Steel hair syndrome

Steel hair syndrome is

[A] Scleroderma
[C] Menkes disease
[D] Osteogenesis imperfecta

Menkes disease

  • Menkes disease is characterized by an unstable body temperature and strikingly tangled hair, which is colorless or steel-colored, sparse, and known to break easily.
  • X-linked disorder of intestinal copper absorption.
  • Metabolic defect – The third phase: Transport across the serosal aspect of the mucosal cell membrane is defective.
  • Defect at molecular level – Mutations in the gene for a “copper binding P type ATPase

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