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)

Descriptor ID	D007706
MeSH Number(s)	C10.228.140.163.100.540 C10.597.606.643.455.687 C16.320.322.500.687 C16.320.400.525.687 C16.320.565.189.540 C16.320.565.618.590 C17.800.329.968 C18.452.132.100.540 C18.452.648.189.540 C18.452.648.618.590
Concept/Terms	Menkes Kinky Hair Syndrome Menkes Kinky Hair Syndrome Hypocupremia, Congenital Kinky Hair Disease Diseases, Kinky Hair Hair Diseases, Kinky Kinky Hair Diseases Kinky Hair Syndrome Menkea Syndrome Menkea Syndromes Syndrome, Menkea Syndromes, Menkea X-Linked Copper Deficiency Menkes Syndrome Menkes' Disease Diseases, Menkes' Menkes' Diseases Steely Hair Disease Disease, Steely Hair Diseases, Steely Hair Hair Diseases, Steely Steely Hair Diseases Steely Hair Syndrome Steely Hair Syndromes Syndrome, Steely Hair Syndromes, Steely Hair Copper Transport Disease Congenital Hypocupremia Congenital Hypocupremias Hypocupremias, Congenital Menkes Disease

Below are MeSH descriptors whose meaning is more general than "Menkes Kinky Hair Syndrome".

Below are MeSH descriptors whose meaning is related to "Menkes Kinky Hair Syndrome".

Below are MeSH descriptors whose meaning is more specific than "Menkes Kinky Hair Syndrome".