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• 6 A 16-year-old girl comes to the physician for a routine examination. She is wearing a sweater despite the
. 7 warm weather and says that she often feels cold. She also complains of difficulty with weight loss_ The
• 8 patient wants to lose about 4.5 kg (10 Ib) because she believes she is "enormous." For the past year, she
• 9 has been on a vegetarian diet because meat makes her feel bloated. Menarche was at age 12 and her last
• 10 menstrual period was 2 months ago. The patient is an honors student and involved in ballet and
• 11 cross-country running. Her mother has Graves' disease. Temperature is 36_2 C (97.2 F), blood pressure is
• 12 88/58 mm Hg, pulse is 42/min, and respirations are 16/min. Her body mass index is 15 kg/m2. The patient is
• 13 alert and cooperative but appears tired. Her skin is dry with lanugo. Laboratory results are as follows:
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Serum chemistry
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Sodium 136 mEdiL
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Potassium 2.9 mEq/L
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Chloride 102 mEq/L
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Bicarbonate 24 mEq/L
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Blood urea nitrogen 18 mg/dL
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Creatinine mg/dL
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Calcium 8 mg/dL
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Glucose 78 mg/dL
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Magnesium 2.3 mg/dL
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Phosphorus 2 mg/dL
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Thyroid function tests
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Thyroid-stimulating hormone 4.5 pU/mL
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Free T4 ng/dL
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Triiodothyronine (T3) 65 ngfdL
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Urine human chorionic gonadotropin Negative
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What is the most appropriate next step in management of this patient's symptoms?
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• 36 A. Administer bupropion
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• B. Administer fluoxetine
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• 39 • C. Administer levothyroxine
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• 4 menstrual period was 2 months ago. The patient is an honors student and involved in ballet and
• 5 cross-country running. Her mother has Graves' disease. Temperature is 36_2 C (972 F), blood pressure is
• 6 88/58 mm Hg, pulse is 42/min, and respirations are 16/min. Her body mass index is 15 kg/m2. The patient is
• 7 alert and cooperative but appears tired. Her skin is dry with lanugo. Laboratory results are as follows:
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Serum chemistry
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Sodium 136 mEq/L
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Potassium 2.9 mEq/L
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Chloride 102 mEq/L
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Bicarbonate 24 mEq/L
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Blood urea nitrogen 18 mg/dL
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Creatinine 0.6 mgldL
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Calcium 8 mg/dL
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Glucose 78 mg/dL
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Magnesium 2.3 mgfdL
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Phosphorus 2 mg/dL
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Thyroid function tests
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Thyroid-stimulating hormone 4.5 pU/mL
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Free T4 GA ng/dL
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Triiodothyronine (T3) 65 ng/dL
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Urine human chorionic gonadotropin Negative
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What is the most appropriate next step in management of this patient's symptoms?
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• 30 O A. Administer bupropion
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0 B. Administer fluoxetine
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O C. Administer levothyroxine
• 34 • D. Administer olanzapine
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• E Hospitalization
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O F. Order serum 25-hydroxy-vitamin
• 38 O G. Order serum cobalamin
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O H. Refer for cognitive behavioral therapy, with follow-up in 2 weeks
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6 A 4-year-old boy is brought to the physician for evaluation of speech that is "difficult to understand." His
▪ 7 parents state that "he doesn't seem to listen" and describe him as a slow learner who only uses single words
▪ 8 and frequently mumbles. The boy prefers to play by himself and is obsessed with building towers with
▪ 9 blocks_ He becomes extremely distressed if prevented from performing this activity. He was born at term
• 10 from an uncomplicated pregnancy and delivery, and his physical growth has been normal. His older brother
• 11 was aggressive as a young child and diagnosed with attention deficit hyperactivity disorder. In the office
• 12 waiting room, the boy plays quietly with blocks and seems oblivious to the other children. When his mother
• 13 calls him to come to her, he turns toward her once and then resumes playing. His physical examination and
• 14 hearing are normal. Which of the following is the most appropriate statement to tell his parents?
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• 17 • A. "He most likely has a developmental language disorder and would benefit from speech therapy."
• 18 • B. "His behavior is most likely due to the inattentive subtype of attention deficit hyperactivity disorder."
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• 20 • C. "I am concerned that his difficulties may indicate intellectual disability.'
• 21 • D. "I understand your concerns; a brain MRI will help establish a definitive diagnosis."
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• 23 E "I understand your concerns; a comprehensive evaluation for autism spectrum disorder will be
• 24 ".•-1 helpful."
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▪ 6 A 12-year-old boy is accused of setting his neighbor's house on fire. His parents say, 'We can't believe he
▪ 7 would do anything like this; he has always been hyperactive but is basically a good kid_ The past year he has
▪ 8 been a little more irritable and argumentative with us, but that's just typical of preteens." They add that he has
▪ 9 wanted to be a firefighter since a young age and spends hours on the internet watching videos about fires and
• 10 firefighting_ The boy's grades are average, and his teachers describe him as a loner who has difficulty making
• 11 friends at school_ Two years ago he was caught setting the interior of his father's car on fire_ The boy has
• 12 also been linked to several suspicious fires in the neighborhood, although no criminal charges have been
• 13 brought against him. Based on this information, which of the following is the most likely diagnosis?
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• 16 • A. Antisocial personality disorder
• 17 • B. Attention deficit hyperactivity disorder
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• C. Conduct disorder
• 20 • D. Normal developmental experimentation
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• E Oppositional defiant disorder
• 23 C. F. Pyromania
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5 Bilateral lower extremities have nonblanching palpable purplish lesions below the knee and mild swelling of the 6 feet.500/pL • 14 • 15 Urinalysis • 16 Specific gravity 1. Bowel sounds are normal. there is no rebound tenderness or guarding.000/p L • 13 Leukocytes 7.025 • 17 pH TO • 18 Protein +1 • 19 Blood Moderate • 20 Glucose Negative • 21 Ketones Negative • 22 Leukocyte esterase Negative • 23 Nitrites Negative • 24 Bacteria None • 25 White blood cells 1-2/hpf • 26 Red blood cells 20-30/hpf • 27 Casts None • 28 Crystals None • 29 • 30 Which of the following is the most likely cause of this child's skin manifestations? • 31 • 32 • 33 • A. Clotting factor deficiency • 36 • 37 • ID. Nutritional deficiency • 38 • E Vasculitis • 39 • Viral infection • 40 • 41 • 42 0 Feedback Suspend End Block . Bone marrow infiltration • 35 • C. 1 2 Item: 4 of 44 F' Mark 3 Previous Next Lab Values. 4 tenderness to deep palpation. Notes Calculator. The remainder of the examination is normal_ 7 ▪ 8 Laboratory results are as follows: ▪ 9 Complete blood count • 10 Hemoglobin 13. Bacterial infection • 34 • B.0 WI:IL • 11 Hematocrit 40% • 12 Platelets 140.

and then suddenly became ill. thrombocytopenia and renal insufficiency_ What is the most likely cause of the patient's ▪ 9 symptoms? • 10 • 11 • A. 4 A I D-year-old boy is brought to the emergency department due to abdominal pain and bloody diarrhea. 1 2 Item: 5 of 44 F' Mark 3 Previous Next Lab Values. Escherichia coli • 13 • 14 • C. Physical examination shows a ▪ 7 pale and jaundiced child_ There is diffuse abdominal tenderness and 2+ pedal edema_ Laboratory studies ▪ 8 show anemia. Campylobacter jejuni • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . The mother says that he was "fine" a few days ago. Crohn's disease • 15 • D. Salmonella poisoning • 18 • F. Lactose intolerance • 16 • 17 • E. Notes Calculator. Vibrio cholera • 12 • B.

• 10 Which of the following is the most likely cause of this patient's current complaints? • 11 • 12 • 13 • A. Ten days ▪ 8 later. 1 2 Item: 6 of 44 F' Mark 3 Previous Next Lab Values. Notes Calculator. the patient presents again with fever. Small tender lymph nodes are palpated in the cervical region_ The symptoms subside quickly on penicillin therapy. Drug-induced reaction • 15 • 16 • C. Lymphoproliferative disorder • 17 • Henoch-Schonlein purpura • 18 • 19 O E Infective endocarditis • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . skin rash and fleeting joint pain in the lower extremities. 4 A 9-year-old Caucasian male complains of fever. sore throat and difficulty swallowing. Rheumatic fever • 14 O B. axillary and inguinal regions. Physical ▪ 9 examination reveals scattered urticaria and palpable lymph nodes in the cervical.

Posterior urethral valves • 20 • 21 O ID. Unilateral renal agenesis • 25 O G. Examination shows mild • 11 bilateral lower-extremity edema but no other abnormalities_ Urinalysis shows mild proteinuria but no white • 12 blood cells or bacteria. A 5-year-old girl with chronic renal insufficiency is brought to the physician for a follow-up visit Since birth. Which of the following is the most likely predisposing factor for this patient's • 14 recurrent infections? • 15 • 16 • 17 • A. Notes Calculator. Item: 7 of 44 F' Mark Previous Next Lab Values. Neurogenic bladder • 19 • C. Vesicoureteral reflux • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . Polycystic kidney disease • 22 • E Recurrent sexual abuse • 23 • 24 O F. The girl has since lived with her grandmother. Her mother lost custody of the girl 3 years ago due to failure to comply with recommended treatment • 10 and prophylaxis of her infections. Renal scintigraphy with dimercaptosuccinic acid shows bilateral focal parenchymal • 13 scarring and blunted calyces. for which she takes trimethoprim-sulfamethoxazole daily for prophylaxis. She has a history of poor growth and mild hypertension but is otherwise developmentally 9 normal. she has had multiple episodes of urinary tract infections. Common variable immunodeficiency • 18 • B.

She feels well 7 and has no concerns. Echocardiography • 19 • ID. Urine toxicology screen • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 ▪ 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . What is the best next step in the evaluation of this patient? • 13 • 14 • 15 • A. Fasting lipid panel • 20 • 21 • E Urine culture • 22 • F. 4- 5 6 A healthy 15-year-old girl comes to the physician for a routine health maintenance examination. She is an honors student in 1 lath grade and plays varsity soccer. Complete blood count • 17 • 18 • C. Her menstrual cycles are regular and last 3-4 days. 9 She has no vaginal discharge or pain. 1 2 Item: 8 of 44 F' Mark 3 Previous Next Lab Values. her last menses was 1 week ago_ 8 The patient has been sexually active with one partner for the past year and takes oral contraceptive pills daily. The patient's body mass index is 23 kgim2. The • 10 patient has tried marijuana ''a few times" but does not use tobacco or alcohol_ Her parents are healthy_ Her • 11 maternal grandfather died of a myocardial infarction at age 68. • 12 Vital signs and physical examination are normal. Notes Calculator. Chtarnydia trachornatis testing • 16 • B.

Attempt nasogastric tube placement • 20 • 21 • E Attempt peripheral intravenous line placement • 22 • F. Notes Calculator. blood pressure is 60/28 mm Hg. On examination. Attempt intraosseous cannulation • 19 • ID. Her capillary refill time is 5 seconds centrally_ Despite numerous attempts. Her temperature is 397 C (1113_5 F). and pulse is 145/min. it is not • 11 possible to start a peripheral intravenous line. • 4- • 5 • 6 A 3-year-old girl is brought to the emergency department with lethargy and fever_ She had diarrhea for several • 7 days. Transport to intensive care unit for central venous catheter placement • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 41 42 0 Feedback Suspend End Block . The girl has refused liquids for the past 12 hours and has not urinated today. she is lethargic • 10 and has poor skin turgor.• 1 • 2 Item: 9 of 44 V. She has no allergies and takes no medications. Which of the • 12 following is the best next step in management of this patient? • 13 • 14 • 15 • A. and the child's condition continues to deteriorate.Mark • 3 Previous Next Lab Values. Attempt arterial line placement • 16 • R. and her parents say that "she suddenly took a turn for the worse". Attempt central venous catheter placement • 17 • 18 • C.

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6 A 5-year-old boy is brought to the physician after he was hit in the head with a baseball_ The incident occurred
7 about two hours ago_ He had no loss of consciousness, but was a little dazed after being hit However, he
8 seemed to improve after a short time. About 30 minutes ago, the patient began to complain of a headache,
vomited twice, and is currently not acting right according to his parents_ On examination, the child is sleepy
and his left pupil is larger than his right A CT scan of his head is shown below_
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Which of the following is the most appropriate next step in management?
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• 34 • A. Cerebral angiogram
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• B. MRI of the brain
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• C. Repeat CT in 24 hours
• 38 .0 ID. IDexamethasone
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• E Emergent craniotomy
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• 2 Item: 11 of 44 V/lark
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▪ 5
▪ 6 A 12-year-old boy is brought to the pediatrician by his mother for a routine examination. The boy has an
▪ 7 eight-year history of episodes in which he utters strange sounds, clears his throat, grimaces, and blinks his
▪ 8 eyes. Recently he has also started to jerk his head during these episodes as well. His medical history is
• 9 otherwise unremarkable and he is earning passing grades in school. Physical examination is unremarkable_
• 10 Which of the following disorders is most likely to develop in this boy?
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• 13 • A. Conduct disorder
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• B. Obsessive-compulsive disorder
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• C. Oppositional defiant disorder
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• ID. Narcissistic personality disorder
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• 19 • E. Antisocial personality disorder
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▪ 5

A 2-year-old boy with cough and difficulty breathing is brought to the emergency department by his mother_
▪ 7 She says that he was well and playing with his toys until 2 hours prior to presentation. He is healthy, but his
▪ 8 6-year old brother has a peanut allergy. The patient's temperature is 36.7 C (98 F), blood pressure is 92148
mm Hg, pulse is 114/min, and respirations are 48/min. The patient's pulse oximetry shows 91% on room air_
• 10 Physical examination shows nasal flaring and grunting with both subcostal and intercostal retractions.
• 11 Wheezing is heard in the right lung field; the left field is clear to auscultation_ No rales or rhonchi are noted.
The remainder of the physical examination is within normal limits. Supplementary oxygen is applied. Chest
x-ray reveals mild hyperinflation of the right lung. Which of the following is the most appropriate next step in
management of this child?

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• A. Bronchoscopy
• 18 • B. Chest computed tomography scan
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• C. Chest physiotherapy
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• 21 • D. Chest tube placement
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• E Intramuscular epinephrine
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• 24 • Nebulized albuterol
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• G. Racemic epinephrine
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and family histories are 9 unremarkable. He is cyanotic and short of breath 7 when he breastfeeds but turns pink when he cries. Vital signs are normal and 4-extremity blood • 10 M pressures are equal. Tetralogy of Fallot • 19 • ID. His prenatal. His weight is average for gestational age. Tracheoesophageal fistula with esophageal atresia • 20 • 21 • E Transient tachypnea of the newborn • 22 • F. Examination shows a non-dysmorphic boy with clear lungs and no murmurs_ There are : h 11 2 no intercostal retractions or nasal flaring. Vocal cord paralysis • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 I • 39 • 40 • 41 • 42 411 0 Feedback Suspend End Block A . Notes Calculator.• 1 • 2 Item: 13 of 44 V-• Mark 3 Previous Next Lab Values. He is voiding normally and passed meconium. Which of the following is the most likely diagnosis? • 14 • 15 • A. Choanal atresia • 115'1 • 17 • 18 I • Laryngomalacia • C. 4 I 5 6 7 A 1-day-old boy is in the newborn nursery with persistent feeding difficulty. 1 . Feeding him expressed breast milk from a bottle results in 8 1 the same findings. His peripheral pulses are full and symmetric. birth.

GGGip L • 19 Neutrophils 90% • 20 Lymphocytes 10% • 21 Monocytes 2% • 22 • 23 Nitroblue tetrazolium test is normal. He has a history 8 of numerous sinus infections and episodes of cellulitis. necrotic periodontal infection with • 11 ulceration. Impaired leukocyte adhesion • 37 • Fl.8 WI:IL • 16 'I Hematocrit 36% • 17 Platelets 240.000. Adenosine deaminase deficiency • 29 O 13.1p L • 18 Leukocyte count 36. • 24 • 25 Which of the following defects is most likely present in this patient? • 26 • 27 • 28 O A. with previous cultures isolating Staphylococcus 9 aureus and Pseuciornorras aerugirrosa. Complement deficiency • 30 0 C. the child has a severe. Opsonization defect • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . 5 6 7 A 3-year-old boy is brought to the emergency department because of severe oral pain along with bleeding and irritation of his gums_ His symptoms began 2 weeks ago and have progressively worsened. • 12 • 13 Laboratory results are as follows: Complete blood count • 15 Hemoglobin 11. His mother also notes that his umbilical cord did not fall off until 5 • 10 weeks after birth_ On physical examination. Defective formation of mature 13 lymphocytes • 31 • 32 O ID. Defective formation of mature T lymphocytes • 33 • E Defective intracellular killing • 34 • E Development defect of the fourth pharyngeal pouch • 35 • 36 O G. 3 =1 1 4 Item: 14 of 44 V-• Mark Previous Next Lab Values. Notes Calculator.

Persistent pulmonary hypertension • 24 • 25 • E Respiratory distress syndrome • 26 • Transient tachypnea of the newborn • 27 • 28 • 29 • 30 • 31 • 32 • 33 ▪ 34 • 35 ▪ 36 • 37 ▪ 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . and 8 the mother received glucocorticoids and magnesium sulfate shortly after the onset of labor. Pulse oximetry showed 75% on room air and improves to 90% with 35% Fi02 by sm 3 continuous positive air pressure ventilation. blood pressure is 65/35 mm Hg. Meconium aspiration syndrome • 23 • ID.8 F) under radiant heat. amniotic fluid was clear. A chest x-ray shows diffuse. Membranes 9 ruptured spontaneously 6 hours prior to delivery. pulse is 140imin. intercostal and subcostal • '1 retractions. Notes Calculator. Congenital diaphragmatic hernia • 20 • ID-transposition of the great arteries • 21 • 22 • C. The boy initially emerged vigorous • 10 with spontaneous respirations but rapidly developed respiratory distress_ Birth weight was 1 kg (2 lb 4 oz)_ • 11 His temperature is 36 C (96. 5 6 7 A 3D-minute-old boy is in the neonatal intensive care unit with central cyanosis_ He was born at 28 weeks gestation by vaginal delivery to a 16-year-old girl. and shallow respirations. nasal flaring. reticular granularity and perihilar linear opacities bilaterally. Bilateral breath sounds are diminished. The pregnancy was complicated by premature labor. fine. What is the most likely •14 • 6 I diagnosis in this patient? • -17 • 18 • 19 • A. and • 12 respirations are 70/min. Physical examination shows grunting. 3 =1 1 4 Item: 15 of 44 V-• Mark Previous Next Lab Values.

Complement deficiency • 17 ▪ C.4Gimin. Impaired oxidative metabolism • 15 • B. and productive cough of two days 7 duration. pulse is 1. His mother reports that he just recovered from prolonged diarrhea due to Garda infection_ His past 8 medical history is also significant for pneumonia and recurrent ear infections since 6 months of age. Thymic hypoplasia • • • 13 I 19 ▪ 11 Adenosine deaminase deficiency • E Abnormal B-lymphocyte maturation • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . his temperature is 383' C (1G1 . Notes Calculator. 3 =1 1 4 Item: 16 of 44 V-• Mark Previous Next Lab Values. 5 6 7 An 18-month-old male is brought to the hospital because of fever. • 10 Examination reveals a young child in mild respiratory distress and bronchial breath sounds in the right lower • 11 lung lobe_ Which of the following is the most likely cause of his recurrent infections? • 12 in • 13 • 14 • A. On 9 physical examination. dyspnea.1O F). and respirations are 40/min.

Karyotyping shows 45 X0_ Which of the following is she most at risk of developing? 9 • 10 • 11 • A. Mitral valve prolapse • 13 • 14 • C. She is at 8th percentile for height and 30th percentile for 7 weight Vital signs are within normal limits. Mental retardation • 15 • ID. 1 2 Item: 17 of 44 V. Notes Calculator. Bipolar disorder • E Breast cancer • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . Osteoporosis • 12 • B. widely 8 spaced nipples.Mark 3 Previous Next Lab Values. Examination shows a high arched palate and inverted. 4- 5 6 An 8-year-old girl is being evaluated for short stature.

The right hemi-scrotum is • 11 slightly swollen and tender to palpation. On 9 examination. 1 2 Item: 18 of 44 V-• Mark 3 Previous Next Lab Values. Notes Calculator.0 • 15 Protein +1 • 16 'I Blood Moderate • 17 Glucose Negative i Ketones Negative • 19 Leukocyte esterase Negative •• 20 Nitrites Negative • 21 • 22 • 23 The boy is admitted to the hospital for further management Which of the following adverse outcomes is he • 24 most likely to develop? • 25 • 26 • A. Mucous membranes are dry and his capillary refill is 3 • 10 seconds. Intussusception • 31 • 32 • E Meckel diverticulum • 33 • F. He has a palpable nonblanching rash on his buttocks and lower legs. Cholecystitis • 28 • 29 • C.016 • 14 pH 7. Appendicitis • 27 • B. His 7 father noticed the swelling today and is sure that "it wasn't there yesterday. Diverticulitis • 30 • D." The boy had a low-grade fever 8 1 and cough 2 weeks ago and "hasn't been himself ever since. 4 5 6 7 A 5-year-old boy is brought to the emergency department by his father with fatigue and scrotal swelling." He has no significant past medical history. the patient looks ill and lethargic. Volvulus • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . Abdominal examination shows mild diffuse tenderness to palpation • 12 without rebound or guarding_ Laboratory results from urinalysis are as follows: 1113 1= Specific gravity 1.

blood pressure is 78/40 mm Hg. Pseudornonas aerugirrosa • 31 O E Salmonella eritentocfis • 32 • 33 O F. pulse is • 11 140.0001pL • 17 Leukocytes 18.• 1 • 2 Item: 19 of 44 V-• Mark ▪ 3 Previous Next Lab Values.imin. The boy was hospitalized during infancy for dactylitis and last summer for splenic sequestration ▪ 9 crisis_ Other than ibuprofen and acetaminophen for fever and pain. • 27 O B. and respirations are 22/min_ Examination shows a lethargic boy with mild pallor. Streptococcus pneurnoniae • 35 • 36 • 37 • 30 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . Escherichia col?. Laboratory results • 12 are as follows: • 13 Complete blood count • 14 Hemoglobin 8.2 g/dL • 15 Hematocrit 24% • 16 Platelets 325. His temperature is 3E4 C (103 F). ▪ 7 He is exhausted and has chills and night sweats. Nesseria rnenwrgibares • 30 O D. Notes Calculator. Haernophdus influerrzae type B • 28 • 29 O C. However. he takes no medications_ His • 10 vaccinations are up to date. he has had no bone pain. cough. or difficulty ▪ 8 breathing.800IpL • 18 Neutrophils 80% Band 6% • 20 Gram stain Pending • 21 Blood culture Pending • 22 • 23 • 24 Which of the following organisms is the most likely cause of this patient's condition? • 25 • 26 a A. • 4- ▪ 5 ▪ 6 A 3-year-old African American boy with sickle cell anemia is brought to the emergency department for fever. Staphylococcus aureus • 34 O G.

malaise. • 10 The wound was rinsed with water and has since healed_ The girl takes no medications. Spider • 22 • 23 • E. Raccoon • 19 • 20 • C. • 4 ▪ 5 ▪ 6 A previously healthy 5-year-old girl is brought to the emergency department with difficulty swallowing. • 12 Examination shows an agitated. Notes Calculator. The girl sustained a bite to her right forearm when she and her ▪ 9 family went camping in Delaware last month_ Family members are unsure of the bite source as it was dark. Mosquito • 18 • B. which • 14 is the most likely source of her symptoms? • 15 • 16 • 17 • A. muscle aches. A well-healed scar is seen on her right forearm_ Of the following disease reservoirs. and sore throat She now refuses to drink fluids ▪ 8 and spits out any water that is given to her. she has had fever. disoriented girl with copious drool and facial grimacing. Rat • 21 • ID. has no allergies. chills. and • 11 is up to date on standard vaccinations_ There is no history of foreign travel_ Vital signs are normal.• 1 • 2 Item: 20 of 44 F' Mark ▪ 3 Previous Next Lab Values. Squirrel • 24 • Tick • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . The neck has full • 13 range of motion. For the ▪ 7 past week.

What is the most likely organism causing this patient's symptoms? • 13 • 14 • 15 • A. Group B Streptococcus • 19 • Haemophilus inffuenzae • 20 I 21 • E Herpes simplex virus • 22 • Listeria monocytogenes • 23 • 24 • G. • 4- ▪ 5 ▪ 6 An 18-month-old child is brought to the emergency department by his mother with fever. and ▪ 7 lethargy_ He developed a fever during the day that initially responded to treatment with ▪ 8 acetaminophen. vomiting. On examination he is drowsy and lethargic_ He flexes his hips when his neck is • 11 flexed. Throughout the day. Borretia burgdorferi • 16 • B. he became increasingly lethargic and developed a rash on his lower ▪ 9 extremities that has acutely worsened during the past few hours. He also has an erythematous. Cytomegaroviras • 17 • 1a • C. Neisseria meningitides • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . nonblanching pinpoint rash on his trunk and bilateral lower • 12 extremities. Notes Calculator. The child's immunizations are up to date • 10 and he is otherwise healthy.• 1 • 2 Item: 21 of 44 V-• Mark ▪ 3 Previous Next Lab Values.

The pertinent physical findings are pallor. He was born at 32 weeks of • 7 gestation with a birth weight of 12GG g. Sickle cell anemia . The laboratory studies are as follows: • 9 Hb 7 WI:IL • 10 WBC 7. Hemolytic disease of newborn • 23 • ID.• 1 • 2 Item: 22 of 44 V-Mark • 3 Previous Next Lab Values. Beta thalassemia • 21 • 22 • C.DDD!cmm • 11 Platelets 230. 25 • 26 Iron deficiency • 27 • G.000/cl-rim • 12 Reticulocytes Low • 13 • 14 • 15 The peripheral smear shows normocytic normochromic RBC. tachycardia. • 4- • 5 • 6 A 2-month-old infant is brought to the clinic for the evaluation of poor feeding. 24 • E Anemia of prematurity . Notes Calculator. Vitamin B12 deficiency • 28 • H. Alpha thalassemia • 20 • B. Pancytopenia • 29 • 30 • G-6-PD deficiency • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 1 11 0 Feedback Suspend End Block . and • 8 flow murmurs. tachypnea. What is the most likely diagnosis? • 16 • • 17 • 19 • A.

Vitamin 13 12 supplementation • 22 • G. At this time. The mother • 10 eats a balanced diet that includes meat products.Mark 3 Previous Next Lab Values. which of the following is the most appropriate • 11 nutritional intervention for this patient? • 12 • 13 • 14 • A. Introduction of pureed vegetables to the diet • 18 • 11 Iron and vitamin ID supplementation • 19 • 20 • E No additional supplementation needed 2= -1 • F. Notes Calculator. She was born at 34 weeks gestation and weighed 2_2 kg (4 lb 14 oz) at birth. Her physical examination is notable for mild pallor but is otherwise normal. Introduction of fruit juices to the diet • 16 • 17 C. 4 5 6 A 1G-week-old girl is brought to the office by her mother for a well-infant examination after missing her visit 2 7 weeks earlier. Vitamin C supplementation • 24 • 25 • 26 I • 27 • 28 I • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . and her growth and development are appropriate for her chronological and 9 gestational ages.• 1 • 2 Item: 23 of 44 V. She has been breastfed exclusively since then. Introduction of eggs and meat to the diet • 15 • B.

Lennox-Gastaut syndrome • 18 • 19 • 20 • 21 • 22 4im • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 41 42 0 Feedback Suspend End Block . Juvenile myoclonic epilepsy • 16 • 17 • E. During these episodes. EEG performed 9 before and after hyperventilation is normal_ Which of the following is the most likely diagnosis? • 10 • 11 • A. Complex partial seizure • 12 ▪ R. and produces lip-smacking 7 movements.11 V-• Mark 3 Previous Next Lab Values. he is unresponsive to verbal or tactile stimuli. Typical absence seizure • 13 • 14 • C. Notes Calculator. He has 8 no family history of any seizure disorder. 4- 5 A previously healthy 8-year-old boy is brought to the office by his mother because he has multiple staring 6 episodes. His neurological examination is unremarkable. after which he remains confused for some time. Atypical absence seizure • 15 ▪ ID. Each episode lasts for a few minutes. 1 2 Item: 24 of 44 .

Examination shows no other ▪ 9 abnormalities. • 4- ▪ 5 ▪ 6 A 15-year-old girl is brought to the physician by her mother because she has not begun menstruating_ She is ▪ 7 otherwise healthy and has no medical problems_ Vitals signs are within normal limits.Mark ▪ 3 Previous Next Lab Values. Kar-yotvping • 18 • 19 • E GnRH stimulation test • 20 O F. Serum FSH level • 17 O ID. Notes Calculator. Which of the following is the most appropriate • 10 next step in management? • 11 • 12 • 13 O A. MRI of pituitary • 15 • 16 O C. Physical examination ▪ 8 shows absence of breast development and no pubic hair is seen. Reassurance • 21 • 22 • 23 • 24 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 42 0 Feedback Suspend End Block . Ultrasound confirms the presence of a uterus.• 1 • 2 Item: 25 of 44 V. Estrogen level • 14 • B.

he has 8 become increasingly sleepy with significantly decreased oral intake. and increased sleepiness_ 7 He has had a fever for 3 days despite taking acetaminophen and ibuprofen_ Over the past day. Intravenous antibiotics followed by head CT scan • 18 • 19 • C. The anterior fontanelle is soft and full. and reactive to light There are no focal neurologic deficits_ A complete blood • 12 count and blood culture are pending. Which of the following are the most appropriate next steps in the • 13 management of this patient? • 14 • 15 • 16 • A. Head CT scan followed by lumbar puncture • 17 • B. Pupils are equal. His temperature is 40 C (104 F). round. vomiting. blood 9 pressure is 92/48 mm Hg. The infant is lethargic but cries when attempts are made to flex his • 11 head. 4 5 6 An 8-month-old boy is brought to the emergency department with fever. Intravenous antibiotics followed by hospital admission • 20 • D. Notes Calculator. Intravenous antibiotics followed by lumbar puncture • 21 3= 2. pulse is 120/min.2 • E Lumbar puncture followed by intravenous antibiotics • 23 • 24 • 25 •s • 27 • 28 I • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . and respirations are 30/min_ Pulse oximetry shows 96% on room • 10 air.• 1 • 2 Item: 26 of 44 F' Mark 3 Previous Next Lab Values.

The patient reports that his right knee and right ankle have swelled 9 several times before_ He has a history of easy bruising since childhood and had an episode of excessive • 10 bleeding after a tooth extraction. He has no history 1 of fever. Avascular necrosis • 15 • B.• 1 • 2 Item: 27 of 44 F Mark 3 Previous Next Lab Values. and the pain has gradually worsened since then. His uncle had similar problems_ Which of the following is the most likely • 11 cause of this patient's joint pain? • 12 • 13 • 14 • A. Hemosiderin deposition and fibrosis • 18 • 11 Immunologic tissue injury • 19 • 20 • E Occult traumatic injury • 21 • Repetitive microtrauma 22 ffmi • 23 • G. Vector-borne illness • 24 • 25 • 26 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . 4 5 6 A 16-year-old boy is brought to the pediatrician with pain and limited motion of the right knee. Notes Calculator. local erythema. Crystal deposition • 16 • 17 C. He first noticed 7 knee pain and swelling 6 months earlier. or trauma.

The most likely cause of the • 12 newborn's symptoms is infection with which of the following organisms? • 13 • 14 • 15 • A. 25 . Toxopfasma gorrarii • 20 • 21 • E Treponerna palfichlin • 22 • Varicella zoster virus • 23 • 24 • 25 . and head circumference are <5th percentile.• 1 • 2 Item: 28 of 44 V. Rubella virus • 19 • D. Physical examination • 10 shows bilateral white pupillary reflexes. His mother traveled to Egypt during ▪ 8 the first trimester of pregnancy and developed a brief febrile illness but otherwise felt well during the ▪ 9 pregnancy. Herpes simplex virus • 17 • 18 • C.Mark ▪ 3 Previous Next Lab Values. Auscultation shows a • 11 continuous systolic-diastolic murmur over the left 2nd intercostal space. The boy does not alert to voice or sound. Cytomegalovirus • 16 • B. 27 m 28 • 29 • 30 • 31 • 32 33 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 42 0 Feedback Suspend End Block . length. The infant's weight. • 4- ▪ 5 ▪ 6 A 4-day-old boy in the newborn nursery has failed a bilateral hearing screening_ He was born at 37 weeks ▪ 7 gestation by elective cesarean section for intrauterine growth retardation. Notes Calculator.

8° C (101. His temperature is 38. blood pressure is 122/74 mm Hg.• 1 • 2 Item: 29 of 44 V. Notes Calculator. Check serum protein and albumin levels • 16 • 17 C. and myalgia_ He has no other medical problems_ Family history is negative for any kidney ▪ 8 disease. 0 Feedback Suspend End Block .9° F). and ▪ 9 respirations are 15/min. with no further workup • 21 • Renal biopsy • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 41. pyuria. pulse is 95/min.Mark ▪ 3 Previous Next Lab Values. Order 24-hour urinary collection for protein • 18 • 11 Order renal ultrasound • 19 • 20 • E Reassure. Examination shows no abnormalities_ An incidental urine dipstick testing shows 2+ • 10 proteinuria but no hematuria. • 4- ▪ 5 ▪ 6 A 14-year-old boy comes to the physician with two days of fever and nasal discharge_ He has also had ▪ 7 malaise. or active urine sediment Serum creatinine is within normal limits_ • 11 Which of the following is the most appropriate next step in management? • 12 • 13 • 14 • A_ Repeat dipstick testing on two subsequent occasions • 15 • 13. fatigue.

▪ 9 and a cleft palate_ Chest radiograph shows an absent thymus_ • 10 • 11 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 . micrognathia. Lab Values. Notes Calculator. She has low-set ears. 28 ▪ 29 • 30 31 32 33 34 35 36 37 38 39 • 40 se 41 42 0 Feedback Suspend End Block .• Mark ▪ 3 Previous Next .• 1 • 2 Item: 30 of 44 A V. On examination. she is cyanotic and tremulous. • 4- ▪ 5 ▪ 6 A newborn girl is evaluated in the neonatal intensive care unit for cyanosis_ She was born at 36 weeks ▪ 7 gestation by cesarean section for fetal distress_ The girl remained hypoxic despite administration of ▪ 8 supplemental oxygen. 27 .

This patient is at greatest risk for which of the following? • 30 • 31 ▪ A. 28 ▪ 29 Echocardiography confirms truncus arteriosus. 27 .• 1 • 2 Item: 30 of 44 'Mark <=1 ▪ 3 Previous Next Lab Values. Notes Calculator. Duodenal atresia • 32 ▪ B. Hypocalcemia 33 • 34 • C. Hypoglycemia • 35 • D. Myelomeningocele ▪ 38 ▪ Neutropenia • 39 • G. • 4 ▪ 5 ▪ 6 ▪ 7 ▪ 8 ▪ 9 • 10 • 11 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 . Thrombocytopenia • 40 • 41 • 42 0 Feedback Suspend End Block . Infertility • 36 • 37 • E.

Apgar scores were 8 and 9 at 1 and 5 minutes. Chronic rhinosinusitis • 18 • B. Upright abdominal • 11 x-ray shows multiple dilated loops of small bowel with paucity of air in the large intestine and rectum_ Left • 12 lateral decubitus view shows free air above the liver. Hypothyroidism • 21 • D. The colon is • 14 diffusely narrow. Today she has been spitting up all 9 feeds and now has bilious emesis_ Her abdomen is tense and distended. Viscous • 13 meconium is irrigated and evacuated. Early-onset Alzheimer's disease • 19 • 20 • C. Notes Calculator. 4 5 6 A 52-hour-old girl in the newborn nursery was born at 40 weeks gestation to a 30-year-old woman by 7 cesarean delivery for failure to progress. respectively.• 1 • 2 Item: 31 of 44 F' Mark 3 Previous Next Lab Values. Which comorbidity will this patient most likely develop? • 15 • 16 • 17 • A. Infertility from cervical obstruction • 22 • 23 • E Sensorineural hearing loss • 24 • 25 • 26 • 27 • 28 29 • 30 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . The 8 patient has been voiding appropriately but has not yet passed meconium. Rectal examination shows no stool • 10 in the rectal vault Feeds are held and a nasogastric tube is placed for decompression. primarily from the ileum with some from the colon. An emergency laparotomy is performed.

The child has flattened facies.7 C (98 F). Bilateral cryptorchidism • 14 • 15 • B. 9 and respirations are 50/min. Wilms tumor • 24 • 25 • 26 • 27 • 28 • 29 H • 31 16 • 31 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . pulse is 144/min. Pulse oximetry is 84% on room air. Notes Calculator. 4 5 6 A newborn boy is evaluated shortly after vaginal delivery for respiratory distress. His temperature is 36. Duodenal atresia • 18 • D. He was born at 37 weeks 7 gestation to a primigravid mother who had no medical problems.• 1 • 2 Item: 32 of 44 F' Mark 3 Previous Next Lab Values. abdominal distension. Congenital diaphragmatic hernia • 16 • 17 C. Second-trimester prenatal ultrasound 8 showed bilateral hydronephrosis and oligohydramnios. Hypospadias • 19 • 20 • E Posterior urethral valves • 21 • Prostatic hyperplasia • 22 • 23 • G. and clubfeet • 11 What is the most likely underlying diagnosis in this patient? • 12 • 13 • A. Examination shows decreased aeration of • 10 both lungs and intercostal retractions.

Physical examination shows a well-appearing adolescent with olive skin. Apply topical corticosteroids to inflamed skin • 17 • 18 • C. Tender. and light-brown • 11 hair. 1 • 2 Item: 33 of 44 . Notes Calculator.• Mark 3 Previous Next Lab Values. brown eyes. She just returned from a week-long 7 vacation to the Bahamas with her family. Apply sun protection factor 50 sunscreen 30 minutes before sun exposure • 16 • B. There is no edema or vesicles. For the past 2 days. Which of • 12 the following is the most appropriate recommendation? • 13 • 14 • 15 • A. Change to sun protection factor 75 sunscreen • 19 • Continue current sun protection regimen • 20 • 21 • E Form a baseline tan with a tanning bed before sun exposure • 22 • F.11 V. Family history is negative for skin • 10 cancer. the patient has had redness on her face and 8 shoulders despite applying sun protection factor 50 sunscreen just before swimming. blanching erythema is present on the face and arms. This is her first 9 sunburn. Her parents say that "everybody tans easily" in their family. Wear light-colored clothing while outdoors • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . Refrain from outdoor activities on cloudless days • 23 • 24 • G. • 4- 5 6 A 13-year-old girl is brought to the physician for evaluation of sunburn.

Lyses microthrombi in the circulation • 30 • C. He was started on hydroxyurea one year ago and has not had 8 any further pain crises since then. He has had no recent illnesses or hospitalizations. Notes Calculator. Increases fetal hemoglobin • 32 • 33 • E. Chelates iron to prevent iron toxicity • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block .0001mm3 • 18 Leukocyte count 9500/mm3 • 19 Neutrophils 56% • 20 Eosinophils 3% • 21 Lymphocytes 36% • 22 Monocytes 5% • 23 • 24 Which of the following best describes the role of hydroxyurea in the treatment of patients with sickle cell • 25 disease? • 26 • 27 • A. His physical 9 examination is unremarkable_ The patient's laboratory results are shown below_ • 10 Complete blood count • 11 Hemoglobin 9. Removes sickled red blood cells from the circulation • 28 • 29 • B. 1 • 2 Item: 34 of 44 F' Mark 3 Previous Next Lab Values. • 4- 5 6 A 10-year-old patient with sickle cell disease comes to the physician for a routine visit The patient has a 7 history of multiple pain crises and pneumonias.0 gIL • 12 Erythrocyte count 2_2 mlnimm • 13 • 14 MCHC 32% • 15 MCV 105 fl • 16 Reticulocytes 2_0% • 17 Platelets 212. Protects against encapsulated bacterial infections • 31 • D.

Aldosterone • 27 • B. Her blood ▪ 9 pressure is 82/55 mm Hg. heart rate is 11 /min. Cortisol • 28 • 29 • C. Insulin • 31 • E Triiodothyronine • 32 • 33 • 34 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . weight loss. and syncope. Glucagon • 30 • D. Her body mass index is 14 • 10 kg/m2. Notes Calculator. On the second day of admission. She is started on ▪ 7 nasogastric feeds and her activities are restricted. and respirations are 22/min. the patient complains of ▪ 8 severe shortness of breath and has recurrent episodes of nonsustained ventricular tachycardia. • 4- ▪ 5 ▪ 6 A 17-year-old girl is admitted to the hospital for purging behavior. Physical examination shows an emaciated girl with bibasilar crackles and jugular venous distension_ • 11 Serum chemistry results are as follows: • 12 Sodium 138 mEq/L • 13 Potassium 2_1 mEq/L • 14 Chloride 92 mEq!L • 15 Bicarbonate 28 mEq!L • 16 Blood urea nitrogen 14 mg/dL • 17 Creatinine 0_8 mg/dL • 18 Calcium 8 mg/dL • 19 Glucose 90 mg/dL • 20 Magnesium 1 mg/dL • 21 Phosphorus 0_9 mg/dL • 22 • 23 • 24 A surge in which of the following hormones best explains this patient's deterioration? • 25 • 26 • A.Mark ▪ 3 Previous Next Lab Values.• 1 • 2 Item: 35 of 44 V.

He has been playing at the park daily and has had ▪ 9 occasional insect bites_ Vital signs are normal.P ▪ 3 Previous Next Lab Values. A few days ago. Some lesions are covered with a thick golden-yellow crust The rest of his • 11 examination is within normal limits_ What is the most appropriate next step in the management of this patient? • 12 • 13 • 14 • A. The bumps became painful as they filled with fluid. The patient has no other ▪ 8 medical problems and his vaccinations are up to date. Swab intact skin for culture • • 19 • 20 • E Topical mupirocin • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 35 I 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . and chin.• 1 • 2 Item: 36 of 44 F' Mark U. Oral cephalexin • 18 ID. Anti-streptolysin 0 antibody titers • 15 • B. Express pus for culture • 16 • 17 C. Physical examination shows multiple pustules on his right • 10 cheek. Notes Calculator. nose. • 4- ▪ 5 ▪ 6 A 4-year-old boy is brought to physician for evaluation of a rash. the boy had a few small red ▪ 7 bumps on his chin and cheek.

Tympanocentesis and culture • 23 C.9 C (102 F). Ototopical antibiotics • 21 • 22 • E.11M Mark ▪ 3 Previous Next Lab Values.• 1 • 2 Item: 37 of 44 . Acetaminophen. erythematous bilateral tympanic membranes with • 11 decreased mobility on air compression. F. For the past few days. Her • 10 temperature is 38. • 4- ▪ 5 ▪ 6 An 11-month-old girl is brought to the physician for fever. She attends ▪ 9 day care and her diet consists of infant formula and finger foods. she has had rhinorrhea and ▪ 7 nasal congestion. Notes Calculator. Myringotomy with tympanostomy tube placement • 18 • 19 • C. follow-up in 2 days • 17 • B. The girl has no medical problems and takes no medications. Otoscopy shows bulging. Oral antibiotics • 20 • ID. Her hearing is intact and • 12 the rest of her examination is normal_ Which of the following is the most appropriate next step in • 13 management of this patient? • 14 • 15 • 16 • A. The fever started 2 days ago and the girl has since been pulling at both ears. Viral nasopharyngeal polymerise chain reaction testing • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 a 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . The ear pain ▪ 8 seems worse when she lies down. Crusted rhinorrhea is present at the nares. Both parents smoke cigarettes.

39 .• 1 • 2 Item: 38 of 44 V-• Mark ▪ 3 Previous Next Lab Values. Pseudomorras species • 16 • 17 ▪ C. He has ▪ 7 sickle cell disease and has been hospitalized previously for sickle cell crisis_ Vital signs are notable for ▪ 8 persistent fever. Which of the following organisms is the most likely cause of • 11 his condition? • 12 • 13 • 14 • A. Streptocomis pneumoniae • 19 • 20 • E Group B streptococcus • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 ▪ 35 ▪ 36 . Notes Calculator. Escherichia cob. 37 • 38 . Examination of the left lower extremity reveals a normal knee joint with marked tenderness ▪ 9 and swelling over the proximal tibia. Salmonella species • 18 ▪ D. • 4- ▪ 5 ▪ 6 A 12-year-old boy is brought to the emergency department because of severe pain near his left knee. 40 ▪ 41 42 0 Feedback Suspend End Block . • 15 • B. Laboratory studies show leukocytosis and elevated ESR_ Imaging • 10 studies confirm the diagnosis of osteomyelitis.

Today she developed a "pink rash" on her face that spread rapidly to her trunk and ▪ 9 extremities.• 1 I Item: 39 of 44 • 2 F' Mark ▪ 3 Previous Next Lab Values. Physical examination shows a cooperative girl with bilateral nonexudative • 13 conjunctivitis and patchy erythema on the soft palate. She lives in Poland and arrived in the United States last ▪ 7 week to visit her family. pulse is 88/min. She completed an antibiotic course for a "throat • 10 infection" 2 months ago but has otherwise been healthy. Small. Review of systems is otherwise negative. • 4- ▪ 5 ▪ 6 A 4-year-old girl is brought to the physician for a rash. 0 I Feedback. posterior auricular. Suspend End Block . She has received no vaccinations and takes no • 11 medications. Notes Calculator. Her temperature is 38. tender lymph nodes are palpable in the • 14 suboccipital. and posterior cervical areas_ The nontender rash is shown below_ • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 se 11. The child was in good health until 3 days ago.3 C(101 F). and • 12 respirations are 181min. blood pressure is 1GG/50 mm Hg. when she developed nasal congestion ▪ 8 and a low-grade fever.

• 4. Small. posterior auricular. tender lymph nodes are palpable in the 6 suboccipital. and posterior cervical areas_ The nontender rash is shown below. respirations are 181min_ Physical examination shows a cooperative girl with bilateral nonexudative 5 conjunctivitis and patchy erythema on the soft palate. 1 • 2 Item: 39 of 44 V/lark 3 Previous Next Lab Values. ▪ 7 ▪ 8 ▪ 9 • 10 • 11 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 35 36 37 38 se 0 Feedback Suspend End Block . Notes Calculator.

rhich of the following is the most likely cause of this patient's condition? 111 0 Suspend End Block Feedback . • 4- ▪ 5 ▪ 6 ▪ 7 ▪ 8 ▪ 9 • 10 • 11 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 The remainder of the examination is normal. ..Mark U.• 1 • 2 Item: 39 of 44 V.P ▪ 3 Previous Next Lab Values.1.. Notes Calculator.

▪ 3 . El70 • 2 Mark ' Previous Next Lab Values Notes Calculator. Mumps • 36 • F. Kawasaki disease • 33 • 34 0 D. Scarlet fever • 39 O H. Measles • 35 O E. Varicella • 40 • 41 0 • 42 Feedback Suspend End Block A'7 . 32 O C.• 1 Item: 39 of 44 I -4(1 I>. Rubella • 37 • 3-8 O G. Erythema multiforme • 31 • B. Exanthematous drug eruption . 4 ▪ 5 6 7 8 ▪ 9 • 10 • 11 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 The remainder of the examination is normal Which of the following is the most likely cause of this patient's • 27 condition? • 28 • 29 • 30 0 A.

• 4- ▪ 5 ▪ 6 A 1-year-old girl is brought to the emergency department because she is not moving her right arm. The girl lives with her father and her mother. Closed reduction and casting of the right arm • 18 • C. Notes Calculator.• 1 I Item: 40 of 44 • 2 F' Mark ▪ 3 Previous Next Lab Values. No bruises or other abnormalities are seen_ Which of the following is the • 12 most appropriate next step in management of this patient? • 13 • 14 • 15 • A. Contact the child protection agency • 16 • 17 B. who is currently out of town_ Physical • 10 examination shows a well-nourished child who holds her right arm in pronation against the chest She avoids • 11 any movement of her right arm. Gently hyperpronate the right forearm • 19 • 20 • D. He was swinging her by the forearms when ▪ 8 she suddenly started crying and refused to use her right arm. Her father ▪ 7 says that the girl acted normally when she awoke this morning. Order a complete skeletal survey • 21 • E Order an x-ray of the right arm • 22 • 23 • Refer the child to an orthopedic surgeon • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 41 42 0 Feedback Suspend End Block A'S . He has swung her by the arms previously and it ▪ 9 usually makes her laugh.

5° F (39.45% saline • 29 • 30 • C. His abdominal exam is unremarkable. He has lost three pounds (1.1' C). On examination. 0. 5% albumin • 32 • E Packed red blood cells • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 42 0 Feedback Suspend End Block . but has had two to 8 three episodes of diarrhea with each bottle.9% saline • 28 • B. Capillary • 13 refill is four seconds.0 mg/dL • 22 Blood glucose 98 mg/dL • 23 • 24 • 25 Which of the following fluids should be used as a bolus in the resuscitation of this infant? • 26 • 27 • A. • 12 Cardiopulmonary exam reveals tachycardia and tachypnea.5 mEq!L • 17 Chloride 1 08 mEq/L • 18 Bicarbonate 14 mEq/L • 19 Blood urea nitrogen (BUN) 20 mgldL • 20 Serum creatinine 0. 5% dextrose • 31 • ID.8 mg/I:IL • 21 Calcium 10. Laboratory results are shown below. • 14 Chemistry panel • 15 Serum sodium 165 mEq/L • 16 Serum potassium 4.4 kg) since his routine check-up two 9 weeks ago. pulse is 200/min. respiratory rate is 42/min. 1 • 2 Item: 41 of 44 RI M Mark 3 Previous Next Lab Values. Notes Calculator. • 4- 5 6 A 9-month-old infant is brought to the emergency department with lethargy and tachypnea_ He was healthy 7 before developing fever and diarrhea four days ago. He has been taking some formula. his temperature is • 10 102. His mucous membranes are dry. and blood pressure is 70/45 mm Hg_ He is • 11 lethargic with decreased tone and decreased deep tendon reflexes. He has had one wet diaper in the past twenty four hours. 0.

• 4- ▪ 5 ▪ 6 A newborn infant is brought to the nursery for evaluation after delivery. While in the ▪ 9 hospital. The infant was born via normal spontaneous vaginal ▪ 8 delivery and required no resuscitation. Notes Calculator. Cytomegalovirus infection • 19 • 20 • E Human immunodeficiency virus infection • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 0 Feedback Suspend End Block . Rubella • 18 • D.• 1 • 2 Item: 42 of 44 A V. the infant requires treatment for anemia and hyperbilirubinemia. On subsequent examinations.Mark ▪ 3 Previous Next Lab Values. Toxoplasmosis • 15 • E. but she had only two prenatal visits. Syphilis • 16 • 17 • C. The infant has hepatosplenomegaly on examination. The mother reports that the pregnancy ▪ 7 was uncomplicated. the • 10 infant has clear rhinorrhea and ulcerative lesions on his feet Which of the following congenital infections is • 11 most likely in this patient? • 12 • 13 • 14 • A.

What is the most common cause of death in this patient population? • 12 • 13 • 14 • A. Diabetes-related complications • 18 ▪ D. His blood pressure is 120/10 ▪ 9 mmHg and pulse is 80/min.• 1 • 2 Item: 43 of 44 F' Mark 3 Previous Next Lab Values. dysmetria. 0 Feedback. Suspend End Block . Malignancy • 19 • 20 • E Septic shock • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 •42 41. • 4 ▪ 5 ▪ 6 A 15-year-old Caucasian male is brought to the office by his mother for the evaluation of a six-month history of ▪ 7 unstable gait and speech difficulty which are getting worse over time_ His past medical history is insignificant ▪ 8 He is not taking any medications. Notes Calculator. Renal failure • 16 • 17 ▪ C. and denies smoking or alcohol consumption. and absence of deep plantar • 11 reflexes on lower extremities.' The neurologic examination showed dysarthria. Cardiomyopathy • 15 • B. Musculoskeletal examination showed scoliosis and feet deformity with 'hammer • 10 toes. nystagmus.

The rest of the examination is unremarkable. Notes Calculator. Bone marrow biopsy • 22 • B. Observation • 26 • 27 • E Platelet transfusion • 28 • F.1pL • 16 • 17 • 18 Peripheral smear shows a few large platelets. Vital signs are • 10 normal. He takes no medications.5gidL • 14 Platelets 1 0. CEDDip L • 15 Leukocytes 1. well-appearing child with scattered petechiae and • 11 ecchymoses over the trunk and extremities. Intravenous antibiotics • 23 • 24 • C. Physical examination shows a cooperative. What is the most appropriate next step in management of this • 19 patient? • 20 • 21 • A. he had an upper ▪ 8 respiratory tract infection that resolved uneventfully. has no allergies. and his ▪ 9 immunizations are up to date_ Family history is negative for bleeding or clotting disorders. Laboratory results • 12 are as follows: • 13 Hemoglobin 13. He has had no bleeding or recent trauma Three weeks ago.• 1 • 2 Item: 44 of 44 F Mark ▪ 3 Previous Next Lab Values. Splenectomy • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 0 Feedback Suspend End Block . • 4- ▪ 5 ▪ 6 A previously healthy 6-year-old boy is brought to the physician after the sudden appearance of bruises ▪ 7 throughout his body.000. Intravenous immunoglobulin • 25 • ID.

Her body mass index is 15 kg/m2. Her skin is dry with lanugo. pulse is 42/min. Administer levothyroxine [14%] • 40 11 Ariminktpr nlAn7Anine 11041 • 41 • 42 End Block Feedback .9 mEq/L • 17 Chloride 102 mEq/L • 18 Bicarbonate 24 mEq/L • 19 Blood urea nitrogen 18 mg/dL • 20 Creatinine mg/dL • 21 Calcium 8 mg/dL • 22 Glucose 78 mg/dL • 23 Magnesium 2. The patient is • 13 alert and cooperative but appears tired. Temperature is 36_2 C (97. blood pressure is • 12 88/58 mm Hg.5 pU/mL • 28 Free T4 ng/dL • 29 Triiodothyronine (T3) 65 ngfdL • 30 • 31 Urine human chorionic gonadotropin Negative • 32 • 33 • 34 What is the most appropriate next step in management of this patient's symptoms? • 35 • 36 A. She also complains of difficulty with weight loss_ The • 8 patient wants to lose about 4. Her mother has Graves' disease. Menarche was at age 12 and her last • 10 menstrual period was 2 months ago. Notes Calculator. The patient is an honors student and involved in ballet and • 11 cross-country running.3 mg/dL • 24 Phosphorus 2 mg/dL • 25 • 26 Thyroid function tests • 27 Thyroid-stimulating hormone 4. Laboratory results are as follows: • 14 Serum chemistry • 15 Sodium 136 mEdiL • 16 Potassium 2.2 F). Administer fluoxetine [59C] • 38 • 39 C. and respirations are 16/min.5 kg (10 Ib) because she believes she is "enormous. • 4 • 5 • 6 A 16-year-old girl comes to the physician for a routine examination.• 2 Item: 1 of 44 V-• Mark • 3 Previous Next Lab Values. she • 9 has been on a vegetarian diet because meat makes her feel bloated. She is wearing a sweater despite the • 7 warm weather and says that she often feels cold. Administer bupropion [1%] • 37 B." For the past year.

Administer olanzapine [1%] • 21 E Hospitalization [67%] • 22 • 23 F. Notes Calculator.• 2 Item: 1 of 44 V-Mark -4(1 • 3 Previous Next Lab Values.4 ngidL ▪ 9 Triiodothyronine (T3) 65 ngldL • 10 • 11 Urine human chorionic gonadotropin Negative • 12 • 13 • 14 What is the most appropriate next step in management of this patient's symptoms? • 15 • 16 A. with follow-up in 2 weeks [10%] • 26 • 27 • 28 Explanation: • 29 • 30 • 31 Anorexia versus bulimia nervosa • 32 . 5 therapy • 37 Anorexia • Intense fear of weight gain • 38 • Nutritional rehabilitation nervosa • 39 • Distorted views of body • Olanzapine. Administer fluoxetine [5%] • 18 • 19 C. Administer levothyroxine [141C] • 20 ID.5 pLlimL ▪ 8 Free T4 0. - • 33 Diagnosis Clinical features Treatment • 34 • 35 • Cognitive-behavioral • 36 • BMI <18.Refer for cognitive behavioral therapy. Administer bupropion [1%] • 17 B. 141L1J114--J14-11 I I IIILJL • 4 Phosphorus 2 mg/dL ▪ 5 ▪ 6 Thyroid function tests ▪ 7 Thyroid-stimulating hormone 4. Order serum cobalamin [19C] • 25 H. if no response se • 40 weight and shape • • 41 42 Feedback a End Block . Order serum 25-hydroxy-vitamin D [1'3C] • 24 G.

and heart failure. patients require close monitoring for refeeding syndrome: • 33 electrolyte depletion. and phobic avoidance of many foods. To lose weight. • 37 • 38 (Choice B) Clear evidence supports the use of selective serotonin reuptake inhibitors (SSR1s) to achieve • 39 remission from binging and purging in bulimia nervosa However. Meals should be supervised. with • 29 the primary goal of weight gain_ Hospitalization and acute stabilization are highly recommended for • 30 dehydration. hypophosphatemia). determined dieting. patients may fast and/or exercise excessively (restricting • 25 subtype) or compensate for binge eating with laxatives or vomiting (purging subtype). • 35 • 36 (Choice A) Bupropion is contraindicated in patients with eating disorders as it can provoke seizures. electrolyte disturbances (eg. running). • 4. arrhythmias. It is characterized by distorted • 22 body image. or severe weight • 31 loss. bradycardia. 11 no iesponse weight and shape ▪ 5 to above ▪ 6 ▪ 7 • Recurrent episodes of ▪ 8 ▪ 9 binge eating • 10 • Binge eating followed by • Cognitive-behavioral • 11 compensatory behavior therapy • 12 Bulimia to prevent weight gain • Nutritional rehabilitation • 13 nervosa • 14 • Excess worrying about • SSRI medication. and some patients require nasogastric tube • 32 feeding. : 1 of 44 E F'Mark ▪ 3 Previous . resulting in unsafe weight loss. which can result from fluid and electrolyte shifts_ Vitamin • 34 deficiencies should be assessed and supplemented if deficiencies are identified (Choices F and G). ballet. Risk • 23 factors include high-achieving "type-A" personalities and participation in activities in which there is pressure to • 24 be slender (eg. SSRIs have not been shown to aid weight • 40 gain in anorexia nervosa and are therefore not recommended. Next Lab Values. • 41 • 42 End Block Feedback . • 27 • 28 Nutritional rehabilitation and psychotherapy are the cornerstones of treatment for anorexia nervosa. Notes Calculator. hypokalemia. Many patients also have • 26 secondary amenorrhea. often in • 15 body shape and weight combination with above • 16 • Maintains normal body • 17 weight (BMI 18_5-30) • 18 • 19 Li5MLEwotldr LLC • 20 • 21 Anorexia nervosa is an eating disorder that usually begins in adolescence. This patient meets the DSM-V diagnostic criteria for anorexia nervosa (Table). as seen in this patient. 1 • ulanzapine. During the onset of anabolism.

Goals of treatment include nutritional rehabilitation • 34 and weight gain. Refeeding hypophosphatemia in adolescents with anorexia nervosa: a systematic review. • 24 • 25 (Choice ID) Olanzapine is the most widely studied antipsychotic for anorexia nervosa and can be considered • 26 in patients who fail to gain weight despite initial nutritional rehabilitation and cognitive behavioral therapy. patients require close monitoring for refeeding syndrome: • 13 electrolyte depletion. with ▪ 9 the primary goal of weight gain_ Hospitalization and acute stabilization are highly recommended for • 10 dehydration. • 35 • 36 References: • 37 • 38 1. and some patients require nasogastric tube • 12 feeding. • 27 • 28 (Choice H) Cognitive behavioral therapy is very important but this patient requires prompt comprehensive • 29 inpatient management. To lose weight. resulting in euthyroid hypothyroxinemia. bradycardia. be slender (eg. and electrolyte derangements. hypophosphatemia). as seen in this patient Meals should be supervised. Item: 1 of 44 F'Mark ▪ 3 Previous Next Lab Values. Identification and management of eating disorders in children and adolescents. hypokalemia. Notes Calculator. Patients should be monitored closely for refeeding syndrome. During the onset of anabolism. patients may fast and/or exercise excessively (restricting ▪ 5 subtype) or compensate for binge eating with laxatives or vomiting (purging subtype). Levothyroxine is not • 23 recommended and treatment should be directed at nutritional rehabilitation instead. severe bradycardia • 33 or cardiac dysrhythmias. and heart failure. • 30 • 31 Educational objective: • 32 Patients with anorexia nervosa require hospitalization when they have unstable vital signs. • 4. which can result from fluid and electrolyte shifts_ Vitamin • 14 deficiencies should be assessed and supplemented if deficiencies are identified (Choices F and G). ▪ 7 ▪ 8 Nutritional rehabilitation and psychotherapy are the cornerstones of treatment for anorexia nervosa. or severe weight • 11 loss. Many patients also have ▪ 6 secondary amenorrhea. • 15 • 16 (Choice A) Bupropion is contraindicated in patients with eating disorders as it can provoke seizures. • 21 (Choice C) Starvation and malnutrition can decrease serum thyroxine-binding globin and albumin • 22 concentrations in anorexia nervosa. • 40 • 41 • 42 End Block Feedback . • 17 • 18 (Choice B) Clear evidence supports the use of selective serotonin reuptake inhibitors (SSR1s) to achieve • 19 remission from binging and purging in bulimia nervosa However. ba-llet. rather than outpatient referral. arrhythmias. SSRIs have not been shown to aid weight • 20 gain in anorexia nervosa and are therefore not recommended. running). electrolyte disturbances (eg. • 39 2. This patient meets the DSM-V diagnostic criteria for anorexia nervosa (Table).

• 2 Item: 1 of 44 F' Mark
• 3 Previous Next Lab Values. Notes Calculator.
• 4
▪ 5 Nutritional rehabilitation and psychotherapy are the cornerstones of treatment for anorexia nervosa, with
▪ 6 the primary goal of weight gain_ Hospitalization and acute stabilization are highly recommended for
▪ 7 dehydration, electrolyte disturbances (eg, hypokalemia, hypophosphatemia), bradycardia, or severe weight
▪ 8 loss, as seen in this patient Meals should be supervised, and some patients require nasogastric tube
▪ 9 feeding. During the onset of anabolism, patients require close monitoring for refeeding syndrome:
• 10 electrolyte depletion, arrhythmias, and heart failure, which can result from fluid and electrolyte shifts. Vitamin
• 11 deficiencies should be assessed and supplemented if deficiencies are identified (Choices F and G).
• 12
• 13 (Choice A) Rupropion is contraindicated in patients with eating disorders as it can provoke seizures.
• 14
• 15 (Choice B) Clear evidence supports the use of selective serotonin reuptake inhibitors (SSR1s) to achieve
• 16 remission from binging and purging in bulimia nervosa. However, SSRIs have not been shown to aid weight
• 17 gain in anorexia nervosa and are therefore not recommended.
• 18
• 19
(Choice C) Starvation and malnutrition can decrease serum thyroxine-binding globin and albumin
• 20
concentrations in anorexia nervosa, resulting in euthyroid hypothyroxinemia. Levothyroxine is not
• 21
recommended and treatment should be directed at nutritional rehabilitation instead.
• 22
(Choice ID) Olanzapine is the most widely studied antipsychotic for anorexia nervosa and can be considered
• 23
in patients who fail to gain weight despite initial nutritional rehabilitation and cognitive behavioral therapy.
• 24
• 25
(Choice H) Cognitive behavioral therapy is very important but this patient requires prompt comprehensive
• 26
inpatient management, rather than outpatient referral.
• 27
• 28 Educational objective:
• 29 Pi
Patients with anorexia nervosa require hospitalization when they have unstable vital signs, severe bradycardia
• 30 or cardiac dysrhythmias, and electrolyte derangements. Goals of treatment include nutritional rehabilitation
• 31 and weight gain. Patients should be monitored closely for refeeding syndrome.
• 32
• 33
References:
• 34
• 35 1. Identification and management of eating disorders in children and adolescents.
• 36 2. Refeeding hypophosphatemia in adolescents with anorexia nervosa: a systematic review.
• 37
• 38
• 39
Copyright © Morld Last updated: [10/13/2014]
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• 41
• 42 End Block
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2 Item: 2 of 44 F' Mark
3 Previous Next Lab Values. Notes Calculator.
• 4 1.
5
6 A 4-year-old boy is brought to the physician for evaluation of speech that is "difficult to understand." His
▪ 7 parents state that "he doesn't seem to listen" and describe him as a slow learner who only uses single words
▪ 8 and frequently mumbles. The boy prefers to play by himself and is obsessed with building towers with
▪ 9 blocks_ He becomes extremely distressed if prevented from performing this activity. He was born at term
• 10 from an uncomplicated pregnancy and delivery, and his physical growth has been normal. His older brother
• 11 was aggressive as a young child and diagnosed with attention deficit hyperactivity disorder. In the office
• 12 waiting room, the boy plays quietly with blocks and seems oblivious to the other children. When his mother
• 13 calls him to come to her, he turns toward her once and then resumes playing. His physical examination and
• 14 hearing are normal. Which of the following is the most appropriate statement to tell his parents?
• 15
• 16
• 17
A. "He most likely has a developmental language disorder and would benefit from speech therapy."
• 18
[2%]
• 19 H. "His behavior is most likely due to the inattentive subtype of attention deficit hyperactivity disorder."
• 20 [1%]
• 21
C. "I am concerned that his difficulties may indicate intellectual disability." [1%]
• 22
• 23
ID_ understand your concerns; a brain MRI will help establish a definitive diagnosis." [0%]
• 24 E. understand your concerns; a comprehensive evaluation for autism spectrum disorder will be
• 25 helpful." [95%]
• 26
• 27
• 28 Explanation:
• 29
• 30
• 31 DM-5 criteria for autism spectrum disorder
• 32
• 33
• Multiple, persistent deficits in social communication & interactions currently
• 34
• 35
or by history involving:
• 36 • Social-emotional reciprocity
• 37 • Nonverbal communicative behaviors
• 38 • Developing, maintaining & understanding relationships
• 39
• Restricted, repetitive patterns of behavior currently or by history:
• 40
• 41
• 42 End Block
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1
2 Item: 2 of 44 r. Mark
▪ 3 Previous Next Lab Values. Notes Calculator.
• 4-
▪ 5
▪ 6 DSM-5 criteria for autism spectrum disorder
▪ 7
▪ 8
▪ 9 • Multiple, persistent deficits in social communication & interactions currently
• 10 or by history involving:
• 11 • Social-emotional reciprocity
• 12
• Nonverbal communicative behaviors
• 13
• Developing, maintaining & understanding relationships
• 14
• 15 • Restricted, repetitive patterns of behavior currently or by history:
• 16 • Repetitive motor movements
• 17 • Insistence on sameness or inflexible adherence to routines
• 18
• Fixated interests of abnormal intensity or focus
• 19
• 20
• Adverse responses to sensory input
• 21 • Symptoms occur in the early developmental period & cause impairment
• 22
• Not better explained exclusively by intellectual disability or another condition
• 23
• 24
4.15M LEWurl d. LLC
• 25
• 26 This patient's impaired social interaction and communication, restricted interests, insistence on
• 27 routine, and delayed language development raise concerns about autism spectrum disorder. Autism
• 28 spectrum disorder can occur with or without language impairment. Language deficits can range from stilted
• 29 speech to language delays (seen in this case) or to complete lack of intelligible speech. Varying degrees of
• 30 intellectual impairment can also accompany autism.
• 31
• 32 The DM-5 diagnosis of autism spectrum disorder encompasses high-functioning autism (Asperger
• 33 syndrome), childhood disintegrative disorder, and any pervasive developmental disorder not otherwise
• 34 specified_ Diagnosis is based on history and behavioral observations (Table}_ The physician should
• 35 empathically listen to the parents' concerns and complete a comprehensive evaluation before making a
• 36 definitive diagnosis. This evaluation includes structured assessments of social, language, and intellectual
• 37 development in addition to hearing, vision, and genetic (eg, Fragile X syndrome) testing.
• 38
• 39 (Choice A) Varying degrees of language impairment frequently accompany autism spectrum disorders.
• 40 Howpvp.r isolated lannuaoa disorripm am not nharantpri7p.el by elpfinits in non-verbal communination sonial
• 41
• 42 End Block
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1
2 Item: 2 of 44 V-Mark
3 Previous Next Lab Values. Notes Calculator.
• 4 intellectual impairment can also accompany autism.
5
6 The DM-5 diagnosis of autism spectrum disorder encompasses high-functioning autism (Asperger
▪ 7 syndrome), childhood disintegrative disorder, and any pervasive developmental disorder not otherwise
▪ 8 specified_ Diagnosis is based on history and behavioral observations (Table). The physician should
▪ 9 empathically listen to the parents' concerns and complete a comprehensive evaluation before making a
• 10 definitive diagnosis. This evaluation includes structured assessments of social, language, and intellectual
• 11 development in addition to hearing, vision, and genetic (eg, Fragile X syndrome) testing.
• 12
• 13 (Choice A) Varying degrees of language impairment frequently accompany autism spectrum disorders.
• 14 However, isolated language disorders are not characterized by deficits in non-verbal communication, social
• 15 reciprocity, and lack of imaginative play.
• 16
• 17
(Choice B) Even though this patient is inattentive and does not listen to his mother, this is likely due to the
• 18
lack of social reciprocity seen in autism spectrum disorders. Diagnosis of attention deficit hyperactivity
• 19
disorder requires the presentation of multiple inattentive symptoms (eg, distractibility, forgetfulness, avoidance
• 20
of tasks, lack of follow through, careless mistakes); these are not exhibited by this patient
• 21
(Choice C) Intellectual disability may accompany autism but would not explain this patient's impaired social
• 22
communication and interactions.
• 23
• 24
(Choice 0) In the absence of focal neurologic findings, routine neuroimaging is not recommended for
• 25
patients with suspected autism spectrum disorder. These patients often require sedation for MRI, and this is
• 26
an unnecessary risk when imaging will likely be low yield.
• 27
• 28 Educational objective:
• 29 Autism spectrum disorder should be suspected in children with impaired social communication/interactions
• 30 and restricted repetitive interests or behaviors. Comprehensive evaluation includes structured assessments
• 31 of social, language, and intellectual development in addition to hearing, vision, and genetic testing.
• 32
• 33
References:
• 34
• 35 1. Diagnostic evaluation of autism spectrum disorders.
• 36 2. Autism spectrum disorders.
• 37
• 38
• 39
Copyright @ UWorld Last updated: [8/11/2014]
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• 41
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but that's just typical of preteens. 1 Item: 3 of 44 V. Conduct disorder [16°.6] • 18 C. he has always been hyperactive but is basically a good kid_ The past year he has ▪ 8 been a little more irritable and argumentative with us.Mark Previous Next Lab Values. although no criminal charges have been • 13 brought against him. arousal prior to act • 34 • 35 • Fascination with fire & its consequences • 36 • Pleasure or relief when settingiwitnessing fires • 37 • 38 • No external gain. Notes Calculator. Antisocial personality disorder [2%] • 17 13. 'We can't believe he ▪ 7 would do anything like this. revenge." They add that he has ▪ 9 wanted to be a firefighter since a young age and spends hours on the internet watching videos about fires and • 10 firefighting_ The boy's grades are average.6] • 19 I • 20 ID. Rased on this information. antisocial • 40 • • 41 • 42 End Block Feedback . Normal developmental experimentation [0%] • 21 E Oppositional defiant disorder [2%] • 22 • E Pyromania [80%] • 23 • 24 • 25 Explanation: • 26 • 27 • 28 Pyromania • 29 • 30 DSM-5 diagnosis • 31 • 32 • Deliberate fire setting on more than 1 occasion • 33 • Tension. which of the following is the most likely diagnosis? • 14 • 15 • 16 A. ▪ 5 ▪ 6 A 12-year-old boy is accused of setting his neighbor's house on fire. and his teachers describe him as a loner who has difficulty making • 11 friends at school_ Two years ago he was caught setting the interior of his father's car on fire_ The boy has • 12 also been linked to several suspicious fires in the neighborhood. psychosis. manic episode. Attention deficit hyperactivity disorder [1°. His parents say. or political motivation. not done to attract attention • 39 • Not better explained bar conduct disorder.

political statement. 1 Item: 3 of 44 Mark Previous Next Lab Values. or political motivation. • 26 profit. There is no obvious motive (eg. They deliberately start fires to reduce tension and feel • 28 pleasure or relief afterward. A separate diagnosis of pyromania is not indicated when a patient who sets fires meets the criteria • 37 for conduct disorder. It involves a • 39 pattern of violation of basic societal rules and the rights of others and requires a history with some symptoms se • 40 • 41 • 42 End Block Feedback . a rare impulse control disorder more commonly found in males. arousal prior to act • 12 • Fascination with fire & its consequences • 13 • 14 • Pleasure or relief when settinglwitnessing fires • 15 • No external gain. not done to attract attention • 16 • 17 • Not better explained by conduct disorder. antisocial • 18 personality disorder. LLC • 22 • 23 This child's fascination with fire and history of repetitive. deceitfulness or theft. Notes Calculator. firefighters). recognition). revenge. psychosis. ▪ 5 ▪ 6 Pyromania ▪ 7 ▪ 8 DSM-5 diagnosis ▪ 9 • 10 • Deliberate fire setting on more than 1 occasion • 11 • Tension. • 38 (Choice A) The diagnosis of antisocial personality disorder is given to individuals MB years. intentional fire setting suggest a diagnosis of • 24 pyromania. destruction of property (includes fire setting). substance • 19 intoxication) • 20 • 21 Cr]USMLEWorld. revenge. The diagnosis of conduct disorder requires at least 3 of 15 behaviors that fall into 4 categories: • 34 aggression toward people and animals. a psychiatric condition of childhood and • 32 adolescence characterized by a repetitive and persistent pattern of violating major societal rules or the rights • 33 of others. Individuals with this condition tend to be fascinated by fire and • 27 anything related to it (eg. DM-5 describes pyromania as • 25 intentional fire setting on more than one occasion to relieve internal tension. manic episode. or impaired judgment (neurocognitive disorder. or substance intoxication_ • 30 • 31 Fire setting is also a symptom of conduct disorder (Choice C). fire stations. Pyromania must be differentiated from fire setting associated with a manic • 29 episode. psychosis. • 35 and serious violation of rules. This patient exhibits only 1 of 15 possible behaviors seen in conduct • 36 disorder.

• 32 • 33 Educational objective: • 34 Pyromania is characterized by intentional and repeated fire setting with no obvious motive_ Individuals with • 35 conduct disorder can also have a history of fire setting. Clinical characteristics and psychiatric el:morbidity of pyromania se • 41 • 42 End Block Feedback . irritable. • 18 (Choice A) The diagnosis of antisocial personality disorder is given to individuals X18 years. Pyromania must be differentiated from fire setting associated with a manic ▪ 9 episode. and accidental or occasional fire setting by unsupervised • 30 children may occur_ This happens most commonly in children age 5-10 who do not understand the dangers • 31 of playing with fire_ In contrast. • 23 there is insufficient information to substantiate a diagnosis of attention deficit hyperactivity disorder (ADHD) or • 24 oppositional defiant disorder (ODD). a psychiatric condition of childhood and • 12 adolescence characterized by a repetitive and persistent pattern of violating major societal rules or the rights • 13 of others. They deliberately start fires to reduce tension and feel ▪ 8 pleasure or relief afterward. but other features (eg. lying. There is no obvious motive (eg. Notes Calculator. and impulsive. hyperactive. • 15 and serious violation of rules. recognition). a rare impulse control disorder more commonly tound in males. firefighters). This patient exhibits only 1 of 15 possible behaviors seen in conduct • 16 disorder. recurrent fire setting to relieve tension. ▪ 6 profit. destruction of property (includes fire setting). • 21 • 22 (Choices B and E) Although this boy's parents describe him as hyperactive. Individuals with this condition tend to be fascinated by fire and ▪ 7 anything related to it (eg. resulting • 25 in academic difficulties and impaired social relationships_ amp is characterized by a pattern of angry/irritable • 26 mood and argumentative/defiant behavior toward authority figures_ Children with ADHD or ODD do not • 27 typically engage in fire setting_ • 28 • 29 (Choice ID) Children are naturally curious about fire. A separate diagnosis of pyromania is not indicated when a patient who sets fires meets the criteria • 17 for conduct disorder. IJSM-5 describes pyromania as ▪ 5 intentional fire setting on more than one occasion to relieve internal tension. political statement. Children with ADHD are inattentive. theft. and argumentative. pyromania involves intentional. or substance intoxication_ • 10 • 11 Fire setting is also a symptom of conduct disorder (Choice C). pyromania. psychosis. fire stations. revenge. deceitfulness or theft. 1 Item: 3 of 44 [Ell V-• Mark Previous Next Lab Values. It involves a • 19 pattern of violation of basic societal rules and the rights of others and requires a history with some symptoms • 20 of conduct disorder before age 15. The diagnosis of conduct disorder requires at least 3 of 15 behaviors that fall into 4 categories: • 14 aggression toward people and animals. cruelty to others) • 36 are also present • 37 • 38 References: • 39 • 40 1.

• 17 there is insufficient information to substantiate a diagnosis of attention deficit hyperactivity disorder (ADHD) or • 18 oppositional defiant disorder (ODD). A separate diagnosis of pyromania is not indicated when a patient who sets fires meets the criteria • 11 for conduct disorder. lying. hyperactive. Clinical characteristics and psychiatric comorbidity of pyromania • 35 2. irritable. resulting • 19 in academic difficulties and impaired social relationships. 34 1. and accidental or occasional fire setting by unsupervised • 24 children may occur_ This happens most commonly in children age 5-10 who do not understand the dangers • 25 of playing with fire_ In contrast. but other features (eg. • 31 • 32 References: • 33 . destruction of property (includes fire setting). a psychiatric condition of childhood and • 6 adolescence characterized by a repetitive and persistent pattern of violating major societal rules or the rights . • 26 • 27 Educational objective: • 28 Pyromania is characterized by intentional and repeated fire setting with no obvious motive_ Individuals with • 29 conduct disorder can also have a history of fire setting. and impulsive. theft.• 1 2 Item: 3 of 44 VI/lark Previous Next Lab Values. • 9 and serious violation of rules. Children with ADHD are inattentive. cruelty to others) • 30 are also present. ODD is characterized by a pattern of angry/irritable • 20 mood and argumentative/defiant behavior toward authority figures_ Children with ADHD or ODD do not • 21 typically engage in fire setting_ • 22 • 23 (Choice 0) Children are naturally curious about fire. • 4 • 5 Fire setting is also a symptom of conduct disorder (Choice C). pyromania involves intentional. It involves a • 14 pattern of violation of basic societal rules and the rights of others and requires a history with some symptoms • 15 of conduct disorder before age 15. and argumentative. recurrent fire setting to relieve tension. • 16 (Choices B and E) Although this boy's parents describe him as hyperactive. This patient exhibits only 1 of 15 possible behaviors seen in conduct • 10 disorder. deceitfulness or theft. Notes Calculator. Prevalence and correlates of fire-setting in the United States: results from the National • 36 Epidemiologic Survey on Alcohol and Related Conditions (NESARC). • 37 • 38 • 39 Copyright © LIWorld Last updated: [11/27/2014] • 40 • 41 • 42 End Block Feedback . 7 of others. • 12 • 13 (Choice A) The diagnosis of antisocial personality disorder is given to individuals L18 years. The diagnosis of conduct disorder requires at least 3 of 15 behaviors that fall into 4 categories: • 8 aggression toward people and animals.

7 extremities following a viral illness 7 days earlier_ He has also had abdominal pain and nausea for 24 hours. The patient has no past medical history and takes no • 9 medications. Vital signs are within normal limits for his age_ The abdomen is soft with mild diffuse • 10 tenderness to deep palpation.000/u L • 19 Leukocytes 7. 5 • 6 A 10-year-old boy is brought to an urgent care center by his mother with a 1-day history of "rash' on his lower .500/4 • 20 • 21 Urinalysis • 22 Specific gravity 1. • 8 He has no travel history and no sick contacts. Bacterial infection [5%] • 40 IRnnp r-r-irrriutr infiltmtinn 1-1 041 so • 41 • 42 End Block Feedback .• 1 2 Item: 4 of 44 V-Mark • • 3 Previous Next Lab Values. 4 A .0 WI:IL • 17 Hematocrit 40% • 18 Platelets 140. • 11 Bilateral lower extremities have nonblanching palpable purplish lesions below the knee and mild swelling of the • 12 feet The remainder of the examination is normal_ • 13 • 14 Laboratory results are as follows: • 15 Complete blood count • 16 Hemoglobin 13. there is no rebound tenderness or guarding.025 • 23 pH • 24 Protein +1 • 25 Blood Moderate • 26 Glucose Negative • 27 Ketones Negative • 28 Leukocyte esterase Negative • 29 Nitrites Negative • 30 Bacteria None • 31 White blood cells 1-2/hpf • 32 Red blood cells 20-30/hpf • 33 Casts None • 34 Crystals None • 35 • 36 Which of the following is the most likely cause of this child's skin manifestations? • 37 • 38 • 39 A. Bowel sounds are normal. Notes Calculator.

Nutritional deficiency [G%] • 14 • E Vasculitis [85%] • 15 E Viral infection [7%] • 16 • 17 • 18 Explanation: • 19 • 20 • 21 lienoch-Schanlein purpura • 22 • 23 • 24 • Immune-mediated leukocAoclastic va5culitis Pathogenesis • 25 • Associated with IgA deposition in affected organs • 26 • 27 • 28 • Palpable purpura with normal platelet count & • 29 coagulation studies • 30 ▪ Arthritis/arthralgia • 31 Clinical • Abdominal pain • 32 • 33 manifestations • Gastrointestinal bleeding • 34 • Intussusception • 35 * Renal disease similar to IgA nephropathy • 36 • 37 • Scrotal pain & swelling • 38 • 39 . 4 Crystals None . Clotting factor deficiency [3%] • 12 • 13 D. Bone marrow infiltration [1%] • -1 -1 C.• 1 2 Item: 4 of 44 F'Mark • .Normal platelet count & coagulation studies v • 40 Lab-oratory • 41 • 42 End Block Feedback . 7 • 8 • 9 A. 5 6 • Which of the following is the most likely cause of this child's skin manifestations? . Bacterial infection [5%] • 10 B. 3 Previous Next Lab Values. Notes Calculator.

Notes Calculator.• 1 • 2 Item: 4 of 44 F'Mark • 3 Previous Next ..LL. Palpable purpura with normal platelet count & 13 coagulation studies • 14 • 15 • Arthritis/a rthralgia • 16 Clinical • Abdominal pain 17 manifestations • Gastrointestinal bleeding • 18 • Intussusception • 19 • 20 • Renal disease similar to IgA nephropathy • 21 • Scrotal pain & swelling • 22 . or Treatment • 34 altered mental status • • Systemic glucocorticoids in patients with severe • 36 • abdominal pain unresponsive to nonsteroldal anti- • 38 inflammatory drugs • 39 • 40 L.41 • 42 End Block. ! • 23 • 24 • Normal platelet count & coagulation studies Laboratory • 25 • Normal to elevated creatinine 1 • 26 findings • Urinalysis: Hematuria. Lab Values. 4 • 5 Henoch-Schonlein purpura • 6 • 7 • 8 • Immune-mediated leukocytoclastic vasculitis 0 Pathogenesis • Associated with IgA deposition in affected organs • 10 11 • 12 . • 28 • 20 . Supportive management (hydration & pain control • 30 with nonsteroidal anti-inflammatory drugs) • 31 • 32 • Hospitalization indicated for severe abdominal pain.0 se • .uSMLEwadd. Feedback . red cell casts & mild proteinuria • '7 Z. • 33 renal insufficiency inability to tolerate oral intake.

or positive histopathology. non-nephrotic-range proteinuria. Treatment is primarily supportive and consists of hydration and pain control with nonsteroidal • 27 anti-inflammatory drugs. Neisseria • 30 rnerringitidis.K • 11 • 12 This child's presentation of hematuria. A. It typically follows an • 15 infection (often minor) and presents with the classic tetrad of palpable purpura on the lower extremities. HSP. In children with atypical • 25 presentations. Streptococcus przeurnornae) that presents with blue or black hemorrhagic. abdominal pain. specifically Henoch-SchOnlein purpura (HSP).1 I 1. Notes Calculator. is the most common systemic vasculitis of childhood. • 21 HSP can be diagnosed clinically in children presenting with classic signs and symptoms_ The diagnosis • 22 requires the presence of lower-extremity purpura or petechiae with at least one of the following: arthritis or • 23 arthralgia. and hemophilia B (factor IX deficiency). and renal disease. • 14 which is immune (IgA) mediated. such as abdominal pain or renal disease_ • 40 se • 41 • 42 End Block Feedback . renal involvement. Arthralgias are most common in the ankles and knees. a renal biopsy may be required to confirm the diagnosis. altered mental status • 5 • Systemic glucocorticoids in patients with severe • 6 • 7 abdominal pain unresponsive to nonsteroidal anti- • 8 inflammatory drugs • 9 • 10 kZi OhtLE wodld. and • 20 legs and is distributed symmetrically. these • 39 conditions do not present with other systemic findings.• 1 • 2 Item: 4 of 44 l 'Mark • 3 Previous Next . abdominal pain. • 31 These patients are typically very ill with fever. abdominal pain. which may be purpuric or petechial. However.1. However. Lab Values. Laboratory testing is not required but • 24 typically demonstrates a normal platelet count and a mildly elevated creatinine. Abdominal pain is typically colicky and is thought to be • 17 secondary to localized vasculitis of the bowel wall. OM IA ■ . and a purpuric rash on the lower extremities without • 13 thrombocytopenia is most consistent with a vasculttfs. hemophilia A (factor VIII deficiency). deposition of IgA in the mesangium • 26 will be seen. neither of which is present in this patient • 36 • 37 (Choice C) Common clotting factor deficiencies that cause purpura in children include von Willebrand • 38 disease. The rash in HSP. 1. hypotension. patients with leukemia • 35 often have an elevated white blood cell count and thrombocytopenia. • 28 • 29 (Choice A) Purpura fulminans is a life-threatening condition seen with bacterial infections (eg.1. thighs. typically involves the buttocks. • 18 Renal manifestations include hematuria. and a mildly elevated serum • 19 creatinine. purpuric lesions. and evidence of disseminated intravascular • 32 coagulation_ • 33 • 34 (Choice B) Bone marrow infiltration by leukemic cells can cause purpura. • 16 arthralgias.

• 37 • 38 • 39 Copyright © UWorld Last updated: [7/20/2014] se • 40 • 41 • 42 End Block Feed back . • 6 • 7 These patients are typically very ill with fever. childhood polyarteritis • 34 nodosa. neither of which this • 23 child has_ • 24 • 25 Educational objective: • 26 Henoch-SchOnlein purpura (HSP) is an IgA-mediated vasculitis of childhood that presents with palpable • 27 purpura on the lower extremities. Notes Calculator. these would be extremely unlikely in a previously healthy child without any dietary • 18 restrictions. arthralgias. neither of which is present in this patient. • 11 • 12 (Choice C) Common clotting factor deficiencies that cause purpura in children include von Willebrand • 13 disease. hemophilia A (factor VIII deficiency). or vesicular rather than purpuric. can cause petechiae. these illnesses often present with fever and additional systemic symptoms. patients with leukemia • 10 often have an elevated white blood cell count and thrombocytopenia. maculopapular. Rocky • 21 Mountain spotted fever and atypical measles can have lesions that progress to generalized purpura. However. However. • 36 3. hypotension. such as abdominal pain or renal disease. purpuric lesions. EULARIPRINTO/PRES criteria for Henoch-Schonlein purpura. Henoch-Schonlein Purpura • 32 • 33 2. these • 14 conditions do not present with other systemic findings. unlike those with other causes of purpura_ • 29 • 30 References: • 31 1. childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara • 35 2008. abdominal pain. • 19 • 20 (Choice F) Viral exanthems are typically macular. and hemophilia B (factor IX deficiency). and renal involvement Children with HSP • 28 have a normal platelet count. • 15 • 16 (Choice 0) Nutritional deficiencies. or • 17 ecchymoses. Streptococcus pneurnoniae) that presents with blue or black hemorrhagic. purpura. Neisseria rnerringitidis. • 22 However. Part II: Final classification criteria. 4 • 5 (Choice A) Purpura fulminans is a life-threatening condition seen with bacterial infections (eg.• 1 • 2 Item: 4 of 44 V-Mark • 3 Previous Next Lab Values. However. such as vitamin C deficiency (scurvy). and evidence of disseminated intravascular • 8 coagulation_ 9 (Choice B) Bone marrow infiltration by leukemic cells can cause purpura. Clinical practice: Diagnosis and management of Henoch-Schanlein purpura.

Lactose intolerance [1%] • 16 E Salmonella poisoning [7%] • 17 F. • 33 diarrhea (sometimes bloody). Vibrio cholera [1%] • 12 0 B. What is the most likely cause of the patient's ▪ 9 symptoms? • 10 • 11 A. dialysis if necessary. Escherichia coli [79%] • 13 C. and most patients are severely dehydrated. abdominal pain. Treatment is generally supportive. Notes Calculator. Antibiotics are not indicated_ • 29 • 30 (Choice A) Cholera usually presents during an epidemic with an abrupt. platelet • 27 count. and produces a bloody diarrhea. and the initial presentation includes abdominal pain and diarrhea. The diagnosis is confirmed v • 40 • 41 • 42 End Block Feedback . and (3) presence of positive reducing sugars. and is not • 34 seen in the very young..to four-years-old. Campylobacter jejuni [9%] • 18 • 19 • 20 Explanation: • 21 • 22 This patient has hemolytic-uremic syndrome. and involves • 28 plasmapheresis. There is no hemolysis or jaundice_ • 35 • 35 (Choice 0) Lactose intolerance has features of chronic diarrhea (not bloody). It can present with abdominal • 37 pain or cramps. painless diarrhea There • 31 may be vomiting. weight loss and rectal fissures_ It generally has a gradual course. which is caused by a toxin released by Escherichia coli. Crohn's disease [2%] • 14 • 15 ID. thrombocytopenia and hemolytic anemia Investigations include CB C. 4 A 1G-year-old boy is brought to the emergency department due to abdominal pain and bloody diarrhea. and is usually associated with ingestion of dairy products. and steroids. BUN and creatinine. 1 2 Item: 5 of 44 F' Mark ' ▪ 3 Previous Next Lab Values. blood smear. Subsequent • 24 activation of the coagulation system and red cell hemolysis results in jaundice. watery. thrombocytopenia and renal insufficiency. The • 26 classic triad of HUS is uremia. The • 23 verotoxin invades and destroys the colonic epithelial lining. The diagnosis is suggested by: (1) improvement of symptoms after abstaining from dairy • 39 products. The treatment is fluid replacement • 32 (Choice C) Crohn's disease can affect any part of the GI tract It can present with fever. (2) acid pH of the stools. . and then suddenly became ill_ Physical examination shows a ▪ 7 pale and jaundiced child_ There is diffuse abdominal tenderness and 2+ pedal edema_ Laboratory studies ▪ 8 show anemia. urinalysis. It is more common in Asians and • 38 Africans. HUS is more common in • 25 children aged one. The ▪ 6 mother says that he was "fine" a few days ago.

bone. The diagnosis is suggested by: (1) improvement of symptoms after abstaining from dairy • 24 products. BUN and creatinine. and involves • 12 plasmapheresis. The treatment is fluid replacement. HUS is more common in ▪ 9 children aged one. and steroids. and the initial presentation includes abdominal pain and diarrhea. abdominal pain. Subsequent ▪ 8 activation of the coagulation system and red cell hemolysis results in jaundice. It generally has a gradual course. dialysis if necessary. and (3) presence of positive reducing sugars. • 38 • 39 Copyright © UWorld Last updated: [8/22/2014] • 40 • 41 • 42 End Block Feedback . There is no hemolysis or jaundice_ • 20 • 21 (Choice ID) Lactose intolerance has features of chronic diarrhea (not bloody). food or milk_ It can • 28 present with general malaise. and triad • 37 of anemia. and is not • 19 seen in the very young. platelet • 11 count. and produces a bloody diarrhea. painless diarrhea There • 15 may be vomiting. If untreated. blood smear. • 34 • 35 Educational Objective: • 35 Suspect hemolytic uremic syndrome in a child with sudden-onset abdominal pain. and most patients are severely dehydrated. • 18 diarrhea (sometimes bloody). weight loss and rectal fissures. bloody diarrhea.to four-years-old.• 1 • 2 Item: 5 of 44 V-Mark ▪ 3 Previous Next Lab Values. urinalysis. watery. • 31 (Choice F) Even though Campytobacterjejurri can cause bloody diarrhea. The ▪ 7 verotoxin invades and destroys the colonic epithelial lining. (2) acid pH of the stools. Stool cultures are diagnostic. and renal failure. thrombocytopenia.. which is caused by a toxin released by Eschend-ria cob. the disease can • 30 progress to involve the brain. kidney and joints. and is usually associated with ingestion of dairy products. The diagnosis is confirmed • 25 by a positive hydrogen breath test • 26 • 27 (Choice E) Salmonella poisoning is usually due to ingestion of contaminated water. Notes Calculator. thrombocytopenia and hemolytic anemia Investigations include CBC. Antibiotics are not indicated_ • 13 • 14 (Choice A) Cholera usually presents during an epidemic with an abrupt. Most • 29 individuals have diarrhea for several weeks. Treatment is generally supportive. and an erythematous rash on the abdomen (Rose spots). • 16 • 17 (Choice C) Crohn's disease can affect any part of the GI tract It can present with fever. it does not cause • 32 thrombocytopenia_ Renal failure can be seen with any diarrhea secondary to dehydration and prerenal • 33 azotemia. It can present with abdominal • 22 pain or cramps. fever. • 4 Explanation: ▪ 6 This patient has hemolytic-uremic syndrome. The • 10 classic triad of HUS is uremia. It is more common in Asians and • 23 Africans.

Treatment with penicillin makes rheumatic fever highly • 35 unlikely in this patient • 36 • 37 (Choice C) Lymphoproliferative disorders such as leukemia may present with symptoms such as fever. subcutaneous nodules and chorea. IMP-SMX and cefaclor • 26 (most classic association}_ It most commonly occurs in young children following the treatment of viral • 27 infections with antibiotic agents. sore throat and difficulty swallowing. amoxicillin. • 38 lymphadenopathy and joint pains. carditis. v • 40 • 41 • 42 End Block Feedback . but. symptoms of fever. erythema • 34 marginatum rash. Rheumatic fever [37%] • 13 • 14 • R. such as penicillin. 4 A s-year-old Caucasian male complains of fever. Henoch-Schonlein purpura [15136] • 17 • 18 E Infective endocarditis [21:k] • 19 • 20 Explanation: • 21 • 22 This young boy's initial presentation is suggestive of an upper respiratory tract infection. The onset of fever. Small tender lymph nodes are palpated in the cervical region_ The symptoms subside quickly on penicillin therapy. • 31 • 32 (Choice A) Rheumatic fever may occur in cases of streptococcal pharyngitis that are not appropriately • 33 treated with antibiotics. Notes Calculator. Physical ▪ 9 examination reveals scattered urticaria and palpable lymph nodes in the cervical. • 28 like true serum sickness. Complete blood count. Drug-induced reaction [31%] • 15 C. 1 2 Item: 6 of 44 V-Mark 3 Previous Next Lab Values. The mechanism of serum sickness-like reaction is not fully understood. blood smear and bone marrow biopsy are useful in • 39 establishing the diagnosis. skin rash and fleeting joint pain in the lower extremities. Ten days ▪ 8 later. which present with rapid-onset symptoms. the patient presents again with fever. urticaria and arthralgias begin one to two weeks after • 29 administration of the offending agent Serum sickness-like reactions are distinct from immediate • 30 hypersensitivity reactions. axillary and inguinal regions. however. most • 24 likely serum sickness-like reaction_ Serum sickness-like reaction is a hypersensitivity reaction that occurs • 25 one to two weeks after administration of certain drugs. • 10 Which of the following is the most likely cause of this patient's current complaints? • 11 • 12 A. This patient's history is more suggestive of a drug reaction. Lymphoproliferative disorder [891)] • 16 ID. Patients may present with a combination of polyarthritis. suggests a separate drug-induced reaction. • 23 rash and joint pain 10 days after penicillin therapy.

which present with rapid-onset symptoms. carditis. heart murmur. suggests a separate drug-induced reaction. • 30 • 31 Educational objective: • 32 Serum sickness-like reaction may occur one to two weeks after administration of certain drugs. Janeway lesions (erythematous macules). it does not • 37 represent a true drug allergy. such as • 33 penicillin. but. The mechanism of serum sickness-like reaction is not fully understood. • 26 • 27 (Choice E) Infective endocarditis may cause fever. Treatment with penicillin makes rheumatic fever highly • 17 unlikely in this patient • 18 • 19 (Choice C) Lymphoproliferative disorders such as leukemia may present with symptoms such as fever. Notes Calculator. amoxicillin. Patients may present with a combination of polyarthritis. urticaria and arthralgias begin one to two weeks after • 11 administration of the offending agent Serum sickness-like reactions are distinct from immediate • 12 hypersensitivity reactions. • 22 • 23 (Choice ID) Henoch-Schonlein purpura is a systemic IgA-mediated vasculitis occurring after viral upper • 24 respiratory infection. an immune-complex mediated hypersensitivity reaction to non-human proteins. abdominal pain and • 25 renal disease. subcutaneous nodules and chorea. Osler nodes (nodules on the • 28 fingers and toes). • 10 like true serum sickness. however. This patient's history is more suggestive of a drug reaction. most likely serum sickness-like reaction_ Serum sickness-like reaction is a hypersensitivity reaction that occurs one to two weeks after administration of certain drugs. and should resolve with withdrawal of the offending agent. symptoms of fever. It presents with arthralgias. This young boy's initial presentation is suggestive of an upper respiratory tract infection. erythema • 16 marginatum rash. polyarthralgia and lymphadenopathy_ The condition derives its name from its similarities to true serum • 35 sickness. Serum-sickness • 36 like reaction is a clinical diagnosis. • 38 • 39 Copyright © UWorld Last updated: [8/22/2014] • 40 • 41 • 42 End Block Feedback . blood smear and bone marrow biopsy are useful in • 21 establishing the diagnosis. amoxicillin or cefaclor in the setting of a viral illness. • 20 lymphadenopathy and joint pains. TMF-SMX and cefaclor • 8 (most classic association)_ It most commonly occurs in young children following the treatment of viral ▪ 9 infections with antibiotic agents. The onset of fever. urticarial • 34 rash. • 13 • 14 (Choice A) Rheumatic fever may occur in cases of streptococcal pharyngitis that are not appropriately • 15 treated with antibiotics. Prominent symptoms include fever. bacteremia. such as penicillin. rash and joint pain 10 days after penicillin therapy. purpuric rash of the lower extremities. splinter hemorrhages and Roth spots (retinal • 29 hemorrhages). Item: 6 of 44 V-Mark -4(1 Previous Next Lab Values. Complete blood count.

Item: 7 of 44 F' Mark Previous Next Lab Values. Neurogenic bladder [1%] • 19 C. The girl has since lived with her grandmother. A 5-year-old girl with chronic renal insufficiency is brought to the physician for a follow-up visit Since birth. Common variable immunodeficiency [1%] • 18 B. Which of the following is the most likely predisposing factor for this patient's • 14 recurrent infections? • 15 • 16 • 17 A. Vesicoureteral reflux [82%] • 26 • 27 Explanation: • 28 • 29 • 30 Vesicoureteral reflux • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 End Block Feedback . Notes Calculator. Renal scintigraphy with dimercaptosuccinic acid shows bilateral focal parenchymal • 13 scarring and blunted calyces. Posterior urethral valves [119C] • 20 • 21 ID. for which she takes trimethoprim-sulfamethoxazole daily for prophylaxis. She has a history of poor growth and mild hypertension but is otherwise developmentally 9 normal. Polycystic kidney disease [5%] • 22 E Recurrent sexual abuse [0%] • 23 • Unilateral renal agenesis [0*] • 24 • 25 • G. Her mother lost custody of the girl 3 years ago due to failure to comply with recommended treatment • 10 and prophylaxis of her infections. Examination shows mild • 11 bilateral lower-extremity edema but no other abnormalities_ Urinalysis shows mild proteinuria but no white • 12 blood cells or bacteria. she has had multiple episodes of urinary tract infections.

Vesicoureteral reflux • 10 • -1 -1 • 12 • 13 • 14 • 15 • 115 • 17 • 18 • 19 • 20 • 21 • 22 • 23 Normal Grade I Grade II Grade III Grade IV Grade V • 24 • 25 • 26 Grade Description • 27 • 28 I Into a nondilated ureter • 29 • 30 II Into the pelvis & calyces without dilation • 31 • 32 Mild to moderate dilation of the ureter. pelvis & calyces. renal pelvis & calyces. Item: 7 of 44 F'Mark -4(1 U. loss of papillary • 38 impressions ureteral tortuosity • 39 • 40 • 41 . • 33 III with minimal blunting of the fornices • 34 • 35 IV Moderate ureteral tortuosity & dilation of the pelvis & calyces • 36 • 37 Gross dilation of the ureter.P Previous Next . • 42 End Block Feedback . Lab Values. Notes Calculator.

• 24 such as hypertension and anemia. • 31 (Choice C) Posterior urethral valves are the most common cause of chronic renal insufficiency/failure in • 32 children_ This distal urinary tract obstruction can cause secondary urinary reflux but the condition affects only • 33 boys. loss of papillary V impressions: ureteral tortuosity 9 • • 10 • 11 ©LIS LEWo rld. • 18 • 19 The definitive diagnosis of VUR is made by contrast voiding cystourethrogram. and out the urethra. Recurrent and/or chronic pyelonephritis can lead to blunting of • 21 calices (calyceal clubbing) and focal parenchymal scarring. Renal ultrasound is • 20 performed to screen for hydronephrosis. Renal function should be followed by • 23 serial creatinine. One of the most common abnormalities is primary • 15 vesicoureteral reflux (VUR). Patients with severe VUR have urinary reflux from the bladder into the kidney. • 17 and the regurgitant urine causes dilation of the ureters (hydroureter) and kidneys (hydronephrosis). • 25 • 26 (Choice A) Patients with common variable immunodeficiency typically have recurrent sinopulmonary or • 27 gastrointestinal infections_ • 28 • 29 (Choice B) Neurogenic bladder can cause recurrent UTIs due to urine stasis and secondary reflux from • 30 inadequate voiding. Patients should be monitored closely for complications of chronic renal insufficiency. • 36 respiratory distress from pulmonary hypoplasia. Item: 7 of 44 Mark -<1 Previous Next Lab Values. micrognathia from • 37 oligohydramnios). Notes Calculator. • 16 bladder. Renal scintigraphy with dimercaptosuccinic acid • 22 is the preferred modality for long-term evaluation for renal scarring. and Potter faces (flattened ears/nose. Normal urine should have unidirectional flow from the kidneys. • 34 • 35 (Choice ID) Autosomal recessive polycystic kidney disease manifests in infancy as large flank masses. LLC • 12 • 13 Recurrent urinary tract infections (UTIs) in infants and children are a serious problem as they often involve • 14 the kidney and signify a congenital urinary tract anomaly. pelvis & calyces. ureters. urinary tract • 40 v • 41 • 42 End Block Feedback . ly Moderate ureterar tortuosity & dilation of the pelvis & calyces Gross dilation of the ureter. Autosomal dominant polycystic kidney disease is usually asymptomatic in childhood_ • 38 • 39 (Choice E) Although frequent sexual intercourse is a risk factor for recurrent UTIs in women. It is unlikely due to lack of other neurologic deficits in this patient.

Complications include • 28 parenchymal scarring. Definitive diagnosis is made by voiding • 29 cystourethrogram_ • 30 • 31 References: • 32 • 33 1. • 37 • 38 • 39 Copyright © Morld Last updated: [8/26/2G14] • 40 • 41 • 42 End Block Feedback . • 17 • 18 (Choice ID) Autosomal recessive polycystic kidney disease manifests in infancy as large flank masses. It is unlikely due to lack of other neurologic deficits in this patient. • 23 • 24 (Choice F) Most patients with a solitary kidney are asymptomatic. urinary tract • 22 anomalies are the principal cause in infants and children. Renal function should be followed by serial creatinine. This distal urinary tract obstruction can cause secondary urinary reflux but the condition affects only • 16 boys.• 1 Item: 7 of 44 VI/lark 3 Previous Next Lab Values. • 13 • 14 (Choice C) Posterior urethral valves are the most common cause of chronic renal insufficiency/failure in • 15 children. Autosomal dominant polycystic kidney disease is usually asymptomatic in childhood_ • 21 (Choice E) Although frequent sexual intercourse is a risk factor for recurrent UTIs in women. and Potter faces (flattened ears/nose. micrognathia from • 20 oligohydramnios). and renal insufficiency. Urinary tract infection: clinical practice guideline for the diagnosis and management of • 36 the initial UTI in febrile infants and children 2 to 24 months. is the preferred modality for long-term evaluation for renal scarring. • 35 2. such as hypertension and anemia • 8 (Choice A) Patients with common variable immunodeficiency typically have recurrent sinopulmonary or • 9 gastrointestinal infections_ • 10 • 11 (Choice B) Neurogenic bladder can cause recurrent UTIs due to urine stasis and secondary reflux from • 12 inadequate voiding. • 19 respiratory distress from pulmonary hypoplasia. • 25 • 26 Educational objective: • 27 Severe vesicoureteral reflux can cause recurrent or chronic pyelonephritis. Notes Calculator. Patients should be monitored closely for complications of chronic renal insufficiency. Summary of the AUA guideline on management of primary vesicoureteral reflux in • 34 children. hypertension.

111M 'Mark a' Previous Next Lab Values Notes Calculator Media Exhibit Bilateral vesicoureteral reflux 9 • 10 • -1 -1 • 12 • 13 • 14 Bilateral grade V • 15 vesicoureteral • 16 reflux and • 17 hydroureter • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 End Block Feedback . Item: 7 of 44 .

Notes Calculator 4 Media Exhibit 5 6 Posterior urethral valves 7 8 9 Posterior urethral valves • 10 • -1 -1 • 12 • 13 • 14 • 15 • 16 • 17 Hydro- • 18 nephrosi5 • 19 • 20 • 21 • 22 • 23 Dilated • 24 ureters • 25 • 26 • 27 Reflux • 28 • 29 • 30 • 31 Distended • 32 bladder • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 0 • 42 End Block Feedback . 3 Previous Next Lab Values. 2 Item: 7 of 44 F'Mark Ff.

LLC • 37 • 38 • 39 • 40 I • 41 • 42 . 2 Item: 7 of 44 .11M 'Mar k 3 Previous Next Lab Values Notes Calculator 4 Media Exhibit 5 6 Posterior urethral valveq 7 8 9 • 10 • 11 Hydro- • 12 nephrosis • 13 • 14 • 15 • 16 Dilated • 17 ureters • 18 • 19 • 20 • 21 Reflux • 22 • 23 • 24 Distended • 25 • 26 bladder • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 Urethra obstructed by congenital • 35 • 36 posterior urethral membrane 01.15MLEWorkl.

Fasting lipid panel [8%] • 20 E Urine culture [2%] • 21 F. infertility. 4- 5 6 A healthy 15-year-old girl comes to the physician for a routine health maintenance examination. She feels well 7 and has no concerns. history of sexually transmitted infections. ectopic pregnancy. Undetected and untreated infection can lead to pelvic inflammatory disease and its ▪ 34 associated complications (eg. Complete blood count [15%] • 17 C. 9 She has no vaginal discharge or pain. Screening is also recommended for any person with a new partner in past 2 months. All sex partners from the preceding 2 months should V • 40 • 41 • 42 End Block Feedback . The patient's body mass index is 23 kgim2. In addition. Urine toxicology screen [7%] • 22 • 23 • 24 Explanation: • 25 • 26 The purpose of screening tests is to identify individuals at increased risk for a particular disease who would • 27 benefit from diagnosis and treatment. incarceration. Notes Calculator. vaginal. and contact with sex workers. 2 of the most common sexually transmitted • 29 infections. The • 37 test can be performed on urine. or urethral specimens with similar accuracy. 1 2 Item: 8 of 44 V-Mark 3 Previous Next Lab Values. endocervical. Chtarnydia trachornatis testing [68%] • 16 B. chronic pelvic pain). • 36 The best screening test is the nucleic acid amplification test. her last menses was 1 week ago_ 8 The patient has been sexually active with one partner for the past year and takes oral contraceptive pills daily. Her menstrual cycles are regular and last 3-4 days. The • 10 patient has tried marijuana ''a few times" but does not use tobacco or alcohol_ Her parents are healthy_ Her • 11 maternal grandfather died of a myocardial infarction at age 68. She is an honors student in 10th grade and plays varsity soccer. • 31 • 32 Cer-vicitis is a common manifestation of chlamydia and gonorrhea. Patients • 38 diagnosed with infection should receive antibiotics immediately and refrain from sexual intercourse until • 39 treatment is complete and symptoms have resolved. All sexually active women age . these chlamydial • 35 and gonorrheal infections can facilitate HIV transmission. which has high sensitivity and specificity. but many patients are • 33 asymptomatic. • 12 Vital signs and physical examination are normal. Echocardiography [11C] • 18 • 19 ID. 24 should undergo testing for • 28 Chlarnyaria trachornatis and Neisseria gorrorrhoeae. What is the best next step in the evaluation of this patient? • 13 • 14 • 15 ▪ A. multiple • 30 partners. illicit drug use.

5 and gonorrheal infections can facilitate HIV transmission. 4. marijuana. echocardiography. • 14 • 15 (Choice C) Electrocardiogram. but adolescents should be counseled on the increased risk of unintentional injuries. pallor. and premature cardiac death • 18 or disability in a close relative age <50_ Routine screening is otherwise not recommended due to risk of false- • 19 positive results and lack of cost efficiency. vaginal. However. 6 The best screening test is the nucleic acid amplification test. 1 2 Item: 8 of 44 V. peiviu pairq_ in dUUILIU11. and exercise testing should be performed in athletes at • 16 risk for sudden cardiac death. abuse. irlieruilLy. dbbULIdLeU 1. fatigue.1. Notes Calculator. eL. as lipid • 22 levels are relatively stable just prior to and after puberty. • 17 or dyspnea on exertion.dLIU116 key. pregnant women should be screened for asymptomatic bacteriuria due to the increased • 28 incidence of pyelonephritis and low birth weight • 29 • 30 (Choice F) A basic drug test can detect amphetamine. but the risks of short.U1114. • 20 • 21 (Choice ID) Universal screening for dyslipidemia is recommended at age 9-11 and at age 17-21. 4. motor ▪ 34 vehicle crashes. Screening outside of these periods should occur in • 23 patients at high risk for cardiovascular disease (eg. High-risk patients include those with a history of Marfan syndrome. but routine screening is not recommended otherwise_ Random urine toxicology is generally not • 39 recommended. • 40 • 41 • 42 End Block Feedback . opioids.11.Mark -4Z1 3 Previous Next Lab Values. • 24 family history of premature coronary disease) and men age • 25 • 26 (Choice E) Routine screening for asymptomatic bacteriuria is not recommended in men and nonpregnant • 27 women. endocervical. or urethral specimens with similar accuracy_ Patients 8 diagnosed with infection should receive antibiotics immediately and refrain from sexual intercourse until 9 treatment is complete and symptoms have resolved. and phencyclidine that • 31 have been used within a few days of the test Testing may be useful if the patient is in a drug abuse • 32 rehabilitation or pain management program or is receiving psychiatric care_ Random drug screening is not • 33 recommended. and dependence.LupiL preyridriLy. The 7 test can be performed on urine. family history of cardiomyopathy or long-QT syndrome. chest pain. • 35 Educational objective: • 36 All sexually active women ages24 should be screened for Chlarnydia trachornatis and Neissena gonorriroeae • 37 by nucleic acid amplification testing. which has high sensitivity and specificity. cocaine.and-long term drug use should be discussed. Athletes with risk factors for sudden death should undergo cardiac • 38 evaluation. history of obesity/diabetes mellitus/tobacco exposure. • 11 • 12 (Choice B) Patients who have no symptoms (eg. dyspnea) and no menorrhagia do not require • 13 routine complete blood count testing. All sex partners from the preceding 2 months should • 10 also be tested and treated for infection.1MT1p114.

and-long term drug use should be discussed. Notes Calculator. Results of random drug testing in an adolescent substance abuse program. abuse. Testing may be useful if the patient is in a drug abuse • 21 rehabilitation or pain management program or is receiving psychiatric care_ Random drug screening is not • 22 recommended. and dependence. and premature cardiac death 7 or disability in a close relative age <50_ Routine screening is otherwise not recommended due to risk of false- 8 positive results and lack of cost efficiency. history of obesity/diabetes mellitus/tobacco exposure.S. and phencyclidine that • 20 have been used within a few days of the test. 1 2 Item: 8 of 44 V-Mark 3 Previous Next Lab Values. However. • 37 • 38 • 39 Copyright © LIWorld Last updated: [11/24/2014] • 40 • 41 • 42 End Block Feedback . opioids. • 33 • 34 2. USPSTF recommendations for STI screening. cocaine. marijuana. • 35 3. but the risks of short. • 24 • 25 Educational objective: • 26 All sexually active women ages 4 should be screened for Chlarnyciria trachornatis and Neissena gonorriloeae • 27 by nucleic acid amplification testing_ Athletes with risk factors for sudden death should undergo cardiac • 28 evaluation. and exercise testing should be performed in athletes at 5 risk for sudden cardiac death_ High-risk patients include those with a history of Mar-fan syndrome. as lipid • 11 levels are relatively stable just prior to and after puberty. pregnant women should be screened for asymptomatic bacteriuria due to the increased • 17 incidence of pyelonephritis and low birth weight. Screening outside of these periods should occur in • 12 patients at high risk for cardiovascular disease (eg. Screening for asymptomatic bacteriuria in adults: evidence for the U. 9 • 10 (Choice 0) Universal screening for dyslipidemia is recommended at age 9-11 and at age 17-21. chest pain. • 18 • 19 (Choice F) A basic drug test can detect amphetamine. but adolescents should be counseled on the increased risk of unintentional injuries. family history of cardiomyopathy or long-QT syndrome. 6 or dyspnea on exertion. • 13 family history of premature coronary disease) and men age • 14 • 15 (Choice E) Routine screening for asymptomatic bacteriuria is not recommended in men and nonpregnant • 16 women. Preventive • 36 Services Task Force reaffirmation recommendation statement. but routine screening is not recommended otherwise_ Random urine toxicology is generally not • 29 recommended. • 30 • 31 References: • 32 1. echocardiography. 4 (Choice C) Electrocardiogram. motor • 23 vehicle crashes.

or • 37 bone fragility (eg. la catheters can be placed manually or with a driver. osteogenesis imperfecta). and pulse is 145/min. 11. it is not • 11 possible to start a peripheral intravenous line. fracture or previous 10 attempts in the chosen extremity. • 4. blood pressure is 60/28 mm Hg. Attempt intraosseous cannulation [69'36] • 18 • 19 ID. cellulitis) overlying the access site. • 5 6 A 3-year-old girl is brought to the emergency department with lethargy and fever_ She had diarrhea for several 7 days. flat surface and distance from the • 34 sternum in case cardiopulmonary resuscitation is performed simultaneously. However. She has no allergies and takes no medications. The girl has refused liquids for the past 12 hours and has not urinated today. Attempt arterial line placement [2°4] • 16 B. any large bone can • 35 be used. and clinical trials have demonstrated 10 lines to be safer and faster than central lines. Attempt central venous catheter placement [12%] • 17 C. 10 access requires less skill and practice than central line • 29 placement. Her capillary refill time is 5 seconds centrally_ Despite numerous attempts. she is lethargic • 10 and has poor skin turgor. Contraindications to 10 placement include • 36 infection (eg. 10 • 30 catheters provide a cannula large enough to deliver fluids and medications rapidly and to obtain blood samples • 31 for laboratory testing_ • 32 • 33 The most common site for 10 access is the proximal tibia due to its wide. Her temperature is 397 C (103. and her parents say that "she suddenly took a turn for the worse''. and the child's condition continues to deteriorate. When intravenous access cannot be obtained in emergency cases. • 38 (Choice A) Arterial lines are used for continuous blood pressure monitoring and to draw laboratory studies. Notes Calculator. On examination. Transport to intensive care unit for central venous catheter placement [15%] • 22 • 23 • 24 Explanation: • 25 • 26 This child's presentation is concerning for hypovolemic or septic shock and requires emergency fluid • 27 resuscitation.5 F). Attempt peripheral intravenous line placement [116] • 21 F. Which of the • 12 following is the best next step in management of this patient? • 13 • 14 • 15 A. 1 • 2 Item: 9 of 44 F' Mark • 3 Previous Next Lab Values. Attempt nasogastric tube placement [1%] • 20 E. • 39 They are not used for fluid resuscitation and should be placed after the patient has been resuscitated as the V • 40 • 41 • 42 End Block Feedback . intraosseous (10) • 28 access should be attempted immediately.

any large bone can 5 be used_ 10 catheters can be placed manually or with a driver_ Contraindications to 10 placement include 6 infection (eg.14. or 7 bone fragility (eg. A central line can be placed later if necessary when the patient is stable... cellulitis) overlying the access site.nallual 1. (Choice A) Arterial lines are used for continuous blood pressure monitoring and to draw laboratory studies_ • 10 They are not used for fluid resuscitation and should be placed after the patient has been resuscitated as the • 11 procedure takes time and great skill_ • 12 • 13 (Choices B and F) Central catheters take longer to place than la lines and require a higher amount of • 14 procedural skill and practice_ This child needs immediate access for fluid resuscitation. II UI II LI 14-. absorption of enteral fluids will not be rapid enough to correct the patient's hypovolemia_ • 20 • 21 (Choice E) Several attempts at peripheral intravenous insertion have already failed_ Given that the child is in • 22 uncompensated shock. when the splanchnic circulation is constricted_ • 19 At this point.. r... LU ILA L..JLE-. Intraosseous versus intravenous vascular access during out-of-hospital cardiac arrest: a • 36 randomized controlled trial. 1 2 Item: 9 of 44 V-• Mark 3 Previous Next Lab Values. they are not recommended in shock. Notes Calculator. • 32 • 33 2.. I LI 14-.14-. I I 14-. • 16 • 17 (Choice 0) Although nasogastric fluids are preferred for mild to moderate dehydration associated with • 18 gastrointestinal disease. 4. fracture or previous la attempts in the chosen extremity. • 37 • 38 • 39 Copyright © UWorld Last updated: [8/27/2014] • 40 • 41 • 42 End Block Feedback . However.-. and transporting her • 15 within the hospital will cause delays.. I I 4.1 4.4-01111114-011 . • 23 • 24 Educational objective: • 25 lntraosseous lines can be placed rapidly when emergency access is necessary and peripheral access cannot • 26 be obtained_ lntraosseous access can be performed with less required skill and practice than central venous • 27 access_ • 28 • 29 References: • 30 1.11.11 6. osteogenesis imperfecta). Comparison of intraosseous versus central venous access in adults under resuscitation • 34 in the emergency department with inaccessible peripheral veins • 35 3. access should be escalated to 10 placement. Comparison of umbilical venous and intraosseous access during simulated neonatal • 31 resuscitation.J11-4-. A sternum in case cardiopulmonary resuscitation is performed simultaneously.11411.111.

but was a little dazed after being hit However. The incident occurred 7 about two hours ago_ He had no loss of consciousness. vomited twice. the patient began to complain of a headache. he 8 seemed to improve after a short time. • 11 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 se • 41 • 42 End Block Feedback . 1 2 Item: 10 of 44 F' Mark 3 Previous Next Lab Values. 4- 5 6 A 5-year-old boy is brought to the physician after he was hit in the head with a baseball. Notes Calculator. and is currently not acting right according to his parents_ On examination. About 30 minutes ago. the child is sleepy and his left pupil is larger than his right A CT scan of his head is shown below.

seizures. 1 2 Item: 10 of 44 Mark 3 Previous Next Lab Values. The patient in this vignette has pupillary abnormalities. signs of increased ICF. • 40 se • 41 • 42 End Block Feedback . Repeat CT in 24 hours [2%] • 19 ID. which • 37 include any of the following: GCS < 8. confusion. Older children and adolescents usually present with more classic • 33 symptoms of headache. The key to evaluating a child with an epidural • 36 hematoma is to search for the presence of clinical features that necessitate an emergent craniotomy. and should therefore have a neurosurgical evaluation for an emergent craniotomy. IDexamethasone [6%] • 20 • 21 E Emergent craniotomy [89%] • 22 • 23 Explanation: • 24 • 25 Epidural hematomas are usually caused by injury to meningeal blood vessels. and a decreased level of • 39 consciousness.Thich of the following is the most appropriate next step in management? • 14 • 15 A. vomiting. Notes Calculator. and do not usually cross suture • 26 lines because of the better dural attachment at the sutures. or • 38 cerebellar signs. vomiting. The classic history of an epidural hematoma • 27 consists of a direct head injury followed by a lucid interval and then rapid neurologic deterioration with • 28 headache. hemiparesis. Affected patients can progress to coma and death • 29 due to herniation if not treated. and deteriorating mental status_ • 34 • 35 A CT scan of the head is the diagnostic test of choice. 4- 5 6 7 8 • 11 • 12 • 13 V. pupillary abnormalities. vomiting. Cerebral angiogram [1%] • 16 MRI of the brain [1%] • 17 • 18 C. • 32 irritability. • 30 • 31 Physical examination findings vary by age_ Infants with open fontanelles may present with bulging fontanelles. and lethargy. seizures. and hypotonia.

and patients with features suggestive of • 32 deteriorating neurological status or increased IP require an emergent craniotomy. Ilfle6 UeLdU6e UI Lilt UeLLel UUldl dadLAIHIefIL dL Lilt 6ULUE e6. riibLury UI dfl epiuurdi fief EldLUElld 5 consists of a direct head injury followed by a lucid interval and then rapid neurologic deterioration with 6 7 headache. pupillary abnormalities. This patient. • 37 • 38 • 39 Copyright © LIWorld Last updated: [12/29/2014] • 40 • 41 • 42 End Block Feedback . and deteriorating mental status. I He L-Id6611. The patient in this vignette has pupillary abnormalities. has a clear mechanism of injury resulting in his • 20 epidural hematoma. however. Older children and adolescents usually present with more classic symptoms of headache. • 27 • 28 (Choice ID) Steroids have no role in the acute management of an epidural hematoma. and lethargy. seizures. vomiting. The key to evaluating a child with an epidural • 13 hematoma is to search for the presence of clinical features that necessitate an emergent craniotomy. • 17 • 18 (Choice A) An angiogram may be helpful when an epidural hematoma is suspected to be secondary to an • 19 arteriovenous malformation (AVM).• 1 • 2 Item: 10 of 44 V-Mark -4Z1 3 Previous Next Lab Values. Notes Calculator. • 29 Educational objective: • 30 Epidural hematomas are often associated with a lucid interval followed by rapid neurological deterioration_ • 31 They appear as a biconvex mass on CT scan of the head. which delays appropriate intervention. Physical examination findings vary by age_ Infants with open fontanelles may present with bulging fontanelles. • 24 • 25 (Choice C) In patients with no neurologic signs and a small epidural hematoma. and a decreased level of • 16 consciousness. hemiparesis. irritability. seizures. signs of increased ICF. • 33 • 34 • 35 References: • 36 1. or • 15 cerebellar signs. which • 14 include any of the following: GCS < 8. vomiting. confusion. vomiting. • 11 • 12 A CT scan of the head is the diagnostic test of choice. a follow-up CT in 24 hours • 26 may be appropriate. making an AVM extremely unlikely_ • 21 • 22 (Choice B) A MRI of the brain is not useful in the acute evaluation of an epidural hematoma because it takes • 23 too long to obtain. and should therefore have a neurosurgical evaluation for an emergent craniotomy. and hypotonia. Affected patients can progress to coma and death due to herniation if not treated. 4. Extradural haematomas in children: a 10-year review.

Narcissistic personality disorder [2%] • 18 E Antisocial personality disorder [4%] • 19 • 20 • 21 Explanation: • 22 Occurring more frequently in males. Tourette syndrome is characterized by multiple motor and one or more • 23 vocal tics that present before the age of 18.. but attention deficit • 37 hyperactivity disorder and obsessive-compulsive disorder are more common comorbid conditions.. • 38 • 39 Educational Objective: • 40 ID afro ni-c. The tics occur many times a day (frequently in bouts) nearly every • 24 day or at regular intervals for at least one year. and impulse • 33 control disorders. and shoulder shrugging. Less common comorbid conditions include anxiety. Obsessive-compulsive disorder (O ID) develops • 31 within 3-6 years after the tics first appeared. C. clears his throat. coprolalia may be observed_ Symptoms are • 27 exacerbated by stress and tend to subside during sleep_ • 28 • 29 • Frequent comorbid conditions in this patient population include attention deficit hyperactivity disorder (60 • 30 percent) and obsessive-compulsive disorder (27 percent). D and E) There is an increased incidence of conduct disorder. The boy has an ▪ 7 eight-year history of episodes in which he utters strange sounds. The motor tics frequently observed include grimacing.. grunting. His medical history is ▪ 9 otherwise unremarkable and he is earning passing grades in school. Obsessive-compulsive disorder [81%] • 15 C. Physical examination is unremarkable_ • 10 Which of the following disorders is most likely to develop in this boy? • 11 • 12 • 13 A. at en a c i n n if i r a ni-hr inr rna c nr-1 ri c lr of tint en lnninn nn r if H1 ma ran fiariizr e s • 41 • 42 End Block Feedback . Recently he has also started to jerk his head during these episodes as well. coughing.• 1 • 2 Item: 11 of 44 VI/lark ▪ 3 Previous Next Lab Values. and blinks his ▪ 8 eyes. • 4- ▪ 5 ▪ 6 A 12-year-old boy is brought to the pediatrician by his mother for a routine examination. It may peak in late adolescence or in early adulthood at a time • 32 when the tics are waning. head jerking. Oppositional defiant disorder [6%] • 17 ID. • 34 • 35 (Choices A.nr-4rnrna F. grimaces. Notes Calculator. oppositional defiant disorder. depression. Conduct disorder [71C] • 14 ko B. eye • 25 blinking. • 36 and antisocial personality disorder in patients suffering from Tourette syndrome. nose twitching. The vocal tics include barking. 1* rifin Tni i rai-Fa c. and throat clearing_ In a minority of cases. • 26 squeaking.

• 1 • 2 Item: 11 of 44 V-• Mark -4Z1 ▪ 3 Previous Next Lab Values.113LiLIC3 II 1 VV1114. C. It may peak in late adolescence or in early adulthood at a time • 29 when the tics are waning.. coughing. coprolalia may be observed_ Symptoms are • 24 exacerbated by stress and tend to subside during sleep_ • 25 • 26 Frequent comorbid conditions in this patient population include attention deficit hyperactivity disorder (&J • 27 percent) and obsessive-compulsive disorder (27 percent). Less common comorbid conditions include anxiety. Conduct disorder [7%] • 10 B.. Notes Calculator. eye • 22 blinking. Tourette syndrome is characterized by multiple motor and one or more • 20 vocal tics that present before the age of 18.111111‘3 1113 • 4. • 23 squeaking. Obsessive-compulsive disorder [81%] • 11 • 12 C. Physical examination is unremarkable_ ▪ 6 ▪ 7 Which of the following disorders is most likely to develop in this boy? ▪ 8 ▪ 9 A.11 11C ULLC13 3L1 01 iyc 3LJUI 1413. and shoulder shrugging. I. CII1U L. head jerking. nose twitching.. • 34 • 35 Educational Objective: • 36 Patients with Tourette syndrome have a significantly increased risk of developing attention deficit hyperactivity • 37 disorder or obsessive-compulsive disorder. and throat clearing_ In a minority of cases. The motor tics frequently observed include grimacing. A 5 eyes. Obsessive-compulsive disorder (O D} develops • 28 within 3. His medical history is otherwise unremarkable and he is earning passing grades in school. oppositional defiant disorder.ICC!! 3 1113 L111 LiCIL. D and E) There is an increased incidence of conduct disorder.GJ . depression. The vocal tics include barking. The tics occur many times a day (frequently in bouts) nearly every • 21 day or at regular intervals for at least one year. grunting.. yi II I IC74. and impulse • 30 • control disorders_ • 31 (Choices A. Antisocial personality disorder [4%] • 15 • 17 Explanation: • 18 • 19 Occurring more frequently in males. Recently he has also started to jerk his head during these episodes as well. ciyi 1113LLII y UI Cl. but attention deficit • 33 hyperactivity disorder and obsessive-compulsive disorder are more common comorbid conditions. Oppositional defiant disorder [65. • 38 • 39 Copyright © LIWorld Last updated: [1611212014] • 40 • 41 • 42 End Block Feedback . • 32 and antisocial personality disorder in patients suffering from Tourette syndrome. Narcissistic personality disorder [2%] • 14 E.6] • 13 ID.years after the tics first appeared.

• 1 • 2 Item: 12 of 44 V. Chest physiotherapy [0%] • 20 D. • 11 Wheezing is heard in the right lung field. blood pressure is 92148 9 mm Hg. Chest computed tomography scan [3%] • 19 C. Notes Calculator. FBA is most common in children age 1-3 • 33 years. Supplementary oxygen is applied. pulse is 1141min. but his 8 s-year old brother has a peanut allergy. Chest tube placement [1%] • 21 • 22 E Intramuscular epinephrine [4%] • 23 F. Bronchoscopy [81%] • 17 • 18 B. The patient's temperature is 3E7 C (98 F). laryngeal and tracheal FBs are far less common_ • 35 • 36 Clinical features of FBA are shown in the table. • 37 • 38 • 39 Clinical manifestations of foreign body aspiration V • 40 • 41 • 42 End Block Feedback . this child's clinical • 31 presentation of sudden-onset respiratory distress without a preceding illness and focal findings on pulmonary • 32 examination is most consistent with foreign body aspiration (FBA). Which of the following is the most appropriate next step in management of this child? A. More than • 34 half of aspirated FBs end up in the right mainstem bronchus. the left field is clear to auscultation. The patient's pulse oximetry shows 91% on room air_ • 10 Physical examination shows nasal flaring and grunting with both subcostal and intercostal retractions. No rales or rhonchi are noted. Nebulized albuterol [7%] • 24 G. • 4- 5 ▪ 6 A 2-year-old boy with cough and difficulty breathing is brought to the emergency department by his mother_ ▪ 7 She says that he was well and playing with his toys until 2 hours prior to presentation. The remainder of the physical examination is within normal limits. He is healthy. and respirations are 48/min.Mark ▪ 3 Previous Next Lab Values. Chest x-ray reveals mild hyperinflation of the right lung. Commonly aspirated FBs include foods such as peanuts and popcorn and pieces of toys. Racemic epinephrine [3%] • 25 • 26 • 27 Explanation: • 28 • 29 Respiratory distress is one of the most common presenting chief complaints in the pediatric emergency • 30 department Although the differential diagnosis for respiratory distress is broad.

• 39 • 40 The standard of care for both diagnosis and treatment of FBA is immediate bronchoscopy. History of choking (80%-90% of cases) • 10 . they are normal in • 35 approximately 2/3 of cases given that most aspirated objects are radiolucent. with air trapping during expiration. -1 2 Item: 12 of 44 I'Mark U. .. A 5 6 Clinical manifestations of foreign body aspiration 7 8 9 . and/or localized bronchiectasis (late • 30 feature). In contrast. • 41 • 42 End Block Feedback . Visualization of foreign body • 30 • 31 USN1L EWorld. 4.P 3 Previous Next Lab Values. post-obstructive pneumonia. t LC • 32 • 33 • 34 Although chest radiographs are often obtained in patients with suspected FBA. • 27 • 28 Radiographic . If an FE causes partial • 36 obstruction. complete • 37 obstruction can result in atelectasis. hyperinflated lungs are seen on imaging. Notes Calculator. Coughing • 11 Signs & •• Sudden-onset respiratory distress symptoms • 13 •• Cyanosis • 14 6 15 . Altered mental status • 17 • 18 • Focal monophonic wheezing on affected side • 19 i Diminished aeration on affected side • 20 Physical • 21 • Generalized wheezing examination • 22 • Inspiratory stridor • 23 findings • 24 • Hoarseness • 25 • Respiratory distress • 26 . Hyperinflation or atelectasis of affected side • 29 findings .

gastrointestinal symptoms (eg. hyperinflated lungs are seen on imaging. his clinical presentation is not consistent with • 34 anaphylaxis. • 23 • 24 (Choice C) Chest physiotherapy is helpful in removing tenacious secretions and mucous plugs. with air trapping during expiration. If an FE causes partial obstruction.1c. as seen • 37 with asthma. . Anaphylaxis typically • 31 presents with respiratory distress. which typically • 41 • 42 End Block Feedback .ILC Although chest radiographs are often obtained in patients with suspected FBA. 1 2 Item: 12 of 44 F' Mark U. I 11%. • 39 • 40 (Choice G) Nebulized racemic epinephrine is used for laryngotracheobronchitis (croup). and/or localized bronchiectasis (late • 18 feature}_ • 19 • 20 The standard of care for both diagnosis and treatment of FBA is immediate bronchoscopy. and • 33 hypotension. crampy abdominal pain.JSMILEWorld. swollen lips and tongue). involvement of the skin-mucosal tissue (eg. complete • 17 obstruction can result in atelectasis.P 3 Previous Next Lab Values. • 29 • 30 (Choice E) Intramuscular epinephrine is the first-line treatment for anaphylaxis. itching. or pleural effusion_ It is not indicated in this patient. they are normal in approximately 2/3 of cases given that most aspirated objects are radiolucent. generalized hives. a scan is • 22 not indicated as it requires patient cooperation and only delays the diagnosis. post-obstructive pneumonia. • 26 • 27 (Choice 0) Chest tube placement is indicated for patients with respiratory distress secondary to a large • 28 pneumothorax. Eronchodilators are not helpful in patients with FBA given that the underlying etiology is a fixed • 38 obstruction in the bronchus. vomiting).111 4. It is • 25 indicated in patients with bronchiectasis. Although the patient's sibling has a peanut allergy. A 5 ▪ Respiratory distress 6 7 8 Radiographic • Hyperinflation or atelectasis of affected side 9 findings • Visualization of foreign body • 10 • 11 1. • 32 flushing. In contrast. • 21 (Choice B) Although an aspirated FE may be seen on computed tomography scan of the chest. hemothorax. Notes Calculator. • 35 • 36 (Choice F) Although this patient has wheezing on examination. it is focal rather than generalized.

Indications for flexible versus rigid bronchoscopy in children with suspected • 31 foreign-body aspiration. which typically • 19 presents with mild upper respiratory symptoms. Tracheobronchial aspiration of foreign bodies and rigid bronchoscopy in children. Notes Calculator. gastrointestinal symptoms (eg. Foreign body aspiration in children: Experience from 2624 patients. ▪ 5 (Choice ID) Chest tube placement is indicated for patients with respiratory distress secondary to a large ▪ 7 pneumothorax. or pleural effusion_ It is not indicated in this patient 8 (Choice El Intramuscular epinephrine is the first-line treatment for anaphylaxis_ Anaphylaxis typically • 10 presents with respiratory distress. if witnessed. and hypotension_ Although the patient's sibling has a peanut allergy.• 1 • 2 Item: 12 of 44 F' Mark ▪ 3 Previous Next Lab Values. crampy abdominal pain. • 36 4. • 34 • 35 3. Extraction of tracheobronchial foreign bodies in children and adults with rigid and • 33 flexible bronchoscopy. it is focal rather than generalized. involvement of the skin-mucosal tissue (eg. vomiting). • 4 indicated in patients with bronchiectasis. • 32 2. hemothorax. a "barky'r cough. is very helpful in diagnosis_ Immediate bronchoscopy is indicated to remove the foreign • 26 body_ • 27 • 28 References: • 29 • 30 1. • 11 flushing. as seen with asthma. and inspiratory stridor. This child has none • 20 of these_ • 21 • 22 Educational objective: • 23 Sudden-onset respiratory distress in a toddler with focal findings on physical examination is most consistent • 24 with foreign body aspiration_ Chest x-ray is of limited help as most objects are radiolucent A history of • 25 choking. his clinical presentation is not consistent with anaphylaxis_ (Choice F) Although this patient has wheezing on examination. itching. generalized hives. Eronchodilators are not helpful in patients with FBA given that the underlying etiology is a fixed • 17 obstruction in the bronchus_ • 18 (Choice G) Nebulized racemic epinephrine is used for laryngotracheobronchitis (croup). • 37 • 38 • 39 Copyright © LIWorld Last updated: [1G/13/2014] • 40 • 41 • 42 End Block Feedback . swollen lips and tongue).

Notes Calculator. and family histories are ▪ 9 unremarkable. • 4 ▪ 5 ▪ 6 7 A 1-day-old boy is in the newborn nursery with persistent feeding difficulty. He is voiding normally and passed meconium. Examination shows a non-dysmorphic boy with clear lungs and no murmurs_ There are • 11 no intercostal retractions or nasal flaring. His peripheral pulses are full and symmetric. Which of the following • 12 is the most likely diagnosis? 13 • 14 • 15 A. Tracheoesophageal fistula with esophageal atresia [14N • 20 E. Choanal atresia [6416] B. Vocal cord paralysis [0%] • 22 • 23 • 24 Explanation: • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 ▪ 38 • 39 • 40 se 41 42 End Block Feedback . birth. His prenatal. Vital signs are normal and 4-extremity blood • 10 pressures are equal. Tetralogy of Fallot [8%] • 18 • 19 D. He is cyanotic and short of breath ▪ 7 when he breastfeeds but turns pink when he cries. Transient tachypnea of the newborn [5%] • 21 F. Laryngomalacia [996] • 17 C. His weight is average for gestational age.• 1 • 2 Item: 13 of 44 F' Mark ▪ 3 Previous Next Lab Values. Feeding him expressed breast milk from a bottle results in ▪ 8 the same findings.

and Ear abnormalitiesIdeafness). Renal • 35 anomalies. 1 . Bilateral obstruction classically presents with cyclic cyanosis that worsens when • 39 the infant cannot breathe through the nose during feeding but recovers during crying. Notes Calculator. Unilateral choanal • 40 rema in i inriinnnQari !Intl! the infant riaidolnnQ his nr her first i inner raQnirtnnf infortinn so • 41 • 42 Feedback End Block A . Heart Defects. leaving either a bony (90%) or membranous (10%) obstruction_ The condition may be isolated or • 34 part of a syndrome (ie. Growth impairment. • 36 • 37 The clinical severity depends on the infant's ability to breathe through the mouth and whether 1 or both • 38 I choanae are obstructed. 4 I A 5 6 7 7 8 1 9 • 10 M : h 11 2 • 14 • 15 • 11151 • 17 • 18 I • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 Choanal atresia should be suspected in a newborn with cyanosis that is aggravated by feeding and relieved • 32 by crying_ The congenital nasal malformation is caused by failure of the posterior nasal passage to canalize • 33 completely. CHARGE syndrome: Coloboma. Atresia of the choanae.• 1 • 2 Item: 13 of 44 F'Mark 3 Previous Next Lab Values.

• 21 (Choice C) Patients who have Tetralogy of Fallot (TOF) with mild obstruction to pulmonary blood flow are • 22 asymptomatic at rest and become cyanotic when stressed. air-fluid levels (red arrow) may develop at the • 15 obstruction site_ The first step in management consists of placing an oral airway and gavage feeding_ • 11151 Definitive treatment involves repairing the obstruction with surgery or endoscopy. Infants may • 37 have unilateral or bilateral paralysis and generally have stridor and respiratory distress_ It cannot be clinically • 38 I differentiated from laryngomalacia. Symptoms appear within the first few weeks of life. making TOF diagnosis unlikely. respiratory distress. which shows a narrowing at the level of the pterygoid plate in the • 14 posterior nasal cavity (green arrows). subcostallintercostal retractions and expiratory • 34 grunting. and Ear abnormalities/deafness). Atresia of the choanae.Mark 3 Previous Next Lab Values. this infant has no stridor or tachypnea. the cyanosis in choanal atresia • 24 is not triggered by stress but by anything that will keep the infant's mouth closed (ie. • 17 • 18 I (Choice B) Laryngomalacia classically presents with inspiratory stridor that is exacerbated by exertion or • 19 distress. 1 . • 40 so • 41 • 42 Feedback End Block A . CHARGE syndrome: Coloboma. 4 I part of a syndrome (ie. and adventitious lung sounds in addition to cyanosis. However. direct visualization of the upper airway via endoscope is necessary. and this infant has no stridor or tachypnea. These patients typically have • 30 coughing. Growth impairment. Unilateral choanal • 10 M atresia may remain undiagnosed until the infant develops his or her first upper respiratory infection. In severe cases. Heart Defects. Bilateral obstruction classically presents with cyclic cyanosis that worsens when 9 the infant cannot breathe through the nose during feeding but recovers during crying. • 31 • 32 (Choice E) Transient tachypnea of the newborn is characterized by tachypnea immediately after birth that is • 33 unrelated to crying. • 39 Cyanosis is uncommon unless the obstruction is severe. The diagnosis is confirmed by CT scan (Image). • 23 which may be confused with the cyanosis seen in choanal atresia.• 1 • 2 Item: 13 of 44 V. Additional symptoms include nasal flaring. • 25 Patients with TOF will also have a systolic ejection murmur of pulmonary stenosis and holosystolic murmur of • 26 ventricular septal defect These are not seen in this patient. : h 11 2 Failure to pass a catheter through the nose into the oropharynx is suggestive of choanal atresia. Cyanosis is uncommon unless the obstruction • 20 is severe_ However. • 27 • 28 (Choice 0) Tracheoesophageal fistula with esophageal atresia can cause feeding problems immediately • 29 after birth as feeds cannot pass the esophagus and end up in the airway. Renal 5 anomalies. 6 7 7 The clinical severity depends on the infant's ability to breathe through the mouth and whether 1 or both 8 1 choanae are obstructed. These desaturations are known as "ter spells. • 35 • 36 (Choice F) Vocal cord paralysis is associated with birth injury or central nervous system insults. Notes Calculator. during feedings).

Choanal atresia: current concepts and controversies • 36 I 2. this infant has no stridor or tachypnea. • 26 I • 27 Educational objective: • 28 I Choanal atresia should be suspected in an otherwise well-appearing infant with intermittent cyanosis and I • 29 distress during feeding that is relieved by crying. (Choice E) Transient tachypnea of the newborn is characterized by tachypnea immediately after birth that is • 19 unrelated to crying. subcostallintercostal retractions and expiratory • 20 grunting. A CT scan shows narrowing at the level of the pterygoid plate in • 31 the posterior nasal cavity. Item: 13 of 44 'Mark 3 Previous Next Lab Values. These desaturations are known as 'let" spells. Symptoms appear within the first few weeks of life. Failure to pass a catheter through the nose into the • 30 oropharynx is suggestive of the diagnosis. making TOF diagnosis unlikely. respiratory distress. Additional symptoms include nasal flaring. and adventitious lung sounds in addition to cyanosis. • 14 • 15 (Choice ID) Tracheoesophageal fistula with esophageal atresia can cause feeding problems immediately after birth as feeds cannot pass the esophagus and end up in the airway. 4 I (Choice B) Laryngomalacia classically presents with inspiratory stridor that is exacerbated by exertion or 5 distress. and this infant has no stridor or tachypnea. 1 i 2. the cyanosis in choanal atresia is not triggered by stress but by anything that will keep the infant's mouth closed (ie. Notes Calculator. direct visualization of the upper airway via endoscope is necessary_ • 25 Cyanosis is uncommon unless the obstruction is severe. However. • 11:1 M which may be confused with the cyanosis seen in choanal atresia. 7 8 1 (Choice C) Patients who have Tetralogy of Fallot (TOF) with mild obstruction to pulmonary blood flow are 9 asymptomatic at rest and become cyanotic when stressed. However. during feedings). Cyanosis is uncommon unless the obstruction 6 7 is severe. Nasal obstruction in newborns • 37 • 38 I • 39 Copyright © LIWorld Last updated: [9/4/2014] • 40 I • 41 • 42 End Block Feedback . Infants may • 23 have unilateral or bilateral paralysis and generally have stridor and respiratory distress_ It cannot be clinically • 24 differentiated from laryngomalacia. : h 12 1 Patients with TOF will also have a systolic ejection murmur of pulmonary stenosis and holosystolic murmur of ventricular septal defect These are not seen in this patient. These patients typically have • • • 11151 17 18 I coughing. • 21 • 22 (Choice F) Vocal cord paralysis is associated with birth injury or central nervous system insults. • 32 • 33 References: • 34 • 35 1.

He has a history 8 of numerous sinus infections and episodes of cellulitis. Opsonization defect [3%] • 38 • 39 • 40 Explanation: V • 41 • 42 End Block Feedback .DDD!pL • 19 Neutrophils 90% • 20 Lymphocytes 10% • 21 Monocytes 2% • 22 • 23 Nitroblue tetrazolium test is normal. Impaired leukocyte adhesion [16%] • 36 • 37 H. Defective formation of mature 13 lymphocytes [5%] • 31 D. • 12 • 13 Laboratory results are as follows: Complete blood count • 15 Hemoglobin 11.8 gfdL • 16 Hematocrit 36% • 17 Platelets 24G. His mother also notes that his umbilical cord did not fall off until 5 • 10 weeks after birth_ On physical examination. the child has a severe. with previous cultures isolating Staphylococcus 9 aureus and Pseudornonas aerugirrosa.COGipL • 18 Leukocyte count 36. Defective formation of mature T lymphocytes [2%] • 32 • 33 E Defective intracellular killing [6%] • 34 • Development defect of the fourth pharyngeal pouch [1%] • 35 G. necrotic periodontal infection with • 11 ulceration. Adenosine deaminase deficiency [4%] • 28 • 29 B. • 24 • 25 Which of the following defects is most likely present in this patient? • 26 • 27 A. Notes Calculator. 3 =1 1 4 Item: 14 of 44 F Mark Previous Next Lab Values. 5 6 7 A 3-year-old boy is brought to the emergency department because of severe oral pain along with bleeding and irritation of his gums_ His symptoms began 2 weeks ago and have progressively worsened. Complement deficiency [3%] • 30 C.

Delayed umbilical cord separation (> 30 days) .Absence of bus formation at sites of infection .Recurrent skin & mucosa) bacterial infections • 16 ▪ Periodontitis. an essential component of certain integrins present on the surface of . • • 25 27 • 28 This patient with recurrent bacterial infections. 1 Item: 14 of 44 -461 II 7 Mark 3 Previous Next Lab Values.33 the complete absence of neutrophils in inflamed or infected tissues. . • 38 Laboratory studies show severe lymphopenia.Cultures often show S oureus & Gram-negative bacilli • 22 • 23 . C. particularly v • 41 • 42 Feedback. and D) Adenosine deaminase deficiency is an autosomal recessive form of severe combined i 36 immunodeficiency that leads to deficient formation of mature B and T lymphocytes due to toxic accumulation • of adenosine_ Most patients present with life-threatening infections. often necrotizing • 17 • 18 • 19 Laboratory • 20 ▪ Leukocytosis with neutrophil predominance • 21 . and a necrotic periodontal • 29 infection is most likely suffering from leukocyte adhesion deficiency type I. A Manifestations of 6 ▪ 7 leukocyte adhesion deficiency type I • 8 • 9 Clinical . delayed umbilical cord separation. LLC Fl.Biopsy of infected tissue shows inflammatory infiltrate • 24 devoid of neutrophils USPALEWor Id.31 leukocytes. End Block . failure to thrive. and persistent diarrhea. Notes Calculator. • 34 • (Choices A. This results in leukocytosis with a neutrophil predominance and . • 39 • 40 (Choice 13) Patients with complement deficiencies are at increased risk of bacterial infections. It is characterized by the inability of leukocytes (particular neutrophils) to exit the vasculature and • 32 migrate to areas of infection or inflammation.Poor wound healing • 15 . This syndrome is caused by • 30 deficient expression of CD18.

Notes Calculator.ri tissups • 41 • 42 End Block Feedback . which is characterized by T-cell deficiency_ DiGeorge syndrome typically has • 29 other associated physical examination findings. In this condition. and D) Adenosine deaminase deficiency is an autosomal recessive form of severe combined • 16 immunodeficiency that leads to deficient formation of mature 13 and T lymphocytes due to toxic accumulation • 17 of adenosine_ Most patients present with life-threatening infections. • 35 • 36 Educational objective: • 37 Patients with leukocyte adhesion defect type 1 suffer from delayed separation of the umbilical cord. recurrent • 38 bacterial infections of the skin and mucosal surfaces. L I C. It is characterized by the inability of leukocytes (particular neutrophils) to exit the vasculature and • 12 migrate to areas of infection or inflammation. and Neisseria • 22 meningitides_ They are also at risk for autoimmune conditions.• 1 • 2 Item: 14 of 44 rMark ▪ 3 Previous Next Lab Values. delayed umbilical cord separation. particularly • 21 with encapsulated bacteria such as Streptococcus pneumoniae. which is required for the clearance of encapsulated organisms. and a necrotic periodontal ▪ 9 infection is most likely suffering from leukocyte adhesion deficiency type I. including facial and cardiac anomalies along with thymic • 30 hypoplasia. • 26 • 27 (Choice F) Developmental defects in the pharyngeal arch system (including the fourth pharyngeal pouch) are • 28 seen in DiGeorge syndrome. and necrotic periodontal infections_ Additional findings • 39 include leukocytosis with a neutrophil predominance and the complete absence of neutrophils in inflamed or • 40 infp. • 4. This • 34 patient has no history of infection by encapsulated organisms. C. This syndrome is caused by • 10 deficient expression of CD18. • 31 • 32 (Choice H) Opsonization defects can result from asplenia. the classic nitroblue tetrazolium test is negative • 25 (abnormal). Haernophilus influenza. an essential component of certain integrins present on the surface of • 11 leukocytes. as the spleen plays a crucial role in the • 33 production of opsonizing antibody. and persistent diarrhea. failure to thrive. This results in leukocytosis with a neutrophil predominance and • 13 the complete absence of neutrophils in inflamed or infected tissues_ • 15 (Choices A.nte. The dihydrorhodamine 123 test is more sensitive and can quantify the severity of illness. • 18 Laboratory studies show severe lymphopenia_ • 19 • 20 (Choice B) Patients with complement deficiencies are at increased risk of bacterial infections. • 23 (Choice E) Chronic granulomatous disease is characterized by defective intracellular killing due to impaired • 24 respiratory burst from activated phagocytes. ▪ 7 ▪ 8 This patient with recurrent bacterial infections. devoid of neutrophils A ▪ 5 ▪ 6 LISMLEftrIel.

ui I lUIULIUI I • 4- of adenosine. particularly 8 with encapsulated bacteria such as Streptococcus pneurnomae. The dihydrorhodamine 123 test is more sensitive and can quantify the severity of illness.11. Leukocyte adhesion deficiency type 1: an important consideration in the clinical • 35 differential diagnosis of prepubertal periodontitis. and persistent diarrhea. including facial and cardiac anomalies along with thymic • 18 hypoplasia. A case report and review of the • 36 literature. at. Periodontal manifestation of leukocyte adhesion deficiency type I.11 I I !ULU! c 1_7 ai IU I iyi i !pi iuti. which is characterized by T-cell deficiency DiGeorge syndrome typically has • 17 other associated physical examination findings. • 29 • 30 References: • 31 1.. • 10 • 11 (Choice El Chronic granulomatous disease is characterized by defective intracellular killing due to impaired • 12 respiratory burst from activated phagocytes. failure to thrive. • 19 • 20 (Choice H) Opsonization defects can result from asplenia. the classic nitroblue tetrazolium test is negative • 13 (abnormal). • 34 3.. • 37 • 38 • 39 Copyright © UWorld Last updated: [8/10/2014] • 40 • 41 • 42 End Block Feedback . recurrent • 26 bacterial infections of the skin and mucosal surfaces. This • 22 patient has no history of infection by encapsulated organisms.ici IL iui I I IULIUI I 1. 5 Laboratory studies show severe lymphopenia_ 6 7 (Choice B) Patients with complement deficiencies are at increased risk of bacterial infections. and Neisseria 9 meningitides_ They are also at risk for autoimmune conditions.. • 15 (Choice F) Developmental defects in the pharyngeal arch system (including the fourth pharyngeal pouch) are • 16 seen in DiGeorge syndrome.3 Lu uciit. as the spleen plays a crucial role in the • 21 production of opsonizing antibody. Hematologically important mutations: leukocyte adhesion deficiency.• 1 • 2 Item: 14 of 44 V-Mark 3 Previous Next Lab Values. which is required for the clearance of encapsulated organisms. Haernophifus influenza. JLc 3 uuu w LUP!. and necrotic periodontal infections_ Additional findings • 27 include leukocytosis with a neutrophil predominance and the complete absence of neutrophils in inflamed or • 28 infected tissues. • 32 • 33 2. Notes Calculator. • 23 • 24 Educational objective: • 25 Patients with leukocyte adhesion defect type 1 suffer from delayed separation of the umbilical cord. II 1 II 1 IUI iuuciit ici iti LI ILL icrau. In this condition.t. Most patients present with life-threatening infections.

A chest x-ray shows diffuse. by • 13 continuous positive air pressure ventilation. Bilateral breath sounds are diminished. What is the most likely • 16 diagnosis in this patient? • 17 • 18 • 19 A. blood pressure is 65/35 mm Hg. ID-transposition of the great arteries [3%] • 21 C. fine. amniotic fluid was clear. Membranes ▪ 9 ruptured spontaneously 6 hours prior to delivery. distress • 32 • 33 Diagnosis Pathophysiology Clinical features • 34 • 35 • • 36 Inadequate alveolar • Tachyphea that begins shortly after Transient birth & resolves by day 2 of life • 37 fluid clearance at birth tachypnea of • 38 results in mild • Chest x-ray: Bilateral perihilar linear • 39 the newborn pulmonary edema 1 streaking • 40 • 41 • 42 ■ Feedback End Block . • 4- ▪ 5 ▪ 6 A 33-minute-old boy is in the neonatal intensive care unit with central cyanosis_ He was born at 28 weeks ▪ 7 gestation by vaginal delivery to a 16-year-old girl. pulse is 143imin. intercostal and subcostal • 14 retractions. and ▪ 8 the mother received glucocorticoids and magnesium sulfate shortly after the onset of labor. and • 12 respirations are 70/min.• 1 I • 2 Item: 15 of 44 F' Mark -4(1 ▪ 3 Previous Next Lab Values. and shallow respirations. Physical examination shows grunting. reticular granularity and perihilar linear opacities bilaterally. Persistent pulmonary hypertension [3%] • 24 • E Respiratory distress syndrome [88%] • 25 F. Pulse oximetry showed 75% on room air and improves to 90% with 35% FiO. The boy initially emerged vigorous • 10 with spontaneous respirations but rapidly developed respiratory distress_ Birth weight was 1 kg (2 lb 4 oz)_ • 11 His temperature is 36 C (96.8 F) under radiant heat. Notes Calculator. Congenital diaphragmatic hernia [0%] • 20 B. Meconium aspiration syndrome [3%] • 22 • 23 ID. Transient tachypnea of the newborn [3%] • 26 • 27 • 28 Explanation: • 29 • 30 • 31 I Common causes of neonatal respirator). The pregnancy was complicated by premature labor. nasal flaring.

the immature alveoli are unable to produce sufficient surfactant to reduce • 33 1 alveolar surface tension. Affected neonates develop the following symptoms within minutes to hours after • 39 birth in an attempt to compensate for diffuse alveolar collapse: • 40 se • 41 • 42 End Block Feedback . reticulogranular. is a common problem • 35 in premature neonates and very low birth weight (<1500 g [3. Notes Calculator.LLC • 33 • 34 Respiratory distress syndrome (RDS). and it occurs in almost all extremely preterm (28 weeks) neonates. • 21 distress collapse & diffuse ground-glass appearance • 22 syndrome atelectasis • 23 • Air bronchograms • 24 • 25 • Low lung volumes • 26 • 27 High pulmonary • Tachypnea & severe cyanosis • 28 Persistent vascular resistance • 29 pulmonary • Chest x-ray: Clear lungs with results in right-to-left • 30 hypertension decreased pulmonary vascularity shunting & hypoxia • 31 • 32 OUSAiLEWorkl. formerly known as hyaline membrane disease. The primary • 37 problem is surfactant deficiency.3 Ib]) infants. 4 1 5 6 7 Common causes of neonatal respiratory distress 8 1 Diagnosis Pathophysiology Clinical features 9 • 10 • Tachypnea that begins shortly after Inadequate alveolar • 11 Transient birth & resolves by day 2 of life fluid clearance at birth • 12 tachypnea of 13 •-h1= results in mild • Chest x-ray: Bilateral perihilar linear the newborn • 14 pulmonary edema streaking • 16 • Severe respiratory distress & • 17 cyanosis after premature birth • 18 • 19 Surfactant deficiency • Chest x-ray: Respiratory • 20 results in alveolar • Diffuse.• • 2 Item: 15 of 44 11 r. The incidence is inversely • 36 related to gestational age.Mark 3 Previous Next Lab Values.

• 21 • 22 (Choice A) Classic x-ray findings of congenital diaphragmatic hernia include loops of bowel in the • 23 hemithorax. • 4. Notes Calculator. This patient's history of • 33 prematurity and clear amniotic fluid makes this diagnosis unlikely. The primary ▪ 7 problem is surfactant deficiency.• 1 • 2 Item: 15 of 44 F' Mark ▪ 3 Previous Next Lab Values. and flattening of the diaphragm. which stimulate fetal surfactant synthesis and secretion_ Neonatal • 19 treatment includes early continuous positive air pressure ventilation_ Intubation. High pulmonary vascular resistance results in right-to-left shunting of deoxygenated • 37 blood through the foramen ovale and ductus arteriosus. Administration of supplemental oxygen would not improve oxygen saturation_ • 29 • 30 (Choice C) Meconium aspiration syndrome occurs in term or post-term infants born through meconium- • 31 stained fluid. • 34 • 35 (Choice 0) Persistent pulmonary hypertension of the newborn should be suspected in all term and post-term • 36 neonates with cyanosis. coarse streaking of both lung fields. a displaced cardiac silhouette. Respiratory distress syndrome (I-WS). In addition. the immature alveoli are unable to produce sufficient surfactant to reduce ▪ 8 alveolar surface tension. resulting in hypoxia. mechanical ventilation.3 Ib]) infants. the x-ray findings in this patient are very typical of RDS. • 20 and exogenous surfactant therapy are reserved for severe cases. The incidence is inversely ▪ 6 related to gestational age. tormerly known as hyaline membrane disease. is a common problem A ▪ 5 in premature neonates and very low birth weight (<1500 g [3. The diagnosis is rare in very low • 38 birth weight infants. • 39 • 40 irrhnirta F1 TrAnqiinnt tArhunnin2 is thin mnqt rnmmnn rai icr3 of rinqnirAtnrur r-litrinqq in fiill_tprm infant nil • 41 • 42 End Block Feedback . and it occurs in almost all extremely preterm (28 weeks) neonates. Patients with congenital diaphragmatic • 24 hernia would worsen with noninvasive positive-pressure ventilation as air pumped into the gastrointestinal • 25 tract can compress the lungs and should instead be intubated_ • 26 (Choice B) D-transposition of the great arteries can cause cyanosis due to systemic circulation of • 27 deoxygenated blood. X-ray would show patchy • 32 infiltrates. The risk of RDS can be reduced by administration of • 18 maternal antenatal glucocorticoids. and a gasless abdomen. Affected neonates develop the following symptoms within minutes to hours after ▪ 9 birth in an attempt to compensate for diffuse alveolar collapse: • 10 • Tachypnea (respiratory rate >601min) • 11 • Grunting (to increase end-expiratory pressure) • 12 • Nasal flaring (decreases nasal airway resistance) • 13 • Retractions (intercostal muscles contract and pull in the compliant chest wall) • 14 • Hypoxia and cyanosis (reflects significant atelectasis) • 16 • 17 This patient has the classic x-ray findings of RDS. Newborns are usually tachypneic without other signs of respiratory • 28 distress. Meconium obstructs the airways and causes respiratory distress.

resulting in hypoxia.W PL) Uy III IUII 14. Respiratory support in preterm infants at birth.IUUG IUUl. • 28 and retractions immediately after birth_ Chest-x ray includes characteristic fine reticular granularity of the • 29 lungs_ Treatment includes early continuous positive air pressure ventilation_ • 30 • 31 References: • 32 1.6011LPII. 11=1111CI II 14. The diagnosis is rare in very low • 20 birth weight infants. 11. X-ray would show patchy • 14 infiltrates. and a gasless abdomen.6. • 21 • 22 (Choice F) Transient tachypnea is the most common cause of respiratory distress in full-term infants and • 23 has no long-term sequelae.11C114111101111.IIJYY1 III LI IG • 4 A hemithorax. Respiratory distress in the newborn. Newborns are usually tachypneic without other signs of respiratory • 10 distress. This patient's history of prematurity and clear amniotic fluid makes this diagnosis unlikely. Notes Calculator. Patients with congenital diaphragmatic ▪ 5 ▪ 6 7 hernia would worsen with noninvasive positive-pressure ventilation as air pumped into the gastrointestinal tract can compress the lungs and should instead be intubated_ ▪ 7 ▪ 8 (Choice B) 0-transposition of the great arteries can cause cyanosis due to systemic circulation of ▪ 9 deoxygenated blood.. Respiratory distress in the setting of surfactant deficiency.JJ UI L.1. In addition. • 25 • 26 Educational objective: • 27 Respiratory distress syndrome should be suspected when a premature infant presents with grunting. and • 24 diffuse reticular granularities on chest x-ray rules out benign transient tachypnea. Meconium obstructs the airways and causes respiratory distress. and flattening of the diaphragm. coarse streaking of both lung fields. • 33 • 34 2. J UI . High pulmonary vascular resistance results in right-to-left shunting of deoxygenated • 19 blood through the foramen ovale and ductus arteriosus. the x-ray findings in this patient are very typical of RDS. The role of surfactant treatment in preterm infants and term newborns with acute • 35 respiratory distress syndrome. a displaced cardiac silhouette.6011WW1111. flaring. • 16 • 17 (Choice 0) Persistent pulmonary hypertension of the newborn should be suspected in all term and post-term • 18 neonates with cyanosis. • 37 ▪ 38 • 39 Copyright © LIWorld Last updated: [8/8/2014] • 40 Ii • 41 • 42 End Block Feedback . Administration of supplemental oxygen would not improve oxygen saturation_ • 11 • 12 (Choice C) Meconium aspiration syndrome occurs in term or post-term infants born through meconium- • 13 stained fluid. • 36 3.1:11 1.11.• 1 • 2 Item: 15 of 44 V-• Mark -<=1 ▪ 3 Previous Next Lab Values. prematurity.

Media Exhibit 5 6 eonaiml respiratory distress syndrome 7 8 9 • 10 • 11 • 12 • 13 • 14 Endotracheal. tube • 16 • 17 • 18 • 19 $111111111116" _. • 20 • 21 • 22 • 23 • 24 round-glass" • 25 opacities Air • 26 bronchograms • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 Orogastric • 36 tube • 37 V • 33 • 39 • 40 • 41 • 42 End Block Feedback . Item: 15 of 44 3 Previous Next Lab Values Notes Calculator 4.

Media Exhibit ▪ 5 ▪ 6 Congenital diaphragmatic hernia ▪ 7 ▪ 8 ▪ 9 • 10 • 11 • 12 • 13 • 14 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 Gasless abdomen • 35 • 36 • 37 V • 38 • 39 • 40 • 41 • 42 .• -1 • 2 Item: 15 of 44 .11M Mark ▪ 3 Previous Next Lab Values Notes Calculator • 4.

influenzae and S. • 35 • 36 (Choice B) Gonococcal and meningococcal infections are common in patients with complement • 37 deficiency. pneurnoniae and H.Trail rlafirianrw • 41 • 42 End Block Feedback .1111-1.c. Adenosine deaminase deficiency [21%] • 19 E. and productive cough of two days 7 duration. • 32 a defect in the NADPH-oxidase system of phagocytic cells results in defective intracellular killing_ Patients are • 33 therefore prone to abscesses secondary to catalase-producing organisms like Aspergdfus and • 34 Staphylococcus. • 27 pneurnorriae because immune destruction of encapsulated organisms depends upon the humoral immune • 28 response. pulse is 1. Notes Calculator.4Gimin. Genetic 0-cell deficiencies present after • 25 6 months of age because this is when the newborn's levels of maternal antibodies drop. his temperature is 383' C (1G1 F). • 30 • 31 (Choice A) Chronic granulomatous disease (CGD) is a condition of impaired oxidative metabolism. Complement deficiency [9%] C. Thymic hypoplasia [7%] • 17 • 18 D. His mother reports that he just recovered from prolonged diarrhea due to Garda infection_ His past 8 1 medical history is also significant for pneumonia and recurrent ear infections since 6 months of age. influenzae infections do occur in this patient population as well.1. Affected infants are • 26 predisposed to recurrent sinopulmonary infections with encapsulated organisms like H. 1 2 Item: 16 of 44 'MY-Mark 3 Previous Next Lab Values. Impaired oxidative metabolism [11%] • 15 13. dyspnea. but • 38 infections with Giardia are not typically seen_ • 39 • 40 nna clamant of nir4artrria c-trnrIrr. In CGD. S. 4 5 6 7 An 18-month-old male is brought to the hospital because of fever. On 9 physical examination. • 10 Examination reveals a young child in mild respiratory distress and bronchial breath sounds in the right lower • 11 lung lobe_ Which of the following is the most likely cause of his recurrent infections? • 12 r i 13 = • 14 A. Abnormal B-lymphocyte maturation [5156] • 20 • 21 • 22 Explanation: • 23 This infant has a history of Garda and recurrent sinopulmonary infections since six months of age. is the specific deficiency that predisposes to Garda • 29 infection.-.rna Tina re. and respirations are 40/min. Deficiency in IgA. which inhabits the GI tract. • 24 suggesting a genetic defect in 0-lymphocyte maturation (Choice E).

is the specific deficiency that predisposes to Garda infection. • 32 • 33 Educational objective: • 34 Patients with genetic B-cell deficiencies begin to develop recurrent infections after passing 6 months of • 35 age. • 14 pneurnomae because immune destruction of encapsulated organisms depends upon the humoral immune • 15 response. Adenosine deaminase deficiency [2116] ▪ 5 wI E Abnormal B-lymphocyte maturation [51%] ▪ 6 ▪ 7 ▪ 8 Explanation: ▪ 9 • 10 This infant has a history of Garda and recurrent sinopulmonary infections since six months of age. ifitluenzae infections do occur in this patient population as well. The deficient humoral immune response in these patients impairs the body's ability to destroy • 36 encapsulated organisms. • 19 a defect in the NADPH-oxidase system of phagocytic cells results in defective intracellular killing_ Patients are • 20 therefore prone to abscesses secondary to catalase-producing organisms like Aspergllus and • 21 Staphylococcus. but • 25 infections with Guardia are not typically seen. Patients with SCID have a deficiency of both B. Affected infants are • 13 predisposed to recurrent sinopulmonary infections with encapsulated organisms like H. pneunioniae and H. • 17 • 18 (Choice A) Chronic granulomatous disease (COD) is a condition of impaired oxidative metabolism. influenzae and S.• 1 • 2 Item: 16 of 44 V-Mark ▪ 3 Previous Next Lab Values. pneurnoniae • 37 are common_ Lack of IgA also predisposes to Garda infection_ • 38 • 39 Copyright © LIWorld Last updated: [12/29/2014] • 40 • 41 • 42 End Block Feedback . In COD. Hence. Notes Calculator. recurrent sinopulmonary infections with H. • 22 • 23 (Choice B) Gonococcal and meningococcal infections are common in patients with complement • 24 deficiency. • 29 (Choice ID) Adenosine deaminase deficiency is commonly the genetic defect underlying severe combined • 30 immunodeficiency (SCID). The resultant T-cell deficiency puts • 28 patients at high risk for viral and fungal infections. • 26 • 27 (Choice C) Thymic hypoplasia is one element of DiGeorge syndrome. intluenzae and S. • 4- ID. • 11 suggesting a genetic defect in B-lymphocyte maturation (Choice Ey Genetic B-cell deficiencies present after • 12 6 months of age because this is when the newborn's levels of maternal antibodies drop.and T-cells_ The T-cell deficiency • 31 predisposes to viral and fungal infections and the B-cell deficiency predisposes to infection by bacteria. S. Deficiency in IgA. which inhabits the GI tract.

high arched palate. • 31 • 32 (Choice 13) There is no higher risk for development of bipolar disorder in patients with Turner syndrome. widely 8 spaced nipples. Mitral valve prolapse is not very common. 4- 5 6 An 8-year-old girl is being evaluated for short stature. Mitral valve prolapse [22%] • 13 C. Examination shows a high arched palate and inverted. Notes Calculator. and 45 XO karyotype are all features of Turner • 39 syndrome. Mental retardation [13%] • 14 • 15 ID. • 27 • 28 (Choice B) The most common cardiovascular abnormalities in Turner syndrome are coarctation of the aorta • 29 and aortic valve abnormalities. Bipolar disorder [1%] E Breast cancer [3%] • 18 • 19 Explanation: • 20 • 21 This patient's short height. and 45 XO karyotype are all consistent • 22 with a diagnosis of Turner syndrome_ Patients with Turner syndrome are prone to the development of • 23 osteoporosis which increases the risk of bone fracture_ This increased risk is in part due to low estrogen • 24 levels from gonadal dysgenesis_ There is also thought to be increased risk from having only one copy of X • 25 chromosome genes that may be involved in bone metabolism_ Estrogen replacement therapy is given to • 26 nearly all patients to promote normal maturation. high arched palate. widely spaced nipples. Osteoporosis [&J%] • 12 B. but it also has the effect of reducing the risk of osteoporosis. while elevated estrogen levels are a risk factor for breast cancer_ • 36 • 37 Educational objective: • 38 Short height. • 30 (Choice C) Most patients with Turner syndrome have normal cognitive abilities. 1 2 Item: 17 of 44 V-Mark 3 Previous Next Lab Values. She is at 8th percentile for height and 30th percentile for 7 weight Vital signs are within normal limits. widely spaced nipples. Patients with Turner syndrome have a higher risk of osteoporosis due to lower estrogen levels • 40 and nnlv hmiinn nnp rnnv of rhrnmnsnmp npnps invnlvprl in hemp nlethnlisrn se • 41 • 42 End Block Feedback . • 33 • 34 (Choice E) Patients with Turner syndrome do not have any additional risk of breast cancer_ Turner syndrome • 35 patients have decreased estrogen levels. Karyotyping shows 45 X0_ Which of the following is she most at risk of developing? 9 • 10 • 11 A.

• 24 • 25 (Choice B) The most common cardiovascular abnormalities in Turner syndrome are coarctation of the aorta • 26 and aortic valve abnormalities. • 29 • 30 (Choice ID) There is no higher risk for development of bipolar disorder in patients with Turner syndrome. but it also has the effect of reducing the risk of osteoporosis. Karyotyping shows 45 X0_ Which of the following is she most at risk of developing? 7 8 A. high arched palate. while elevated estrogen levels are a risk factor for breast cancer_ • 33 • 34 Educational objective: • 35 Short height. She is at 8 — percentile for height and 30`" percentile for 5 weight Vital signs are within normal limits. Turner syndrome • 32 patients have decreased estrogen levels. • 38 • 39 Copyright © UWorld Last updated: [8/22/2014] • 40 • 41 • 42 End Block Feedback . high arched palate. and 45 XO karyotype are all consistent • 19 with a diagnosis of Turner syndrome_ Patients with Turner syndrome are prone to the development of • 20 osteoporosis which increases the risk of bone fracture_ This increased risk is in part due to low estrogen • 21 levels from gonadal dysgenesis_ There is also thought to be increased risk from having only one copy of X • 22 chromosome genes that may be involved in bone metabolism. Examination shows a high arched palate and inverted. widely spaced nipples. Osteoporosis [60%] 9 • 10 B. 4. An 8-year-old girl is being evaluated for short stature. Bipolar disorder [1°. Estrogen replacement therapy is given to • 23 nearly all patients to promote normal maturation.11V-• Mark 3 Previous Next Lab Values. widely 6 spaced nipples. Notes Calculator. Mental retardation [13%] • 12 ID.0] • 13 • 14 E Breast cancer [3%] • 15 Explanation: • 18 This patient's short height. Mitral valve prolapse [22%] • 11 C. widely spaced nipples. • 31 (Choice E) Patients with Turner syndrome do not have any additional risk of breast cancer. Patients with Turner syndrome have a higher risk of osteoporosis due to lower estrogen levels • 37 and only having one copy of X chromosome genes involved in bone metabolism. • 27 • 28 (Choice C) Most patients with Turner syndrome have normal cognitive abilities.. Mitral valve prolapse is not very common.• 1 • 2 Item: 17 of 44 . and 45 XO karyotype are all features of Turner • 36 syndrome.

He has a palpable nonblanching rash on his buttocks and lower legs.'" The boy had a low-grade fever 8 and cough 2 weeks ago and 'hasn't been himself ever since. Appendicitis [694)] • 27 B. Intussusception [66%] • 31 E Meckel diverticulum [3%] • 32 F. Cholecystitis [81C] • 28 C." He has no significant past medical history_ On examination. 3 =1 1 4 Item: 18 of 44 F' Mark Previous Next Lab Values. Notes Calculator. the patient looks ill and lethargic. Abdominal examination shows mild diffuse tenderness to palpation • 12 without rebound or guarding_ Laboratory results from urinalysis are as follows: .016 • u pH 7. 5 6 7 A 5-year-old boy is brought to the emergency department by his father with fatigue and scrotal swelling. The right hemi-scrotum is • ii slightly swollen and tender to palpation. Volvulus [15%] • 33 • 34 • 35 Explanation: • 36 • 37 Henoch-Schonlein purpura • 38 • 39 V • 40 • 41 • 42 End Block Feedback . His 7 father noticed the swelling today and is sure that 'it wasn't there yesterday. 1=3 Specific gravity 1. Mucous membranes are dry and his capillary refill is 3 • io seconds. Diverticulitis [39C] • 29 • 30 ID.8 • -15 Protein +1 • 16 Blood Moderate • 17 Glucose Negative Ketones Negative • 19 Leukocyte esterase Negative • 20 Nitrites Negative • 21 • 22 The boy is admitted to the hospital for further management Which of the following adverse outcomes is he • 23 most likely to develop? • 24 • 25 • 26 A.

red cell casts & mild proteinuria • 27 • 28 • 29 • Supportive management (hydration & pain control • 30 with nonsteroidal anti-inflammatory drugs) • 31 • Hospitalization indicated for severe abdominal pain.4K se • 41 • 42 End Block Feedback . • 4. or Treatment • 34 altered mental status • 35 • Systemic glucocorticoids in patients with severe • 36 • 37 abdominal pain unresponsive to nonsteroidal anti- • 33 inflammatory drugs • 39 • 40 uSIALVicw121. 1 - • 2 Item: 18 of 44 F'Mark 3 Previous Next Lab Values. inability to tolerate oral intake. • 32 • 33 renal insufficiency. Notes Calculator. A 5 Fienoch-Schonlein purpura 6 7 8 • Immune-mediated leukocytoclastic vasculitis 9 Pathogenesis • Associated with IgA deposition in affected organs • 10 • -1 -1 • 12 • Palpable purpura with normal platelet count & • 13 coagulation studies • 14 • Arthritisiarthralgia • 15 • 16 Clinical • Abdominal pain • 17 manifestations • Gastrointestinal bleeding • Intussusception • 19 • 20 • Renal disease similar to IgA nephropathy • 21 • Scrotal pain & swelling • 22 • 23 • 24 • Normal platelet count & coagulation studies Laboratory • 25 • Normal to elevated creatinine • 26 findings • Urinalysis: Hematuria.

palpable purpura on • 15 the lower extremities.C • 10 • 11 • 12 Henoch-Schbnlein purpura (HSP) is an immune-mediated vasculitis of childhood that often occurs after mild • 13 illnesses such as upper respiratory tract infections_ HSP is more common in boys and occurs more • 14 frequently in the fall and winter months. however. Because of their location. which can act as lead-points for the intussusception. but ileo-ileal intussusceptions that do not reduce • 27 spontaneously often require surgical management • 28 • 29 (Choice A) Appendicitis is no more common in HP than in normal children. vomiting. Ileocolic • 26 intussusceptions can be treated with air or contrast enema. the presence of severe abdominal pain • 19 should prompt further workup for gastrointestinal hemorrhage or intussusception. which is caused by inflammation of colonic divertula. The presence of Meckel diverticulum. is associated with an increased risk of • 41 • 42 End Block Feedback . altered mental status A ▪ 5 • Systemic glucocorticoids in patients with severe ▪ 6 ▪ 7 abdominal pain unresponsive to nonsteroidal anti- ▪ 8 inflammatory drugs ▪ 9 Q.I. Notes Calculator. anorexia. The increased risk for intussusception is due to bowel • 22 wall edema and localized hemorrhage. which are ileo-colic. Risk factors • 33 for cholecystitis in children include sickle cell anemia. patients can have scrotal pain and • 16 swelling as the initial presenting symptoms_ • 17 18 Although the majority of patients with HSP develop abdominal pain. Lab Values. presents with severe episodic • 21 abdominal pain and "currant jelly" or bloody stools. • 23 Unlike most cases of intussusception in children. and renal disease. • 4. is exceedingly rare in children • 36 and typically presents with left lower-quadrant pain_ An increased incidence of colonic diverticula and • 37 abdominal walliinguinal hernias is seen with autosomal dominant polycystic kidney disease. which occurs in up to 4% of cases. ▪ 38 • 39 (Choice El Meckel diverticulum usually presents with painless rectal bleeding in young children_ It is not • 40 associated with HSP. Classic manifestations include abdominal pain. ci.• 1 • 2 Item: 18 of 44 Al Mark ▪ 3 Previous Next . and obesity. intussusceptions in HSP are more likely • 24 to be small-bowel or ileo-ileal (60% of cases).EWadkl. and right lower-quadrant pain_ • 31 • 32 (Choice B) Cholecystitis is rare in otherwise healthy children and is not associated with HS13. In rare cases. • 34 • 35 (Choice C) Diverticulitis. small-bowel intussusceptions cannot • 25 be seen on contrast enema and are diagnosed by the presence of a "target" sign on ultrasound.) u$M. Appendicitis typically presents • 30 with fever. hereditary spherocytosis. both of which are known • 20 complications_ Intussusception. arthralgias.

abdominal pain. Intra-abdominal manifestations of Henoch-Schbnlein purpura. •• 20 • 21 (Choice F) Malrotation with midgut volvulus is a very common cause of intestinal obstruction in the neonatal • 22 period. Volvulus is not a recognized complication of HSP. • 29 • 30 References: • 31 1. and it can also occur later in childhood or adolescence. Notes Calculator. • 32 • 33 2. Typical features include palpable purpura. Item: 18 of 44 V-Mark 3 Previous Next Lab Values. Over 90% of patients with midgut volvulus • 23 present with vomiting (often bilious). hereditary spherocytosis. hematuria. Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 • 34 cases over a 5-year period and review of literature. vomiting. • 37 • 38 • 39 Copyright © LIWorld Last updated: [12/31/2014] • 40 • 41 • 42 End Block Feedback . • 16 • 17 (Choice El Pdeckel diverticulum usually presents with painless rectal bleeding in young children. Henoch Schtinlein purpura in childhood: clinical analysis of 254 cases over a 3-year • 36 period. The presence of Meckel diverticulum. Risk factors • 11 for cholecystitis in children include sickle cell anemia. 1 2. 4 I oe seen on contrast enema and are diagnosed oy me presence or a . anorexia. is associated with an increased risk of • 19 recurrent intussusception. is exceedingly rare in children • 14 and typically presents with left lower-quadrant pain_ An increased incidence of colonic diverticula and • 15 abdominal wall/inguinal hernias is seen with autosomal dominant polycystic kidney disease.target sign on uitrasouno_ iieocoiic 5 intussusceptions can be treated with air or contrast enema. arthralgias and occasionally • 28 scrotal swelling_ Children with HSP are at increased risk for ileo-ileal intussusception. however. which is caused by inflammation of colonic divertula. and right lower-quadrant pain_ 9 • 10 (Choice B) Cholecystitis is rare in otherwise healthy children and is not associated with HSF. and obesity_ • 12 1113 1= (Choice C) Diverticulitis. • 24 • 25 Educational objective: • 26 Henoch-Schdrilein purpura (HSP) is an immune-mediated vasculitis that is most common in children age • 27 <15. • 35 3. but ileo-ileal intussusceptions that do not reduce 6 7 spontaneously often require surgical management 7 (Choice A) Appendicitis is no more common in HP than in normal children_ Appendicitis typically presents 8 with fever. It is not i associated with HSP.

Media Exhibit ▪ 5 ▪ 6 Bloody stools in intussusizeption ▪ 7 ▪ 8 ▪ 9 • 10 • -1 -1 • 12 • 13 • 14 • 15 • 16 • 17 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 =Pr • 37 • 33 • 39 • 40 • 41 • 42 End Block Feedback .Mark V"' ▪ 3 Previous Next Lab Values Notes Calculator. • 4.• -1 • 2 Item: 18 of 44 Al I .

-1 2 Item: 18 of 44 'Mark 3 Previous Next Lab Values Notes Calculator 4. Media Exhibit X 5 6 neoaolic intussusception 7 8 9 • 10 • -1-1 • 12 13 11= • 14 • 15 • 16 • 17 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 33 • 39 • 40 • • 41 42 A Feedback a End Block .

▪ 7 He is exhausted and has chills and night sweats. Neisseria meningitides [7%] • 29 • 30 D. Pseudomonas aeruginosa [3%] • 31 E Salmonella enteritidis [16%] • 32 E Staphylococcus aureus [13%] • 33 • 34 G. and respirations are 22/min.000/pL • 17 Leukocytes 18. Escherichia coIi [3%] • 27 Haemophilus intluenzae type 13 [5%] • 28 C. cough. he has had no bone pain. • 4- ▪ 5 ▪ 6 A 3-year-old African American boy with sickle cell anemia is brought to the emergency department for fever. His temperature is 39A C (1G3 F). Streptococcus pneumoniae [53%] • 35 • 36 Explanation: • 37 • 30 • 39 • 40 Sickle-cell anemia se • 41 • 42 End Block Feedback . The boy was hospitalized during infancy for dactylitis and last summer for splenic sequestration ▪ 9 crisis_ Other than ibuprofen and acetaminophen for fever and pain. Examination shows a lethargic boy with mild pallor.2 WI:IL • 15 Hematocrit 24% • 16 Platelets 325. he takes no medications_ His • 10 vaccinations are up to date. However. Notes Calculator. pulse is • 11 140/min.800/pL • 18 Neutrophils 8G% Band 6% • 20 Gram stain Pending • 21 Blood culture Pending • 22 • 23 Which of the following organisms is the most likely cause of this patient's condition? • 24 • 25 • 26 A.• 1 • 2 Item: 19 of 44 V-• Mark -4(1 ▪ 3 Previous Next Lab Values. Laboratory results • 12 are as follows: • 13 Complete blood count • 14 Hemoglobin 8. blood pressure is 1814G mm Hg. or difficulty ▪ 8 breathing.

• 4 • 5 Sickle-cell anemia • 6 • 7 Inheritance Autosomal recessive • 8 0 • 10 • Hemolytic anemia Clinical 11 presentation • Acute vasoocclusive pain crises • 12 13 • 14 • NI. Next .• 1 • 2 : 19 of 44 r Flilark • 3 Previous . Lab Values. • 15 Laboratory \ unconjugated bilirubin • 16 'I findings • 17 • Peripheral smear: Sickled red cells. Hematocrit. Feedback . Notes Calculator. • 28 • Hydration • 29 Acute pain crises • Analgesia • 30 • 31 • Transfusion • 32 • 33 • 34 • Infection with encapsulated organisms • • Streptococcal pneumoniae bacterem ia & pneumonia • 36 • Salmonella osteomyelitis • • 38 • Osteonecrosis • 39 Complications • Acute chest syndrome • 40 • 41 • 42 End Block. lk lactate dehydrogenase. '1k reticulocytes. Howell-Jolly bodies • 18 I • 20 • Vaccination • 21 • Penicillin (until age 5) • 22 Maintenance • Folic acid supplementation • 23 • 24 • Hydroxyurea (for patients with • 25 Management recurrent vasooccrusive events) • 26 • '7 Z.

which leads to • 10 repeated microinfarctions in any part of the body. Haemophilus influenzae type E. hypotension. All patients with sickle cell anemia should receive routine pediatric vaccinations (eg. • 31 • 32 (Choice C) N meningitides is an encapsulated organism that is responsible for most cases of bacterial • 33 meningitis in children and young adults in the United States. this patient has no bone pain suggestive of • 33 osteomyelitis. The slow blood flow in the spleen • 11 permits splenic sequestration of red blood cells. • 25 • 26 (Choices A and D) Given the patient's history of sickle cell anemia and likelihood of splenic dysfunction. photophobia. high fever. neutrophil-predominant leukocytosis with • 17 bandemia) is alarming for sepsis_ S pneumoniae is the most common cause of bacteremia. Neisseria meningitides. However. Infected patients can have fever. with H • 18 influenzae type B (Choice B) as a distant second. • 39 • 40 se • 41 • 42 End Block Feedback _ A . but this patient has no related symptoms (eg. • 27 bacteremia due to pneumococcus is more likely than Escherichia coli or Pseudomonas aeruginosa_ E coli is • 28 the most common cause of urinary tract infection in all patients. • 29 dysuria. • 34 tachycardia. The incidence of • 21 bacteremia has decreased dramatically with widespread vaccination. and patients with functional • 13 asplenia are at extremely high risk of overwhelming infection with Streptococcus pneurnoniae • 14 (pneumococcus). • 15 • 16 This patient's presentation (eg. but children with sickle cell disease • 22 continue to be at serious risk of invasive pneumococcal disease from other pneumococcal serotypes. but particularly the spleen. and neck pain makes meningitis • 35 unlikely in this patient • 36 (Choices E and F) Salmonella species and Staphylococcus aureus are the most common causes of • 37 osteomyelitis in sickle cell patients in the United States. hypotension. and drowsiness. Unfortunately. • 4 • Pnapisrn ▪ 5 • Stroke ▪ 6 ▪ 7 ODUSPALEWorktr U_C ▪ 8 ▪ 9 The primary problem in homozygous sickle cell disease is constant clumping of sickle cells. P aeruginosa is a common cause of bacteremia in patients with burn • 30 wounds and neutropenia. hesitancy.• 1 • 2 Item: 19 of 44 V/lark ▪ 3 Previous Next Lab Values. penicillin prophylaxis should also be given until at least age 5 years. The absence of headache. Notes Calculator. and Salmonella species. resulting in recurrent splenic infarction and splenic • 12 dysfunction_ The spleen is the site of removal for encapsulated organisms. abdominal pain). 13-valent conjugate pneumococcus and H influenzae type B vaccines) plus the • 20 23-valent polysaccharide pneumococcus and meningococcal conjugate vaccines. this patient • 24 was not taking penicillin. lethargy. • 23 Therefore.

Increase in invasive Streptococcus pneumoniae infections in children with sickle cell • 36 disease since pneumococcal conjugate vaccine licensure. Incidence of invasive pneumococcal disease among individuals with sickle cell disease • 34 before and after the introduction of the pneumococcal conjugate vaccine. this patient has no bone pain suggestive of • 22 osteomyelitis. • 12 dysuria. Pneumococcus is the most common • 27 cause of sepsis in this population. Infected patients can have fever. Notes Calculator. ▪ 8 ▪ 9 (Choices A and D) Given the patient's history of sickle cell anemia and likelihood of splenic dysfunction. but this patient has no related symptoms (eg. • 29 • 30 References: • 31 1. and neck pain makes meningitis • 18 unlikely in this patient • 20 (Choices E and F) Salmonella species and Staphylococcus aureus are the most common causes of • 21 osteomyelitis in sickle cell patients in the United States. • 23 • 24 Educational objective: • 25 Sickle cell anemia is associated with functional asplenia due to recurrent splenic infarction. Unfortunately. hesitancy. photophobia. r ' • 4- bacteremia has decreased dramatically with widespread vaccination. penicillin prophylaxis should also be given until at least age 5 years. hypotension. • 17 tachycardia. ▪ 6 Therefore. Sickle-cell disease. abdominal pain}_ P aeruginosa is a common cause of bacteremia in patients with burn • 13 wounds and neutropenia. which puts these • 26 patients at risk of dangerous infection with encapsulated organisms. The absence of headache.• 1 • 2 Item: 19 of 44 [MI V-Mark ▪ 3 Previous Next Lab Values. this patient ▪ 7 was not taking penicillin. Vaccination and penicillin prophylaxis are extremely important infection • 28 prevention measures. but children with sickle cell disease ▪ 5 continue to be at serious risk of invasive pneumococcal disease from other pneumococcal serotypes. • 10 bacteremia due to pneumococcus is more likely than Escherichia coli or Pseudomocas aeruginosa_ E coli is • 11 the most common cause of urinary tract infection in all patients. • 37 • 30 • 39 Copyright © UWorld Last updated: [10/25/2014] • 40 • 41 • 42 End Block Feedback . However. • 35 3. and drowsiness. • 14 • 15 (Choice C) N meningitides is an encapsulated organism that is responsible for most cases of bacterial • 16 meningitis in children and young adults in the United States. • 32 • 33 2.

Mark ▪ 3 Previous Next Lab Values.ctir. Notes Calculator. Mosquito [1 °A] • 18 Raccoon [79%] • 19 C. has no allergies. • 12 Examination shows an agitated. and • 11 is up to date on standard vaccinations_ There is no history of foreign travel_ Vital signs are normal. For the ▪ 7 past week. The neck has full • 13 range of motion. • 10 The wound was rinsed with water and has since healed_ The girl takes no medications.• 1 • 2 Item: 20 of 44 V.01 cri.ocrn en. she has had fever. Spider [3%] • 22 E. Rat [3%] • 20 • 21 ID. malaise. muscle aches. chills. Squirrel [4%] • 23 _ F. disoriented girl with copious drool and facial grimacing. • 41 • 42 End Block Feedback . • 4- ▪ 5 ▪ 6 A previously healthy 5-year-old girl is brought to the emergency department with difficulty swallowing. A well-healed scar is seen on her right forearm_ Of the following disease reservoirs.o. The girl sustained a bite to her right forearm when she and her ▪ 9 family went camping in Delaware last month_ Family members are unsure of the bite source as it was dark. which • 14 is the most likely source of her symptoms? • 15 • 16 • 17 A. and sore throat She now refuses to drink fluids ▪ 8 and spits out any water that is given to her. Tick [1 ON • 24 • 25 • 26 Explanation: • 27 • 28 • 29 Human rabies • 30 • 31 Pathogenesis Transmission of rabies virus by bite from infected animal • 32 • 33 • 34 • Wild carnivores Reservoir • 35 • Bats • 36 • 37 • 39 • Hydrophobia • 39 • Acrophobia Encephalitic • 40 Phorionnn.

transmission can occur by contact with mucous membranes.• 1 • 2 ▪ 3 Previous Next Lab Values. • 32 Raccoons are the most prevalent rabid animal in the United States. Those who miss • 39 the opportunity for passive and active immunization after a high-risk animal bite are in danger of deadly rabies se • 40 • 41 • 42 End Block Feedback . especially on the east coast In rare • 33 cases. Post-exposure prophylaxis includes both rabies immune globulin and rabies • 38 vaccine to prevent progression to life-threatening encephalitic or paralytic rabies disease. spastic paralysis • 17 • 18 features • Agitation • 19 Paralytic Ascending flaccid paralysis • 21 • 22 • 23 Post-exposure Rabies immune globulin & rabies vaccine immediately • 24 prophylaxis after exposure to high-risk wild animal • 25 • 26 • 27 Prognosis Coma. respiratory failure & death within weeks • 28 OUSMLEWuddr LLC • 29 • 30 Rabies is a fatal disease that is transmitted to humans by contact with infected animals. Transmission of the • 31 neurotropic rabies virus typically involves exposure to saliva from an infected animal through a bite. and corneal • 34 and organ transplantations_ • 35 • 36 Bites from wild carnivores or bats require thorough wound cleansing with soap and water and urgent • 37 post-exposure prophylaxis. infected aerosol particles. • 4 ▪ 5 Human rabies ▪ 6 ▪ 7 Pathogenesis Transmission of rabies virus by bite from infected animal ▪ 8 ▪ 9 • 10 • Wild carnivores • 11 Reservoir • Bats • 12 • 13 • 14 • Hydrophobia • 15 • • Acrophobia • 16 Encephalitic Clinical • Pharyngeal spasm. Notes Calculator.

• 31 • 32 References: • 33 . • 37 • 33 • 39 Copyright © UWorld Last updated: [11/16/2014] • 40 Ii • 41 • 42 End Block Feedback . transmission can occur by contact with mucous membranes. Hydrophobia and aerophobia are pathognomonic features of encephalitic rabies. infected aerosol particles. Almost all patients die within weeks_ • 20 (Choices A. ticks are • 21 vectors for Lyme meningitis and Rocky Mountain spotted fever. 34 1. • 4 ▪ 5 OLISMLEWarld. the feeling of • 18 water or air triggers involuntary pharyngeal muscle spasms_ Many patients are disoriented and agitated.• 1 • 2 Item: 20 of 44 F' Mark ▪ 3 Previous Next Lab Values. chipmunks. D and F) Mosquitos are vectors for West Nile virus and Dengue hemorrhagic fever. • 23 • 24 (Choices C and E) Small rodents such as squirrels. ▪ 9 Raccoons are the most prevalent rabid animal in the United States. Rabies surveillance in the United States during 2012. Those who miss • 16 the opportunity for passive and active immunization after a high-risk animal bite are in danger of deadly rabies • 17 disease. Post-exposure prophylaxis includes both rabies immune globulin and rabies • 15 vaccine to prevent progression to life-threatening encephalitic or paralytic rabies disease. with . management and post-mortem findings of a human case of rabies imported • 36 into the United Kingdom from India: a case report. LLC ▪ 6 ▪ 7 Rabies is a fatal disease that is transmitted to humans by contact with infected animals. Transmission of the ▪ 8 neurotropic rabies virus typically involves exposure to saliva from an infected animal through a bite. and rats are less common reservoirs of • 25 rabies. • 35 2. • 22 including necrosis in severe cases. and affected patients • 30 typically die within weeks of illness onset. and spider bites can cause local inflammation. especially on the east coast In rare • 10 cases. insects do not transmit rabies. and corneal • 11 and organ transplantations_ • 12 • 13 Bites from wild carnivores or bats require thorough wound cleansing with soap and water and urgent • 14 post-exposure prophylaxis. Hydrophobia and aerophobia are pathognomonic for encephalitic rabies. 19 fluctuating mental status. Diagnosis. • 26 • 27 Educational objective: • 28 Post-exposure rabies prophylaxis can be life-saving and should be initiated after a bite wound from a wild • 29 carnivore. Notes Calculator. However.

• 1 • 2 Item: 21 of 44 V-• Mark ▪ 3 Previous Next Lab Values. On examination he is drowsy and lethargic_ He flexes his hips when his neck is • 11 flexed. with rates 10 times higher than those of the general • 31 population. vomiting. and • 40 erythematous. wrists. Lyme disease typically presents with a rash (ie. annular. it does not se • 41 • 42 End Block Feedback . It appears within 24 hours of the infection. he became increasingly lethargic and developed a rash on his lower ▪ 9 extremities that has acutely worsened during the past few hours. He also has an erythematous. It is usually acquired in the late summer months after • 39 an ixodes sp. The child's immunizations are up to date • 10 and he is otherwise healthy. He developed a fever during the day that initially responded to treatment with ▪ 8 acetaminophen_ Throughout the day. Notes Calculator. Haemophilus influenzae [4%] • 19 • 20 E Herpes simplex virus [1%] I 21 • Listeria monocytogenes [6%] • 22 • G. tick bite. flanks and ankles. • 33 • 34 Prompt identification and treatment are critical given that the disease progresses rapidly over the course of a • 35 few hours and carries a very high morbidity and mortality rate even with appropriate treatment • 36 • 37 (Choice A) Borrefia burgdorferi causes Lyme disease. • 4 ▪ 5 ▪ 6 7 An 18-month-old child is brought to the emergency department by his mother with fever. erythema chronicum migrans). Borrefia burgdorferi [1%] • 15 • 16 E Cytomegalovirus [1%] • 17 C. • 39 headaches. Neisseria meningitides [77%] • 23 • 24 • 25 Explanation: • 26 • 27 This patient with signs of meningitis and a petechial rash most likely has Neisseria meningitides_ Neisseria • 28 meningitides is the most common cause of bacterial meningitis in children and young adults in the United • 29 States_ The mortality rate of meningococcal meningitis is extremely high. and malaise. fevers. Group B Streptococcus [10%] • 1a ID. approaching nearly 15%. Children • 30 age < 2 are at greatest risk for contracting this illness. nonblanching pinpoint rash on his trunk and bilateral lower • 12 extremities. Seventy-five percent of patients with Meningococcus meningitis present with a petechial rash that • 32 is prominent on the axilla. chills. The skin rash is very characteristic and is large. occasionally with central clearing_ Although Lyme disease can cause meningitis. and ▪ 7 lethargy. What is the most likely organism causing this patient's symptoms? • 13 • 14 A.

annular. Another mode of transmission is ingestion of • 25 unpasteurized milk or cheese from infected cows. neck stiffness. • 36 2. and ▪ 7 erythematous. fevers. tick bite. The skin rash is very characteristic and is large. • 37 • 39 • 39 Copyright © UWorld Last updated: [8/24/2014] • 40 • 41 • 42 End Block Feedback . is 1 of the 3 most common causes of • 24 meningitis in newborns but does not present with a rash. It usually does not cause meningitis or present with a • 12 rash. erythema chronicum migrans). Multicenter surveillance of invasive meningocaccal infections in children. and a petechial or purpuric • 30 rash. splenomegaly. It is usually acquired in the late summer months after ▪ 5 an ixodes sp. Prompt diagnosis and treatment are critical given that it has a high morbidity and mortality rate even 1 • 31 with appropriate treatment • 32 • 33 References: • 34 • 35 1.• 1 • 2 Item: 21 of 44 V-Mark ▪ 3 Previous Next Lab Values. chills. and malaise. • 20 (Choice E) Herpes simplex virus generally causes temporal lobe encephalitis in neonates and infants and I 21 typically presents with seizures. • 29 It typically presents with fever. and pharyngitis. It is usually acquired • 15 from the mother during childbirth. • 13 • 14 (Choice C) Group 13 Streptococcus is the most common cause of meningitis in infants. • 16 • 17 (Choice 0) Haernopfulus inthierrzae may cause meningitis but typically does not present with a rash. It would be less likely to occur in an 18-month-old child_ • 26 • 27 Educational objective: • 28 Meningococcal meningitis is the most common cause of bacterial meningitis in children and young adults. which presents with high • 11 fevers. The rate of H irrfluerrzae infections has decreased • 19 dramatically since the vaccine was introduced. it does not ▪ 8 present with a petechial rash. occasionally with central clearing_ Although Lyme disease can cause meningitis. epiglottis. ▪ 9 • 10 (Choice B) Cytornegaiovirus can cause infectious mononucleosis like illness. malaise. altered mental status. It is not associated with a rash and is quite unlikely in an 18-month-old. headache. It may •18 also cause rhinorrhea. • 22 • 23 (Choice F) Listeria rnorrocytogenes. fever. which is transmitted vaginally. • 4 (Choice A) Borrefia burgdorferi causes Lyme disease. ▪ 6 7 headaches. fatigue. It would be less likely in an 18-month-old child. Lyme disease typically presents with a rash (ie. and ear infections. Clinical recognition of meningococcal disease in children and adolescents. Notes Calculator.

The pertinent physical findings are pallor.maturitv is defined by the followinn laboratory studips- • 41 • 42 End Block Feedback . lethargy. • 38 tachypnea. poor feeding. Symptoms include poor weight gain. (2) shorter life span of • 36 red blood cells in neonates. • 4 11. Hemolytic disease of newborn [4%] • 22 • 23 ID.DDD!cmm • 11 Platelets 230. 25 • Iron deficiency [99C] • 26 • 27 G. Notes Calculator. tachycardia. The causes are usually • 35 pathophysiologic. and pallor_ The hemoglobin level is usually around 7-10 gldL. Beta thalassemia [5%] • 21 C.• 1 • 2 Item: 22 of 44 V. and (3) diminished fetoplacental transfusion (occurs when the baby is held above • 37 the level of the placenta after delivery). tachypnea. Pancytopenia [15C] • 29 G-6-PD deficiency [2%] • 30 • 31 • 32 Explanation: • 33 • 34 Anemia of prematurity is seen in hospitalized. • 5 • 6 A 2-month-old infant is brought to the clinic for the evaluation of poor feeding. Vitamin 812 deficiency [156] • 28 H. premature or low birth weight infants. Sickle cell anemia [29C] . He was born at 32 weeks of • 7 gestation with a birth weight of 12GG g. • 39 • 40 Anemia of orp. The laboratory studies are as follows: • 9 Hb 7 WI:IL • 10 WBC 7. Alpha thalassemia [3%] • 20 B. 24 ✓ E Anemia of prematurity [74%] .000/cl-rim • 12 Reticulocytes Low • 13 • 14 • 15 The peripheral smear shows normocytic normochromic RBC. tachycardia.Mark • 3 Previous Next Lab Values. What is the most likely diagnosis? • 16 • 17 • 19 A. and include: (1) transitions in the erythropoiesis sites of the neonate. and • 8 flow murmurs.

• 8 tachypnea. pallor. poor feeding. lethargy. and (3) diminished fetoplacental transfusion (occurs when the baby is held above • 7 the level of the placenta after delivery). • 13 3. (2) shorter life span of • 6 red blood cells in neonates. and hydrops • 27 fetalis in severe cases. 25 (Choice C) Hemolytic disease of the newborn is due to Rh or ARO incompatibility. • 28 • 29 (Choice ID) Sickle cell anemia presents much later because the presence of hemoglobin F protects the • 30 infants from sickling during the first 4-6 months of life_ Dactylitis may be the initial presentation that warrants • 31 further work-up for sickle cell disease. • 36 • 37 (Choice G) Low levels of folic acid or vitamin B 12 do not cause anemia of prematurity. and pallor_ The hemoglobin level is usually around 7-10 gidL. Iron is given because it will be stored and reutilized during • 35 the active phase of erythropoiesis. and red blood cell precursors in the bone marrow are decreased. The clinical features • 26 depend on the severity of the disease. • 9 • 10 Anemia of prematurity is defined by the following laboratory studies: • 11 1. • 15 • 16 • 17 The treatment involves iron supplementation. the • 38 anemia in such cases is megaloblastic_ • 39 • 40 Educational Objective: • 41 • 42 End Block Feedback . Notes Calculator. • 19 • 20 (Choice A) Alpha thalassemia is caused by abnormalities in the synthesis of alpha chains of hemoglobin_ It is • 21 usually a hypochromic. . • 22 (Choice B) Beta thalassemia is also a hypochromic. and a positive family history is usually present. The reticulocyte count is low. 24 . microcytic anemia that usually presents at 6-12 months • 23 of age. microcytic anemia. premature or low birth weight infants. Furthermore. Symptoms include poor weight gain. The reticulocyte count is increased due to hemolysis. iron deficiency is not the usual cause.• 1 • 2 Item: 22 of 44 V-Mark • 3 Previous Next Lab Values. The causes are usually • 5 pathophysiologic. hepatosplenomegaly. Normal total bilirubin level. periodic hemoglobin checking and blood transfusion. and include: (1) transitions in the erythropoiesis sites of the neonate. if needed. Normal WE C and platelet counts. • 4 Anemia of prematurity is seen in hospitalized. • 32 • 33 (Choice F) Even though iron supplementation is routinely administered in the management of anemia of • 34 prematurity. Symptoms include jaundice. Erythropoietin is not routinely used. • 14 4. Peripheral smear shows normocytic and normochromic anemia_ No other abnormal forms are seen_ • 12 2. tachycardia.

1 ici 1110 UI pi CI 1101411 ILy 13 uciii ICU LI IC 1U111. 24 infants from sickling during the first 4. shortened REC life span. pallor. • 30 (Choice G) Low levels of folic acid or vitamin E 12 do not cause anemia of prematurity. Normal total bilirubin level. Furthermore. and red blood cell precursors in the bone marrow are decreased. 25 further work-up for sickle cell disease. Symptoms include jaundice.• 1 • 2 Item: 22 of 44 Bli. The pathology • 35 involves a combination of diminished RE C production.Mark -<1 . Iron • 36 supplementation does not prevent falling hemoglobin levels. iron deficiency is not the usual cause. Iron is given because it will be stored and reutilized during • 29 the active phase of erythropoiesis. and iron deficiency is not the cause of anemia of • 37 prematurity. The clinical features • 20 depend on the severity of the disease. • 15 • 16 (Choice B) Beta thalassemia is also a hypochromic. Notes Calculator. hepatosplenomegaly. and blood loss. Peripheral smear shows normocytic and normochromic anemia_ No other abnormal forms are seen. .JYY11 Iy.months of life_ Dactylitis may be the initial presentation that warrants . • 6 2. • 32 • 33 Educational Objective: . 3 Previous Next Lab Values. • 4 • 5 1. periodic hemoglobin checking and blood transfusion. The reticulocyte count is low. • 9 • 10 The treatment involves iron supplementation. 7 3. • 8 4. • 22 ▪ 23 (Choice 0) Sickle cell anemia presents much later because the presence of hemoglobin F protects the . if needed. The reticulocyte count is increased due to hemolysis. It is • 14 usually a hypochromic. microcytic anemia. !OULU CILIA y 3LUUIC-3. • 26 • 27 (Choice F) Even though iron supplementation is routinely administered in the management of anemia of • 28 prematurity. • 11 Erythropoietin is not routinely used. and a positive family history is usually present. microcytic anemia that usually presents at 6-12 months • 17 of age. the • 31 anemia in such cases is megaloblastic. • 38 • 39 Copyright © UWorld Last updated: [8/19/2014] • 40 • 41 • 42 End Block Feedback . • 19 (Choice C) Hemolytic disease of the newborn is due to Rh or ABC incompatibility. 34 Anemia of prematurity is the most common anemia in premature and low birth weight infants. • 12 • 13 (Choice A) Alpha thalassemia is caused by abnormalities in the synthesis of alpha chains of hemoglobin. and hydrops ▪ 21 fetalis in severe cases. Normal VVEC and platelet counts. 1-4.

4 5 6 A 1G-week-old girl is brought to the office by her mother for a well-infant examination after missing her visit 2 7 weeks earlier. The mother • 10 eats a balanced diet that includes meat products. She was born at 34 weeks gestation and weighed 2_2 kg (4 lb 14 oz) at birth. Vitamin C supplementation [0%] • 22 • 24 Explanation: • 25 • 26 I • 27 Timeline of infant nutrition • 28 I • 29 • 30 • 31 Birth 6 months 1 year • 32 • 33 • 34 • 35 Exclusive breastfeeding • 36 • 37 Introduction of Introduction • 38 • 39 pureed foods of cow's milk • 40 • 41 • 42 Feedback End Block . Introduction of fruit juices to the diet [0%] • 16 C. and her growth and development are appropriate for her chronological and 9 gestational ages. which of the following is the most appropriate • 11 nutritional intervention for this patient? • 12 • 13 A. Vitamin 13 12 supplementation [116] G. Notes Calculator. Introduction of pureed vegetables to the diet [1%] • 17 tO ID. Her physical examination is notable for mild pallor but is otherwise normal. Iron and vitamin ID supplementation [85%] • 18 • 19 E No additional supplementation needed [12%] • 20 F. Introduction of eggs and meat to the diet [0%] • 14 • 15 B. She has been breastfed exclusively since then.Mark 3 Previous Next Lab Values. 2 Item: 23 of 44 V. At this time.

• 34 (Choices A and C) The American Academy of Pediatrics (AAP) recommends exclusive breastfeeding • 35 until age 6 months given that earlier introduction of solid foods is associated with an increase in • 36 gastrointestinal infections_ Pureed fruits and vegetables should be introduced first followed by pureed proteins • 37 • such as meats. V • • 41 42 Feedback a End Block . 1 2 Item: 23 of 44 F'Mark 3 Previous Next Lab Values. and both iron and vitamin ID supplementation should be initiated. 4 Timeline of infant nutrition 5 6 7 1 Birth 6 months 1 year 9 • 10 • 11 • 12 • 13 Exclusive breastfeeding • 14 • 15 Introduction of Introduction • 16 pureed foods of cow's milk • 17 • 18 • 19 OUVVorId • 20 Iron deficiency anemia is the single most common nutritional deficiency in infants and children and is often • 22 asymptomatic. However. Fullterm. and early introduction of cow's milk before age 12 months • 25 increases the risk of iron deficiency anemia in infants_ • 26 I • 27 Human breast milk contains only small amounts of vitamin ID that are inadequate for meeting the infant's daily • 28 I requirement All exclusively breastfed infants should be started on 400 International Units of vitamin ID daily • 29 within the first month of life_ • 30 • 31 Given that this infant was born prematurely and is exclusively breastfed. the presence • 24 of maternal iron deficiency. she is at significantly increased risk • 32 for iron deficiency anemia. prematurity. Iron • 33 supplementation should be continued until age 1 year in preterm infants. Notes Calculator. There is no evidence suggesting that early introduction of highly allergenic foods such as 38 • 39 eggs is associated with an increased risk of allergies. these foods can be introduced any time after age 6 • 40 months. regardless of dietary intake. healthy infants are born with robust iron stores that generally prevent them from developing iron deficiency anemia until age 4-6 months.

7 L4L il1111114.L411uyII 14. Breastfeeding and the use of human milk..1). Diagnosis and prevention of iron deficiency and iron-deficiency anemia in infants and • 35 young children (0-3 years of age). There is no evidence suggesting that early introduction of highly allergenic foods such as • 11 eggs is associated with an increased risk of allergies. Iron 6 supplementation should be continued until age 1 year in preterm infants.L4ilLI . All exclusively breastfed infants should also be started on vitamin ID supplementation_ • 30 • 31 References: • 32 1. and both iron and vitamin ID supplementation should be initiated.111L4LL41 4-..1 I LI IL4L LI Mil II II L41 IL TYL4il 4-. 1 2 Item: 23 of 44 Mark -4Z1 3 Previous Next Lab Values. these foods can be introduced any time after age 6 • 12 months. 1. 14-. Preterm infants are at significantly increased risk for iron deficiency anemia Iron • 28 I supplementation should be started at birth in exclusively breastfed preterm infants and continued until age 1 • 29 year. Iron homeostasis in the neonate.7GU 1 1. %-4 14r4-. For families who choose to offer juice. • 36 3. • 37 • 38 • 39 Copyright © LIWorld Last updated: [1D/27/2014] • 40 • 41 • 42 Feedback End Block . • 24 • 25 Educational objective: • 26 I Full-term infants are born with adequate iron stores to prevent anemia for the first 4. • 22 (Choice G) Vitamin C deficiency is exceedingly rare in exclusively breastfed infants given that breast milk contains adequate amounts of vitamin C to meet the infant's daily requirement until at least age 6 months.months of life • 27 regardless of dietary intake. 4-.71'. 7 (Choices A and C) The American Academy of Pediatrics (AAP) recommends exclusive breastfeeding until age 6 months given that earlier introduction of solid foods is associated with an increase in 9 gastrointestinal infections_ Pureed fruits and vegetables should be introduced first followed by pureed proteins • 10 such as meats.1 GQ. no more than 4-6 ounces per day should be • 16 given (after age 6 months) and it should never be given in a bottle given the significantly increased risk of • 17 dental caries_ • 18 • 19 (Choice F) Supplementation of vitamin B12 is recommended for exclusively breastfed infants born to strict • 20 vegetarian mothers. the AAP does not recommend routine introduction of • 15 juice into an infant's diet. • 13 • 14 (Choice B) Although some fruit juices contain vitamins. 4 5 for iron deficiency anemia. Notes Calculator.LA. • 33 • 34 2.

• 34 • 35 (Choice 0) Juvenile myoclonic epilepsy presents in adolescents with unilateral or bilateral myoclonic jerks. Juvenile myoclonic epilepsy [4%] • 16 E. but do not have a post-ictal • 29 state. picking movements of the hand). His neurological examination is unremarkable. he is unresponsive to verbal or tactile stimuli. staring spells. Notes Calculator... Typical absence seizure [229C] • 13 C. and may be precipitated by sleep deprivation. Atypical absence seizure [37%] • 14 • 15 D. automatisms (e. During these episodes. Unlike in absence seizures. The characteristic EEG pattern is slow spike-and-wave • 33 activity with a frequency less than 2_5Hz. after which he remains confused for some time. and produces lip-smacking 7 movements. The EEG pattern is usually normal or may show brief discharges. 2 Item: 24 of 44 .11V-Mark 3 Previous Next Lab Values.e. lip i smacking.e. He has no family history of any seizure disorder. Complex partial seizure [32%] • 12 B. hyperventilation during the EEG cannot • 25 simulate a complex partial seizure_ • 26 I • 27 (Choice B) A typical absence seizure is characterized by brief (i. failure to respond to various stimuli during the episode. • 37 • 38 (Choice E) Lennox-Gastaut syndrome usually presents in childhood as seizures of multiple types. typically lasting for a few seconds) • 28 periods of impaired consciousness. EEG performed 9 before and after hyperventilation is normal_ Which of the following is the most likely diagnosis? • 10 • 11 wI A.g. 4 5 A previously healthy 8-year-old boy is brought to the office by his mother because he has multiple staring 6 episodes.. • 31 • 32 (Choice C) An atypical absence seizure lasts longer. lasting for a few minutes) episodes of impaired • 22 consciousness. swallowing. Hyperventilation during the EEG reveals a generalized 3Hz spike-and-wave pattern on a normal • 30 background. Patients may also present with automatisms. and post-ictal confusion. • 40 se • 41 • 42 End Block Feedback . Each episode lasts for a few minutes. and slow spike-and-wave activity on EEG. Lennox-Gastaut syndrome [4%] • 17 • 18 • 19 Explanation: • 20 • 21 A complex partial seizure is characterized by brief (i. • 36 The symptoms take place in the morning. impaired • 39 cognitive function.

Hyperventilation during the EEG reveals a generalized 3Hz spike-and-wave pattern on a normal i background. typically lasting for a few seconds) • 21 periods of impaired consciousness. Patients may also present with automatisms.. and post-ictal confusion. The EEG pattern is usually • 17 normal or may show brief discharges. Complex partial seizure [32%] 6 B. Lennox-Gastaut syndrome [4%] • 11 • 12 • 13 Explanation: • 14 A complex partial seizure is characterized by brief (Le. lip • 16 smacking. 1 2 Item: 24 of 44 V. picking movements of the hand). automatisms (e. Notes Calculator. failure to respond to • 36 various stimuli during the episode.Mark 3 Previous Next Lab Values. • 33 • 34 Educational objective: • 35 A complex partial seizure is characterized by brief episodes of impaired consciousness. failure to respond to various stimuli during the episode. hyperventilation during the EEG cannot • 18 simulate a complex partial seizure_ • 19 • 20 (Choice B) A typical absence seizure is characterized by brief (Le. Unlike in absence seizures. swallowing. staring spells. The characteristic EEG pattern is slow spike-and-wave • 26 I activity with a frequency less than 2_5Hz. • 25 (Choice C) An atypical absence seizure lasts longer. • 29 The symptoms take place in the morning. and may be precipitated by sleep deprivation. Juvenile myoclonic epilepsy [4%] • 10 E.. but do not have a post-ictal • 22 state. and post-ictal confusion.g. 4 5 A. impaired • 32 cognitive function. The EEG pattern is • 37 usually normal or may show brief discharges.. • 30 • 31 (Choice E) Lennox-Gastaut syndrome usually presents in childhood as seizures of multiple types. Atypical absence seizure [37%] 8 9 ID. lasting for a few minutes) episodes of impaired • 15 consciousness. • 38 • 39 Copyright © LIWorld Last updated: [12/29/2014] • 40 • • 41 • 42 End Block Feed back . and slow spike-and-wave activity on EEG. staring spells. Typical absence seizure [22%] 7 C. automatisms. • 27 • 28 (Choice ID) Juvenile myoclonic epilepsy presents in adolescents with unilateral or bilateral myoclonic jerks.

Examination shows no other ▪ 9 abnormalities. ▪ 3 Previous Next Lab Values. Which of the following is the most appropriate • 10 next step in management? • 11 • 12 A. Notes Calculator.• 1 • 2 Item: 25 of 44 V-Mark .4111°. Physical examination ▪ 8 shows absence of breast development and no pubic hair is seen. XX 46„ XV V • 40 • 41 • 42 End Block Feedback . Karyotyping [20%] • 18 E GnRH stimulation test [14%] • 19 • Reassurance [12%] • 20 • 21 • 22 Explanation: • 23 • 24 Evaluation of primary amenorrhea • 26 Pelvic examination • 27 or ultras-410g FaITI • 28 • 29 • 30 • • 31 Uterus present Uterus absent • 32 • 33 • 34 • 35 Serum FSI-1 Ha ryotype • 36 serum testosterone • 37 • 38 Increased Decreased • 39 46. Serum FSH level [41%] • 16 • 17 D. Estrogen level [13%] • 13 • 14 • MRI of pituitary [1%] • 15 C. • 4- ▪ 5 ▪ 6 A 15-year-old girl is brought to the physician by her mother because she has not begun menstruating_ She is ▪ 7 otherwise healthy and has no medical problems_ Vitals signs are within normal limits. Ultrasound confirms the presence of a uterus.

or to gonadal ▪ 37 (peripheral) abnormalities. However. Notes Calculator. ▪ 3 Previous Next Lab Values. so • 34 measuring the estrogen level provides no additional information (Choice A). as with this patient. If the amenorrhea is of central origin.4111°. if secondary sexual characteristics are absent. Increased FSH • 38 (hypergonadotropic amenorrhea) indicates a peripheral cause. The absence of breast development indicates a lack of estrogen. and decreased FSH (hypogonadotropic ▪ 39 amenorrhea) indicates a central cause. This distinction can be made by measurement of the FSH level. • 4- ▪ 5 Evaluation of primary amenorrhea ▪ 6 ▪ 7 Pelvic examination ▪ 8 or ultrasonogram ▪ 9 • 10 • 11 • 12 Uterus present Uterus absent • 13 • 14 • 15 1 • 16 Serum 151-I Ha ryotype • 17 serum testosterone • 18 • 19 Increased Decreased • 20 46. XY • 21 Normal female Normal male • 22 1 testosterone testosterone 1 Karyotyping Cranial MRI • 23 levels levels • 24 • 26 • 27 Abnormal Mullerian Androgen development insensitivity syndrome • 28 utactwoktut c ail • 29 • 30 This patient has primary amenorrhea and underdevelopment of secondary sexual characteristics. work-up should not be • 33 delayed beyond age 14 (Choice F). Isolated • 31 amenorrhea with well-developed secondary sexual characteristics can be considered normal up to the age of • 32 16. • 35 • 36 Primary amenorrhea can be due to either hypothalamic/pituitary (central) abnormalities.cAritni-itninn wni ilri hp the • 41 • 42 End Block Feedback . a pituitary MRI is indicated to • 40 Innk fnr a leginn in the cella tl irrir inhnirta RI If AMPrinfrhPA is of nerinherAl nrinin 1.• • 2 Item: 25 of 44 F'Mark . XX 46.

or to gonadal • 20 (peripheral) abnormalities.• 1 • 2 Item: 25 of 44 V-. If the amenorrhea is of central origin. work-up should not be • 16 delayed beyond age 14 (Choice F). ▪ 37 • 38 ▪ 39 Copyright © LIWorld Last updated: [10/30/2014] • 40 • 41 • 42 End Block Feedback . • 4. Normal female Normal male Jr ▪ 5 testosterone testosterone Karyotyping Cranial MAI ▪ 6 levels levels ▪ 7 ▪ 8 ▪ 9 I Abnormal Muilerian Androgen • 10 development insensitivity syndrome • 11 iD 201 1 • 12 • 13 This patient has primary amenorrhea and underdevelopment of secondary sexual characteristics. Notes Calculator. karyotyping would be the • 24 next step (Choice I3). Evaluation and management of adolescent amenorrhea. The absence of breast development indicates a lack of estrogen. Increased FSH • 21 (hypergonadotropic amenorrhea) indicates a peripheral cause. If amenorrhea is of peripheral origin. However. Mark -<1 ▪ 3 Previous Next Lab Values. and decreased FSH (hypogonadotropic • 22 amenorrhea) indicates a central cause. if secondary sexual characteristics are absent. as with this patient. a pituitary PORI is indicated to • 23 look for a lesion in the sells turcica (Choice 6). • 18 • 19 Primary amenorrhea can be due to either hypothalamic/pituitary (central) abnormalities. This distinction can be made by measurement of the FSH level. • 26 • 27 Educational objective: • 28 In a patient with primary amenorrhea: • 29 • 30 • FSH measurement should be ordered if there is no breast development • 31 • Pituitary MRI is the next step if FSH is decreased • 32 • Karyotyping is the next step if FSH is increased • 33 • 34 • 35 References: • 36 1. (Choice E) A GnRH stimulation test is used for the evaluation of precocious puberty. so • 17 measuring the estrogen level provides no additional information (Choice A). Isolated • 14 amenorrhea with well-developed secondary sexual characteristics can be considered normal up to the age of • 15 16.

Althouah the antibiotics aiven before LP can affect the • 41 • 42 11. lethargy.Mark -461 M=E-- Previous . End Block. Feedback. Streptococcus prieumoniae and Neisseria meningiticlis are the most • 36 common causes of bacterial meningitis in children age >1 month. nuchal rigidity. to LP • Coma or focal neurologic findings ▪ 27 0 intravenous vancomycin & ceftriaxone OR • 28 I cefotaxime Treatment • 29 0 Dexannethasone for Haotnophilus influenzae type b • 30 meningitis • • 32 OUSMLEWarld. lethargy. fever. Item: 26 of 44 V. vomiting) is concerning for bacterial meningitis. Bacterial meningitis in children age >1 month 7 • 8 * Fever • 0 • Vomiting/poor feeding • 10 Clinical * Seizures • 11 • 12 features • Altered mental status (eg. Lab Values. Kernig & Brudzinski signs • 14 • Bulging anterior fontanelle • 15 • 16 • 17 • Complete blood count & electrolytes • 18 Workup • Blood cultures • 19 • LP & cerebrospinal fluid studies • 20 • 21 • 22 Indications for • History of hydrocephalus or neurosurgical procedure • imaging prior • History of head trauma • 24. Management requires prompt administration of empiric antibiotics and cerebrospinal fluid (CSF) analysis to establish the diagnosis and • 38 identify the offending pathogen. ill-appearing patients with high likelihood of bacterial meningitis should receive empiric • 40 antibiotic therapy as soon as blood cultures are sent. irritability) • 13 0 Nuchal rigidity. Notes Calculator. Next . As it can take >30 minutes to prepare and perform a lumbar puncture (LP) • 39 with sterile technique. . LLC • 34 This infant's clinical presentation (altered mental status.

ceftriaxone is avoided as it displaces bilirubin • 27 from albumin and increases the risk of kernicterus. pathogens other than meningococcus can usually be • 22 identified up to several hours after antibiotic administration_ • 23 • 24 Empiric antibiotic therapy should be directed at the most common pathogens. herniation is less likely in infants age <1 • 31 year as the fontanelles have not fully closed and can accommodate some brain swelling. fever. ill-appearing patients with high likelihood of bacterial meningitis should receive empiric • 20 antibiotic therapy as soon as blood cultures are sent Although the antibiotics given before LP can affect the • 21 yield of cerebrospinal fluid Gram stain and culture.• 1 • 2 Item: 26 of 44 F'Mark ▪ 3 Previous Next Lab Values. Ceftriaxone is effective • 25 against most strains of S pneurnorriae and N meningitidis. focal neurologic findings. The risk of • 32 herniation is low in the absence of coma. it would not be appropriate to hospitalize the • 37 infant without first performing LP. Head CT would • 30 delay LP and expose the infant to high doses of radiation. Streptococcus pneumoniae and Neisseria meningitidis are the most • 16 common causes of bacterial meningitis in children age >1 month. In neonates age X28 days. • 38 • 39 (Choice El LP should be performed prior to administering antibiotics in patients with lower suspicion for • 40 se • 41 • 42 End Block Feedback . vomiting) is • 15 concerning for bacterial meningitis. Notes Calculator. or history of a neurosurgical procedure • 33 (eg. In addition. Therefore. Management requires prompt • 17 administration of empiric antibiotics and cerebrospinal fluid (CSF) analysis to establish the diagnosis and • 18 identify the offending pathogen_ As it can take >30 minutes to prepare and perform a lumbar puncture (LP) • 19 with sterile technique. This risk does not exist in older infants who are unlikely to • 28 have hyperbilirubinemia. vancomycin is given due to increasing prevalence of resistant strains of S pneurnorriae. • 4 to LP • Coma or focal neurologic findings ▪ 5 ▪ 6 ▪ 7 • ntravenous vancomycin & cariaxone OR I ▪ 8 cefotaxi me Treatment ▪ 9 • Dexamethasone for Haotnophilus infiuenzae type b • 10 meningitis • 11 • 12 QUSHLEWarkl. nuchal rigidity. ventriculoperitoneal shunt) (Table). LLC • 13 • 14 This infant's clinical presentation (altered mental status. • 29 (Choices A and B) Intravenous antibiotics are the priority when meningitis is suspected. head CT is not usually indicated in infants such as this • 34 patient • 35 • 36 (Choice C) Although ceftriaxone and vancomycin are indicated. lethargy.

fever. • 37 • 38 • 39 Copyright © LIWorld Last updated: [1G/16/n14] • 40 • 41 • 42 Feedback End Block .• • sm 22 (Choice El LP should be performed prior to administering antibiotics in patients with lower suspicion for • 23 meningitis and with a stable neurologic examination. herniation is less likely in infants age <1 • 14 year as the fontanelles have not fully closed and can accommodate some brain swelling_ The risk of • 15 herniation is low in the absence of coma. This risk does not exist in older infants who are unlikely to • 10 have hyperbilirubinemia. Imaging should be performed before the lumbar puncture in infants and • 29 children in coma. with focal neurologic findings. poor oral intake. • 24 • 25 Educational objective: Bacterial meningitis in infants and children presents with lethargy. • 34 • 35 2. and vomiting. It is • 27 crucial to administer empiric antibiotics (ceftriaxone and vancomycin) first and then perform a lumbar • 28 I puncture as soon as possible.J111U1 I ILIIU Ul I I LUII I Ul ULI ici uiaiI Iiiciiiii utiutitiu3 tiraiI 4 5 identified up to several hours after antibiotic administration. 1 2 Item: 26 of 44 V-• Mark -4Z1 3 Previous Next Lab Values..I. • 11 • 12 (Choices A and B) Intravenous antibiotics are the priority when meningitis is suspected. In neonates ages28 days. 1998-2007. In addition. Clinical features suggestive of meningitis in children: a systematic review of prospective • 33 data. 6 Empiric antibiotic therapy should be directed at the most common pathogens.. vancomycin is given due to increasing prevalence 8 of resistant strains of S pneurnorriae. ULJI 5. Head CT would • 13 delay LP and expose the infant to high doses of radiation. head CT is not usually indicated in infants such as this • 17 patient • 18 • 19 (Choice C) Although ceftriaxone and vancomycin are indicated. Ceftriaxone is effective 7 against most strains of S pneurnorriae and N meningitidis. • 36 3. Therefore. or history of a neurosurgical procedure • 16 (eg. ceftriaxone is avoided as it displaces bilirubin 9 from albumin and increases the risk of kernicterus. it would not be appropriate to hospitalize the • 20 infant without first performing LP. Notes Calculator. ventriculoperitoneal shunt) (Table). • 21 . Bacterial meningitis in the United States. Acute bacterial meningitis in infants and children: epidemiology and management. or with a history of a neurosurgical procedure_ • 30 • 31 References: • 32 1. focal neurologic findings..

Vector-borne illness [TA] • 22 • 23 • 24 Explanation: • 25 • 26 Hemophilia A & B • 28 • 29 • 30 X-linked recessive disorder of factor VIII deficiency Pathophysiology • 31 (hemophilia A) or factor IX deficiency (hemophilia B) • 32 • 33 • 34 • Occurs almost exclusively in males Epidemiology • 35 • Female carriers may have mild symptoms • 36 • 37 • 38 • Hemarthrosis. He first noticed 7 knee pain and swelling 6 months earlier.stinRi nr irinAry tr rt hIppriinn • 41 • 42 End Block Feedback . He has no history 8 of fever. • 4- 5 6 A 16-year-old boy is brought to the pediatrician with pain and limited motion of the right knee. His uncle had similar problems_ Which of the following is the most likely • 11 cause of this patient's joint pain? • 12 • 13 A. The patient reports that his right knee and right ankle have swelled 9 several times before_ He has a history of easy bruising since childhood and had an episode of excessive • 10 bleeding after a tooth extraction. Notes Calculator. Mark 3 Previous Next Lab Values. Hemosiderin deposition and fibrosis [56%] • 17 ID. and the pain has gradually worsened since then. 1 • 2 Item: 27 of 44 V-. Avascular necrosis [3%] • 14 • 15 B. Crystal deposition [1%] • 16 C. local erythema. or trauma. Immunologic tissue injury [8%] • 18 • 19 E Occult traumatic injury [12%] • 20 • Repetitive microtrauma [2094)] • 21 G. hemophilic arthropathy • 39 Clinical features • Intramuscular hernatornas V • 40 • inAstrriiritp.

4 A 5 6 Hemophilia A & B 7 8 X-linked recessive disorder of factor VIII deficiency 9 Pathophysiology (hemophilia A) or factor IX deficiency (hemophilia B) • 10 • -1-1 • 12 • Occurs almost exclusively in males • 13 Epidemiology • Female carriers may have mild symptoms I • 14 . which are caused by deficiencies of Factor VIII and Factor IX. Lab Values. respectively. Hemophilia A and • 34 B. a late complication that occurs in both types of hemophilia and is a significant source • 40 of morbidity is thounht to ho hausp. .adinn to svnovitis and fibrosis so • 41 • 42 End Block Feedback . excessive bleeding after a dental procedure.d by ironihamosidarin danosition 1p.• 1 • 2 Item: 27 of 44 F'Mark 3 Previous Next . • 33 • 39 Hemophilic arthropathy. are most consistent with hemophilia. Hemophilia can affect nearly every organ system and result in long-term complications • 37 such as hemophilic arthropathy. female carriers may be • 36 mildly symptomatic. along with his family history. hemophilic arthropathy • 17 Clinical features • Intramuscular hematomas • 18 • Gastrointestinal or genitourinary tract bleeding • 19 • 20 • 21 • Normal platelet count ffmi 2 • Normal bleeding time • 23 Laboratory findings • Normal prothrombin time • 24 • 25 • Prolonged activated partial thromboplastin time • 26 • Decreased or absent factor VIII or IX activity • 28 Treatment Administration of factor VIII or factor IX • 29 • 30 VUSAILEWurld.LLC • 31 • 32 This patient's history of easy bruising since childhood. are bleeding disorders inherited • 35 in an X-linked recessive pattern. and • 33 recurrent joint swelling. Notes Calculator. Although only male patients are severely affected. • 15 • 16 • Hemarthrosis.

• 37 • 38 (Choice G) Lyme disease is a vector-borne illness that can result in arthritis of the large joints as a late • 39 finding. it is more consistent with a chronic inflammatory process due to iron/hemosiderin deposition. Both conditions are uncommon in children. which are caused by deficiencies of Factor VIII and Factor IX. female carriers may be ▪ 6 mildly symptomatic. • 34 • 35 (Choice F) Repetitive microtrauma. • 4. Sickle cell • 22 disease may also cause recurrent vaso-occlusive episodes. which is most common in adults age >55. it would be unlikely to • 40 account for this patient's other findinas_ so • 41 • 42 End Block Feedback . LCP. respectively. Notes Calculator. early prophylactic treatment with factor concentrates can significantly reduce the risk of • 17 developing arthropathy. which may affect the joints.• 1 • 2 Item: 27 of 44 V-Mark ▪ 3 Previous Next Lab Values. Although only male patients are severely affected. are bleeding disorders inherited A ▪ 5 in an X-linked recessive pattern. or idiopathic avascular necrosis of the femoral head. Hemophilic arthropathy typically presents • 12 with chronic worsening joint pain and swelling and can result in contractures of the joint and limited range of • 13 motion. Hemophilic arthropathy cannot be entirely prevented. • 18 • 19 (Choice A) Avascular necrosis of the femoral head is seen in children with sickle cell disease and in • 20 Legg-Calve-Perthes disease (LCP). B. Although severe hemophilic • 14 arthropathy may be visible on plain films. It is most common in patients with a history of recurrent hemarthroses. magnetic resonance imaging allows for much earlier detection • 15 and characterization of the degree of joint damage. is thought to be caused by ironihemosiderin deposition leading to synovitis and fibrosis • 11 within the joint However. also known as "the wear and tear" phenomenon. respectively. • 30 • 31 (Choice El Although minor unnoticed trauma can result in episodes of hemarthrosis in patients with • 32 hemophilia. these conditions would not explain • 29 the patient's bleeding symptoms. Although Lyme arthritis can present with swelling and limited range of motion. • 16 However. • 26 (Choice 0) Immunologic tissue injury occurs in a variety of conditions. Hemophilia can affect nearly every organ system and result in long-term complications ▪ 7 such as hemophilic arthropathy. other mechanisms may also be involved. is thought to play a role • 36 the development of osteoarthritis. It is rare in adolescents. typically • 21 presents with limp or hip pain and is most common in children age 4-12. the prolonged course of symptoms in this patient does not suggest acute bleeding into the joint • 33 Rather. such as rheumatoid arthritis and • 28 psoriatic arthritis_ But while inflammatory arthritis may cause joint swelling. ▪ 8 ▪ 9 Hemophilic arthropathy. • 24 • 25 (Choice B) Urate and calcium pyrophosphate deposition are the mechanisms of arthritis in gout and pseudogout. a late complication that occurs in both types of hemophilia and is a significant source • 10 of morbidity. but are not • 23 associated with excessive bruising or bleeding.

the prolonged course of symptoms in this patient does not suggest acute bleeding into the joint • 18 Rather.C VING131-U1111 I ICU! U3I3 UI LI IC ICI I IUI CH I ICGIU 13 3CCI I II I 4. ILL. • 37 • 38 • 39 Copyright © UWorld Last updated: [7/16/2014] • 40 • 41 • 42 End Block Feedback .IIIJIL. Sickle cell 7 disease may also cause recurrent vaso-occlusive episodes. Hemophilic arthropathy: a review of imaging and staging.1111U1 CI I MU I 311. Both conditions are uncommon in children. respectively. Pathobiology of hemophilic synovitis I: overexpression of mdm2 oncogene. • 15 • 16 (Choice E) Although minor unnoticed trauma can result in episodes of hemarthrosis in patients with • 17 hemophilia. these conditions would not explain • 14 the patient's bleeding symptoms. which is most common in adults age >55.. 9 (Choice B) Urate and calcium pyrophosphate deposition are the mechanisms of arthritis in gout and • 10 pseudogout. Pathogenesis of haemophilic arthropathy. • 11 • 12 (Choice ID) Immunologic tissue injury occurs in a variety of conditions. it would be unlikely to • 25 account for this patient's other findings.pdc LCII U13CCI3C AI IU II I 5 Legg-Calve-Perthes disease (LOP)_ LOP. It is rare in adolescents. also known as "the wear and tear' phenomenon. typically 6 presents with limp or hip pain and is most common in children age 4-12. • 26 Educational objective: • 28 Hemophilic arthropathy is a delayed consequence of recurrent hemarthrosis. or idiopathic avascular necrosis of the femoral head. It is associated with 4 • 29 hemosiderin deposition leading to synovitis and fibrosis within the joint The risk of hemophilic arthropathy can • 30 be significantly reduced by prophylactic treatment with factor concentrates_ • 31 • 32 References: • 33 1. which may affect the joints. • 4. • 22 • 23 (Choice G) Lyme disease is a vector-borne illness that can result in arthritis of the large joints as a late • 24 finding_ Although Lyme arthritis can present with swelling and limited range of motion. is thought to play a role • 21 the development of osteoarthritis.• 1 • 2 Item: 27 of 44 V-Mark 3 Previous Next Lab Values. • 34 • 35 2.. ft is more consistent with a chronic inflammatory process due to iron/hemosiderin deposition. but are not 8 associated with excessive bruising or bleeding. • 19 • 20 (Choice F) Repetitive microtrauma. such as rheumatoid arthritis and • 13 psoriatic arthritis_ But while inflammatory arthritis may cause joint swelling. Notes Calculator. • 36 3.

3 Previous Next Lab Values Notes Calculator 4. • 23 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 33 • 39 ••••• • 40 • • 41 42 Feedback a End Block .111M 'Mark FF. 2 Item: 27 of 44 . Media Exhibit X 5 6 7 8 9 • 10 • -1 -1 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 z= 22 • 23 • 24 • 25 • 26 rF.

• 4- ▪ 5 ▪ 6 A 4-day-old boy in the newborn nursery has failed a bilateral hearing screening_ He was born at 37 weeks ▪ 7 gestation by elective cesarean section for intrauterine growth retardation.• 1 • 2 Item: 28 of 44 V. cervical • 36 presentation Children lymphadenopathy. His mother traveled to Egypt during ▪ 8 the first trimester of pregnancy and developed a brief febrile illness but otherwise felt well during the ▪ 9 pregnancy. Toxoptasma gondii [4%] • 20 E. The boy does not alert to voice or sound. Cytomegalovirus [14%] • 16 B. The most likely cause of the • 12 newborn's symptoms is infection with which of the following organisms? • 13 • 14 • 15 A. Treporrema paltidurn [1'36] • 21 F. coryza. • 38 erythematous maculopapular rash ▪ 39 • 40 so • 41 • 42 . Notes Calculator. glaucoma • 32 • 33 • Low-grade fever • 34 ▪ 35 Clinical • Conjunctivitis. patent ductus anenosus) • 31 • Cataracts. Herpes simplex virus [0%] • 17 C.Mark ▪ 3 Previous Next Lab Values. Auscultation shows a • 11 continuous systolic-diastolic murmur over the left 2nd intercostal space. Rubella virus [80'36] • 18 • 19 D. length. 27 Rubella (German measles) m 28 • 29 • Sensorineural hearing loss • 30 Congenital • Cardiac anomalies (eg. and head circumference are <5th percentile. The infant's weight. Forschheimer spots ▪ 37 • Cephalocaudal spread of blanching. Varicella zoster virus [0%] • 22 • 23 • 24 Explanation: • 25 . Physical examination • 10 shows bilateral white pupillary reflexes. 25 .

Forschheimer spots • 16 • Cephalocaudal spread of blanching. • 35 First-trimester infections are the most devastating. Live attenuated rubella vaccine 'Do • 29 Treatment . 25 . 27 Prevention . patent ductus artenosus) • 10 • Cataracts. cervical • 14 • 15 presentation Children lym phadenopathy. • 17 erythematous maculopapular rash • 18 • 19 • 20 Adolescents] • Same -as children + arthralgiasiarthritis • 21 Adults • 22 • 23 • Polymerase chain reaction • 24 Diagnosis • Acute & convalescent serology for ant-rubella IgM & IgG • 25 . LLC • 31 • 32 Rubella infection in adults can be asymptomatic in 25%-50% of patients or characterized by mild symptoms 33 (eg. • 4- ▪ 5 ▪ 6 Rubella (German measles) ▪ 7 ▪ 8 • Sensorineural hearing loss ▪ 9 Congenital • Cardiac anomalies (eg. low-grade fever. Notes Calculator. However. with potential outcomes such as spontaneous abortion • 36 and congenital rubella syndrome. and hearing loss_ • 30 • 39 A probable diagnosis can be made clinically and confirmed by culture.• -1 • 2 Item: 28 of 44 F'Mark ▪ 3 Previous Next Lab Values. Congenital rubella syndrome can be prevented by selective immunization of women of childbearing so • 41 • 42 End Block Feedback . maculopapular rash). murmur of patent ductus arteriosus. coryza. or polymerase chain reaction • 40 (Table). Supportive care • 30 © SWILEVYarld. infection during pregnancy is dangerous due to high risk • 34 for transmission of the rubella virus from the mother's bloodstream to the placenta and into fetal circulation. serology. glaucoma • -1 -1 • 12 • Low-grade fever • 13 Clinical • Conjunctivitis. This newborn has the classic triad of leukocoria (white papillary reflex) from • 37 cataracts.

However. and cataracts. murmur of patent ductus arteriosus. serology. The • 36 classic triad of congenital rubella syndrome consists of sensorineural deafness. purpura. and cataracts with a maternal history of a pruritic. with potential outcomes such as spontaneous abortion ▪ 6 and congenital rubella syndrome. It is also associated with sensorineural hearing loss. cardiac defects (eg. which can result in permanent hearing loss and/or • 21 blindness. Cataracts. This newborn has the classic triad of leukocoria (white pupillary reflex) from ▪ 7 cataracts. blindness is usually due to • 18 chorioretinitis. for transmission of the rubella virus from the mother's bloodstream to the placenta and into fetal circulation. and hearing loss_ ▪ 8 ▪ 9 A probable diagnosis can be made clinically and confirmed by culture. intrauterine • 31 growth retardation. 25 . • 19 (Choice B) Herpes simplex virus is usually acquired perinatally from vaginal delivery. but congenital • 16 infection shares similar manifestations with in utero rubella infection. generalized vesicular rash. • 29 • 30 (Choice F) Congenital varicella syndrome is characterized by microcephaly. • 14 • 15 (Choice A) Maternal cytomegalovirus infection can be mild and minimally symptomatic.. including deafness. and the heart is unaffected. • 38 • 39 References: • 40 se • 41 • 42 End Block Feedback . 27 (Choice E) Congenital syphilis infection can cause cataracts and sensorineural hearing loss but is not m 28 associated with congenital heart disease. hydrocephalus. but not cardiac defects_ . deafness. • 23 • 24 (Choice ID) Congenital toxoplasmosis causes the triad of chorioretinitis. but widespread immunization is preferable for complete eradication of the virus from herd immunity. and heart defects would not be present at birth as the fetus was not infected • 22 in utero. or polymerase chain reaction • 10 (Table). Notes Calculator. the deafness is typically unilateral.• 1 • 2 Item: 28 of 44 'Mark -<1 ▪ 3 Previous Next Lab Values. and intracranial • 25 calcifications. patent • 37 ductus arteriosus). limb hypoplasia. • 12 Rubella immunization is contraindicated during pregnancy due to a theoretical risk for the live vaccine to • 13 cause fetal infection. 33 • 34 Educational objective: • 35 Maternal-fetal transmission of the rubella virus is most teratogenic during the first trimester of pregnancy. Newborns can • 20 deteriorate rapidly from invasive meningoencephalitis. Congenital • 32 heart defects are not a typical finding in this disease. and jaundice. • 4. • 17 hepatosplenomegaly. Congenital rubella syndrome can be prevented by selective immunization of women of childbearing • 11 age. ▪ 5 First-trimester infections are the most devastating.

• 21 • 22 (Choice F) Congenital varicella syndrome is characterized by microcephaly. • 10 • 11 (Choice B) Herpes simplex virus is usually acquired perinatally from vaginal delivery. and heart defects would not be present at birth as the fetus was not infected • 14 in utero. ▪ 6 (Choice A) Maternal cytomegalovirus infection can be mild and minimally symptomatic. Newborns can • 12 deteriorate rapidly from invasive meningoencephalitis. It is also associated with sensorineural hearing loss. • 37 ▪ 38 • 39 Copyright © UWorld Last updated: [1011/2014] • 40 Ii • 41 • 42 End Block Feedback .• 1 • 2 Item: 28 of 44 F Mark -4Z1 ▪ 3 Previous Next Lab Values. blindness is usually due to ▪ 9 chorioretinitis. 33 34 2. • 35 3. 2012. Rubella.Tor tne live vaccine to ▪ 5 cause fetal infection. deafness. and cataracts with a maternal history of a pruritic. The I m 28 classic triad of congenital rubella syndrome consists of sensorineural deafness. Notes Calculator. intrauterine • 23 growth retardation. which can result in permanent hearing loss and/or • 13 blindness. the deafness is typically unilateral. hydrocephalus. but congenital ▪ 7 infection shares similar manifestations with in utero rubella infection. but not cardiac defects_ • 18 • 19 (Choice El Congenital syphilis infection can cause cataracts and sensorineural hearing loss but is not • 20 associated with congenital heart disease. and Illinois. Kuaeiia immunization is contrainoicatea airing pregnancy aue to a tneoreticai risk. • 15 • 16 (Choice ID) Congenital toxoplasmosis causes the triad of chorioretinitis. Three cases of congenital rubella syndrome in the postelimination era--Maryland. limb hypoplasia. and cataracts. Cataracts. 25 Educational objective: . cardiac defects (eg. • 4. However. • 30 • 31 References: • 32 1. and intracranial • 17 calcifications. 27 Maternal-fetal transmission of the rubella virus is most teratogenic during the first trimester of pregnancy. including deafness. generalized vesicular rash_ Congenital • 24 heart defects are not a typical finding in this disease_ • 25 . ▪ 36 Alabama. Rubella and congenital rubella (German measles). patent • 29 ductus arteriosus). and jaundice. and the heart is unaffected. purpura. ▪ 8 hepatosplenomegaly.

Mark ▪ 3 Previous Next Lab Values. or volume depletion. seizures. renal ultrasound. with no further workup [27%] • 20 • Renal biopsy [2%] • 21 • 22 • 23 Explanation: • 24 • 25 A urine dipstick can be positive in up to 10% of school-aged children. and myalgia_ He has no other medical problems_ Family history is negative for any kidney ▪ 8 disease. renal biopsy (Choices C. Order renal ultrasound [3%] • 19 E. Transient and orthostatic • 32 proteinurias are usually benign conditions that require no further evaluation_ • 33 • 34 If the proteinuria persists on the repeat sample or if any of the initial studies are abnormal.• 1 • 2 Item: 29 of 44 V. and ▪ 9 respirations are 15/min. pyuria. His temperature is 38. Check serum protein and albumin levels [10%] • 16 C. fatigue.9° F). blood pressure is 122/74 mm Hg. or persistent Transient proteinuria is the most common cause of proteinuria and • 27 can be caused by fever. the patient should • 35 be referred to a pediatric nephrologist and evaluated for underlying renal disease. Repeat dipstick testing on two subsequent occasions [3296] • 15 B. Examination shows no abnormalities_ An incidental urine dipstick testing shows 2+ • 10 proteinuria but no hematuria. orthostatic. This patient presents with proteinuria during a febrile illness and should be tested again in the future to rule • 38 out persistent proteinuria. • 40 • 41 • 42 End Block Feedback . Further investigation may • 36 include 24-hour urinary collection for protein. Orthostatic proteinuria is very • 28 common in adolescent boys and is defined as increased protein when the patient is in an upright position that returns to normal when the patient is recumbent If the urinalysis shows no hematuria and is otherwise • 30 normal. Order 24-hour urinary collection for protein [26%] • 17 • 18 D.8° C (101. and. possibly. or active urine sediment Serum creatinine is within normal limits_ • 11 Which of the following is the most appropriate next step in management? • 12 • 13 • 14 ▪ A. If these • 31 subsequent tests are negative for protein. • 39 (Choice B) Checking serum protein (albumin) has little value in evaluating proteinuria since many patients „„. pulse is 95/min. exercise. Proteinuria in children can be transient • 26 (intermittent). D. the diagnosis is transient proteinuria. Notes Calculator. and • 37 F). stress. the urine dipstick should be repeated on at least two additional specimens (Choice A). • 4- ▪ 5 ▪ 6 A 14-year-old boy comes to the physician with two days of fever and nasal discharge. He has also had ▪ 7 malaise. Reassure.

with no further workup [7°•0] 7 Renal biopsy [2%] 8 9 • 10 Explanation: • 11 • 12 A urine dipstick can be positive in up to 10% of school-aged children_ Proteinuria in children can be transient • 13 (intermittent). 1 • 2 Item: 29 of 44 V-Mark 3 Previous Next Lab Values. • 4- 5 D. Transient and orthostatic • 19 proteinurias are usually benign conditions that require no further evaluation_ • 20 • 21 If the proteinuria persists on the repeat sample or if any of the initial studies are abnormal. and. and • 24 F). stress. seizures. or persistent Transient proteinuria is the most common cause of proteinuria and • 14 can be caused by fever. This patient presents with proteinuria during a febrile illness and should be tested again in the future to rule • 25 out persistent proteinuria. the patient should • 22 be referred to a pediatric nephrologist and evaluated for underlying renal disease_ Further investigation may • 23 include 24-hour urinary collection for protein. possibly. exercise. 13. the urine dipstick should be repeated on at least two additional specimens (Choice A). which requires • 32 further evaluation for underlying renal disease_ • 33 • 34 • 35 References: • 36 1. orthostatic. • 26 • 27 (Choice B) Checking serum protein (albumin) has little value in evaluating proteinuria since many patients • 28 with even nephrotic-range proteinuria have normal serum protein levels. renal ultrasound. Order renal ultrasound [3%] 6 E Reassure. renal biopsy (Choices C. the diagnosis is transient proteinuria. or volume depletion_ Orthostatic proteinuria is very • 15 common in adolescent boys and is defined as increased protein when the patient is in an upright position that • 16 returns to normal when the patient is recumbent If the urinalysis shows no hematuria and is otherwise • 17 normal. If these • 18 subsequent tests are negative for protein. Educational objective: • 30 Transient proteinuria is the most common cause of isolated proteinuria in children and should be reevaluated • 31 with a repeat urine dipstick testing on two separate occasions to rule out persistent proteinuria. Notes Calculator. Isolated proteinuria: analysis of a school-age population • 37 • 38 • 39 Copyright © LNVorld Last updated: [11/4/2014] • 40 • 41 • 42 End Block Feedback .

Notes Calculator. 27 .• 1 • 2 Item: 30 of 44 F' Mark ▪ 3 Previous Next Lab Values. On examination. • 10 • 11 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 . • 4 ▪ 5 ▪ 6 A newborn girl is evaluated in the neonatal intensive care unit for cyanosis. 28 ▪ 29 • 30 • 31 • 32 33 • 34 • 35 • 36 • 37 ▪ 38 • 39 • 40 se • 41 • 42 End Block Feedback . she is cyanotic and tremulous. micrognathia. Chest radiograph shows an absent thymus. She has low-set ears. She was born at 36 weeks ▪ 7 gestation by cesarean section for fetal distress_ The girl remained hypoxic despite administration of ▪ 8 supplemental oxygen. ▪ 9 and a cleft palate.

• 4- ▪ 5 ▪ 6 ▪ 7 ▪ 8 ▪ 9 • 10 • 11 • 12 • 13 • 14 • 15 Echocardiography confirms truncus arteriosus_ This patient is at greatest risk for which of the following? • 16 • 17 A.• 1 • 2 Item: 30 of 44 F' Mark ▪ 3 Previous Next Lab Values. Duodenal atresia [10%] • 18 B.2 deletion Pathogenesis 36 • Defective development of pharyngeal pouches 37 38 • 39 • Conotruncal cardiac defects • 40 se • 41 • 42 End Block Feedback . Thrombocytopenia [1a4] • 26 . Infertility [4%] • 22 E Myelomeningocele [3%] • 23 • 24 • Neutropenia [5%] • 25 G. 27 . 28 Explanation: ▪ 29 • 30 • 31 DiGeorge syndrome • 32 33 • 34 35 • Chromosome 22q11. Notes Calculator. Hypoglycemia [2%] • 21 ID. Hypocalcemia [7€9C] • 19 • 20 C.

Notes Calculator.2 deletion • 10 Pathogenesis • 11 • Defective development of pharyngeal pouches • 12 • 13 • 14 • Conotruncal cardiac defects • 15 • Abnormal facies • 16 Clinical • 17 • Thymic aplasiaihypoplasia • 18 features • Cleft palate • 19 • 20 • Hypocalcemia • 21 • 22 L6MLEWorld. and septal defects.• 1 • 2 Item: 30 of 44 r Mark ▪ 3 Previous Next Lab Values. intranasal influenza. 28 ▪ 29 Once DOS is suspected. and hypocalcemia . 35 36 Depending on the degree of thymic hypoplasia. and arrhythmias that can further exacerbate co-existing heart problems and may require • 32 aggressive calcium repletion. and • 26 mediastinum. Echocardiography is critical as the cardiac anomalies in DOS are variable and 33 place the patient at great risk for heart failure and hypoperfusion. serum calcium levels and echocardiography must be ordered immediately_ • 30 Hypocalcemia results from hypoplasia of the parathyroid glands_ Neonates are at high risk for life-threatening 1 • 31 tetany. Truncus arteriosus is strongly associated • 34 with DGS. rotavirus. LLC • 23 • 24 DiGeorge syndrome (DOS) is disorder usually caused by the microdeletion of chromosome 2201. the safety of live • 40 vaccines (measles-mumps-rubella. seizures. The clinical presentation typically includes the pentad of congenital heart disease. neck. resulting • 25 in poor development of the pharyngeal pouch system and subsequent abnormalities of the face. cleft palate. • 4- ▪ 5 ▪ 6 DiGeorge syndrome ▪ 7 ▪ 8 ▪ 9 • Chromosome 2411. interrupted aortic arch. all • 39 patients with DOS should receive routine killed or subcomponent vaccines_ However. and oral polio virus vaccines) for these • 41 • 42 End Block Feedback . and other common conditions include tetralogy of Fallot. thymic hypoplasia. 27 dysmorphia. facial . patients can have T-cell lymphopenia and increased risk of 37 viral and fungal infections_ Humoral immunodeficiency can also result from defective T-cell help in B-cell 38 activation for antibody production. increasing susceptibility to bacterial infections as well_ Therefore.

29 (Choice 0) Congenital heart disease (especially coarctation of the aorta) and ovarian failure are common • 30 manifestations of Turner syndrome. However. Neonates are at high risk for life-threatening • 11 tetany. is a feature of DGS. However. • 10 Flypocalcemia results from hypoplasia of the parathyroid glands. and oral polio virus vaccines) for these • 21 patients depends on the degree of immunodeficiency_ Decisions regarding administration should be tailored • 22 to the patient's immune status. However. patients can have T-cell lymphopenia and increased risk of • 17 viral and fungal infections_ Humoral immunodeficiency can also result from defective T-cell help in B-cell • 18 activation for antibody production. the safety of live • 20 vaccines (measles-mumps-rubella. 28 . intranasal influenza. 27 seen in infants of diabetic mothers and in disorders of glycogen metabolism or gluconeogenesis. serum calcium levels and echocardiography must be ordered immediately. A DiGeorge syndrome (DGS) is disorder usually caused by the microdeletion of chromosome 2201. rotavirus. • 15 • 16 Depending on the degree of thymic hypoplasia. and hypocalcemia. 34 35 (Choice F) Lymphopenia. and arrhythmias that can further exacerbate co-existing heart problems and may require • 12 aggressive calcium repletion. Therefore. phenytoin. • 26 (Choice C) Patients with DGS do not have problems with glycemic control. cleft palate. However.• 1 • 2 Item: 30 of 44 V-Mark ▪ 3 Previous Next Lab Values. 31 32 (Choice E) Folic acid antagonists (eg. all • 19 patients with DGS should receive routine killed or subcomponent vaccines_ However. resulting ▪ 5 in poor development of the pharyngeal pouch system and subsequent abnormalities of the face. rather than neutropenia. Notes Calculator. interrupted aortic arch. 36 37 (Choice G) Patients with congenital rubella syndrome can have congenital heart disease and 38 thrombocytopenia. increasing susceptibility to bacterial infections as well. patients with DOS generally do not have platelet abnormalities. and other common conditions include tetralogy of Fallot. neck. Truncus arteriosus is strongly associated • 14 with DGS. hypoglycemia can be . The clinical presentation typically includes the pentad of congenital heart disease. thymic hypoplasia. and ▪ 6 mediastinum. and septal defects. These patients are at • 25 increased risk of duodenal atresia. • 4. methotrexate) increase the risk of neural tube defects and 33 possibly cardiac anomalies and oral clefts. Echocardiography is critical as the cardiac anomalies in DGS are variable and • 13 place the patient at great risk for heart failure and hypoperfusion. • 39 • 40 Educational objective: • 41 • 42 End Block Feedback . • 23 • 24 (Choice A) Congenital heart disease and low-set ears are seen in Down syndrome. neural tube defects are not associated with DOS. seizures. Infertility is not a feature of DOS. DGS is not associated with intestinal malformations. facial ▪ 7 dysmorphia. ▪ 8 ▪ 9 Once DOS is suspected. .

and oral polio virus vaccines) for these • 11 patients depends on the degree of immunodeficiency_ Decisions regarding administration should be tailored • 12 to the patient's immune status. rotavirus. DGS is not associated with intestinal malformations. These patients are at • 14 increased risk of duodenal atresia. • 4 with Lg. and congenital heart disease. However. • 21 • 22 (Choice El Folic acid antagonists (eg.• 1 • 2 Item: 30 of 44 F'Mark ▪ 3 Previous Next Lab Values. ▪ 29 Educational objective: • 30 Newborns with DiGeorge syndrome must be assessed immediately for potentially life-threatening • 31 hypocalcemia. increasing susceptibility to bacterial infections as well. 27 (Choice G) Patients with congenital rubella syndrome can have congenital heart disease and . The diagnosis should be suspected in the setting of craniofacial anomalies. However. Therefore. • 15 • 16 (Choice C) Patients with DGS do not have problems with glycemic control. neural tube defects are not associated with DGS.. • 13 (Choice A) Congenital heart disease and low-set ears are seen in Down syndrome. methotrexate) increase the risk of neural tube defects and • 23 possibly cardiac anomalies and oral clefts.3. 28 thrombocytopenia_ However. patients can have T-cell lymphopenia and increased risk of ▪ 7 viral and fungal infections_ Humoral immunodeficiency can also result from defective T-cell help in B-cell ▪ 8 activation for antibody production. The spectrum of parathyroid gland dysfunction associated with the microdeletion • 37 • 38 • 39 Copyright @ UWorld Last updated: [1018/2014] • 40 • 41 • 42 End Block Feedback . Notes Calculator. patients with DGS generally do not have platelet abnormalities. However. hypoglycemia can be • 17 seen in infants of diabetic mothers and in disorders of glycogen metabolism or gluconeogenesis. is a feature of DGS. • 18 • 19 (Choice ID) Congenital heart disease (especially coarctation of the aorta) and ovarian failure are common • 20 manifestations of Turner syndrome. all ▪ 9 patients with DGS should receive routine killed or subcomponent vaccines_ However. phenytoin. intranasal influenza. 33 • 34 • 35 References: • 36 1. • 26 . and septa) detects_ ▪ 5 ▪ 6 Depending on the degree of thymic hypoplasia. interrupted aortic arch. • 24 • 25 (Choice F) Lymphopenia. and other common conditions include tetralogy of allot. rather than neutropenia. thymic • 32 hypoplasia. Infertility is not a feature of DGS. the safety of live • 10 vaccines (measles-mumps-rubella.

27 . Media Exhibit ▪ 5 ▪ 6 DiGeorge syndrome ▪ 7 o's ▪ 8 ▪ 9 • 10 • -1-1 • 12 • 13 • 14 • 15 • 16 • 17 Classic location • 18 • 19 and shape of • 20 thymus in • 21 newborn • 22 ("sail sign") • 23 • 24 • 25 • 26 . 28 ▪ 29 • :30 • 31 • 32 33 • 34 35 36 37 se 33 • 39 • 40 • 41 • 42 End Block Feedback .• -1 • 2 Item: 30 of 44 V-Mark ▪ 3 Previous Next Lab Values Notes Calculator • 4.

Notes . • 16 Pulmonary • 17 drtry • 18 • 19 • 20 • 21 • 22 Trumus • 23 arteriosus • 24- • 25 • 26 Ventricular • 27 septa! • 2a defect 29 • 31 • 32 • 33 • 34 35 36 -47 C WAIILEwerld. LL( 38 39 40 41 0 42 Feedback End Block. • 3 • 4 Media Exhibit • 6 ▪ 6 Truncus arteriosus ▪ 7 A • 8 Truncus arteriosus • 10 • 11 • 12 • V • 14 Ao r ta • 15. Calculator. .• 1 A • 2 Item: 30 of 44 !Mark Previous Next Lab Values.

underused colon • 28 I (microcolon). Although only 20% of patients with CF • 24 develop meconium ileus. The disease is most commonly • 25 due to a mutation in the CF transmembrane conductance regulator gene. the free air above the liver in this patient's x-ray was indicative of intestinal perforation and required • 30 emergency surgery. °pacification of all sinuses can be seen as • 34 early as age 8 months. resulting in abnormal chloride and • 26 sodium transport and resultant tenacious secretions in multiple organs. resulting in obstruction at the level of the ileum and a narrow. Although contrast enema is typically performed to assess level of bowel obstruction in stable 29 patients. Pseudornorras • 37 aentginosa). and patients often require surgical debridement of their sinuses during childhood and • 35 adulthood. the earliest life-threatening manifestation • 23 of cystic fibrosis (CF). Chronic lung inflammation and recurrent pneumonias can progress to irreversible bronchiectasis_ • 36 Because of frequent treatment with aminoglycosides for Gram-negative infections (eg. • 19 These 2 conditions have overlapping clinical features but can usually be differentiated by the level of intestinal • 20 obstruction and consistency of the meconium (Table). almost all newborns with meconium ileus have CF. Notes Calculator. • 38 (Choices B and C) Patients with CF are no more likely to develop Alzheimer's disease or hypothyroidism • 39 compared to the general population_ On the other hand. 4 5 6 Differential diagnosis of delayed passage of meconium 7 8 Diagnosis Level of obstruction Meconium consistency 9 • 10 Hirschsprung • 11 Rectosigmoid Normal disease • 12 • 13 Meconium ileus Ileum Inspissated • 14 • 15 OUSMLEWorld. The discovery of inspissated meconium should prompt sweat chloride testing to confirm the diagnosis of CF • 32 • 33 Nearly all patients with CF develop sinopulmonary disease. • 21 • 22 This patient's presentation is most consistent with meconium ileus.LLC I • 16 • 17 Meconium ileus and Hirschsprung disease (congenital aganglionic megacolon) should be considered in any • 18 neonate who has delayed passage of meconium as 99% of full-term infants stool within 48 hours of birth. 2 Item: 31 of 44 F'Mark 3 Previous Next Lab Values. Hirschsprung disease is associated with Down St • 40 • 41 • 42 End Block Feedback . 10%-50% of patients may develop sensorineural hearing loss (Choice E). Meconium ileus is virtually diagnostic for CF. The meconium is as thick as glue • 27 and difficult to propel.

almost all newborns with meconium ileus have CF The disease is most commonly ▪ 8 due to a mutation in the CF transmembrane conductance regulator gene. The meconium is as thick as glue • 10 and difficult to propel. • 32 • 33 References: • 34 • 35 1. • 4- ▪ 5 This patient's presentation is most consistent with meconium ileus. Rhinosinusitis in cystic fibrosis: not a simple story. Notes Calculator. Pseudornonas • 20 aeruginosa). • 26 Infertility is due to the combination of secondary amenorrhea from malnutrition and thick cervical mucus • 27 obstructing sperm entry. • 28 • 29 Educational objective: • 30 Meconium ileus is virtually diagnostic for cystic fibrosis. Although contrast enema is typically performed to assess level of bowel obstruction in stable • 12 patients. Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis? • 36 2. only 20% women with CF have fertility problems. which in turn is associated with these comorbidities_ • 24 • 25 (Choice 0) In contrast to men. the free air above the liver in this patient's x-ray was indicative of intestinal perforation and required • 13 emergency surgery. • 21 (Choices B and C) Patients with CF are no more likely to develop Alzheimer's disease or hypothyroidism • 22 compared to the general population_ On the other hand. Hirschsprung disease is associated with Down • 23 syndrome. who are usually infertile. and patients often require surgical debridement of their sinuses during childhood and • 18 adulthood. resulting in abnormal chloride and ▪ 9 sodium transport and resultant tenacious secretions in multiple organs. 10%-50% of patients may develop sensorineural hearing loss (Choice E).• 1 • 2 Item: 31 of 44 F' Mark ▪ 3 Previous Next Lab Values. Opacification of all sinuses can be seen as • 17 early as age 8 months. resulting in obstruction at the level of the ileum and a narrow. The discovery of inspissated meconium should prompt sweat chloride testing to • 14 confirm the diagnosis of CF • 15 • 16 Nearly all patients with CF develop sinopulmonary disease. underused colon • 11 (microcolon). the earliest life-threatening manifestation ▪ 6 of cystic fibrosis (CF)_ Meconium ileus is virtually diagnostic for CF Although only 20% of patients with CF ▪ 7 develop meconium ileus. • 37 • 38 • 39 Copyright © LIWorld Last updated: [11/23/2014] • 40 • 41 • 42 End Block Feedback . Inspissated meconium is responsible for life-threatening obstruction at the level of the ileum. Chronic lung inflammation and recurrent pneumonias can progress to irreversible bronchiectasis_ • 19 Because of frequent treatment with aminoglycosides for Gram-negative infections (eg.

-1 2 Item: 31 of 44 . 11 . 3 Previous Next Lab Values Notes Calculator 4 Media Exhibit X ▪ 5 ▪ 6 ezonium ileus ▪ 7 • 8 • 9 • 10 • -1-1 • 12 • 13 • 14 • 15 • 16 • 17 • 19 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 23 29 • 30 • 32 • 33 • 34 • 35 • 36 • 37 • 39 • 39 • 40 • L1-1 • 42 End Block Feedback .111M Mark FF.

• -1 • 2 Item: 31 of 44 .11M Mark 11 . Media Exhibit ▪ 5 ▪ 6 Meconium ileus ▪ 7 ▪ 8 ▪ 9 • 10 • 11 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 Contrast enema • 25 • 26 demonstrating • 27 microcolon • 23 • 29 • 30 • 32 • 33 • 34 • 35 • 36 • 37 V • 33 • 39 • 40 • • 41 42 Feedback a End Block . ▪ 3 Previous Next Lab Values Notes Calculator • 4.

2 Item: 31 of 44 V/lark 3 Previous Next Lab Values Notes Calculator 4. A 5 6 7 9 • 10 • -1 -1 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 29 • 30 • 32 • 33 • 34 • 35 • 36 • 37 • 30 • 39 • 40 • • 41 42 4.# Feedback a End Block .

11 1. ▪ 3 Previous Next Lab Values Notes Calculator • 4. Media Exhibit ▪ 5 ▪ 6 Bilateral maxillary sinusitis ▪ 7 o's ▪ 8 ▪ 9 • 10 • -1-1 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 32 • 33 • 34 • 35 • 36 • 37 se • 30 • 39 • 40 • 41 • 42 End Block Feedback . Mark 11 .• -1 • 2 Item: 31 of 44 .

Duodenal atresia [9%] • 17 • 18 D. Hypospadias [3%] • 19 • E. Pulse oximetry is 84% on room air. The child has flattened facies. 2 Item: 32 of 44 11 V. Notes Calculator. 4 5 6 A newborn boy is evaluated shortly after vaginal delivery for respiratory distress. Second-trimester prenatal ultrasound 8 showed bilateral hydronephrosis and oligohydramnios. His temperature is 36. Examination shows decreased aeration of • 10 both lungs and intercostal retractions. Bilateral cryptorchidism [1%] • 14 • 15 B. Wilms tumor [8%] • 23 • 24 Explanation: • 25 • 26 Potter sequence • 27 • 28 • 29 Urinary tract anomaly • 30 • 31 • 33 1 • 34 • 35 Anurialoliguria in utero • 36 • 37 • 38 • 39 V • 40 • 41 • 42 End Block Feedback . 9 and respirations are 50/min. and clubfeet • 11 What is the most likely underlying diagnosis in this patient? • 12 • 13 A. Congenital diaphragmatic hernia [10%] • 16 C. Posterior urethral valves [68%] • 20 H Prostatic hyperplasia [1%] • 21 • 22 G.7 C (98 F). He was born at 37 weeks 7 gestation to a primigravid mother who had no medical problems.Mark -4(1 3 Previous Next Lab Values. abdominal distension. pulse is 144/min.

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• 2 Item: 32 of 44 I'Mark
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1 Potter sequence
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Urinary tract anomaly
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• 13 Anuriaioliguna in utero
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• 19 Oligohydramnio7
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• 26 Pulmonary hypoplasia Flat facies Limb deformities
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LISMLEWorld, LLC
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Posterior urethral valves (PUV) are the most common cause of urinary tract obstruction in newborn
• 33 boys. Abnormal folds in the distal prostatic urethra obstruct urinary flow, resulting in progressive dilation of
• 34 the bladder, ureters and kidneys_ Prenatal ultrasonography findings of bladder distention, bilateral
• 35 hydroureters, and bilateral hydronephrosis are highly suggestive of PUV. Poor urine output in utero
• 36 results in oligohydramnios as fetal urine is a major source of amniotic fluid. Oligohydramnios in the second
• 37 trimester is ominous and associated with high perinatal mortality because normal amniotic fluid levels are
• 30 1 required for lung development Low amniotic fluid also restricts fetal movement, leading to a cascade of
• 39 physical anomalies including flat facies and limb deformities (Potter sequence).
• 40 so
• 41
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• 2 Item: 32 of 44 Mark
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▪ 3 Previous Next Lab Values. Notes Calculator.
• 4 t
▪ 5
▪ 6 Pulmonary hypoplasia Flat facies Limb deformities
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0 USMLEWorld, LLC
• 10
• 11
• 12 Posterior urethral valves (PUV) are the most common cause of urinary tract obstruction in newborn
• 13 boys. Abnormal folds in the distal prostatic urethra obstruct urinary flow, resulting in progressive dilation of
• 14 the bladder, ureters and kidneys_ Prenatal ultrasonography findings of bladder distention, bilateral
• 15 hydroureters, and bilateral hydronephrosis are highly suggestive of PUV. Poor urine output in utero
• 16 results in oligohydramnios as fetal urine is a major source of amniotic fluid. Oligohydramnios in the second
• 17 trimester is ominous and associated with high perinatal mortality because normal amniotic fluid levels are
• 18 required for lung development Low amniotic fluid also restricts fetal movement, leading to a cascade of
• 19 physical anomalies including flat facies and limb deformities (Potter sequence).
• 20
• 21 Other affected infants can present with poor urinary stream, straining with voiding, urosepsis, failure to thrive,
• 22 and renal failure. Diagnosis is confirmed by voiding cystourethrogram and cystoscopy. Treatment options
• 23 include PUV ablation and urinary diversion. Despite prenatal diagnosis and early surgical intervention, patients
• 24 are at high risk for permanent kidney damage.
• 25
(Choice A) Cr-yptorchidism is the failure of one or both testes to descend from the abdomen through the
• 26
inguinal canal(s) into the scrotum. Examination shows a hypoplastic or poorly rugated scrotum. However.
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cryptorchidism would not cause obstructive uropathy and oligohydramnios_
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• 29
(Choice B) Congenital diaphragmatic hernia is a life-threatening defect of the diaphragm that allows
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abdominal viscera to herniate into the chest, resulting in restriction of lung development Patients can present
• 31
with respiratory distress, but on examination the abdomen appears scaphoid due to loss of the abdominal
contents into the chest. Prenatal ultrasound demonstrates a mass in the chest (abdominal contents) and
• 33
sometimes polyhydramnios from esophageal compression, making this diagnosis unlikely.
• 34
• 35 (Choice C) Fetal swallowing is responsible for amniotic fluid removal. An atretic duodenum can obstruct
• 36 amniotic fluid clearance, resulting in polyhydramnios, in contrast to the oligohydramnios seen in this patient
• 37 Another significant finding on prenatal ultrasound includes the characteristic "double bubble" sign due to
• 38 dilation of the stomach and the first part of the duodenum.
• 39
• 40 frhnir-a rll 1-11.(nnqnqrliqQ is a rnnnnnitql qhnnrrnqIihf in inshirh thin ninnilin I innthrq nninnQ nn thin lainntrql rsi ir-Fqria se
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• 2 Item: 32 of 44 F'Mark
▪ 3 Previous Next Lab Values. Notes Calculator.
• 4- are at nigh risk ror permanent Kidney damage.
▪ 5
(Choice A) Cryptorchidism is the failure of one or both testes to descend from the abdomen through the
▪ 6
inguinal canal(s) into the scrotum. Examination shows a hypoplastic or poorly rugated scrotum. However,
▪ 7
cryptorchidism would not cause obstructive uropathy and oligohydramnios_
▪ 8
▪ 9
(Choice B) Congenital diaphragmatic hernia is a life-threatening defect of the diaphragm that allows
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abdominal viscera to herniate into the chest, resulting in restriction of lung development Patients can present
• 11
with respiratory distress, but on examination the abdomen appears scaphoid due to loss of the abdominal
• 12
contents into the chest Prenatal ultrasound demonstrates a mass in the chest (abdominal contents) and
• 13
sometimes polyhydramnios from esophageal compression, making this diagnosis unlikely.
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• 15 (Choice C) Fetal swallowing is responsible for amniotic fluid removal. An atretic duodenum can obstruct
• 16 amniotic fluid clearance, resulting in polyhydramnios, in contrast to the oligohydramnios seen in this patient
• 17 Another significant finding on prenatal ultrasound includes the characteristic "double bubble" sign due to
• 18 dilation of the stomach and the first part of the duodenum.
• 19
• 20 (Choice DO Hypospadias is a congenital abnormality in which the penile urethra opens on the ventral surface
• 21 of the penis rather than the tip. These patients are able to urinate but may have difficulty controlling the urinary
• 22 stream.
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• 24 (Choice F) Benign prostatic hyperplasia can cause obstructive uropathy at the same level as posterior
• 25 urethral valves. However, this condition is common in men age >513 and does not affect children.
• 26
• 27 (Choice G) Wilms tumor (nephroblastoma) is the most common renal malignancy in childhood. Most
• 28 children present at age 2-5 years with a large, palpable flank mass but no other symptoms. It is extremely
• 29 rare for Wilms tumor to present in utero.
• 30
• 31
Educational objective:
Posterior urethral valves are the most common cause of urinary tract obstruction in newborn boys. Classic
findings on prenatal ultrasonography include bladder distension, bilateral hydroureters, and bilateral
• 33
hydronephrosis_ Oligohydramnios from low urine production in utero can cause pulmonary hypoplasia and
• 34
postnatal respiratory distress_
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• 36
• 37 References:
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1. Congenital urethral anomalies in boys. Part I: posterior urethral valves.
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• 40 2. Risk factors for end stage renal disease in children with posterior urethral valves. se
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• 42 End Block
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2 Item: 32 of 44 V- Mark
3 Previous Next Lab Values. Notes Calculator.
4
5 (Choice B) Congenital diaphragmatic hernia is a life-threatening defect of the diaphragm that allows
6 abdominal viscera to herniate into the chest, resulting in restriction of lung development Patients can present
7 with respiratory distress, but on examination the abdomen appears scaphoid due to loss of the abdominal
8 contents into the chest Prenatal ultrasound demonstrates a mass in the chest (abdominal contents) and
1 sometimes polyhydramnios from esophageal compression, making this diagnosis unlikely.
9
• 10
(Choice C) Fetal swallowing is responsible for amniotic fluid removal. An atretic duodenum can obstruct
• 11
amniotic fluid clearance, resulting in polyhydramnios, in contrast to the oligohydramnios seen in this patient
• 12
Another significant finding on prenatal ultrasound includes the characteristic "double bubble" sign due to
• 13
dilation of the stomach and the first part of the duodenum.
• 14
• 15 (Choice 0) Hypospadias is a congenital abnormality in which the penile urethra opens on the ventral surface
• 16 of the penis rather than the tip. These patients are able to urinate but may have difficulty controlling the urinary
• 17 stream.
• 18
• 19 (Choice F) Benign prostatic hyperplasia can cause obstructive uropathy at the same level as posterior
• 20 urethral valves. However, this condition is common in men age >5Q and does not affect children.
• 21
• 22 (Choice G) Wilms tumor (nephroblastoma) is the most common renal malignancy in childhood. Most
• 23 children present at age 2-5 years with a large, palpable flank mass but no other symptoms. It is extremely
• 24 rare for Wilms tumor to present in utero.
• 25
• 26 Educational objective:
• 27 Posterior urethral valves are the most common cause of urinary tract obstruction in newborn boys. Classic
findings on prenatal ultrasonography include bladder distension, bilateral hydroureters, and bilateral 4
• 28
• 29 hydronephrosis_ Oligohydramnios from low urine production in utero can cause pulmonary hypoplasia and
• 31h:1 postnatal respiratory distress_
• 31
References:
• 33
1. Congenital urethral anomalies in boys. Part I: posterior urethral valves.
• 34
• 35 2. Risk factors for end stage renal disease in children with posterior urethral valves.
• 36 3. Fetal surgery for posterior urethral valves: long-term postnatal outcomes.
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Copyright © UWorld Last updated: [1018/2014]
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LL1: posterior urethral membrane • 30 • 31 • 33 • 34 • 35 • 36 • 37 • 38 • 39 • 40 • 41 • 42 End Block Feedback .• -1 • 2 Item: 32 of 44 V/lark ▪ 3 Previous Next Lab Values Notes Calculator • 4. Media Exhibit X ▪ 5 ▪ 6 Posterior urethral valves ▪ 7 ▪ 8 Posterior urethral valves ▪ 9 Normal urinary system Posterior urethral valves • 10 • 11 • 12 • 13 Kidney Hydro- • 14 nephrosis • 15 • 16 • 17 • 18 Ureter Dilated • 19 ureters • 20 • 21 • 22 Distended • 23 Bladder bladder • 24 • 25 • 26 • 27 • 28 Urethra Urethra obstructed by congenital • 29 IDUSIALEVO-dd.

• -1 • 2 Item: 32 of 44 V/lark ▪ 3 Previous Next Lab Values Notes Calculator • 4. 141edia Exhibit ▪ 5 ▪ 6 Cryptordiidism ▪ 7 ▪ 8 ▪ 9 • 10 • -1 -1 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 33 • 34 • 35 • 36 • 37 • 30 • 39 • 40 • 41 • 42 End Block Feedback .

Which of • 12 the following is the most appropriate recommendation? • 13 • 14 • 15 A. Apply sun protection factor 50 sunscreen 30 minutes before sun exposure [72°4] • 16 B. thick. long-sleeved shirts • 34 Prevention O Tightly woven. Physical examination shows a well-appearing adolescent with olive skin. Tender. Her parents say that "everybody tans easily" in their family. blanching erythema is present on the face and arms. Wear light-colored clothing while outdoors [5%] • 24 • 25 Explanation: • 26 • 27 • 28 Sunburn • 29 • 30 • 31 • Remain indoors between 10 AM-4 PM • 32 • \Near protective clothing: O Hats. Refrain from outdoor activities on cloudless days [6%] • 22 • 23 G.11V-Mark ▪ 3 Previous Next Lab Values. Apply topical corticosteroids to inflamed skin [4%] • 17 C. pants. Notes Calculator. brown eyes. or dark-colored fabrics • 35 • Apply sunscreen 3D minutes before sun exposure • 36 • 37 • Avoid tanning beds • 38 • 39 • Mild-moderate sunburn: • 40 so • 41 • 42 End Block Feedback . She just returned from a week-long ▪ 7 vacation to the Bahamas with her family. and light-brown • 11 hair. This is her first ▪ 9 sunburn. Change to sun protection factor 75 sunscreen [11%] • 18 • 19 D. There is no edema or vesicles.• 1 • 2 Item: 33 of 44 . Continue current sun protection regimen [2970] • 20 E Form a baseline tan with a tanning bed before sun exposure [0°•0] • 21 F. Family history is negative for skin • 10 cancer. the patient has had redness on her face and ▪ 8 shoulders despite applying sun protection factor 50 sunscreen just before swimming. • 4- ▪ 5 ▪ 6 A 13-year-old girl is brought to the physician for evaluation of sunburn. For the past 2 days.

P 3 Previous Next Lab Values. Mark U. everyone is at risk of • 35 the carcinogenic effects of excessive UV exposure and could benefit from education regarding • 36 photo-protection. If it is • 39 impractical to stay indoors (Choice F).. • 4. especially during peak hours of UV intensity. A 5 6 Sunburn 7 . thick. • 37 • 38 The best method of photo-protection is sun avoidance. Excessive exposure • 34 to UV light increases the risk of photoaging and skin cancer_ Regardless of skin type. Notes Calculator. broad-spectrum sunscreens with sun protection factor (SPF) 15-30 • 40 or higher should be used reoularlv when outdoors Sunscreen should be =lied 15-30 minutes before sun so • 41 • 42 End Block Feedback . aloe vera • 19 0 Oral: NSAIDs Treatment • 20 • Severe sunburn: Hospitalization • 21 0 Intravenous fluids & analgesia • 22 0 Wound care • 23 • 24 • 25 • Cancer: • 26 0 Melanoma • 27 Complications 0 Basal cell carcinoma • 28 0 Squamous cell carcinoma • 29 • Photoaging • 30 • 31 LIWorld • 32 Sunburn is an inflammatory response to excessive exposure of ultraviolet (UV) radiation.• 2 Item: 33 of 44 r. 8 • Remain indoors between 10 AM-4 PM 9 • Wear protective clothing: • 10 0 Hats. calamine lotion. or dark-colored fabrics • 13 • Apply sunscreen 30 minutes before sun exposure • 14 • Avoid tanning beds • 15 • 16 • Mild-moderate sunburn: • 17 • 18 0 Topical: Cool compresses. long-sleeved shirts • 11 Prevention • 12 0 Tightly woven. pants.

• 36 • 37 Educational objective: • 38 Sun avoidance is the best form of photo-protection_ Sunscreen with sun protection factor 15-30 or higher • 39 should be applied 15-30 minutes prior to sun exposure to allow time for development of a protective film. everyone is at risk of 5 the carcinogenic effects of excessive UV exposure and could benefit from education regarding 6 photo-protection. However. broad-spectrum sunscreens with sun protection factor (SPF) 15-30 • 10 or higher should be used regularly when outdoors. Notes Calculator. and 98%. which increases exposure to sunscreen chemicals. snow. 7 The best method of photo-protection is sun avoidance. and 50 sunscreens is 93%. The family should be educated • 29 about applying sunscreen 15-30 minutes prior to sun exposure. • 16 As a result. V • 40 r • 41 • 42 End Block Feedback . The amount of UVB radiation filtered by SPF • 25 15. tanning bed use in teens and young adults is associated with a 75% increase in melanoma. • 34 (Choice G) Although dark fabrics attract more heat than light fabrics. • 21 (Choice B) Topical corticosteroids are commonly used to soothe sunburns. SPF 100 sunscreen filters 99% of UVB rays). they actually offer greater UV protection • 35 because the rays cannot penetrate the fabric as well. patients should be informed that cloud • 15 coverage does not block UV rays and that UV rays can be reflected off water. a small amount of • 20 sunscreen can be used if sun exposure is unavoidable and additional protection is necessary. but there is little evidence that • 22 they reduce symptoms and healing time. patients can burn in the shade or while skiing in the winter. patients should be reminded that sunscreen • 14 alone does not fully protect against sun damage_ In addition. Regardless of skin type. • 30 1. Sunscreens with SPF >50 provide a • 26 negligible increase in UV protection (eg. Sunscreen should be applied 15-30 minutes before sun • 11 exposure to allow the formation of a protective film on the skin and reapplied at least every 2 hours. • 27 • 28 (Choice 0) This patient has been applying sunscreen just before swimming. 97%. If it is 9 impractical to stay indoors (Choice F). In fact. reapplication is needed even for products • 13 labeled "water-resistant" or "very water-resistant. and concrete. especially during peak hours of UV intensity. respectively. 4 to UV light increases the risk of photoaging and skin cancer. sand. 2 Item: 33 of 44 V-Mark 3 Previous Next Lab Values. • 31 11= (Choice E) "Fre-tanning" with tanning beds can increase skin pigmentation but does not protect against • 32 sunburn. • 17 • 18 Sunscreen is generally avoided in infants age < months because of their thin skin and high surface • 19 area-to-body weight ratio. 30. • 23 • 24 (Choice C) Sunscreens are rated by the strength of their SPF." However. • 12 Because sunscreen is washed off with swimming and sweating.

• 17 • 18 (Choice 0) This patient has been applying sunscreen just before swimming_ The family should be educated • 19 about applying sunscreen 15-30 minutes prior to sun exposure. and concrete. Sunscreens with SPF >50 provide a • 16 negligible increase in UV protection (eg. The amount of LJVB radiation filtered by SPF • 15 15. • 4. tanning bed use in teens and young adults is associated with a 75% increase in melanoma • 23 • 24 (Choice G) Although dark fabrics attract more heat than light fabrics. 30. Sunscreen with sun protection factor 15-30 or higher • 29 should be applied 15-30 minutes prior to sun exposure to allow time for development of a protective film. snow. respectively. which increases exposure to sunscreen chemicals. The association of use of sunbeds with cutaneous malignant melanoma and other skin cancers: A systematic review. and 50 sunscreens is 93%. SPF 100 sunscreen filters 99% of UVB rays). and 98%. they actually offer greater UV protection • 25 because the rays cannot penetrate the fabric as well. ▪ 6 As a result. a small amount of • 10 sunscreen can be used if sun exposure is unavoidable and additional protection is necessary. • 11 (Choice B) Topical corticosteroids are commonly used to soothe sunburns.• 1 • 2 Item: 33 of 44 F Mark ▪ 3 Previous Next Lab Values. However. • 37 • 38 • 39 Copyright © UWorld Last updated: [9/23/2014] • 40 • 41 • 42 End Block Feedback . • 30 • 31 References: • 32 1. Ultraviolet radiation: a hazard to children and adolescents. patients should be informed that cloud ▪ 5 coverage does not block UV rays and that UV rays can be reflected off water. 97%. sand. • 20 • 21 (Choice El "Pre-tanning' with tanning beds can increase skin pigmentation but does not protect against • 22 sunburn. • 36 3. • 26 • 27 Educational objective: • 28 Sun avoidance is the best form of photo-protection. Notes Calculator. but there is little evidence that • 12 they reduce symptoms and healing time. Photoprotection. • 13 • 14 (Choice C) Sunscreens are rated by the strength of their SPF. alone does not fully protect against sun damage_ In addition. In fact. • 34 • 35 2. ▪ 7 ▪ 8 Sunscreen is generally avoided in infants age < months because of their thin skin and high surface ▪ 9 area-to-body weight ratio. patients can burn in the shade or while skiing in the winter.

0 WI_ • 12 Erythrocyte count 21 mlnimm • 13 • 14 MCHC 32% • 15 MCV 105 fl • 16 Reticulocytes 2_0% • 17 Platelets 212. Notes Calculator. Removes sickled red blood cells from the circulation [3%] • 28 • 29 B. • 10 Complete blood count • 11 Hemoglobin 9. The patient's laboratory results are shown below. 1 • 2 Item: 34 of 44 V-Mark 3 Previous Next Lab Values. His physical 9 examination is unremarkable. Increasing the fetal hemoglobin dilutes the number of sickled cells in the circulation and • 40 v • 41 • 42 End Block Feedback . • 4- 5 6 A 10-year-old patient with sickle cell disease comes to the physician for a routine visit The patient has a 7 history of multiple pain crises and pneumonias. Hydroxyurea works by increasing the amount of fetal hemoglobin • 39 in the circulation. Lyses microthrombi in the circulation [3%] • 30 C. He was started on hydroxyurea one year ago and has not had 8 any further pain crises since then.0001mm3 • 18 Leukocyte count 9500/mm3 • 19 Neutrophils 56% • 20 Eosinophils 3% • 21 Lymphocytes 36% • 22 Monocytes 5% • 23 • 24 Which of the following best describes the role of hydroxyurea in the treatment of patients with sickle cell • 25 disease? • 26 • 27 A. Protects against encapsulated bacterial infections [11C] • 31 • ID. Increases fetal hemoglobin [91%] • 32 • 33 E Chelates iron to prevent iron toxicity [1%] • 35 Explanation: • 36 • 37 Hydroxyurea is a chemotherapy agent used in sickle cell disease to decrease vaso-occlusive pain crises as • 38 well as episodes of acute chest syndrome. He has had no recent illnesses or hospitalizations.

2 Item: 34 of 44 V-Mark 3 Previous Next Lab Values. and episodes of acute chest syndrome. • 22 • 23 (Choice A) Hydroxyurea does not remove sickled cells from the circulation. • 27 Hydroxyurea does not prevent or lyre these microthrombi. Hydroxyurea works by increasing the amount of fetal hemoglobin • 17 in the circulation. Removes sickled red blood cells from the circulation [3%] 6 7 B. The spleen is responsible for • 24 filtering out the sickled red blood cells. Protects against encapsulated bacterial infections [1561 9 D. Patients can be treated with iron chelators such as deferoxamine to help reduce the effects of e the transfusion related hemosiderosis_ • 35 •• 36 I Educational objective: • 37 Hydroxyurea benefits patients with sickle cell disease by increasing fetal hemoglobin. • 38 • 39 Copyright © LIWorld Last updated: [10/24/2014] • 40 • 41 • 42 End Block Feedback . Increases fetal hemoglobin [91%] • 10 • 11 E Chelates iron to prevent iron toxicity [196] • 12 • 13 Explanation: • 14 • 15 Hydroxyurea is a chemotherapy agent used in sickle cell disease to decrease vaso-occlusive pain crises as • 16 well as episodes of acute chest syndrome. the need for • 19 transfusions. Side effects occur because hydroxyurea suppresses • 20 the bone marrow. anemia. and thrombocytopenia may occur. 4 A 5 A. Frequent transfusions may lead to • 33 iron toxicity. • 32 (Choice E) Patients with sickle cell disease often require transfusions. but decreases the proportion of sickled cells in the • 28 circulation_ • 29 • 30 (Choice C) Prophylactic penicillin is given to sickle cell patients to prevent infection with encapsulated 31 irm organisms. Notes Calculator. These effects are generally • 21 temporary and reversible but may predispose the patient to infection. Hydroxyurea has been shown to decrease pain crises. Lyses microthrombi in the circulation [3%] 8 C. Increasing the fetal hemoglobin dilutes the number of sickled cells in the circulation and • 18 reduces vaso-occlusive episodes. • 25 • 26 (Choice B) Microthrombi caused by the sickled cells are the cause for vaso-occlusive pain crises. Leukopenia.

P 3 Previous Next Lab Values.101 . • 4- 5 6 A 17-year-old girl is admitted to the hospital for purging behavior. Physical examination shows an emaciated girl with bibasilar crackles and jugular venous distension_ • 11 Serum chemistry results are as follows: • 12 Sodium 138 mEq/L • 13 Potassium 2_1 • 14 Chloride 92 mEgil_ • 15 Bicarbonate 28 mail • 16 Blood urea nitrogen 14 mg/dL • 17 Creatinine 0_8 mg/dL • 18 Calcium 8 mg/dL • 19 Glucose 90 mgldL • 20 Magnesium 1 mg/dL • 21 Phosphorus 0_9 mg/dL • 22 • 23 A surge in which of the following hormones best explains this patient's deterioration? • 24 • 25 • 26 A. Glucagon [5%] • 29 • 30 ID. On the second day of admission. the patient complains of 8 severe shortness of breath and has recurrent episodes of nonsustained ventricular tachycardia. and syncope. heart rate is 11 /min. Her body mass index is 14 • 10 kg/m2. Notes Calculator. 1 • 2 Item: 35 of 44 V-Mark U. and respirations are 22/min. weight loss. Aldosterone [27%] • 27 Cortisol [18%] • 28 C. Her blood 9 pressure is 82/55 mm Hg. She is started on 7 nasogastric feeds and her activities are restricted. Oh se • 41 • 42 End Block Feedback .1. Insulin [42%)] • 31 E Triiodothyronine [8%] • 32 • 33 Explanation: • 36 • 37 Pathogenesis of refeeding syndrome • 38 • 39 • 40 I ORM .11 NA All ARAI 11.

4 Pathogenesis of refeeding syndrome 5 6 7 Starvation: Clinical manifestations 8 catabolic state • Arrhythmia 9 • 10 • Congestive heart failure • 11 (pulmonary edema.• 1 • 2 Item: 35 of 44 F'Mark 3 Previous Next Lab Values. • 30 magnesium & thiamine • 32 Depletion of fat. potassium. Serum thiamine • 22 use in brain Sodium & water retention • 23 • 24 • 25 • 26 Glycogenolysis I Glycogen synthesis • 27 t Lipolysis I Protein synthesis • 28 Intracellular uptake of t Protein catabolism • 29 phosphorus. Serum phosphorus. minerals & • 34 intracellular electrolytes 1 Insulin L • 36 • 37 • 30 I Start refeeding: • 39 anabolic state • 40 v • 41 • 42 End Block Feedback . protein. • 12 peripheral edema) 1 Insulin • 13 • Seizures Glucagon • 14 • Wernicke encephalopathy Cortisol • 15 • 16 • 17 • 18 1 Ketone bodies 1. • 19 use in muscle potassium. • 33 vitamins. magnesium • 20 • 21 ¶ Ketone bodies j. Notes Calculator.

sodium conservation. • 13 Phosphorus is the primary deficient electrolyte as it is required for energy (adenosine triphosphate).111.. they do not directly impact phosphorus or magnesium uptake.P 3 Previous Next Lab Values. Therefore. • 23 • 24 (Choice E) Patients with anorexia nervosa have euthyroid hypothyroxinemia. and potassium • 20 secretion in an attempt to maintain blood pressure. which in turn promotes cellular uptake of phosphorus. and magnesium. 5 anabolic state 6 7 USIPALEWorkl. • 14 Deficiencies in potassium and magnesium potentiate cardiac arrhythmias in a heart that is already atrophic • 15 from prolonged malnutrition. Notes Calculator. • 22 (Choice C) Glucagon activity is elevated during starvation but decreased during refeeding. which is characterized by • 25 normal thyroid-stimulating hormone and normal to decreased serum thyroxine and triiodothyronine_ • 26 • 27 Educational objective: • 28 Refeeding syndrome is a potentially fatal complication of nutritional rehabilitation in anorexia nervosa. • 31 • 32 References: • 33 1. 11. and magnesium. • 4 bian refeeding. and bibasilar lung crackles_ • 18 • 19 (Choices A and 6) Aldosterone and cortisol stimulate water resorption. Hypophosphatemia during nutritional rehabilitation in anorexia nervosa: implications for • 36 refeeding and monitoring. • 30 potassium. potassium. Clinical manifestations include arrhythmias and cardiopulmonary failure. aggressive initiation of nutrition without adequate electrolyte repletion • 16 can quickly precipitate cardiopulmonary failure. whether enteral or intravenous. 2. • 37 • 38 • 39 Copyright © LIWorld Last updated: [11/18/2014] • 40 • 41 • 42 End Block Feedback . • 29 Carbohydrate intake stimulates insulin activity. These steroid hormones are high during starvation but • 21 less so during refeeding_ In addition. This patient has signs of volume overload from heart failure • 17 based on increased pulse and respiratory rate.• 1 • 2 Item: 35 of 44 IM V-• Mark U. • 12 causes pancreatic insulin secretion and cellular uptake of phosphorus. Carbohydrate ingestion. jugular venous distension. LLC 8 9 Chronic starvation and acute refeeding are potentially life-threatening in patients with anorexia nervosa_ • 10 Refeeding syndrome is the constellation of pathologic derangements resulting from a surge in insulin activity • 11 as the body resumes anabolism (Flow chart). Nutritional rehabilitation: practical guidelines for refeeding the anorectic patient.

Physical examination shows multiple pustules on his right • 10 cheek.. Topical antibiotics (eg.1. Swab intact skin for culture [3%] • 18 • 19 • E Topical mupirocin [50%] • 20 • 21 Explanation: • 22 • 23 • 24 • 25 Impetigo • 26 • 27 Type Non-bullous Bullous • 28 • 29 • 30 Staphylococcus aureus • 31 Microbiology &/or Streptococcus Staphylococcus aureus • 32 pyogenes • 33 • 34 • 35 Rapidly enlarging flaccid bullae with Clinical Painful non-itchy pustules 36 yellow fluid.le-rieLe-. • 39 • 40 Tre. Anti-streptolysin 0 antibody titers [10%] • 14 • 15 B. . Oral cephalexin [29%] • 17 ID. • 4- 5 6 A 4-year-old boy is brought to physician for evaluation of a rash. Oral antibiotics (eg. the boy had a few small red 7 bumps on his chin and cheek. 1 • 2 Item: 36 of 44 F Mark 3 Previous Next Lab Values. cephalexin. Notes Calculator. A few days ago. N • 41 • 42 End Block Feedback . Some lesions are covered with a thick golden-yellow crust The rest of his • 11 examination is within normal limits. Express pus for culture [8%] • 16 C. nose. The patient has no other 8 medical problems and his vaccinations are up to date. What is the most appropriate next step in the management of this patient? • 12 • 13 A. and chin. The bumps became painful as they filled with fluid.n. He has been playing at the park daily and has had 9 occasional insect bites_ Vital signs are normal. ucollarette" of scale at features & honey-crusted lesions • 37 the periphery of ruptured lesions • 38 .

Topical antibiotics (eg. uccillarette" of scale at features & honey-crusted lesions • 18 the periphery of ruptured lesions • 19 • 20 • 21 Topical antibiotics (eg. Notes Calculator.an takp wp.d for natip. poor personal hygiene. insect bite). • 30 • 31 The superficial skin infection manifests with multiple painful pustules on the exposed areas of the face and • 32 extremities_ aver the course of a week. Predisposing factors include warm and humid climate. 36 mupirocin) are preferred due to less side effects and antibiotic resistance risk compared to oral therapy_ • 37 Thorough handwashing is important to prevent the spread of this contagious infection. or ciindamycin) • 23 QUSMLEWOrkl.• 1 • 2 Item: 36 of 44 V. Treatment • 22 mupirocin) didoxacillin.nprallv not • 41 • 42 End Block Feedback . and atopic dermatitis (eczema). • 4- ▪ 5 ▪ 6 Impetigo ▪ 7 ▪ 8 Type Non-bullous Bullous ▪ 9 • 10 • 11 Staphylococcus aureus • 12 Microbio logy 8Lior Streptococcus Staphylococcus aureus • 13 pyogenes • 14 I • 15 • 16 Rapidly enlarging flaccid bullae with Clinical Painful non-itchy pustules • 17 yellow fluid.Mark ▪ 3 Previous Next Lab Values.p.ks to riso and am np. cephalexin. • 34 • 35 Antibiotics are indicated to reduce infection transmission and recovery time. Colonization with staphylococci • 29 or streptococci is also a risk factor. Culture of pustular fluid can be • 40 nonsidprp. LLC • 24 • 25 The patient's rash is consistent with localized non-bullous impetigo_ Impetigo is a common pediatric rash that • 26 is typically caused by Staphylococcus aureus or group A beta-hemolytic Streptococcus (Streptococcus • 27 pyogerres). • 28 pre-existing skin trauma (eg. poverty. • 38 • 39 (Choices A and B) Impetigo diagnosis is based on clinical presentation. crowding.nts whn fail pninirin thpranv Antibody titprs r. the pustules eventually rupture and harden into a characteristic • 33 golden-yellow ("honey") crust Local lymphadenopathy can be present but fever and chills are unusual. Oral antibiotics (eg.

Oral antibiotics are also • 24 indicated when topical therapy is impractical for widespread non-bullous impetigo. Colonization with staphylococci ▪ 8 or streptococci is also a risk factor. the pustules eventually rupture and harden into a characteristic golden-yellow ("honey") crust Local lymphadenopathy can be present but fever and chills are unusual. Notes Calculator. It is usually caused by • 23 S aureus and should be treated with oral cephalexin.• 1 • 2 Item: 36 of 44 F' Mark ▪ 3 Previous Next Lab Values. • 37 • 38 • 39 Copyright © UWorld Last updated: [8/2G/2014] • 40 • 41 • 42 End Block Feedback . Treatment of impetigo: oral antibiotics most commonly prescribed. I ne patients rasn IS consistent vim localizeo non-ounous impetigo_ impetigo is a common peal-al-sic rasn mat ▪ 5 is typically caused by Staphylococcus aureus or group A beta-hemolytic Streptococcus (Streptococcus ▪ 6 pyogenes). • 31 • 32 References: • 33 1. • 14 mupirocin) are preferred due to less side effects and antibiotic resistance risk compared to oral therapy_ • 15 Thorough handwashing is important to prevent the spread of this contagious infection. crowding. 36 3. insect bite). • 30 Topical mupirocin is the treatment of choice. diagnosis is clinical. and atopic dermatitis (eczema). Impetigo . • 25 • 26 (Choice 13) Swabs of intact skin are not useful as skin flora would be detected. Over the course of a week. Bacterial resistance and impetigo treatment trends: a review. • 12 • 13 Antibiotics are indicated to reduce infection transmission and recovery time.review. poverty. • 27 • 28 Educational objective: • 29 Non-bullous impetigo is characterized by painful pustules and honey-crusted lesions. ▪ 7 pre-existing skin trauma (eg. • 21 • 22 (Choice C) Bullous impetigo is characterized by flaccid bullae containing yellow fluid. Antibody titers can take weeks to rise and are generally not • 19 useful in acute illness_ Anti-streptolysin 0 titers can be considered for patients who develop complications • 20 such as post-streptococcal glomerulonephritis and rheumatic fever. Topical antibiotics (eg. dicloxacillin. Predisposing factors include warm and humid climate. or clindamycin. ▪ 9 The superficial skin infection manifests with multiple painful pustules on the exposed areas of the face and • 10 • 11 extremities. • 4. poor personal hygiene. Culture of pustular fluid can be • 18 considered for patients who fail empiric therapy. • 34 • 35 2. • 16 • 17 (Choices A and El) Impetigo diagnosis is based on clinical presentation.

.• 1 • 2 Item: 37 of 44 ......A .:11.... she has had rhinorrhea and ▪ 7 nasal congestion.. Otoscopy shows bulging. The girl has no medical problems and takes no medications. Oral antibiotics [80%] • 19 • 20 ID. Ototopical antibiotics [4%] • 21 E Tympanocentesis and culture [2°/0] • 22 E Viral nasopharyngeal polymerise chain reaction testing [11.‘.. • 4 ▪ 5 ▪ 6 An 11-month-old girl is brought to the physician for fever..P ▪ 3 Previous Next Lab Values.. Her • 10 temperature is 38. Notes Calculator. Both parents smoke cigarettes. She attends ▪ 9 day care and her diet consists of infant formula and finger foods...-. follow-up in 2 days [9%] • 17 Myringotomy with tympanostomy tube placement [3%] • 18 ▪ C..1. Her hearing is intact and • 12 the rest of her examination is normal_ Which of the following is the most appropriate next step in • 13 management of this patient? • 14 • 15 • 16 A.:4. Crusted rhinorrhea is present at the nares.. Acetaminophen. erythematous bilateral tympanic membranes with • 11 decreased mobility on air compression. • 41 • 42 End Block Feedback .. For the past few days.9 C (102 F). The fever started 2 days ago and the girl has since been pulling at both ears.11M Mark U.6] • 23 • 24 • 25 Explanation: • 26 • 27 Acute otitis media • 28 • 29 • 30 • Streptococcus pneurnoniae • 31 Microbiology • Nontypeable Haemophilus influenzae • 32 • Moraxella catarrhafis • 33 • 34 • 35 • Middle ear effusion • 36 Clinical plus a 37 features • Bulging tympanic membrane • 38 • 39 • 40 _ 1. The ear pain ▪ 8 seems worse when she lies down.

• 1 • 2 Item: 37 of 44 Al I Mark ▪ 3 Previous Next .. craniofacial anomalies. ri- se • 41 • 42 End Block Feedback . • 39 • 40 rs Ali.41. and Moraxelta catarrhafis_ The first-line treatment is a 1D-day course of high-dose amoxicillin. I{5 J I . exposure to cigarette smoke.. AOM is an extremely common • 29 condition in children age 6-36 months as their Eustachian tubes are short and easily clogged. fr. • 32 • 33 The most common offending pathogens include Streptococcus pneurnoniae.r r. cholesteatoma. Potential complications of recurrent AOM are numerous and include chronic suppurative otitis media. Risk factors • 30 include formula intake (rather than breast milk)..k. allergic rhinitis or viral upper • 31 respiratory infection. tympanosclerosis. ". and chronic middle ear effusion. If • 35 AOM returns within a month of initial treatment. mastoiditis. • 4 ▪ 5 Acute otitis media ▪ 6 ▪ 7 ▪ 8 • Streptococcus pneumomae ▪ 9 Microbiology • Nontypeable Haeinophilus int7uenzae • 10 • Moraxella catarrhaiis • 11 • 12 • 13 • Middle ear effusion Clinical • 14 plus • 15 features • Bulging tympanic membrane • 16 • 17 • 18 • Initial: Amoxicillin • 19 Treatment • 2nd line Amoxicillin-clavulanic acid • 20 • 21 • 22 • Conductive hearing loss • 23 Complications • Mastoiditis • 24 • Meningitis • 25 • 26 cif USMLEWorldr LLC • 27 • 28 This patient's clinical presentation is consistent with acute otitis media (ADM).1-.-4 Ir. amoxicillin-clavulanic acid should be given in anticipation of • 36 infection with beta-lactamase-resistant strains. 111•FMI eardrum perforation. nontypeable Haemopbllus • 34 influenzae. Notes Calculator. Lab Values. and conductive hearing loss. labyrinthitis..

but not for AOM. labyrinthitis. but delaying • 13 antibiotics in infants and young children could increase the risk of developing complications. non-typeable Haernophitus influeraae. Notes Calculator. Potential complications of recurrent AOM are numerous and 9 include chronic suppurative otitis media. especially with cigarette smoke • 29 exposure. • 23 • 24 (Choice F) Although viral upper respiratory infections often precede or occur with A011. has a normal immune system. L3 episodes within 6 months or L. chronic • 22 suppurative otitis media. • 11 • 12 (Choice A) Analgesia (eg. • 38 • 39 Copyright © LIWorld Last updated: [10/20/2014] • 40 • 41 • 42 End Block Feedback .Mark 3 Previous Next Lab Values. Tympanocentesis and culture during myringotomy with tympanostomy tube placement should be • 19 considered in children with multiple episodes of ACM (eg. and Moraxetta catarrhatis. The first-line treatment is a 10-day course of high-dose amoxicillin. nontypeable Haernoplidus 6 influenzae. cholesteatoma. day care attendance. or external otitis. • 16 • 17 (Choices B and E) Empiric antibiotics are usually adequate for complete treatment of • 18 AOM. If 7 ACM returns within a month of initial treatment. 1 2 Item: 37 of 44 V. recent or concurrent upper respiratory infection. Otitis media: diagnosis and treatment. •36 2. ibuprofen. Observation is a • 14 reasonable option if the child is age years. amoxicillin-clavulanic acid should be given in anticipation of 8 infection with beta-lactamase-resistant strains. • 31 and Moraxella catarrhafis_ Oral amoxicillin should be administered to prevent complications (eg. tympanosclerosis. and symptoms are mild and • 15 unilateral. • 26 • 27 Educational objective: • 28 Acute otitis media is a common infection in infants and young children. The • 30 most common causative organisms are Streptococcus prreurnorriae. 4- 5 The most common offending pathogens include Streptococcus prreurnornae. acetaminophen) is appropriate for relief from otalgia. • 10 eardrum perforation. and conductive hearing loss. mastoiditis. viral identification • 25 would not change management. which consists of empiric antibiotic treatment. • 32 • 33 References: • 34 • 35 1. mastoiditis). The diagnosis and management of acute otitis media. and formula intake.4 episodes within • 20 12 months) despite appropriate antibiotic treatment • 21 (Choice 0) Therapy with otic drops is appropriate for otorrhea from tympanostomy tubes.

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Normal pneumatized
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mastoid air cells
°pacified middle ear cavity
• 20 °pacified mastoid air cells
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A

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• 2 Item: 38 of 44 V-• Mark
▪ 3 Previous Next Lab Values. Notes Calculator.
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5
▪ 6 A 12-year-old boy is brought to the emergency department because of severe pain near his left knee. He has
▪ 7 sickle cell disease and has been hospitalized previously for sickle cell crisis_ Vital signs are notable for
▪ 8 persistent fever. Examination of the left lower extremity reveals a normal knee joint with marked tenderness
▪ 9 and swelling over the proximal tibia. Laboratory studies show leukocytosis and elevated ESR_ Imaging
• 10 studies confirm the diagnosis of osteomyelitis. Which of the following organisms is the most likely cause of
• 11 his condition?
• 12
• 13
• 14
A. Escherichia cob. [1%]
• 15 Pseudornonas species [29C]
• 16
• C. Salmonella species [929C]
• 17
• 18
D. Streptococcus pneurnoniae [4%]
• 19 E. Group B streptococcus [1%]
• 20
• 21
• 22 Explanation:
• 23
This patient presents with clinical findings suggestive of osteomyelitis in the setting of sickle cell disease.
• 24
Because of the early decline in their splenic function, patients with sickle cell disease are more susceptible to
• 25
infections of the bone and joints_ Salmonella and Staph aureus are the most common cause of osteomyelitis
• 26
in patients with sickle cell disease. Although controversial, more recent studies have shown that S. aureus is
• 27
more common than Salmonella in children with sickle cell.
• 28
• 29
(Choices A and El E. colt and group B streptococcus are common causes of septic arthritis and
• 30
osteomyelitis in neonates_ Group B streptococcus is the most common organism responsible for
• 31
osteomyelitis in children less than one year of age.
• 32
• 33 (Choice B) Pseudomonal osteomyelitis is seen in diabetic patients and after puncture wounds of the foot and
• 34 is a less common cause of osteomyelitis in children with sickle cell_
▪ 35
• 36 (Choice 0) Sickle cell patients are more susceptible to encapsulated organisms due to abnormal function of
- 37 the spleen. and Streptococcus pneurnoniae is the most common cause of bacteremia in these children. S.
• 38 pneurnoniae typically causes meningitis and pneumonia and is a less likely cause of osteomyelitis.
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- 40 Educational objective: se
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A'S

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• 2 Item: 38 of 44 V/lark
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• 4 14.F..le Lel! dl IU ridgy =el I I iuNmi.dilLeu reviuuNy iur 14...Kle Lel! LI VILdI iyri dl e I IULdUle IUI

▪ 5 persistent fever_ Examination of the left lower extremity reveals a normal knee joint with marked tenderness
▪ 6 and swelling over the proximal tibia Laboratory studies show leukocytosis and elevated ER_ Imaging
▪ 7 studies confirm the diagnosis of osteomyelitis. Which of the following organisms is the most likely cause of
▪ 8 his condition?
▪ 9
• 10 A. Escherichia colt [1%]
• 11
Pseudomonas species [2%]
• 12
• 13 C. Salmonella species [92%]
• 14 D. Streptococcus pneumonilae [4%]
• 15
E Group B streptococcus [1%]
• 16
• 17
• 18 Explanation:
• 19
• 20 This patient presents with clinical findings suggestive of osteomyelitis in the setting of sickle cell disease.
• 21 Because of the early decline in their splenic function, patients with sickle cell disease are more susceptible to
• 22 infections of the bone and joints_ Salmonella and Stapir aureus are the most common cause of osteomyelitis
• 23 in patients with sickle cell disease. Although controversial, more recent studies have shown that S. aureus is
• 24 more common than Salmonella in children with sickle cell.
• 25
• 26 (Choices A and E) E. colt and group B streptococcus are common causes of septic arthritis and
• 27 osteomyelitis in neonates. Group B streptococcus is the most common organism responsible for
• 28 osteomyelitis in children less than one year of age.
• 29
• 30 (Choice B) Pseudomonal osteomyelitis is seen in diabetic patients and after puncture wounds of the foot and
• 31 is a less common cause of osteomyelitis in children with sickle cell_
• 32
(Choice 0) Sickle cell patients are more susceptible to encapsulated organisms due to abnormal function of
• 33
the spleen, and Streptococcus pneurnorriae is the most common cause of bacteremia in these children. S.
• 34
pneurnorriae typically causes meningitis and pneumonia and is a less likely cause of osteomyelitis.
• 35
• 36
Educational objective:
37
Salmonella and Staph aureus are the most common cause of osteomyelitis in patients with sickle cell disease.
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Copyright @ UWorld Last updated: [7/23/2014]
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▪ 6 A 4-year-old girl is brought to the physician for a rash. She lives in Poland and arrived in the United States last
▪ 7 week to visit her family. The child was in good health until 3 days ago, when she developed nasal congestion
▪ 8 and a low-grade fever_ Today she developed a "pink rash" on her face that spread rapidly to her trunk and
▪ 9 extremities. Review of systems is otherwise negative. She completed an antibiotic course for a 'throat
• 10 infection" 2 months ago but has otherwise been healthy. She has received no vaccinations and takes no
• 11 medications. Her temperature is 38.3 C (101 F), blood pressure is 100/50 mm Hg, pulse is 88/min, and
• 12 respirations are 18/min. Physical examination shows a cooperative girl with bilateral nonexudative
• 13 conjunctivitis and patchy erythema on the soft palate. Small, tender lymph nodes are palpable in the
• 14 suboccipital, posterior auricular, and posterior cervical areas_ The nontender rash is shown below_
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• -1 • 2 Item: 39 of 44 .111 II Mark U. • 4- ▪ 5 ▪ 6 ▪ 7 ▪ 8 ▪ 9 • 10 • -1 -1 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 ▪ 34 • 35 • 36 • 37 • 30 • 40 41 42 End Block Feedback .P ▪ 3 Previous Next Lab Values. Notes Calculator.

cervical • 40 nrpnAntntinn l errurthsui nnrwtorth%i Fanrer+11-tebi r-nn r cnntc se • 41 42 . Which of the following is the most likely cause of this patient's • 14 condition? • 15 • 16 A.6] • 26 • 27 • 28 Explanation: • 29 • 30 • 31 Rubella (German measles) • 32 • 33 • Sensorineural hearing loss • 34 Congenital • Cardiac anomalies (eg. Scarlet fever [9%] • 25 1 H. coryza. Kawasaki disease [7%] • 20 D. Erythema.• -1 • 2 Item: 39 of 44 Mark ▪ 3 Previous Next Lab Values. multiforme [1%] • 17 B. Varicella [19. Notes Calculator. Measles [35%] • 21 E Mumps [1%] • 22 • 23 Rubella [45%] • 24 G. • 4- ▪ 5 ▪ 6 ▪ 7 ▪ 8 ▪ 9 • 10 • -1 -1 • 12 • 13 The remainder of the examination is normal. patent ductus artenosus) • 35 • Cataracts. glaucoma • 36 • 37 • 38 • Low-grade fever Clinical • Conjunctivitis. Exanthematous drug eruption [1%] • 18 • 19 C.

Some patients also have a nonexudative • 36 conjunctivitis and patchy erythema (Forschheimer spots) on the soft palate. and lasts <3 days_ A low-grade fever and • 35 tender lymphadenopathy may precede or occur with the rash. Vaccination is important to prevent se • 40 • • 41 • 42 End Block Feedback .• 1 • 2 Item: 39 of 44 Mark ▪ 3 Previous Next Lab Values. an RNA togavirus infection. spreads quickly in a • 34 cephalocaudal and centrifugal pattern to the rest of the body. congenital disease is devastating. • 4 ▪ 5 Rubella (German measles) ▪ 6 ▪ 7 • Sensorineural hearing loss ▪ 8 Congenital • Cardiac anomalies (eg. Forschheimer spots • 15 • 16 • Cephalocaudal spread of blanching. "pink" maculopapular exanthem begins on the face. coryza. glaucoma • -1 -1 • 12 • Low-grade fever • 13 Clinical • Conjunctivitis. Supportive care • 29 USMLEWarkl. Notes Calculator. • 30 In contrast to postnatal infection. Treatment is aimed at • 37 symptomatic relief (eg. Children • 32 remain asymptomatic or develop mild disease 2-3 weeks after inhalation of infected respiratory • 33 droplets_ An erythematous. • 17 erythematous maculopapular rash • 18 • 19 Adolescents} • 20 • Same as children + arthralgiasiarthritis Adults • 21 • 22 • 23 • Polymerase chain reaction Diagnosis • 24 • Acute & convalescent serology for anti-rubella IgM & IgG • 25 • 26 Prevention • Live attenuated rubella vaccine • 27 • 28 Treatment . patent ductus arteriosus) ▪ 9 • 10 • Cataracts. LLC • 30 • 31 This unvaccinated child most likely has rubella (German measles). cervical • 14 presentation Children lymphadenopathy. acetaminophen) as no specific antiviral therapy is available.

congenital disease is devastating. irs. CCIU3 LAUIL. and painful vesicular eruption. I I 1C14. rash pattern. This patient has no pustules or vesicles. Vaccination is important to prevent • 10 spread of infection to nonimmune pregnant women. Feedback End Block I . and a • 38 maculopapular rash that spreads cephalocaudally. A low-grade fever and tender lymphadenopathy may precede or occur with the rash. coryza. the rash spreads more gradually and appears darker (eg. and a diffuse. • 34 pruritic.4111°. and Forschheimer spots are more suggestive of rubella • 23 • 24 (Choice ID) Measles tends to be more severe than rubella.11. Some patients also have a nonexudative 6 conjunctivitis and patchy erythema (Forschheimer spots) on the soft palate. • 35 • 36 Educational objective: • 37 Rubella infection in children is mild and characterized by low-grade fever. reddish-brown) in comparison • 26 to rubella. Treatment is aimed at ▪ 7 symptomatic relief (eg. Conjunctivitis is • 32 not a feature of scarlet fever_ • 33 (Choice H) Primary varicella zoster infection (chickenpox) is characterized by fever. and • 25 malaise_ In addition. acetaminophen) as no specific antiviral therapy is available. • 15 • 16 (Choice B) Exanthematous (morbilliform) drug eruptions are erythematous and maculopapular and • 17 sometimes cause a low-grade fever. but the rash has a • 31 "sandpaper" texture due to numerous tiny papules and is most pronounced in the skin folds. tender lymphadenopathy.C. Pregnant women who are infected during the 1st • 11 trimester are at extremely high risk for miscarriage or severe birth defects. 1 • 2 Item: 39 of 44 V-• Mark . the allergic rash presents within 1-2 weeks of treatment and • 18 rarely has mucosal involvement • 19 • 20 (Choice C) Although patients with Kawasaki disease may have high fever and mucocutaneous • 21 abnormalities. 1. making this diagnosis unlikely. with high fevers up to 40 C (104 F). 3 Previous Next Lab Values. the fever lasts days and the patient is irritable_ This patient's vaccine status. and lasts <3 days. ▪ 8 ▪ 9 In contrast to postnatal infection. • 12 • 13 (Choice A) Erythema multiforme is characterized by the sudden onset of erythematous 'target' lesions and • 14 usually follows herpes simplex infection. malaise. However. Vaccination is important to prevent infection of vulnerable pregnant women due to the risk of debilitating congenital rubella syndrome.1 I CI yLl ICH ICILUL13. • 29 • 30 (Choice G) Scarlet fever can cause fever and a rash that spreads cephalocaudally.1J1JICLM_ /-1. • 22 behavior.UILIIJCIIJUICII CACI! ILI ICI I I L1 III 4.11 I LI IC I CIL. fever duration.rtly II I CI • 4- 5 cephalocaudal and centrifugal pattern to the rest of the body. but the infection causes parotitis and not a rash. Notes Calculator. • 27 • 28 (Choice E) Mumps can cause a low-grade fever.

• 1 • 2 Item: 39 of 44 'Mark ▪ 3 Previous Next Lab Values. and Forschheimer spots are more suggestive of rubella. with high fevers up to 40 C (104 F).0. and painful vesicular eruption. In addition. and a • 29 maculopapular rash that spreads cephalocaudally. • 25 pruritic. but the infection causes parotitis and not a rash. • 12 behavior. and • 15 malaise. malaise. 11 LI IC1110 111. fever duration. tender lymphadenopathy. coryza. the fever lasts days and the patient is irritable_ This patient's vaccine status. However. Rubella and congenital rubella (German measles). Rubella immunity and vaccination coverage of the population of northern Greece in 2006. • 13 • 14 (Choice ID) Measles tends to be more severe than rubella.LL. • 31 • 32 References: • 33 1. the allergic rash presents within 1-2 weeks of treatment and ▪ 8 rarely has mucosal involvement ▪ 9 • 10 (Choice C) Although patients with Kawasaki disease may have high fever and mucocutaneous • 11 abnormalities.. rash pattern. • 34 2.I ILGU Ur LI IL. Conjunctivitis is • 22 not a feature of scarlet fever_ • 23 • 24 (Choice H) Primary varicella zoster infection (chickenpox) is characterized by fever.Ail LE-11 W.1“1•%•V6P1 ILII L4L.1 I IGLU. • 19 • 20 (Choice G) Scarlet fever can cause fever and a rash that spreads cephalocaudally.L • 4.411. Notes Calculator.. 10. making this diagnosis unlikely_ • 26 • 27 Educational objective: • 28 Rubella infection in children is mild and characterized by low-grade fever. A ▪ 5 usually follows herpes simplex infection. il UUUGI I LOI GI LI 14-. reddish-brown) in comparison • 16 to rubella_ • 17 • 18 (Choice El Mumps can cause a low-grade fever.11. but the rash has a • 21 "sandpaper" texture due to numerous tiny papules and is most pronounced in the skin folds. Copyright © LIWorld Last updated: [9/21/2014] Feedback End Block . Vaccination is important to prevent infection of vulnerable • 30 pregnant women due to the risk of debilitating congenital rubella syndrome. This patient has no pustules or vesicles. ▪ 6 (Choice B) Exanthematous (morbilliform) drug eruptions are erythematous and maculopapular and ▪ 7 sometimes cause a low-grade fever. and a diffuse. the rash spreads more gradually and appears darker (eg. Rubella 3.-.

-1 2 Item: 39 of 44 V-Mark 3 Previous Next Lab Values Notes Calculator 4 Media Exhibit X 5 6 Erythema multforrne 7 8 9 • 10 • 11 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 35 36 37 30 I 39 Feedback C End Block. .

11 . Media Exhibit ▪ 5 ▪ 6 Measles-3 ▪ 7 ▪ 8 ▪ 9 • 10 • -1 -1 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 33 3 • 40 • 41 • 42 End Block Feedback .• -1 • 2 Item: 39 of 44 V-Mark EF. ▪ 3 Previous Next Lab Values Notes Calculator • 4.

Mark ▪ 3 Previous Next Lab Values Notes Calculator • 4. Media Exhibit ▪ 5 ▪ 6 Sprlet fever ▪ 7 ▪ 8 ▪ 9 • 10 • -1 -1 • 12 • 13 • 14 • 15 • 16 • 17 • 18 • 19 • 20 • 21 • 22 • 23 • 24 • 25 • 26 • 27 • 28 • 29 • 30 • 31 • 32 • 33 • 34 • 35 • 36 • 37 • 30 • 40 • • 41 42 Feedback a End Block .• -1 • 2 Item: 39 of 44 V.

Order a complete skeletal survey [2%] • 19 • 20 E Order an x-ray of the right arm [22%] • 21 • Refer the child to an orthopedic surgeon [11C] • 22 • 23 • 24 Explanation: • 25 • 26 Radial head subluxation (nursemaid's elbow) • 27 • 28 Axial traction on forearm with elbow extended • 29 Mechanism • 30 (child pulled. Gently hyperpronate the right forearm [66%] • 18 D. No bruises or other abnormalities are seen. lifted. Which of the following is the • 12 most appropriate next step in management of this patient? • 13 • 14 A. LLC 41 42 End Block Feedback A'S . Contact the child protection agency [1°4] • 15 • 16 E Closed reduction and casting of the right arm [7%] • 17 ▪ C. He was swinging her by the forearms when ▪ 8 she suddenly started crying and refused to use her right arm_ He has swung her by the arms previously and it ▪ 9 usually makes her laugh. The girl lives with her father and her mother.• 1 • 2 Item: 40 of 44 V-• Mark ▪ 3 Previous Next Lab Values. deformity. who is currently out of town_ Physical • 10 examination shows a well-nourished child who holds her right arm in pronation against the chest She avoids • 11 any movement of her right arm. or focal tenderness • 34 • 35 • 36 • Forearm hyperpronation • 37 Treatment OR • 38 • Forearm supination & elbow flexion • 39 CP USMLEVI1011d. Notes Calculator. or swung by arm) • 31 • 32 Physical • Arm held extended & pronated • 33 examination • No swelling. • 4- 5 ▪ 6 A 1-year-old girl is brought to the emergency department because she is not moving her right arm. Her father ▪ 7 says that the girl acted normally when she awoke this morning.

and the patient should experience • 22 immediate return of normal function after manual reduction. ▪ 36 2. If the historian is inconsistent with the details or the examination is incongruent with the • 19 reported mechanism. • 16 • 17 (Choices A and D) The likelihood of child abuse is very low as the mechanism of injury is consistent with the • 18 patient's examination. Forearm supination with elbow flexion is also commonly performed • 14 but may be less successful and more painful. Randomized comparison of pain perception during radial head subluxation reduction • 35 using supination-flexion or forced pronation. Reduction is typically performed by primary care • 23 or emergency physicians_ • 24 • 25 (Choice E) The diagnosis is made clinically as radiographs are often normal. • 37 • 38 • 39 Copyright © UWorld Last updated: [7/30/2014] • 40 • 41 • 42 End Block Feedback . Manipulative interventions for reducing pulled elbow in young children. a complete skeletal survey should be performed to look for healing fractures in other • 20 parts of the body_ • 21 (Choices B and F) Immobilization with slings or casts is not needed. • 26 • 27 Educational objective: • 28 Subluxation of the radial head is common in preschool children. No post-reduction films are needed when the patient resumes • 15 full use of the extremity. • 4 reatrnent OR ▪ 5 * Forearm supination & elbow flexion ▪ 6 lArvILEWOr Id. Notes Calculator. • 31 • 32 References: • 33 • 34 1. Next Lab Values. A pop may also be heard on successful reduction.• 1 • 2 ▪ 3 Previous . Full recovery after closed reduction by forearm hyperpronation confirms the • 30 diagnosis. The child is usually not in distress at presentation but may cry at any attempt to move the elbow or • 12 forearm_ The preferred treatment of this condition is closed reduction by applying pressure on the radial head • 13 and hyperpronating the forearm. The classic mechanism is swinging or • 29 pulling a child by the arm. LLC ▪ 7 ▪ 8 ▪ 9 Radial head subluxation (nursemaid's elbow) is a common injury in preschool children. The injury • 10 characteristically occurs from innocent swinging of a young child by the arms or pulling a child's arm while • 11 in a hurry.

5% albumin [29C] • 32 E Packed red blood cells [1%] • 33 • 34 • 35 Explanation: • 36 • 37 The signs and symptoms of hypernatremia are mainly neurologic and include lethargy. • 38 irritability. but has had two to ▪ 8 three episodes of diarrhea with each bottle. his temperature is • 10 102.8 mg/dL • 21 Calcium 10.• 1 • 2 Item: 41 of 44 F' Mark ▪ 3 Previous Next Lab Values. muscle weakness.1' C). His abdominal exam is unremarkable. He has lost three pounds (1A kg) since his routine check-up two ▪ 9 weeks ago. Laboratory results are shown below.9% saline [6296] • 28 B. His mucous membranes are dry. 0. 5% dextrose [89C] • 30 • 31 ID. Capillary • 13 refill is four seconds. pulse is 200/min. • 12 Cardiopulmonary exam reveals tachycardia and tachypnea.45% saline [27%] • 29 C. • 40 42 End Block Feedback . Notes Calculator. • 4- 5 ▪ 6 A 9-month-old infant is brought to the emergency department with lethargy and tachypnea_ He was healthy ▪ 7 before developing fever and diarrhea four days ago. altered mental status. and blood pressure is 70/45 mm Hg_ He is • 11 lethargic with decreased tone and decreased deep tendon reflexes. and decreased • 39 deep tendon reflexes. On examination. respiratory rate is 42/min.5 mEq/L • 17 Chloride 108 mEcilL • 18 Bicarbonate 14 mEq/L • 19 Blood urea nitrogen (BUN) 2G mgidL • 20 Serum creatinine 0.0 mg/dL • 22 Blood glucose 98 mg/dL • 23 • 24 Which of the following fluids should be used as a bolus in the resuscitation of this infant? • 25 • 26 • 27 A. and seizures_ Hypernatremia can also cause muscle cramps. He has had one wet diaper in the past twenty four hours. 0. • 14 Chemistry panel • 15 Serum sodium 165 mailL • 16 Serum potassium 4.5° F (39. He has been taking some formula.

5% albumin [2%] ▪ 7 ▪ 8 E. and decreased • 14 deep tendon reflexes. muscle weakness. • 34 • 35 Educational objective: • 36 Isotonic solutions such as normal saline are the fluid of choice for initial resuscitation in severe hypovolemic • 37 hypernatremia. hyperaldosteronism). • 19 • 20 When treating a patient with hypernatremia.• 1 • 2 Item: 41 of 44 V/lark ▪ 3 Previous Next Lab Values. • 24 • 25 (Choices B and C) Half normal saline and 5% dextrose are hypotonic solutions. diuretic use. glycosuria) or extrarenal • 17 losses (eg. U. Hypervolemic hypernatremia occurs due to • 18 exogenous sodium intake or mineralocorticoid excess (eg. Precipitous drops in sodium levels can cause cerebral edema. • 28 • 29 (Choice 0) Multiple studies have demonstrated that the expensive colloid solutions are no better than • 30 crystalloids at fluid resuscitation. In this infant with • 21 hypernatremia and dehydration. isotonic solutions • 33 remain the mainstay of initial fluid resuscitation (even in individuals with massive bleeding). they should never • 26 be used for initial resuscitation because they quickly exit the intravascular system and lower the sodium too • 27 rapidly. • 31 (Choice E) Packed red blood cells may be appropriate in initial fluid resuscitation when bleeding is a major • 32 issue_ Because of the limited availability of packed red blood cells on short notice. only isotonic solutions such as normal saline or lactated Ringer's should be • 23 used. Notes Calculator. the initial goal is to stabilize him with fluid resuscitation as needed. • 13 irritability. altered mental status. however. excessive sweating). When • 22 giving intravenous fluid boluses. and seizures. • 4. gastrointestinal upset. 5% dextrose [8%] ▪ 6 ID. Hypernatremia can also cause muscle cramps. b. As such. the sodium must be slowly returned to normal. • 15 • 16 Hypovolemic hypernatremia develops secondary to renal losses (eg.41buk saline viIpj 5 C. Packed red blood cells [1%] ▪ 9 • 10 Explanation: • 11 • 12 The signs and symptoms of hypernatremia are mainly neurologic and include lethargy. • 38 • 39 Copyright © LIWorld Last updated: [12/29/2014] • 40 42 Feedback End Block .

jaundice. The initial screening is with nontreponemal tests such as • 32 the venereal disease research laboratory (VDRL) test. hepatosplenomegaly. • 28 saddle nose. The infant was born via normal spontaneous vaginal 8 delivery and required no resuscitation. Most • 24 infants born with congenital syphilis are initially asymptomatic.rF rlafari-c. v • 41 Feedback End Block . The infant has hepatosplenomegaly on examination. high arched palate.ani-c. • 38 chorioretinitis. confirmatory testing is with treponemal tests such as the fluorescent treponemal • 34 antibody absorption (FTA-ABS) test or the treponema pallidum particle agglutination assay (TPPAY • 35 Parenteral penicillin G is the treatment of choice for syphilis_ • 36 • 37 (Choice A) Major clinical findings of congenital toxoplasmosis include hepatosplenomegaly.inrith Ina-mrinn ke. Human immunodeficiency virus infection [8%] • 20 • 21 • 22 Explanation: • 23 Congenital syphilis is contracted through the transplacental transmission of Treporierna parlidurn. but she had only two prenatal visits. While in the 9 hospital. The mother reports that the pregnancy 7 was uncomplicated. and rhinorrhea. Late congenital manifestations (presenting • 27 after two years of age) include frontal bossing. or enzyme • 33 immunoassay (EIA) test. anemia. Those with early symptoms present with • 25 cutaneous lesions on the palms and soles. hydrocephalus. rapid plasma reagin (RPR) test. Hutchinson teeth. and intracranial calcifications_ • 39 • 40 frq% rtrac. Notes Calculator. On subsequent examinations. These late manifestations can be prevented with early treatment of the • 29 infant • 30 • 31 Diagnosis of syphilis is made with serologic testing. • 4- 5 6 A newborn infant is brought to the nursery for evaluation after delivery. Syphilis [55%] • 16 C. 1 • 2 Item: 42 of 44 A V-Mark 3 Previous Next Lab Values. Toxoplasmosis [4%] • 15 B. the infant requires treatment for anemia and hyperbilirubinemia. and perioral fissures. the • 10 infant has clear rhinorrhea and ulcerative lesions on his feet Which of the following congenital infections is • 11 most likely in this patient? • 12 • 13 • 14 A. interstitial keratitis. Cytomegalovirus infection [25%] • 19 E. Rubella [8%] • 17 • 18 D. • 26 Radiographs demonstrate metaphyseal dystrophy and periostitis.

petechiae or purpura. • 28 • 29 (Choice 0) Congenital cytomegalovirus is characterized by intrauterine growth retardation. interstitial keratitis. • 23 chorioretinitis. and • 31 periventricular calcifications.11M Mark 3 Previous Next Lab Values. chorioretinitis. 1 • 2 Item: 42 of 44 . • 26 hepatosplenomegaly. • 24 • 25 (Choice C) Congenital rubella presents with sensorineural hearing loss. t_ human immunodeficiency virus infection pluki A 5 6 Explanation: 7 8 Congenital syphilis is contracted through the transplacental transmission of Treponerna parlidurn. Notes Calculator. Late congenital manifestations (presenting • 12 after two years of age) include frontal bossing. • 20 Parenteral penicillin G is the treatment of choice for syphilis_ • 21 • 22 (Choice A) Major clinical findings of congenital toxoplasmosis include hepatosplenomegaly. or enzyme • 18 immunoassay (EIA) test. microcephaly. and 37 rhinorrhea_ Metaphyseal dystrophy and periostitis may be seen on radiography. high arched palate. heart defects. jaundice. • 13 saddle nose. hepatosplenomegaly. Hutchinson teeth. hydrocephalus. cataracts. • 11 Radiographs demonstrate metaphyseal dystrophy and periostitis. These late manifestations can be prevented with early treatment of the • 14 infant • 15 F • 16 Diagnosis of syphilis is made with serologic testing. Most 9 infants born with congenital syphilis are initially asymptomatic. and intracranial calcifications. and rhinorrhea. and perioral fissures. and thrombocytopenic purpura (commonly described as a "blueberry • 27 muffin' rash). confirmatory testing is with treponemal tests such as the fluorescent treponemal • 19 antibody absorption (FTA-ABS) test or the treponema pallidum particle agglutination assay (TPPA). • 30 hepatosplenomegaly. Those with early symptoms present with • 10 cutaneous lesions on the palms and soles. • 34 35 Educational objective: 36 Congenital syphilis presents early on with hepatosplenomegaly. sensorineural hearing loss. anemia. cutaneous lesions. jaundice. 38 39 Copyright © LIWorld Last updated: [12/29/2014] • 40 • 41 a 42 End Block Feedback . microcephaly. rapid plasma reagin (RPR) test. • 32 (Choice E) Infants with congenital human immunodeficiency virus (HIV) infection are usually clinically • 33 asymptomatic at birth. anemia. • 4. The initial screening is with nontreponemal tests such as • 17 the venereal disease research laboratory (VDRL) test.

posterior columns.• 1 • 2 Item: 43 of 44 F' Mark ▪ 3 Previous Next Lab Values. His blood pressure is 120/10 ▪ 9 mmHg and pulse is 80/min. • 28 scoliosis and 'hammer toes'). • 33 • 34 (Choice C) Diabetes develops in 10-20% of the patients with Friedreich ataxia. • 35 ▪ 36 (Choice 0) Friedreich ataxia does not predispose to cancer. and absence of deep plantar • 11 reflexes on lower extremities.6] • 16 C. Notes Calculator. and its symptoms usually begin before 22 years of age. which is the most common type of spinocerebellar • 24 ataxias. dysmetria. diabetes. and these include concentric hypertrophic cardiomyopathy. Renal failure [13?. It is an autosomal recessive disorder. • 37 ▪ 38 (Choice E) Septic shock may develop due to respiratory complications. • 4- 5 • ▪ 6 A 15-year-old Caucasian male is brought to the office by his mother for the evaluation of a six-month history of ▪ 7 unstable gait and speech difficulty which are getting worse over time_ His past medical history is insignificant ▪ 8 He is not taking any medications. but it is not as common as cardiac • 39 complications_ • 40 se • 41 • 42 End Block Feedback . but it is a rare cause of death. dysarthria) result from degeneration of the spinal • 26 tracts (spinocerebellar tracts. Diabetes-related complications [3%] • 17 • 18 ID Malignancy [10%] • 19 E.. and skeletal deformities (e.' The neurologic examination showed dysarthria. Non-neurological manifestations may also • 27 occur. nystagmus. gait ataxia. Cardiomyopathy [699/0] • 15 B. frequent falling. pyramidal tract). Septic shock [4%] • 20 • 21 • 22 Explanation: • 23 This clinical scenario is typical for Friedreich ataxia. • 25 Neurological manifestations (e_g_. Cardiomyopathy develops in up • 30 to 90% of the patients_ • 31 • 32 (Choice B) Renal failure is not characteristic of Friedreich ataxia.. The median survival after disease onset rarely exceeds 20 years_ The most • 29 common causes of death are cardiomyopathy and respiratory complications. Musculoskeletal examination showed scoliosis and feet deformity with 'hammer • 10 toes.g. and denies smoking or alcohol consumption. What is the most common cause of death in this patient population? • 12 • 13 • 14 A.

▪ 36 • 37 *very high-yield topic for LISMLE1 ▪ 38 • 39 Copyright © UWorld Last updated: [8/22/2014] • 40 • 41 • 42 End Block Feedback . Cardiomyopathy develops in up • 21 to 90% of the patients_ • 22 • 23 (Choice 13) Renal failure is not characteristic of Friedreich ataxia. It is an autosomal recessive disorder. but it is a rare cause of death.6] ▪ 9 • 10 E. and its symptoms usually begin before 22 years of age_ • 16 Neurological manifestations (e_g_. The most • 20 common causes of death are cardiomyopathy and respiratory complications. but it is not as common as cardiac • 30 complications_ • 31 Educational Objective: • 32 Friedreich ataxia is the most common type of spinocerebellar ataxias.. • 4 ▪ A.0] ▪ 7 C. dysarthria). Diabetes-related complications [3%] ▪ 8 D. and skeletal deformities (e_g_. diabetes. Renal failure [13?. Cardiomyopathy [69°/0] 5 ▪ 6 B. • 26 • 27 (Choice 0) Friedreich ataxia does not predispose to cancer. Remember the combination of • 33 neurologic (ataxia. and these include concentric hypertrophic cardiomyopathy. Malignancy [10?.. • 24 • 25 (Choice C) Diabetes develops in 10-20% of the patients with Friedreich ataxia. Notes Calculator.• 1 • 2 Item: 43 of 44 Al II Mark ▪ 3 Previous Next Lab Values. • 28 • 29 (Choice E) Septic shock may develop due to respiratory complications. Non-neurological manifestations may also • 18 occur. frequent falling. skeletal (scoliosis. Septic shock [4%] • 11 • 12 Explanation: • 13 • 14 This clinical scenario is typical for Friedreich ataxia. posterior columns. dysarthria) result from degeneration of the spinal • 17 tracts (spinocerebellar tracts. The median survival after disease onset rarely exceeds 2G years. pyramidal tract). • 19 scoliosis and 'hammer toes'). gait ataxia. feet deformities) and cardiac (concentric hypertrophic • 34 cardiomyopathy) manifestations of the disease_ The most common causes of death are cardiomyopathy and • 35 respiratory complications. which is the most common type of spinocerebellar • 15 ataxias.

well-appearing child with scattered petechiae and • 11 ecchymoses over the trunk and extremities. Vital signs are • 10 normal. He has had no bleeding or recent trauma Three weeks ago. Intravenous antibiotics [19q • 23 • 24 C. Intravenous immunoglobulin [38%] • 25 D. In severe cases.1pL • 16 • 17 • 18 Peripheral smear shows a few large platelets.0001pL). What is the most appropriate next step in management of this • 19 patient? • 20 • 21 A. He takes no medications. Observation [28%] • 26 E Platelet transfusion [25%] • 27 • 28 • Splenectomy [2%] • 29 • 30 Explanation: • 31 • 32 • 33 • 34 • 35 Immune thrombocytopenia (ITP) is characterized by an abnormally low platelet count (<1113. resulting • 36 in increased propensity for bruising and bleeding. he had an upper ▪ 8 respiratory tract infection that resolved uneventfully. Bone marrow biopsy [716] • 22 B. • 4- ▪ 5 ▪ 6 A previously healthy 6-year-old boy is brought to the physician after the sudden appearance of bruises ▪ 7 throughout his body. The rest of the examination is unremarkable. Physical examination shows a cooperative. and his ▪ 9 immunizations are up to date_ Family history is negative for bleeding or clotting disorders. Notes Calculator.5gidL • 14 Platelets 10. patients may experience mucosal • 40 • 41 • 42 End Block Feedback . has no allergies.• 1 • 2 Item: 44 of 44 F Mark ▪ 3 Previous Next Lab Values. CEDDip L • 15 Leukocytes 1. Laboratory results • 12 are as follows: • 13 Hemoglobin 13. The disorder can affect people of any age but is most • 37 common at age 2-5 years_ The pathogenesis involves antibodies that bind to platelets and subsequent • 38 destruction of these antibody-platelet complexes in the spleen_ The condition is usually preceded by a viral • 39 infection and presents with purpura and petechiae.000.

anemia. fatigue). Adults with ITP and platelet count <30. the course is usually ▪ 9 self-limited with spontaneous recovery within 6 months. regardless of platelet count Children with bleeding should receive intravenous immunoglobulin • 31 or glucocorticoids. • 4 destruction of these antibody-platelet complexes in the spleen_ The condition is usually preceded by a viral ▪ 5 infection and presents with purpura and petechiae. leukopenia. The American Society of Hematology 2011 evidence-based practice guideline for immune • 36 thrombocytopenia. ▪ 7 ▪ 8 The approach to treatment is different in children compared to adults.000!pL should receive glucocorticoids or intravenous • 13 immunoglobulin as they are less likely to experience spontaneous recovery. In severe cases. Observation without treatment is recommended • 10 for children who experience only cutaneous symptoms. Notes Calculator. unexplained weight loss. • 20 • 21 (Choice E) Platelet transfusion is very rarely indicated as it generally results in further platelet • 22 destruction. regardless of platelet count Intravenous • 11 immunoglobulin (IVIg) (Choice C) or glucocorticoids are the first-line drugs in patients who experience • 12 bleeding. Laboratory studies shows isolated thrombocytopenia and megakaryocytes on peripheral smear.• 1 • 2 Item: 44 of 44 V-Mark ▪ 3 Previous Next Lab Values. • 17 • 18 (Choice B) Antibiotics are not indicated in immune thrombocykopenia as the condition is not related to a • 19 bacterial infection. • 14 • 15 (Choice A) Bone marrow biopsy is not required in well-appearing children with isolated thrombocytopenia • 16 and no other symptoms (eg. • 37 • 38 • 39 Copyright © Morld Last updated: [11/2G/2014] • 40 X • 41 • 42 End Block Feedback . fever of unknown origin. patients may experience mucosal ▪ 6 bleeding. In children. • 32 • 33 • 34 References: • 35 1. It can be considered in severe cases with active or intracranial bleeding_ 111 • 23 • 24 (Choice F) The spleen is the site of platelet destruction and splenectomy can increase the life span and • 25 quantity of platelets_ Splenectomy is a last resort for catastrophic bleeding or chronic ITP that is refractory to • 26 IVIG and glucocorticoids_ • 27 Educational objective: • 28 Immune thrombocytopenia should be suspected in children who develop isolated thrombocytopenia and • 29 petechiae after a viral infection_ Children usually recover spontaneously within 6 months and require only • 30 observation.