AHA Coding Clinic® for ICD-9 - 2004 Issue 4; VOLUMES 1 AND 2 NEW/REVISED DIAGNOSIS CODES

Peroxisomal Disorders

Effective October 1, 2004, code 277.86, Peroxisomal disorders, has been created to uniquely identify peroxisomal disorders. Peroxisomal disorders are rare congenital diseases that are characterized by the absence of normal peroxisomes in the cells of the body. The most common is X-linked adrenoleukodystrophy with an incidence of 1 in 17,000. Others have an overall estimate of about 1 in 50,000. Peroxisomes contain enzymes that function as catalysts in metabolic processes. They are responsible for oxidation of fatty acids, biosynthesis of membrane phospholipids (plasmalogens), cholesterol and bile acids, conversion of amino acids into glucose, reduction of hydrogen...

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