Menkes syndrome

Alternative names:

kinky hair disease; Menkes kinky hair syndrome; steely hair disease

Definition:

An inherited disorder that results in reduced absorption of copper from the intestines.

Causes, incidence, and risk factors:

Menkes syndrome is inherited as an X-linked recessive disorder. The symptom is related to deficient levels of available copper. Symptoms include progressive mental deterioration, kinked or twisted brittle hair, and skeletal changes. Death usually occurs in the first few years of life.