1-100 notchtop superexams

Question 1

Which phase of Kawasaki disease is associated with coronary aneurysms?

A. Acute febrile phase
B. Subacute phase
C. Convalescent phase
D. Phase of complications
E. All of the above

Answer 1

B. Subacute phase

> Kawasaki disease presents with characteristically high, unremitting fever and 4 out of the 5 principal features:

1. Bilateral nonexudative bulbar conjunctival injection with limbal sparing
2. erythema of the oral and pharyngeal mucosa with strawberry tongue and dry, cracked lips
3. edema and erythema of the hands and feet
4. rash of various forms (scarlatiniform, maculopapular, erythema multiforme)
5. nonsuppurative cervical lymphadenopathy (usually unilateral, with node size >1.5cm)

Question 2

Fever and the acute signs of illness and usually lasts 1-2wks

Answer 2

Acute febrile phase

Question 3

Desquamation, thrombocytosis, coronary aneurysms and highest risk of sudden death usually lasts 2 weeks

Answer 3

Subacute febrile phase

Question 4

All clinical signs have disappeared until ESR normalizes typically 6-8 weeks after onset on illness

Answer 4

Convalescent phase

Question 5

A four year old presents with low-grade fever, intermittent crampy abdominal pain with emesis and swollen knees of 3 days duration. A purpuric rash distributed below the knees of both lower extremities is noted on physical examination. This patient most likely has:

A. Meningococcemia
B. Idiopathic thrombocytopenic purpura
C. Henoch-Scholein purpura
D. SLE
E. Juvenile Rheumatoid arthritis

Answer 5

C. Henoch-Scholein purpura

> HSP is a common vasculitis among children and presents with the classic findings of abdominal pain with or without rectal bleeding, vasculitis rash, arthritis and nephritis. The platelet count is normal. Gastrointestinal involvement may progress to intussusception.

Question 6

A 3 year old female presents with 1 week history of daily (“quotidian”) fever associated with arthritis of both ankles and her right knee. Her mother notes that she has decreased activity and also notes rashes that come and go in different parts of her body but not on the face. On physical examination, salmon colored patches were noted on the trunk and extremities. Palpation revealed splenomegaly. What is the diagnosis?

A. Henoch Schoenlein Purpura
B. Systemic Lupus Erythematosus
C. Juvenile Rheumatoid arthritis
D. Juvenile Dermatomyositis
E. Dengue fever

Answer 6

C. Juvenile Rheumatoid arthritis

Question 7

Salmon colored patch, evanescent, spares the face

Answer 7

Juvenile Rheumatoid arthritis

Question 8

Malar rash, photosensitive

Answer 8

Systemic Lupus Erythematosus

Question 9

Heliotrope (periorbital rash), gottron papules (rash on the knuckles)

Answer 9

Dermatomyositis

Question 10

Purpura in dependent portions

Answer 10

Henoch Schonlein purpura

Question 11

A 4 yr old boy presents with his third episode of painful cervical lymphadenitis. Each was treated with incision and drainage and grew S. aureus. He also experiences recurrent skin infections. A year ago, he was hospitalized for osteomyelitis. The most important laboratory test is:

A. PCR for ADA deficiency
B. Nitroblue tetrazolium test
C. MAC-I assay
D. Neutrophil count
E. Genetic chromosomal analysis

Answer 11

B. Nitroblue tetrazolium test

This is a case of CHRONIC GRANULOMATOUS DISEASE wherein patients are susceptible to catalase positive organisms like S. aureus. The nitroblue tetrazolium tests the neutrophils ability to generate superoxide anion and thus kill ingested bacteria.

Question 12

Which among the following is NOT a prominent feature of Wiskott-Aldrich syndrome?

A. X-linked recessive inheritance
B. Atopic dermatitis
C. Thrombocytopenia
D. Recurrent infections with encapsulated bacteria
E. Leukopenia

Answer 12

E. Leukopenia

> The prominent immunologic impairment in Wiskott Aldrich Syndrome is against polysaccharides of encapsulated organisms.

Question 13

A 12 year old seeks consult for 1 day history of sneezing, clear rhinorrhea and nasal itching not associated with fever or any other systemic symptoms. PE reveals boggy, pale nasal edema with a clear discharge. The most likely diagnosis is?

A. Foreign body
B. Vasomotor rhinitis
C. Allergic rhinitis
D. Neutrophilic rhinitis
E. Rhinitis medicamentosa

Answer 13

C. Allergic rhinitis

> Allergic rhinitis is often seasonal and associated with allergic conjunctivitis. Eosinophils predominate in the nasal secretions.

It is classified as:
- Seasonal (cyclical) or perinneal (all year)
- Intermittent (symptoms occur <4 days per week or <4 consecutive weeks) or persistent (symptoms occur >4 days per week or >4 consecutive weeks)
- Mild-moderate (no impairment) or severe (with impairment of daily living/sleep)

Question 14

The Kasai procedure is indicated for which of the following?

A. Neonatal hepatitis
B. Biliary atresia
C. Metabolic liver disease
D. Fulminant hepatic failure
E. None of the above

Answer 14

B. Biliary atresia

> Despite initial success of the Kasai operation, patients with biliary atresia eventually get liver transplantation.

Question 15

An 8 month old manifests with fussiness and emesis and refuses to eat. 1 week ago, he was brought for consult and was diagnosed with URTI. On the morning of consult, his mother noted currant jelly like material on his diaper. On PE, a sausage shaped mass was palpable in the abdomen. There is likewise abdominal distention and rectal exam reveals blood in the stool. The most likely diagnosis is:

A. Viral diarrhea
B. Duodenal atresia
C. Intussusception
D. Hypertrophic pyloric stenosis
E. Intestinal adenoma

Answer 15

C. Intussusception

> Aside from that, intussusception may present with lethargy out of proportion to the intestinal signs and symptoms.
Intussusception in children is usually due to lymphoid hyperplasia in the intestines. This becomes the lead point.
The most common location is ILEOCOLIC.

Question 16

A 3 year old female presents to your office with unilateral nasal discharge. The discharge is described by the caretaker to be malodorous and lately has been blood tinged. The most likely diagnosis is:

A. Tertiary syphilis
B. Unilateral choanal atresia
C. Nasopharyngeal carcinoma
D. Foreign body
E. Angiosarcoma of the nose

Answer 16

D. Foreign body

Question 17

A 3 year old is brought to the ER. On PE, inspiratory stridor and a barking cough were noted. There is mild respiratory distress, tachypnea, and fever. The signs and symptoms are aggravated by agitation and crying. The most likely diagnosis is:

A. Trachiomalacia
B. Laryngotracheobronchitis
C. Epiglotittis
D. Bacterial tracheitis
E. Peritonsillar abscess

Answer 17

B. Laryngotracheobronchitis

> This is a case of croup and is common in this age group.
Barking cough = croup
Epiglotitis and bacterial tracheitis patients are more toxic appearing and not in merely mild respi distress

Question 18

A newborn was noted to be cyanotic. Unfortunately the imaging facilities are unavailable for the day. A hyperoxia test was thus done which showed no improvement of PaO2 after administration of 100% O2. The next step in the management of this neonate is:

A. Low tidal volume mechanical ventilation
B. Positive pressure ventilation
C. Digoxin
D. Emergency laparotomy
E. Prostaglandin E

Answer 18

E. Prostaglandin E

> In the absence of imaging for definitive diagnosis of the congenital heart disease, the clinician should not hesistate to administer prostaglandin in case the patient has a ductus dependent congenital heart disease. Prostaglandin prevents the ductus from closing.

Question 19

The ______________ is used to differentiate whether the etiology of cyanosis is cardiac or not. PaO2 does not improve in cardiac etiologies.

Answer 19

hyperoxia test

Question 20

A patient was diagnosed with tetralogy of fallot. His chest xray reveals the classic “Le Couer en Sabot”. Which of the following components of TOF is responsible for this shape?

A. Pulmonary stenosis
B. VSD
C. RVH
D. Overriding of the aorta
E. Right sided aorta

Answer 20

C. RVH

> The typical configuration on AP view consists of a narrow base, concavity of the left heart border in the area usually occupied by the pulmonary artery and normal overall heart size. The hypertrophied right ventricle causes the rounded apical shadow to be uptilted so that it is situated higher above the diaphragm than normal and pointing horizontally to the left of the chest wall. The cardiac shadow has been likened to that of a boot or a wooden shoe.

Question 21

A 5 year old presents with multiple petichiae on her lower extremities and oral-mucosal bleeding of 3 days duration. 2 weeks prior, she had a mild respiratory tract infection but other than that, her caretakers describe her to be active and generally fine. On PE, she is afebrile. No lymphadenopathy nor hepatosplenomegaly is noted. Which is the best next diagnostic step to confirm the diagnosis?

A. CBC with platelet count
B. PT
C. PTT
D. Bleeding time
E. Clotting time

Answer 21

A. CBC with platelet count

> CBC with PC would reveal decreased platelets and normal other parameters suggesting that this is a case of ITP.

Question 22

An infant has a hemangioma that grows rapidly in size, leading to thrombocytopenia and microangiopathic hemolytic anemia. Which term describes this condition?

A. Kaposi like form of infantile hemangioma
B. McCune Albright syndrome
C. Kasabach-Merritt syndrome
D. Maffucci syndrome
E. Evan’s syndrome

Answer 22

C. Kasabach-Merritt syndrome

Question 23

Polyostotic fibrous dysplasia, café-au-lait skin pigmentation, autonomous endocrine hyperfunction

Answer 23

McCune Albright syndrome

Question 24

Multiple enchondromas and hemangiomas

Answer 24

Mafucci syndrome

Question 25

Autoimmune anemia + thrombocytopenia

Answer 25

Evan’s syndrome

Question 26

A previously healthy 8 year old male presents with progressive painless proptosis and decreased visual acuity of the left eye during the past 10 weeks. The most likely diagnosis is:

A. Orbital cellulitis
B. Trichinosis
C. Retinoblastoma
D. Rhabdomyosarcoma
E. None of the above

Answer 26

D. Rhabdomyosarcoma

Question 27

The most common sarcoma of childhood.

Answer 27

Rhabdomyosarcoma

Question 28

_______________ is a common site for rhadomyosarcoma which produces local signs as it grows and displaces normal tissues.

Answer 28

Orbital rhabdomyosarcoma

Question 29

________________ may be endophytic or exophytic from the retina. They present with leukocoria or strabismus.

Answer 29

Retinoblastoma

Question 30

A 4 year old male experienced an upper respiratory tract infection that was followed in 2 weeks by generalized edema. His blood pressure is normal. Urinalysis reveals 2-5 rbc/hpf and 4+ protein. His BUN is 19mg/dl. Serum albumin 0.9g/dl and C3 was 93mg/dl. The most likey diagnosis is:

A. Post streptococcal glomerulonephritis
B. Membranous glomerulonephritis
C. Minimal change disease
D. Focal sclerosis
E. IgA nephropathy

Answer 30

C. Minimal change disease

> Patient is presenting with nephrotic syndrome. The most common of which is minimal change disease in children.
Hypoalbuminemia, proteinuria, edema and hyperlipidemia constitute the nephrotic syndrome.

Question 31

The following listed phrases are characteristics of simple partial seizures EXCEPT:

A. Loss of consciousness
B. Duration of 10-20 sec
C. Versive seizures
D. May have secondary generalization
E. Abnormal EEG

Answer 31

A. Loss of consciousness

> There is never a loss of consciousness in simple partial seizures. Some patients may actually be conversant and talk to you during the event.

Question 32

Pincer grasp is usually noted at what age?

A. 2mos
B. 4mos
C. 6mos
D. 8mos
E. 10 mos

Answer 32

D. 8mos

Question 33

A 12 year old male presents with complaints of polyarthritis and an evanescent rash which spares the face. On PE, patient appears to be in respiratory distress and auscultation revealed tachycardia and a gallop rhythm, and some hard painless freely movable swellings over the extensor surfaces like the elbows. Chest xray reveals cardiomegaly and ASOT is 350 Todd units. The following are appropriate in the management of this patient except:

A. Penicillin V 200-500mg QID x 1 week
B. Complete bed rest
C. Morphine
D. Digoxin
E. 02 supplementation

Answer 33

A. Penicillin V 200-500mg QID x 1 week

> The antibiotic treatment for eradication of streptococcus can be either:

1. Penicillin VK x 10days (not just one week)
2. Benzathine PCN 0.6-1.2 MU IM
3. Erythromycin 250mg TID x 10 days

Question 34

A 5 year old presents with complaint of chronic cough of 1 month duration with associated weight loss and recurrent fever. PPD was done. Which of the following is true.

A. Equal to or > 5 is positive. Anything below this value is negative.
B. Equal to or > 10 is positive. Anything below this value is negative.
C. Equal to or > 15 is positive. Anything below this value is negative.
D. Equal to or > 20 is positive. Anything below this value is negative.
E. Equal to or > 25 is positive. Anything below this value is negative.

Answer 34

B. Equal to or > 10 is positive. Anything below this value is negative.

> Equal to or > 5 – positive if with history of close contact, with suggestive clinical findings, suggestive CXR, immunocompromised
Equal to or > 10 = positive

Question 35

Which one of the following forms of juvenile idiopathic arthritis is most likely to be associated with serious eye complications?

A. Polyarticular arthritis that is seropositive for rheumatoid factor
B. Polyarticular arthritis that is seronegative for rheumatoid factor
C. Oligoarticular arthritis without axial spine involvement
D. Oligoarticular arthritis with axial spine involvement
E. Systemic-onset juvenile rheumatoid arthritis

Answer 35

C. Oligoarticular arthritis without axial spine involvement

> Patients with oligoarticular arthritis without axial spine involvement are most likely to develop chronic and potentially severe anterior uveitis, which can be clinically quite subtle even as it leads to progressive visual loss. Up to 25% of patients in this subset may develop anterior uveitis, and the group that is ANA positive appears to be at highest risk. Patients with axial spine involvement can also develop anterior uveitis, but this tends to be acute, self-limited, and easily treatable.

Question 36

A 7-year-old boy presents with palpable purpura on the buttocks and legs, fever, abdominal pain and vomiting, arthritis in his knees and ankles, melena, and hematuria. His mother states that he had an upper respiratory illness approximately 1 week ago, but has otherwise been well. Blood tests reveal mild renal insufficiency. The most likely cause of the bleeding into the skin observed in this patient is:

A. coagulation factor deficiency
B. qualitative platelet dysfunction
C. quantitative platelet dysfunction
D. vasculitis
E. vitamin deficiency

Answer 36

D. vasculitis

> The clinical description is that of Henoch-Schönlein purpura, a form of leukocytoclastic angiitis (hypersensitivity vasculitis) resulting from an immune reaction that damages the vascular endothelium. Henoch-Schönlein purpura is closely related to IgA nephropathy, a glomerulopathy resulting in nephritic syndrome, and may represent a systemic version of this disease.

Question 37

A mother calls you frantic because she has just been diagnosed with varicella (chicken pox). She delivered a term infant 7 days ago that appears to be eating, stooling, and urinating without difficulty. The child has been afebrile and seems to be doing well. Which of the following is the most appropriate step in management?

a. Isolate the infant from the mother.
b. Hospitalize the infant in the isolation ward.
c. Administer acyclovir to the infant.
d. Administer varicella-zoster immunoglobulin to the infant.
e. Advise the mother to continue regular well-baby care for the infant.

Answer 37

e. Advise the mother to continue regular well-baby care for the infant.

> Per CDC recommendations, varicella-zoster immunoglobulin (VZIG) should be administered to the infant immediately after delivery if the other had the onset of varicella within 5 days prior to delivery, and immediately upon diagnosis if her chicken pox started within 2 days after delivery. If untreated, about half of these infants will develop serious varicella as early as 1 day of age. If a normal full-term newborn is exposed to chicken pox 2 or more days postnatally, VZIG and isolation are not necessary because these babies appear to be at no greater risk for complications than older children. Acyclovir may be used in infants at risk for severe varicella, such as those infants exposed perinatally.

Question 38

A 10-month-old baby boy has a 5-hour history of intermittent crying, and with intermittent drawing up of his knees to his chest in between crying episodes. On the way to the emergency room he passes a loose, bloody stool. He has had no vomiting and has refused his bottle since the crying began. Physical examination is noteworthy for an irritable infant whose abdomen is very difficult to examine because of constant crying. His temperature is 38.8°C (101.8°F). The rectal ampulla is empty, but there is some gross blood on the examining finger. Which of the following studies would be most helpful in the immediate management of this patient?

a. Stool culture
b. Examination of the stool for ova and parasites
c. Air contrast enema
d. Examination of the blood smear
e. Coagulation studies

Answer 38

c. Air contrast enema

Question 39

The usual presentation of ____________ is that of an infant between 4 and 10 months of age who has a sudden onset of intermittent colicky abdominal pain. The child can appear normal when the pain abates, but as it recurs with increasing frequency, the child can begin to vomit and become progressively more obtunded. The passage of stool containing blood and mucus, frequently described as resembling currant jelly, is often observed. Early examination of the abdomen can be unremarkable, but as the problem persists, a sausage-shaped mass in the right upper quadrant is frequently palpated. An air, barium, or saline enema examination under fluoroscopic or ultrasound control can be therapeutic as well as diagnostic when the hydrostatic effects of the contrast serve to reduce the intussusception, but should be performed with surgical backup, as a complication of attempted reduction is intestinal perforation. Rates of intestinal perforation are lowest with air reduction. Early diagnosis prevents bowel ischemia. The cause of most intussusceptions is unknown, but a Meckel diverticulum or polyp can serve as a lead point. None of the other choices would result in a correct diagnosis (and potential therapy) for the child with a classic presentation for intussusception.

Answer 39

intussusception

Question 40

A 13-year-old boy presents to the pediatrician with extrapyramidal signs, including a resting and kinetic tremor. An ophthalmologic examination demonstrates the presence of dark rings that appear to encircle the iris of the eye, and his laboratory studies demonstrate elevated liver enzymes. It is likely that the patient’s condition is associated with the accumulation of:

A. copper
B. Councilman bodies
C. eosinophilic hyaline inclusions
D. glycogen
E. iron

Answer 40

A. copper

Question 41

______________ is a hereditary condition associated with the accumulation of copper in the liver, brain, and eye.

Answer 41

Wilson disease

Question 42

Accumulation of copper in the Descemet membrane of the eye results in the pathognomonic lesion known as the _____________.

Answer 42

Kayser-Fleischer ring.

Question 43

__________________ are apoptotic hepatocytes that were first identified in yellow fever.

Answer 43

Councilman bodies

Question 44

A 10-month-old infant on long-term aspirin therapy for Kawasaki disease develops sudden onset of high fever, chills, diarrhea, and irritability. A rapid swab in your office dentifies influenza A, adding her to the long list of influenza patients you have seen this December. Over the next few days, she slowly improves and becomes afebrile. However, 5 days after your last encounter you hear from the hospital that she has presented to the emergency center obtunded and posturing with evidence of liver dysfunction. Which of the following statements about her current condition is correct?

A. With proper supportive care, the overall mortality rate is low.
B. With her progressive liver dysfunction, increased total serum bilirubin is anticipated.
C. Administration of N-acetylcysteine is first-line therapy
D. Seizures are uncommon with this condition.
E. Death is usually associated with increased intracranial pressures and herniation.

Answer 44

E. Death is usually associated with increased intracranial pressures and herniation.

Question 45

____________ is an acquired mitochondrial hepatopathy that results from the interaction of an influenza (or varicella) infection and aspirin use. While prevalence has decreased over the last few decades and it is now a rare disease, mortality remains the same at more than 40% of cases. Liver enzymes and ammonia are elevated, but total bilirubin is not. Patients initially present toward the end of a viral infection with sleepiness, emesis, and abnormal liver functions. As the disease progresses, the patient may develop seizures, coma, hyperventilation, and decorticate posturing. Ultimately they may develop respiratory arrest, loss of deep tendon reflexes (DTRs), and fixed and dilated pupils. Death is usually from cerebral edema and subsequent herniation. While aspirin is no longer routinely used in children as an antipyretic or pain reliever, the increase in the use of aspirin in adults with heart disease requires specific counseling for parents of children with influenza and varicella to avoid aspirin use. In addition, both of these infections are preventable with proper immunization. N-acetylcysteine is protective of hepatocytes in acetaminophen overdose.

Answer 45

Reye syndrome

Question 46

A 4-year-old boy in your practice has been having many infections. You suspect that he may have an immune dysfunction. Quantitative immunoglobulins were sent and were normal. Which of the following immunodeficiency disorders is associated with normal immunoglobulin G (IgG) levels?

A. X-linked agammaglobulinemia
B. DiGeorge syndrome
C. Late-onset hypogammaglobulinemia
D. Ataxia-telangiectasia
E. Severe combined immunodeficiency

Answer 46

B. DiGeorge syndrome

> Although reduced levels of immunoglobulin A (IgA) or IgE may be seen in patients with DiGeorge syndrome—a T cell deficiency disorder—the total serum immunoglobulin level usually is normal and IgG levels are normal.
In X- linked agammaglobulinemia (Bruton’s) and late-onset hypogammaglobulinemia, IgG, IgM, and IgA levels are all reduced, and the total immunoglobulin level is less than 100 mg/dL.
Patients with ataxia-telangiectasia have a defect in their DNA repair mechanism. One of the clinical features is low IgA and IgG.
Severe combined immunodefi ciency results in a decrease in all immunoglobulins.

Question 47

A 5-month-old child was normal at birth, but the family reports that the child does not seem to look at them any longer. They also report the child seems to “startle” more easily than he had before. Testing of his white blood cells (WBCs) identifies the absence of β-hexosaminidase A activity, confirming the diagnosis of which of the following?

a. Niemann-Pick disease, type A
b. Infantile Gaucher disease
c. Tay-Sachs disease
d. Krabbe disease
e. Fabry disease

Answer 47

Tay-Sachs disease

Question 48

Children who have ______________ are characterized by progressive developmental deterioration; physical signs include MACULAR CHERRY-RED SPOTS and exquisite and characteristic sensitivity to noise.

Answer 48

Tay-Sachs disease

Question 49

Diagnosis of this disorder can be confirmed biochemically by the absence of β-hexosaminidase A activity in WBCs.

Answer 49

Tay-Sachs disease

Question 50

The other GM2 gangliosidosis, ____________, results from a deficiency of both β-hexosaminidase A and B.

Answer 50

Sandhoff disease

Question 51

Tay-Sachs disease is inherited as an _______________; frequently, affected children are of Eastern European Jewish ancestry.

Answer 51

Autosomal recessive trait

Question 52

Niemann-Pick disease (type A), characterized by the absence of _______________, results in a normal-appearing child at birth who then develops hepatosplenomegaly, lymphadenopathy, and psychomotor retardation in the first 6 months, followed by regression after that.

Answer 52

sphingomyelinase

Question 53

Infantile Gaucher disease, deficient in ______________, presents in infancy with increased tone, strabismus, organomegaly, failure to thrive, strider, and several years of psychomotor regression before death.

Answer 53

β-glucosidase

Question 54

A disease deficient in galactocerebroside β-galactosidase, which presents early in infancy with irritability, seizures, hypertonia, and optic atrophy, with severe delay and death usually occurring in the first 3 years of life.

Answer 54

Krabbe disease (globoid cell leukodystrophy)

Question 55

Deficient in α-galactosidase, which presents in childhood with angiokeratomas in the “bathing trunk area,” ultimately resulting in severe pain episodes.

Answer 55

Fabry disease

Question 56

The parents of a 2-week-old infant who was born at home bring him to the emergency center in your local hospital for emesis and listlessness. On examination, you find a dehydrated, listless, and irritable infant. Although you do not have a birth weight, the parents do not feel that he has gained much weight. He has significant jaundice. His abdominal examination is significant for both hepatomegaly and splenomegaly. Laboratory values include a total bilirubin of 15.8 mg/dL and a direct bilirubin of 5.5 mg/dL. His liver function tests are elevated and his serum glucose is 38 mg/dL. His admit urinalysis is negative for glucose but positive for Gram-negative rods; his urine and his blood ultimately grow E. coli. Which of the following nutritional considerations should be considered in this child?

A. Administration of high doses of vitamin B6
B. Initial diet free of branched-chain amino acids
C. Lactose free formula
D. Protein restriction and supplementation with citrulline
E. Initiation of a diet low or free of phenylalanine

Answer 56

C. Lactose free formula

Question 57

A 2-year-old child is admitted to your hospital. The child’s pediatrician has been following the child for several days and has noted her to have had high fever, peeling skin, abdominal pain, and a bright red throat. You are concerned because two common pediatric problems that could explain this child’s condition have overlapping presenting signs and symptoms. Which of the following statements comparing these two diseases in your differential is true?

A. Neither has a cardiac complication.
B. Serologic tests are helpful in diagnosing both.
C. Only one of the diseases has mucocutaneous and lymph node involvement.
D. Pharyngeal culture aids in the diagnosis of one of the conditions.
E. A specific antibiotic therapy is recommended for one of the conditions, but only supportive care is recommended for the other.

Answer 57

D. Pharyngeal culture aids in the diagnosis of one of the conditions.

Question 58

A 3-year-old girl presents with generalized edema shortly after recovery from an upper respiratory infection. Laboratory studies reveal marked albuminuria, as well as hypoalbuminemia and hyperlipidemia. Prior similar episodes responded to steroids. The most likely diagnosis is:

A. FSGS
B. membranous GN
C. lipoid nephrosis
D. PSGN
E. RPGN

Answer 58

C. lipoid nephrosis

Question 59

4-year-old boy develops weakness of proximal lower back and extremity muscles, manifested by lordosis, a waddling gait, and the need to push on his knees in order to stand. Examination reveals proximal muscle weakness and bilateral enlargement of the calves. His younger brother has begun to display similar findings, as has his older half-brother, who has the same mother. Serum CK is markedly elevated. Which of the following is characteristic of this disorder?

A. Aberrant protein coded by a very small gene sequence on the Y chromosome
B. Autosomal dominant mode of inheritance
C. Mitochondrial inheritance
D. Regression of findings in late adolescence and adult life
E. Total absence or marked decrease of an important gene product

Answer 59

E. Total absence or marked decrease of an important gene product

> The clinical picture is that of Duchenne muscular dystrophy, the most common and most severe of the muscular dystrophies. This X-linked disorder is characterized by failure of synthesis of dystrophin, most often because of deletion of one or many exons in the DMD gene. Patients manifest with proximal muscle weakness, progressing to muscle necrosis. Serum CK is markedly increased. Compensatory hypertrophy is followed by pseudohypertrophy, in which necrotic muscle is replaced by fat and connective tissue. Most patients become wheelchair- bound and die of respiratory or cardiac failure in their late teenage years or in their early twenties.

Question 60

A 3-year-old boy, an inner city resident, has multiple bony abnormalities, including bowlegs and knock-knees, thickening of the skull with frontal bossing, knobby deformities of the costochondral junctions and, at the ends of the long bones, distortion of the rib cage with flaring over the diaphragm, and pigeon-breast deformity. A decrease in which of the following is characteristic of this condition?

A. Bony osteoblastic activity
B. Calcification of osteoid
C. Release of parathyroid hormone
D. Serum alkaline phosphatase activity
E. Synthesis of osteoid

Answer 60

B. Calcification of osteoid

Question 61

The child has rickets, which is caused by failure of action of _____________________, the active form of vitamin D. The bony abnormalities in rickets are caused by failure of osteoid matrix to calcify, leading to excess accumulation of osteoid, increased thickness of the epiphyseal growth plates, and other skeletal deformities. Many of the effects of calcitriol deficiency are mediated by increased release of parathyroid hormone (PTH). PTH stimulates bony osteoblastic activity, which is mirrored by an increase in serum alkaline phosphatase.

Answer 61

calcitriol (1,25-dihydroxycholecalciferol)

Question 62

A 10-year-old boy presents with a large abdominal mass. Computed tomography of the abdomen reveals enlarged retroperitoneal and mesenteric lymph nodes. Biopsy of one of the involved lymph nodes shows a “starry-sky” appearance, with prominent debris-containing macrophages. Which of the following statements about this disorder is correct?

A. The sporadic form is most frequently associated with EBV.
B. The disorder is considered to be a derivative of Hodgkin lymphoma, lymphocyte depletion subtype.
C. The most common cytogenetic change is t(8;14), with increased expression of c-myc.
D. The tumor cells are derivatives of T lymphocytes.
E. The tumor most often has an indolent clinical course.

Answer 62

C. The most common cytogenetic change is t(8;14), with increased expression of c-myc.

Question 63

The typical cytogenetic change associated with Burkitt lymphoma is _______ with increased expression of the c-myc gene. This disorder is an aggressive B-cell non- Hodgkin ymphoma most commonly affecting children. The endemic (African) form is characterized by the involvement of the maxilla or mandible, whereas the sporadic
(Western) form usually involves the abdominal organs. Burkitt lymphoma is generally a rapidly growing neoplasm, and the endemic form has a frequent association with EBV.

Answer 63

t (8;14)

Question 64

A 2-year-old boy presents with his third bone fracture within the past several months. There is no history or evidence of trauma. Several close family members have been similarly affected. The child is small for his age, and the sclerae are tinged a bluish color. Radiographs reveal generalized osteopenia and evidence of multiple fractures, both old and new. Which of the following is the usual mode of inheritance of this disorder?

A. Autosomal dominant
B. Autosomal recessive
C. X-linked dominant
D. X-linked recessive
E. Mitochondrial

Answer 64

A. Autosomal dominant

Question 65

________________ (or brittle bone disease) is an autosomal dominant disorder characterized by multiple fractures with minimal trauma. It is caused by mutations in either of the genes that code for type I collagen and is manifested by connective tissue abnormalities affecting the bones, teeth, skin, and eyes. The sclerae can appear blue due to translucency of the thin connective tissue overlying the choroid. The disorder occurs in several variants defined by the age of onset and the severity of the fractures. In the less obvious cases, the principal differential diagnostic consideration is child abuse.

Answer 65

Osteogenesis imperfecta

Question 66

A fully immunized 2-year-old presents to the emergency room with several days of low-grade fever, barking cough, and noisy breathing. Over the past few hours he has developed a fever of 40°C (104°F) and looks toxic. He has inspiratory and expiratory stridor. The family has not noticed drooling, and he seems to be drinking without pain. Direct laryngoscopy reveals a normal epiglottis. The management of this disease process includes which of the following?

A. Intubation and intravenous antibiotics
B. Inhaled epinephrine and oral steroids
C. Intravenous methylprednisolone
D. Observation in a cool mist tent
E. Oral antibiotics and outpatient follow-up

Answer 66

A. Intubation and intravenous antibiotics

Question 67

A 15-year-old athlete with knee pain and severe point tenderness at the tibial tubercle most likely has:

A. Osgood-Schlatter disease
B. Legg-Calvé-Perthes disease
C. Slipped capital femoral epiphysis (SCFE)
D. Tibial torsion
E. Charcot-Marie-Tooth Disease

Answer 67

A. Osgood-Schlatter disease

Question 68

_____________ is a very common problem most often seen in athletically active adolescents. This disorder is characterized by ossification in the distal patellar tendon at the point of its insertion onto the tibial apophysis. This disorder is thought to result from mechanical stress on the tendinous insertional area.

Answer 68

Osgood-Schlatter disease

> X-ray views of the involved knee show a characteristic irregularity in the insertional area and often show separately discrete ossicles within the tendon itself. The disease will present with severe local pain and exquisite tenderness in the area of the tibial tubercle. In almost every case, symptoms do regress after skeletal maturity or the discontinuance of active athletic participation. In rare cases, persistive symptoms into adulthood can occur.

Question 69

_________________, also known as coxa plana, is a condition of the pediatric hip characterized by a flattened, misshapen femoral head. The etiology of the problem is related to osteonecrosis of the proximal femoral epiphysis and is thought to result from vascular compromise.

Answer 69

Legg-Calvé- Perthes disease

Question 70

A __________________ is an acquired disorder of the epiphysis thought to be associated with weakness in the perichondrial ring of the growth plate. Children within the ages of 10 to 16 years old are noted to have the displacement of the epiphysis on the femoral neck. In most cases, there is no identifiable trauma history. It is associated with African American heritage and obesity and is somewhat more common in boys than in girls.

Answer 70

slipped capital femoral epiphysis (SCFE)

Question 71

________________ is the most common cause of an intoeing gait. This is most frequently noted in 1- and 2-year-old children. This is often bilateral. Although occasionally intoeing can be marked, pediatric tibial torsion will completely resolve without treatment in almost every case.

Answer 71

Tibial torsion

Question 72

Which of the following indicates a poor prognosis in a patient with neuroblastoma?

A. Hyperdiploid DNA
B. Absence of N-myc amplification
C. Age 1 year
D. Age >13 years
E. Low serum ferritin levels

Answer 72

D. Age >13 years

Question 73

A 5-year-old boy diagnosed with poststreptococcal glomerulonephritis was admitted to the hospital several weeks ago. Over the last several weeks, his clinical state has not improved. Severe oliguria has developed, his serum creatinine has continued to rise, and his glomerular filtration rate has decreased by 50% since his admission to the hospital. Which of the following is the most likely diagnosis?

A. Alport syndrome
B. Membranoproliferative glomerulonephritis
C. Membranous glomerulonephritis
D. Rapidly progressive glomerulonephritis
E. Renal papillary necrosis

Answer 73

D. Rapidly progressive glomerulonephritis

Question 74

The clinical description is that of RPGN, which is defined as the _____________ that progresses rapidly to fulminant renal failure within weeks or months. RPGN is classified into three categories: antiglomerular basement membrane (anti- GBM) antibody disease, immune complex deposit disease, and pauciimmune disease (ANCA positive). Characteristic histologic findings in RPGN include crescents between the Bowman capsule and the glomerular tuft. In approximately 50% of cases of RPGN, the disease is of post streptococcal etiology. It should be noted, however, that the most common outcome of poststreptococcal glomerulonephritis is complete recovery, and only a small minority of patients progress to RPGN.

Answer 74

nephritic syndrome

Question 75

A 9-year-old child has developed headaches that are more frequent in the morning and are followed by vomiting. Over the previous few months, his family has noted a change in his behavior (generally more irritable than usual) and his school performance has begun to drop. Imaging of this child is most likely to reveal a finding in which of the following regions?

A. Infratentorial
B. Supratentorial
C. Intraventricular
D. Spinal canal
E. Peripheral nervous system

Answer 75

A. Infratentorial

> While supratentorial tumors predominate in the first year of life (including choroid plexus tumors and teratomas), brain tumors in children 1 to 10 years old are more frequently infratentorial (posterior fossa) and include cerebellar and brainstem tumors such as medulloblastoma or cerebellar astrocytoma. After 10 years of age, supratentorial tumors (eg, diffuse astrocytoma) are again more common.

Question 76

___________ are the most common solid tumor in childhood, and account for 25% to 30% of all pediatric malignancies.

Answer 76

Brain tumors

Question 77

What is the treatment of choice in vaso-occlusive crisis of sickle cell anemia?

A. Hydration and pain medications
B. Exchange transfusion
C. Splenectomy
D. Antibiotic prophylaxis
E. NOTA

Answer 77

A. Hydration and pain medications

> For vaso-occlusive crisis - Hydration and pain medications
For CVA - Exchange transfusion with chronic transfusion protocol to keep Hgb S <30%
For splenic sequestration - Emergent transfusion with subsequent splenectomy
For aplastic crisis - Supportive care; transfusion if necessary
For infections - Appropriate antibiotics, penicillin prophylaxis, vaccines for S. pneumoniae, H. influenzae, N. meningitidis

Question 78

Inheritance of glucose-6-phosphate dehydrogenase deficiency:

A. Autosomal dominant
B. Autosomal recessive
C. X-liked dominant
D. X-recessive
E. Non-mendelian

Answer 78

D. X-recessive

> Mutation in the G6PD gene on the X chromosome. G6PD deficiency is an X-linked recessive disorder.

Question 79

Gaucher disease is deficiency of which enzyme:

A. Sphingomyelinase
B. Hexosaminidase A
C. Alpha-galactosidase
D. Beta-glucuronidase
E. Glucocerebrosidase

Answer 79

E. Glucocerebrosidase

Question 80

Parahemophilia is deficiency of what coagulation factor?

A. Factor 5
B. Factor 8
C. Factor 9
D. Factor 11
E. NOTA

Answer 80

A. Factor 5

> Factor 5 - Parahemophilia
Factor 8 - Hemophilia A
Factor 9 - Hemophilia B
Factor 11 - Hemophilia C

Question 81

What 4 elements comprise the tetralogy of Fallot, except?

A. VSD
B. Pulmonic stenosis
C. Overriding of the aorta
D. RVH
E. NOTA

Answer 81

E. NOTA

Question 82

Gross Motor development: Sits without support

A. 3 months
B. 6 months
C. 4 months
D. 8 months
E. NOTA

Answer 82

B. 6 months

Question 83

A 5 y/o M came into the ER because of marked increase in cyanosis followed by loss of consciousness. He was previously diagnosed with Tetralogy of Fallot. Which mamong the following physical examination finding will support the diagnosis of a hypercyanotic spell?

A. Loss of murmur
B. Bounding pulses
C. Arrhythmia
D. Retractions
E. NOTA

Answer 83

A. Loss of murmur

Question 84

What is the duration of antibiotic treatment for meningitis caused by penicillin-sensitive pneumococcus?

A. 3-5 days
B. 5-7 days
C. 7-10 days
D. 10-14 days
E. NOTA

Answer 84

D. 10-14 days

Question 85

The anterior fontanel usually closes by:

A. 10-12months
B. 13-15 months
C. 16-18 months
D. 19-21 months
E. NOTA

Answer 85

C. 16-18 months

Question 86

The plasticity of the brain continues into:

A. Infancy
B. Childhood
C. Adolescence
D. Adulthood
E. NOTA

Answer 86

C. Adolescence

Question 87

True of object permanence (constancy) except:

A. Defined as the understanding that objects continue to exist even when not seen
B. Infants persist in searching
C. Peek-a-boo
D. Achieved at 10 months old
E. NOTA

Answer 87

D. Achieved at 10 months old

> A major milestone is the achievement by 9 mo of object permanence (constancy). Stranger anxiety almost same time. Introduction of a transitional object may allow the infant to self comfort in the parents’ absence. The object cannot have any potential for asphyxiation or strangulation.

Question 88

A 9 month M with a purpuric rash is unresponsive when brought to the ER. The pulse is barely palpable. After several attempts, you are unable to establish peripheral venous access. Of the ff, which is the BEST site for the next attempt to provide fluid resuscitation for this patient?

A. Femoral artery
B. Internal jugular vein
C. Intraosseous space
D. Saphenous vein
E. Superficial temporal artery

Answer 88

C. Intraosseous space

Question 89

Major criteria for diagnosis of acute rheumatic fever includes the following, except:

A. Polyarthritis
B. Erythema nodosum
C. Subcutaneous nodules
D. Carditis
E. NOTA

Answer 89

B. Erythema nodosum

> B should be erythema marginatum.

Question 90

What is the ideal dose to give a 10 kg child with iron deficiency anemia?

A. Elemenal iron, 3-6 mg/day
B. Elemenal iron, 30-60 mg/day
C. Elemenal iron, 300-600 mg/day
D. Elemenal iron, 33-66 mg/day
E. Elemenal iron, 0.3-0.6 mg/day

Answer 90

B. Elemenal iron, 30-60 mg/day

> The ideal dose for iron supplemention is 3-6 mg/kg/day. For ths child, it should be 30-60 mg/day of elemental iron.

Question 91

Which pf the following Plasmodium species causes the most severe form of malaria:

A. P. falciparum
B. P. malariae
C. P. knowlesi
D. P. ovale
E. P. vivax

Answer 91

A. P. falciparum

Question 92

The severe acute respiratory syndrome outbreak in the early 2000s was caused by what virus:

A. Rhinovirus
B. Influenza virus
C. Coronavirus
D. Parainfluenza virus
E. Respiratory syncitial virus

Answer 92

C. Coronavirus

> The SARS coronavirus, sometimes shortened to SARS- CoV, is the virus that causes severe acute respiratory syndrome (SARS).

Question 93

A 5 y/o boy come in the ER with generalized edema, BP 90/60, HR 80 bpm, BUN 250 mg/dL, Cr 0.5 mg/dL, urine protein is 3600 mg/day. The most common histologic finding in this patient is:

A. Membranous nephropathy
B. Focal segmental glomerulosclerosis
C. Minimal change disease
D. Membranoproliferative nephropathy
E. Mesangioproliferative nephropathy

Answer 93

C. Minimal change disease

> The most common cause of nephrotic syndrome amongst children is MCD, responsive to steroids most of the time.

Question 94

The most common etiologic agent of the most common major complication of the case above is:

A. Klebsiella oxytoca
B. Streptococcus pneumoniae
C. Pseudomonas aeruginosa
D. Staphylococcus aureus
E. Enterococcus faecalis

Answer 94

B. Streptococcus pneumoniae

> The most common cause of spontaneous bacterial peritonitis among patient with MCD is Streptococcus pneumoniae.

Question 95

Anaphylaxis results in what type of shock:

A. Hypovolemic
B. Cardiogenic
C. Distributive
D. Septic
E. Adrenal crisis

Answer 95

C. Distributive

Question 96

Koplik spots are often seen in this stage of Measles infection:

A. Incubation period
B. Prodromal phase
C. Full blown stage
D. Convalescent stage
E. AOTA

Answer 96

B. Prodromal phase

Question 97

An 11 month old baby presents for evaluation of fever and rash. His mother noted a fever of 40 ̊C two days ago. He appeared well and was eating and playing normally, so his mother was not alarmed. After the fever resolved, he developed a red rash on his trunk that progressed rapidly over the past 24 hours? What is the most lilkely diagnosis?

A. Exanthem subitum
B. Rubella
C. Rubeola
D. Erythema infectiosum
E. Menigococcemia

Answer 97

A. Exanthem subitum

> Roseola infantum/exanthem subitum is characterized by the abrupt onset of high fever that usualy resovles after 72 hours coincident with the appearance of a faint pink-rose coloured morbiliform rash on the trunk.

Question 98

In relation to the previous question, what do you call the ulcers at the uvulopalatoglossal junction characteristic of this disease?

A. Koplik’s spots
B. Herpangina
C. Dewdrop on a rose petal
D. Forscheimer’s spots
E. Nagayama’s spots

Answer 98

E. Nagayama’s spots

> IN asian countries, ulcers at the uvulopalatoglossal junction called Nagayama’s spots are commonly reported in infants with roseola

Question 99

An 8 year old male was brought to the pediatrician by his mother because he developed low grade fevers several days ago, and now has red cheeks and a new rash on his body? What is the most lilkely diagnosis?

A. Exanthem subitum
B. Rubella
C. Rubeola
D. Erythema infectiosum
E. Infectious mononucleosis

Answer 99

D. Erythema infectiosum

> The initial stage of erythema infectiosum is an erythematouus facial flushing often described as a slapped cheek appearance. The causative agent is Parvovirus B19

Question 100

In relation to the previous question, what is trhe primary target of the causative organism?

A. Lymphoid cells
B. Myeloid cells
C. Erythroid cells
D. Peyer’s patches
E . Skin langerhans cells

Answer 100

C. Erythroid cells

> The primary target of B19 infection is the erythroid cell line, specifcially erythroid precursors near the pronormoblast stage. There is progressive depletion of erythroid precursos and a transient arrest of erythropoesis.

Question 101

A mother is concerned because her 6 year old son developed low grade fevers, painful ulcers in the mouth, and rashes on his hands and feet. What could be the likely causative agent?

A. Adenovirus
B. Coxsackievirus A16
C. Cytomegalovirus
D. Echovirus 22
E. Epstein Barr virus

Answer 101

B. Coxsackievirus A16

> Coxsackie virus A 16 is the most common cause of hand, foot and mouth disease which is characteristic of the case.

Question 102

A previously healthy girl presents with a 1 week history of cough, runny nose, fever, sore throat and red eyes.
She went to her pediatrician 2 days ago and was prescribed Augmentin (amoxicillin and clavulanate) for presumed pharyngitis. Yesterday, Ana developed a red rash which started on her face and has spread to her trunk. Her mother insists that the rash is from her new medication. What is the most likely diagnosis?

A. Exanthem subitum
B. Rubella
C. Rubeola
D. Erythema infectiosum
E. Fixed drug eruption

Answer 102

C. Rubeola

Question 103

___________ will classically present with the the prodrome of fever, malaise, conjunctivitis, cough, coryza and an exanthem of erythematous macules and papules beginning on the face and spreading cephalocaudally and centrifugally (by the 3rd day, the whole body is involved).

Answer 103

Rubeola

Question 104

Pertaining to the previosu question all of the ff are true regardign this condition EXCEPT?

A. In measles with no bacterial infection, the ESR and CRP are normal
B. The portal of entry is both the respirartory tract and the conjunctivae
C. Measles infection causes necrosis of the respiraroty tract epithelium with lymphocytic infiltrates
D. Patients are infctious from 3 days before up to 4- 6 days after the onet of rashes.
E. In individuals with passively aquired antibody such as infants and recipeinets of blood products, a subclinical form of measles may occur called atypical measles

Answer 104

E. In individuals with passively aquired antibody such as infants and recipeinets of blood products, a subclinical form of measles may occur called atypical measles

Question 105

In individuals with passively aquired antibody such as infants and recipeinets of blood products, a subclinical form of measles may occur called __________.

Answer 105

inapparent measles

Question 106

_______________ is a more severe form of measles due to the use of the original formalin containing vaccine.

Answer 106

Atypical measles

Question 107

What is the most common cause of death of pediatric patients afflicted with measles?

A. Otitis media
B. Meningitis
C. Encephalitis
D. Dehydration due to diarrhea and vomiting
E. Pneumonia

Answer 107

E. Pneumonia

> Pneumonia is the most common cause of death in measles. It may manifest as giant cell pneumonia caused directly by the viral infection or as superimposed bacterial infection.

Question 108

Which of the ff screening tests results is condiered to be positive for sepsis?

A. Total WBC 3000/mm3; immature/total neutrophil ratio of 0.1 and gastric aspirate with neutrophils 3/hpf
B. Micro-ESR 10 mm in 1 hr, total WBC of 4500/mm3 and gastric aspirate with neutrophil of 2/hpf
C. CRP 3 mg/dl; immature/total neutrophil ratio of 0.5 and gastric aspirate with neutrophil 6/hpf
D. Total WBC 6300/mm3; CRP 0.5 mg/dl immature/total neutrophil ratio of 0.2
E. All of the above

Answer 108

C. CRP 3 mg/dl; immature/total neutrophil ratio of 0.5 and gastric aspirate with neutrophil 6/hpf

An immature/total neutrophil ratio of more than 0.2, WBC of less tha 5000, ANC of less than 1,500 and platelet of less than 100,000 is indicative of sepsis in the newborn

Question 109

Which of the ff statements describes accurately meconium aspiration syndrome?

A. 10-15% of births occur in premature babies
B. Respiratory distress usually manifests within 24 hours after birth
C. Chest xray is usually normal
D. Routine intubation is not indicated for vigorous babies
E. None of the above

Answer 109

D. Routine intubation is not indicated for vigorous babies

Routine intubation to aspirate the lungs of vigorous infnts born through meconium stained fluid is not effective in reducing MAS. 10-15% occurs in term or postterm babies. Respiratry distress occurs within the first hour after birth. A typcial CXR is char by patchy infiltrates and coarse streaking of both lung fields.

Question 110

A newly born baby girl os observed to have tremors and hyperirritability. Moter finally admitted taking an illicit drug. Which of the ff substances/drugs is commonly associated with the symptoms?

A. Tobacoo
B. Alcohol
C. Cocaine
D. Heroin
E. All of the above

Answer 110

D. Heroin

Heroin is associated with tremors and hyperirrtiablity of the infant

Question 111

You have been following an 8 year old child in your iffce for the past several years and have noted that durign the past year, his height has remained below the third percentile. You are conerned about his short stature and decide to begin a workup. On bone age determination it was discoviered that the px’s bone age is 4 years younger than his chornological age. Which ofthe ff diagnosis should be considered?

A. Genetic short stature
B. Skeletal dysplasias
C. Intrauterine grwoth retardation
D. Turner’s syndrome
E. Growth hormone deficiency

Answer 111

E. Growth hormone deficiency

Question 112

An 8 year old girl is brought to the ER with a fever of 38.5C, stiff neck, photophobia and headache. Lumbar pucnture was perfromed revealign the ff results: WBC 38- cells/mm3 with 40% PMN and 60% lymphocyte; normal protein and glucose and negative gram stain. Which of the ff is the most likely cause of her mengitis?

A. Neisseria mengitidis
B. Streptococcus pneumoniae
C. Enterovirus
D. Cryptococcus neoformans
E. Mycobacterium tuberculosis

Answer 112

C. Enterovirus

> The CSF analysis is most consistent with aseptic mengitis, specifically viral menigitis. Enterovirus are the most common cause of viral menigitis. There is lymphocytic predominance and normal protein and sugar.

Question 113

An 8 year old girl presents with sore throat, fever and a rough sandpaper like rash over her trunk and extremities. A throat culture is positive for group A Beta hemolytic streptococcus. Treatment of her infection with antibiotics will prevent which of the ff complications?

A. Reactive arthritis
B. Rheumatic fever
C. Post strep glomerlonephritis
D. Guillan barre syndrome
E. All of the above

Answer 113

B. Rheumatic fever

> The patient’s clinical presentation is consistent wit hscarlet fever, caused by the erythrogenic strain of GAHBS. Althoigh there are multiple complication of GABHS as mentioned in the question, only rheumatic fever will be prevented by treatment of antibiotics.

Question 114

A newborn male had cyanosis on birth. On auscultation, you hear a single S2 but no murmur. O2 sat is 72% in room air. An ECG reveals left axis deviation and elft ventricular hypertrophy. What is the most likely diagnosis?

A. Tetralogy of Fallot
B. Transposition of the great arteries
C. Truncus arteriosus
D. Total anomalous pulmonary venous connection
E. Tricuspid atresia with intact ventricular septum

Answer 114

E. Tricuspid atresia with intact ventricular septum

> Tricuspid atresia is the only casue of cyanosis in the newborn period that manifests with left axis deviation and left ventricular hypertrophy on ECG. Patients with TA withot a ventricular septal defect have a single S2 as a result of the sual coexistence of pulmonary atresia and do not have a murmur.

Question 115

An 8 year old has a chest radiograph that shows cadiomegaly with a snowman appearance. What is the most likely diagnosis?

A. Tetraloogy of Fallot
B. Transposition of the great arteries
C. Truncus arteriosus
D. Total anomalous pulmonary venous connection
E. Tricuspid atresia with intact ventricular septum

Answer 115

D. Total anomalous pulmonary venous connection

> The classic CXR appearance of TAPVR is the snowman appearance

Question 116

A 3 year old boy presents with high grade fever, anorexia and drooliong. He has been previously well. His immunixation records are up to date. On examination he appears very ill and prefers to lean forward on his hands and his neck hyperextended. His voice is muffled. Which of the ff is correct regarding his diagnosis?

A The patient likely has bacterial tracheitis and should be started on antistaphylococcal antibiotics.
B. Racemic epinephrine should be immediately administered.
C. The patient should have his airway visualized and intubated in a controlled environment
D. A neck radiograph will show a steeple sign
E. The throat should be examined to rule out a retropharngeal abscess

Answer 116

C. The patient should have his airway visualized and intubated in a controlled environment

> All of the symptoms and PE findings of the patient points to a possible epiglottitis. One must avoid erxcessive stimulation, including examination of the pharynx with a tongue depressor bec this will induce respiratory distress. Evaluation of the airway and intubation is necessary.

Question 117

A 5 month old infant in a daycare facility develops low grade fever, rhonorrhea and cough. A few days later, she is brought to the ER with tachypnea, chest retractions, diffuse expiratory whezing and fine inspiratory rales bilateraly. Which of the ff is correct regarding his diagnosis?

A. Chest xray will demonstrate decreased lung volumes with bilateral lobar consolidation
B. Chlamydia trachomatis should be condered as a possible etiologic agent
C. Supportive care is the most important management
D. IV antibiotics should be started
E. Ribavarin should be administered.

Answer 117

C. Supportive care is the most important management

Question 118

A 9 month old girl is brought to the ER with a 1 day history of intermittent, inconsolable crying. She seems comfrotable in between the attacks. She has vomited twice and has had one bowel movement that the mother described as bloody. Based on the clinical presentation what is the most appropriate diagnostic procedure?

A. Plain abdominal xray
B. Plain whole abdomen CT scan
C. Barium enema
D. Surgical exploration
E. Abdominal UTZ

Answer 118

C. Barium enema

Question 119

A 14 year old boy has 3+ protein and 4+ blood with RBC casts on urinalysis. Further questioning revelaed 2 prior episodes of tea coloured urine concurrent with upper respiratory tract infection during the last 3 years. Which of the ff is the most likely diagnosis?

A. Membranous nephropathy
B. SLE nephritis
C. IgA nephropathy
D. Membranoproliferatifve GN
E. Post streptococcal GN

Answer 119

C. IgA nephropathy

> This patient’s clinical presentation are consistnet with IgA nephropathy. They usually present with recurrent bouts of gross hematuria assoc with respiratory infections.

Question 120

A 5 year old female came in for complaints of cough and watery nasal discharges 1 week PTA. Upon examination at the ER of CVG hospital, T = 38C RR = 42 with nasal flaring and subcostal retractions. Crackles are heard on both lung fields. Abdominal distention was also noted. CXR revealed hyperinflation with bilateral interstitial infiltrates and peribronchial cuffing. Which of the following is the most likely diagnosis?

a. bronchiolitis
b. pneumonia
c. acute bronchitis
d. asthma
e. NOTA

Answer 120

b. pneumonia

Question 121

An 18 year old male came in for complaints of cough and watery nasal discharges 1 week PTA. Upon examination at the ER, T = 38.5C, RR = 65, dyspnea and difficulty to console the child. After 1 hour, pulse oximetry revealed 70% thus patient was intubated. Auscultation of chest revealed fine crackles on both lung fields with occasional wheezes. CBC taken was normal. CXR revealed hyperinflation and air trapping. Which of the following is the most likely diagnosis?

a. bronchiolitis
b. pneumonia
c. acute bronchitis
d. asthma
e. NOTA

Answer 121

a. bronchiolitis

Question 122

A 2 year old female comes in for complaints of low grade fever and watery nasal discharges 1 week PTA. Two days PTA, dry hacking cough was noted now becoming productive with yellowish sputum. Auscultation of the chest revealed coarse breath sounds. CXR and CBC were unremarkable. Which of the following is the most likely diagnosis?

a. bronchiolitis
b. pneumonia
c. acute bronchitis
d. asthma
e. NOTA

Answer 122

c. acute bronchitis

Question 123

A five year old male presents to the emergency room with sudden onset right sided hemiparesis. Two months ago, patient was diagnosed with iron deficiency anemia and ascariasis. Which of the following is the most likely diagnosis?

a. Todd paralysis
b. migraine
c. meningitis (CNS infection)
d. ischemic stroke
e. NOTA

Answer 123

d. ischemic stroke

> The acute onset of a focal neurologic deficit in a child is stroke until proven otherwise. THe msot common focal presentation is hemiparesis but acute visual, speech, sensory or balance deficits also occur. Three main etiologies are arteriopathic, cardiac, and hematological (sickle cell anemia and IDA)

Question 124

Which of the following laboratory tests in the above situation would confirm your impression?

a. blood cultures
b. MRI
c. EEG
d. lumbar tap
e. CT scan

Answer 124

B. MRI

> acute ischemic stroke is a clinical and radiographic diagnosis. CT imaging can demonstrate larger mature AIS and exclude hemorrhage, however, MRI identifies early and small infarcts and is therefore required to exclude ischemic stroke.

Question 125

Which of the following would be the most appropriate initial management strategy for the patient above?

a. call your friendly pediatric neurologist
b. neuroprotective strategies
c. antithrombotic strategies
d. correction of iron deficiency anemia
e. rehabilitation

Answer 125

a. call your friendly pediatric neurologist

Question 126

What is the definitive treatment in the above condition?

a. broad spectrum antibiotics
b. carbamazepine
c. careful control of cerebral perfusion pressure
d. antithrombosis
e. NOTA

Answer 126

D. Antithrombosis

> emergency thrombolysis is not yet established for children. Antithrombotic strategies depend on the suspected cause but include anticoagulation with heparins or antiplatelet strategies such as aspirin.

Question 127

A previously healthy male 3 year old child followed up in your clinic for official reading of TST which turned out to be 5mm. Which of the following is true?

a. patient is immunosuppressed
b. results is equivocal
c. negative skin test
d. positive skin test
e. confirmation of history of exposure

Answer 127

C. Negative skin test

Question 128

A 2 year old male, CF, presents to the clinic with fever and rash which began on the forehead and on upper neck spreading downward to extremities which subsequently faded leaving a fine desquamation. CF also has a brother who is 13 months old who has been exposed 4 days ago when his grandmother brought him along to visit him. Which of the following is the most appropriate management?

a. give measles vaccine to CF’s brother
b. give measles immune globulin to CF’s brother
c. give both vaccine and immune globulin to CF’s brother
d. Give vitamin A 50,000 IU to CF
e. give vitamin A 100,000 IU to CF’s brother

Answer 128

b. give measles immune globulin to CF’s brother

> Measles vaccine only effective in prevention or modification of measles if given within 72 hours of exposure. Immune globulin may be given up to 6 days after exposure.
Vitamin A dosages is 50,000 IU for less than 6 months, 100,000 6mo-1 yr, and 200,000 >= 1 year old

Question 129

A 25 year old G3P2 (2002) mother, KEC, was admitted to VCMC hospital due to hyperemesis gravidarum. Her 2 year old child started to have intensely pruritic erythematous macules that evolve through papules then vesicles distributed centripetally 2 days ago. KEC also has a 12 month old child at home and a 14 year old neighbor who got exposed. Which of the following is the most appropriate management?

a. give varicella vaccine to the 12month old child
b. give VariZIG to KEC
c. give VariZIG to the 14 year old neighbor
d.both A and B
e.both B and C

Answer 129

D. Both A and B

Question 130

When should you suspect the 14 year old neighbor to develop the same symptoms with the 2 year old child of KEC?

a. 3-5days
b. 10-21days
c. 24-48hours
d. 7-10days
e. 3-4weeks

Answer 130

B. 10-21 days

Question 131

After 2 days, KEC was discharged. Nurse JoAnne who is also a neighbor of KEC came to visit her to check on her blood pressure at home but was also exposed to KEC’s 2 year old child. Which of the following is not true regarding prevention of VZV transmission?

a. person is contagious 24-48 hours before rash appears
b. unvaccinated health care workers who are exposed should be furloughed for 8 - 21 days
c. varicella vaccination can be given to KEC
d. evidence of immunity may be through verification of history of herpes zoster disease by health care provider
e. NOTA

Answer 131

c. varicella vaccination can be given to KEC

Question 132

A 3 year old female comes to you with complaints of a rapidly growing left abdominal mass that crosses the midline associated with a purplish rash and weight loss. Which of the following is the most likely diagnosis?

a. rhabdomyosarcoma
b. hepatoblastoma
c. wilm’s tumor
d. neuroblastoma

Answer 132

D. Neuroblastoma

Question 133

RL, a 16 year old male, came in for recurrent tonsilitis 5x this year. Patient underwent bilateral tonsillectomy and during the 1st postoperative day, recurrent bleeding at operative site was noted. Laboratories requested were as follows: bleeding time = 3 minutes (2 - 8); PTT= 78 seconds (21 - 35); INR = 1.0; mixing studies = correction of PTT; CBC and thrombin time within normal limits. Which of the following is the most likely diagnosis?

a. von willebrand’s disease
b. antiphospholipid antibody syndrome
c. hemophilia
d. DIC
e. parahemophilia

Answer 133

C. Hemophilia

> In hemophilia, PTT value is usually 2 - 3 times the upper limit of normal. REsults of other screening tests (platelet count, bleeding time, PT, thrombin time) are normal.

Question 134

A 7 year old male came in for high grade fever 6 days PTC associated with a polymorphous rash, diffuse injection of pharynx, cervical lymphadenopathy, and periungual peeling of toes. Which of the following is the most appropriate management for this patient?

a. ASA
b. IVIG
c. dexamethasone
d. Both A and B
e. all of the above

Answer 134

D. Both A and B

> In the acute stage of Kawasaki disease, give IVIG and aspirin. Long term therapy for patients with coronary abnormalities include aspirin, clopidogrel, warfarin, LMWH.

Question 135

A 12 year old female came in for knee pain and swelling 3 months PTA. Patient cannot participate in physical education classes due to limited range of motion. One month PTA, patient now noted ankle and wrist pain and swelling associated with fever, generalized lymphadenopathy, and migratory salmon colored rash. Upon physical examination, pericardial friction rub was heard. ANA was negative while CBC showed anemia. Which of the following is the most likely diagnosis?

a. systemic JIA
b. sarcoidosis
c. SLE
d. rheumatoid arthritis
e. acute rheumatic fever

Answer 135

a. systemic JIA

> It is characterized by arthritis, fever, and prominent visceral involvement including HSM, LAD, and serositis (pericarditis). The evanescent salmon colored lesions classic for systemic onset disease are linear or circular and most commonly distributed over trunk and proximal extremities.

Question 136

An 8 year old presented to the OPD of CVGH with multiple subcutaneous nodules. On physical examination, HR = 120, RR = 42, O2 sats = 89%, multiple palatal petechiae, pale palpebral conjunctivae, coarse crackles on left lung field, subcostal retractions, generalized lymphadenopathy. Patient was then transported immediately to ER and the following labs were taken: CBC = anemia with leukocytosis; PTT and PT were elevated. Patient had difficulty breathing and went into respiratory arrest. Upon intubation, pinkish frothy sputum was noted and IV sites were oozing with blood. Which of the following is not true?

a. this is diagnosed through bone marrow biopsy
b. a t(15;17) has favorable prognosis
c. M2 subtype usually presents with DIC
d. remission occurs in 85 - 90% with chemotherapy
e. cell of origin is myeloblast

Answer 136

c. M2 subtype usually presents with DIC

> M3 subtype acute promyeloblastic leukemia presents with DIC due to granules present in the blast cells which trigger the coagulation cascade

Question 137

Which of the following congenital heart diseases presents with increased pulmonary blood flow?

a. total anomalous pulmonary venous return
b. hypoplastic left heart syndrome
c. double outlet right ventricle
d. both A and B
e. both B and C

Answer 137

D. Both A and B

Question 138

A neonate 36 weeks by BS was admitted to the NICU for further evaluation due to an episode of apnea and cyanosis. Currently, patient is grunting with subcostal retractions. CXR revealed ground glass infiltrates on both lung fields. O2 sats remain 89%. Which of the following is not true?

a. hyperoxia test can distinguish cyanotic congenital heart disease from pulmonary disease
b. neonates with Congenital heart disease can increase PaO2 when given 100% oxygen
c. if due to a CNS disorder, PaO2 will not normalize with 100% oxygen through artificial ventilation
d. Both A and B
e. Both B and C

Answer 138

e. Both B and C

Question 139

A 6 month old infant came in for complaints of stridor. Upon workup, a barium esophagram shows a posterior indentation of the esophagus. Which of the following is the most likely diagnosis?

a. laryngomalacia
b. vascular ring
c. posterior laryngeal cleft
d. congenital subglottic hemangioma
e. congenital subglottic stenosis

Answer 139

B. Vascular ring

> vascular ring results from abnormal development of aortic arch complex. THe double aortic arch is the most common complete vascular ring encircling both trachea and esophagus compressing both.