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

2. A 6-MONTH OLD BABY WAS BROUGHT TO THE ER BECAUSE OF DOB. ON PE, SHE WAS TACHYPNEIC,, WITH RALES AND NOTE OF A GR 3/6 HOLOSYSTOLIC MURMUR ON THE LLSB. CXR SHOWED CONGESTION AND CARDIOMEGALY LV FORM. ABG SHOWED PO2 OF 175 OFF OXYGEN. THE MOST LIKELY CARDIAC LESION IS:
a. b.

c.
d.

Pulmonary stenosis Atrial septal defect Ventricular septal defect Transposition of the great arteries

RECALL

Congenital Heart Diseases Acyanotic vs Cyanotic Acyanotic Increased volume load L R shunting (ASD, VSD, PDA, AV septal defects) Increased pressure load Normal blood flow is obstructed (pulmonic stenosis, etc) Cyanotic Decreased pulmonary blood flow Obstruction and a shunt (ex. 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

Tools in the Assessment of Heart Disease

FIVE BASIC QUESTIONS
1. Is the patient cyanotic or acyanotic? 2. Is the pulmonary arterial flow increased or not ? 3. Does the malformation originate in the left or right side of the heart? 4. Which is the dominant ventricle? 5. Is pulmonary hypertension present or not ?
HISTORY PHYSICAL EXAM

CHEST X- Ray
EKG (15 lead)

Others: 2D Echo Cardiac cath

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= < 0.5cm2, RV pressure is N, shunt is LR Non restrictive VSDs >1.0 cm2 RV and LV pressure is equalized

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

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, bounding peripheral pulses with wide PP Indomethacin

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. THE FOLLOWING ARE ANTI-

HEART FAILURE MEDICATIONS, EXCEPT:

a. b. c. d.

Dopamine Furosemide Phenytoin Digoxin

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, enalapril, nitroprusside)

4. A 10 YEAR OLD WENT TO YOU FOR CONSULT DUE TO DIFFICULTY IN WALKING. ON PE, YOU NOTE THAT HE IS ALMOST PULSELESS. YOU SUSPECT:
a. b. c. d.

Juvenile Rheumatoid Arthritis Takayasu Arteritis Panarteritis nodosa Systemic lupus erythematosus

CLUE: PULSELESSNESS

Differentials

Coarctation of the aorta

Femoral pulses are weak and delayed or absent

Vasculitis syndromes

Vasculitis syndromes
HSP, Takayasu arteritis, PAN Takayasu involves large arteries, pulseless disease PAN- small to medium sized arteries, 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 (CHILDRENS 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

8. A PX COMES IN WITH TRISMUS, DIFFICULTY OF SWALLOWING AND HOT POTATO VOICE. YOUR DX IS
a.
b. c. d.

Retropharyngeal abscess Croup Peritonsillar abscess Epiglottitis

NECK ABSCESSES

Retropharyngeal and Lateral pharyngeal abscess

Common in young children 3-4 years old or younger Fever , irritability, decreased oral intake and drooling Bulging posterior pharyngeal wall polymicrobial
Relatively common Older child, adolescent Sore throat, fever, trismus, dysphagia Asymmetric tonsillar bulge with displaced uvula hot potato voice

Peritonsillar cellulitis/abscess

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

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

Pneumonia Spontaneous pneumothorax Lung tumor Atelectasis

PNEUMOTHORAX

Accumulation of extrapulmonary air within the chest Primary vs secondary Spontaneous, traumatic, 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, doxycycline, iodopovidone)

10. TRUE ABOUT EPIGLOTITTIS

a.

b.

c. d.

Rapidly progressive respiratory obstruction Etiologic agent: Haemophilus influenza b A medical emergency All of the above

EPIGLOTITIS

bacterial (H influenza) life-threatening high fever, drooling, toxic child acute onset sitting forward critical airway thumb sign

11. WHICH OF THE FOLLOWING INFECTIONS PRESENT AS PHARYNGEAL INJECTION, WITH TONSILLAR MEMBRANE AND BULL NECK APPEARANCE?
a. b. c. d.

Diphtheria Pertussis Tetanus Polio

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)

12. GOLD STANDARD IN THE TREATMENT OF UNCOMPLICATED SALMONELLOSIS

a.
b. c. d.

Penicillin Ceftriaxone Aspirin Chloramphenicol

SALMONELLOSIS

Nontyphoidal salmonellosis
Acute enteritis, bacteremia, extraintestinal focal infections Tx: antibiotics for bacteremia and extra intestinal infections (ceftriaxone) For acute enteritis: no antibiotics, only for infants <3 mos, malnourished, immunocompromised

Enteric or typhoid fever

S. Ser Typhi, Paratyphi A, Paratyphi B, Paratyphi C Relative bradycardia, rose spots Dx: bone marrow culture Tx: Chloramphenicol remains the gold standard

13. WHICH IS CONSIDERED AS POSITIVE PPD?

a.

b.
c. d.

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

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, clinical findings suggestive of TB, cxr suggestive of TB and immunocompromised condition Otherwise, >10mm is considered positive

POSITIVE PPD

INDURATION > 5 MM

Close contacts of TB pxs, immunocompromised, those with clinical and radio findings
Infants and children <4 yrs, those with underlying medical conditions, frequently exposed to high risk adults > 4yrs old with no risk factors

INDURATION > 10 MM

INDURATION > 15 MM

14. WHICH OF THE FOLLOWING ANTITUBERCULOSIS MEDICATIONS IS CONTRAINDICATED FOR CHILDREN BELOW 7 YEARS OLD?
a. b.

c.
d.

Rifampicin Pyrazinamide Streptomycin Ethambutol

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, cytological, biochemical, etc)

Signs and Symptoms

Cough/ wheezing of 2 weeks or more Unexplained fever of 2 weeks or more Loss of appetite, 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, reduced playfullness, or lethargy

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, clinical findings suggestive of TB, cxr suggestive of TB and immunocompromised condition Otherwise, >10mm is considered positive

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, TB adenitis, CNS TB, etc

Extrapulmonary/Extrathoracic

Management of TB
Intensive Phase (2 mos) Continuation Phase (4 mos) DOTS : decrease the risk of infection, 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

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

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

Rubella Roseola Rubeola Mumps

ROSEOLA INFANTUM
Exanthem subitum or 6th disease Recall: 1st measles, 2nd scarlet, 3rd rubella, 4th atypical scarlet( Filatov-Dukes), 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

16. WHICH IS THE VECTOR OF DENGUE FEVER?

a. b. c.

d.

Anopheles mosquito Culex mosquito Aedes aegypti mosquito Aedes africanus

Aedes aegypti

Flight range: < 25 m in open, urban environmt Dispersal: 30-50 m/d Visits not more than 2-3 houses Remains infective during lifetime High humidity, longer mosquito survival Ave lifespan: 8-15 d

17. MOST SEVERE FORM OF MALARIA, PRESENTING WITH INTENSE PARASITISM

a. b.

c.
d.

Malariae Falcifarum Vivax Ovale

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

18. DRUG OF CHOICE FOR PREVENTION OF RELAPSES OF P. VIVAX AND P. OVALE INFECTION
a. b.

c.
d.

Quinine Chloroquine Primaquine Pyrimethamine sulfadoxime

RELAPSE
Common

in P. Vivax, 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

19. WHICH OF THE FOLLOWING INFESTATIONS IS NOT DETECTED BY DIRECT FECAL SMEAR?
a. b.

c.
d.

Enterobiasis Trichuriasis Hookworms Diphyllobotriasis

HELMINTHIC DISEASES
Direct

fecal exam Ascaris, hookworms, trichuriasis, strongyloides, diphyllobotriasis Microscopic exam of cellophane tape enterobius Demonstration in the blood - filariasis Stool exam by Kato thick smear schistosomiasis

20. WHICH OF THE FOLLOWING VACCINES IS PART OF THE EPI?

a. b. c.

d.

Tetanus toxoid Varicella vaccine Rotavirus vaccine Typhoid vaccine

EXPANDED PROGRAM ON IMMUNIZATION

BCG HEPATITIS B DPT POLIO MEASLES RECENT INCLUSIONS: HiB, MMR, ROTAVIRUS

21. WHAT IS THE MOST COMMON HEMATOLOGIC DISEASE OF INFANCY AND CHILDHOOD?
a. b. c. d.

Acute leukemia Iron deficiency anemia Idiopathic thrombocytopenic purpura Hereditary spherocytosis

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, 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, Meckel, polyp, hemangioma, or inflammatory bowel disease Hookworm infestation

22. IN PHYSIOLOGIC ANEMIA OF INFANCY, HEMOGLOBIN DECLINES AT ____ WEEKS FOR TERM INFANTS
a. b.

c.
d.

3-6 10-12 6-8 2-4

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)

23. WHICH OF THE FOLLOWING PRODUCES PERIPHERAL PANCYTOPENIA WITH HYPOPLASTIC MARROW?
a. b. c.

d.

Acute leukemia Iron deficiency anemia Idiopathic thrombocytopenic purpura Aplastic anemia

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, PC 20,000-100,000/mm3, retic count <1% after correction for hematocrit) Moderate aplastic anemia ANC 500 -1,500/mm3, PC 20,000 -100,000 and retic count <1%

24. MOST COMMON EXTRACRANIAL TUMOR OF CHILDHOOD, MOST FREQUENTLY DIAGNOSED DURING INFANCY, AND MOSTLY ARISING FROM THE ABDOMEN
a. b. c.

d.

Wilms tumor Hepatoblastoma Neuroblastoma Nephroblastoma

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; 2nd most common malignant abdominal tumor in childhood 3 elements: blastema, epithelia and stroma Associated with hemihypertrophy, aniridia, and other congenital anomalies (WAGR, Denys-Drash, Beckwith-Wiedemann) Occurs in children between 2-5 years old CT: no calcification

Wilms Tumor

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

d.

Osteosarcoma Ewings sarcoma Rhabdomyosarcoma Enchondroma

CANCER AND BENIGN TUMORS

Bone Tumors
Osteosarcoma Second decade All races 1.5:1 Spindle cell Retinoblastoma, Pagets Metaphyses of long bones Local pain & swelling Sunburst pattern Lungs, bones Chemotherapy Without mets: 70% cured With mets: < 20% survival Ewings Second decade Primarily whites 1.5:1 Small round cell None Diaphyses, 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

26. DEFICIENCY IN WHICH OF THE FOLLOWING FACTORS WILL YOU NEED TO GIVE VITAMIN K?
a.
b. c. d.

Factors II, V, VII, IX Factors V, VIII, IX, X, XI Factors II, VII, IX, X Factors II, VIII, IX, X

27. A NEWBORN PRESENTED WITH BLUEBERRY MUFFIN RASH, CATARACTS, PULMONARY STENOSIS AND HEARING LOSS. YOU SUSPECT THAT THE BABY HAS
a. b. c.

d.

Congenital varicella syndrome Neonatal herpes syndrome Congenital syphillis Congenital rubella syndrome

CONGENITAL INFECTIONS

Congenital Syphilis

Early and late signs Early snuffles; late Hutchinsons teeth, saber shins, olympian brow

Congenital varicella malformations of the extremities with cicatricial scarring Congenital rubella cataracts, heart defects, deafness, blueberry muffin lesions (same as in CMV) Neonatal herpes

28. A BABY WAS BROUGHT TO YOUR CLINIC

BECAUSE OF FAILURE TO THRIVE. HE HAD DELAYED PASSAGE OF MECONIUM AND CHRONIC CONSTIPATION. HE HAS ABOMINAL DISTENTION AND A PALPABLE MASS ON THE LLQ. THE RECTAL VAULT WAS EMPTY BUT A GUSH OF FOUL-SMELLING GAS WAS NOTED ON RECTAL EXAM. BARIUM ENEMA SHOWED A DILATED PROXIMAL COLON AND SMALL CALIBER DISTAL COLON. SHORT OF A BIOPSY, YOUR DIAGNOSIS IS
a.

b.
c. d.

Intussusception Congenital aganglionic megacolon Hypertrophic pyloric stenosis Gastric volvulus

 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, 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.
b. c. d.

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

30. WHICH OF THE FOLLOWING HORMONES IS NOT FOUND IN THE ANTERIOR PITUITARY
a. b.

c.
d.

Oxytocin Prolactin Leutinizing hormone Follicle stimulationg hormone

PITUITARY HORMONES
Anterior

Pituitary GH, prolactin, TSH, ACTH, LH, FSH, POMC Posterior Pituitary Vasopressin and oxytocin

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

Insulin challenge test DDAVP test Erythropoietin test Beta agonist challenge

DIABETES INSIPIDUS
Polyuria, polydipsia (exceeding 2L/m2/24hr), 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, congenital malformations of the hypothalamus or pituitary, neoplasms, infections, autoimmune diseases, inc vasopressin metabolism

Nephrogenic DI
Vasopressin insensitive Genetic (less common but more severe) or acquired causes

32. A KNOWN INSULIN DEPENDENT DIABETIC

COMES TO YOUR CLINIC AND COMPLAINS OF EARLY MORNING HYPERGLYCEMIA. YOU SUSPECT BRITTLE DIABETES. YOU MEASURE HIS 3, 4, AND 7 AM GLUCOSE CONCENTRATION. THE 3 AND 4 AM LEVELS ARE BELOW 60 MG/DL, WHILE THE 7 AM IS MARKEDLY HIGH. THE PX IS EXPERIENCING
a. b. c. d.

Diabetic ketoacidosis Insulin shock Somogyi phenomenon Dawn phenomenon

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

33. A NEIGHBOR SHOWS YOU HER 3-WEEK OLD

BABY FOR CONSULT. SHE TELLS YOU THAT THE BABY IS SLUGGISH, DOES NOT FEED WELL AND IS CONSTIPATED. YOU NOTE THAT SHE IS JAUNDICED WITH A GLAZED LOOK, ENLARGED ABDOMEN, UMBILICAL HERNIA, AND MOTTLED SKIN. WHICH OF THE FOLLOWING LABORATORY EXAMS WILL YOU PRIORITIZE TO HELP YOU WITH YOUR DIAGNOSIS?
a. b. c. d.

Chest x-ray Lumbar tap Serum T3, T4, TSH Serum Na, K, Ca

CONGENITAL HYPOTHYROIDISM
Most common cause is thyroid dysgenesis (8085%); 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, sluggishness, lack of interest, somnolence, choking spells during nursing, constipation, large abdomen, umbilical hernia Low serum T4 and elevated TSH levels Tx: levothyroxine

34. YOUR ADOLESCENT NIECE TALKS TO YOU ABOUT HER HEIGHT. SHE IS VERY TALL (90TH PERCENTILE), AND ASKS IF THERE IS SOMETHING WRONG WITH HER. HER PARENTS AND SIBLINGS ARE ALL TALL, AND SHE IS IN EXCELLENT HEALTH. WHAT WILL YOU DO?
a. b.

c.
d.

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

35. A WEEK OLD BABY WAS BROUGHT TO THE ER

BECAUSE OF WEAKNESS. SHE HAS BEEN VOMITING AND FEEDING POORLY. HER LAB EXAMS ARE UNREMARKABLE EXCEPT FOR SEVERE METABOLIC ACIDOSIS. YOU TELL THE ATTENDING PHYSICIAN THAT WHILE DOING YOUR PE, YOU NOTED THAT HER URINE SMELLED SWEET, LIKE MAPLE SYRUP. YOU THEN ASK FOR AMINOACID ASSAY, AND EXPECT THE FOLLOWING RESULT:
a.

b.

c. d.

High leucine, isoleucine and valine levels and low alanine levels High isovaleric acid, isovaleryglycerine and hydroxyvaleric acid levels High methylmalonyl acid levels High glutamine and alanine levels

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, Acrid Swimming pool Sweaty feet, Acrid Maple syrup Boiled cabbage Tomcat urine Mousy or musty Rotting fish Boiled cabbage, rancid butter

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

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

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, associated hunger and crying In older children- trismus (masseter muscle spasm or lockjaw), sardonic smile ( intractable spasms of the facial and buccal muscles)

Treatment
Human Tetanus Ig Penicillin G antibiotic of choice; alternatives: metronidazole, erythromycin, Tetracycline Surgery

37. A TEENAGER WAS BROUGHT TO THE ER

FOR RESPIRATORY DISTRESS. HE WAS IMMEDIATELY INTUBATED. THE ILLNESS STARTED AS DIFFICULTY IN WALKING, THEN WEAKNESS OF THE UPPER TRUNK AND EXTREMITIES AND FINALLY FACIAL WEAKNESS. THE HX IS UNREMARKABLE EXCEPT FOR A NON SPECIFIC VIRAL ILLNESS 2 WEEKS AGO. PE SHOWS COMPLETE PARALYSIS WITH ABSENT TENDON REFLEXES. THE LIKELY DX IS
a.

b.
c. d.

Bell palsy Transverse myelitis Spinal muscular atrophy Guillain-Barre Syndrome

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, the upper limbs, 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

38. MOST COMMON INFRATENTORIAL TUMOR IN CHILDREN

a. b. c.

d.

Medulloblastoma Brainstem glioma Cerebellar astrocytoma ependymoma

CANCER AND BENIGN TUMORS

Brain Tumors in Children

Categories
Juvenile pilocytic astrocytoma MOST COMMON Medulloblastoma Diffuse astrocytoma Ependymoma craniopharyngioma

Location
Infratentorial (43.2%) Supratentorial (40.9%) Spinal cord (4.9%) Multiple sites (11%)

Important!
Within the 1st year supratentorial 1-10 yr infratentorial Cranial exposure to ionizing radiation inc incidence

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

TB meningitis Bacterial meningitis Normal CSF Viral meningitis

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, wbc up to 5 (for NB up to15), no rbcs, protein 10-40 mg/dl (120 in a neonate), glucose 60%

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

Porencephaly Lissencephaly Schizencephaly Holoprosencephaly

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

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

Night terror Rage attack Choreoathetosis Nightmares

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

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

Lindane Topical corticosteroid Selsun blue Tretinoin

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

43. A NEWBORNS WEIGHT GAIN IS APPROXIMATELY __ GMS/MONTH AT AGE 2-6 MONTHS

a. b.

c.
d.

300 400 500 600

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

44. THE FIRST PRIMARY TEETH TO ERUPT ARE

a. b. c.

d.

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

45. HANDEDNESS IS USUALLY ESTABLISHED AT AGE ___ YEARS

a. b. c.

d.

2 3 4 5

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; bedwetting up to age 4 in girls and 5 years in boys Piagets preoperational stage: magical thinking, egocentrism and thinking that is dominated by perception, not abstraction

46. THE FIRST VISIBLE SIGN OF PUBERTY IN MALES IS

a. b. c.

d.

Voice change Pubic hair Growth spurt Testicular enlargement

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

47. A 3-YEAR OLD WAS BROUGHT TO YOU FOR EVALUATION OF DELAYED SPEECH. THE CHILD HAS REPETITIVE BEHAVIOR, NO EYE CONTACT, ALONE. HE IS SUSPECTED TO HAVE
a.

b.
c. d.

Asperger disorder Autism Schizophrenia Attention deficit disorder

PERVASIVE DEVELOPMENTAL DISORDERS

Autistic Disorder Asperger Disorder Childhood Disintegrative Disorder Rett Disorder Childhood Schizophrenia

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

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

Asperger Disorder no severe language impairments as compared to patients with autism Rett Disorder x-linked dominant disorder affecting girls exclusively; boys die at birth

development is normal until about 1-2 years old until motor and language development regress

48. YOU EXPECT A SEVEN MONTH OLD TO BE ABLE TO

a. b. c.

d.

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

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

FINE MOTOR MILESTONES

Mean Age (mos)
3.5 4 4 5.5 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

49. XEROPHTHALMIA, NYCTALOPIA, AND PHOTOPHOBIA ARE CAUSED BY DEFICIENCY IN WHICH VITAMIN?
a. b.

c.
d.

A B C D

VITAMIN DEFICIENCIES

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

50. WHICH OF THE FOLLOWING MINERAL WILL CAUSE MOTTLING OF THE TEETH WHEN TAKEN IN EXCESS?
a. b.

c.
d.

Calcium Iron Magnesium Fluoride

FLUOROSIS
Mottled enamel Results from systemic fluoride consumption (>0.05mkd) 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

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

b.
c. d.

Kwashiorkor Marasmus Pellagra Rickets

PROTEIN/ENERGY MALNUTRITION (PEM)

Edematous

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

52. A CHILD WITH HYPOTONIA, SLANTED PALPEBRAL FISSURES, EPICANTHAL FOLD, SIMIAN CREASE, COMPLETE ATRIO-VENTRICULAR SEPTAL DEFECT, AND SHORT BROAD HANDS HAS
a. b. c. d.

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

53. PREMATURE BIRTH IS DEFINED AS LIVE BIRTH BEFORE __ WEEKS FROM THE FIRST DAY OF THE LAST MENSTRUAL PERIOD:
a.

b.
c. d.

39 38 37 36

54. VERY LOW BIRTHWEIGHT INFANTS WEIGH LESS THAN ____ GMS
a. b. c. d.

1,000 2,500 2,000 1,500

Perinatal Mortality - influenced by prenatal, maternal and fetal factors

IUGR, asphyxia, placental insufficiency, severe congenital malformations, 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

55. DIFFERENTIAL DIAGNOSES FOR JAUNDICE IN THE FIRST 24 HOURS OF LIFE, EXCEPT:
a. b.

c.
d.

Physiologic jaundice ABO incompatibility Sepsis Hemorrhage

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 >0.5 mg/dl/hr >8 days (term), 14 days (preterm) symptomatic

KERNICTERUS
Bilirubin encephalopathy resulting from the deposition of unconjugated (indirect) bilirubin in the basal ganglia and brainstem nuclei > 25mg/dl, range: 21-50 Clinical features

Acute
Phase 1: poor suck, hypotonia, seizures Phase 2: hypertonia of extensor muscles, opisthotonos, retrocolis, fever Phase 3: hypertonia

Chronic
1st year : hypotonia, active deep tendon reflexes After 1st year: movement disorders, SNHL, upward gaze

KERNICTERUS

Treatment of Hyperbilirubinemia

Phototherapy
Photoisomerization Complication; loose stools, rash, overheating, dehydration, 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

56. A CHILD WAS BROUGHT TO THE ER DUE TO

EDEMA. IT STARTED ON THE FEET PROGRESSING TOWARDS THE THIGHT. THE EYES WERE NOTED TO BE PUFFY PARTICULARLY IN THE MORNING. THE MOTHER CANNOT RECALL ANY PRIOR ILLNESS. ON PE, YOU NOTICE SEVERAL HEALING WOUNDS ON THE LEGS, AND HIS BP IS HIGH. ON FURTHER PROBING, THE CHILD CONFESSES THAT HIS URINE IS COLA COLORED. YOUR PRIMARY DIAGNOSIS IS:
a.

b.
c. d.

Nephrotic syndrome Renal tubular acidosis Acute glomerulonephritis IgA nephropathy

POST STREPTOCOCCAL GLOMERULONEPHRITIS

Most

common in children age 5 to 12 years Uncommon before the age of 3 Phases:

Latent: hematuria, edema Oliguric: edema, hypertension, azotemia, oligoanuria Diuretic: spontaneous voiding, clinical improvement Early convalescent: increased well being

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

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

58. THE MOST COMMON CAUSE OF IDIOPATHIC NEPHROTIC SYNDROME IS

a.

b.
c. d.

Focal segmental sclerosis Membranous nephropathy Membranoproliferative GN type 1 Minimal change nephrotic syndrome

NEPHROTIC SYNDROME
Heavy

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

59. YOU NOTE THAT A NEWBORN BABY BOYS PREPUCE CANNOT BE RETRACTED. HE HAS
a. b. c.

d.

Hypospadias Phimosis Chordee Paraphimosis

ANOMALIES OF THE PENIS AND URETHRA

Hypospadias- 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

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

c.
d.

1 month 2 months 3 months 4 months

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

61. WHICH IS TRUE ABOUT STATUS EPILEPTICUS?

a.

b.

c. d.

Continuous convulsion lasting for 25 minutes Serial convulsions with return of consciousness in between Always generalized A medical emergency

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

62. THE FOLLOWING ARE PART OF THE MANAGEMENT OF HYPERCYANOTIC SPELLS, EXCEPT:
a. b. c. d.

Hydration Oxygen support Lactate administration Beta blocker administration

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

63. HOW WILL YOU MANAGE A PATIENT WITH ACUTE OTITIS MEDIA WHO HAS BEEN TREATED WITH AMOXICILLIN AT 50 MKD FOR 3 DAYS, BUT STILL HAS FEVER, EAR PAIN AND EAR DISCHARGE? THE PATIENT HAS NOT BEEN TREATED WITH ANY OTHER ANTIBIOTIC PRIOR TO THIS EPISODE?
a. b.

c.
d.

Continue his medication Do tympanocentesis Give clindamycin at 40 mkd Increase the amoxicillin dose to 80-90 mkd

ACUTE OTITIS MEDIA

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

65. A MOTHER IS CONCERNED ABOUT HER DAUGHTERS WEIGHT. THE 9 YEAR OLD GIRL WEIGHS 26 KG. NUTRITIONAL HISTORY REVEALS A WELL-BALANCED DIET. WHAT ADVICE WILL YOU GIVE?
a.

b.

c.

d.

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

WEIGHT

Best index of growth and nutrition Mnemonics:

Infants < 6 months

Wt. 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

66. WHICH OF THE FOLLOWING DISEASES IS PART OF THE NEONATAL NEWBORN SCREENING?
a.
b. c. d.

Congenital toxoplasmosis Congenital rubella Congenital adrenal hyperplasia Congenital varicella

NEWBORN SCREENING

METABOLIC DISEASES

Inborn Errors of Metabolism

Aminoacids: PKU, MSUD, Tyrosinemia Lipids: ALD Carbohydrates: Von Gierkes, Pompes, Galactosemia Mucopolysaccharides: Hurlers, Hunters, Sanfilippo Purine and Pyrimidines: Gout, Lesch-Nyhan

Newborn Screening
PKU, CAH, CH, GAL, G6PD Republic Act 9288: Newborn Screening Act of 2004 Done after the 24th HOL and not later than 72 HOL

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

7 8 9 10

PHYSICAL EXAM
APGAR SCORE
Dictates the need to resuscitate Normal: 7- 9 Sign of distress: </= 6 Severe asphyxia: 1-3 5 mins: prolonged distress in utero, some degree of damage, expect neurologic sequelae 10 mins: if still< 7, continue taking APGAR until >7 is REACHED!!!

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, irregular 2 Over 100 Good, crying

Some flexion of Active motion extremities Grimace Cough or sneeze Completely pink

Blue, pale

Body pink, extremities blue

68. A PATIENT WAS BROUGHT TO THE ER DUE

TO FLACCID PARALYSIS. YOU SUSPECT THAT HE HAS AN ELECTROLYTE IMBALANCE. 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. b. c. d.

Hypercalcemia Hypocalcemiia Hypokalemia Hyperkalemia

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

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 <2.5meqL 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 Lorenzos oil Baclofen

ALD
X-linked

Accumulation

of unbranched saturated VLCFA Treatment Corticosteroid BMT benefits those with neurologic disability Lorenzos 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

71. WHICH OF THE FOLLOWING IS TRUE ABOUT ANOREXIA NERVOSA/

a.

b.

c.

d.

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 ones 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

72.A TEENAGE GIRL WITH ENDOMETRIOSIS IS COMPLAINING OF PAINFUL CRAMPS DURING MENSES. SHE HAS
a. b. c. d.

Dysfunctional uterine bleeding Primary dysmenorrhea Secondary dysmenorrhea Premenstrual syndrome

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 , endometriosis or endometritis

73. DRUG OF CHOICE FOR ANAPHYLAXIS

a. b. c.

d.

Epinephrine Diphenhydramine Loratadine Cyproheptadine

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, laryngeal edema and urticaria and angioedema Activation of mast cells and basophils via cell bound allergen specific IgE molecules Treatment: IM or IV epinephrine

74. WHICH OF THE FOLLOWING IS RARELY TRANSMITTED PERCUTANEOUSLY?

a. b. c.

d.

Hepatitis A Hepatitis B Hepatitis C Hepatitis D

75. WHICH OF THE FOLLOWING MARKERS TELLS YOU THAT YOUR PATIENT WITH HEPATITIS B IS HIGHLY INFECTIVE?
a. b. c. d.

HBsAg HBeAg IgM anti-HBc Anti-HBc

HEPATITIS B MARKERS
first marker to appear; 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

76. THE BEST WAY TO PREVENT DIARRHEA IN INFANCY IS

a.

b.
c.

d.

Proper hygiene Immunization Boil water Breastfeed

77. A 1 YEAR OLD CHILD WITH MEASLES MUST BE GIVEN VIT A. WHICH IS THE CORRECT DOSE?
a.
b. c. d.

50,000 IU 100,000 IU 150,000 IU 200,000 IU

VITAMIN A IN MEASLES
Reduces

morbidity and mortality 6 mos- 1yr : 100,000 IU 1 yr older : 200,000 IU

78. WHICH OF THE FOLLOWING IS EXPECTED OF A 4 YEAR OLD?

a. b. c.

d.

Tell a story Name 4 colors Draw a triangle Dress and undress

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

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,age,sex

8
10 12

Says dada,mama non-specifically

First words other than mama, dada Immature jargon,second word

4
5 6

Repeats 3-word sentence Speech 100% intelligible Uses past tense of eat, run, go Gives word for definition

79. WHICH OF THE FOLLOWING IS A CONTRAINDICATION TO ROUTINE CHILHOOD IMMUNIZATION?

a. b.

c.
d.

Maternal viral illness Antibiotic treatment of a child Fever (temperature of 37.8C) None of the above

80. PULMONARY VASCULAR RESISTANCE FALLS RAPIDLY AT BIRTH DUE TO

a.
b. c. d.

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

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

Marfan syndrome Down syndrome Palau syndrome Turner syndrome

TURNER SYNDROME
Incidence: 1 /4000 Complete or partial absence of the x chromosome 45x Findings

Phenotypically female Short stature Underdeveloped gonads Webbed neck

82. NOT A CONTRAINDICATION TO LUMBAR TAP

a. b. c.

d.

Papilledema Gaping wound at L2-L5 area Pending cerebral herniation Platelet count of 100 x 109 /L

83. A PATIENT WITH HEMOPHILIA B IS BLEEDING PROFUSELY. WHICH OF THE FOLLOWING WILL YOU TRANSFUSE
a. b. c.

d.

Fresh frozen plasma Factor IX concentrate Cryoprecipitate Platelet concentrate

HEMORRHAGIC AND THROMBOTIC DISEASES

Hereditary Clotting Factor Deficiencies

Factor VIII or IX (Hemophilia A or B) Inc APTT, normal PC, BT, PT, thrombin time Hallmark: hemarthrosis

Von Willebrand Disease

Most common hereditary bleeding disorder Sx of mucocutaneous bleeding Inc BT, PTT, normal PC

Liver Disease
Clotting factors produced in the liver except VIII Treatment consists of replacement with FFP or cryoprecipitate

84. THE PATENT FORAMEN OVALE FUNCTIONALLY CLOSES AT:

a. b. c.

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

85. CYSTIC HYGROMA OCCURS MOST FREQUENTLY IN THE

a. b. c.

d.

Abdomen Extremity Head and neck Chest

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

86. WHICH IS THE DRUG OF CHOICE FOR PROPHYLACTIC TREATMENT OF FEBRILE SEIZURES?
a. b.

c.
d.

Phenobarbital Phenytoin Carbamazepine None of the above

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, tonic-clonic and lasts for a few seconds Simple vs complex (>15 min, repeated convulsions, focal findings) LP, EEG, imaging procedures Anticonvulsants and Diazepam?

87. WHICH OF THE FOLLOWING IS ASSOCIATED WITH GASTRITIS AND PEPTIC ULCER DISEASE?
a. b.

c.
d.

Escherichia coli Mycoplasma pneumoniae Helicobacter pylori Enterobius vermicularis

88. WHICH OF THE FOLLOWING IS AN INDICATION FOR PACKED RBC TRANSFUSION IN CHILDREN?
a.

b.

c.

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

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

89. WHICH OF THE FOLLOWING VALUES TELLS YOU THAT A CHILD HAS SEVERE MALNUTRITION BASED ON WATERLOWE CLASSIFICATION?
a.

b.
c. d.

60% 70% 80% 90%

91. ANTIDOTE FOR IRON TOXICITY

a. b. c.

d.

N-acetylcysteine Deferoxamine Naloxone EDTA

POISONING

Acetaminophen
Acute toxic dose: 200mg/k Tx: N-acetylcysteine (NAC)

Salicylates
150mg/k Gastric decontamination, hydration, urinary excretion, Dialysis

Iron
>60 mg/k of elemental iron Deferoxamine

92. WHICH OF THE FOLLOWING IS A DIAGNOSTIC ABDOMINAL X-RAY FINDING OF NECROTIZING ENTEROCOLITIS?
a. b. c. d.

Air fluid levels in the bowels Dilated bowel loops Pneumatosis intestinalis Dilated proximal bowel with constriction distally

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

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

97. AT WHAT AGE CAN THE SNELLEN CHART BE USED TO ASSESS VISUAL ACUITY
a.
b. c. d.

2 years 3 years 4 years 5 years

98. A 15 YEAR OLD HAD HIS UPPER CENTRAL INCISOR KNOCKED OUT OFF THE SOCKET. HE WANTS IT REPLANTED. IN WHAT MEIUM SHOULD THE TOOTH BE TRANSPORTED?
a. b. c. d.

Cows milk Tap water Normal saline Water with sugar

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. A suitable storage medium should have a physiologic osmolality in order to keep the PDL cells in as healthy a condition as possible. Milk has an osmolality within physiologic limits. In vitro studies suggest that it is superior to saliva (Blomlof and Otteskog 1980) and to Eagles medium (Blomlof 1981) and other media (Ashkenazi et al. 1999, Lekic et al. 1998) in terms of PDL cell viability. Milk appears to be the storage medium of choice in an emergency situation, although a new storage medium: Propolis may be better (Martin and Pileggi 2004)

99. WHICH OF THE FOLLOWING SLEEPING POSITIONS HAS BEEN NOTED TO DECREASE THE INCIDENCE OF SUDDEN INFANT DEATH SYNDROME?
a. b.

c.
d.

Supine Prone Side Any of the above

SIDS
Sudden

death of an infant that is unexpected by history and unexplained by thorough post mortem examination, which includes autopsy, investigation of the scene of death, 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

100. A CHILD ACCIDENTALLY INGESTED A BLEACHING SOLUTION (ZONROX). WHICH OF THE FOLLOWING SHOULD BE THE INITIAL MANAGEMENT?
a. b. c. d.

Give large amount of milk Induce emesis Gastric lavage Rinse the mucosa

POISONING

Caustics

Acids and Alkali Thorough removal Emesis and lavage are contraindicated Activated charcoal should not be used

Insecticides
SLUDGE ( salivation, lacrimation, urination, defacation, gastrointestinal cramps, emesis) Antidotes: atropine and pralidoxime

THANK YOU!