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Pediatric Board Review

August 28, 2012 (4:55 p.m.)
Elumalai Appachi MD., MRCP (UK)., DCH.,FAAP

1. 2 weeks old male infant presents with fever and irritability for 3 days. On
examination he lies motionless and has an erythematous rash, which has started to
blister. One of the following is true regarding the diagnosis and management:

• a.May have acquired the condition in utero
• b.May have associated pharyngitis, conjunctivitis, or
• c.Fluid restriction is required to prevent bulla
• d.The skin lesions heal and leave a pigmented whorl
like pattern
• e.Ampicillin is the drug of choice

• Toxin mediated complication of Staph Aureus
• Epidermolytic or exfoliative toxins
• Erythematous rash, bullous impetigo, pharyngitis,
pneumonia, and conjunctivitis
• Separation of epidermis to gentle force: Nikolsky
sign- sheets of epidermis can peel away
• Semi synthetic penicillins are indicated
• Incontinentia pigmenti: Whorl like pigmentation

The risk of having similar child from future pregnancies is 50% • d.30% of infants with this condition have signs of ano-rectal anomalies Fetal Alcohol syndrome • Specific pattern of malformation • Prenatal onset and persistent growth deficiency • Facial abnormalities: thin upper lip.The infant may develop moderate to severe learning disability • c. Your information to the mother includes all of the following EXCEPT: • a.90% of infants with this condition will have congenital heart disease • b.The infant needs an ECHO cardiogram • e. She says her 5-year-old was similar at this age and has delayed development.The infant has a minimal risk for developmental delay • c. • a.There is a minimal risk for future pregnancies to have similar condition • d. During routine postnatal examination you notice this abnormality. small palpebral fissures and maxillary hypoplasia • Cardiac defects: septal defects • Minor joint and limb abnormalities • Delayed development and mental retardation 3.Major/minor components of this condition is expressed in 1-2/1000 live births • e.Thyroid hormone abnormality is common and it needs to be evaluated . The following is true of the infant’s condition. 1-month-old infant is seen in the pediatric outpatient for routine checkup.2.The infant and parents need chromosomal analysis • b. Mother gives a history of poor feeding and unsatisfactory weight gain. 24-year-old primi-gravida delivered at term.

hemiparesis. • Buphthalmos and glaucoma of the ipsilateral eye are common complications.000 live births.Trisomy 21 • 1 in 600-800 live births • Non dysjunction 95% and translocation accounts for 5% • Translocation accounts for 10% of children born with Trisomy 21 to mothers under 30years • Half the translocation occurs de nova in the affected individual and half are inherited from a translocation carrier parent • Chromosome studies should be performed on all parents if translocation is identified 4. intracranial calcifications. laser therapy often provides excellent clearing of the port-wine stain . • Intracranial calcification in the occipitoparietal region assumes a serpentine or railroad-track appearance • The facial nevus is often a target for ridicule by classmates.She may be at risk of developing a seizure disorder • d. and. strokelike episodes. in many cases. leading to psychologic trauma. All of the following are appropriate/true about her EXCEPT: • a.She needs an Ophthalmologic evaluation Sturge Weber Syndrome • Sporadic disorder: facial nevus (port-wine stain).Inform the parents that imaging of her brain is part of initial work-up • c. Inform the parents that the birthmark will disappear as she grows older • b.She may develop moderate to severe learning disability • e. mental retardation 1/50. The parents of this neonate are concerned about the birthmark. seizures.

defective DNA repair and risk for leukemia • Presents as aplastic anemia and treatment is BMT 6. He should receive broad spectrum intravenous antibiotics • c. He has been bottle-fed and was doing well until that day. fever. The following are true regarding his management EXCEPT: • a.5. irritability.He may develop Pancytopenia • c. He is a FTNVD. and a seizure.He needs an Echocardiogram to evaluate for any associated cardiac anomalies • b. 5days old neonate is brought in to the ER with a history of poor feeding. The parent of this neonate is concerned regarding his physical features.Chromosomal studies are required • d. Neuro imaging and EEG should be scheduled • e. The following are true regarding his condition EXCEPT: • a.He may have an increased predisposition to malignancies Fanconi anemia • • • • Autosomal recessive Physical abnormalities in 80% May have associated cardiac anomalies Increased chromosomal breakages. A septic work up is indicated • b. You should await Herpes PCR before initiating therapy with acyclovir • d.Future pregnancies are not at risk • e. You should inform the parents that he may have acquired this illness during the delivery .

undescended testes.Rectal biopsy is required to confirm diagnosis • d. urinary tract abnormalities.The culture of the fluid from the pustule may grow Staph species • d. The following is true regarding his rash. • a.You should reassure the parents that it is a benign condition and doesn’t require any treatment .He needs investigation for associated renal abnormalities • c.Urgent upper GI contrast study is required Prune belly syndrome • 95% of affected patients are males • Deficient abdominal muscles.It is less common in male infants • b.Microscopic examination of the fluid may show many polymorphs • e. and oligohydramnios • Cardiac anomalies in 10% • Prognosis: depends on the pulmonary hypoplasia and renal dysplasia 8.Most of the infants with this condition have associated cardiac anomaly • e.7. 2-days-old neonate’s parents are concerned about this rash.He is at risk to develop seizure disorder later • c.He needs urgent parenteral acyclovir therapy • b. Routine postnatal examination on this neonate revealed the following physical finding. The following is true regarding his condition: • a.

Chromosomal analysis for karyotyping • b.Prognosis is good with aggressive therapy Congenital Rubella • Virtually affects all organ systems • IUGR. pneumonia. The next thing to do is: • a. micropthalmia. Examination reveals an irritable infant who has hypertonia and absent red reflex. cataracts.Features are difficult to differentiate from congenital CMV virus infection • e. hepatitis and thrombocytopenia • Virus is shed in the urine for 1 year or longer • Prognosis with multi organ involvement is poor.Abdominal ultrasound to rule out renal abnormalities • d.She needs an ECHO cardiogram • b. blue berry muffin lesions.Start local testosterone therapy • e. hearing loss.She needs hearing assessment • c. meningoencephalitis. All of the following is true regarding congenital rubella syndrome EXCEPT: • a.Diagnosis can be confirmed by isolating the virus in urine • d.Start hydrocortisone therapy • c.Reassure the parents that future pregnancies are not at risk . Parents of this neonate are concerned regarding the feature shown.9. 7-days-old neonate presents with jaundice and rash. myocarditis. She was a SGA infant at birth and has not gained any weight. 10.

Request an abdominal ultrasound to rule out infantile hypertrophic pyloric stenosis Duodenal atresia • • • • • 1/10000 live births Accounts for 25-40% of intestinal obstruction 50% of infants are premature Common form is membranous Presentation: bilious vomiting and moderate abdominal distension.Consult a pediatric surgeon immediately • e.Administer a fluid bolus to improve intravascular volume • d. The following is true regarding small left colon syndrome: • a. Contrast study not necessary • History of polyhydramnios 12. 2-days-old breast-fed neonate presents with bilious vomiting.Sweat test is indicated to confirm diagnosis • c. mild abdominal distension and lethargy. Mother has gestational diabetes.Physical examination almost always show features of Trisomy-21 • b.Start IV fluids and hold his enteral feeding • b. 3 days old neonate presents with abdominal distension delayed passage of meconium. and vomiting. His management includes all of the following EXCEPT: • a.Prognosis is good for bowel function and recovery • e.Place a nasogastric tube • c.Anal manometry and rectal suction biopsy is required • d.11.Surgery is the treatment of choice in all of the cases .

Mother’s blood group is O+ve. and Platelets 240. pale. Administer erythropoietin therapy immediately • c. Hb is 8g/dl. Arrange for exchange transfusion . Administer RhoGAM to the mother • e. Baby was born by NVD and has no prenatal problems. 4 hours old term neonate is found to be tachypneic. and has poor peripheral perfusion. Administer a blood transfusion at 10-15cc/kg • b. Start 100% oxygen and observe the baby in the nursery • d.13. The next thing to do is: • a. HCT 25.