Thieffry and Sorrell Dejerine syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Thieffry and Sorrell Dejerine syndrome is a rare inherited syndrome characterized by kidney problems and progressive destruction of the wrist and ankle bones which may spread to adjoining bones.

Pathophysiology

Inheritence occurs in both sporadic and autosomal dominant patterns.

Diagnosis

• Hereditary osteolysis
• Hypertension
• Marfanoid appearence
• Micrognathia
• Scoliosis
• Overlapping toes
• Plantar cysts
• Disorder begins in childhood, with painless and progressive loss of bone in carpal and tarsal bones,