The most severe form of Ehler Danlos syndrome is
Correct Answer: Type 4
Description: Ehlers danlos syndrome *A genetic defect in the synthesis of fibrillar collagen*Of all the types, type IV (vascular type) is the most severe form as it involves internal organs*Common type - Type VI (kyphoscoliosis type) caused due to a defect in lysyl hydroxylase*Type VII - defect in type 1 collagen*Type IV - Defect in type 3 collagen*Type I and II - Defect in type 5 collagen ( Ref: Robbins 8/e p146)
Category:
Pathology
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