PEDIATRICS BOARD REVIEW

EDWARD M. SANTOS, MD DEPARTMENT OF PEDIATRICS UERMMMC

1. WHICH OF THE FOLLOWING IS NOT A
COMPONENT OF TETRALOGY OF FALLOT?

a.
b. c. d.

Atrial septal defect Ventricular septal defect Pulmonary stenosis Overriding aorta

TETRALOGY OF FALLOT

TOF
Dyspnea is relieved by squatting  Paroxysmal hypercyanotic attacks (hypoxic, “blue”, or “tet” spells
      

Place infant on the abdomen in knee chest position Oxygen Morphine sq not in excess of 0.2mg/k NaHCO3 if spell is unusually severe Other meds: IV phenylephrine, propranolol

Systolic thrill and murmur  On CXR- boot or wooden shoe (coeur en sabot)  Blalock-Taussig shunt (subclavian and ipsilateral PA) and Gore-Tex interposition shunt  TAPVR - Snowman appearance

THE MOST LIKELY CARDIAC LESION IS: a.. d. b. A 6-MONTH OLD BABY WAS BROUGHT TO THE ER BECAUSE OF DOB.2. SHE WAS TACHYPNEIC. c. ABG SHOWED PO2 OF 175 OFF OXYGEN. WITH RALES AND NOTE OF A GR 3/6 HOLOSYSTOLIC MURMUR ON THE LLSB. Pulmonary stenosis Atrial septal defect Ventricular septal defect Transposition of the great arteries . CXR SHOWED CONGESTION AND CARDIOMEGALY LV FORM. ON PE.

PDA. VSD. etc) Cyanotic  Decreased pulmonary blood flow  Obstruction and a shunt (ex. AV septal defects)  Increased pressure load  Normal blood flow is obstructed (pulmonic stenosis.RECALL     Congenital Heart Diseases Acyanotic vs Cyanotic Acyanotic  Increased volume load  L  R shunting (ASD. TOF)  Increased pulmonary blood flow  Not associated with obstruction but with abnormal mixing of systemic venous and pulmonary venous blood ( ex TGA) .

RECALL No cyanosis  (+) congestion  Increased volume load  decreased pulmonary compliance  increased work of breathing  Acyanotic CHD with increased volume load  VSD  .

Which is the dominant ventricle? 5. Is the pulmonary arterial flow increased or not ? 3. Is the patient cyanotic or acyanotic? 2. Does the malformation originate in the left or right side of the heart? 4.Ray EKG (15 lead) Others: 2D Echo Cardiac cath .Tools in the Assessment of Heart Disease FIVE BASIC QUESTIONS 1. Is pulmonary hypertension present or not ? HISTORY PHYSICAL EXAM CHEST X.

PHYSIOLOGIC CLASSIFICATION OF CONGENITAL HEART DISEASE ACYANOTIC Increased PBF CYANOTIC Increased PBF Decreased PBF Normal or Decreased PBF With SHUNT RVH ASD LVH VSD/PDA AP.Window No SHUNT RVH PS LVH AS RVH BVH RVH LVH TGA TRUNCUS TAPVR TOF/ PVA DORV EBSTEIN .

0 cm2 RV and LV pressure is equalized  . shunt is LR  Non restrictive VSDs >1.5cm2. RV pressure is N.VENTRICULAR SEPTAL DEFECT Most common form of CHD (25% of CHDs)  Most defects are of the membranous type  Size of the VSD is a major determinant of the LR shunt  Restrictive VSDs= < 0.

ATRIAL SEPTAL DEFECT Most common form is the ostium secundum defect in the region of the fossa ovalis  Other forms: primum and sinus venosus defects  Infants and children with ASDs are usually asymptomatic  Widely split and fixed S2  Gr 2-3/6 SEM  .

bounding peripheral pulses with wide PP  Indomethacin  .PATENT DUCTUS ARTERIOSUS Common problem in premature infants  Left PA and the descending aorta  If smallasymptomatic  If large tachycardia and exertional dyspnea  Dynamic precordium. systolic thrill at the ULSB.

CLOSURE OF THE DUCTUS ARTERIOSUS  Functional closure occurs by constriction of the medial smooth muscles in the ductus : occurs 10 15 hours after birth Anatomic closure completed in 2-3 weeks of life by permanent changes in the endothelium and subintimal layers of the ductus  .

FACTORS THAT MAY FAVOR CLOSURE OF THE DUCTUS ARTERIOSUS  Oxygen .strongest stimulus for constriction of smooth muscles Decrease Prostaglandin E2 levels Responsiveness of the ductal smooth muscle to oxygen for closure depends on :     gestational age of the newborn does not depend on the lack of smooth muscle development .

d. EXCEPT: a. Dopamine Furosemide Phenytoin Digoxin . b.3. THE FOLLOWING ARE ANTI- HEART FAILURE MEDICATIONS. c.

enalapril. nitroprusside)  .CONGESTIVE HEART FAILURE Results from CHDs or acquired heart diseases  Volume and pressure overload or from myocardial insufficiency  Treatment  Elimination of precipitating causes  Control of heart failure (meds)   Drug Therapy Diuretics (rapid acting like furo)  Digitalis glycosides (Digoxin)  Other Inotropic agents (Dopamine)  Afterload reducing agents (hydralazine. nitroglycerine. captopril.

Juvenile Rheumatoid Arthritis Takayasu Arteritis Panarteritis nodosa Systemic lupus erythematosus . ON PE.4. YOU NOTE THAT HE IS ALMOST PULSELESS. c. A 10 YEAR OLD WENT TO YOU FOR CONSULT DUE TO DIFFICULTY IN WALKING. b. YOU SUSPECT: a. d.

pulseless disease  PAN. PAN  Takayasu – involves large arteries. Takayasu arteritis.small to medium sized arteries.CLUE: PULSELESSNESS  Differentials   Coarctation of the aorta  Femoral pulses are weak and delayed or absent Vasculitis syndromes  Vasculitis syndromes HSP. presents with fever  Difficulty in walking – could be due to arthritis  .

5. WHICH OF THE FOLLOWING IS THE
EXCEPTION TO FULFILLING JONES CRITERIA IN ORDER TO DIAGNOSE RHEUMATIC FEVER
a. b. c. d.

Subcutaneous nodules Migratory polyarthritis Endocarditis Chorea

JONES CRITERIA

5 major
Carditis, polyarthritis, erythema marginatum, subcutaneous nodules, chorea  Chorea may occur as the only manifestation of acute RF

4 minor

Arthralgia, fever, elevated ESR, elevated CRP, prolonged PR interval

Evidence of recent strep group A infection
(+) throat culture  Elevated ASO

JONES: 2 major or 1 major + 2 minor, with an evidence of recent strep infection

6. WHICH OF THE FOLLOWING IS NOT AN INDICATION FOR TONSILLECTOMY?
a.

b.

c. d.

Obstructive sleep apnea due to hypertrophic adenoids and tonsils Severe rheumatic fever/rheumatic heart disease manifestation To rule out tumor 5 infections in each of the last 2 years for chronic tonsillitis patients

INDICATIONS FOR TONSILLECTOMY (CHILDREN’S HOSPITAL OF PITTSBURG)
7 or more throat infections treated with antibiotics in the preceding year  5 or more throat infections treated in each of the preceding 2 years  3 or more throat infections treated in each of the preceding 3 years

YOUR DX IS a. c. DIFFICULTY OF SWALLOWING AND “HOT POTATO” VOICE. d. A PX COMES IN WITH TRISMUS.8. Retropharyngeal abscess Croup Peritonsillar abscess Epiglottitis . b.

irritability. decreased oral intake and drooling Bulging posterior pharyngeal wall polymicrobial Relatively common Older child. dysphagia Asymmetric tonsillar bulge with displaced uvula “hot potato” voice  Peritonsillar cellulitis/abscess      . adolescent Sore throat. fever. trismus.NECK ABSCESSES  Retropharyngeal and Lateral pharyngeal abscess     Common in young children 3-4 years old or younger Fever .

toxic child. drooling. non toxic. critical airway. life-threatening. no distress.. acute onset. “thumb sign”  .bacterial (H influenza).CROUP VS EPIGLOTITIS Croup – viral (parainfluenza). high fever. sitting forward. “steeple sign”  Epiglotitis. low grade fever. responds to racemic epinephrine and dexamethasone .

THE MOST LIKELY DIAGNOSIS IS a. c.9. AND ON CXR THERE IS A MEDIASTINAL SHIFT TOWARDS THE UNINVOLVED SIDE. d. Pneumonia Spontaneous pneumothorax Lung tumor Atelectasis . A LANKY TEENAGER WENT TO THE ER DUE TO SUDDEN ONSET DYSPNEA. b. HE HAS RETRACTIONS AND DECREASED BREATH SOUNDS OVER THE INVOLVED AREA.

PNEUMOTHORAX       Accumulation of extrapulmonary air within the chest Primary vs secondary Spontaneous. iodopovidone) . traumatic. doxycycline. iatrogenic or catamenial Spontaneous pneumothorax – common among young adults and teenagers who are tall and thin On CXR – evidence of tension includes shift of mediastinal structures away from the side of the air leak Treatment  Small (<5%) or moderate – resolves (1 week)  100% oxygen hastens resolution  Analgesics  Chest tube drainage  Chemical pleurodesis (talc.

Rapidly progressive respiratory obstruction Etiologic agent: Haemophilus influenza b A medical emergency All of the above . b.10. TRUE ABOUT EPIGLOTITTIS a. d. c.

drooling. toxic child acute onset sitting forward critical airway “thumb sign” .EPIGLOTITIS        bacterial (H influenza) life-threatening high fever.

d. c. b. WHICH OF THE FOLLOWING INFECTIONS PRESENT AS PHARYNGEAL INJECTION. WITH TONSILLAR MEMBRANE AND “ BULL NECK” APPEARANCE? a. Diphtheria Pertussis Tetanus Polio .11.

CLUE: BULLNECK AND THE MEMBRANE Caused by Corynebacterium diphtheriae  62 k polypeptide exotoxin  Pseudomembrane  Bull neck appearance – underlying soft tissue edema and enlarged lymph nodes  Toxic cardiomyopathy – 50-60% of deaths  Tx: antitoxin. antibiotics (erythro and penicillin)  .

GOLD STANDARD IN THE TREATMENT OF UNCOMPLICATED SALMONELLOSIS a.12. Penicillin Ceftriaxone Aspirin Chloramphenicol . c. d. b.

Paratyphi C  Relative bradycardia. rose spots  Dx: bone marrow culture  Tx: Chloramphenicol remains the gold standard  . Paratyphi B.SALMONELLOSIS  Nontyphoidal salmonellosis Acute enteritis. extraintestinal focal infections  Tx: antibiotics for bacteremia and extra intestinal infections (ceftriaxone)  For acute enteritis: no antibiotics. Ser Typhi. malnourished. immunocompromised   Enteric or typhoid fever S. Paratyphi A. bacteremia. only for infants <3 mos.

Below age 5 years with BCG > 5 mm 5 years and above: > 5 mm 7 years and above : > 10 mm 7 years and above : > 12 mm . WHICH IS CONSIDERED AS POSITIVE PPD? a.13. d. c. b.

Mantoux test 5 TU PPD-S read at 48-72 hrs  Induration of >5mm is considered positive in the presence of any of the following: history of a close contact with a known or suspected infectious case of TB. >10mm is considered positive  . cxr suggestive of TB and immunocompromised condition  Otherwise. clinical findings suggestive of TB.

frequently exposed to high risk adults > 4yrs old with no risk factors  INDURATION > 10 MM   INDURATION > 15 MM  . those with clinical and radio findings Infants and children <4 yrs. immunocompromised. those with underlying medical conditions.POSITIVE PPD  INDURATION > 5 MM  Close contacts of TB pxs.

d.14. Rifampicin Pyrazinamide Streptomycin Ethambutol . WHICH OF THE FOLLOWING ANTITUBERCULOSIS MEDICATIONS IS CONTRAINDICATED FOR CHILDREN BELOW 7 YEARS OLD? a. b. c.

cytological.TB In Infancy and Childhood  Diagnosis: if 3 or more of the following criteria are present      Exposure to an adult/adolescent with active TB disease Signs and symptoms suggestive of TB Positive tuberculin test Abnormal chest radiograph suggestive of TB Other lab finings suggestive of TB (histological. etc) . biochemical.

failure to gain weight  Failure to respond to 2 weeks of appropriate antibiotics  Failure to gain previous state of health after two weeks of a viral infection or exanthem  Fatigue. reduced playfullness. or lethargy  . loss of weight.Signs and Symptoms Cough/ wheezing of 2 weeks or more  Unexplained fever of 2 weeks or more  Loss of appetite.

clinical findings suggestive of TB. cxr suggestive of TB and immunocompromised condition  Otherwise. >10mm is considered positive  .Mantoux test 5 TU PPD-S read at 48-72 hrs  Induration of >5mm is considered positive in the presence of any of the following: history of a close contact with a known or suspected infectious case of TB.

CNS TB.Clinical Forms of TB  Pulmonary/Intrathoracic TB          Primary Disease Progressive primary disease Pleurisy with effusion Endobronchial Tb chronic pulmonary TB Miliary TB Tuberculoma Pericardial TB Scrofula. etc Extrapulmonary/Extrathoracic  . TB adenitis.

reduce morbidity and the transmission of infection. prevent TB deaths  Anti TB drugs       INH: 10-15mkd Rifampicin: 10-20mkd Pyrazinamide: 20-40mkd Streptomycin: 20-40mkd Ethambutol: 15-25 .Management of TB Intensive Phase (2 mos)  Continuation Phase (4 mos)  DOTS : decrease the risk of infection.

ANTI TB MEDS  Isoniazid  Peripheral neuritis and hepatotoxicity Orange discoloration of urine and tears. thrombocytopenia and influenza like syndrome hyperuricemia Hearing loss  Rifampicin   Pyrazinamide   Streptomycin   Ethambutol Optic neuritis and red-green color blindness  Not recommended for use in children  .

NON-PRURITIC RASH ON THE TRUNK WHICH SPREADS TO THE NECK.15. RAISED . c. Rubella Roseola Rubeola Mumps . d. b. WHICH OF THE FOLLOWING VIRAL INFECTIONS PRESENTS WITH HIGH FEVER WHICH ABRUPTLY LYSES AND DISCRETE PINK. FACE AND PROXIMAL EXTREMITIES? a.

5th erythema infectiosum  Human herpesvirus 6 and 7 ( more of 6)  Nagayama spots – ulcers at the uvulopalatoglossal junction  Primary HHV 6 infxn – 1/3 of febrile seizure cases  . 2nd scarlet. 3rd rubella.ROSEOLA INFANTUM Exanthem subitum or 6th disease  Recall: 1st – measles. 4th atypical scarlet( Filatov-Duke’s).

16. WHICH IS THE VECTOR OF DENGUE FEVER? a. Anopheles mosquito Culex mosquito Aedes aegypti mosquito Aedes africanus . b. d. c.

longer mosquito survival Ave lifespan: 8-15 d . urban environm’t Dispersal: 30-50 m/d Visits not more than 2-3 houses Remains infective during lifetime High humidity.Aedes aegypti Flight range: < 25 m in open.

b.17. PRESENTING WITH INTENSE PARASITISM a. c. d. Malariae Falcifarum Vivax Ovale . MOST SEVERE FORM OF MALARIA.

MALARIA P. Malariae – every other or third day fever spikes  Periodicity of fever.quinine  Blackwater fever. Ovale – daily fever spikes  P. ruptured spleen  P.less apparent in falciparum  Tx. algid malaria  . chloroquine. for chloroquine resistance. Vivax and P. falcifarum is the most severe form and is associated with more intense parasitemia  P. Vivax – less severe. malariae is the mildest and most chronic  P.

d. c. DRUG OF CHOICE FOR PREVENTION OF RELAPSES OF P. Quinine Chloroquine Primaquine Pyrimethamine –sulfadoxime . VIVAX AND P.18. OVALE INFECTION a. b.

Vivax.RELAPSE  Common in P. ovale and malariae  Caused by release of merozoites from an exoerythrocytic source in the liver (vivax an ovale) or persistence within the erythrocyte (malariae)  Primaquine OD x 14 days .

d. c. WHICH OF THE FOLLOWING INFESTATIONS IS NOT DETECTED BY DIRECT FECAL SMEAR? a.19. Enterobiasis Trichuriasis Hookworms Diphyllobotriasis . b.

diphyllobotriasis  Microscopic exam of cellophane tape enterobius  Demonstration in the blood .filariasis  Stool exam by Kato thick smear schistosomiasis . hookworms.HELMINTHIC DISEASES  Direct fecal exam  Ascaris. trichuriasis. strongyloides.

WHICH OF THE FOLLOWING VACCINES IS PART OF THE EPI? a. Tetanus toxoid Varicella vaccine Rotavirus vaccine Typhoid vaccine . d.20. b. c.

EXPANDED PROGRAM ON IMMUNIZATION BCG  HEPATITIS B  DPT  POLIO  MEASLES  RECENT INCLUSIONS: HiB. MMR. ROTAVIRUS  .

Acute leukemia Iron deficiency anemia Idiopathic thrombocytopenic purpura Hereditary spherocytosis . c.21. b. d. WHAT IS THE MOST COMMON HEMATOLOGIC DISEASE OF INFANCY AND CHILDHOOD? a.

it is unusual to have anemia due to inadequate dietary iron before 6 months (usually occurs around 9-24 months)  Chronic iron def : peptic ulcer.IRON DEFICIENCY ANEMIA Most common hematologic disease of infancy and childhood  Newborn – contains 0.5g of iron  Adult – 5g  Iron stores in term infants are sufficient for blood formation in the first 6-9 months  Therefore. polyp. Meckel. hemangioma. or inflammatory bowel disease  Hookworm infestation  .

c. IN PHYSIOLOGIC ANEMIA OF INFANCY. 3-6 10-12 6-8 2-4 . HEMOGLOBIN DECLINES AT ____ WEEKS FOR TERM INFANTS a.22. b. d.

Hgb starts to decrease and may persist for 6-8 weeks  Increase in blood oxygen content and tissue oxygen delivery downregulates erythropoietin (EPO) production  Peak: 8-12 weeks (Hgb 9-11)  Prematures: more exaggerated (3-6 wks.PHYSIOLOGIC ANEMIA OF INFANCY Newborns have higher Hgb and Hct  Within the 1st week. Hgb 7-9)  .

d. b.23. WHICH OF THE FOLLOWING PRODUCES PERIPHERAL PANCYTOPENIA WITH HYPOPLASTIC MARROW? a. Acute leukemia Iron deficiency anemia Idiopathic thrombocytopenic purpura Aplastic anemia . c.

coupled with hypoplastic or aplastic bone marrow  Severe aplastic anemia – 2 or more cell components have become seriously compromised (ANC < 500/mm3.000 -100.000-100.Aplastic Anemia Hallmark is peripheral pancytopenia.000 and retic count <1%  . PC 20. retic count <1% after correction for hematocrit)  Moderate aplastic anemia – ANC 500 -1. PC 20.500/mm3.000/mm3.

AND MOSTLY ARISING FROM THE ABDOMEN a. MOST FREQUENTLY DIAGNOSED DURING INFANCY. c.24. b. Wilm’s tumor Hepatoblastoma Neuroblastoma Nephroblastoma . d. MOST COMMON EXTRACRANIAL TUMOR OF CHILDHOOD.

90% before 5 yr Most cases arise in the abdomen CT scan: calcification and hemorrhage Tumor markers: VMA and HVA Also known as Nephroblastoma Most common primary malignant renal tumor of childhood. Denys-Drash. aniridia. Beckwith-Wiedemann) Occurs in children between 2-5 years old CT: no calcification  Wilm’s Tumor       . and other congenital anomalies (WAGR. 2nd most common malignant abdominal tumor in childhood 3 elements: blastema.CANCER AND BENIGN TUMORS  Neuroblastoma       Most common extracranial solid tumor in children and the most commonly diagnosed malignancy in infants Embryonal cancer of the peripheral SNS Median age of dx is 2 yr. epithelia and stroma Associated with hemihypertrophy.

Osteosarcoma Ewing’s sarcoma Rhabdomyosarcoma Enchondroma .25. c. b. A 15-YEAR OLD CONSULTED FOR ARM SWELLING AND PAIN. X-RAY SHOWED SCLEROTIC DESTRUCTION OF THE PROXIMAL HUMERUS WITH SUNBURST PATTERN. d. THE MOST LIKELY DIAGNOSIS IS a.

bones Chemotherapy Without mets: 70% cured With mets: < 20% survival Ewing’s Second decade Primarily whites 1. flat bones Local pain &swelling Onion skinning Lungs.5:1 Small round cell None Diaphyses. bones Chemotherapy W/o mets: 60% cured With mets: 20-30% survival Feature Age Race Sex (M:F) Cell Predisposition Site Presentation Radiographic finding Metastasis Tx Outcome .CANCER AND BENIGN TUMORS  Bone Tumors Osteosarcoma Second decade All races 1.5:1 Spindle cell Retinoblastoma. Paget’s Metaphyses of long bones Local pain & swelling Sunburst pattern Lungs.

Factors II.26. XI Factors II. VIII. IX. d. VII. c. VIII. IX. DEFICIENCY IN WHICH OF THE FOLLOWING FACTORS WILL YOU NEED TO GIVE VITAMIN K? a. b. V. X . IX Factors V. VII. X Factors II. IX. X.

.

YOU SUSPECT THAT THE BABY HAS a. c. Congenital varicella syndrome Neonatal herpes syndrome Congenital syphillis Congenital rubella syndrome . A NEWBORN PRESENTED WITH “BLUEBERRY MUFFIN” RASH. CATARACTS. PULMONARY STENOSIS AND HEARING LOSS.27. b. d.

late –Hutchinsons teeth. blueberry muffin lesions (same as in CMV)  Neonatal herpes  . olympian brow Congenital varicella – malformations of the extremities with cicatricial scarring  Congenital rubella – cataracts. heart defects. saber shins.CONGENITAL INFECTIONS  Congenital Syphilis   Early and late signs Early – snuffles. deafness.

THE RECTAL VAULT WAS EMPTY BUT A GUSH OF FOUL-SMELLING GAS WAS NOTED ON RECTAL EXAM. A BABY WAS BROUGHT TO YOUR CLINIC BECAUSE OF FAILURE TO THRIVE. b. Intussusception Congenital aganglionic megacolon Hypertrophic pyloric stenosis Gastric volvulus . d.28. BARIUM ENEMA SHOWED A DILATED PROXIMAL COLON AND SMALL CALIBER DISTAL COLON. HE HAS ABOMINAL DISTENTION AND A PALPABLE MASS ON THE LLQ. c. YOUR DIAGNOSIS IS a. SHORT OF A BIOPSY. HE HAD DELAYED PASSAGE OF MECONIUM AND CHRONIC CONSTIPATION.

meconium is passed within the first 48 hours) Rectal exam demonstrates normal anal tone and is usually followed by an explosive discharge of foul smelling feces and gas Rectal manometry and rectal suction biopsy Barium enema is useful in determining the extent of aganglionosis Definitive treatment is operative intervention . Congenital Aganglionic Megacolon (Hirschspung Disease)        Most common cause of lower intestinal obstruction in neonates Caused by abnormal innervation of the bowel (absence of ganglion cells). beginning in the internal anal sphincter and extending proximally to involve a variable length of gut Symptoms usually begin at birth with the delayed passage of meconium (In 99% of full term infants.

b. c. d.29. PRECOCIOUS PUBERTY IS DEFINED AS ONSET OF PUBERTY BEFORE AGE 8 IN GIRLS AND __ IN BOYS a. 8 9 10 11 .

McCune –Albright)  Gonadotropin independent  Incomplete .PRECOCIOUS PUBERTY  Onset of secondary sexual characteristics before 8 years of age in girls and 9 years in boys  Conditions causing precocious puberty  Gonadotropin dependent puberty  Combined gonadotropin dependent and independent (ex.

b. Oxytocin Prolactin Leutinizing hormone Follicle stimulationg hormone . WHICH OF THE FOLLOWING HORMONES IS NOT FOUND IN THE ANTERIOR PITUITARY a.30. c. d.

ACTH. prolactin. POMC  Posterior Pituitary  Vasopressin and oxytocin . TSH.PITUITARY HORMONES  Anterior Pituitary  GH. FSH. LH.

31. c. HE HAS BEEN POLYURIC AND POLYDIPSIC FOR QUITE SOME TIME. HE HAS LOW URINE AND SERUM OSMOLALITY. A 12 YEAR OLD MALE WAS BROUGHT IN FOR DEHYDRATION. AND HYPERNATREMIA. TO DIFFERENTIATE BETWEEN CENTRAL AND NEPHROGENIC ORIGIN. YOU DO: a. d. Insulin challenge test DDAVP test Erythropoietin test Beta agonist challenge . b. YOU SUSPECT DIABETES INSIPIDUS.

polydipsia (exceeding 2L/m2/24hr). inc vasopressin metabolism  Nephrogenic DI Vasopressin insensitive  Genetic (less common but more severe) or acquired causes  . infections. congenital malformations of the hypothalamus or pituitary. neoplasms. hypernatremia  Serum osmolality >300 mOsm/kg and urine osmolality <300 mOsm/kg  Central vs nephrogenic  Central DI   Genetic mutations to the vasopressin gene. trauma to vasopressin neurons.DIABETES INSIPIDUS Polyuria. autoimmune diseases.

YOU MEASURE HIS 3. Diabetic ketoacidosis Insulin shock Somogyi phenomenon Dawn phenomenon . d. AND 7 AM GLUCOSE CONCENTRATION. c. b.32. THE 3 AND 4 AM LEVELS ARE BELOW 60 MG/DL. YOU SUSPECT BRITTLE DIABETES. 4. A KNOWN INSULIN DEPENDENT DIABETIC COMES TO YOUR CLINIC AND COMPLAINS OF EARLY MORNING HYPERGLYCEMIA. WHILE THE 7 AM IS MARKEDLY HIGH. THE PX IS EXPERIENCING a.

DIABETES MELLITUS  Somogyi phenomenon Theoretical rebound from late night or early morning hypoglycemia  Exaggerated counterregulatory response  rare   Dawn phenomenon Due to overnight GH secretion and inc insulin clearance  Usually recurrent and modestly elevates morning levels   Brittle diabetes Unexplained wide fluctuations in glucose levels  Usually an adolescent female with recurrent DKA taking large doses of insulin  .

K. DOES NOT FEED WELL AND IS CONSTIPATED. T4. b. TSH Serum Na. d. UMBILICAL HERNIA. A NEIGHBOR SHOWS YOU HER 3-WEEK OLD BABY FOR CONSULT.33. AND MOTTLED SKIN. c. Ca . SHE TELLS YOU THAT THE BABY IS SLUGGISH. ENLARGED ABDOMEN. WHICH OF THE FOLLOWING LABORATORY EXAMS WILL YOU PRIORITIZE TO HELP YOU WITH YOUR DIAGNOSIS? a. YOU NOTE THAT SHE IS JAUNDICED WITH A GLAZED LOOK. Chest x-ray Lumbar tap Serum T3.

15% .inborn error of thyroxine synthesis  Detected by newborn screening  F>M  May be normal at birth (but head size may be slightly increased due to myxedema of the brain)  Prolongation of physiologic jaundice – earliest sign  Other findings: feeding difficulties. somnolence. lack of interest. choking spells during nursing. constipation.CONGENITAL HYPOTHYROIDISM Most common cause is thyroid dysgenesis (8085%). sluggishness. large abdomen. umbilical hernia  Low serum T4 and elevated TSH levels  Tx: levothyroxine  .

c. WHAT WILL YOU DO? a. AND ASKS IF THERE IS SOMETHING WRONG WITH HER. AND SHE IS IN EXCELLENT HEALTH. SHE IS VERY TALL (90TH PERCENTILE). d. YOUR ADOLESCENT NIECE TALKS TO YOU ABOUT HER HEIGHT. Assure her that she is normal Have x-rays done to check her epiphysis Get her growth hormone levels Have her undergo cranial CT scan . HER PARENTS AND SIBLINGS ARE ALL TALL.34. b.

LIKE MAPLE SYRUP. isoleucine and valine levels and low alanine levels High isovaleric acid. SHE HAS BEEN VOMITING AND FEEDING POORLY. AND EXPECT THE FOLLOWING RESULT: a. d. isovaleryglycerine and hydroxyvaleric acid levels High methylmalonyl acid levels High glutamine and alanine levels . b. YOU THEN ASK FOR AMINOACID ASSAY. A WEEK OLD BABY WAS BROUGHT TO THE ER BECAUSE OF WEAKNESS. c. YOU NOTED THAT HER URINE SMELLED SWEET. High leucine. YOU TELL THE ATTENDING PHYSICIAN THAT WHILE DOING YOUR PE. HER LAB EXAMS ARE UNREMARKABLE EXCEPT FOR SEVERE METABOLIC ACIDOSIS.35.

INBORN ERRORS OF METABOLISM ASSOCIATED WITH ABNORMAL ODOR Inborn Error of Metabolism Urine Odor Glutaric Acidemia (Type III) Hawkinsinuria Isovaleric Acidemia Maple Syrup Urine Disease Hypermethioninemia Multiple carboxylase deficiency Phenylketonuria Trimethylaminuria Tyrosinemia Sweaty feet. rancid butter . Acrid Maple syrup Boiled cabbage Tomcat urine Mousy or musty Rotting fish Boiled cabbage. Acrid Swimming pool Sweaty feet.

MAPLE SYRUP URINE DISEASE  Diagnosis   Peculiar odor of maple syrup Elevated levels of branched chain amino acids (ILV)  Treatment Aim: rapid removal of branched chain amino acids and their metabolites  Diet  Hydration  Dialysis .

Ceftriaxone d. THE MOTHER DID NOT HAVE PRENATAL CHECK-UP AND HER IMMUNIZATION STATUS WAS UNKNOWN. THE DRUG OF CHOICE IS a. A NEWBORN WAS BROUGHT TO THE ER FOR SEIZURES. YOUR DIAGNOSIS IS NEONATAL TETANUS. Chloramphenicol . Penicillin b. Metronidazole c. SHE WAS BORN AT HOME AND A SHARPENED BAMBOO STICK WAS USED TO CUT THE CORD.36.

NEONATAL TETANUS     Most common form of tetanus An acute spastic paralytic illness caused by the neurotoxin (tatanospasmin) produced by Clostridium tetani Tetanospasmin – 2nd most poisonous substance known (surpassed in potency only by the botulinum toxin) Manifestations In infants – progressive difficulty in feeding. erythromycin. associated hunger and crying  In older children.trismus (masseter muscle spasm or lockjaw). Tetracycline  Surgery  . alternatives: metronidazole. sardonic smile ( intractable spasms of the facial and buccal muscles)   Treatment Human Tetanus Ig  Penicillin G – antibiotic of choice.

THEN WEAKNESS OF THE UPPER TRUNK AND EXTREMITIES AND FINALLY FACIAL WEAKNESS. HE WAS IMMEDIATELY INTUBATED. b.37. THE ILLNESS STARTED AS DIFFICULTY IN WALKING. d. THE HX IS UNREMARKABLE EXCEPT FOR A NON SPECIFIC VIRAL ILLNESS 2 WEEKS AGO. Bell palsy Transverse myelitis Spinal muscular atrophy Guillain-Barre Syndrome . c. PE SHOWS COMPLETE PARALYSIS WITH ABSENT TENDON REFLEXES. THE LIKELY DX IS a. A TEENAGER WAS BROUGHT TO THE ER FOR RESPIRATORY DISTRESS.

GUILLAIN – BARRE SYNDROME         Postinfectious polyneuropathy involving mainly motor but sometimes also sensory and autonomic nerves Usually follows a nonspecific viral infection Weakness begins in the lower extremities and progressively involves the trunk. and finally the bulbar muscles (a pattern known as Landry ascending paralysis) Proximal and distal muscles are involved relatively symmetrically CSF – protein is elevated to more than 2x the upper limit of normal Dissociation between high CSF protein and a lack of cellular response is diagnostic Treatment: IVIG Alternative Tx: Plasmapheresis and immunosuppressive drugs . the upper limbs.

c. MOST COMMON INFRATENTORIAL TUMOR IN CHILDREN a. Medulloblastoma Brainstem glioma Cerebellar astrocytoma ependymoma . b. d.38.

9%)  Multiple sites (11%)   Important! Within the 1st year –supratentorial  1-10 yr – infratentorial  Cranial exposure to ionizing radiation – inc incidence  .2%)  Supratentorial (40.9%)  Spinal cord (4.CANCER AND BENIGN TUMORS  Brain Tumors in Children  Categories Juvenile pilocytic astrocytoma – MOST COMMON  Medulloblastoma  Diffuse astrocytoma  Ependymoma  craniopharyngioma   Location Infratentorial (43.

PROTEIN 10 MG/DL. WBC 10/MM2.39. THE PATIENT HAS: a. GLUCOSE 60% OF SERUM GLUCOSE. TB meningitis Bacterial meningitis Normal CSF Viral meningitis . A NEWBORN IS SUSPECTED TO HAVE SEPSIS. c. b. CSF STUDIES SHOWED RBC=0. LUMBAR TAP WAS DONE AS PART OF THE WORK-UP. d.

CEREBROSPINAL FLUID LP: L3-L4 or L4-L5  OP: 100 mm/Hg ( 60-180)  Contraindications  Elevated ICP  s/sx of pending cerebral herniation  Critical illness  Skin infection at the site  Thrombocytopenia (<20 x 109)  Normal CSF  Clear. glucose 60%  . protein 10-40 mg/dl (120 in a neonate). no rbcs. wbc up to 5 (for NB up to15).

CRANIAL CT SCAN OF A CHILD WITH MULTIPLE CONGENITAL ANOMALIES SHOWED ABSENCE OF CONVOLUTIONS. d. Porencephaly Lissencephaly Schizencephaly Holoprosencephaly . THIS IS KNOWN AS a. c. b.40.

DISORDERS OF NEURONAL MIGRATION Lissencephaly  Absence of convolutions (agyria)  Schizencephaly  Presence of unilateral or bilateral clefts within the hemispheres  Porencephaly  Presence of cysts or cavities within the brain  Holoprocencephaly  Defective cleavage of the procencephalon  .

d.41. b. AND ACTS AS IF NOTHING HAPPENED IN THE MORNING. THE DIAGNOSIS IS a. THE CHILD GOES BACK TO SLEEP IN A FEW MINUTES. c. Night terror Rage attack Choreoathetosis Nightmares . YOUR FRIEND TELLS YOU THAT HIS CHILD WAKES UP AROUND MIDNIGHT SCREAMING AND HYPERVENTILATING.

scary or exciting events which the child can recall upon waking up  Night terrors  Less common  Last 10-15 minutes.NIGHT TERRORS VS NIGHTMARES  Nightmares Common and usually involve vivid. during which the child is not easily aroused and appears frightened or agitated  No recollection of the event  .

THERE ARE EXCORIATED. Lindane Topical corticosteroid Selsun blue Tretinoin . A CHILD COMPLAINS OF INTENSE PRURITUS OF THE SKIN. b. THE INTERDIGITAL SPACES AND FLEXOR AREAS.42. AND SCALING PAPULES ON THE TRUNK. THE TREATMENT OF CHOICE IS a. PARTICULARLY AT NIGHT. d. PARTICULARLY THE WAIST AREA. c. CRUSTING.

classic lessions  Treatment: Permethrin 5%  Additional therapies: Lindane 1% lotion or cream and oral ivermectin  .SCABIES Caused by burrowing and release of toxic and antigenic substances by the female mite Sarcoptes scabiei var hominis  Most important factor that determines spread of scabies is the extent and duration of physical contact with an affected individual  Intense pruritus particularly at night  Threadlike burrows.

b. 300 400 500 600 . d. c. A NEWBORN’S WEIGHT GAIN IS APPROXIMATELY __ GMS/MONTH AT AGE 2-6 MONTHS a.43.

WEIGHT GAIN DURING THE FIRST YEAR  0-2 MOS  30 g/day  2-6 MOS  20 g/day  6-9 MOS  15 g/day  9-12 MOS  12 g/day .

d. b. c.44. THE FIRST PRIMARY TEETH TO ERUPT ARE a. Upper central incisors Lower central incisors Upper lateral incisors cuspids .

CHRONOLOGY OF HUMAN DENTITION (PRIMARY) AGES (MOS) Maxillary AGES (MOS) Mandibular Central incisors Lateral incisors Cuspids First molars Second molars 6-8 8-11 16-20 10-16 20-30 5-7 7-10 16-20 10-16 20-30 .

2 3 4 5 . HANDEDNESS IS USUALLY ESTABLISHED AT AGE ___ YEARS a.45. c. d. b.

bedwetting up to age 4 in girls and 5 years in boys  Piaget’s preoperational stage: magical thinking. egocentrism and thinking that is dominated by perception. not abstraction  .Preschool Years Between 2 and 5 years old  Emergence of language and exposure of children to an expanding social sphere  Handedness is usually established at 3 y/o  Bowel and bladder control.

Voice change Pubic hair Growth spurt Testicular enlargement .46. d. THE FIRST VISIBLE SIGN OF PUBERTY IN MALES IS a. b. c.

physiology. and psychological and social functioning  In boys: testicular enlargement  In girls: appearance of breast buds  . shape. middle and late adolescence  Rapid changes in body size.ADOLESCENCE 10-20 years old  Early.

d. A 3-YEAR OLD WAS BROUGHT TO YOU FOR EVALUATION OF DELAYED SPEECH.47. Asperger disorder Autism Schizophrenia Attention deficit disorder . THE CHILD HAS REPETITIVE BEHAVIOR. c. NO EYE CONTACT. b. ALONE. HE IS SUSPECTED TO HAVE a.

PERVASIVE DEVELOPMENTAL DISORDERS Autistic Disorder  Asperger Disorder  Childhood Disintegrative Disorder  Rett Disorder  Childhood Schizophrenia  .

little symbolic play. and reliance on non verbal communication with delay in use of words  .Autism Develops before 36 months and is typically diagnosable at 18 mos of age  Qualitative impairment in verbal and non verbal communication. limited joint attention or orienting to one’s name. repetitive behaviors. in imaginative activity and in reciprocal social interactions  Males>females  Poor eye contact.

Autism CHAT. behavior modification  Pharmacotherapy: SSRIs.checklist for autism in toddlers  Intensive behavioral therapy before 3 yrs old targeted toward speech and language development  Educational programming. clonidine  .

boys die at birth   development is normal until about 1-2 years old until motor and language development regress .Asperger Disorder – no severe language impairments as compared to patients with autism  Rett Disorder – x-linked dominant disorder affecting girls exclusively.

YOU EXPECT A SEVEN MONTH OLD TO BE ABLE TO a. d. Pull to stand Sit without support Walk alone Grasp object with thumb and forefinger . b. c.48.

MOTOR DEVELOPMENT Dependent on brain maturity Sequence : Gross: cephalocaudal Fine : proximodistal Weakest correlation to IQ .

GROSS MOTOR MILESTONES Mean Age (mos) Milestones 3 Head control 5 6 Roll over Sit with support 8 9 Sit alone Pulls to stand holding on 11 15 24 36 Stand independently Walk alone Climb up and down stairs Ride a trike 48 Hop on one foot .

5 4 4 5.FINE MOTOR MILESTONES Mean Age (mos) 3.5 8 12 13 15 22 Milestones Grasps rattle Reaches for objects Palmar grasp gone Transfers objects hand to hand Pincer grasp Turns pages of books Scribbles Builds tower of 2 cubes Builds tower of 6 cubes .

c. NYCTALOPIA. b. A B C D . d. XEROPHTHALMIA. AND PHOTOPHOBIA ARE CAUSED BY DEFICIENCY IN WHICH VITAMIN? a.49.

anorexia. seborrhea  Pyridoxine: irritability. constipation  Riboflavin  Niacin: pellagra  Folacin: megaloblastic anemia  Cyanocobalamin: pernicious anemia  Biotin: dermatitis. fatigue. xerophthalmia.VITAMIN DEFICIENCIES       Vitamin A: photophobia. convulsions Vitamin C: scurvy and poor wound healing Vitamin D: rickets Vitamin E: red blood cell hemolysis Vitamin K: hemorrhage . blindness Vitamin B: beriberi.

Calcium Iron Magnesium Fluoride .50. d. c. b. WHICH OF THE FOLLOWING MINERAL WILL CAUSE MOTTLING OF THE TEETH WHEN TAKEN IN EXCESS? a.

05mkd) during enamel formation  Severe brownish discoloration and hypoplasia – also seen with fluoride concentrations in the drinking water >5ppm  For prevention of dental caries – 1ppm  Children < 6y/o : pea sized amount of toothpaste  Children < 2 y/o : “smear”  .FLUOROSIS Mottled enamel  Results from systemic fluoride consumption (>0.

HIS EXTREMITIES ARE COOL. THE DIAGNOSIS IS a. HIS FACE LOOKS WIZENED DUE TO LACK OF SUBCUTANEOUS FAT. b. SLIGHTLY DISTENDED ABDOMEN. MUSCLE ATROPHY. Kwashiorkor Marasmus Pellagra Rickets . d. HE HAS WRINKLED LOOSE SKIN.51. AND TEMP IS SUBNORMAL. c. AN EMACIATED CHILD WAS BROUGHT TO THE ER FOR MEDICAL MANAGEMENT.

obvious muscle wasting.150 cal/kg/d and 4g/kg/d of protein  . flaky dermatosis. flag sign. moonface. potbelly. psychomotor changes. diarrhea  Marasmus: gross loss of subcutaneous fat. winged scapula  Treatment of PEM: 3 phases First : stabilization. antibiotics  Second: Diet providing maintenance requirements of protein and energy  Third: Recovery diet. anemia.PROTEIN/ENERGY MALNUTRITION (PEM)  Edematous (Kwashiorkor) and Nonedematous (Marasmus) PEM  Edematous: edema. correction of dehydration.

d. SIMIAN CREASE. c. 7q23 deletion Trisomy 18 Trisomy 21 22q11 deletion . AND SHORT BROAD HANDS HAS a. b.52. A CHILD WITH HYPOTONIA. EPICANTHAL FOLD. COMPLETE ATRIO-VENTRICULAR SEPTAL DEFECT. SLANTED PALPEBRAL FISSURES.

TRISOMY 21 (DOWN SYNDROME) Incidence: 1/600-800 births  Findings        Hypotonia Upward and slanted palpebral fissures and epicanthic folds Speckled irises (Brushfield spots) Varying degrees of mental and growth retardation Cardiac malformations Simian crease .

.

b. c. PREMATURE BIRTH IS DEFINED AS LIVE BIRTH BEFORE __ WEEKS FROM THE FIRST DAY OF THE LAST MENSTRUAL PERIOD: a. 39 38 37 36 .53. d.

1.000 2.500 2.54. VERY LOW BIRTHWEIGHT INFANTS WEIGH LESS THAN ____ GMS a. c.000 1. b.500 . d.

maternal and fetal factors  IUGR.influenced by prenatal.associated with preterm birth and low birth weight and lethal congenital anomalies  Low Birth Weight    LBW : 2500g or less VLBW: 1500g or less . Perinatal Mortality . severe congenital malformations. early onset neonatal infections Neonatal Deaths . placental insufficiency. asphyxia.

EXCEPT: a.55. c. d. b. Physiologic jaundice ABO incompatibility Sepsis Hemorrhage . DIFFERENTIAL DIAGNOSES FOR JAUNDICE IN THE FIRST 24 HOURS OF LIFE.

5 mg/dl/hr >8 days (term). 14 days (preterm) symptomatic .HYPERBILIRUBINEMIA Newborns appear jaundiced when the bil level is > 7 mg/dl  Physiologic hyperbilirubinemia         Onset > 24 hours Inc RBC production Inc enterohepatic circulation Defective uptake Decreased conjugation Decreased hepatic secretion Pathologic     Onset < 24 hrs Rise of >0.

active deep tendon reflexes  After 1st year: movement disorders. upward gaze  . range: 21-50  Clinical features   Acute Phase 1: poor suck.KERNICTERUS Bilirubin encephalopathy resulting from the deposition of unconjugated (indirect) bilirubin in the basal ganglia and brainstem nuclei  > 25mg/dl. SNHL. retrocolis. fever  Phase 3: hypertonia   Chronic 1st year : hypotonia. seizures  Phase 2: hypertonia of extensor muscles. opisthotonos. hypotonia.

loose stools. overheating. bronze baby syndrome  Intravenous Immunoglobulin  Metalloporphyrins   Competitive enzymatic inhibition of the rate limiting conversion of heme protein to biliverdin by heme oxygenase double volume exchange transfusion  Exchange Transfusion  . rash.KERNICTERUS  Treatment of Hyperbilirubinemia  Phototherapy Photoisomerization  Complication. dehydration.

b. YOU NOTICE SEVERAL HEALING WOUNDS ON THE LEGS. ON PE. YOUR PRIMARY DIAGNOSIS IS: a.56. Nephrotic syndrome Renal tubular acidosis Acute glomerulonephritis IgA nephropathy . ON FURTHER PROBING. THE EYES WERE NOTED TO BE PUFFY PARTICULARLY IN THE MORNING. THE MOTHER CANNOT RECALL ANY PRIOR ILLNESS. c. AND HIS BP IS HIGH. THE CHILD CONFESSES THAT HIS URINE IS COLA COLORED. A CHILD WAS BROUGHT TO THE ER DUE TO EDEMA. d. IT STARTED ON THE FEET PROGRESSING TOWARDS THE THIGHT.

hypertension. clinical improvement Early convalescent: increased well being . edema Oliguric: edema. azotemia. oligoanuria Diuretic: spontaneous voiding.POST STREPTOCOCCAL GLOMERULONEPHRITIS  Most common in children age 5 to 12 years  Uncommon before the age of 3  Phases:     Latent: hematuria.

POST-STREPTOCOCCAL GLOMERULONEPHRITIS  Serochemical findings:      Decreased C3 Elevated ASO or anti DNAse B Hyperkalemia Normal CBC. rbcs . may have dilutional anemia Elevated BUN and creatinine during the oliguric phase  Urinalysis: proteins.

hypertension.ACUTE POSTSTREP GN Acute onset of gross hematuria. and renal insufficiency  Follows infection of the throat or skin by nephritogenic strains of group A B-hemolytic strep  Kidneys are enlarged. edema. on EM – there are “humps” on the GBM  Low C3 levels  Edema is due to salt and water retention  .

b. c. d.58. THE MOST COMMON CAUSE OF IDIOPATHIC NEPHROTIC SYNDROME IS a. Focal segmental sclerosis Membranous nephropathy Membranoproliferative GN type 1 Minimal change nephrotic syndrome .

NEPHROTIC SYNDROME  Heavy proteinuria . mesangial proliferation (5%). hypoalbuminemia. edema and hyperlipidemia  90% idiopathic  Causes of idiopathic: minimal change disease(85%). and focal segmental glomerulosclerosis (10%) .

YOU NOTE THAT A NEWBORN BABY BOY’S PREPUCE CANNOT BE RETRACTED. d. c. b. Hypospadias Phimosis Chordee Paraphimosis .59. HE HAS a.

a urethral opening that is on the ventral surface of the penile shaft (1/250)  Chordee without hypospadias – mild or moderate ventral penile curvature (chordee) and incomplete development of the foreskin but the urethral meatus is at the tip of the glans  Phimosis – inability to retract the prepuce  Paraphimosis – occurs when the foreskin is retracted past the coronal sulcus and the prepuce cannot be pulled back over the glans  .ANOMALIES OF THE PENIS AND URETHRA Hypospadias.

1 month 2 months 3 months 4 months . THE MOTHER ASKS IF SURGERY IS REQUIRED TO BRING IT DOWN. b. HOW LONG WILL YOU OBSERVE? a. d. YOU TELL HER THAT YOU WILL OBSERVE FOR THE MEANTIME. c. A 3-MONTH OLD BABY WITH UNILATERAL UNDESCENDED TESTIS WAS BROUGHT TO YOU FOR THE FIRST TIME.60.

UNDESCENDED TESTES (CRYPTORCHIDISM)  Most common disorder of sexual differentiation in boys  4.5% of boys at birth  Bilateral in 10% of cases  Majority descend spontaneously by 3 months  If the testis does not descend by 4 months. it will remain undescended .

d. c. Continuous convulsion lasting for 25 minutes Serial convulsions with return of consciousness in between Always generalized A medical emergency . b. WHICH IS TRUE ABOUT STATUS EPILEPTICUS? a.61.

STATUS EPILEPTICUS  Continuous convulsion lasting longer that 30 minutes  Serial convulsions with no return of consciousness in between  May be generalized or partial  Medical emergency .

d. b. c.62. Hydration Oxygen support Lactate administration Beta blocker administration . THE FOLLOWING ARE PART OF THE MANAGEMENT OF HYPERCYANOTIC SPELLS. EXCEPT: a.

TREATMENT OF HYPOXIC SPELLS        Knee chest position – traps systemic venous blood in the legs thereby temporarily decreasing the systemic venous return and helping calm the baby. This also increases the systemic venous resistance (SVR) by reducing arterial blood flow through the femoral arteries Morphine sulfate – suppresses the respiratory center and abolishes hyperpnea NaHCO3 – corrects acidosis and eliminates the respiratory center-stimulating effects of acidosis Administration of oxygen may improve arterial oxygen saturation a little Vasoconstrictors such as phenylephrine raise SVR Ketamine – increases SVR and sedates the patient Propranolol .

HOW WILL YOU MANAGE A PATIENT WITH ACUTE OTITIS MEDIA WHO HAS BEEN TREATED WITH AMOXICILLIN AT 50 MKD FOR 3 DAYS. b. EAR PAIN AND EAR DISCHARGE? THE PATIENT HAS NOT BEEN TREATED WITH ANY OTHER ANTIBIOTIC PRIOR TO THIS EPISODE? a. c. d. Continue his medication Do tympanocentesis Give clindamycin at 40 mkd Increase the amoxicillin dose to 80-90 mkd .63. BUT STILL HAS FEVER.

children who are exposed to large number of children  2nd line: Amoxicillin-clavulanic acid. children who have recently received treatment with B lactam drugs. ceftriaxone  . cefuroxime. Moraxella catarrhalis  Treatment:  1st line : Amoxicillin 40 mkd 80-100mkd  Higher dose for children < 2 yrs.ACUTE OTITIS MEDIA Etiology: Strep pneumoniae. non-typable H influenzae.

WHAT ADVICE WILL YOU GIVE? a. NUTRITIONAL HISTORY REVEALS A WELL-BALANCED DIET. Enrol her daughter on a weight loss program Prescribe multivitamins and mineral supplements Assure her that weight for age is normal and continue with her diet Tell her to wait for her menarche and then you will reassess . A MOTHER IS CONCERNED ABOUT HER DAUGHTER’S WEIGHT.65. c. d. THE 9 YEAR OLD GIRL WEIGHS 26 KG. b.

in gms = Age in mos x 600 + BW 6-12 months Wt. in gms = Age in mos x 500 + BW 2 years and up Wt. in kgs = Age in years x 2 + 8 .WEIGHT • • Best index of growth and nutrition Mnemonics: • • • • • • Infants < 6 months Wt.

WHICH OF THE FOLLOWING DISEASES IS PART OF THE NEONATAL NEWBORN SCREENING? a. d. b.66. Congenital toxoplasmosis Congenital rubella Congenital adrenal hyperplasia Congenital varicella . c.

NEWBORN SCREENING .

METABOLIC DISEASES  Inborn Errors of Metabolism      Aminoacids: PKU. GAL. Galactosemia Mucopolysaccharides: Hurler’s. Lesch-Nyhan  Newborn Screening PKU. G6PD  Republic Act 9288: Newborn Screening Act of 2004  Done after the 24th HOL and not later than 72 HOL  . Tyrosinemia Lipids: ALD Carbohydrates: Von Gierke’s. CAH. Sanfilippo Purine and Pyrimidines: Gout. Hunter’s. CH. Pompe’s. MSUD.

7 8 9 10 . ACTIVE MOTION. d. SHOW IRREGULAR BREATHING. b. A NEONATE WITH A HEART RATE OVER 100/MIN. c. PINK BODY BUT BLUE EXTREMITIES HAS AN APGAR SCORE OF __ AT 1 MINUTE a.67. GRIMACE.

PHYSICAL EXAM APGAR SCORE Dictates the need to resuscitate Normal: 7. expect neurologic sequelae 10 mins: if still< 7.9 Sign of distress: </= 6 Severe asphyxia: 1-3 5 mins: prolonged distress in utero. some degree of damage. continue taking APGAR until >7 is REACHED!!! .

pale Body pink. crying Some flexion of Active motion extremities Grimace Cough or sneeze Completely pink Blue.APGAR EVALUATION OF NEWBORNS SIGN Heart Rate Respiratory Effort Muscle tone Response to catheter in nostril Color Absent Absent Limp No response 0 1 Below 100 Slow. extremities blue . irregular 2 Over 100 Good.

68. b. THESE FINDINGS ARE CONSISTENT WITH WHICH ELECTROLYTE ABNORMALITY? a. d. A PATIENT WAS BROUGHT TO THE ER DUE TO FLACCID PARALYSIS. WIDENED QRS AND PEAKED T WAVES. Hypercalcemia Hypocalcemiia Hypokalemia Hyperkalemia . THE PATIENT WAS HOOKED TO A CARDIAC MONITOR AND YOU NOTE THAT HE HAS PROLONGED PR INTERVAL. c. YOU SUSPECT THAT HE HAS AN ELECTROLYTE IMBALANCE.

flattening of the P wave and widened QRS complex  Can lead to ventricular fibrillation  Paresthesias.HYPERKALEMIA Most important effects are due to the role of K in membrane polarization  Peaked T waves . increased PR interval. weakness and even an ascending paralysis  Cardiac toxicity precedes all these symptoms  . ST segment depression. fasciculations.

HYPOKALEMIA Common in children with most cases related to gastroenteritis  Clinical Manifestations

Heart and skeletal muscles are vulnerable – ECG shows flattened T waves, depressed ST segment, and the appearance of a U wave  Muscle weakness and cramps  Paralysis if <2.5meq’L  Poluria and polydipsia

69. YOU DIAGNOSE AN 8-YEAR OLD BOY AS HAVING ADRENOLEUKOYSTROPHY WITH MILD CEREBRAL INVOLVEMENT. WHAT IS THE MOST EFFECTIVE THERAPY FOR HIM?
a.

b.
c. d.

Steroids Bone marrow transplantation Lorenzo’s oil Baclofen

ALD
 X-linked

 Accumulation

of unbranched saturated VLCFA  Treatment  Corticosteroid  BMT – benefits those with neurologic disability  Lorenzo’s oil  Baclofen- for spasms

70. A CHILD WITH UNEXPLAINED BONE PAIN, EASY BRUISABILITY, AND HEPATOMEGALY WAS SUSPECTED TO HAVE LIPID STORAGE DISEASE. ENZYME ASSAY OF CULTURED FIBROBLASTS SHOWED DEFECTS IN B-GLUCOSIDASE ACTIVITY. WHICH OF THE FOLLOWING DISEASES DOES HE HAVE?
a.

b.
c. d.

Gaucher disease Niemann-Pick disease Tay-sachs disease Sandhoff disease

LIPIDOSES Disorder Enzyme defect Gaucher B glucosidase Niemann-Pick Acid sphingomyelinase Tay-Sachs B hexoseaminidase Sandhoff B hexoseaminidase .

c.71. d. b. WHICH OF THE FOLLOWING IS TRUE ABOUT ANOREXIA NERVOSA/ a. Fear of not being able to stop eating Refusal to maintain body weight over a minimal weight for age and height Absence of at least 2 consecutive expected menstrual cycles Self evaluation is not influenced by weight and height .

ANOREXIA NERVOSA AND BULIMIA  Anorexia Intense fear of becoming obese. which does not diminish as weight loss progresses  Disturbance in the way in which one’s body weight. size or shape is experienced  Refusal to maintain BW over a minimal normal weight for age and height  Absence of at least 3 consecutive menstrual cycles   Bulimia      Recurrent episodes of binge eating Fear of not being able to stop eating Self induced vomiting Minimum average of 2 binge eating per week x 3 mos Self evaluation is unduly influenced by boy weight and shape .

SHE HAS a. Dysfunctional uterine bleeding Primary dysmenorrhea Secondary dysmenorrhea Premenstrual syndrome . c.72. d. b.A TEENAGE GIRL WITH ENDOMETRIOSIS IS COMPLAINING OF PAINFUL CRAMPS DURING MENSES.

endometriosis or endometritis .PAINFUL MENSTRUAL CRAMPS  Primary Dysmenorrhea  Absence of any specific pelvic pathologic condition  Most common  Prostaglandins F2 and E2  Secondary dysmenorrhea  Results from an underlying structural abnormality of the cervix or uterus. a foreign body .

c. d. Epinephrine Diphenhydramine Loratadine Cyproheptadine . DRUG OF CHOICE FOR ANAPHYLAXIS a. b.73.

laryngeal edema and urticaria and angioedema  Activation of mast cells and basophils via cell bound allergen specific IgE molecules  Treatment: IM or IV epinephrine  . pulmonary edema. visceral congestion.ANAPHYLAXIS A serious allergic reaction that is rapid in onset and may cause death  Pathologic features – acue pulmonary hyperinflation. intra alveolar hemorrhaging.

b. c. WHICH OF THE FOLLOWING IS RARELY TRANSMITTED PERCUTANEOUSLY? a. d. Hepatitis A Hepatitis B Hepatitis C Hepatitis D .74.

HBsAg HBeAg IgM anti-HBc Anti-HBc . c. WHICH OF THE FOLLOWING MARKERS TELLS YOU THAT YOUR PATIENT WITH HEPATITIS B IS HIGHLY INFECTIVE? a. b. d.75.

its rise coincide with appearance of symptoms  HBeAg – present during acute phase and indicates a highly infectious state  Anti-HBcAg IgM – rises early after infection  Most valuable single serolologic marker of acute HBV infection  Anti-HBc IgG – replaces IgM  Anti HBsAg – present among immunized persons  HBsAg .HEPATITIS B MARKERS – first marker to appear.

b. THE BEST WAY TO PREVENT DIARRHEA IN INFANCY IS a. Proper hygiene Immunization Boil water Breastfeed . d. c.76.

d. A 1 YEAR OLD CHILD WITH MEASLES MUST BE GIVEN VIT A.000 IU 100. WHICH IS THE CORRECT DOSE? a. b.000 IU 200.77. c. 50.000 IU 150.000 IU .

1yr : 100.VITAMIN A IN MEASLES Reduces morbidity and mortality 6 mos.000 IU .000 IU 1 yr older : 200.

WHICH OF THE FOLLOWING IS EXPECTED OF A 4 YEAR OLD? a. c. Tell a story Name 4 colors Draw a triangle Dress and undress . b. d.78.

GROSS MOTOR MILESTONES Mean Age (mos) 3 5 Milestones Head control Roll over 6 8 9 11 15 24 36 48 Sit with support Sit alone Pulls to stand holding on Stand independently Walk alone Climb up and down stairs Ride a trike Hop on one foot .

second word 4 5 6 Repeats 3-word sentence Speech 100% intelligible Uses past tense of eat. dada Immature jargon.sex 8 10 12 Says dada.EXPRESSIVE LANGUAGE MILESTONES Age Milestones (mos) 3 Vocalizes and coos 6 Babbles Age Milestones (yrs) 2 2-word phrases 3 3-word sentence Gives full name. go Gives word for definition . run.mama non-specifically First words other than mama.age.

Maternal viral illness Antibiotic treatment of a child Fever (temperature of 37. c. d. WHICH OF THE FOLLOWING IS A CONTRAINDICATION TO ROUTINE CHILHOOD IMMUNIZATION? a.8C) None of the above . b.79.

80. d. c. PULMONARY VASCULAR RESISTANCE FALLS RAPIDLY AT BIRTH DUE TO a. b. Rise in arterial pO2 Closure of the ductus arteriosus Increase in pulmonary blood flow Decrease in tortuosity of the pulmonary vasculature .

 Pulmonary resistance & pressure  L atrial pressure blood from R ventricle enter the pulmonary circulation  O2 content physiologic closure of foramen ovale muscular constriction and functional closure of patent ductus arteriosus .

THE MOST LIKELY DIAGNOSIS IS: a. AN 18-YEAR OLD GIRL WITH PRIMARY AMENORRHEA. d. Marfan syndrome Down syndrome Palau syndrome Turner syndrome . WEBBED NECK AND COARCTATION OF THE AORTA CONSULTS AT YOUR CLINIC.81. SHORT STATURE. b. c.

TURNER SYNDROME Incidence: 1 /4000  Complete or partial absence of the x chromosome  45x  Findings  Phenotypically female  Short stature  Underdeveloped gonads  Webbed neck  .

d.82. c. b. Papilledema Gaping wound at L2-L5 area Pending cerebral herniation Platelet count of 100 x 109 /L . NOT A CONTRAINDICATION TO LUMBAR TAP a.

c. Fresh frozen plasma Factor IX concentrate Cryoprecipitate Platelet concentrate . d. WHICH OF THE FOLLOWING WILL YOU TRANSFUSE a. b. A PATIENT WITH HEMOPHILIA B IS BLEEDING PROFUSELY.83.

PT. BT. thrombin time  Hallmark: hemarthrosis   Von Willebrand Disease    Most common hereditary bleeding disorder Sx of mucocutaneous bleeding Inc BT. normal PC  Liver Disease Clotting factors produced in the liver except VIII  Treatment consists of replacement with FFP or cryoprecipitate  . normal PC. PTT.HEMORRHAGIC AND THROMBOTIC DISEASES  Hereditary Clotting Factor Deficiencies Factor VIII or IX (Hemophilia A or B)  Inc APTT.

b. c.84. THE PATENT FORAMEN OVALE FUNCTIONALLY CLOSES AT: a. d. 10-15 hours of life 24 hours of life 1 month of life 3 months of life .

CLOSURE OF SHUNTS  Foramen ovale  Functionally closed by the 3rd month  Dustus arteriosus  Functional closure by the 10-15th HOL .

Abdomen Extremity Head and neck Chest . d. c. b. CYSTIC HYGROMA OCCURS MOST FREQUENTLY IN THE a.85.

CYSTIC HYGROMAS AND LYMPHANGIOMAS Arise in the embryonic lymph sac and are the 2nd most common benign vascular tumors in children (1st – hemangiomas)  Half – located in the head and neck area  50% are present at birth. with most presenting by 2 y/o  .

Phenobarbital Phenytoin Carbamazepine None of the above . WHICH IS THE DRUG OF CHOICE FOR PROPHYLACTIC TREATMENT OF FEBRILE SEIZURES? a. c. b.86. d.

repeated convulsions. EEG.FEBRILE SEIZURES         6 mos – 60 mos Rare before 9 mos and after 5 years old Rapid increase in temperature (>39C) r/o CNS infection or metabolic causes Usually generalized. focal findings) LP. imaging procedures Anticonvulsants and Diazepam? . tonic-clonic and lasts for a few seconds Simple vs complex (>15 min.

WHICH OF THE FOLLOWING IS ASSOCIATED WITH GASTRITIS AND PEPTIC ULCER DISEASE? a.87. b. c. Escherichia coli Mycoplasma pneumoniae Helicobacter pylori Enterobius vermicularis . d.

Acute blood loss of 15% of total blood volume Hemoglobin of 11 mg/dl preoperatively Hemoglobin of 9 mg/dl but asymptomatic Hemoglobin of 10 mg/dl in congestive heart failure . c. WHICH OF THE FOLLOWING IS AN INDICATION FOR PACKED RBC TRANSFUSION IN CHILDREN? a. d. b.88.

PEDIATRIC RBC TRANSFUSIONS  Acute loss > 25% of circulating blood volume  Hgb < 8 g/dl in perioperative period  Hgb <13 g/dl and severe cardiopulmonary disease  Hgb <8 g/dl and symptomatic chronic anemia  Hgb < 8 g/dl and marrow failure .

WHICH OF THE FOLLOWING VALUES TELLS YOU THAT A CHILD HAS SEVERE MALNUTRITION BASED ON WATERLOWE CLASSIFICATION? a. c.89. 60% 70% 80% 90% . d. b.

c. d. ANTIDOTE FOR IRON TOXICITY a. b. N-acetylcysteine Deferoxamine Naloxone EDTA .91.

urinary excretion.POISONING  Acetaminophen Acute toxic dose: 200mg/k  Tx: N-acetylcysteine (NAC)   Salicylates 150mg/k  Gastric decontamination. hydration. Dialysis   Iron >60 mg/k of elemental iron  Deferoxamine  .

WHICH OF THE FOLLOWING IS A DIAGNOSTIC ABDOMINAL X-RAY FINDING OF NECROTIZING ENTEROCOLITIS? a. d.92. Air fluid levels in the bowels Dilated bowel loops Pneumatosis intestinalis Dilated proximal bowel with constriction distally . b. c.

most common life threatening emergency of the GI tract in the newborn period  Pneumatosis intestinalis  Triad: abdominal distention.NECROTIZING ENTEROCOLITIS Necrotizing Enterocolitis. bloody stools  Tx: no definitive tx for established NEC. only supportive care and preventing further injury  If tx is needed: surgery and Metronidazole . gastric retention.

93. A CHILD WITH ACRODERMATITIS ENTEROPATHICA, POOR WOUND HEALING AND GROWTH RETARDATION HAS:
a.

b.
c. d.

Iron deficiency Selenium deficiency Taurine deficiency Zinc deficiency

ZINC

Acrodermatitis enteropathica – rare autosomal disorder caused by an inability to absorb sufficient zinc from the diet

94. CONTRAINDICATION TO MMR VACCINATION
a. b. c.

d.

Breastfeeding Pregnancy Tuberculosis Diarrhea

95. PEUTZ-JEGHERS SYNDROME PRESENTS AS POLYPOSIS OF THE SMALL BOWELS AND WHICH OF THE FOLLOWING?
a. b. c.

d.

Nasal polyposis Hemangioma Hyperpigmented macules on the face lips, and buccal mucosa Periungual desquamation

PEUTZ-JEGHERS SYNDROME
Characterized by melanotic papules on the lips and mucous membranes and by gastrointestinal polyposis  Autosomal dominant  Polyposis usually involves the jejunum and ileum but may also involve the stomach, duodenum, colon and rectum

96. AN IMPORTANT REFLEX TO FACILITATE BREASTFEEDING
a. b. c.

d.

Moro reflex Palmar grasp reflex Rooting reflex Parachute reflex

NUTRITION  Breastfeeding vs Formula feeding  Advantages  Techniques  Rooting reflex  Sucking reflex: prolactin (AP) – milk secretion oxytocin (PP) .contraction  Swallowing reflex  Contraindications  Septicemia  HIV  Active TB  Typhoid fever  Breast CA  Malaria  Substance abuse and severe neuroses or psychoses .

c.97. d. 2 years 3 years 4 years 5 years . AT WHAT AGE CAN THE SNELLEN CHART BE USED TO ASSESS VISUAL ACUITY a. b.

IN WHAT MEIUM SHOULD THE TOOTH BE TRANSPORTED? a. HE WANTS IT REPLANTED. c.98. d. b. A 15 YEAR OLD HAD HIS UPPER CENTRAL INCISOR KNOCKED OUT OFF THE SOCKET. Cow’s milk Tap water Normal saline Water with sugar .

1999. 1998) in terms of PDL cell viability. Milk has an osmolality within physiologic limits. A viable periodontal ligament (PDL) is important for proper physiologic healing of replanted teeth. One of the critical factors affecting outcome is the way the tooth is handled during the extraoral period. Lekic et al. A suitable storage medium should have a physiologic osmolality in order to keep the PDL cells in as healthy a condition as possible. although a new storage medium: Propolis may be better (Martin and Pileggi 2004) . Milk appears to be the storage medium of choice in an emergency situation. In vitro studies suggest that it is superior to saliva (Blomlof and Otteskog 1980) and to Eagle’s medium (Blomlof 1981) and other media (Ashkenazi et al.

d. WHICH OF THE FOLLOWING SLEEPING POSITIONS HAS BEEN NOTED TO DECREASE THE INCIDENCE OF SUDDEN INFANT DEATH SYNDROME? a. Supine Prone Side Any of the above . c. b.99.

SIDS  Sudden death of an infant that is unexpected by history and unexplained by thorough post mortem examination. which includes autopsy. and review of the medical history  Environmental and genetic risk factors  Infant sleep environment: sleeping prone has consistently shown to increase the risk of SIDS  Recommendation: supine position . investigation of the scene of death.

Give large amount of milk Induce emesis Gastric lavage Rinse the mucosa . b. A CHILD ACCIDENTALLY INGESTED A BLEACHING SOLUTION (ZONROX). c.100. d. WHICH OF THE FOLLOWING SHOULD BE THE INITIAL MANAGEMENT? a.

lacrimation. gastrointestinal cramps. urination. emesis)  Antidotes: atropine and pralidoxime  . defacation.POISONING  Caustics     Acids and Alkali Thorough removal Emesis and lavage are contraindicated Activated charcoal should not be used  Insecticides SLUDGE ( salivation.

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