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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 > ICD-9-CM Diagnosis 272 Disorders of lipoid metabolism 272 is a non-specific code that cannot be used to specify a diagnosis ICD-9-CM Diagnosis 272.0 Pure hypercholesterolemia A familial disorder characterized by increased plasma concentration of cholesterol carried in low density lipoproteins (LDL) and by a deficiency in a cell surface receptor which regulates LDL degradation and cholesterol synthesis. 272.0 is a specific code that can be used to specify a diagnosis 272.0 contains 22 index entries ICD-9-CM Diagnosis 272.1 Pure hyperglyceridemia A familial disorder characterized by an isolated elevation in the plasma level of endogenously synthesized triglyceride carried in VLDL. It is considered to be an autosomal dominant trait. 272.1 is a specific code that can be used to specify a diagnosis 272.1 contains 20 index entries ICD-9-CM Diagnosis 272.2 Mixed hyperlipidemia A disorder genetically distinct from the other inherited hyperlipidemias characterized by the type II or type IV lipoprotein pattern (the pattern may change from time to time and the lipid level may be normal at one time and abnormal at another time). 272.2 is a specific code that can be used to specify a diagnosis 272.2 contains 30 index entries ICD-9-CM Diagnosis 272.3 Hyperchylomicronemia A rare familial condition characterized by massive chylomicronemia and decreased levels of other lipoproteins. It is due to deficiency of lipoprotein lipase, an alkaline triglyceride hydrolase which catalyzes an important step in the extrahepatic removal of triglyceride-rich lipoproteins from the blood. 272.3 is a specific code that can be used to specify a diagnosis 272.3 contains 18 index entries ICD-9-CM Diagnosis 272.4 Other and unspecified hyperlipidemia An excess of lipids in the blood. Metabolic disease characterized by excess plasma LIPOPROTEINS. 272.4 is a specific code that can be used to specify a diagnosis 272.4 contains 14 index entries ICD-9-CM Diagnosis 272.5 Lipoprotein deficiencies A disease in which the low density lipoprotein (beta-lipoprotein) concentrations are far below normal. A disorder of lipid metabolism inherited as an autosomal recessive trait characterized by the near absence of APOLIPOPROTEINS B and apoB-containing lipoproteins in plasma. Microsomal triglyceride transfer protein is deficient or absent in enterocytes. Clinical and laboratory findings include acanthocytosis, hypocholesterolemia, peripheral neuropathy, posterior column degeneration, ataxia, and steatorrhea. Intellectual abilities may also be impaired. (Menkes, Textbook of Child Neurology, 5th ed, p118; Curr Opin Lipidol 1994 Apr;5(2):81-6) The presence of abnormally low levels of lipoproteins in the serum. 272.5 is a specific code that can be used to specify a diagnosis 272.5 contains 19 index entries ICD-9-CM Diagnosis 272.6 Lipodystrophy A collection of rare conditions resulting from defective fat metabolism and characterized by atrophy of the subcutaneous fat. They include total, congenital or acquired, partial, abdominal infantile, and localized lipodystrophy. 272.6 is a specific code that can be used to specify a diagnosis 272.6 contains 10 index entries ICD-9-CM Diagnosis 272.7 Lipidoses A disturbance of lipid metabolism with abnormal deposit of lipids in the cells. (Dorland, 27th ed) A group of diseases marked by autosomal recessive inheritance and accumulation of sphingomyelin in cells of the RETICULOENDOTHELIAL SYSTEM. They are divided into 5 subtypes: A-E. Type A (classic infantile form) is caused by a deficiency of SPHINGOMYELIN PHOSPHODIESTERASE and presents at age 6-12 months with progressive hepatosplenomegaly and neurologic deterioration. Type B (non-neuronopathic form) presents in childhood with hepatosplenomegaly and pulmonary infiltrates. Type C (chronic neuronopathic form) is caused by defective intracellular cholesterol transport and is divided into severe infantile, late infantile, juvenile, and neonatal hepatitis forms. Type D (Nova Scotian Variant) is phenotypically similar to type C. Type E is an adult non-neuronopathic form. (From Menkes, Textbook of Child Neurology, 5th ed, pp101-4) An autosomal recessive disorder caused by deficiency of the enzyme glucocerebrosidase (see GLUCOSYLCERAMIDASE) featuring the pathological storage of glycosylceramide in mononuclear PHAGOCYTES (Gaucher Cells). The most common subtype is the non-neuronopathic form, a slowly progressive condition characterized by hepatosplenomegaly and skeletal deformities. The neuronopathic forms are divided into infantile and juvenile forms. The infantile form presents at 4-5 months of age with anemia, loss of cognitive gains, neck retraction, dysphagia, and hepatosplenomegaly. The juvenile form features a slowly progressive loss of intellect, hepatosplenomegaly, ATAXIA, myoclonic SEIZURES, and spasticity. The neuronopathic forms are characterized by neuronal loss with neuronophagia, and accumulation of glucocerebroside in neurons. (From Baillieres Clin Haematol 1997 Dec;10(4):711-23; Menkes, Textbook of Child Neurology, 5th ed, p97) Intestinal fat transport defect with hypobetalipoproteinemia and accumulation of apolipoprotein B-like protein in intestinal cells, deficient blood apolipoproteins, and avitaminosis E manifested by malnutrition, steatorrhea, and growth and mental retardation. Some clinical (but not biochemical) manifestations may disappear later in life. Lysosomal storage disease caused by a deficiency of alpha-galactosidase A and resulting in an accumulation of globotriaosylceramide in the renal and cardiovascular systems. The disease is X-linked and is characterized by telangiectatic skin lesions, renal failure, and disturbances of the cardiovascular, gastrointestinal, and central nervous systems. The severe infantile form of inherited lysosomal lipid storage diseases, due to deficiency of acid lipase. It results in accumulation of neutral lipids, particularly cholesterol esters, within cells (particularly leukocytes, fibroblasts, and liver cells). An allelic variant of CHOLESTEROL ESTER STORAGE DISEASE, it is also known as Wolman's xanthomatosis. 272.7 is a specific code that can be used to specify a diagnosis 272.7 contains 82 index entries ICD-9-CM Diagnosis 272.8 Other disorders of lipoid metabolism A systemic disease, often associated with arthritis, fever, and weight loss. The cutaneous lesions consist of histiocytes, which are usually confined to the dermis. A large number of patients develop malignant tumors (carcinomas, sarcomas, and lymphomas). --2003 272.8 is a specific code that can be used to specify a diagnosis 272.8 contains 50 index entries ICD-9-CM Diagnosis 272.9 Unspecified disorder of lipoid metabolism 272.9 is a specific code that can be used to specify a diagnosis 272.9 contains 10 index entries