PEDIATRICS SYNDROMES quiz (BEST FOR REVISION) Q & A | GUARANTEED PASS.

PEDIATRICS SYNDROMES quiz (BEST FOR REVISION) Q & A Pediatrics #1 – Clinical: Part One 1) What is the most common etiology of pediatric cardiac arrest? a) Respiratory b) Cardiac c) CNS d) Me tabolic e) Renal 2) When assessing a pediatric patient in cardiorespiratory arrest, the primary survey involves ABCDE (airway, breathing, circulation, disability, and exposure). Which of the following components of the primary survey is contraindicated in children? a) Head-tilt chin-lift b) Jaw-thrust maneuver c) Blind finger sweep d) Using heart rate to assess intravascular volume status e) Using capillary refill to assess adequate circulation 3) Which of the following is a primary treatment option for a pediatric patient in pulseless ventricular tachycardia (VT) or ventricular fibrillation (VF)? a) Cardioversion b) Amiodarone or procainamide c) Vagal maneuvers and adenosine d) Atropine and epinephrine e) Defibrillation 4) A child presents with a cardiac arrhythmia. The Emergency Department physician puts a bag of ice-cold water on the child’s face to induce the mammalian diving reflex (vagal maneuver). Which of the following did the patient likely have? a) Hemodynamically stable VT b) Hemodynamically stable SVT c) Hemodynamically unstable VT or SVT d) Pulseless VT or VF e) Bradycardia or asystole 5) Which of the following drugs would be useful for multiple ventricular ectopy (PVCs) as well as for refractory VT/VF to improve the susceptibility for cardioversion? a) Atropine b) Bicarbonate c) Calcium and magnesium d) Lidocaine e) Epinephrine 6) What is the most common type of shock seen in children? a) Hypovolemic (electrolyte loss, blood loss, third spacing) b) Cardiogenic (congenital heart disease, cardiomyopathy) c) Disruptive (anaphylactic, neurologic, drug toxicity) d) Septic, compensated (“warm”) e) Septic, uncompensated (“cold”) 7) Approximately what percentage of childhood poisonings occur in children younger than 5 years of age? a) 15% b) 35% c) 50% d) 65% e) 85% 8) A child presents with irritability, anorexia, vomiting, and hyperactivity. The clinician suspects lead poisoning and draws labs looking for >20mcg/dL, but the labs return >45mcg/dL so the clinician immediately begins chelation treatment. What is the most likely source of this child’s poisoning? a) Playground equipment b) Paint from an old building c) Men’s multivitamin tablets d) Window blinds e) Graphite pencils 9) What is the leading cause of accidental death in children older than 1 year and adolescents? a) Drowning b) Lead poisoning c) Burns d) Motor vehicle accidents e) Foreign body aspiration 10) Which of the following pediatric patients is most likely to be involved in a drowning accident (bathtub submersion)? a) Caucasian males b) Caucasian females c) African American males d) African American females 11) Foreign body aspiration has the highest incidence in children aged 6-30 months. What is the most common object involved, accounting for over 50% of aspirations? a) Small toys (e.g. lego, marble) b) Nuts c) Bread d) Crayons e) Fish bones 12) Burns are the 3rd leading cause of injury in children and the 2nd most frequent cause of accidental death. They involve abuse in 15-25% of cases. What is the most common type of burn seen in children? a) Scald burns b) Flame burns c) Electrical burns d) Contact burns e) Chemical burns 13) Which of the following is NOT true of child abuse? a) Neglect is the most common cause of failure to thrive b) Sexual abusers of children are usually stepfathers, fathers, or other male family members c) 10% of ER visits involving children younger than 5 years old result from abuse d) 80% of sexual abuse victims are girls e) Molestation by strangers and babysitters is common 14) It has been shown that home apnea monitors do not decrease the likelihood of sudden infant death syndrome (SIDS). Which of the following is NOT a risk factor for SIDS? a) Prematurity b) Low birth weight c) Female gender d) Prone sleeping position e) Intrauterine growth restriction (IUGR) f) Maternal smoking during pregnancy 15) Newborn cyanosis is most likely due to cardiac, pulmonary, neurologic, or hematologic disorders. It is clinically evident when absolute concentrations of deoxygenated hemoglobin rise above: a) 1g/dL b) 2g/dL c) 3g/dL d) 4g/dL e) 5g/dL 16) Which of the following should be given to an unstable infant with suspected congenital heart disease? a) Prostaglandin E1 (alprostadil) b) Prostaglandin E2 (dinoprostone) c) Phosphodiesterase 3 inhibitor (milrinone) d) Phosphodiesterase 4 inhibitor (mesembrine) e) Phosphodiesterase 5 inhibitor (sildenafil) 17) During a routine cardiac examination of an infant, a murmur is heard. Further examination reveals a fixed split S2, regardless of inspiration or expiration. An ECG shows mild right ventricular hypertrophy (RVH). Which of the following is most likely? a) Atrial septal defect b) Ventricular septal defect c) Patent ductus d) Tetrology of Fallot e) Coarctation of the aorta f) Transposition of the great arteries 18) Which of the following presents with a holosystolic murmur and is the most common congenital heart defect? a) Patent ductus b) Atrial septal defect c) Tetrology of Fallot d) Coarctation of the aorta e) Ventricular septal defect f) Transposition of the great arteries 19) A continuous “machinery” murmur is heart in which of the following? a) Patent ductus b) Atrial septal defect c) Tetrology of Fallot d) Coarctation of the aorta e) Ventricular septal defect f) Transposition of the great arteries 20) A young child presents with cyanosis. The mother says the child has periodic episodic cyanosis, which is accompanied by agitation and rapid, deep breathing. The child turns blue for a couple minutes then normal color returns. Chest x-ray shows a “boot shaped” heart and ECG shows right-axis deviation. Which of the following would NOT be seen in this child? a) Over-riding aorta b) Atrial septal defect c) Right ventricular hypertrophy d) Pulmonary stenosis 21) A newborn is found to have Turner syndrome. Blood pressure measurements show a difference between upper and lower extremities. Which of the following is most likely? a) Patent ductus b) Atrial septal defect c) Tetrology of Fallot d) Coarctation of the aorta e) Ventricular septal defect f) Transposition of the great arteries 22) A newborn boy presents to the Emergency Room with cyanosis. A chest x-ray shows an egg-on-a-string (egg-shaped) heart. Which of the following is most likely? a) Patent ductus b) Atrial septal defect c) Tetrology of Fallot d) Coarctation of the aorta e) Ventricular septal defect f) Transposition of the great arteries 23) Conotruncal anomalies (e.g. truncus arteriosus, tetralogy of Fallot, VSD) are associated with what chromosomal micro-deletion? a) 11p22 b) 11q22 c) 22p11 d) 22q11 24) What is the most common cardiac lesion found in rheumatic heart disease? a) Hypoplastic left heart syndrome b) Coronary artery aneurysms c) Aortic stenosis d) Mitral regurgitation e) Ventricular septal defect 25) A young child is found to have pericarditis, myocarditis, and transient rhythm disturbances. If Kawasaki disease is suspected, which of the following may develop? a) Hypoplastic left heart syndrome b) Coronary artery aneurysms c) Aortic stenosis d) Mitral regurgitation e) Ventricular septal defect 26) Endocarditis is seen in adult IV drug abusers and with prosthetic replacement valves after dental procedures. In children, it is seen with congenitally abnormal valves. What is the most common causative agent? a) Staphylococcus aureus b) Streptococcus viridans c) Haemophilus influenzae (HACEK) d) Staphylococcus epidermidis e) Coxsackie B virus 27) A child presents with fever, dyspnea, tachycardia, and mild ST-and T-wave changes on ECG. Physical exam reveals S3 ventricular gallop. If myocarditis is suspected, which of the following is most likely? a) Staphylococcus aureus b) Streptococcus viridans c) Haemophilus influenzae (HACEK) d) Staphylococcus epidermidis e) Coxsackie B virus 28) Dyspnea, orthopnea, paroxysmal nocturnal dyspnea (PND), pulsus alternans, and neuromuscular disease etiology (e.g. Duchenne Muscular Dystrophy) is seen in which of the following? a) Hypertrophic cardiomyopathy b) Dilated cardiomyopathy c) Endocarditis d) Myocarditis e) Myocardial infarction 29) A young high school basketball player suddenly collapses during a game. Which of the following is most likely? a) Hypertrophic cardiomyopathy b) Dilated cardiomyopathy c) Endocarditis d) Myocarditis e) Myocardial infarction 30) Which of the following developmental tests evaluates children from 0 to 6 years of age and involves gross motor, fine motor-adaptive, language, and personal-social? a) Denver II b) CAT c) CLAMS d) B & C 31) What is the best indicator of future intellectual achievement? a) Gross motor b) Fine motor-adaptive c) Language d) Personal-social 32) A development quotient below what level would constitute developmental delay? a) 100 b) 90 c) 80 d) 70 e) 60 33) Which of the following intelligence quotients (IQs) would signify severely mentally retarded? a) 80 b) 60 c) 45 d) 30 e) 10 34) Which of the following is NOT true of attention deficit hyperactivity disorder (ADHD)? a) Most of those affected as a child do not have persisting symptoms b) Involves hyperactivity, inattention, and impulsivity c) Requires information from parents and teachers using a scale such as the Conner Parent and Teacher Scales d) Symptoms must be present for at least 6 months e) Treatment may include drugs such as methylphenidate (Ritalin), dextroamphetamine (Dexedrine) and mixed amphetamine salts (Adderall) 35) A child is able to balance on one foot, can copy a cross, can catch a ball, can dress himself, and tells an intelligible story to a stranger. How old is this child? a) 1-year b) 2-years c) 3-years d) 4-years e) 5-years 36) A child is able to discriminate the use of “mama” and “dada”, stands alone, cooperates with dressing, and imitates actions. How old is this child? a) 36-months b) 24-months c) 12-months d) 6-months e) 2-months 37) A child can roll in both directions, sits still, reaches with one hand, and babbles. How old is this child? a) 2-months b) 4-months c) 6-months d) 9-months e) 12-months 38) Autism and Asperger syndrome are pervasive development disorders (PDD). Asperger is characterized by intense interest in specific topics (e.g. dinosaurs) as well as which of the following? a) Lack of language ability b) Lack of showing interest c) Lack of emotional reciprocity d) Lack of abstract form understanding (e.g. sarcasm) e) Lack of motor mannerisms and inflexibility to rituals 39) The measles-mumps-rubella (MMR) vaccine and thimerosal have been shown to be associated with the development of autism. a) True b) False Match the disease with the causative agent: 40) Measles a) Parvovirus B19 41) Rubella b) Paramyxovirus 42) Roseola infantum c) Varicella-Zoster virus 43) Fifth disease (erythema infectiosum) d) RNA Togavirus 44) Hand-Foot-and-Mouth e) HHV-6 45) Chicken pox f) Coxsackie A virus Match the disease with the type of rash: 46) Measles a) Mouth ulcers, football shaped vesicles 47) Rubella b) Pruritic rash, red papules, vesicles, scabs 48) Roseola infantum c) Maculopapular on trunk to periphery 49) Fifth disease (erythema infectiosum) d) Discrete maculopapular, <5 days 50) Hand-Foot-and-Mouth e) Koplik spots, maculopapular at head 51) Chicken pox f) Slapped-cheek, arms spread to trunk 52) An infant presents with abrupt onset of diffuse erythema, marked skin tenderness, and fever. Flaccid bullae develop and rupture, leading beefy red, weeping surfaces. Physical exam reveals separation of the epidermis on light rubbing (Nikolsky sign). Labs show S. aureus as the causative agent. Which of the following is most likely? a) Bullous impetigo b) Nonbullous impetigo c) Scalded skin syndrome d) Folliculitis e) Cellulitis 53) A child presents with a hyperpigmented scaly lesion on the upper back, chest, and neck. Labs are negative for septated branching hyphae. However, a “spaghetti and meatballs” arrangement is seen on microscopy. Further lab testing shows Malassezia furfur. Which of the following is most likely? a) Tinea capitis b) Tinea corporis c) Tinea cruris d) Tinea pedis e) Tinea versicolor 54) A boy with oily skin presents with complaints of facial blemishes that began as he reached puberty. The clinician believes Propionibacterium acnes is to blame. What treatment option is reserved for severe cases that involve inflammatory papules, cysts, abscesses, and scaring? a) Benzoyl peroxide b) Topical antibiotics (clindamycin) c) Topical retinoids (tretinoin) d) Oral antibiotics (tetracycline) e) Oral retinoic acid (Accutane) 55) A child is found to have a silvery scale rash on areas of physical or thermal trauma (Köbner phenomenon). The child scratches at the rash, which causes pinpoint bleeding (Auspitz sign). Which of the following is most likely? a) Acne b) Tinea corporis c) Folliculitis d) Psoriasis e) Bullous impetigo 56) Which of the following is NOT characteristic of diabetic ketoacidosis? a) Type II diabetes b) Polyuria, polydipsia c) Kussmaul breathing d) Tachycardia and hypotension e) Fruity breath and cerebral edema 57) An infant girl is found to have short statue and deformities of the neck, chest, and heart. Physical exam reveals a knuckle-knuckle-dimple-dimple sign. Which of the following is most likely? a) Central diabetes insipidus b) Growth hormone deficiency c) Primary hypothyroidism d) Cushing syndrome e) Turner syndrome 58) What is the most common cause of hyperthyroidism in children? a) Hashimoto thyroiditis b) Graves disease c) Toxic multinodular goiter d) Amiodarone toxicity e) Knuckle-knuckle-dimple-knuckle pseudohypothyroidism 59) What is the most common cause of congenital adrenal hyperplasia? a) 21-hydroxylase deficiency b) 17alpha-hydroxylase deficiency c) Aromatase deficiency d) 11beta-hydroxylase deficiency e) 3beta-hydroxylase deficiency 60) Precocious puberty is defined as development of secondary sex characteristics before age 7.5 in girls and before what age in boys? a) 5 b) 6 c) 7.5 d) 9 e) 10 61) A child presents with weakness, nausea, vomiting, and weight loss. History reveals salt cravings. Physical exam reveals increased skin pigmentation and postural hypotension. Lab testing shows hyponatremia, hyperkalemia, hypoglycemia, and metabolic acidosis. Which of the following is most likely? a) Cushing syndrome b) Cushing disease c) Addison disease d) Pubertal delay e) Pseudopseudohypothyroidism 62) What is the Holliday-Segar method used for? a) Fluid bolus calculation in burn victims b) Fluid infusion rate for traumatic injuries c) Fluid amount for daily maintenance d) Fluid allowance for diabetes insipidus e) Fluid loss rate for dehydration 63) A child presents with suspected dehydration due to immature kidneys. Evaluation shows a depressed anterior fontanelle, sunken eyes, altered mental status, and increased heart rate. It is estimated that the child has lost 7% of their body weight to water. Labs reveal 800mOsm/L urine, slightly elevated urine specific gravity, elevated BUN, and mild acidosis. Which of the following best describes this patient? a) Normal b) Mild shock c) Compensated shock d) Uncompensated shock 64) The hemolysis of red blood cells during sample collections is the most common causes of (artifactual): a) Hyponatremia b) Hypernatremia c) Hypokalemia d) Hyperkalemia 65) A child presents with constipation after a bout of severe vomiting. The clinician notes weakness and tetany. An ECG shows depressed ST segments with biphasic T-waves and prominent U-waves. Which of the following is most likely? a) Hyponatremia b) Hypernatremia c) Hypokalemia d) Hyperkalemia 66) A child with a congenital renal tubule defect has large losses of bicarbonate. Labs reveal a blood pH of 7.20. Which of the following is most likely? a) Metabolic acidosis b) Metabolic alkalosis c) Respiratory acidosis d) Respiratory alkalosis Pediatrics #2 – Clinical: Part Two 1) What is the most common medical cause of abdominal pain in children? a) Sickle cell disease b) Lactase deficiency c) Gastroenteritis d) Mesenteric lymphadenitis e) Group A strep infections 2) What is the most common surgical cause of abdominal pain in children? a) Trauma b) Intussusception c) Cholecystitis d) Appendicitis e) Testicular torsion 3) Which of the following cases would suggest colicky pain or obstruction? a) Blood or mucinous diarrhea b) Child lies still c) Child cannot remain still d) Sore throat e) Dysuria 4) Which of the following cases would suggest inflammatory pain, infection, or perforated organ/viscus? a) Blood or mucinous diarrhea b) Child lies still c) Child cannot remain still d) Sore throat e) Dysuria 5) An infant presents with violent episodes of irritability, colicky pain, and emesis that are interspersed with relatively normal periods. A barium enema is performed, showing a coiled-spring appearance to the bowel. Which of the following is most likely? a) Trauma b) Intussusception c) Cholecystitis d) Appendicitis e) Testicular torsion 6) Which of the following is an uncommon cause of emesis in children? a) Crohn disease b) Gastroesophageal reflux c) Acute gastroenteritis d) Tonsillitis e) Otitis media 7) A three-week-old child presents with non-bilious projectile vomiting. Which of the following is most likely? a) Gastroesophageal reflux b) GI malrotation c) Pyloric stenosis d) Hirschsprung disease e) Esophageal stricture 8) A newborn is brought in with gastrointestinal problems. An upper GI series is performed, showing abnormal positioning of the ligament of Treitz and the cecum. Which of the following is most likely? a) Gastroesophageal reflux b) GI malrotation c) Pyloric stenosis d) Hirschsprung disease e) Esophageal stricture 9) What is the most common cause of acute diarrhea in children within most developed countries? a) Bacterial enterocolitis b) Appendicitis c) Iron, mercury, lead, or fluoride ingestion d) Intussusception e) Viral gastroenteritis 10) Hirschsprung disease (i.e. congenital megacolon) results from failure of ganglion cells of the myenteric plexus to migrate down to the distal colon. This results in: a) Constipation b) Diarrhea c) Hematochezia d) Obstipation 11) Which of the following is defined as bright red blood per rectum (BRBPR) and usually results from a lower GI bleed, distal to the ligament of Treitz? a) Constipation b) Diarrhea c) Hematochezia d) Obstipation e) Melena 12) Which of the following is the most common anomaly of the GI tract, is due to vestigial remnant of the omphalomesenteric duct (within 100cm of the ileocecal valve), and presents with painless rectal bleeding? a) Gastroschisis b) Meckel diverticulum c) Hirschsprung disease d) Omphalocele e) Diaphragmatic hernia 13) Which of the following is NOT true of congenital defects? a) Autosomal dominant disorders typically code for structural proteins b) Autosomal recessive disorders typically code for enzymes c) Environmental factors cause 10% of birth defects d) Major anomalies have a low incidence up to newborns with the presence of three minor anomalies e) Ornithine transcarbamylase deficiency (OTC), like all other inborn errors of metabolism, is autosomal recessive Match the genetic defect with the characteristics: 14) Horseshoe kidney, rocker-bottom feet, low-set ears a) Downs (21) 15) Gynecomastia, small phallus, small testes, taller b) Edwards (18) 16) Wide-spaced hypoplastic nipples, webbed neck, shield chest c) Patau (13) 17) High leukemia risk, short, broad hands, dysmorphic face d) Turner 18) Microcephaly, sloped forehead, cryptorchidism, polydactyly e) Klinefelter 19) A mother brings in her young boy because he is gaining weight rapidly. She has been trying to keep food away from him, but finds him digging through the garbage and eating non-edible items (pica). Physical exam reveals almond-shaped eyes that are different from the mother’s, a down-turned mouth, and small hands and feet. Which of the following is most likely? a) Maternal chromosome 15 deletion b) Paternal chromosome 15 deletion c) Trisomy 21 d) Trisomy 18 e) Trisomy 13 20) A girl is brought in by her mother with the concern that the child is not learning properly. Physical exam reveals maxillary hypoplasia, a large mouth, prognathism, and short stature. Mental assessment reveals the child is severely mentally retarded. During a discussion with the mother, the clinician notes the child making jerky arm movements, marionette-like movements, and laughing a great deal (happy puppet syndrome). Which of the following is most likely? a) Maternal chromosome 15 deletion b) Paternal chromosome 15 deletion c) Deletion of 22q11 d) Deletion of 22p11 e) Trisomy 21 21) A newborn is found to have macrosomia at birth. Testing as the child begins to grow reveals macroorchidism, large ears, and a large jaw. Genetic testing reveals a large amount of CGG trinucleotide repeats. Which of the following is most likely? a) Angelman syndrome b) Prader-Willi syndrome c) Fragile X syndrome d) DiGeorge syndrome e) Hurler syndrome 22) Microdeletion of 22q11.2 can lead to tetralogy of Fallot, interrupted aortic arch, and thymus absence. It is associated with velocardiofacial syndrome as well as: a) Angelman syndrome b) Prader-Willi syndrome c) Fragile X syndrome d) DiGeorge syndrome e) Hurler syndrome 23) A 2-week-old presents with vision problems, anorexia, and emesis after breast- feeding. Testing reveals liver failure, renal dysfunction, and developing cataracts. A deficiency in G1P uridyltransferase is found. The clinician is concerned about the risk of E. coli sepsis. Which of the following is most likely? a) OTC deficiency b) Gaucher disease c) Hurler syndrome d) Phenylketonuria e) Galactosemia f) Homocystinuria Match the glycogen storage disease with the deficiency: 24) Acid alpha-glucosidase (GAA) a) Von Gierke disease (Type I) 25) Glucose-6-phosphate (G6P) b) Pompe disease (Type II) 26) Myophosphorylase c) McArdle disease (Type V) 27) A fair-haired, fair-skinned, blue-eyed child presents with projectile vomiting, mental retardation, tremors, and mouse-like smelling urine. Testing reveals a defect in tyrosine formation. Which of the following is most likely? a) OTC deficiency b) Gaucher disease c) Hurler syndrome d) Phenylketonuria e) Galactosemia f) Homocystinuria 28) Which of the following can cause eye lens dislocation and involves a defect in the pathway that converts methionine to cysteine and serine? a) OTC deficiency b) Gaucher disease c) Hurler syndrome d) Phenylketonuria e) Galactosemia f) Homocystinuria 29) A patient is diagnosed with OTC deficiency after developing severe hyperammonemia after the consumption of proteins. How is this disorder inherited? a) Autosomal dominant b) Autosomal recessive c) X-linked d) Y-linked e) Mitochondrial 30) Which of the following is due to a deficiency in alpha-iduroindase leading to dermatan and heparan sulfate accumulation, coarse facies, corneal clouding, and exaggerated kyphosis? a) OTC deficiency b) Gaucher disease c) Hurler syndrome d) Phenylketonuria e) Galactosemia f) Homocystinuria 31) Which of the following is due to a deficiency in beta-glucosidase leading to glucocerebrosidase accumulation, anemia, leucopenia, and an Erlenmeyer flask-shape of the distal femur? a) OTC deficiency b) Gaucher disease c) Hurler syndrome d) Phenylketonuria e) Galactosemia f) Homocystinuria 32) An infant presents with anorexia, apathy, easy fatigability, and irritability. Physical exam reveals skin pallor, glossitis, and koilonychias. Labs reveal iron deficiency. Which of the following is most likely? a) Hypochromic microcytic anemia b) Hyperchromic microcytic anemia c) Hypochromic macrocytic anemia d) Hyperchromic macrocytic anemia e) Normocytic anemia 33) During the first year of life, a child develops severe hemolytic anemia and splenomegaly. A physical exam reveals a tower skull, frontal bossing, and prominent cheekbones. Labs are negative for excess beta-globin tetramers. The child is started on folate supplementation, RBC transfusions, and iron chelation. Which of the following is most likely? a) Homozygous alpha thalassemia (Bart) b) Hemoglobin H disease c) Alpha thalassemia minor d) Beta thalassemia major e) Beta thalassemia minor 34) A 2-year-old develops bone marrow suppression after a viral infection. Pure red cell aplasia is found and the child is diagnosed with transient erythroblastopenia of childhood (TEC). The mother is told it will pass. What type of anemia is this? a) Hypochromic microcytic anemia b) Hyperchromic microcytic anemia c) Hypochromic macrocytic anemia d) Hyperchromic macrocytic anemia e) Normocytic anemia 35) An African-American child presents with pain in the hands, knees, and a general aching sensation. Physical exam reveals splenomegaly, dactylitis, and pulmonary rales. Labs show anemia. The father has a history of some type of RBC disorder. Which of the following does the child most likely have? a) Beta-globin position 4 substitution of glutamine for valine b) Beta-globin position 4 substitution of valine for glutamine c) Beta-globin position 6 substitution of glutamine for valine d) Beta-globin position 6 substitution of valine for glutamine 36) A blood test on a child reveals Heinz bodies. Further testing reveals G6PD, which is a lack of the hexose monophosphate shunt pathway enzyme that results in a depletion of NADPH and inability to regenerate reduced glutathione. How is this disorder inherited? a) Autosomal dominant b) Autosomal recessive c) X-linked d) Y-linked e) Mitochondrial 37) A child is brought to the emergency center with traumatic hemorrhages after a soccer game. Physical exam reveals significant knee hemarthroses. Further testing reveals an X- linked recessive disorder involving factor VIII deficiency. Which of the following is most likely? a) Sickle cell disease b) Alpha thalassemia c) Hemophilia A d) Hemophilia B e) Von Willebrand disease 38) A child presents with complaints of bleeding gums when she brushes her teeth and even when she eats. Testing reveals a deficiency in von Willebrand factor. What is the drug of choice for this patient for bleeding episodes? a) Vitamin K b) Clopidogrel c) Desmopressin acetate d) Cryoprecipitate e) Fresh-frozen plasma 39) What clotting factor is deficient in hemophilia B? a) Factor V b) Factor VII c) Factor VIII d) Factor IX e) Factor X 40) Which of the following is NOT true? a) Hypocalcemic tetany with absence of the thymic shadow and cell-mediated immunodeficiency suggests 22q11 deletion b) Humoral immunodeficiency predisposes patients to infections with encapsulated organisms (e.g. H. influenzae, S. pneumonia) c) Cell-mediated immunodeficiency predisposes patients to autoimmune disorders, intracellular organisms, and opportunistic infections d) Typical signs of infections are often absent in the presence of neutropenia e) The most common immunodeficiency seen in pediatrics is cell-mediated 41) A patient presents with recurrent infections involving catalase-producing bacteria (S. aureus, C. albicans, Aspergillus). A nitroblue tetrazolium test (NBT) and dihydrorhodamine reduction (DHR) test are positive for the detection of chronic granulomatous disease (CGD). Along with interferon-gamma, what prophylactic daily drug should this patient receive? a) Penicillin G b) Amphotericin B c) Doxycycline d) TMP-SMX e) Isoniazid 42) A child presents with seasonal rhinorrhea and upper respiratory tract symptoms. He has dark circles under his eyes and a horizontal crease across the middle of the nose. A Type I hypersensitivity response is suspected. What is the treatment of choice? a) Sedating H1 blockers (e.g. diphenhydramine) b) Non-sedating H1 blockers (e.g. fexofenadine) c) H2 blocker (e.g. famotidine) d) Corticosteroid (e.g. prednisone) e) Antibiotic (e.g. amoxicillin) 43) What is the gold standard for diagnosis of food allergies, such as to peanuts, eggs, milk, soy, wheat, or fish? a) Patient history b) Allergy scratch test c) Allergy blood panel d) H1 suppression test e) Double-blind placebo-controlled food challenge 44) A 13-year-old girl presents with joint pain lasting for 2-months. She admits to joint stiffness in the morning that resolves with movement. Her mother says she has been eating less, fatigues easier, and is irritable. Physical exam reveals a rash and limited range of motion in the affected joints. Which of the following is most likely? a) Systemic lupus erythematosus (SLE) b) Polyarteritis nodosa (PAN) c) Kawasaki disease d) Juvenile rheumatoid arthritis (JRA) e) DiGeorge syndrome 45) A late adolescent presents with a malar facial rash, photosensitivity, and painless oral ulcerations. Testing reveals anemia, positive ANA, and signs of a type III hypersensitivity reaction. What is the treatment of choice? a) Sedating H1 blockers (e.g. diphenhydramine) b) Non-sedating H1 blockers (e.g. fexofenadine) c) H2 blocker (e.g. famotidine) d) Corticosteroid (e.g. prednisone) e) Antibiotic (e.g. amoxicillin) 46) PAN usually presents with waxing and waning symptoms of painful erythematous skin nodules, purpura, hypertension, hematuria, and systemic complaints. Henoch- Schönlein purpura is an IgA-mediated vasculitis that peaks in winter months, is usually preceded by a group A streptococcal upper respiratory infection, and causes non- thrombocytopenic palpable purura. What is the treatment of choice for both of these? a) Sedating H1 blockers (e.g. diphenhydramine) b) Non-sedating H1 blockers (e.g. fexofenadine) c) H2 blocker (e.g. famotidine) d) Corticosteroid (e.g. prednisone) e) Antibiotic (e.g. amoxicillin) 47) An infant presents with high fever, lymphadenopathy, and mucocutaneous lesions. Physical exam reveals bilateral conjunctivitis, dry fissured lips, strawberry tongue, indurative edema of the feet, and truncal polymorphous rash. Kawasaki disease is suspected. What is the treatment of choice for the convalescent phase? a) Acetaminophen b) Aspirin c) Indomethacin d) Ibuprofen e) Hydrocodone 48) Fever of unknown origin (FUO) implies a fever greater than 38.3C degrees (101F) for equal to or greater than how many days? a) 3 days b) 5 days c) 7 days d) 14 days e) 21 days 49) The most common bacteria implicated in acute otitis media (AOM) in pediatrics are S. pneumoniae, H. influenzae, and M. catarrhalis. What is the treatment of choice? a) Amoxicillin b) Doxycycline c) Penicillin d) Ketoconazole e) Ceftriaxone 50) In general, children with pharyngitis should not be treated with antibiotics empirically as most episodes are viral. If Group A Streptococcus (GAS) is suspected, what is the drug of choice? a) Amoxicillin b) Doxycycline c) Penicillin d) Ketoconazole e) Ceftriaxone 51) A 14-year-old boy presents with extreme fatigue. Testing shows fever, generalized lymphadenopathy, atypical lymphocytes, and a positive heterophile antibody test. Epstein-Barr virus is suspected. Which of the following is most likely? a) Croup b) Epiglottitis c) Mononucleosis d) Bronchiolitis e) Pertussis 52) A child arrives at the Emergency Department with a hoarse voice and barky seal-like cough. Physical exam reveals stridor and the clinician orders steroids and nebulized epinephrine. Parainfluenza virus is suspected. Which of the following is most likely? a) Croup b) Epiglottitis c) Mononucleosis d) Bronchiolitis e) Pertussis 53) What is the treatment of choice for epiglottitis? a) Supportive only b) IV epinephrine c) IV prednisone d) IV amoxicillin e) Endotracheal intubation 54) Bronchiolitis is characterized by 5-10 days of wheezing, rhonchi, and crackles typically between November and April. Prophylactic use of palivizumab may be beneficial. What is the most common cause of bronchiolitis in children? a) Influenza virus b) Parainfluenza virus c) Respiratory syncytial virus d) Epstein-Barr virus e) S. pneumoniae 55) A child presents to the Emergency Department with a long, stridorous inspiration after a paroxysmal “whooping” cough. The child is started on erythromycin estolate, a macrolide. Which of the following is most likely? a) Croup b) Epiglottitis c) Mononucleosis d) Bronchiolitis e) Pertussis 56) A patient is asked to come into her obstetrician at 35-weeks of gestation to get a bacteria test. The test will help determine if she will receive penicillin during labor to prevent neonatal pneumonia and meningitis. Which of the following is being tested for? a) Streptococcus pneumoniae b) Haemophilus influenzae c) Mycoplasma pneumoniae d) Group B strep (S. agalactiae) e) Group D strep (Enterococcus) 57) An adolescent child (school age) presents with photophobia, fever, and neck pain. Testing reveals a positive Brudzinski sign and a positive Kernig test. Which of the following is most likely? a) Streptococcus pneumoniae b) Haemophilus influenzae c) Mycoplasma pneumoniae d) Group B strep (S. agalactiae) e) Group D strep (Enterococcus) 58) Bacterial meningitis involving S. pneumoniae or H. influenzae is most commonly seen in what population? a) Neonates b) Children ages 3-6 c) Children ages 6-14 d) Teenagers e) Adults 59) What is the most common cause of gastroenteritis in infants and toddlers? a) Giardia lamblia b) Campylobacter jejuni c) Yersinia enterocolitica d) Norwalk virus e) Rotavirus Match the form of gastroenteritis with the treatment: 60) Shigellosis a) No antibiotics unless systemic 61) C. jejuni b) Stop antibiotics, metronidazole 62) Salmonella c) TMP/SMX 63) C. difficile d) Erythromycin 64) Giardiasis e) Metronidazole 65) A child from the South Atlantic U.S. presents with a headache and rash that began about a week after a hike in the woods. The rash began on the wrists and ankles and spread proximally to the trunk. What is the drug of choice? a) Amoxicillin b) Ceftriaxone c) Penicillin d) Doxycycline e) Clindamycin 66) A five-year-old child is brought in with multiple erythema migrans lesions. Physical exam reveals lymphadenopathy and cranial nerve palsy. ECG shows a slight AV block. History reveals the child’s brothers brought him into the Minnesota woods to go hunting, but the child was never examined for ticks after the trip. What is the initial drug of choice for this child, who is younger than 8-years-old? a) Amoxicillin b) TMP/SMX c) Ketoconazole d) Doxycycline e) Clindamycin Pediatrics #3 – Clinical: Part Three 1) What is the Apgar (APGAR) score for a child with a heart rate of 90, irregular and weak cry, cyanotic extremities, weak and slightly flexed extremities, and grimacing facial expression? a) 0 b) 3 c) 5 d) 7 e) 9 2) Which of the following forms of birth trauma involves a diffuse, edematous, and often dark swelling of the soft tissues of the scalp that extends across the midline and/or suture lines? a) Erb palsy b) Fractured clavicle c) Klumpke palsy d) Caput succedaneum e) Cephalhematoma 3) A newborn presents with an arm that is extended, internally rotated, and flexed at the wrist. Moro reflex of the right arm is absent but right hand grasp is intact. Which of the following is most likely? a) Erb palsy b) Fractured clavicle c) Klumpke palsy d) Caput succedaneum e) Cephalhematoma 4) Very low birth weight (VLBW) is defined as less than: a) 1,500g b) 2,500g c) 3,500g d) 4,500g e) 5,500g 5) Which of the following is NOT common in postmature (>42 weeks) infants? a) Meconium aspiration b) Persistent pulmonary hypertension c) Hyperglycemia d) Hypocalcemia e) Polycythemia 6) Which of the following congenital infections presents with cataracts, “blueberry muffin” skin syndrome, vertical bone striation, and patent ductus? a) Toxoplasma gondii b) Treponema pallidum c) Rubella d) Cytomegalovirus e) Herpes simplex 7) Which of the following congenital infections is the most common and presents with skin vesicles or denuded skin, keratoconjunctivitis, and seizures? a) Toxoplasma gondii b) Treponema pallidum c) Rubella d) Cytomegalovirus e) Herpes simplex 8) Early-onset neonatal sepsis usually involves bacteria that colonize the mother’s genitourinary tract. On the other hand, nosocomial-acquired sepsis involves drug- resistant bacterial pathogens that are more commonly seen in the neonatal intensive care unit (NICU), such as: a) Group B strep (agalactiae) b) E. coli c) Klebsiella d) S. aureus e) L. monocytogenes 9) Respiratory distress syndrome (RDS) involves the formation of a hyaline membrane (hazy ground-glass appearance chest radiograph) and a deficiency in surfactant. Measuring the lecithin-to-sphingomyelin ratio can help predict RDS. What is the major factor predisposing a newborn to RDS? a) Birth involving meconium b) Breech presentation c) Delayed cord clamping d) Pre-maturity (<34 weeks) e) Post-maturity (>45 weeks) 10) What is the most common cause of neonatal unconjugated hyperbilirubinemia? a) Post-birth UV-light exposure b) Hemolytic disorder c) Bacterial sepsis d) Extrahepatic obstruction e) Genetic disorder (e.g. Rotor, Dubin-Johnson) 11) Necrotizing enterocolitis (NEC) is seen in premature infants. Which of the following is the most common cause of neonatal polycythemia? a) Birth involving meconium b) Breech presentation c) Delayed cord clamping d) Pre-maturity (<34 weeks) e) Post-maturity (>45 weeks) 12) A newborn has a positive Coombs test, spherocytes, increased bilirubin, and increased reticulocytes. Which of the following is most likely? a) Anemia of infancy b) Intraventricular hemorrhage c) Immune hemolysis d) Hereditary spherocytosis e) Glucose-6-phosphate dehydrogenase deficiency 13) A preterm infant is found to have bleeding of the germinal matrix, an area of immature vasculature that is the site of pluripotent cells that migrate to form neurons and glia. Which of the following is most likely? a) Anemia of infancy b) Intraventricular hemorrhage c) Immune hemolysis d) Hereditary spherocytosis e) Glucose-6-phosphate dehydrogenase deficiency 14) What is the drug of choice for neonatal seizures? a) Phenobarbital b) Succinylcholine c) Midazolam d) Fentanyl e) Rohypnol 15) All states require newborn screening for hypothyroidism. Which of the following would be suggestive of primary hypothyroidism? a) Low T4 level and low TSH level b) Low T4 level and high TSH level c) High T4 level and low TSH level d) High T4 level and high TSH level 16) Which of the following describes infants with Beckwith-Wiedemann syndrome or islet cell adenomas? a) Transient hypoglycemia b) Protracted hypoglycemia c) Transient hyperinsulinemia d) Protracted hyperinsulinemia 17) What is the most common type of tracheoesophageal fistula (TEF)? a) Distal TEF with esophageal atresia b) H-type TEF without esophageal atresia c) Proximal TEF with esophageal atresia d) Proximal and distal TEF with esophageal atresia e) Esophageal atresia without TEF (no air in GI tract) 18) A double bubble sign on radiograph is indicative of: a) Distal TEF b) Proximal TEF c) Gastric atresia d) Duodenal atresia e) Bile duct atresia 19) What defect is seen here? a) Gastroschisis b) Meckel diverticulum c) Hirschsprung disease d) Omphalocele e) Diaphragmatic hernia 20) Failure of the tongue to descent by the 9th week of gestation causes: a) Unilateral cleft lip b) Bilateral cleft lip c) Midline cleft palate d) Lateral cleft palate 21) Which of the following are transient dark blue-black pigmented macules over the lower back and buttocks that are sharply demarked (do not fade into surrounding skin)? a) Child abuse bruises b) Erythema toxicum neonatorum c) Seborrheic dermatitis d) Mongolian spots 22) A newborn with IUGR is born with microcephaly and mental retardation. Physical exam reveals mid-facial hypoplasia, micrognathia, and a flattened philtrum. Which of the following did the mother likely use during pregnancy? a) Cocaine b) Heroine c) Methamphetamine d) Marijuana e) Alcohol 23) Infants of narcotic-abusing mothers should never be given naloxone (Narcan) in the delivery room as it can precipitate: a) Seizures b) Myocardial infarction c) Stroke d) Respiratory distress syndrome e) Ascites 24) Multicystic kidney is always unilateral and is the most common type of renal dysplasia. It consists of numerous non-communicating, fluid filled cysts, and a diagnosis is best confirmed by: a) MRI b) CT scan c) Abdominal x-ray d) Ultrasound e) Barium study 25) What is the most common cause of hydronephrosis in childhood? a) Ureteropelvic junction obstruction b) Ureterovesical junction obstruction c) Autosomal dominate polycystic kidney disease d) Autosomal recessive polycystic kidney disease e) Posterior urethral valves 26) What is the treatment of choice for infants with recurrent UTIs caused by vesicoureteral reflex (VUR)? a) Nitrofurantoin b) TMP/SMX c) Amoxicillin d) Ciprofloxacin e) Ceftriaxone 27) What is the most common cause of end-stage renal disease in childhood? a) Ureteropelvic junction obstruction b) Ureterovesical junction obstruction c) Autosomal dominate polycystic kidney disease d) Autosomal recessive polycystic kidney disease e) Posterior urethral valves 28) During an exam of an adolescent boy, the left side of the scrotal sac appears like a bag of worms. Palpation reveals tortuous veins. Which of the following is most likely? a) Varicocele b) Hydrocele c) Testicular torsion d) Hypospadias e) Cryptorchidism 29) What is the most common pathogen seen in pediatric UTIs? a) S. aureus b) C. difficile c) E. coli d) H. influenzae e) S. saprophyticus 30) A child is found to have extreme proteinuria, hypoalbuminemia, hyperlipidemia, and edema. What is the most common cause of primary nephrotic syndrome in the pediatric population? a) Minimal change disease b) Cystic renal dysplasia c) Membranous glomerulopathy d) Acute post-streptococcal glomerulonephritis e) Membranoproliferative glomerulonephritis 31) A child presents with hematuria that is overt on microscopic examination and contains red cell casts. What is the most common glomerulonephritis seen in childhood? a) Minimal change disease b) Cystic renal dysplasia c) Membranous glomerulopathy d) Acute post-streptococcal glomerulonephritis e) Membranoproliferative glomerulonephritis 32) A child presents with painless hematuria and sensorineural hearing loss. Damage to type IV collagen is suspected (Alport syndrome). How is this disorder inherited? a) Autosomal dominant b) Autosomal recessive c) X-linked d) Y-linked e) Mitochondrial 33) Which of the following is the most common type of renal tubular acidosis (RTA) seen in children and adults? a) Distal Type I b) Distal Type IV c) Proximal Type II with Fanconi syndrome d) Proximal Type II without Fanconi syndrome 34) A patient is found to have a low serum pH and low serum bicarbonate. A workup for hyperchloremic metabolic acidosis is begun. The patient’s urine anion gap (Na+ + K+ - Cl-) is found to be positive. The patient is hyperkalemic and has a urine pH < 5.5. Which of the following describes this patient? a) Normal b) Proximal RTA (type 2) c) Distal RTA (type 1) d) Distal RTA (type 4) e) None of the above 35) Which of the following would cause diabetes insipidus (DI)? a) Excess ADH release b) Inability to release ADH c) Excess ACTH release d) Inability to release ACTH e) Hyperglycemia 36) What is the most common form of acute renal failure (ARF) in the pediatric population? a) Pre-renal b) Intra-renal (intrinsic) c) Post-renal 37) What is the prophylaxis for preventing neural tube defects, which may be diagnosed by an increase in maternal alpha-fetoprotein? a) Niacin b) Diabetes control c) Electrolyte balance d) Sodium control e) Folic acid 38) A newborn is found to have a tuft of hair on the lower back. Testing shows an underlying bony vertebral lesion without herniation of any spinal contents. Which of the following is most likely? a) Myelomeningocele b) Meningocele c) Spina bifida occulta d) Arnold-Chari malformation 39) A child is found to have an Arnold-Chari malformation and begins developing signs of hydrocephalus. Which of the following is NOT a component of the Cushing triad? a) Upward gaze paralysis (“setting sun” sign) b) Bradycardia c) Hypertension d) Cheyne-Stokes respiration 40) Which of the following is the term for bilateral lower extremity spasticity, which may be seen in cerebral palsy? a) Diplegia b) Hemiplegia c) Quadriplegia d) Seizure 41) A child is bought in for neurologic screening after the mother finds him “blanking out” for a few seconds. She says the boy will be talking, stop taking, then pick back up at the same part of the sentence about 5 seconds later. EEG during one of these episodes shows a characteristic 3-per-second spike and wave pattern. Which of the following is most likely? a) Generalized seizure b) Petit mal seizure c) Infantile spasm d) Febrile seizure 42) Reye syndrome is characterized by acute-onset encephalopathy and degenerative liver disease when what drug is given following a viral illness (e.g. chicken pox)? a) Acetaminophen b) Aspirin c) Ibuprofen d) Naproxen e) Indomethacin 43) A child presents with complaints of weakness. History reveals a viral illness about a week earlier. The weakness has been progressive, ascending, and of acute onset. Testing reveals likely autoimmune-mediated demyelination of peripheral nerves. Plasmapheresis is started to hasten resolution. Which of the following is most likely? a) Duchenne muscular dystrophy (DMD) b) Becker muscular dystrophy c) Guillain-Barré syndrome d) Myasthenia gravis (MG) e) Multiple sclerosis (MS) 44) Which of the following is an autoimmune disorder of the neuromuscular junction with autoantibodies binding to postsynaptic Ach receptors? a) Duchenne muscular dystrophy (DMD) b) Becker muscular dystrophy c) Guillain-Barré syndrome d) Myasthenia gravis (MG) e) Multiple sclerosis (MS) 45) A child presents with motor difficulties. Physical exam reveals hypertrophy of the calves. Watching the child stand up involves them pushing their trunk up with their arms, then standing (Gower sign). An X-linked recessive disorder is suspected. Which of the following is most likely? a) Duchenne muscular dystrophy (DMD) b) Multiple sclerosis (MS) c) Guillain-Barré syndrome d) Myasthenia gravis (MG) e) Spinal muscle atrophy (SMA) 46) Which of the following primarily affects the anterior horn cell of the spine and has mostly been eradicated due to a killed virus vaccination? a) Duchenne muscular dystrophy (DMD) b) SMA Type I (Werdnig-Hoffman disease) c) SMA Type II d) Poliomyelitis e) Guillain-Barré syndrome 47) Hydrocephalus is most associated with: a) Head size two standard deviations below mean (microcephaly) b) Head size one standard deviation below mean c) Abnormally shaped head (craniosynostosis) d) Head size one standard deviation above mean e) Head size two standard deviations above mean (macrocephaly) 48) Failure to thrive (FTT) is defined as weight below what percentile? a) 10th b) 7th c) 5th d) 3rd e) 1st 49) Pediatric obesity is defined as weight above what percentile? a) 99th b) 97th c) 95th d) 93rd e) 90th 50) What is the most common cause of amblyopia in children? a) Retinal trauma b) Strabismus c) Retinoblastoma d) Congenital cataract e) Retinopathy of prematurity (ROP) 51) What is the most common intraocular malignancy of childhood, which presents with leukocoria? a) Malignant melanoma b) Neuroblastoma c) Retinoblastoma d) Metastatic adenocarcinoma e) Ocular carcinoma 52) What is the most common cause of leukocoria in children? a) Retinal trauma b) Strabismus c) Retinoblastoma d) Congenital cataract e) Retinopathy of prematurity (ROP) 53) Fluorescein drops with a blue-filter light would most likely be used in which of the following cases? a) Corneal abrasions b) Retinoblastoma c) Congenital cataracts d) Retinopathy of prematurity e) Malignant melanoma 54) Steroid drops should NOT be given in which of the following causes of conjunctivitis? a) Neisseria gonorrhoeae b) Corynebacterium c) Streptococci d) Staphylococci e) HSV1 55) An infant presents with suspected developmental dysplasia of the hip (DHH). A Barlow maneuver is positive and an Ortolani maneuver confirms the finding as the hip being abnormally positioned through most of the exam. Which is most likely? a) Subluxatable b) Dislocatable c) Dislocated d) Fused 56) Limp is the most common musculoskeletal complaint in children. What is the most common cause of limp? a) Talipes equinovarus (clubfoot) b) Metatarsus adductus c) Genu valgum d) Genu varum e) Trauma 57) Legg-Calvé-Perthes disease is defined as avascular necrosis of the: a) Navicular bone of the hand b) Lunate bone of the hand c) Sacroiliac joint d) Femoral head e) Talus bone of the ankle 58) Which of the following is NOT a contributing factor to slipped capital femoris epiphysis (SCFE)? a) Trauma b) Weight c) Male gender d) Puberty 59) A young male child presents with painless limp and knee pain. Which of the following is most likely? a) SCFE b) Duchenne muscular dystrophy c) Legg-Calvé-Perthes disease d) DHH e) PCL tear 60) Which of the following is particularly common in toddlers due to twisting forces on the tibia during falling? a) Torus (buckle) fracture b) Greenstick fracture c) Spiral fracture d) Salter-Harris fracture type V e) Salter-Harris fracture type IV 61) What type of osteogenesis imperfecta (OI) is autosomal dominant, has conductive hearing loss, blue sclerae, and presents with bow legs or neonatal fractures? a) Type I b) Type II c) Type III d) Type IV 62) A child is brought to the clinic by his mother after the child’s father was swinging the child around the living room by his arms. The child holds his right arm close to the body, slightly flexing and pronating the hand. Motion at the elbow is limited. Which of the following is most likely? a) Osteomyelitis b) Osteogenesis imperfecta c) Lateral epicondylitis d) Medial epicondylitis e) Radial head subluxation 63) What is the most common pathogen seen in osteomyelitis in children? a) Shigella b) Salmonella c) S. aureus d) S. epidermidis e) N. gonorrhoeae 64) An adolescent presents with a painful knee, fever, and refusal to bear weight on the affected limb. Aspiration reveals diplococci that grow on chocolate agar with carbon dioxide, ruling out the most common cause. Which of the following is most likely? a) Shigella b) Salmonella c) S. aureus d) S. epidermidis e) N. gonorrhoeae 65) What is the most common reason for hospitalization in pediatric practice? a) Renal dysfunction b) Congenital heart defects c) Asthma d) Fever of unknown origin 66) A child presents with acute respiratory distress with dyspnea and wheezing. Exam reveals subcostal retractions, tripod positioning, tracheal tugging, and nasal flaring. Which of the following drugs would be most useful for this acute attack? a) Beclomethasone (inhaled corticosteroid) b) Flunisolide (inhaled corticosteroid) c) Epinephrine IV d) Albuterol (beta2-agonist) e) Montelukast (leukotriene receptor antagonist) 67) A newborn presents with failure to thrive and pulmonary problems. An abdominal radiograph shows a mottle appearance, suggesting meconium ileus. Elevated levels of what ion would be expected in this child’s sweat? a) Potassium b) Sodium c) Calcium d) Magnesium e) Chloride 68) A mother brings in her 2-month-old son to the pediatrician. The mother states the child rolled off the sofa onto the carpeted floor. Testing shows subdural hematomas. Physical exam reveals retinal hemorrhages. Which of the following is most likely? a) Caput succedaneum b) Cephalhematoma c) Traumatic fall d) Child abuse Pediatrics #4 – Extra: New Treatments For Multiple Sclerosis 1) Which of the following is NOT true of multiple sclerosis (MS)? a) Affects more than 30 per 100,000 in middle North America b) Has a higher prevalence in temperate zones c) Most commonly affects women between ages 20 and 40 d) In most cases, is caused by a viral infection e) Genetic factor may play a role in susceptibility 2) Which of the following is characteristic of phase-2 MS, not phase-1? a) Hyperreflexic spasticity b) Bladder and bowel affected c) Hyperreflexia with good axial tone d) Diminution of deep tendon reflexes e) Ataxia of gait with asymmetric paraparesis 3) Which of the following is characteristic of phase-2 MS, not phase-3? a) Bulbar difficulties with swallowing b) Gaze palsies c) Frequency of urination and inability to completely void d) Bulbar difficulties with speaking e) Limb and trunk spasticity 4) Which of the following during early-onset MS correlates to a more severe course of disease? a) Length of initial event b) Loss of urinary control c) Number of relapses d) Intensity of initial event e) The appears to be no correlation between early-onset and disease course 5) A young adult with MS has ocular pain, abnormal visual acuity and fields, reduced vision, relative afferent pupillary defect, and abnormal visual evoked potentials (VEPs). Which of the following is most likely? a) Uveitis b) Glaucoma c) Retinoblastoma d) Coats disease e) Optic neuritis 6) What is the central pathological CNS event suspected in the etiology of MS? a) Destruction of myelin b) Destruction of the putamen c) Destruction of the caudate nucleus d) Destruction of the substantia nigra e) Lysosomal accumulation of lipids 7) The T2-lesion volume (gadolinium-enhanced) on MRI at what locations may be an associated predictor of depression, which occurs in 25-50% of patients with MS? a) Left anterior inferior prefrontal cortex and left anterior temporal CSF b) Right anterior inferior prefrontal cortex and right anterior temporal CSF c) Left anterior inferior prefrontal cortex and right anterior temporal CSF d) Right anterior inferior prefrontal cortex and left anterior temporal CSF 8) Which of the following tests is useful in evaluating demyelination in the posterior columns of the spinal cord? a) Visual evoked response (VER) test b) Sensory evoked response (SER) test c) Rapid plasma reagin (RPR) test d) Erythrocyte sedimentation rate (ESR) e) Vitamin B12 levels 9) The presence of gamma globulins with oligoclonal bands within the CSF indicates: a) The destruction of myelin b) The extent of future MS disease course c) The synthesis of immunoglobulins in the CNS d) The destruction of autoantibodies e) An autoimmune reaction in the PNS 10) What is the treatment for acute exacerbations of multiple sclerosis? a) Prednisone 500mg IV BID for 3-5 days b) Prednisone 100mg IV QID for 3-5 days c) Prednisone 1000mg IM QD for 3-5 days d) Methylprednisolone 100mg IV BID for 3-5 days e) Methylprednisolone 1000mg IV QD for 3-5 days 11) Which of the following has been reported to increase in incidence in MS patient receiving interferon-beta? a) Headaches b) Influenza-like symptoms c) Asthenia d) Anemia e) Seizures 12) Modafinil and amantadine are used in MS for patients complaining of: a) Spasticity b) Depression c) Insomnia d) Fatigue e) Nausea 13) 3,4-Diaminopyridine (3,4-DAP) may improve motor function and fatigue in some patients with MS. What is its mechanism of action? a) Sodium channel blocker b) Beta blocker c) Potassium channel blocker d) Calcium channel blocker e) Alpha adrenergic agonist 14) Baclofen and tizanidine are used in MS for patients complaining of: a) Spasticity b) Depression c) Insomnia d) Fatigue e) Nausea 15) Tolterodine may be used for hyperactive bladder in MS patients. Which of the following is a common side effect of this drug? a) Vomiting b) Dry mouth c) Bradycardia d) Conjunctival injection e) Profuse sweating 16) Which of the following is NOT part of the Barthof criteria in the diagnosis of MS, which requires 3 of 6 criteria to be met? a) Objective neurologic abnormalities on clinical examination with involvement of white matter long tracts b) One area of CNS involvement c) Two or more episodes of progression over 6 months d) Age between 15 and 60 years e) Exclusion of other diseases that may produce similar symptoms f) Presence of oligoclonal bands and/or myelin basic protein in CSF 17) Which of the following disease-modifying drugs for MS has the highest cost and is dosed as 20mg SC once a day? a) Interferon beta-1a b) Interferon beta-1b c) Glatiramer d) Mitoxantrone e) Natalizumab 18) Which of the following has been shown to decrease the number and volume of gadolinium-enhancing lesions in MS patients, with a decline of about 44%? a) Botulinum-A toxin (Botox) b) Amantadine (Symmetrel) c) Simvastatin (Zocor) d) Natalizumab (Tysabri) e) Tolterodine (Detrol) 19) Which of the following time periods shows an increase in MS relapses? a) Pre-pregnancy b) First trimester c) Third trimester d) First 3-months post pregnancy e) 3 to 6-months post pregnancy Pediatrics #5 – Clinical: Textbook Questions 1) A 2-year-old female child presents with VT, severe ventricular dysfunction, hypotension, and metabolic acidosis. The patient is cardioverted into ventricular fibrillation, which degenerates into asystole. What is the most appropriate indication for using intravenous epinephrine in this patient? a) Ventricular ectopy b) Asystole c) Severe refractory metabolic acidosis and/or hyperkalemia d) Bradycardia e) Supraventricular tachycardia 2) A 16-year-old female patient presents with short stature and no secondary sexual characteristics. What diagnosis must be considered? a) Turner syndrome b) Isolated growth hormone deficiency c) Cushing disease d) Familial short stature e) Addison disease 3) Galactosemia, a disorder of carbohydrate metabolism, is inherited in an autosomal recessive fashion. What is the risk of galactosemia in a child whose parents are both carriers for the disorder? a) 100% b) 75% c) 50% d) 25% e) 0% 4) Which of the following statements is true regarding children with sickle cell disease? a) Vaccinations are not required because they receive penicillin prophylaxis b) Gallstones typically develop before the age of 3 years c) Episodes of dactylitis should be treated with antibiotics d) Hydroxyurea maintenance therapy decreases the number and severity of vaso- occlusive crises e) Acute chest syndrome requires only supportive care 5) A mother brings her 5-year-old son to your office in New Mexico for his regular health maintenance visit. A quick review of the patient's chart reveals that he and his family are strict vegans. Their house is very small, so all the children spend a good deal of time outside. The mother states that her son eats plenty of dark green vegetables and iron- fortified grains. She does not believe in providing supplemental vitamins and minerals. This child is most at risk for nutritional deficiency involving which of the following? a) Vitamin B12 b) Vitamin B6 c) Niacin d) Riboflavin e) Vitamin D 6) A 6-year-old boy presents with a newly appreciated heart murmur. He is asymptomatic, with normal growth and development and normal exercise tolerance. On examination S1 and S2 are normal; a II/VI low-frequency midsystolic murmur is heard at the left lower sternal border. His pulses are normal. The most likely diagnosis is: a) Bicuspid aortic valve b) Still's murmur c) Ventricular septal defect d) Atrial septal defect e) Coarctation of the aorta 7) You are called to the delivery room for a routine birth. The infant cries when the cord is cut. You examine the child under the warmer and notice that when he stops crying, his chest heaves, and he turns blue. You are unable to pass the NG tube through the nose for suctioning. Which condition is most likely causing this infant's respiratory distress? a) Choanal atresia or stenosis b) Vocal cord paralysis c) Subglottic stenosis d) Recurrent laryngeal nerve damage e) Laryngeal web 8) A 3-year-old girl is diagnosed with new-onset insulin-dependent diabetes mellitus. Which of the following laboratory findings is consistent with diabetic ketoacidosis? a) Hypoglycemia b) Hypercarbia c) Ketones in urine d) Increased venous blood pH e) Decreased BUN 9) During a male newborn examination, the testes are not palpable in the scrotal sacs. One testis is palpable high in the right inguinal canal and cannot be gently manipulated into the anatomically correct position. The left testis is not palpable but is discovered in the abdomen after consultation with a pediatric urologist and an abdominal ultrasound. In counseling the parents, which one of these statements regarding cryptorchidism is true? a) More than 99% of males have bilateral descended testes at age 1 year b) Impaired sperm production is not a concern if neither testis descends c) Malignant degeneration is not a risk factor for testes, which do not descend as long as they are placed within the scrotal sac through surgery by 1 year of age d) This infant is no more likely than his peers to manifest an inguinal hernia e) Microphallus is a common associated condition 10) A 5-year-old boy presents with a waddling limp and has had a stiff right hip for the last 2 months. He has minimal complaints of pain. The most likely diagnosis is: a) Legg-Calve-Perthes disease b) Slipped capital femoral epiphysis c) Toddler's fracture d) Septic arthritis e) Juvenile idiopathic arthritis 11) A 17-year-old young girl on oral contraceptive therapy for regulation of her menstrual periods presents with a 1-week history of left leg pain and swelling. Evaluation with a Doppler ultrasound reveals absence of flow in the left femoral and popliteal veins. The clot extends proximally to the left external iliac vein. The most important potential complication that one should be cautious about in this girl is: a) Venous insufficiency b) Limb overgrowth c) Pulmonary embolism d) Edema e) Gangrene 12) A woman with a seizure disorder under medical management wants to conceive a child. Her risk of having a child with a neural tube defect is greatest if her current medical regimen includes which of the following? a) Phenobarbital b) Phenytoin c) Ethosuximide d) Carbamazepine e) Primidone 13) A 2-month-old infant presents to your emergency department with a heart rate of 220 beats/minute, pulses, and adequate perfusion. After giving the infant oxygen, you note abnormal P waves and a narrow QRS (!0.08 sec) on the cardiac monitor. Which of the following is the best course of action? a) Administer IV/IO epinephrine b) Administer IV adenosine by rapid bolus c) Administer IV calcium chloride d) Administer IV atropine by rapid bolus e) Administer IV sodium bicarbonate 14) A 3-month-old infant presents with a history of abnormal movements that his parents think might be seizures. You observe an episode of recurrent rhythmic flexor-extensor spasms that repeat about 30 times before subsiding. The EEG shows hypsarrhythmia, and a Wood lamp exam is positive for several flat, hypopigmented macules scattered over the skin surface. This child's infantile spasms are most likely a result of which of the following underlying disorders? a) Von Recklinghausen disease b) Tuberous sclerosis c) Von Hippel-Lindau disease d) Sturge-Weber disease e) Bilateral acoustic neurofibromatosis 15) A 21-month-old girl arrives at clinic in May with a vaccination record that indicates that she has received 3 DTaP doses, 3 Hib doses, 3 IPV doses, 3 pneumococcal conjugate vaccine doses, 2 hepatitis A vaccine doses, and 3 hepatitis B vaccine doses. Which of the following should be administered at this visit? a) DTaP, Hib, IPV, varicella b) DTaP, Hib, pneumococcal conjugate vaccine, MMR, and varicella c) DTaP, hepatitis A, IPV, pneumococcal conjugate vaccine d) DTaP, hepatitis B, MMR, and varicella e) DTaP, hepatitis A, IPV, MMR, and varicella 16) The mother of a 30-month-old boy is concerned that the child's speech is “garbled.” The child uses “ma-ma” and “da-da” appropriately. He uses about 30 other words, but most of them are mispronounced (for instance, “boo” instead of “blue”). The boy's aunt, uncle, and cousins came to visit for a weekend and were unable to understand more than half of what he said. Examination of the ears reveals normal canals with translucent, mobile tympanic membranes, and visible landmarks. Which of the following evaluations for speech delay should be performed first? a) Receptive language testing b) Phonetic testing c) Dysfluency evaluation d) Tympanogram testing e) Audiologic (hearing) assessment 17) A 13-year-old girl presents with recurrent abdominal pain over the last 3 months. She has missed a total of 8 days of school. There is no associated fever, weight loss, gastrointestinal bleeding, and the pain does not occur in relation to meals or awaken her from sleep. There is diffuse abdominal tenderness but no other abnormal findings on examination. Which approach is likely to help in the diagnosis and management of her condition? a) Abdominal CT scan with contrast b) Upper and lower endoscopy and biopsies c) Explaining the likely etiology of her symptoms using a biopsychosocial model and symptomatic therapy d) A diet history and a diet elimination trial e) Referral to a psychiatrist 18) A newborn male child has a flat facial profile, upslanted palpebral fissures, epicanthal folds, a small mouth with a protruding tongue, small genitalia, and simian creases on his hands. What of the following chromosomal disorders is most likely in this child? a) Trisomy 21 b) Trisomy 18 c) Trisomy 13 d) Klinefelter syndrome e) Turner syndrome 19) At a 2-year well-child visit, you collect information that your patient lives in a very old rental home with peeling paint. Both the capillary (screening) and venous blood lead measu