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Comprehensive Pediatrics Glossary

W

Werdnig-Hoffmann Disease (Spinal Muscular Atrophy Type 1)

  • Definition: Severe autosomal recessive neuromuscular disorder characterized by degeneration of anterior horn cells in the spinal cord, leading to progressive muscle weakness.

  • Epidemiology: Most severe form of SMA; onset usually before 6 months of age.

  • Clinical Features: Hypotonia (“floppy infant”), generalized weakness, absent deep tendon reflexes, difficulty feeding, respiratory compromise.

  • Investigations: Genetic testing (SMN1 gene deletion), EMG (denervation changes), muscle biopsy rarely needed.

  • Management: Supportive care: respiratory support, nutritional support, physiotherapy. Disease-modifying therapy: nusinersen, onasemnogene abeparvovec, risdiplam.

  • Complications: Respiratory failure, recurrent infections, early mortality without treatment.

Wilms Tumor (Nephroblastoma)

  • Definition: Most common pediatric renal malignancy, usually affecting children aged 2–5 years.

  • Clinical Features: Abdominal mass (usually unilateral, firm, painless), hematuria, hypertension, fever, malaise.

  • Investigations: Ultrasound (initial), CT/MRI abdomen for staging, chest imaging for metastasis, biopsy if diagnosis uncertain.

  • Management: Multimodal: nephrectomy, chemotherapy (vincristine, actinomycin D, doxorubicin), radiotherapy for advanced disease.

  • Complications: Recurrence, metastasis (lungs), chemotherapy-related toxicity.

  • Prognosis: Favorable with early diagnosis; 5-year survival >90% for localized disease.

Wasting (Pediatric Malnutrition)

  • Definition: Acute malnutrition characterized by low weight-for-height (WHZ < -2 SD).

  • Etiology: Inadequate dietary intake, infectious diseases, chronic illness.

  • Clinical Features: Muscle wasting, loss of subcutaneous fat, apathy, edema in severe cases (kwashiorkor overlap).

  • Investigations: Anthropometry (weight-for-height, MUAC), serum albumin, hemoglobin, electrolytes.

  • Management: Nutritional rehabilitation (therapeutic milk F-75/F-100), micronutrient supplementation, treatment of infections.

  • Complications: Immune dysfunction, increased mortality risk, growth retardation.

Whooping Cough (Pertussis)

  • Definition: Highly contagious respiratory infection caused by Bordetella pertussis.

  • Clinical Features: Catarrhal phase (1–2 weeks): mild cough, rhinorrhea, low-grade fever; Paroxysmal phase: severe coughing fits, inspiratory “whoop,” post-tussive vomiting; Convalescent phase: gradual recovery.

  • Investigations: Nasopharyngeal swab for PCR or culture, CBC (lymphocytosis).

  • Management: Macrolide antibiotics (azithromycin, clarithromycin), supportive care (oxygen, hydration), isolation to prevent spread.

  • Prevention: DTaP vaccination (primary series + booster).

  • Complications: Pneumonia, apnea (especially in infants), seizures, encephalopathy, death.

Wolff-Parkinson-White Syndrome (WPW)

  • Definition: Congenital pre-excitation syndrome due to accessory pathway between atria and ventricles, leading to tachyarrhythmias.

  • Clinical Features: Palpitations, syncope, dizziness, possible sudden cardiac arrest; may be asymptomatic.

  • Investigations: ECG: short PR interval, delta wave, widened QRS; Holter monitoring; electrophysiological study for risk stratification.

  • Management: Acute: vagal maneuvers, antiarrhythmic drugs (procainamide), cardioversion if unstable. Chronic: catheter ablation of accessory pathway.

  • Complications: Supraventricular tachycardia, atrial fibrillation, sudden cardiac death (rare).

Wernicke Encephalopathy (Pediatric)

  • Definition: Rare pediatric neurological disorder due to thiamine (vitamin B1) deficiency.

  • Clinical Features: Triad: ophthalmoplegia, ataxia, confusion; may also present with irritability, seizures.

  • Etiology: Malnutrition, prolonged parenteral nutrition without thiamine, chronic gastrointestinal disease.

  • Investigations: Clinical diagnosis; MRI brain may show mammillary body changes; low serum thiamine supports diagnosis.

  • Management: Prompt intravenous thiamine replacement, supportive care, treatment of underlying cause.

  • Complications: Korsakoff syndrome (memory impairment), irreversible neurological deficits, death if untreated.

Wiskott-Aldrich Syndrome

  • Definition: X-linked primary immunodeficiency characterized by thrombocytopenia, eczema, and recurrent infections.

  • Etiology: Mutation in WAS gene affecting actin cytoskeleton of hematopoietic cells.

  • Clinical Features: Petechiae, bruising, eczema, recurrent bacterial/viral/fungal infections, autoimmune manifestations.

  • Investigations: CBC (thrombocytopenia), immunoglobulin levels, flow cytometry, genetic testing.

  • Management: Supportive: IVIG, antibiotics, platelet transfusions; curative: hematopoietic stem cell transplantation.

  • Complications: Autoimmune disease, malignancies (lymphoma), severe infections, early mortality if untreated.

Waterhouse-Friderichsen Syndrome (Pediatric)

  • Definition: Acute adrenal insufficiency due to adrenal hemorrhage, most commonly from meningococcemia.

  • Clinical Features: Rapid onset fever, purpura fulminans, hypotension, shock, vomiting, lethargy.

  • Investigations: Blood culture, cortisol levels (low), electrolytes (hyponatremia, hyperkalemia), coagulation profile.

  • Management: Aggressive IV fluids, IV antibiotics (ceftriaxone/penicillin), IV hydrocortisone, ICU support.

  • Complications: Multiorgan failure, death if not treated promptly.

 

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