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Menkes Kinky Hair Syndrome (Menkes Disease) Summary

Description: An inherited disorder of copper metabolism transmitted as an X-linked trait and characterized by the infantile onset of HYPOTHERMIA, feeding difficulties, hypotonia, SEIZURES, bony deformities, pili torti (twisted hair), and severely impaired intellectual development. Defective copper transport across plasma and endoplasmic reticulum membranes results in copper being unavailable for the synthesis of several copper containing enzymes, including PROTEIN-LYSINE 6-OXIDASE; CERULOPLASMIN; and SUPEROXIDE DISMUTASE. Pathologic changes include defects in arterial elastin, neuronal loss, and gliosis. (From Menkes, Textbook of Child Neurology, 5th ed, p125)

Also Known As: Menkes Disease; Kinky Hair Syndrome; Hypocupremia, Congenital; Menkes Syndrome; Steely Hair Syndrome Show All >>

Networked: 599 relevant articles (7 outcomes, 38 trials/studies) for this Disease

Key Drugs and Agents for Menkes Kinky Hair Syndrome

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Copper : 3 outcomes 23 studies in 467 results : IBA
  2. Dopamine beta hydroxylase deficiency : 1 outcome in 2 results : IBA
  3. Tocopherols : 1 outcome in 1 result : FDA 2
  4. Monophenol Monooxygenase (Tyrosinase) : 1 outcome in 1 result : IBA
  5. Pyruvaldehyde (Methylglyoxal) : 1 outcome in 1 result : IBA
  6. Pyridoxine (Pyridoxin) : 1 outcome in 1 result : FDA 27 Generic
  7. Niacin (Nicotinic Acid) : 1 outcome in 1 result : FDA 21 Generic
  8. Adenosine Triphosphatases (ATPase) : 4 studies in 82 results : IBA
  9. Proteins (Proteins, Gene) : 2 studies in 17 results : IBA
  10. Protons (Proton) : 2 studies in 3 results : IBA
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Diseases Related to Menkes Kinky Hair Syndrome

  1. Oculocutaneous Albinism (Albinism, Yellow Mutant)
  2. Neurofibromatoses (Neurofibromatosis)
  3. Exfoliative Dermatitis (Erythroderma)
  4. Hepatolenticular Degeneration (Wilson's Disease)
  5. Menkes Kinky Hair Syndrome (Menkes Disease)
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Key Therapies for Menkes Kinky Hair Syndrome

Efficacy Chart >>
  1. Chelation Therapy (Therapy, Chelation) : 1 study in 1 result
  2. Therapeutics : 1 study in 1 result
  3. Injections : 5 results
  4. Anesthesia : 2 results
  5. Airway Management : 1 result
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