๐Ÿฉธ Chapter 526 ยท Von Willebrand Disease (VWD)

Nelson Textbook of Pediatrics 22nd Edition | Von Willebrand disease is the most common inherited bleeding disorder (prevalence 1:100 to 1:10,000). Mucocutaneous bleeding: epistaxis, menorrhagia, easy bruising, bleeding after dental extractions. Types: Type 1 (quantitative deficiency, 60-80%), Type 2 (qualitative defects: 2A, 2B, 2M, 2N), Type 3 (severe deficiency). Diagnosis: VWF:Ag, VWF:RCo (activity), FVIII, multimer analysis. Treatment: desmopressin (DDAVP) for type 1, VWF-containing concentrates for type 2/3, antifibrinolytics for mucosal bleeding.

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๐Ÿ“‹ 30 Clinical Scenarios โ€” Von Willebrand Disease

๐Ÿ“‡ Highโ€‘Yield Review Cards (von Willebrand Disease)

๐Ÿฉบ Clinical Recognition: Von Willebrand Disease

Select a presentation for diagnostic clues and management.

๐Ÿ“‹ Stepwise Management of Von Willebrand Disease

๐Ÿ”‘ Key Principles โ€” Nelson Chapter 526
โ€ข Type 1 (most common): Quantitative deficiency. Desmopressin (DDAVP) is first-line (IV 0.3 mcg/kg or intranasal). Rises VWF and FVIII.
โ€ข Type 2A/2B/2M: Qualitative defects. Desmopressin may be ineffective or contraindicated (type 2B โ€” thrombocytopenia). Use VWF-containing concentrates (Humate-P, Wilate).
โ€ข Type 2N: Decreased FVIII binding (mimics hemophilia A). Desmopressin may increase FVIII temporarily; VWF concentrate needed.
โ€ข Type 3: Severe deficiency. VWF concentrates. Desmopressin ineffective.
โ€ข Antifibrinolytics (tranexamic acid): For mucosal bleeding (epistaxis, menorrhagia, dental procedures).
โ€ข Hormonal therapy: For menorrhagia (oral contraceptives, levonorgestrel IUD).

    โšก Reflex Prompts โ€” Clinical Decisions in VWD

    ๐Ÿ“– Summary: Von Willebrand Disease โ€” Nelson Chapter 526