title: Catecholamine metabolism in steely hair disease.
abstract: Steely hair disease, a neurodegenerative disorder, is characterized by slow growth, progressive cerebral dysfunction, kinky friable hair, x-linked inheritance, and death before three years of age. Low-serum copper has been found to play a role in steely hair disease. This article describes the role of copper, the pathophysiology of this disease, and current treatment and research. A case history and pertinent aspects of nursing care of these patients are also presented.
mesh_terms: D001928:Brain Diseases, Metabolic / Q000378:metabolism*; D002395:Catecholamines / Q000378:metabolism*; D003300:Copper / Q000097:blood / Q000378:metabolism / Q000627:therapeutic use; D004298:Dopamine / Q000097:blood / Q000378:metabolism; D015103:Droxidopa / Q000627:therapeutic use; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D007706:Menkes Kinky Hair Syndrome / Q000188:drug therapy / Q000378:metabolism* / Q000451:nursing; D009638:Norepinephrine / Q000097:blood / Q000134:cerebrospinal fluid / Q000378:metabolism; D009732:Nursing Care
chemical_list: D002395:Catecholamines; D003300:Copper; D015103:Droxidopa; D004298:Dopamine; D009638:Norepinephrine