Pediatrics Questions Flashcards

1
Q

Smooth, nontender abdominal mass that does not cross midline. Associated with abdominal pain, low-grade fever, HTN, and hematuria. Normal renal function.

A. Gastric duplication cyst
B. Neuroblastoma
C. Polycystic kidney diseawse
D. Rhabdomyosarcoma
E. Wilm's tumor
A

E. Wilm’s tumor

Dx with abdominal ultrasound, confirm with biopsy. CT chest and abdomen to look for mets.

Tx with nephrectomy and post-surgical chemo, possibly radiation

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2
Q

A 5-year-old boy presents to the emergency department with two days of abdominal pain and one day of rash. He has not had any vomiting. He had a normal stool today. Medical history is significant for an upper respiratory tract infection one week ago. He is otherwise healthy and takes no medications. Vital signs are normal. On examination he has petechia and purpura on his buttocks and lower extremities. Palpation of the abdomen reveals mild tenderness. The remainder of the exam is normal. Laboratory studies are normal. A clinical diagnosis is made. Which of the following is most important to monitor at this patient’s follow-up visit in two days?

A. BP and urinalysis
B. Platelet count
C. PT/PTT
D. White blood cells and hemoglobin
E. Signs of intracranial hemorrhage
A

A. BP and urinalysis

This patient likely has Henoch-Schonlein Purpura (HSP). The exact etiology of HSP is unknown, but it is believed to involve an IgA-mediated immune response to infection or other triggers. The incidence is 10 cases per 100,000 children with a peak at ages 4 to 6 years (range of 2 to 17 years). HSP is characterized by a rash consisting of petechiae and palpable purpura. Other findings include a colicky diffuse or periumbilical abdominal pain, arthritis or arthralgia, and renal disease. Given the incidence of renal disease, it is important to check the urine for signs of hematuria or proteinuria; sudden changes in blood pressure can potentially suggest a change in renal function. With abnormal findings, serum BUN and creatinine must be checked.

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3
Q

1 week old male with uncomplicated vaginal delivery presents with mom d/t complaint of recurrent epistaxis. VSS. PE reveals murmur and a TTE is ordered which reveals a bicuspid aortic valve and a PDA. Which of the following is most likely also present?

A. coarctation of aorta
B. ostium primum ASD
C. ostium secundum ASD
D. tetralogy of fallot
E. total anomalous pulmonary venous return
A

A. coarctation of aorta

[Coarctations are often associated with bicuspid aortic valve, PDA, and/or VSD. Due to increase in upper body flow and pressure, epistaxis, headache, LE ischemic sxs, dilated costal arteries, and CHF are commonly found]

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4
Q

You dx a pt with ataxia telangiectasia. Which of the following immunologic abnormalities would be expected in this pt?

A. elevated IgA and IgE with reduced levels of IgM
B. IgA deficiency
C. low or absent levels of all immunoglobulins
D. normal immunoglobulins with ineffective neutrophils
E. undetectable IgA levels with elevated IgM and normal IgG

A

E. undetectable IgA levels with elevated IgM and normal IgG

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5
Q

7 y/o female presents to ED with 5 day hx of fever, malaise, and anorexia. PMH includes aortic stenosis. PE reveals loud systolic ejection murmur at cardiac base that radiates to carotids and systolic ejection click. Splenomegaly and petechial eruption on palms and soles also present. Labs reveal WBC 19,000 and ESR of 78. Which of the following is correct regarding most likely dx?

A. Abnormal rheumatoid factor is unusual
B. TTE is most sensitive test for detecting vegetations
C. Gram negative rods are most common cause
D. Ophthalmologic eval is useful in dx
E. Abx should be administered prior to blood culture

A

D. Ophthalmologic eval is useful in dx

[This is bacterial endocarditis; Roth spots and retinal hemorrhages on ophtho exam can help confirm dx]

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6
Q

Pt presents later in life with low urinary excretion of calcium and high urinary excretion of magnesium leading to hypomagnesemia. They also have hypokalemia and hypochloremic metabolic alkalosis

A. Bartter syndrome
B. Gitelman syndrome
C. Goodpasture syndrome
D. Gullner syndrome
E. Liddle syndrome
A

B. Gitelman syndrome

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7
Q

10 y/o brought in with tibial fracture extending from the epiphysis, through the physis, and into the metaphysis. What is salter-harris grade?

A. Grade I
B. Grade II
C. Grade III
D. Grade IV
E. Grade V
A

D. Grade IV

Grade I = within the physis
Grade II = within the physis and extends into metaphysis
Grade III = in the physis and extends into epiphysis
Grade IV = from epiphysis, through physis, and into metaphysis
Grade V = crush injury of the physis

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8
Q

Sickle cell patient presents with abrupt development of nausea, vomiting, and crampy abdominal pain followed by moderate watery diarrhea with specks of blood and mucus. The patient has a pet snake. Most appropriate therapy?

A. Amoxicillin
B. Amoxicillin-clav
C. Cefotaxime
D. Ciprofloxacin
E. Gentamicin
A

C. Cefotaxime

Pt has acute bacterial gastroenteritis d/t Salmonella – risk factor is infected domestic animals like dogs, cats, reptiles, and amphibians.

Tx with 3rd gen cephalosporin like cefotaxime, ceftriaxone, or cefixime. Ampicillin may also be used.

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9
Q

A previously healthy 4-year-old girl is brought to her clinician because her parents have noticed that she has been less active than usual for the past three weeks. Her father explains that it is difficult to get his daughter out of bed in the mornings and that she no longer plays outside with her older brother. Physical examination is notable for a temperature of 38.4 C, heart rate of 125 bpm, pallor, truncal bruising, and diffuse lymphadenopathy. The remainder of the exam, including a thorough neurologic assessment, is unremarkable. Which of the following is the most likely diagnosis?

A. Aseptic meningitis
B. Kawasaki disease
C. Non-accidental trauma
D. Acute lymphoblastic leukemia
E. Mononucleosis
A

D. Acute lymphoblastic leukemia

The child presents with evidence of anemia (fatigue, tachycardia, pallor) and thrombocytopenia (unexplained bruising). Failure of two or more hematologic cell lines should always raise suspicion for malignant invasion of the marrow. Furthermore, the child’s chief complaint, fatigue, is the most common presenting symptom of acute leukemia. Finally, the incidence of ALL peaks at age 4 years.

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10
Q

You are examining a LGA infant born via vaginal delivery at 39 weeks. Pregnancy was complicated by poorly controlled gestational DM requiring insulin therapy. PE of infant reveals asymmetrical Moro reflex with impaired movement of LUE. The left SCM is in spasm. Grasp reflex is intact. Dx?

A. Clavicle fracture
B. Erb-Duchenne palsy
C. Klumpke palsy
D. Shoulder dislocation
E. Torticollis
A

A. Clavicle fracture

Clavicle fracture results in absent Moro on affected side, along with SCM spasm

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11
Q

Which of the following electrolyte abnormalities is most likely in an infant born to a diabetic mother?

A. Hypermagnesemia
B. Hypernatremia
C. Hypocalcemia
D. Hypokalemia
E. Hyponatremia
A

C. Hypocalcemia

The metabolic/electrolyte abnormalities most frequently observed in infants of diabetic mothers are hypoglycemia, hypocalcemia, and hypomagnesemia]

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12
Q

A 6 y/o girl presents with uncomplicated Lymes. Which of the following is most appropriate tx?

A. Oral doxycycline
B. Oral amoxicillin
C. IV penicillin
D. IV ceftriaxone
E. No abx
A

B. Oral amoxicillin

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13
Q

A 4 y/o male presents w/ 2 week history of viral URI sxs and 2-day hx of swelling around eyes. PE reveals normal vitals, moderate periorbital edema, mild scrotal edema, and pedal edema. UA reveals 3+ proteinuria. Which of the following is most accurate regarding the most likely dx?

A. Low serum cholesterol
B. Predisposed to hemorrhage
C. If pt becomes febrile, start empiric abx to cover for encapsulated organisms
D. Refer for diagnostic renal bx
E. Pt will not respond to corticosteroids and requires cyclophosphamide or cyclosporin

A

C. If pt becomes febrile, start empiric abx to cover for encapsulated organisms

Pts with nephrotic syndrome are at increased risk for infection with encapsulated organisms such as strep pneumo

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14
Q

Polymorphous rash with high fever lasting at least 5 days, plus: adenopathy, edema/erythema/peeling of oral cavity, edema/erythema/desquamation of extremities, conjunctivitis. Dx?

A. Kawasaki's disease
B. Roseola
C. Rubella
D. Rubeola
E. Scarlet fever
F. Varicella Zoster
A

A. Kawasaki’s disease

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15
Q

Pts with homocystinuria require a methionine-restricted diet, and supplementation with folic acid and pyridoxine. What other medication should be added to the regimen?

A. Propranolol
B. Vitamin C
C. D-penicillamine
D. Aspirin
E. Zinc
A

D. Aspirin

[due to hypercoagulability]

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16
Q

A 6 wk old male presents in September with 2 day hx of cough and increased work of breathing. PMH is significant for uncomplicated vaginal delivery at 38 wks. At 10 days of age, he was treated for conjunctivitis with erythromycin ointment. On PE, he is afebrile, coughing rapidly and has RR of 54. Diffuse wheezes are present throughout lung fields with mild subcostal retractions. Which of the following is the most likely cause?

A. Mycoplasma pneumoniae
B. Chlamydia trachomatis
C. Respiratory syncytial virus
D. Streptococcus pneumoniae
E. Asthma
A

B. Chlamydia trachomatis

Chlamydia trachomatis is a common cause of afebrile PNA in a child 1-3 months of age. It presents with staccato cough, respiratory distress, and a history of conjunctivitis in 50%. Often presents with tachypnea and wheezing.

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17
Q

A 6-week-old infant presents to your office for a check-up. The baby was born full-term by NSVD to a 29-year-old G1P0 mother with no complications. Mother states the baby was feeding well until a week ago, when he developed increased sleepiness, prolonged feeding, and greater duration between feeds. His mother notes he stops to take breaks during feeds because he seems to be trying to catch his breath. He has four to six wet diapers per day and stools three or four times per day. Vital signs: Temperature is 37.6 C (99.7F), respiratory rate is 68 breaths/minute, pulse is 138 beats/minute, blood pressure is 88/58 mmHg, and oxygen saturation is 98%. The physical examination is notable for increased respiratory effort and retractions, and, upon cardiac examination, a murmur with a hyperactive precordium and no cyanosis. Abdominal exam reveals a liver edge palpable to 4 cm below the right costal margin. Which condition would be LEAST LIKELY to be the cause of the infant’s symptoms?

A. Aortic stenosis
B. Atrial septal defect
C. Coarctation of the aorta
D. Patent ductus arteriosus
E. Ventricular septal defect
A

B. Atrial septal defect

Choice B is correct because atrial septal defects (ASDs) do not cause CHF. ASDs often go undiagnosed for decades due to subtle physical examination findings and/or a lack of appreciable symptoms. Children with ASD’s are generally asymptomatic

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18
Q

A 13 y/o trauma victim presents unconscious with suspected head injury. On exam, he does not open his eyes to voice or pain. He has decerebrate posturing of the extremities to pain, and he moans with stimulation (you are not able to understand any words). What is his GCS?

A. 4
B. 5
C. 6
D. 7
E. 8
A

B. 5

Does not open eyes to pain or voice = +1
Decerebrate posturing = +2
Moans to stimulation with incomprehensible words = +2

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19
Q

11 day old infant with bilateral eye discharge. PE reveals conjunctival injection, lid swelling, and scant mucopurulent discharge. No rashes or skin lesions present. Most likely pathogen?

A. Chlamydia trachomatis
B. Neisseria gonorrhoeae
C. Staphylococcus aureus
D. Adenovirus
E. Herpes simplex virus
A

A. Chlamydia trachomatis

This is neonatal conjunctivitis – MCC is Chlamydia trachomatis. Usually occurs second week of life with mild to moderate lid and conjunctival inflammation, and a small amount of purulent discharge. Tx with 14 days of oral erythromycin

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20
Q

Which of the following findings is characteristic of the galactosemias?

A. Elevated creatine kinase
B. Direct hyperbilirubinemia
C. Hyperglycemia
D. Hypertriglyceridemia
E. Hypertrophic cardiomyopathy
A

B. Direct hyperbilirubinemia

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21
Q

Pt presents with polyhydramnios, premature delivery, severe hypokalemia, hypochloremia, metabolic alkalosis, and normal BP

A. Bartter syndrome
B. Gitelman syndrome
C. Goodpasture syndrome
D. Gullner syndrome
E. Liddle syndrome
A

A. Bartter syndrome

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22
Q

Asthma symptoms occurring more than 2 times per week with 3-4 nocturnal awakenings per month. Classification?

A. Exercise-induced
B. Intermittent
C. Mild persistent
D. Moderate persistent
E. Severe persistent
A

C. Mild persistent

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23
Q

Low-pitched, musical or vibratory sound best heard at the left lower sternal border or between the left border and the apex. Louder in supine position and increases with fever and/or anemia. Decreases in upright position.

A. ASD
B. PDA
C. Peripheral pulmonary stenosis
D. Stills murmur
E. Venous hum
A

D. Stills murmur

Most common innocent murmur, usually heard in children 3-6.

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24
Q

A newborn is dx with Trisomy 21. TTE is performed demonstrating a cleft anterior mitral valve leaflet. Which of the following is most likely also present?

A. coarctation of aorta
B. ostium primum ASD
C. ostium secundum ASD
D. tetralogy of fallot
E. total anomalous pulmonary venous return
A

B. ostium primum ASD

[Ostium primum ASDs are located in the inferior portion of the septum and are associated with clefts in the anterior leaflet of the mitral valve. Ostium secundum ASDs are associated with mitral valve prolapse and regurgitation]

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25
Q

A previously healthy and developmentally normal 16-month-old male comes to the urgent care clinic with his father with a chief complaint of his first reported seizure. The child was reported to have dropped to the floor with loss of consciousness and had sporadic twitchy movements of his legs and arms that lasted for five minutes. The child has had URI symptoms for the past two days, with a fever to 103 degrees F without any changes in mental status. Neither parent has a seizure disorder, but the child’s mother reports having a single seizure as a young girl once after developing a high fever after a cold. What is the most likely diagnosis?

A. Epilepsy
B. Simple febrile seizure
C. Complex febrile seizure
D. Absence seizure
E. Cyanotic breath-holding spell
A

B. Simple febrile seizure

Simple febrile seizures are characterized by < 15 minutes duration, occurring only once in a 24-hour period, and are generalized (in this patient’s case, generalized tonic-clonic). A positive family history for febrile seizures in the parents makes it a more likely diagnosis in their children. This particular diagnosis fits this patient the best.

A COMPLEX febrile seizure is characterized by > 15 minute duration, occurring more than once during a 24-hour period, and being focal. Given that this child’s seizure was associated with loss of consciousness and tonic-clonic movements lasting five minutes, and occurred only once during a 24-hour period, complex febrile seizure is not the most likely diagnosis.

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26
Q

Best empiric treatment for pediatric pneumonia in neonate at risk for sepsis?

A. IV ampicillin + cefotaxime
B. IV azithromycin
C. IV ceftriaxone
D. Oral amoxicillin
E. Oral amoxicillin-clavulanate
A

A. IV ampicillin + cefotaxime

First-line empiric treatment for pediatric pneumonia should consist of ampicillin to cover Listeria and group B strep AND an amino glycoside like gentamicin OR a 3rd gen cephalosporin like cefotaxime for expanded coverage of gram negative organisms like E.coli

27
Q

A 3 y/o female is dx with cervical adenitis and is discharged home with a 7 day course of amoxicillin-clavulanate. 5 days into therapy, she presents with no decrease in size of the enlarged cervical node. The child’s fevers have also been persistent. PE shows a febrile toddler with conjunctivitis, pharyngitis, right anterior cervical LAD (the node is 2 cm in diameter), and an erythematous macular rash on the chest and back. Which of the following courses of management is most appropriate at this time?

A. Send pt home on oral PCN with presumptive dx of Scarlet fever
B. Perform TST and discharge on oral dicloxacillin, f/u in 48 hrs
C. Admit pt for rule-out sepsis workup that includes evaluation of blood, urine, and CSF cultures for bacterial infection
D. Admit pt for high-dose IVIG
E. Admit pt for high-dose corticosteroid therapy

A

D. Admit the pt for high-dose IVIG

Dx is Kawasaki disease. Tx is high-dose IVIG and ASA

28
Q

Asthma symptoms that are daily with nocturnal awakenings more than once per week with some limitation in normal activity. Classification?

A. Exercise-induced
B. Intermittent
C. Mild persistent
D. Moderate persistent
E. Severe persistent
A

D. Moderate persistent

29
Q

Pt with Down syndrome presents with prominent second heart sound, and systolic ejection murmur best appreciated at left upper sternal border. Which of the following is the most likely cause of the cardiac murmur?

A. Coarctation of aorta
B. Complete AV canal defect
C. Mitral valve prolapse
D. Patent ductus arteriosus
E. Pulmonic stenosis
A

B. Complete AV canal defect

30
Q

A 5-year-old boy is brought to his pediatrician with two days of right ankle pain and rash over both lower extremities. He has not had any fever and is previously healthy. He recovered from an upper respiratory infection two weeks ago. Vital signs are normal. His right ankle is swollen and mildly tender. He has palpable purpura over both lower extremities. Which of the following statements is true about his likely diagnosis?

A. CBC would likely reveal thrombocytopenia
B. This disease is classified as a small vessel vasculitis
C. Urinalysis is not warranted for this patient’s work-up
D. Treatment options include IVIG
E. This disease is equally common in girls and boys

A

B. This disease is classified as a small vessel vasculitis

HSP is classified as a small vessel vasculitis. The exact mechanism of HSP is unknown; however, it is thought to be an IgA-mediated immune response affecting small vessels (skin, GI tract, joints, kidneys). Approximately 50% of cases follow viral or bacterial URIs. Biopsy of affected organs shows leukocytoclastic vasculitis with IgA deposition

31
Q

Infant with suckling difficulty. What CN affected?

A. accessory
B. facial
C. glossopharyngeal
D. trigeminal
E. vagus
A

C. glossopharyngeal

[Irritation of glossopharyngeal and hypoglossal nerves from compression of jugular foramen]

32
Q

4 day old infant with bilateral eye discharge. PE reveals severe conjunctival inflammation and profuse purulent discharge. Most likely pathogen?

A. Chlamydia trachomatis
B. Neisseria gonorrhoeae
C. Staphylococcus aureus
D. Adenovirus
E. Herpes simplex virus
A

B. Neisseria gonorrhoeae

[Tx with IM ceftriaxone]

33
Q

You are called to evaluate a blue infant. On admin of 100% O2, there is no significant increase in pts PaO2. Which of the following is the most likely dx?

A. Neonatal pneumonia
B. Tetralogy of fallot
C. Respiratory distress syndrome
D. Truncus arteriosus
E. Meconium aspiration syndrome
A

B. Tetralogy of fallot

34
Q

Pathologic mechanism of fragile X syndrome

a. gene deletion
b. multifactorial
c. nonsense mutation
d. trinucleotide repeat
e. trisomy

A

d. trinucleotide repeat

Caused by CGG repeat in FMR1 region of X chromosome. X-linked dominant inheritance.

35
Q

Which of the following defects is associated with exstrophy of the bladder?

A. cleft palate
B. cloacal exstrophy
C. epispadias
D. meningocele
E. posterior urethral valves
A

C. epispadias

Other associated defects include neuro abnormalities, cloacal defects, and omphalocele

36
Q

Gastroschisis causes a defect to the right of the umbilicus. What is the etiology?

A. failure of closure of umbilical ring
B. intrauterine mesenteric ischemia
C. involution of the second umbilical vein
D. patent processus vaginalis
E. persistence of the umbilical ring
A

C. involution of the second umbilical vein

37
Q

A 5-year-old boy is noted to have a grade II systolic murmur and a widely split S2 murmur on cardiac exam. His vital signs are stable and he has been asymptomatic. Which of the following statement is accurate regarding this child’s presentation and likely condition?
A. Chest x-ray, ECG, and echocardiogram would be indicated as next steps to work up a presumed ventricular septal defect
B. No further work-up for a presumed venous hum
C. This patient’s murmur is caused by excessive flow through the pulmonary outflow tract and should be evaluated
D. This patient should be scheduled for cardiac catheterization

A

C. This patient’s murmur is caused by excessive flow through the pulmonary outflow tract and should be evaluated

This patient’s murmur is likely caused by an atrial septal defect, which causes additional blood flow through the pulmonary outflow tract (representing the combined normal systemic venous return to the right heart plus additional blood coming across the ASD) and should be evaluated.

38
Q

8 y/o presents with low-grade fever, headache, and pruritic rash. Mom reports rash began on cheeks, then spread to trunk and extremities. Exam reveals maculopapular rash with lacy, reticulated appearance. dx?

A. Erlichiosis
B. Measles
C. Erythema infectiosum
D. Roseola infantum
E. Rubella
A

C. Erythema infectiosum

39
Q

A 2-year-old female with normal birth and developmental history presents with increased agitation and decreased arousability. Her father suffers from chronic pain secondary to a back injury, and her mother found an open container of pills on the bed. Vitals reflect bradycardia, bradypnea, hypotension, and slight hypothermia. On physical exam, she exhibits somnolence, constricted pupils, hypoactive bowel sounds, and hyporeflexia. What substance was most likely ingested?

A. Iron
B. Amitriptyline
C. Insecticides
D. Hydromorphone

A

D. Hydromorphone

40
Q

You are examining a LGA infant born via vaginal delivery at 39 weeks. Pregnancy was complicated by poorly controlled gestational DM requiring insulin therapy. PE of infant reveals asymmetric Moro reflex and one arm remains adducted and internally rotated at child’s side with the palm pronated. Dx?

A. Clavicle fracture
B. Erb-Duchenne palsy
C. Klumpke palsy
D. Shoulder dislocation
E. Torticollis
A

B. Erb-Duchenne palsy

Caused by injury to C5-C6 – stretching or tearing of upper portion of brachial plexus as result of excess flexion of head away from shoulder

41
Q

The American Academy of Pediatrics recommends that all children receive autism screening at 18 and 24 months. Which of the following tools is used for this?

A. ASQ:SE
B. Conner's rating scale
C. Denver Developmental Screening Test-II
D. M-CHAT
E. Vanderbilt Parent Rating Scale
A

D. M-CHAT

[Modified Checklist for Autism in Toddlers]

42
Q

Daytime asthma symptoms occurring 2 or fewer days per week, 2 or fewer nocturnal awakenings per month, and SABA use less than 2 times per week. Classification?

A. Exercise-induced
B. Intermittent
C. Mild persistent
D. Moderate persistent
E. Severe persistent
A

B. Intermittent

43
Q

You are examining an infant born via uncomplicated vaginal delivery at 39 weeks. PE of infant reveals paralysis of one hand with impaired wrist and finger flexion. Dx?

A. Clavicle fracture
B. Erb-Duchenne palsy
C. Klumpke palsy
D. Shoulder dislocation
E. Torticollis
A

C. Klumpke palsy

Caused by injury to C7-C8 (lower portion of brachial plexus)

44
Q

Which of the following findings BY ITSELF is most suggestive of child abuse?

A. Bruises on the knees, shins, and elbows in different stages of healing
B. Metaphyseal “corner” fracture of the distal left humerus
C. Burns to the right arm that have irregular splashlike configuration
D. Nondisplaced spiral fracture of left tibia
E. Displaced supracondylar fracture of right elbow

A

B. Metaphyseal “corner” fracture of the distal left humerus

45
Q

6 month old pt presents with seizures and CT findings include intracranial calcifications. You diagnose Sturge-Weber syndrome. Which of the following is an expected associated skin finding?

A. Strawberry hemangioma
B. Nevus flammeus
C. Cutis marmorata
D. Acrocyanosis
E. Pustular melanosis
A

B. Nevus flammeus

Aka port-wine stain

46
Q

10 y/o male presents with fever, hypotension, diarrhea, and diffuse erythroderma of skin. Nikolsky sign absent. Lab studies reveal thrombocytopenia and elevated CK. Cultures of blood, urine, and CSF are negative. Most likely causative agent?

A. Group A beta-hemolytic streptococcus
B. Borrelia burgdorferi
C. Staph aureus
D. Kawasaki disease
E. E.coli O157:H7
A

C. Staph aureus

[toxic shock syndrome, MCC is staph aureus]

47
Q

Pt presents with hemarthrosis following minor trauma to knee and you suspect hemophilia A. Which of the following is true of hemophilia A?

A. aPTT is prolonged
B. Males and females equally affected
C. Management includes replacement of factor IX
D. Bleeding time is prolonged
E. The cause is a deficiency of a Vitamin K-dependent clotting factor

A

A. aPTT is prolonged

Hemophilia A = X-linked deficiency in factor VIII. Prolonged aPTT but PT and bleeding time are normal.

48
Q

Etiology of omphalocele?

A. failure of closure of umbilical ring
B. intrauterine mesenteric ischemia
C. involution of the second umbilical vein
D. patent processus vaginalis
E. persistence of the umbilical ring
A

A. failure of closure of umbilical ring

49
Q

Nontender abdominal mass that crosses midline, hypertension, elevated catecholamines

A. Gastric duplication cyst
B. Neuroblastoma
C. Polycystic kidney diseawse
D. Rhabdomyosarcoma
E. Wilm's tumor
A

B. Neuroblastoma

50
Q

7 month old female presents with episodes of tachypnea, failure to thrive, doll-like facial features, thin limbs, hypotonia, and a protruding abdomen. Labs reveal elevated lactate and uric acid. Further evaluation will likely reveal:

A. Elevated creatine kinase
B. Direct hyperbilirubinemia
C. Hyperglycemia
D. Hypertriglyceridemia
E. Hypertrophic cardiomyopathy
A

D. Hypertriglyceridemia

Dx is von Gierke’s disease (glycogen storage disorder type I); autosomal recessive disorder resulting in glucose-6-phosphatase deficiency. Features include hypoglycemia during fasting resulting in lactic acidosis and Kussmaul breathing, and hypertriglyceridemia that may lead to xanthomas and pancreatitis. Tx with frequent carb feedings while avoiding galactose and fructose.

51
Q

Acid maltase deficiency is diagnostic of Pompe disease (GSD II). Which of the following is an associated feature?

A. Elevated creatine kinase
B. Direct hyperbilirubinemia
C. Hyperglycemia
D. Hypertriglyceridemia
E. Hypertrophic cardiomyopathy
A

E. Hypertrophic cardiomyopathy

52
Q

A 5-week-old infant is brought to the pediatrician for failure to thrive with a history of prolonged feeding time and tachypnea. What other features does this infant most likely have?

A. Cyanosis from a right-to-left shunt
B. Early systolic click without a murmur
C. Hyperdynamic precordium with a holosystolic murmur
D. Systolic murmur with a widely split second heart sound

A

C. Hyperdynamic precordium with a holosystolic murmur

A heart murmur from a VSD is typically not appreciated in the immediate newborn period, as the pulmonary vascular resistance is still quite elevated. During this time, since the pulmonary vascular resistance equals the systemic vascular resistance, there is no shunting of blood through the VSD. However, after a few days to weeks after birth, the pulmonary vascular resistance decreases, and the murmur appears, reflecting the shunted flow of blood through the VSD (from left to right). As flow through the VSD increases and becomes audible, the child is at risk for developing symptoms related to excessive pulmonary flow: tachypnea and poor weight gain.

53
Q

A newborn has thrombocytopenia, hepatosplenomegaly, hearing loss, heart murmur, and bilateral cateracts. Congenital infection with which of the following?

A. Toxoplasmosis
B. Rubella
C. Varicella
D. HSV
E. CMV
A

B. Rubella

54
Q

9 month old presents with purulent conjunctival discharge. Tx?

A. Topical tobramycin
B. Topical acyclovir
C. Oral erythromycin
D. Oral acyclovir
E. IV penicillin
A

A. Tobical tobramycin

55
Q

PE reveals non-radiating 1/6 continuous murmur that is best heard inferior to the right clavicle. Louder in upright position and disappears with compression of jugular vein.

A. ASD
B. PDA
C. Peripheral pulmonary stenosis
D. Stills murmur
E. Venous hum
A

E. Venous hum

Second most common innocent murmur, usually heard in children 2-5. Caused by rapid blood flow through internal jugular veins, causing venous walls to vibrate.

56
Q

8 y/o presents with acute onset high fever. 4 days later the fever resolves and you note a red, non-confluent, maculopapular rash on the trunk that begins to spread to face and extremities. Dx?

A. Erlichiosis
B. Measles
C. Erythema infectiosum
D. Roseola infantum
E. Rubella
A

D. Roseola infantum

aka HHV-6

57
Q

Can present either at birth with severe generalized hypotonia and weakness or in adolescence with slowly progressive facial and distal extremity weakness. Characteristic facial features include hollowing of muscles around temples, jaw, and neck, ptosis, facial weakness, and drooping of lower lip.

A. Becker's muscular dystrophy
B. Charcot Marie Tooth
C. Duchenne muscular dystrophy
D. Limb girdle dystrophy
E. Myotonic dystrophy
A

E. Myotonic dystrophy

Autosomal dominant progressive triple repeat expansion in gene coding for myotonin protein kinase

58
Q

Pt presents with immunodeficiency, eczema, thrombocytopenia, and bleeding abnormalities. Which of the following immunologic abnormalities would be expected in this pt?

A. elevated IgA and IgE with reduced levels of IgM
B. IgA deficiency
C. low or absent levels of all immunoglobulins
D. normal immunoglobulins with ineffective neutrophils
E. undetectable IgA levels with elevated IgM and normal IgG

A

A. elevated IgA and IgE with reduced levels of IgM

[this is Wiskott-Aldrich]

59
Q

Pt presents with HTN at a young age. Labs reveal hypokalemia and metabolic alkalosis, as well as decreased levels of serum renin and aldosterone.

A. Bartter syndrome
B. Gitelman syndrome
C. Goodpasture syndrome
D. Gullner syndrome
E. Liddle syndrome
A

E. Liddle syndrome

60
Q

Most common cause of cardiac arrest in a child?

A. Poisoning
B. Cardiac dysrhythmia secondary to heart disease
C. Seizures
D. Hypoxia
E. Trauma
A

D. Hypoxia

61
Q

Asthma symptoms that occur throughout the day and nocturnal awakenings that occur nightly with extreme limitations in activity. Classification?

A. Exercise-induced
B. Intermittent
C. Mild persistent
D. Moderate persistent
E. Severe persistent
A

E. Severe persistent

62
Q

8 y/o presents with erythematous, tender, maculopapular rash that began on the face and has begun to spread to the rest of the body. On exam you note tender posterior auricular lymph nodes that mom notes began prior to the rash. Dx?

A. Erlichiosis
B. Measles
C. Erythema infectiosum
D. Roseola infantum
E. Rubella
A

E. Rubella

63
Q

A 4 y/o male presents with fragile bones, easy bruisability, and blue sclerae. What screening test is most appropriate?

A. Head US
B. Audiology evaluation
C. Ophtho exam
D. Thyroid screen
E. Cervical spine radiographs
A

B. Audiology evaluation

Osteogenesis imperfecta type I pts are at risk for early conductive hearing loss