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2008 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 > Other And Unspecified Disorders Of Metabolism 277.* > 2008 ICD-9-CM Diagnosis 277.86 Peroxisomal disorders A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional PEROXISOMES. Peroxisomal enzymatic abnormalities may be single or multiple. Biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. Diseases in this category include ZELLWEGER SYNDROME; infantile Refsum disease; rhizomelic chondrodysplasia (CHONDRODYSPLASIA PUNCTATA, RHIZOMELIC); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and ADRENOLEUKODYSTROPHY (X-linked). Neurologic dysfunction is a prominent feature of most peroxisomal disorders. A syndrome combining the characteristics of adrenocortical insufficiency (Addison disease) with those of cerebral sclerosis (Schilder disease). Skin bronzing and sclerosis of the brain and demyelination are the principal manifestations. An autosomal recessive disorder due to defects in PEROXISOME biogenesis which involves more than 13 genes encoding peroxin proteins of the peroxisomal membrane and matrix. Zellweger syndrome is typically seen in the neonatal period with features such as dysmorphic skull; MUSCLE HYPOTONIA; SENSORINEURAL HEARING LOSS; visual compromise; SEIZURES; progressive degeneration of the KIDNEYS and the LIVER. Zellweger-like syndrome refers to phenotypes resembling the neonatal Zellweger syndrome but seen in children or adults with apparently intact peroxisome biogenesis. An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS). 277.86 is a specific code that can be used to specify a diagnosis 277.86 contains 11 index entries View the ICD-9-CM Volume 1 277.* hierarchy 277.86 also known as: Adrenomyeloneuropathy Neonatal adrenoleukodystrophy Rhizomelic chrondrodysplasia punctata X-linked adrenoleukodystrophy Zellweger syndrome Index entries containing 277.86: Aciduria 791.9 glutaric type III 277.86 Adrenoleukodystrophy 277.86 neonatal 277.86 x-linked 277.86 Adrenomyeloneuropathy 277.86 Chondrodysplasia 756.4 rhizomelic punctata 277.86 Disorder - see also Disease peroxisomal 277.86 Neonatal - see also condition adrenoleukodystrophy 277.86 Rhizomelic chrondrodysplasia punctata 277.86 Syndrome - see also Disease Zellweger 277.86 Zellweger syndrome 277.86