🩸 Section 511.1 · Sickle Cell Disease

Nelson Textbook of Pediatrics 22nd Edition | Sickle cell disease (HbSS, HbSC, HbSβ-thal). Pathophysiology: HbS polymerization → RBC sickling → hemolysis, vaso-occlusion. Newborn screening, penicillin prophylaxis, hydroxyurea (from 9 months), TCD for stroke prevention, chronic transfusions, acute pain management, acute chest syndrome, priapism, splenic sequestration, aplastic crisis (parvovirus B19). Gene therapy (Casgevy, Lyfgenia) approved for ≥12 years.

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📋 30 Clinical Scenarios — Sickle Cell Disease

📇 High‑Yield Review Cards (Sickle Cell Disease)

🩺 Clinical Recognition: Sickle Cell Disease Complications

Select a presentation for diagnostic clues and management.

📋 Stepwise Management of Sickle Cell Disease (Nelson 511.1)

🔑 Key Recommendations — NHLBI 2014 & Nelson
• Newborn screening → confirm, start penicillin VK by 2-4 months.
• Hydroxyurea 20-35 mg/kg/day for all children with HbSS starting at 9 months.
• TCD screening annually ages 2-16 years. Abnormal (>200 cm/sec) → chronic transfusions.
• Fever → emergency evaluation, IV ceftriaxone, admit.
• Gene therapy (Casgevy, Lyfgenia) approved for ≥12 years with recurrent VOC.

    ⚡ Reflex Prompts — Clinical Decisions in Sickle Cell Disease

    📖 Summary: Sickle Cell Disease — Nelson Section 511.1