2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Endocrine, Nutritional And Metabolic Diseases, And Immunity Disorders 240-279 > Other Metabolic Disorders And Immunity Disorders 270-279 > Disorders Of Mineral Metabolism 275.* > 2006 ICD-9-CM Diagnosis 275.1 Disorders of copper metabolism View the most recent version of ICD-9-CM 275.1 A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years. 275.1 is a specific code that can be used to specify a diagnosis 275.1 contains 24 index entries View the ICD-9-CM Volume 1 275.* hierarchy Alternate Terminology Hepatolenticular degeneration Wilson's disease  Index entries containing 275.1: Cirrhosis, cirrhotic 571.5 due to hepatolenticular degeneration 275.1 Wilson's disease 275.1 Deficiency, deficient ceruloplasmin 275.1 copper NEC 275.1 Degeneration, degenerative hepatolenticular (Wilson's) 275.1 lenticular (familial) (progressive) (Wilson's) (with cirrhosis of liver) 275.1 striate artery 437.0 Wilson's hepatolenticular 275.1 Disease, diseased - see also Syndrome hepatolenticular 275.1 Kinnier Wilson's (hepatolenticular degeneration) 275.1 Wilson's (hepatolenticular degeneration) 275.1 Disorder - see also Disease metabolism NEC 277.9 copper 275.1 Hepatolenticular degeneration 275.1 Kinnier Wilson's disease (hepatolenticular degeneration) 275.1 Lenticular degeneration, progressive 275.1 Pseudosclerosis (brain) of Westphal (-Strümpell) (hepatolenticular degeneration) 275.1 Strümpell-Westphal pseudosclerosis (hepatolenticular degeneration) 275.1 Syndrome - see also Disease amyostatic 275.1 chorea-athetosis-agitans 275.1 lenticular 275.1 Westphal-Strümpell (hepatolenticular degeneration) 275.1 Wilson's (hepatolenticular degeneration) 275.1 Westphal-Strümpell syndrome (hepatolenticular degeneration) 275.1 Wilson's disease or syndrome (hepatolenticular degeneration) 275.1 hepatolenticular degeneration 275.1