10.4 Pediatric Neurology, Nephrology & Musculoskeletal Disorders
Key Takeaways
Post-streptococcal glomerulonephritis shows hematuria, edema, hypertension, and low C3 1–2 weeks after strep pharyngitis; minimal change disease is nephrotic with normal complement and responds to steroids.
Kawasaki disease (fever ≥5 days plus ≥4 of 5 criteria) is treated with IVIG and aspirin to prevent coronary artery aneurysms.
Guillain-Barré syndrome (ascending flaccid paralysis with areflexia) is treated with IVIG; monitor for respiratory failure via serial forced vital capacity.
A septic arthritis of the pediatric hip is an emergency — apply the Kocher criteria and obtain urgent ultrasound plus surgical washout.
Pediatric Neurology, Nephrology & Musculoskeletal Disorders
The SMLE Pediatrics domain includes dedicated subsections on the central nervous system, nephrology, and the musculoskeletal system. High-yield content covers seizure disorders, neuromuscular weakness, glomerular and nephrotic disease, and inflammatory joint and bone conditions. Saudi-relevant considerations (consanguinity and autosomal recessive neurometabolic disease, post-streptococcal glomerulonephritis, and Kawasaki disease) appear frequently.
Pediatric Neurology
Seizure Disorders
- Febrile seizures occur in children 6 months–5 years with fever and no CNS infection. Simple febrile seizures are generalized, last <15 minutes, and do not recur within 24 hours; management is supportive with antipyretics — antiepileptics are not routinely indicated. Complex febrile seizures (>15 min, focal, or recurrent within 24 h) carry a higher risk of later epilepsy.
- Epilepsy is recurrent unprovoked seizures. First-line for focal seizures is carbamazepine or levetiracetam; for absence seizures, ethosuximide or valproate.
- Status epilepticus: a seizure lasting ≥5 minutes or recurrent seizures without recovery between them. First-line is IV lorazepam (or rectal diazepam), repeated once if needed, followed by a loading dose of fosphenytoin; simultaneously identify and treat the precipitant (fever, non-adherence, metabolic derangement, CNS infection).
Cerebral Palsy
- A non-progressive motor disorder from a static brain lesion acquired before age 2; spastic diplegia is associated with premature birth (periventricular leukomalacia). The SMLE trap: a child with early hypotonia that later progresses to hypertonia/spasticity — distinguish this static picture from a progressive neurodegenerative disorder.
Guillain-Barré Syndrome (GBS)
- Acute ascending symmetric flaccid paralysis with areflexia, often following a Campylobacter jejuni diarrheal illness. Diagnosis is clinical, supported by albuminocytologic dissociation on CSF (elevated protein, normal cell count). Treatment is IV immunoglobulin (IVIG) or plasmapheresis; monitor respiratory function with serial forced vital capacity.
Myasthenia Gravis
- Fatigable weakness with ocular onset (ptosis, diplopia); acetylcholine-receptor antibodies. The ice pack test is a bedside aid. Treat with pyridostigmine ± thymectomy.
Pediatric Nephrology
Nephrotic vs Nephritic Syndromes
| Feature | Nephrotic | Nephritic |
|---|---|---|
| Proteinuria | Massive (>40 mg/m²/hr; serum albumin <25 g/L) | Moderate (non-nephrotic) |
| Hematuria | Minimal | Prominent (dysmorphic RBCs, casts) |
| Edema | Heavy (periorbital, ascites) | Mild–moderate |
| Complement | Normal (minimal change disease) | Low (PSGN, MPGN) |
| Blood pressure | Normal | Elevated |
- Minimal change disease: the most common nephrotic syndrome in children (peak 2–6 years); normal light microscopy with foot-process effacement on electron microscopy; responds to corticosteroids.
- Post-streptococcal glomerulonephritis (PSGN): 1–2 weeks after group A strep pharyngitis (or 3–6 weeks after strep pyoderma). Presents with hematuria (cola-colored urine), edema, hypertension, and low C3. Management is supportive; confirm with elevated antistreptolysin O (ASO) and anti-DNase B. Immunocompromise is not usually required.
Enuresis & Vesicoureteral Reflux
- Enuresis: repeated involuntary voiding in a child ≥5 years. For monosymptomatic nocturnal enuresis, first-line is behavioral — the bedwetting alarm; desmopressin is the pharmacologic option for short-term use (e.g., sleepovers), with attention to fluid restriction to avoid hyponatremia.
- Vesicoureteral reflux (VUR): the most common cause of recurrent childhood UTI and a recognized risk for renal scarring and hypertension. Low-grade reflux is managed with continuous prophylactic antibiotics and surveillance ultrasound; high-grade reflux or recurrent pyelonephritis warrants surgical reimplantation.
Pediatric Musculoskeletal
Kawasaki Disease
- A medium-vessel vasculitis in children <5 years. Diagnostic criteria: fever ≥5 days plus ≥4 of 5: bilateral non-exudative conjunctivitis, polymorphous rash, strawberry tongue/cracked lips, erythema and swelling of extremities (later desquamation), and cervical lymphadenopathy ≥1.5 cm.
- Coronary artery aneurysms are the major complication; echocardiography is mandatory.
- Treatment: IVIG + high-dose aspirin; low-dose aspirin continues until coronary arteries normalize.
Juvenile Idiopathic Arthritis (JIA)
- Arthritis persisting ≥6 weeks in a child <16 years with other causes excluded. Systemic JIA presents with quotidian fever, salmon-pink rash, and arthritis. Oligoarticular JIA is the most common subtype and carries the highest antinuclear antibody (ANA) and uveitis risk — mandatory slit-lamp screening.
Osteomyelitis & Septic Arthritis
- A child with acute refusal to bear weight or move a limb raises concern for septic arthritis of the hip. The Kocher criteria (fever, ESR >40, WBC >12,000, inability to bear weight) stratify risk; high-probability hips require urgent ultrasound and surgical washout. Staphylococcus aureus is the most common organism across all ages.
Orthopedic Conditions of Childhood
- Developmental dysplasia of the hip (DDH): risk factors are female sex, breech presentation, firstborn, and oligohydramnios. Neonates are screened with the Barlow (dislocatable) and Ortolani (reducible) maneuvers; confirmed DDH is treated with a Pavlik harness in early infancy.
- Legg-Calvé-Perthes disease: idiopathic avascular necrosis of the femoral head in children 4–8 years, presenting with a painless limp and limited hip abduction.
- Slipped capital femoral epiphysis (SCFE): displacement through the proximal femoral physis in overweight early adolescents (10–16 years); presents with hip or referred knee pain and external rotation — managed with urgent in situ pinning to prevent avascular necrosis.
- Osgood-Schlatter disease: traction apophysitis of the tibial tuberosity in active adolescents; managed with activity modification, ice, and short courses of NSAIDs.
A 4-year-old boy presents with cola-colored urine, periorbital edema, and hypertension 10 days after a sore throat. Laboratory testing shows a low C3 and elevated antistreptolysin O (ASO). What is the most likely diagnosis?
Post-streptococcal glomerulonephritis
Minimal change disease
IgA nephropathy
Hemolytic uremic syndrome
A 3-year-old has fever for 6 days, bilateral non-exudative conjunctivitis, a strawberry tongue, a polymorphous rash, and swollen, erythematous hands. What is the most appropriate treatment and why?
Antipyretics and discharge home
IVIG and high-dose aspirin to prevent coronary artery aneurysms
Intravenous antibiotics for suspected cellulitis
Corticosteroids for systemic juvenile idiopathic arthritis
A 6-year-old boy develops ascending weakness in his legs two weeks after a diarrheal illness. Deep tendon reflexes are absent. What is the most appropriate next step in management?
Order an MRI of the spine
Start corticosteroids
Give pyridostigmine
Administer IV immunoglobulin and monitor respiratory function
Sections you finish are checked off in the contents.