PEDIA LE6 Flashcards
A 2-year-old boy presents to the ER due to a painless abdominal mass. Physical examination reveals aniridia, cryptorchidism, hypospadias, horseshoe kidney, and hemihypertrophy. Urinalysis shows microscopic hematuria. The most likely diagnosis is:
A. Neuroblastoma
B. Hepatoblastoma
C. Rhabdomyosarcoma
D. Wilms tumor
D. Wilms tumor
Rationale: Wilms tumor is associated with WAGR syndrome (Wilms tumor, Aniridia, Genitourinary anomalies, and mental Retardation), presenting as a painless abdominal mass with hematuria.
A 15-month-old female presents with a mass noted on a chest X-ray in the superior mediastinal region, associated with left-sided miosis and ipsilateral ptosis. The most likely diagnosis is:
A. Wilms tumor
B. Neuroblastoma
C. Acute leukemia
D. Rhabdomyosarcoma
B. Neuroblastoma
Rationale: Neuroblastoma in the superior mediastinum may cause Horner syndrome (ptosis, miosis), originating from neural crest cells.
A 6-year-old boy has been complaining of headaches and difficulty seeing objects on the sides for 4 months. On examination, he is not mentally retarded, his school grades are good, and visual acuity is diminished in both eyes. Visual field testing shows significant defects. A CT scan of the head shows a suprasellar mass with calcification. Which of the following is the most probable diagnosis?
A. Astrocytoma
B. Craniopharyngioma
C. Pituitary adenoma
D. Meningioma
B. Craniopharyngioma
Rationale: Craniopharyngiomas are suprasellar tumors with calcifications, causing bitemporal hemianopia and headaches due to optic chiasm compression.
A 6-year-old boy has been complaining of headaches and difficulty seeing objects on the sides for 4 months. On examination, he is not mentally retarded, his school grades are good, and visual acuity is diminished in both eyes. Visual field testing shows significant defects. A CT scan of the head shows a suprasellar mass with calcification. Which of the following is the most probable diagnosis?
A. Astrocytoma
B. Craniopharyngioma
C. Pituitary adenoma
D. Meningioma
B. Craniopharyngioma
Rationale: Craniopharyngiomas are suprasellar tumors with calcifications, causing bitemporal hemianopia and headaches due to optic chiasm compression.
Which one of the following is the most common cause of congenital hydrocephalus?
A. Craniosynostosis
B. Intrauterine meningitis
C. Aqueductal stenosis
D. Malformation of the great vein of Galen
C. Aqueductal stenosis
Rationale: Congenital hydrocephalus is most commonly caused by aqueductal stenosis, leading to obstructive (non-communicating) hydrocephalus.
A 4-month-old male infant has generalized weakness, hypotonia, areflexia, and tongue fasciculations. The most likely diagnosis is:
A. Infant botulism
B. Dandy-Walker malformation
C. Werdnig-Hoffman disease
D. Juvenile myasthenia gravis
C. Werdnig-Hoffman disease
Rationale: Werdnig-Hoffman disease (spinal muscular atrophy type 1) presents with hypotonia, tongue fasciculations, areflexia, and progressive muscle weakness due to anterior horn cell degeneration.
Which of the following statements about Simple Febrile Seizure is correct?
A. Seizures are focal, lasting for >15 minutes
B. Child has normal neurologic examination findings
C. It is caused by severe metabolic disturbances
D. Seizures recur twice within 24 hours of fever
B. Child has normal neurologic examination findings
Rationale: Simple febrile seizures are generalized, last <15 minutes, do not recur within 24 hours, and are associated with normal neurological examination findings.
A 12-year-old boy develops a severe headache, photophobia, and vomiting. He is febrile, with BP 140/90 mmHg. Cranial nerve and motor examinations are non-focal. He has nuchal rigidity and a positive extensor plantar response. The most appropriate study to obtain at this time is:
A. CSF analysis
B. EEG
C. CT scan
D. Skull X-ray
C. CT scan
Rationale: In a febrile child with signs of increased intracranial pressure (hypertension, headache, vomiting, positive Babinski), a CT scan is needed before lumbar puncture to rule out brain herniation risk.
A 6-year-old boy complains of a 4-day history of low back pain and difficulty walking. On examination, you note weakness in the lower extremities and absent tendon reflexes. Sensory function is intact. MRI of the spine is normal. Which one of the following is the most likely diagnosis?
A. Duchenne muscular dystrophy
B. Guillain-Barré syndrome
C. Myasthenia gravis
D. Acute poliomyelitis
B. Guillain-Barré syndrome
Rationale: Guillain-Barré syndrome presents with progressive lower limb weakness, areflexia, and normal sensory function. MRI is usually normal, distinguishing it from spinal cord pathology.
Patients with tuberculous (TB) meningitis have these characteristic CSF findings:
A. Decreased glucose, increased proteins, decreased lymphocytes
B. Increased glucose, decreased proteins, decreased lymphocytes
C. Increased glucose, increased proteins, increased lymphocytes
D. Decreased glucose, increased proteins, increased lymphocytes
D. Decreased glucose, increased proteins, increased lymphocytes
Rationale: TB meningitis presents with a lymphocytic pleocytosis, elevated protein, and decreased glucose due to Mycobacterium tuberculosis consuming glucose in the CSF.
A 12-week pregnant woman makes an appointment with her OB to discuss spina bifida, as her cousin had an affected infant. Which of the following statements is most likely?
A. The defect invariably leads to severe neurological impairment
B. Neural tube defects cannot affect the brain directly or indirectly
C. Neural tube defects arise due to a failure of the neural tube to close
D. Screening will show a low alpha-fetoprotein (AFP) if she has an affected infant
C. Neural tube defects arise due to a failure of the neural tube to close
Rationale: Neural tube defects (e.g., spina bifida) result from incomplete closure of the neural tube during early embryogenesis, leading to varying degrees of neurological impairment.
One month prior, a 5-year-old boy with known asthma presents with puffy eyelids upon waking in the morning, initially subsiding by midday but persisting throughout the day, along with a tightening waistline. Urinalysis shows 4+ proteinuria, serum albumin 2 g/dL, and elevated serum cholesterol. C3 is normal. What should be your next step?
A. Give furosemide
B. Start prednisone
C. Request ANA titer
D. Perform kidney biopsy
B. Start prednisone
Rationale: Nephrotic syndrome in children (most commonly minimal change disease) is treated with corticosteroids as first-line therapy, given the classic presentation of proteinuria, hypoalbuminemia, and hyperlipidemia.
One of the following statements regarding “breath-holding spells” is NOT true:
A. It is fairly common in the first 2 years of life
B. It does not contribute to an increased risk of seizure disorders
C. Parents are advised to ignore and not reinforce these attacks
D. It must be immediately attended to prevent hypoxia and onset of seizures
D. It must be immediately attended to prevent hypoxia and onset of seizures
Rationale: Breath-holding spells are benign and self-limited; they do not cause long-term hypoxia or increase seizure risk. Parents should be reassured and not reinforce the behavior.
The appropriate approach to a neonate presenting with vaginal bleeding on day 4 of life is:
A. Administration of vitamin K
B. Investigation for a bleeding disorder
C. Administration of 10 mL/kg of fresh frozen plasma over 4 hours
D. No specific therapy
D. No specific therapy
Rationale: Neonatal vaginal bleeding is due to withdrawal of maternal estrogen and is self-limited, requiring no treatment.
A 1-month-old infant was hospitalized for acute gastroenteritis caused by rotavirus. Her diarrhea improved with ORS, but when the mother resumed her usual cow’s milk formula, she had an increased number of watery stools. She appears well-hydrated, and abdominal examination is normal. The most appropriate approach to treat this infant’s diarrhea is:
A. Give her only ORS until the diarrhea resolves
B. Administer IV fluid therapy
C. Change to a lactose-free formula for the next few days
D. Dilute the cow’s milk formula with ORS for the next few days
C. Change to a lactose-free formula for the next few days
Rationale: Post-infectious lactose intolerance is common after rotavirus gastroenteritis due to temporary lactase deficiency; switching to a lactose-free formula resolves symptoms.
A true statement regarding migraine headache in children is:
A. The headache is usually associated with aura
B. Headache is usually squeezing, tight-band-like in quality
C. The headache is usually associated with nausea, vomiting, and photophobia
D. Location of the pain is usually posterior and associated with neck muscle pain
C. The headache is usually associated with nausea, vomiting, and photophobia
Rationale: Pediatric migraine often presents with a pulsating headache, nausea, vomiting, and photophobia, though an aura is not always present.
Which of the following is an autoimmune disorder of the neuromuscular junction caused by autoantibodies binding to postsynaptic acetylcholine receptors?
A. Duchenne muscular dystrophy
B. Spinal muscular atrophy
C. Myasthenia gravis
D. Guillain-Barré syndrome
C. Myasthenia gravis
Rationale: Myasthenia gravis is an autoimmune disease caused by autoantibodies targeting acetylcholine receptors at the neuromuscular junction, leading to muscle weakness and fatigability.
Secondary nephrotic syndrome should be suspected in patients with proteinuria and:
A. Age of 3 years old
B. Normal C3
C. Serum albumin of 1.5 g/dL
D. High blood pressure
D. High blood pressure
Rationale: Secondary nephrotic syndrome should be suspected when hypertension, hematuria, low complement levels, or systemic symptoms are present, distinguishing it from primary nephrotic syndrome.
Characteristic laboratory findings in Henoch-Schönlein purpura (HSP) include all of the following EXCEPT:
A. Increased ESR
B. Mild leukocytosis
C. Thrombocytopenia
D. Increased serum IgA
C. Thrombocytopenia
Rationale: HSP is an IgA-mediated vasculitis with normal or elevated platelet count, increased ESR, mild leukocytosis, and elevated serum IgA.
Characteristic laboratory findings in Henoch-Schönlein purpura (HSP) include all of the following EXCEPT:
A. Increased ESR
B. Mild leukocytosis
C. Thrombocytopenia
D. Increased serum IgA
C. Thrombocytopenia
Rationale: HSP is an IgA-mediated vasculitis with normal or elevated platelet count, increased ESR, mild leukocytosis, and elevated serum IgA.
Acute post-streptococcal glomerulonephritis (APSGN) in childhood:
A. Is most common between 6 months and 3 years of age
B. Heavy proteinuria is a common clinical feature
C. Typically develops 2-3 weeks after streptococcal infection of the skin or throat
D. Usually presents as severe renal failure
C. Typically develops 2-3 weeks after streptococcal infection of the skin or throat
Rationale: APSGN is a post-infectious glomerulonephritis occurring 2-3 weeks after a group A streptococcal infection, presenting with hematuria, edema, and hypertension.
A 15-year-old patient was brought to the pediatrician due to decreased urine output for 1 week. Physical examination reveals stunted growth and pallor. Lab results show Hgb 6 g/dL, BUN 100 mg/dL, and creatinine 20 mg/dL. His anemia is primarily due to:
A. Fluid retention
B. Iron deficiency
C. Probable bleeding
D. Inadequate erythropoietin production
D. Inadequate erythropoietin production
Rationale: Chronic kidney disease (CKD) leads to anemia due to deficient erythropoietin production, resulting in decreased red blood cell synthesis.
An 8-year-old girl presents with a distended abdomen and periorbital swelling. She is otherwise well and has no gastrointestinal symptoms. You suspect nephrotic syndrome. This condition is typically accompanied by:
A. Enlarged kidneys on ultrasound
B. Heavy proteinuria on urinalysis
C. Hypertension
D. Macroscopic hematuria
B. Heavy proteinuria on urinalysis
Rationale: Nephrotic syndrome is characterized by massive proteinuria (>3.5 g/day), hypoalbuminemia, edema, and hyperlipidemia.
The presence of renal parenchymal scarring due to vesicoureteral reflux is best demonstrated by:
A. Renal DMSA scan
B. Renal ultrasonography
C. Voiding cystourethrogram (VCUG)
D. Intravenous pyelography (IVP)
A. Renal DMSA scan
Rationale: A renal DMSA scan is the best test for detecting renal parenchymal scarring due to vesicoureteral reflux.
A 2-year-old boy with chronic renal failure is brought to the ER for evaluation of nausea, fatigue, and muscle weakness. On examination, HR is 140/min, perfusion is decreased, and pulses are feeble. ECG shows peaked T waves, and lab results reveal serum potassium 7.5 mEq/L and glucose 72 mg/dL. The most appropriate initial treatment is administration of:
A. Calcium gluconate 10%
B. Plain NSS 20 cc/kg
C. Verapamil
D. Insulin
A. Calcium gluconate 10%
Rationale: Calcium gluconate stabilizes cardiac membranes in hyperkalemia with ECG changes (peaked T waves), preventing life-threatening arrhythmias.
There is an increased risk for neonatal infection when rupture of membranes exceeds how many hours?
A. 8
B. 12
C. 18
D. 24
C. 18
Rationale: Prolonged rupture of membranes (>18 hours) increases the risk of neonatal sepsis, especially due to Group B Streptococcus.
A 6-year-old boy presents with bright red blood and clots in the urine. There is no history of trauma, dysuria, frequency, urgency, or back pain. On examination, he is afebrile, HR 76, RR 14, BP 110/68. Urinalysis shows 3+ RBCs, pH 6.5, SG 1.025, no casts, and WBC 0-5/hpf. BUN/creatinine ratio is normal. The most appropriate next test is:
A. Renal scan
B. Intravenous pyelogram (IVP)
C. Renal ultrasound
D. Voiding cystourethrogram (VCUG)
C. Renal ultrasound
🔹 Rationale: Gross hematuria with clots and no signs of infection or glomerular disease (no casts) suggests structural causes (e.g., Wilms tumor, stones, trauma). Renal ultrasound is the best initial test to assess kidney structure and rule out masses or stones.
Which of the following conditions primarily affects the anterior horn cells of the spinal cord and has mostly been eradicated due to attenuated/killed virus vaccination?
A. Guillain-Barré syndrome
B. Poliomyelitis
C. Myasthenia gravis
D. Rabies
B. Poliomyelitis
Rationale: Poliomyelitis is caused by poliovirus and primarily affects the anterior horn cells, leading to asymmetric flaccid paralysis. It has been nearly eradicated due to vaccination.
A 10-year-old boy suddenly develops brief, jerky, non-stereotyped movements that are difficult to describe and occur only while awake, affecting his daily activities. His penmanship deteriorated, and occasionally, his pens or utensils would fling out of his grasp. The primary consideration for this previously well boy should be:
A. Motor Tic
B. Sydenham’s chorea
C. Myoclonus
D. Tourette syndrome
B. Sydenham’s chorea
Rationale: Sydenham’s chorea is a major manifestation of acute rheumatic fever, characterized by involuntary, jerky movements, deteriorating handwriting, and emotional instability.
Delayed speech in a 5-year-old child with normal motor and adaptive development is most likely due to:
A. Deafness
B. Kernicterus
C. Cerebral palsy
D. Mental retardation
A. Deafness
Rationale: Isolated delayed speech development in a child with normal motor and adaptive skills is most commonly due to undiagnosed hearing impairment.
A 2-year-old boy with a history of recurrent cervical adenitis and pneumonia presents with a perianal abscess, consistent with a diagnosis of:
A. Wiskott-Aldrich syndrome
B. DiGeorge syndrome
C. Ataxia-telangiectasia
D. Chronic granulomatous disease
D. Chronic granulomatous disease
Rationale: CGD is an immunodeficiency characterized by recurrent bacterial and fungal infections (e.g., pneumonia, abscesses, lymphadenitis) due to defective phagocytic oxidative burst.
At birth, a term newborn infant is noted to have hydrocephalus and intracranial calcifications on CT scan of the head. Eye examination shows bilateral chorioretinitis. He most likely has an intrauterine infection with:
A. Varicella
B. Syphilis
C. Toxoplasmosis
D. Cytomegalovirus
C. Toxoplasmosis
Rationale: Congenital toxoplasmosis presents with the classic triad of hydrocephalus, intracranial calcifications, and chorioretinitis.
- A 3-month-old female presents with a generalized seizure lasting for 25 minutes without regaining consciousness. The seizure is refractory to rectal diazepam and IV lorazepam in the ER. Her temperature is 37.5°C, and there is no sign of trauma. What should be the best intervention at this point?
A. Get glucose level and correct hypoglycemia.
B. Support the airway.
C. Start IV phenobarbital.
D. Start hypertonic saline for suspected hyponatremia.
B. Support the airway.
Rationale:
In status epilepticus, ensuring airway protection is the first priority before administering additional medications.
Seizures lasting >5 minutes can impair airway reflexes, leading to hypoxia and respiratory failure.
Tracheal intubation should be considered if the child becomes obtunded, loses airway reflexes, or has respiratory insufficiency.
Once the airway is secured, additional anticonvulsants (e.g., phenobarbital, fosphenytoin) can be administered to stop the seizure.
- A 5-month-old child was diagnosed with pneumococcal bacterial meningitis. Which of the following statements about pneumococcal meningitis is incorrect?
A. T-lymphocyte defect increases the risk of developing pneumococcal meningitis.
B. The duration of antibiotic therapy is 10-14 days.
C. Streptococcus pneumoniae can alter the microbiome of the nasopharynx by producing hydrogen peroxide against the growth of H. influenzae type B.
D. It is more common among patients with cochlear implants.
A. T-lymphocyte defect increases the risk of developing pneumococcal meningitis
Rationale: Pneumococcal meningitis is primarily associated with B-cell and complement deficiencies, not T-cell defects, which are more relevant to fungal and viral infections.
- An infant presents with delayed gross motor skills, motor stiffness, and spastic paraplegia. Imaging reveals Dandy-Walker malformation. Which of the following statements is NOT CORRECT about Dandy-Walker malformation?
A. Cystic dilation of the fourth ventricle
B. Displacement of the cerebellar tonsils through the foramen magnum
C. Hypoplasia of the cerebellar vermis
D. An enlarged posterior fossa
B. Displacement of the cerebellar tonsils through the foramen magnum
Rationale: Dandy-Walker malformation involves cystic dilation of the fourth ventricle, cerebellar vermis hypoplasia, and an enlarged posterior fossa, but tonsillar herniation is a feature of Chiari malformations, not Dandy-Walker.
- A 12-year-old child falls from a 3-story building, leading to increased intracranial pressure, possibly due to intracranial hemorrhage. Which of the following management steps is the least priority for this patient?
A. Hyperventilation with bag-mask ventilation
B. Normal saline fluid boluses
C. Elevation of the head to at least 30 degrees
D. Start mannitol therapy
D. Start mannitol therapy
- A 7-year-old child with a brain mass presents to the ER with BP 150/100 mmHg, HR 60 bpm, and irregular breathing. You inserted an IV access and started mannitol therapy. Aside from osmotic diuresis, what is another mechanism by which mannitol decreases intracranial pressure?
A. Mannitol increases serum tonicity.
B. Mannitol may cause cerebrovasodilation.
C. Mannitol decreases blood viscosity.
D. Mannitol increases serum sodium level, which is oftentimes low.
C. Mannitol decreases blood viscosity
Rationale: Mannitol decreases intracranial pressure not only via osmotic diuresis but also by reducing blood viscosity, leading to improved cerebral perfusion and oxygenation.
- EEG shows high-voltage, slow, chaotic background with multifocal spikes. The most likely diagnosis is:
A. West syndrome
B. Dravet syndrome
C. Ohtahara syndrome
D. Doose syndrome
A. West syndrome
Rationale: West syndrome (infantile spasms) presents with hypsarrhythmia on EEG, characterized by high-voltage, chaotic slow waves with multifocal spikes.
- A 10-year-old appears well and has seizures under control. On physical examination, he has gingival hyperplasia. Which anticonvulsant most likely caused this side effect?
A. Valproic acid
B. Carbamazepine
C. Phenytoin
D. Levetiracetam
C. Phenytoin
Rationale: Phenytoin is commonly associated with gingival hyperplasia, a side effect resulting from fibroblast stimulation and increased extracellular matrix production.
- Electrolyte imbalances are common in patients with symmetrical and non-structural causes of coma among children. Aside from hyponatremia and hypernatremia, which of the following can also cause decreased sensorium?
A. Hypomagnesemia
B. Hypermagnesemia
C. Hypocalcemia
D. Hyperphosphatemia
B. Hypermagnesemia ✅
Causes depressed sensorium, muscle weakness, respiratory depression, and cardiac conduction abnormalities.
High levels inhibit neuromuscular transmission, leading to hyporeflexia, lethargy, and coma.
- In patients with Guillain-Barré syndrome (GBS), which of the following should be monitored to test respiratory function?
A. Pleocytosis (20–300 cells) with CNS involvement
B. Predominantly PMNs
C. Near-normal CSF cell values
D. Serial spirometry to assess vital capacity
D. Serial spirometry to assess vital capacity
- Myasthenia gravis (MG) should be differentiated from other causes of neuromuscular blockade, such as organophosphate poisoning, botulism, and tick paralysis. The most specific test for MG is:
A. Muscle biopsy
B. Electromyography
C. Serum creatine kinase
D. Acetylcholine receptor antibody test
D. Acetylcholine receptor antibody test
Rationale: The most specific test for myasthenia gravis is the detection of anti-acetylcholine receptor antibodies, confirming the autoimmune pathology.
B. Electromyography (EMG) ✅ (Partially Correct)
EMG, especially single-fiber electromyography (SFEMG), is highly sensitive for MG.
However, it is not the most specific test.
- An adolescent was brought to the ER due to unsteady gait. Which of the following medications is least likely to cause ataxia?
A. Imipramine
B. Alcohol
C. Benzodiazepines
D. Phenytoin
A. Imipramine
Rationale: While imipramine can cause dizziness and sedation, it is not commonly associated with ataxia, unlike alcohol, benzodiazepines, and phenytoin, which affect cerebellar function.
- An 18-year-old boy has a severe headache (10/10 pain scale) and is brought to the ER unconscious with BP 160/90 mmHg, HR 70 bpm, and unilateral pupillary dilatation. Cranial CT scan shows subarachnoid hemorrhage with cerebral edema. Which of the following mechanisms is expected as a result of vasogenic cerebral edema?
A. Increased permeability of capillaries
B. Intracellular accumulation of excess fluid
C. Greater swelling in gray matter than in white matter
D. Increased permeability of neuronal membranes
A. Increased permeability of capillaries
Rationale: Vasogenic cerebral edema occurs due to blood-brain barrier disruption, leading to increased capillary permeability and extracellular fluid accumulation, particularly in white matter.
- A 12-year-old boy is diagnosed with Guillain-Barré syndrome. You admit him to the ward and monitor for disease progression. Which of the following should be monitored to assess respiratory function?
A. Chest radiograph
B. Peak expiratory flow rate
C. Arterial blood gas
D. Vital capacity
D. Vital capacity
Rationale: Vital capacity monitoring via serial spirometry is essential in Guillain-Barré syndrome to detect impending respiratory failure.
- Myasthenia gravis should be differentiated from other causes of neuromuscular blockade, such as organophosphate poisoning, botulism, and tick paralysis. The most specific test for MG is:
A. Muscle biopsy
B. Electromyography
C. Anti-AChR antibodies
D. Serum creatine kinase
C. Anti-AChR antibodies
Rationale: The most specific test for myasthenia gravis is detecting anti-acetylcholine receptor (AChR) antibodies, which confirm the autoimmune etiology.
- An infant presents with delayed gross motor skills, motor stiffness, and spastic paraplegia. Imaging reveals Dandy-Walker malformation. Which of the following statements is NOT CORRECT about Dandy-Walker malformation?
A. Hypoplasia of the cerebellar vermis
B. An enlarged posterior fossa
C. Cystic dilatation of the fourth ventricle
D. Displacement of the cerebellar tonsils through the foramen magnum
D. Displacement of the cerebellar tonsils through the foramen magnum
Rationale: This feature is characteristic of Chiari malformation, not Dandy-Walker malformation, which involves hypoplasia of the cerebellar vermis, an enlarged posterior fossa, and a cystic fourth ventricle.
- A child with spastic diplegia cerebral palsy is seen for the first time in your clinic. As part of the workup, you request a cranial MRI. Which of the following findings is commonly seen in these patients?
A. Periventricular leukomalacia
B. White matter injury
C. Multicystic cortical encephalomalacia
D. Enlarged thalamocortical pathways
A. Periventricular leukomalacia
Rationale: Periventricular leukomalacia (PVL) is the most common MRI finding in spastic diplegia, resulting from hypoxic-ischemic injury to the periventricular white matter.
- A 3-year-old boy presents with fever, anorexia, and headache. Examination reveals positive meningeal irritation. CSF analysis shows increased lymphocytes and decreased glucose. He has a history of prolonged steroid therapy for nephrotic syndrome. The most likely diagnosis is:
A. Fungal meningitis
B. Viral meningitis
C. Parasitic meningitis
D. Partially treated bacterial meningitis
A. Fungal meningitis
Rationale: A child on prolonged steroid therapy is immunocompromised, making fungal meningitis (e.g., Cryptococcus or Histoplasma) a likely cause, presenting with lymphocytic pleocytosis and low glucose in CSF.
- The electroencephalography (EEG) of an 8-year-old girl reveals generalized 3-Hz spike-and-wave activity. Which of the following is the most likely diagnosis?
A. Benign childhood epilepsy
B. Generalized absence seizure
C. West syndrome
D. Simple partial seizure
B. Generalized absence seizure
Rationale: Absence seizures are characterized by brief staring episodes and a classic 3-Hz spike-and-wave pattern on EEG.
- The duration of anticonvulsant treatment varies according to seizure type and epilepsy syndrome. For most children, anticonvulsant medications can be weaned off after 2 years without seizures. Of the following, which condition usually requires lifelong treatment?
A. Simple partial seizure
B. Complex partial seizure
C. Juvenile myoclonic epilepsy
D. Infantile spasm
C. Juvenile myoclonic epilepsy
Rationale: Juvenile myoclonic epilepsy (JME) requires lifelong treatment, as seizures tend to recur even after long seizure-free periods.
- A 6-month-old infant presents with severe hypotonia and generalized weakness. His creatine phosphokinase (CK) is mildly elevated, EMG shows fasciculations and fibrillations, and muscle biopsy shows grouped atrophy. The most likely finding in this infant is:
A. Absent deep tendon reflexes
B. Absent sensation
C. Bilateral ptosis
D. Persistent Moro reflex
A. Absent deep tendon reflexes
Rationale: Severe hypotonia, muscle atrophy, and fasciculations in an infant suggest spinal muscular atrophy (SMA), which presents with absent deep tendon reflexes due to anterior horn cell degeneration.
- Which complication may be detrimental to the patient if there is hyperkalemia?
A. Hypotension
B. Arrhythmia
C. Hematuria
D. Renal failure
B. Arrhythmia
Rationale:
Hyperkalemia can cause life-threatening cardiac arrhythmias (e.g., ventricular fibrillation, asystole).
ECG findings: Peaked T waves, widened QRS, prolonged PR interval.
- Arthritis, abdominal pain, and a petechial eruption on the buttocks are consistent with a diagnosis of:
A. Henoch-Schönlein purpura
B. Systemic lupus erythematosus (SLE)
C. Kawasaki disease
D. Dermatomyositis
A. Henoch-Schönlein purpura (HSP)
Rationale:
HSP is an IgA-mediated vasculitis that presents with:
Palpable purpura (buttocks, legs)
Arthritis/arthralgia
Abdominal pain (due to GI vasculitis)
Renal involvement (IgA nephropathy)
- You suspect a brain tumor in a 2-year-old girl with a recent history of ataxia, slurred speech, and early morning vomiting. Which statement about childhood brain tumors is true?
A. Most common location is infratentorial
B. Brain tumors are a rare type of cancer in childhood
C. Signs of increased intracranial pressure are rare on presentation
D. Seizures are the presenting complaint in most cases
A. Most common location is infratentorial
Rationale:
Most common pediatric brain tumors are infratentorial (posterior fossa, cerebellum).
Common tumors: Medulloblastoma, pilocytic astrocytoma.
Signs of increased ICP are common (early morning vomiting, ataxia).
- Which of the following movement disorders describes slow, writhing movements?
A. Tremor
B. Chorea
C. Athetosis
D. Dystonia
C. Athetosis
Rationale:
Athetosis: Slow, writhing movements, commonly seen in cerebral palsy (dyskinetic type).
Other choices:
A. Tremor → Rhythmic oscillations.
B. Chorea → Rapid, jerky movements.
D. Dystonia → Sustained, twisting muscle contractions.
- An 8-year-old boy is referred for new-onset seizures. Which of the following most strongly supports a diagnosis of complex partial seizures?
A. Normal mental state, consciousness, and responsiveness during seizure
B. A brief tonic-clonic seizure
C. Automatisms
D. 3/second spike-and-wave pattern on EEG
C. Automatisms
Rationale:
Complex partial seizures involve loss of consciousness and automatisms (lip-smacking, hand movements).
Other choices:
A. Normal mental state → Incorrect, complex partial seizures cause altered consciousness.
B. Brief tonic-clonic seizure → Suggests generalized seizure, not complex partial.
D. 3-Hz spike-and-wave EEG → Seen in absence seizures.
- A 3-month-old infant is brought to the hospital due to altered mental status. Examination shows a sleepy baby who is difficult to arouse. Fundoscopic exam reveals retinal hemorrhages. The best next diagnostic test is:
A. Lumbar puncture
B. CT scan of the brain
C. Skull X-ray
D. EEG
B. CT scan of the brain
Rationale:
Retinal hemorrhages in an infant suggest abusive head trauma (shaken baby syndrome).
CT scan is the best initial test to check for subdural hematoma.
Other choices:
A. Lumbar puncture → Not first-line unless meningitis is suspected.
C. Skull X-ray → Only detects fractures, not intracranial bleeding.
D. EEG → Not helpful in diagnosing trauma.
- A 9-year-old girl presents with severe, intermittent headache. She is febrile, and physical and neurological exams are normal. Which of the following characteristics best supports the diagnosis of childhood migraine?
A. Strong family history of migraine
B. Frequently isolated to the occipital region
C. Duration of headache more than 24 hours
D. Persistence of headache after sleep
A. Strong family history of migraine
Rationale:
Childhood migraines often have a strong genetic component.
Other choices:
B. Occipital region → Rare in children.
C. Duration >24 hours → Migraines typically last <24 hours.
D. Persistence after sleep → Migraines often improve with sleep.
- An 18-month-old child is brought to the ER due to a seizure. Which of the following supports febrile seizures?
A. Focal seizure is typical
B. Such seizures usually occur in association with infections of the CNS
C. Such seizures often last more than 15 minutes
D. Affected children usually are between 5 months and 5 years of age
D. Affected children are between 5 months and 5 years of age
Rationale:
Simple febrile seizures occur in children 6 months – 5 years, are generalized, and last <15 min.
Other choices:
A. Focal seizures are typical → Incorrect, simple febrile seizures are generalized.
B. Associated with CNS infections → Incorrect, no CNS infection is present.
C. Often last >15 min → Incorrect, complex febrile seizures last >15 min.
- The CSF examination of an 8-year-old child with mild fever, nuchal rigidity, and intermittent stupor shows: WBC 200 (all lymphocytes), negative Gram stain, protein 150 mg/dL, glucose 15 mg/dL. CT scan shows enhancement of the basal cisterns. The most likely diagnosis is:
A. Aseptic meningitis
B. Tuberculous meningitis
C. Pseudotumor cerebri
D. Acute bacterial meningitis
B. Tuberculous meningitis
Rationale:
TB meningitis findings:
Lymphocytic pleocytosis
Very low glucose (<40 mg/dL)
High protein (>100 mg/dL)
Basal meningeal enhancement on CT
Other choices:
A. Aseptic meningitis → Normal glucose.
C. Pseudotumor cerebri → Normal CSF, increased ICP.
D. Acute bacterial meningitis → Would have predominantly PMNs, not lymphocytes.
- A 1-year-old male is brought to the ER due to focal tonic-clonic seizures refractory to anticonvulsant treatment. Skull X-ray shows intracranial calcifications with a “railroad track” appearance. The most likely diagnosis is:
A. Tuberous sclerosis
B. Sturge-Weber syndrome
C. Multiple sclerosis
D. Neurofibromatosis
B. Sturge-Weber syndrome
Rationale:
Sturge-Weber syndrome findings:
Port-wine stain
Seizures (focal, refractory)
Leptomeningeal angiomas → Cause intracranial calcifications (“railroad track” pattern).
Glaucoma.
Other choices:
A. Tuberous sclerosis → Would have multiple cortical tubers, renal angiomyolipomas.
C. Multiple sclerosis → Rare in infants, no calcifications.
D. Neurofibromatosis → Café-au-lait spots, optic gliomas, not intracranial calcifications.
- About 12 days after a mild upper respiratory infection, a 12-year-old boy complains of progressive weakness in his lower extremities. Over several days, it involves his trunk. On PE, he has lower extremity muscle weakness and absent deep tendon reflexes, but no muscle atrophy or pain. CSF analysis shows elevated protein only. The most likely diagnosis is:
A. Duchenne muscular dystrophy
B. Guillain-Barré syndrome
C. Charcot-Marie-Tooth disease
D. Werdnig-Hoffmann disease
B. Guillain-Barré syndrome
Rationale:
GBS presents with progressive ascending weakness following a viral illness.
Key findings:
Lower limb weakness → spreads upward
Absent deep tendon reflexes
No sensory loss or muscle atrophy
CSF: Elevated protein, normal WBC (albuminocytologic dissociation)
Other choices:
A. Duchenne muscular dystrophy → No acute onset or preceding infection, progressive proximal muscle weakness.
C. Charcot-Marie-Tooth disease → Chronic, not post-viral, associated with pes cavus, foot drop.
D. Werdnig-Hoffmann disease → Presents at birth or infancy, not post-viral.
- A newborn infant has respiratory distress and trouble feeding in the nursery. Pregnancy was complicated by decreased fetal movement. On PE, the infant has shallow respirations, twitching of the fingers and toes, and is very hypotonic. There is pooled saliva in the mouth, tongue fasciculations, and absent deep tendon reflexes. CSF examination is normal. Which of the following statements is true?
A. The condition is caused by the absence of the muscle cytoskeletal protein dystrophin
B. The condition is caused by the degeneration of anterior horn cells in the spinal cord
C. The condition is caused by antibodies that bind the acetylcholine receptor at the postsynaptic muscle membrane
D. The condition is caused by progressive axonal neuropathy
B. The condition is caused by the degeneration of anterior horn cells in the spinal cord
Rationale:
Werdnig-Hoffmann disease (Spinal Muscular Atrophy Type 1):
Severe hypotonia, tongue fasciculations, absent reflexes, respiratory failure.
Caused by degeneration of anterior horn cells (SMN1 gene mutation).
Other choices:
A. Dystrophin absence → Duchenne MD, presents later.
C. Acetylcholine receptor antibodies → Myasthenia gravis, affects older children.
D. Progressive axonal neuropathy → Seen in metabolic diseases (e.g., adrenoleukodystrophy).
- Unsteadiness in sitting, walking, or gait, along with clumsiness of the arms and legs, is most commonly seen in:
A. Cerebral dysfunction
B. Spinal cord disease
C. Cerebellar dysfunction
D. Peripheral nerve disease
C. Cerebellar dysfunction
Rationale:
Cerebellar dysfunction causes ataxia, poor coordination, intention tremors.
Other choices:
A. Cerebral dysfunction → Affects cognition.
B. Spinal cord disease → Causes weakness, sensory loss.
D. Peripheral nerve disease → Causes hyporeflexia, distal weakness.
- All of the following statements about hydrocephalus are true, EXCEPT:
A. Most children with hydrocephalus go on to have IQ consistent with mental retardation
B. Staphylococcus epidermidis is the most likely etiologic agent after VP shunt insertion
C. Ultrasound is a useful imaging study to detect hydrocephalus in the fetal and neonatal periods
D. Some early signs are delay in walking, waddling gait, seizures, and hypertonia
A. Most children with hydrocephalus go on to have IQ consistent with mental retardation
Rationale:
Many hydrocephalus patients have normal intelligence if treated early with VP shunting.
Other choices (True statements):
B. Staph epidermidis → Most common VP shunt infection.
C. Ultrasound → Used for fetal & neonatal hydrocephalus detection.
D. Early signs → Gait disturbances, seizures, hypertonia.
- An infant was delivered with a myelomeningocele on the thoracolumbar area. The mother asks about possible complications before surgical repair. Which of the following is least likely to happen prior to surgery?
A. Sudden infant death
B. Paralysis
C. Hip dysplasia
D. Bladder and bowel dysfunction
A. Sudden infant death
Rationale:
Myelomeningocele causes:
Paralysis (spinal cord defect).
Bladder & bowel dysfunction (neurogenic).
Hip dysplasia (poor limb development).
SIDS is NOT a typical complication before surgery.
- A 10-year-old boy has gingival hyperplasia. Which anticonvulsant most likely caused this side effect?
A. Valproic acid
B. Carbamazepine
C. Phenytoin
D. Levetiracetam
C. Phenytoin
Rationale:
Phenytoin causes gingival hyperplasia due to fibroblast stimulation.
Other choices:
A. Valproic acid → Hepatotoxicity, neural tube defects.
B. Carbamazepine → Aplastic anemia, SIADH.
D. Levetiracetam → Behavioral issues, not gingival hyperplasia.
- Which of the following autosomal recessive lysosomal storage diseases results in demyelination, progressive dementia, and ataxia, caused by an accumulation of sulfatide due to deficient Galactosyl-3-sulfate ceramide sulfatase in the brain, kidneys, liver, and peripheral nerves?
A. Huntington disease
B. Parkinson disease
C. Krabbe disease
D. Metachromatic leukodystrophy
D. Metachromatic leukodystrophy
Rationale:
Deficiency: Arylsulfatase A → Sulfatide accumulation → demyelination.
Key signs: Progressive ataxia, dementia, peripheral neuropathy.
Other choices:
A. Huntington disease → AD, no demyelination.
B. Parkinson disease → Dopamine-related, no leukodystrophy.
C. Krabbe disease → Similar but involves globoid cells, not sulfatide storage.
- Which of the following is the most common movement disorder described as a rhythmic, involuntary, oscillatory movement?
A. Tremor
B. Chorea
C. Athetosis
D. Dystonia
A. Tremor
Rationale:
Tremors = rhythmic, oscillatory movements.
Other choices:
B. Chorea → Jerky, irregular.
C. Athetosis → Slow, writhing.
D. Dystonia → Sustained, twisting.
- A mother is concerned about her 2-year-old child, who is nonverbal. The child sat alone at 7 months, walked at 13 months, but now has a wide-based stance, no longer walks, and does not manipulate toys. Exam shows height and weight at the 50th percentile, but head circumference below the 5th percentile, with no increase over the past 8 months. Fundi are normal, and there is no hepatosplenomegaly. The most likely diagnosis is:
A. Adrenoleukodystrophy
B. Tay-Sachs disease
C. Cerebral palsy
D. Rett syndrome
D. Rett syndrome
Rationale:
Key features:
Normal development → loss of motor & verbal skills.
Microcephaly, hand-wringing movements.
X-linked dominant, MECP2 mutation (girls only).
Other choices:
A. Adrenoleukodystrophy → Later onset (school-age).
B. Tay-Sachs → Macrocephaly, hyperacusis.
C. Cerebral palsy → Non-progressive.
- A 1-day-old newborn was born at term, weighing 3.2 kg. Head circumference is 40 cm. A cystic mass protrudes from the thoracolumbar spine. There is flaccid paralysis of both lower extremities, and the hips are dislocated. The most likely diagnosis is:
A. Encephalocele
B. Meningocele
C. Meningomyelocele
D. Spina bifida occulta
C. Meningomyelocele
Rationale:
Meningomyelocele (most severe form of spina bifida) → Neural tube defect with spinal cord involvement.
Other choices:
A. Encephalocele → Occipital skull defect, not spinal.
B. Meningocele → Only meninges herniate, no paralysis.
D. Spina bifida occulta → No visible cystic mass, normal neuro exam.
- In the treatment of urinary tract infection (UTI), the main goal is:
A. Prevent emergence of resistant strains
B. Prevent gram-negative sepsis
C. Prevent renal scarring
D. Preserve bladder function
C. Prevent renal scarring
Rationale:
Renal scarring is the most serious long-term complication of UTI, leading to hypertension and chronic kidney disease.
Other choices:
A. Prevent emergence of resistant strains → Important but secondary to preventing kidney damage.
B. Prevent gram-negative sepsis → UTI can cause sepsis, but renal scarring is the primary long-term concern.
D. Preserve bladder function → UTI typically does not cause permanent bladder dysfunction.
- Wilms’ Tumor is the most frequent malignant tumor of the genitourinary tract. The most common presenting sign is:
A. Hematuria
B. Abdominal pain
C. Loss of weight
D. Abdominal mass
D. Abdominal mass
Rationale:
Painless abdominal mass is the most common presentation of Wilms’ tumor (nephroblastoma).
Other choices:
A. Hematuria → Occurs in some cases but is not the most common sign.
B. Abdominal pain → Less common and usually occurs later.
C. Weight loss → Rare, occurs in advanced disease.
- A patient presents with dark urine and healing impetigo, along with periorbital edema. The most likely diagnosis is:
A. IgA nephropathy
B. Post-streptococcal glomerulonephritis (PSGN)
C. Henoch-Schönlein purpura
D. Minimal change disease
B. Post-streptococcal glomerulonephritis (PSGN)
Rationale:
PSGN follows streptococcal infections (impetigo, pharyngitis) and presents with:
Hematuria (tea-colored urine)
Edema (periorbital, generalized)
Hypertension
Low C3 levels
Other choices:
A. IgA nephropathy → Occurs after an upper respiratory infection, NOT impetigo.
C. Henoch-Schönlein purpura → Has a characteristic palpable purpura.
D. Minimal change disease → Causes nephrotic syndrome, NOT hematuria.
- The most common cause of gross hematuria in pediatrics is:
A. IgA nephropathy
B. Alport syndrome
C. Polycystic kidney disease
D. Goodpasture syndrome
A. IgA nephropathy
Rationale:
IgA nephropathy is the most common cause of recurrent gross hematuria in children.
Other choices:
B. Alport syndrome → Causes hematuria + hearing loss + ocular issues.
C. Polycystic kidney disease → Hematuria is possible but less common.
D. Goodpasture syndrome → Rare in children, presents with lung hemorrhage + kidney disease.
- Which of the following is the least commonly observed clinical feature in post-streptococcal glomerulonephritis (PSGN)?
A. Hematuria
B. Edema
C. Renal failure
D. Hypertension
C. Renal failure
Rationale:
PSGN is usually self-limiting, and renal failure is rare unless severe.
Other choices (common findings in PSGN):
A. Hematuria → Tea-colored urine is a hallmark.
B. Edema → Common due to fluid retention.
D. Hypertension → Due to sodium and water retention.
- A 1-year-old child presents with recurrent UTI and enlarged kidneys on ultrasound. The best diagnostic test for evaluation is:
A. Renal tomography
B. Plain film KUB
C. Voiding cystourethrogram (VCUG)
D. MRI
C. Voiding cystourethrogram (VCUG)
Rationale:
VCUG is the gold standard for diagnosing vesicoureteral reflux (VUR), which can cause recurrent UTI and kidney enlargement.
Other choices:
A. Renal tomography (CT) → Not first-line.
B. Plain film KUB → Detects kidney stones, not VUR.
D. MRI → Not necessary for UTI evaluation.
- Children with chronic renal failure may present with:
A. Mental retardation
B. Growth retardation
C. Hypotension
D. Hypokalemia
B. Growth retardation
Rationale:
Chronic kidney disease (CKD) leads to growth failure due to:
Metabolic acidosis
Poor nutrition
Endocrine dysfunction (low growth hormone sensitivity)
Other choices:
A. Mental retardation → Uncommon in CKD.
C. Hypotension → CKD often causes hypertension, not hypotension.
D. Hypokalemia → CKD usually causes hyperkalemia.
- Which of the following statements about the management of nephrotic syndrome is correct?
A. Renal biopsy prior to giving immunosuppressive drugs
B. Steroid-responsive in minimal change lesions
C. Steroid-resistant if failure to respond despite 1 week of prednisone
D. Complete remission is possible if urinary protein is >40 mg/m²/hr
B. Steroid-responsive in minimal change lesions
Rationale:
Minimal change disease (MCD) is the most common cause of nephrotic syndrome in children and is highly steroid-responsive.
Other choices:
A. Renal biopsy before immunosuppressants → Not necessary unless steroid-resistant.
C. Steroid-resistant if no response in 1 week → Steroid resistance is defined after 4 weeks of prednisone, not 1 week.
D. Complete remission if urinary protein >40 mg/m²/hr → Incorrect, remission is defined as proteinuria resolution.
- A sexually active 16-year-old boy presents with arthritis, proximal muscle weakness, erythematous hypertrophic papules over the knuckles, and a violaceous heliotrope rash around the eyes. The most likely diagnosis is:
A. Henoch-Schönlein purpura
B. Systemic lupus erythematosus (SLE)
C. Kawasaki disease
D. Dermatomyositis
D. Dermatomyositis
Rationale:
Dermatomyositis presents with:
Proximal muscle weakness
Heliotrope rash (violet discoloration around the eyes)
Gottron papules (erythematous plaques on knuckles)
Other choices:
A. Henoch-Schönlein purpura → Palpable purpura, abdominal pain, arthritis.
B. SLE → Would include malar rash, nephritis, systemic symptoms.
C. Kawasaki disease → Coronary artery involvement, conjunctivitis, fever.
- A 5-year-old girl presents with arthritis, abdominal pain, and a petechial eruption on her buttocks. The most likely diagnosis is:
A. Henoch-Schönlein purpura
B. Systemic lupus erythematosus (SLE)
C. Kawasaki disease
D. Dermatomyositis
A. Henoch-Schönlein purpura (HSP)
Rationale:
HSP is an IgA-mediated vasculitis characterized by:
Palpable purpura on buttocks/legs
Arthritis/arthralgia
Abdominal pain (intussusception risk)
IgA nephropathy (hematuria, proteinuria)
Other choices:
B. SLE → Would have systemic signs, malar rash, nephritis.
C. Kawasaki disease → Conjunctivitis, fever, mucositis.
D. Dermatomyositis → Proximal muscle weakness, heliotrope rash.
- The most sensitive imaging test for osteomyelitis is:
A. X-ray
B. CT scan
C. MRI
D. Bone scan
C. MRI
Rationale:
MRI is the most sensitive test for early osteomyelitis because it detects bone marrow edema and soft tissue involvement before changes appear on X-ray.
Other choices:
A. X-ray → Often normal in early stages; bone destruction appears 7-10 days later.
B. CT scan → Good for bone detail but not as sensitive as MRI for marrow infection.
D. Bone scan → Useful if MRI is unavailable, but less specific due to uptake in other inflammatory conditions.
- WAGR syndrome includes the following features:
A. Wilms tumor, aniridia, genitourinary malformation, and mental retardation
B. Wilms tumor, aniridia, glaucoma, and retinoblastoma
C. Wilms tumor, ambiguous genitalia, gonadal dysgenesis, and renal failure
D. Wilms tumor, aniridia, growth retardation, and renal cysts
A. Wilms tumor, aniridia, genitourinary malformation, and mental retardation
Rationale:
WAGR syndrome is caused by a deletion on chromosome 11p13 (WT1 gene mutation) and includes:
Wilms tumor (nephroblastoma)
Aniridia (absence of the iris)
Genitourinary anomalies (e.g., cryptorchidism, ambiguous genitalia)
Retardation (mental and developmental delay)
Other choices:
B. Glaucoma, retinoblastoma → Not part of WAGR.
C. Gonadal dysgenesis, renal failure → More consistent with Denys-Drash syndrome.
D. Growth retardation, renal cysts → Incorrect features for WAGR.
- Eruption due to scabies is characterized by papules involving many sites of the body, with sparing of the face and scalp. In which of the following age groups are these sites usually involved?
A. Neonates
B. Infants
C. Adolescents
D. Adults
B. Infants
Rationale:
Scabies typically spares the face and scalp in older children and adults but can involve the head, neck, palms, and soles in infants.
Other choices:
A. Neonates → Less commonly affected than infants.
C. Adolescents → Usually affects interdigital spaces, wrists, axillae, and waist, sparing the face and scalp.
D. Adults → Similar to adolescents, sparing the head.
- Pediculosis may be transmitted by direct contact or indirect spread through contact with personal items such as utensils and clothes. Which of the following manifestations indicates active infestation?
A. White nits
B. Brown nits
C. Excoriation marks
D. Scalp redness
B. Brown nits
Rationale:
Brown nits contain viable eggs and indicate active infestation.
Other choices:
A. White nits → Empty eggshells, indicating past infestation.
C. Excoriation marks → Suggest itching but not necessarily active infestation.
D. Scalp redness → Can be from scratching but not specific for active lice.
