PEDIATRICS BOARD REVIEWEDWARD 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 SHE WAS TACHYPNEIC. b. A 6-MONTH OLD BABY WAS BROUGHT TO THE ER BECAUSE OF DOB.2. d. CXR SHOWED CONGESTION AND CARDIOMEGALY LV FORM. ABG SHOWED PO2 OF 175 OFF OXYGEN.. c. ON PE. THE MOST LIKELY CARDIAC LESION IS: a. Pulmonary stenosis Atrial septal defect Ventricular septal defect Transposition of the great arteries . WITH RALES AND NOTE OF A GR 3/6 HOLOSYSTOLIC MURMUR ON THE LLSB. 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) . etc) Cyanotic Decreased pulmonary blood flow Obstruction and a shunt (ex. PDA. VSD. AV septal defects) Increased pressure load Normal blood flow is obstructed (pulmonic stenosis. 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.Ray EKG (15 lead) Others: 2D Echo Cardiac cath . Is the patient cyanotic or acyanotic? 2. Does the malformation originate in the left or right side of the heart? 4. Is pulmonary hypertension present or not ? HISTORY PHYSICAL EXAM CHEST X. Is the pulmonary arterial flow increased or not ? 3.Tools in the Assessment of Heart Disease FIVE BASIC QUESTIONS 1. 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 . 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 LR shunt Restrictive VSDs= < 0. shunt is LR Non restrictive VSDs >1.5cm2.0 cm2 RV and LV pressure is equalized . RV pressure is N. 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 smallasymptomatic 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 . 3. EXCEPT: a. b. c. THE FOLLOWING ARE ANTI- HEART FAILURE MEDICATIONS. Dopamine Furosemide Phenytoin Digoxin . d. captopril. nitroprusside) . nitroglycerine.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. enalapril. A 10 YEAR OLD WENT TO YOU FOR CONSULT DUE TO DIFFICULTY IN WALKING. c. b. YOU SUSPECT: a. Juvenile Rheumatoid Arthritis Takayasu Arteritis Panarteritis nodosa Systemic lupus erythematosus . ON PE.4. d. YOU NOTE THAT HE IS ALMOST PULSELESS. pulseless disease PAN.small to medium sized arteries. Takayasu arteritis.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 . PAN Takayasu – involves large arteries. 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.8. b. d. A PX COMES IN WITH TRISMUS. DIFFICULTY OF SWALLOWING AND “HOT POTATO” VOICE. c. Retropharyngeal abscess Croup Peritonsillar abscess Epiglottitis . adolescent Sore throat. fever. trismus. irritability.NECK ABSCESSES Retropharyngeal and Lateral pharyngeal abscess Common in young children 3-4 years old or younger Fever . dysphagia Asymmetric tonsillar bulge with displaced uvula “hot potato” voice Peritonsillar cellulitis/abscess . decreased oral intake and drooling Bulging posterior pharyngeal wall polymicrobial Relatively common Older child. life-threatening. high fever. non toxic.CROUP VS EPIGLOTITIS Croup – viral (parainfluenza). “steeple sign” Epiglotitis. sitting forward. acute onset. critical airway. drooling. low grade fever. responds to racemic epinephrine and dexamethasone . no distress.. toxic child. “thumb sign” .bacterial (H influenza). 9. Pneumonia Spontaneous pneumothorax Lung tumor Atelectasis . A LANKY TEENAGER WENT TO THE ER DUE TO SUDDEN ONSET DYSPNEA. c. d. THE MOST LIKELY DIAGNOSIS IS a. HE HAS RETRACTIONS AND DECREASED BREATH SOUNDS OVER THE INVOLVED AREA. AND ON CXR THERE IS A MEDIASTINAL SHIFT TOWARDS THE UNINVOLVED SIDE. b. iodopovidone) .PNEUMOTHORAX Accumulation of extrapulmonary air within the chest Primary vs secondary Spontaneous. 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. traumatic. Rapidly progressive respiratory obstruction Etiologic agent: Haemophilus influenza b A medical emergency All of the above . TRUE ABOUT EPIGLOTITTIS a. d.10. b. c. toxic child acute onset sitting forward critical airway “thumb sign” .EPIGLOTITIS bacterial (H influenza) life-threatening high fever. drooling. 11. d. c. Diphtheria Pertussis Tetanus Polio . WITH TONSILLAR MEMBRANE AND “ BULL NECK” APPEARANCE? a. WHICH OF THE FOLLOWING INFECTIONS PRESENT AS PHARYNGEAL INJECTION. b. 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) . Penicillin Ceftriaxone Aspirin Chloramphenicol . GOLD STANDARD IN THE TREATMENT OF UNCOMPLICATED SALMONELLOSIS a. b.12. d. c. malnourished. rose spots Dx: bone marrow culture Tx: Chloramphenicol remains the gold standard . extraintestinal focal infections Tx: antibiotics for bacteremia and extra intestinal infections (ceftriaxone) For acute enteritis: no antibiotics. Paratyphi A. immunocompromised Enteric or typhoid fever S. only for infants <3 mos. bacteremia. Paratyphi C Relative bradycardia.SALMONELLOSIS Nontyphoidal salmonellosis Acute enteritis. Ser Typhi. Paratyphi B. c. d. b. 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. clinical findings suggestive of TB.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. those with underlying medical conditions. 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.POSITIVE PPD INDURATION > 5 MM Close contacts of TB pxs. 14. Rifampicin Pyrazinamide Streptomycin Ethambutol . d. WHICH OF THE FOLLOWING ANTITUBERCULOSIS MEDICATIONS IS CONTRAINDICATED FOR CHILDREN BELOW 7 YEARS OLD? a. c. b. 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) . cytological. biochemical. loss of weight. 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.Signs and Symptoms Cough/ wheezing of 2 weeks or more Unexplained fever of 2 weeks or more Loss of appetite. reduced playfullness. or lethargy . clinical findings suggestive of TB. >10mm is considered positive . cxr suggestive of TB and immunocompromised condition Otherwise.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. TB adenitis.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. CNS TB. etc Extrapulmonary/Extrathoracic . 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. reduce morbidity and the transmission of infection. 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 .ANTI TB MEDS Isoniazid Peripheral neuritis and hepatotoxicity Orange discoloration of urine and tears. FACE AND PROXIMAL EXTREMITIES? a. RAISED . d. NON-PRURITIC RASH ON THE TRUNK WHICH SPREADS TO THE NECK. WHICH OF THE FOLLOWING VIRAL INFECTIONS PRESENTS WITH HIGH FEVER WHICH ABRUPTLY LYSES AND DISCRETE PINK. c. b.15. Rubella Roseola Rubeola Mumps . 3rd rubella.ROSEOLA INFANTUM Exanthem subitum or 6th disease Recall: 1st – measles. 4th atypical scarlet( Filatov-Duke’s). 2nd scarlet. 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 . c. Anopheles mosquito Culex mosquito Aedes aegypti mosquito Aedes africanus . b. WHICH IS THE VECTOR OF DENGUE FEVER? a.16. d. Aedes aegypti Flight range: < 25 m in open. 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. Malariae Falcifarum Vivax Ovale . c. b. PRESENTING WITH INTENSE PARASITISM a.17. MOST SEVERE FORM OF MALARIA. d. Malariae – every other or third day fever spikes Periodicity of fever.MALARIA P. Ovale – daily fever spikes P. chloroquine. Vivax and P.quinine Blackwater fever. malariae is the mildest and most chronic P. algid malaria . for chloroquine resistance.less apparent in falciparum Tx. Vivax – less severe. falcifarum is the most severe form and is associated with more intense parasitemia P. ruptured spleen P. 18. b. VIVAX AND P. Quinine Chloroquine Primaquine Pyrimethamine –sulfadoxime . DRUG OF CHOICE FOR PREVENTION OF RELAPSES OF P. d. c. OVALE INFECTION a. 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 . Vivax. d. Enterobiasis Trichuriasis Hookworms Diphyllobotriasis . WHICH OF THE FOLLOWING INFESTATIONS IS NOT DETECTED BY DIRECT FECAL SMEAR? a. b.19. c. diphyllobotriasis Microscopic exam of cellophane tape enterobius Demonstration in the blood . trichuriasis. hookworms.filariasis Stool exam by Kato thick smear schistosomiasis . strongyloides.HELMINTHIC DISEASES Direct fecal exam Ascaris. b. c. WHICH OF THE FOLLOWING VACCINES IS PART OF THE EPI? a.20. d. Tetanus toxoid Varicella vaccine Rotavirus vaccine Typhoid vaccine . EXPANDED PROGRAM ON IMMUNIZATION BCG HEPATITIS B DPT POLIO MEASLES RECENT INCLUSIONS: HiB. ROTAVIRUS . MMR. b. c.21. d. WHAT IS THE MOST COMMON HEMATOLOGIC DISEASE OF INFANCY AND CHILDHOOD? a. Acute leukemia Iron deficiency anemia Idiopathic thrombocytopenic purpura Hereditary spherocytosis . polyp. 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. or inflammatory bowel disease Hookworm infestation .IRON DEFICIENCY ANEMIA Most common hematologic disease of infancy and childhood Newborn – contains 0. Meckel.5g of iron Adult – 5g Iron stores in term infants are sufficient for blood formation in the first 6-9 months Therefore. hemangioma. HEMOGLOBIN DECLINES AT ____ WEEKS FOR TERM INFANTS a. 3-6 10-12 6-8 2-4 . b.22. IN PHYSIOLOGIC ANEMIA OF INFANCY. c. d. PHYSIOLOGIC ANEMIA OF INFANCY Newborns have higher Hgb and Hct Within the 1st week. 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. Hgb 7-9) . d. WHICH OF THE FOLLOWING PRODUCES PERIPHERAL PANCYTOPENIA WITH HYPOPLASTIC MARROW? a. c. b.23. Acute leukemia Iron deficiency anemia Idiopathic thrombocytopenic purpura Aplastic anemia . 000 and retic count <1% .000/mm3.Aplastic Anemia Hallmark is peripheral pancytopenia. coupled with hypoplastic or aplastic bone marrow Severe aplastic anemia – 2 or more cell components have become seriously compromised (ANC < 500/mm3. retic count <1% after correction for hematocrit) Moderate aplastic anemia – ANC 500 -1.500/mm3. PC 20.000-100. PC 20.000 -100. Wilm’s tumor Hepatoblastoma Neuroblastoma Nephroblastoma .24. MOST COMMON EXTRACRANIAL TUMOR OF CHILDHOOD. d. c. b. MOST FREQUENTLY DIAGNOSED DURING INFANCY. AND MOSTLY ARISING FROM THE ABDOMEN a. 2nd most common malignant abdominal tumor in childhood 3 elements: blastema. Denys-Drash. aniridia. epithelia and stroma Associated with hemihypertrophy. Beckwith-Wiedemann) Occurs in children between 2-5 years old CT: no calcification Wilm’s Tumor . and other congenital anomalies (WAGR.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. 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. 25. d. b. Osteosarcoma Ewing’s sarcoma Rhabdomyosarcoma Enchondroma . X-RAY SHOWED SCLEROTIC DESTRUCTION OF THE PROXIMAL HUMERUS WITH SUNBURST PATTERN. A 15-YEAR OLD CONSULTED FOR ARM SWELLING AND PAIN. c. THE MOST LIKELY DIAGNOSIS IS a. Paget’s Metaphyses of long bones Local pain & swelling Sunburst pattern Lungs.5:1 Small round cell None Diaphyses.CANCER AND BENIGN TUMORS Bone Tumors Osteosarcoma Second decade All races 1.5:1 Spindle cell Retinoblastoma. 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. 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 . XI Factors II. d. c. VIII. X . VII. IX Factors V. VII. IX. VIII. DEFICIENCY IN WHICH OF THE FOLLOWING FACTORS WILL YOU NEED TO GIVE VITAMIN K? a. V. IX. b. Factors II. IX. X.26. X Factors II. . 27. Congenital varicella syndrome Neonatal herpes syndrome Congenital syphillis Congenital rubella syndrome . c. PULMONARY STENOSIS AND HEARING LOSS. YOU SUSPECT THAT THE BABY HAS a. CATARACTS. d. A NEWBORN PRESENTED WITH “BLUEBERRY MUFFIN” RASH. b. late –Hutchinsons teeth. saber shins. deafness. heart defects. blueberry muffin lesions (same as in CMV) Neonatal herpes .CONGENITAL INFECTIONS Congenital Syphilis Early and late signs Early – snuffles. olympian brow Congenital varicella – malformations of the extremities with cicatricial scarring Congenital rubella – cataracts. THE RECTAL VAULT WAS EMPTY BUT A GUSH OF FOUL-SMELLING GAS WAS NOTED ON RECTAL EXAM. SHORT OF A BIOPSY. HE HAD DELAYED PASSAGE OF MECONIUM AND CHRONIC CONSTIPATION. A BABY WAS BROUGHT TO YOUR CLINIC BECAUSE OF FAILURE TO THRIVE. d. BARIUM ENEMA SHOWED A DILATED PROXIMAL COLON AND SMALL CALIBER DISTAL COLON.28. Intussusception Congenital aganglionic megacolon Hypertrophic pyloric stenosis Gastric volvulus . YOUR DIAGNOSIS IS a. c. HE HAS ABOMINAL DISTENTION AND A PALPABLE MASS ON THE LLQ. b. 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. 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). 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 . 29. PRECOCIOUS PUBERTY IS DEFINED AS ONSET OF PUBERTY BEFORE AGE 8 IN GIRLS AND __ IN BOYS a. c. d. b. 8 9 10 11 . 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. McCune –Albright) Gonadotropin independent Incomplete . Oxytocin Prolactin Leutinizing hormone Follicle stimulationg hormone . d. c.30. b. WHICH OF THE FOLLOWING HORMONES IS NOT FOUND IN THE ANTERIOR PITUITARY a. LH. ACTH. TSH.PITUITARY HORMONES Anterior Pituitary GH. POMC Posterior Pituitary Vasopressin and oxytocin . FSH. prolactin. YOU DO: a. YOU SUSPECT DIABETES INSIPIDUS. HE HAS BEEN POLYURIC AND POLYDIPSIC FOR QUITE SOME TIME. c. d. TO DIFFERENTIATE BETWEEN CENTRAL AND NEPHROGENIC ORIGIN. HE HAS LOW URINE AND SERUM OSMOLALITY. A 12 YEAR OLD MALE WAS BROUGHT IN FOR DEHYDRATION. AND HYPERNATREMIA.31. Insulin challenge test DDAVP test Erythropoietin test Beta agonist challenge . b. DIABETES INSIPIDUS Polyuria. congenital malformations of the hypothalamus or pituitary. neoplasms. inc vasopressin metabolism Nephrogenic DI Vasopressin insensitive Genetic (less common but more severe) or acquired causes . trauma to vasopressin neurons. infections. autoimmune diseases. hypernatremia Serum osmolality >300 mOsm/kg and urine osmolality <300 mOsm/kg Central vs nephrogenic Central DI Genetic mutations to the vasopressin gene. polydipsia (exceeding 2L/m2/24hr). b. THE PX IS EXPERIENCING a. A KNOWN INSULIN DEPENDENT DIABETIC COMES TO YOUR CLINIC AND COMPLAINS OF EARLY MORNING HYPERGLYCEMIA. c. Diabetic ketoacidosis Insulin shock Somogyi phenomenon Dawn phenomenon . WHILE THE 7 AM IS MARKEDLY HIGH. YOU SUSPECT BRITTLE DIABETES.32. THE 3 AND 4 AM LEVELS ARE BELOW 60 MG/DL. AND 7 AM GLUCOSE CONCENTRATION. 4. YOU MEASURE HIS 3. d. 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 . DOES NOT FEED WELL AND IS CONSTIPATED. YOU NOTE THAT SHE IS JAUNDICED WITH A GLAZED LOOK. WHICH OF THE FOLLOWING LABORATORY EXAMS WILL YOU PRIORITIZE TO HELP YOU WITH YOUR DIAGNOSIS? a. AND MOTTLED SKIN. d. Ca . UMBILICAL HERNIA. SHE TELLS YOU THAT THE BABY IS SLUGGISH. Chest x-ray Lumbar tap Serum T3.33. ENLARGED ABDOMEN. b. K. T4. c. TSH Serum Na. A NEIGHBOR SHOWS YOU HER 3-WEEK OLD BABY FOR CONSULT. 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. umbilical hernia Low serum T4 and elevated TSH levels Tx: levothyroxine .CONGENITAL HYPOTHYROIDISM Most common cause is thyroid dysgenesis (8085%). constipation. somnolence. lack of interest. large abdomen. 15% . sluggishness. choking spells during nursing. 34. AND ASKS IF THERE IS SOMETHING WRONG WITH HER. WHAT WILL YOU DO? a. 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 . SHE IS VERY TALL (90TH PERCENTILE). b. HER PARENTS AND SIBLINGS ARE ALL TALL. c. AND SHE IS IN EXCELLENT HEALTH. YOUR ADOLESCENT NIECE TALKS TO YOU ABOUT HER HEIGHT. d. SHE HAS BEEN VOMITING AND FEEDING POORLY. YOU THEN ASK FOR AMINOACID ASSAY. High leucine.35. HER LAB EXAMS ARE UNREMARKABLE EXCEPT FOR SEVERE METABOLIC ACIDOSIS. A WEEK OLD BABY WAS BROUGHT TO THE ER BECAUSE OF WEAKNESS. isovaleryglycerine and hydroxyvaleric acid levels High methylmalonyl acid levels High glutamine and alanine levels . YOU TELL THE ATTENDING PHYSICIAN THAT WHILE DOING YOUR PE. isoleucine and valine levels and low alanine levels High isovaleric acid. LIKE MAPLE SYRUP. b. AND EXPECT THE FOLLOWING RESULT: a. c. YOU NOTED THAT HER URINE SMELLED SWEET. d. Acrid Swimming pool Sweaty feet.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. 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 . Penicillin b. SHE WAS BORN AT HOME AND A SHARPENED BAMBOO STICK WAS USED TO CUT THE CORD. YOUR DIAGNOSIS IS NEONATAL TETANUS. Ceftriaxone d. Metronidazole c. A NEWBORN WAS BROUGHT TO THE ER FOR SEIZURES.36. THE MOTHER DID NOT HAVE PRENATAL CHECK-UP AND HER IMMUNIZATION STATUS WAS UNKNOWN. THE DRUG OF CHOICE IS a. Chloramphenicol . alternatives: metronidazole. Tetracycline Surgery . associated hunger and crying In older children. sardonic smile ( intractable spasms of the facial and buccal muscles) Treatment Human Tetanus Ig Penicillin G – antibiotic of choice.trismus (masseter muscle spasm or lockjaw).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. A TEENAGER WAS BROUGHT TO THE ER FOR RESPIRATORY DISTRESS. THEN WEAKNESS OF THE UPPER TRUNK AND EXTREMITIES AND FINALLY FACIAL WEAKNESS. PE SHOWS COMPLETE PARALYSIS WITH ABSENT TENDON REFLEXES. THE LIKELY DX IS a. HE WAS IMMEDIATELY INTUBATED. THE HX IS UNREMARKABLE EXCEPT FOR A NON SPECIFIC VIRAL ILLNESS 2 WEEKS AGO. c. Bell palsy Transverse myelitis Spinal muscular atrophy Guillain-Barre Syndrome .37. d. b. THE ILLNESS STARTED AS DIFFICULTY IN WALKING. 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.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. c. b.38. d. Medulloblastoma Brainstem glioma Cerebellar astrocytoma ependymoma . MOST COMMON INFRATENTORIAL TUMOR IN CHILDREN a. 2%) Supratentorial (40.CANCER AND BENIGN TUMORS Brain Tumors in Children Categories Juvenile pilocytic astrocytoma – MOST COMMON Medulloblastoma Diffuse astrocytoma Ependymoma craniopharyngioma Location Infratentorial (43.9%) Multiple sites (11%) Important! Within the 1st year –supratentorial 1-10 yr – infratentorial Cranial exposure to ionizing radiation – inc incidence .9%) Spinal cord (4. WBC 10/MM2. TB meningitis Bacterial meningitis Normal CSF Viral meningitis . LUMBAR TAP WAS DONE AS PART OF THE WORK-UP. PROTEIN 10 MG/DL.39. d. b. A NEWBORN IS SUSPECTED TO HAVE SEPSIS. c. GLUCOSE 60% OF SERUM GLUCOSE. CSF STUDIES SHOWED RBC=0. THE PATIENT HAS: a. glucose 60% . wbc up to 5 (for NB up to15). protein 10-40 mg/dl (120 in a neonate).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. no rbcs. CRANIAL CT SCAN OF A CHILD WITH MULTIPLE CONGENITAL ANOMALIES SHOWED ABSENCE OF CONVOLUTIONS. c.40. Porencephaly Lissencephaly Schizencephaly Holoprosencephaly . d. b. THIS IS KNOWN AS a. 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 . AND ACTS AS IF NOTHING HAPPENED IN THE MORNING. Night terror Rage attack Choreoathetosis Nightmares . THE DIAGNOSIS IS a. YOUR FRIEND TELLS YOU THAT HIS CHILD WAKES UP AROUND MIDNIGHT SCREAMING AND HYPERVENTILATING. c. THE CHILD GOES BACK TO SLEEP IN A FEW MINUTES.41. b. d. NIGHT TERRORS VS NIGHTMARES Nightmares Common and usually involve vivid. scary or exciting events which the child can recall upon waking up Night terrors Less common Last 10-15 minutes. during which the child is not easily aroused and appears frightened or agitated No recollection of the event . THE INTERDIGITAL SPACES AND FLEXOR AREAS. Lindane Topical corticosteroid Selsun blue Tretinoin . b. CRUSTING. THERE ARE EXCORIATED. AND SCALING PAPULES ON THE TRUNK. PARTICULARLY AT NIGHT.42. c. d. PARTICULARLY THE WAIST AREA. THE TREATMENT OF CHOICE IS a. A CHILD COMPLAINS OF INTENSE PRURITUS OF THE SKIN. 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.classic lessions Treatment: Permethrin 5% Additional therapies: Lindane 1% lotion or cream and oral ivermectin . d. A NEWBORN’S WEIGHT GAIN IS APPROXIMATELY __ GMS/MONTH AT AGE 2-6 MONTHS a. 300 400 500 600 . c.43. b. 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 . b.44. d. Upper central incisors Lower central incisors Upper lateral incisors cuspids . THE FIRST PRIMARY TEETH TO ERUPT ARE a. c. 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 . HANDEDNESS IS USUALLY ESTABLISHED AT AGE ___ YEARS a. b. c. 2 3 4 5 . d.45. egocentrism and thinking that is dominated by perception. bedwetting up to age 4 in girls and 5 years in boys Piaget’s preoperational stage: magical thinking. 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. 46. Voice change Pubic hair Growth spurt Testicular enlargement . c. THE FIRST VISIBLE SIGN OF PUBERTY IN MALES IS a. b. d. ADOLESCENCE 10-20 years old Early. and psychological and social functioning In boys: testicular enlargement In girls: appearance of breast buds . middle and late adolescence Rapid changes in body size. shape. physiology. 47. THE CHILD HAS REPETITIVE BEHAVIOR. c. d. b. Asperger disorder Autism Schizophrenia Attention deficit disorder . ALONE. HE IS SUSPECTED TO HAVE a. NO EYE CONTACT. A 3-YEAR OLD WAS BROUGHT TO YOU FOR EVALUATION OF DELAYED SPEECH. PERVASIVE DEVELOPMENTAL DISORDERS Autistic Disorder Asperger Disorder Childhood Disintegrative Disorder Rett Disorder Childhood Schizophrenia . limited joint attention or orienting to one’s name. in imaginative activity and in reciprocal social interactions Males>females Poor eye contact. little symbolic play.Autism Develops before 36 months and is typically diagnosable at 18 mos of age Qualitative impairment in verbal and non verbal communication. repetitive behaviors. and reliance on non verbal communication with delay in use of words . checklist for autism in toddlers Intensive behavioral therapy before 3 yrs old targeted toward speech and language development Educational programming.Autism CHAT. behavior modification Pharmacotherapy: SSRIs. 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.48. c. d. b. Pull to stand Sit without support Walk alone Grasp object with thumb and forefinger . 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 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 .FINE MOTOR MILESTONES Mean Age (mos) 3.5 4 4 5. 49. AND PHOTOPHOBIA ARE CAUSED BY DEFICIENCY IN WHICH VITAMIN? a. A B C D . XEROPHTHALMIA. NYCTALOPIA. d. b. c. anorexia. xerophthalmia. seborrhea Pyridoxine: irritability.VITAMIN DEFICIENCIES Vitamin A: photophobia. constipation Riboflavin Niacin: pellagra Folacin: megaloblastic anemia Cyanocobalamin: pernicious anemia Biotin: dermatitis. fatigue. blindness Vitamin B: beriberi. convulsions Vitamin C: scurvy and poor wound healing Vitamin D: rickets Vitamin E: red blood cell hemolysis Vitamin K: hemorrhage . WHICH OF THE FOLLOWING MINERAL WILL CAUSE MOTTLING OF THE TEETH WHEN TAKEN IN EXCESS? a.50. b. c. Calcium Iron Magnesium Fluoride . d. 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. MUSCLE ATROPHY. SLIGHTLY DISTENDED ABDOMEN. Kwashiorkor Marasmus Pellagra Rickets . THE DIAGNOSIS IS a. b. HE HAS WRINKLED LOOSE SKIN. c.51. HIS FACE LOOKS WIZENED DUE TO LACK OF SUBCUTANEOUS FAT. AN EMACIATED CHILD WAS BROUGHT TO THE ER FOR MEDICAL MANAGEMENT. d. HIS EXTREMITIES ARE COOL. AND TEMP IS SUBNORMAL. psychomotor changes. moonface. flag sign. flaky dermatosis. anemia.150 cal/kg/d and 4g/kg/d of protein . winged scapula Treatment of PEM: 3 phases First : stabilization. correction of dehydration. obvious muscle wasting. potbelly. diarrhea Marasmus: gross loss of subcutaneous fat. antibiotics Second: Diet providing maintenance requirements of protein and energy Third: Recovery diet.PROTEIN/ENERGY MALNUTRITION (PEM) Edematous (Kwashiorkor) and Nonedematous (Marasmus) PEM Edematous: edema. SIMIAN CREASE. COMPLETE ATRIO-VENTRICULAR SEPTAL DEFECT. A CHILD WITH HYPOTONIA.52. b. EPICANTHAL FOLD. SLANTED PALPEBRAL FISSURES. d. c. AND SHORT BROAD HANDS HAS a. 7q23 deletion Trisomy 18 Trisomy 21 22q11 deletion . 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.53. 39 38 37 36 . d. c. PREMATURE BIRTH IS DEFINED AS LIVE BIRTH BEFORE __ WEEKS FROM THE FIRST DAY OF THE LAST MENSTRUAL PERIOD: a. 000 2. c. 1. VERY LOW BIRTHWEIGHT INFANTS WEIGH LESS THAN ____ GMS a.000 1. d.500 2.500 . b.54. early onset neonatal infections Neonatal Deaths .associated with preterm birth and low birth weight and lethal congenital anomalies Low Birth Weight LBW : 2500g or less VLBW: 1500g or less . asphyxia. maternal and fetal factors IUGR.influenced by prenatal. Perinatal Mortality . placental insufficiency. severe congenital malformations. DIFFERENTIAL DIAGNOSES FOR JAUNDICE IN THE FIRST 24 HOURS OF LIFE. EXCEPT: a. Physiologic jaundice ABO incompatibility Sepsis Hemorrhage . c. b. d.55. 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. 14 days (preterm) symptomatic .5 mg/dl/hr >8 days (term). fever Phase 3: hypertonia Chronic 1st year : hypotonia. retrocolis. opisthotonos. SNHL. seizures Phase 2: hypertonia of extensor muscles. upward gaze .KERNICTERUS Bilirubin encephalopathy resulting from the deposition of unconjugated (indirect) bilirubin in the basal ganglia and brainstem nuclei > 25mg/dl. hypotonia. active deep tendon reflexes After 1st year: movement disorders. range: 21-50 Clinical features Acute Phase 1: poor suck. 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 .KERNICTERUS Treatment of Hyperbilirubinemia Phototherapy Photoisomerization Complication. rash. dehydration. THE MOTHER CANNOT RECALL ANY PRIOR ILLNESS.56. THE CHILD CONFESSES THAT HIS URINE IS COLA COLORED. ON PE. YOUR PRIMARY DIAGNOSIS IS: a. A CHILD WAS BROUGHT TO THE ER DUE TO EDEMA. AND HIS BP IS HIGH. b. d. IT STARTED ON THE FEET PROGRESSING TOWARDS THE THIGHT. YOU NOTICE SEVERAL HEALING WOUNDS ON THE LEGS. c. Nephrotic syndrome Renal tubular acidosis Acute glomerulonephritis IgA nephropathy . ON FURTHER PROBING. THE EYES WERE NOTED TO BE PUFFY PARTICULARLY IN THE MORNING. oligoanuria Diuretic: spontaneous voiding. clinical improvement Early convalescent: increased well being . edema Oliguric: edema. hypertension. azotemia.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. may have dilutional anemia Elevated BUN and creatinine during the oliguric phase Urinalysis: proteins. rbcs . hypertension. on EM – there are “humps” on the GBM Low C3 levels Edema is due to salt and water retention . edema.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. b. c. d. Focal segmental sclerosis Membranous nephropathy Membranoproliferative GN type 1 Minimal change nephrotic syndrome . THE MOST COMMON CAUSE OF IDIOPATHIC NEPHROTIC SYNDROME IS a.58. edema and hyperlipidemia 90% idiopathic Causes of idiopathic: minimal change disease(85%). mesangial proliferation (5%).NEPHROTIC SYNDROME Heavy proteinuria . and focal segmental glomerulosclerosis (10%) . hypoalbuminemia. c. HE HAS a.59. d. YOU NOTE THAT A NEWBORN BABY BOY’S PREPUCE CANNOT BE RETRACTED. b. Hypospadias Phimosis Chordee Paraphimosis . 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. THE MOTHER ASKS IF SURGERY IS REQUIRED TO BRING IT DOWN. A 3-MONTH OLD BABY WITH UNILATERAL UNDESCENDED TESTIS WAS BROUGHT TO YOU FOR THE FIRST TIME. 1 month 2 months 3 months 4 months . c.60. b. HOW LONG WILL YOU OBSERVE? a. d. YOU TELL HER THAT YOU WILL OBSERVE FOR THE MEANTIME. 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 .UNDESCENDED TESTES (CRYPTORCHIDISM) Most common disorder of sexual differentiation in boys 4. b. Continuous convulsion lasting for 25 minutes Serial convulsions with return of consciousness in between Always generalized A medical emergency . WHICH IS TRUE ABOUT STATUS EPILEPTICUS? a. d. c.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 . THE FOLLOWING ARE PART OF THE MANAGEMENT OF HYPERCYANOTIC SPELLS.62. EXCEPT: a. d. Hydration Oxygen support Lactate administration Beta blocker administration . b. c. 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 . c. d.63. b. EAR PAIN AND EAR DISCHARGE? THE PATIENT HAS NOT BEEN TREATED WITH ANY OTHER ANTIBIOTIC PRIOR TO THIS EPISODE? a. Continue his medication Do tympanocentesis Give clindamycin at 40 mkd Increase the amoxicillin dose to 80-90 mkd . BUT STILL HAS FEVER. HOW WILL YOU MANAGE A PATIENT WITH ACUTE OTITIS MEDIA WHO HAS BEEN TREATED WITH AMOXICILLIN AT 50 MKD FOR 3 DAYS. cefuroxime. children who have recently received treatment with B lactam drugs. Moraxella catarrhalis Treatment: 1st line : Amoxicillin 40 mkd 80-100mkd Higher dose for children < 2 yrs. ceftriaxone . children who are exposed to large number of children 2nd line: Amoxicillin-clavulanic acid.ACUTE OTITIS MEDIA Etiology: Strep pneumoniae. non-typable H influenzae. d. WHAT ADVICE WILL YOU GIVE? a.65. b. 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 . THE 9 YEAR OLD GIRL WEIGHS 26 KG. NUTRITIONAL HISTORY REVEALS A WELL-BALANCED DIET. c. A MOTHER IS CONCERNED ABOUT HER DAUGHTER’S WEIGHT. in kgs = Age in years x 2 + 8 . in gms = Age in mos x 500 + BW 2 years and up Wt.WEIGHT • • Best index of growth and nutrition Mnemonics: • • • • • • Infants < 6 months Wt. in gms = Age in mos x 600 + BW 6-12 months Wt. b. d.66. WHICH OF THE FOLLOWING DISEASES IS PART OF THE NEONATAL NEWBORN SCREENING? a. Congenital toxoplasmosis Congenital rubella Congenital adrenal hyperplasia Congenital varicella . c. NEWBORN SCREENING . Lesch-Nyhan Newborn Screening PKU. GAL. 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. Pompe’s. CAH. Galactosemia Mucopolysaccharides: Hurler’s.METABOLIC DISEASES Inborn Errors of Metabolism Aminoacids: PKU. MSUD. Hunter’s. Sanfilippo Purine and Pyrimidines: Gout. CH. b. SHOW IRREGULAR BREATHING. ACTIVE MOTION.67. A NEONATE WITH A HEART RATE OVER 100/MIN. c. GRIMACE. 7 8 9 10 . PINK BODY BUT BLUE EXTREMITIES HAS AN APGAR SCORE OF __ AT 1 MINUTE a. d. continue taking APGAR until >7 is REACHED!!! . expect neurologic sequelae 10 mins: if still< 7.9 Sign of distress: </= 6 Severe asphyxia: 1-3 5 mins: prolonged distress in utero.PHYSICAL EXAM APGAR SCORE Dictates the need to resuscitate Normal: 7. some degree of damage. 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. pale Body pink. irregular 2 Over 100 Good. extremities blue . crying Some flexion of Active motion extremities Grimace Cough or sneeze Completely pink Blue. c. YOU SUSPECT THAT HE HAS AN ELECTROLYTE IMBALANCE. A PATIENT WAS BROUGHT TO THE ER DUE TO FLACCID PARALYSIS. d. Hypercalcemia Hypocalcemiia Hypokalemia Hyperkalemia . b. THE PATIENT WAS HOOKED TO A CARDIAC MONITOR AND YOU NOTE THAT HE HAS PROLONGED PR INTERVAL. WIDENED QRS AND PEAKED T WAVES. THESE FINDINGS ARE CONSISTENT WITH WHICH ELECTROLYTE ABNORMALITY? a.68. flattening of the P wave and widened QRS complex Can lead to ventricular fibrillation Paresthesias. fasciculations. ST segment depression. weakness and even an ascending paralysis Cardiac toxicity precedes all these symptoms . increased PR interval.HYPERKALEMIA Most important effects are due to the role of K in membrane polarization Peaked T waves . 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 . b.71. c. 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 . d. WHICH OF THE FOLLOWING IS TRUE ABOUT ANOREXIA NERVOSA/ a. which does not diminish as weight loss progresses Disturbance in the way in which one’s body weight.ANOREXIA NERVOSA AND BULIMIA Anorexia Intense fear of becoming obese. 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 . 72. d. b. SHE HAS a. c.A TEENAGE GIRL WITH ENDOMETRIOSIS IS COMPLAINING OF PAINFUL CRAMPS DURING MENSES. Dysfunctional uterine bleeding Primary dysmenorrhea Secondary dysmenorrhea Premenstrual syndrome . a foreign body . 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. b.73. d. c. Epinephrine Diphenhydramine Loratadine Cyproheptadine . DRUG OF CHOICE FOR ANAPHYLAXIS a. laryngeal edema and urticaria and angioedema Activation of mast cells and basophils via cell bound allergen specific IgE molecules Treatment: IM or IV epinephrine .ANAPHYLAXIS A serious allergic reaction that is rapid in onset and may cause death Pathologic features – acue pulmonary hyperinflation. pulmonary edema. intra alveolar hemorrhaging. visceral congestion. c. Hepatitis A Hepatitis B Hepatitis C Hepatitis D . WHICH OF THE FOLLOWING IS RARELY TRANSMITTED PERCUTANEOUSLY? a. d. b.74. b. d.75. 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. 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. 76. b. c. d. THE BEST WAY TO PREVENT DIARRHEA IN INFANCY IS a. Proper hygiene Immunization Boil water Breastfeed . d.000 IU 150. c.77. 50.000 IU 100. b. A 1 YEAR OLD CHILD WITH MEASLES MUST BE GIVEN VIT A.000 IU . WHICH IS THE CORRECT DOSE? a.000 IU 200. VITAMIN A IN MEASLES Reduces morbidity and mortality 6 mos.1yr : 100.000 IU 1 yr older : 200.000 IU . b. c. d. WHICH OF THE FOLLOWING IS EXPECTED OF A 4 YEAR OLD? a. Tell a story Name 4 colors Draw a triangle Dress and undress .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 . dada Immature jargon. run.second word 4 5 6 Repeats 3-word sentence Speech 100% intelligible Uses past tense of eat.sex 8 10 12 Says dada. go Gives word for definition .age.mama non-specifically First words other than mama.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. c. d.8C) None of the above . Maternal viral illness Antibiotic treatment of a child Fever (temperature of 37.79. WHICH OF THE FOLLOWING IS A CONTRAINDICATION TO ROUTINE CHILHOOD IMMUNIZATION? a. b. b. Rise in arterial pO2 Closure of the ductus arteriosus Increase in pulmonary blood flow Decrease in tortuosity of the pulmonary vasculature . PULMONARY VASCULAR RESISTANCE FALLS RAPIDLY AT BIRTH DUE TO a.80. d. c. 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 . SHORT STATURE.81. d. Marfan syndrome Down syndrome Palau syndrome Turner syndrome . WEBBED NECK AND COARCTATION OF THE AORTA CONSULTS AT YOUR CLINIC. c. THE MOST LIKELY DIAGNOSIS IS: a. b. AN 18-YEAR OLD GIRL WITH PRIMARY AMENORRHEA. TURNER SYNDROME Incidence: 1 /4000 Complete or partial absence of the x chromosome 45x Findings Phenotypically female Short stature Underdeveloped gonads Webbed neck . Papilledema Gaping wound at L2-L5 area Pending cerebral herniation Platelet count of 100 x 109 /L . d. b. c. NOT A CONTRAINDICATION TO LUMBAR TAP a.82. c. A PATIENT WITH HEMOPHILIA B IS BLEEDING PROFUSELY. WHICH OF THE FOLLOWING WILL YOU TRANSFUSE a.83. Fresh frozen plasma Factor IX concentrate Cryoprecipitate Platelet concentrate . b. d. PT. normal PC Liver Disease Clotting factors produced in the liver except VIII Treatment consists of replacement with FFP or cryoprecipitate . BT. PTT. normal PC. thrombin time Hallmark: hemarthrosis Von Willebrand Disease Most common hereditary bleeding disorder Sx of mucocutaneous bleeding Inc BT.HEMORRHAGIC AND THROMBOTIC DISEASES Hereditary Clotting Factor Deficiencies Factor VIII or IX (Hemophilia A or B) Inc APTT. 84. THE PATENT FORAMEN OVALE FUNCTIONALLY CLOSES AT: a. b. 10-15 hours of life 24 hours of life 1 month of life 3 months of life . d. c. CLOSURE OF SHUNTS Foramen ovale Functionally closed by the 3rd month Dustus arteriosus Functional closure by the 10-15th HOL . b. d. CYSTIC HYGROMA OCCURS MOST FREQUENTLY IN THE a. c. Abdomen Extremity Head and neck Chest .85. with most presenting by 2 y/o .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. 86. b. c. WHICH IS THE DRUG OF CHOICE FOR PROPHYLACTIC TREATMENT OF FEBRILE SEIZURES? a. Phenobarbital Phenytoin Carbamazepine None of the above . d. 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. imaging procedures Anticonvulsants and Diazepam? . focal findings) LP. tonic-clonic and lasts for a few seconds Simple vs complex (>15 min. repeated convulsions. EEG. b. WHICH OF THE FOLLOWING IS ASSOCIATED WITH GASTRITIS AND PEPTIC ULCER DISEASE? a. c. Escherichia coli Mycoplasma pneumoniae Helicobacter pylori Enterobius vermicularis . d.87. d. c. b. 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 .88. WHICH OF THE FOLLOWING IS AN INDICATION FOR PACKED RBC TRANSFUSION IN CHILDREN? a. 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 . 60% 70% 80% 90% . c. d. WHICH OF THE FOLLOWING VALUES TELLS YOU THAT A CHILD HAS SEVERE MALNUTRITION BASED ON WATERLOWE CLASSIFICATION? a.89. b. ANTIDOTE FOR IRON TOXICITY a.91. c. b. d. N-acetylcysteine Deferoxamine Naloxone EDTA . urinary excretion. hydration. Dialysis Iron >60 mg/k of elemental iron Deferoxamine .POISONING Acetaminophen Acute toxic dose: 200mg/k Tx: N-acetylcysteine (NAC) Salicylates 150mg/k Gastric decontamination. d. b. WHICH OF THE FOLLOWING IS A DIAGNOSTIC ABDOMINAL X-RAY FINDING OF NECROTIZING ENTEROCOLITIS? a. c. Air fluid levels in the bowels Dilated bowel loops Pneumatosis intestinalis Dilated proximal bowel with constriction distally .92. NECROTIZING ENTEROCOLITIS Necrotizing Enterocolitis. bloody stools Tx: no definitive tx for established NEC.most common life threatening emergency of the GI tract in the newborn period Pneumatosis intestinalis Triad: abdominal distention. 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 . d. c. 2 years 3 years 4 years 5 years .97. b. AT WHAT AGE CAN THE SNELLEN CHART BE USED TO ASSESS VISUAL ACUITY a. b. c.98. HE WANTS IT REPLANTED. IN WHAT MEIUM SHOULD THE TOOTH BE TRANSPORTED? a. d. A 15 YEAR OLD HAD HIS UPPER CENTRAL INCISOR KNOCKED OUT OFF THE SOCKET. Cow’s milk Tap water Normal saline Water with sugar . A suitable storage medium should have a physiologic osmolality in order to keep the PDL cells in as healthy a condition as possible. Lekic et al. 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. One of the critical factors affecting outcome is the way the tooth is handled during the extraoral period. 1999. although a new storage medium: Propolis may be better (Martin and Pileggi 2004) . A viable periodontal ligament (PDL) is important for proper physiologic healing of replanted teeth. 1998) in terms of PDL cell viability. Milk appears to be the storage medium of choice in an emergency situation. Milk has an osmolality within physiologic limits. Supine Prone Side Any of the above . b. WHICH OF THE FOLLOWING SLEEPING POSITIONS HAS BEEN NOTED TO DECREASE THE INCIDENCE OF SUDDEN INFANT DEATH SYNDROME? a. d. c.99. SIDS Sudden death of an infant that is unexpected by history and unexplained by thorough post mortem examination. 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 . which includes autopsy. investigation of the scene of death. 100. WHICH OF THE FOLLOWING SHOULD BE THE INITIAL MANAGEMENT? a. c. d. Give large amount of milk Induce emesis Gastric lavage Rinse the mucosa . A CHILD ACCIDENTALLY INGESTED A BLEACHING SOLUTION (ZONROX). b. lacrimation.POISONING Caustics Acids and Alkali Thorough removal Emesis and lavage are contraindicated Activated charcoal should not be used Insecticides SLUDGE ( salivation. emesis) Antidotes: atropine and pralidoxime . gastrointestinal cramps. urination. defacation. THANK YOU! .