Kinky hair disease, congenital defect of copper metabolism manifested in short, sparse, poorly pigmented kinky hair; associated with failure to thrive, physical and mental retardation, and progressive severe deterioration of the brain; apparently a defect of copper transport; X-linked recessive inheritance.
Synonym: kinky-hair disorder, Menkes' syndrome, trichopoliodystrophy.
(05 Mar 2000)