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 > Disorders Of Lipoid Metabolism 272.* >  2008 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
- View the ICD-9-CM Volume 1 272.* hierarchy
272.7 also known as:- Chemically induced lipidosis
- Disease:
- Anderson's
- Fabry's
- Gaucher's
- I cell [mucolipidosis I]
- lipoid storage NOS
- Niemann-Pick
- pseudo-Hurler's or mucolipidosis III
- triglyceride storage, Type I or II
- Wolman's or triglyceride storage, Type III
- Mucolipidosis II
- Primary familial xanthomatosis
272.7 excludes: - cerebral lipidoses (330.1)
- Tay-Sachs disease (330.1)
Index entries containing 272.7:- Anderson's disease
 272.7
Anemia 285.9- splenic 285.8
- familial (Gaucher's) 272.7
Angiokeratoma (M9141/0) - see also Neoplasm, skin, benign- corporis diffusum 272.7
Angiokeratosis- diffuse 272.7
Angiomatosis 757.32- corporis diffusum universale 272.7
Cerebrosidosis 272.7
Ceroid storage disease 272.7
Diabetes, diabetic (brittle) (congenital) (familial) (mellitus) (poorly controlled) (severe) (slight) (without complication) 250.0Disease, diseased - see also Syndrome- Anderson's (angiokeratoma corporis diffusum) 272.7
- ceroid storage 272.7
- Fabry's (angiokeratoma corporis diffusum) 272.7
- Gaucher's (adult) (cerebroside lipidosis) (infantile) 272.7
- I cell 272.7
- lipidosis 272.7
- lipoid storage NEC 272.7
- mucolipidosis (I) (II) (III) 272.7
- Niemann-Pick (lipid histiocytosis) 272.7
- Pick's
- lipid histiocytosis 272.7
- pseudo-Hurler's (mucolipidosis III) 272.7
- storage
- lipid 272.7
- triglyceride-storage, type I, II, III 272.7
- Wolman's (primary familial xanthomatosis) 272.7
Disorder - see also Disease- lipid
- storage 272.7
- metabolism NEC 277.9
Disturbance - see also Disease- metabolism (acquired) (congenital) (see also Disorder, metabolism) 277.9
- phosphatides 272.7
Fabry's disease (angiokeratoma corporis diffusum) 272.7
Gaucher's- disease (adult) (cerebroside lipidosis) (infantile) 272.7
- hepatomegaly 272.7
- splenomegaly (cerebroside lipidosis) 272.7
Hepatomegaly (see also Hypertrophy, liver) 789.1- Gaucher's 272.7
Histiocytosis (acute) (chronic) (subacute) 277.89- lipid, lipoid (essential) 272.7
Hyperlipidosis 272.7- hereditary 272.7
Lipidosis 272.7- cephalin 272.7
- cerebral (infantile) (juvenile) (late) 330.1
- cerebroretinal 330.1 [362.71]
- cerebroside 272.7
- cerebrospinal 272.7
- chemically-induced 272.7
- cholesterol 272.7
- diabetic 250.8 [272.7]
- dystopic (hereditary) 272.7
- glycolipid 272.7
- hepatosplenomegalic 272.3
- hereditary, dystopic 272.7
- sulfatide 330.0
Lipoid - see also condition- histiocytosis 272.7
- essential 272.7
Lipoidosis (see also Lipidosis) 272.7
Mucolipidosis I, II, III 272.7
Niemann-Pick disease (lipid histiocytosis) (splenomegaly) 272.7
Pick's- disease
- lipid histiocytosis 272.7
Pick-Niemann disease (lipid histiocytosis) 272.7
Pseudo-Hurler's disease (mucolipidosis III) 272.7
Purpura 287.2- hemorrhagic (see also Purpura, thrombocytopenic) 287.39
- nodular 272.7
Ruiter-Pompen (-Wyers) syndrome (angiokeratoma corporis diffusum) 272.7
Sea-blue histiocyte syndrome 272.7
Sphingolipidosis 272.7
Sphingolipodystrophy 272.7
Sphingomyelinosis 272.7
Splenomegaly 789.2- Gaucher's (cerebroside lipidosis) 272.7
- Niemann-Pick (lipid histiocytosis) 272.7
Sweeley-Klionsky disease (angiokeratoma corporis diffusum) 272.7
Syndrome - see also Disease- cardiovasorenal 272.7
- Fabry (-Anderson) (angiokeratoma corporis diffusum) 272.7
- Niemann-Pick (lipid histiocytosis) 272.7
- Ruiter-Pompen (-Wyers) (angiokeratoma corporis diffusum) 272.7
- sea-blue histiocyte 272.7
Thesaurismosis- kerasin 272.7
- lipoid 272.7
- phosphatide 272.7
Trihexosidosis 272.7
Wolman's disease (primary familial xanthomatosis) 272.7
Xanthoma(s), xanthomatosis 272.2- bone 272.7
- cutaneotendinous 272.7
- disseminatum 272.7
- familial 272.7
- hereditary 272.7
- infantile 272.7
- joint 272.7
- juvenile 272.7
- multiple 272.7
- multiplex 272.7
- primary familial 272.7
- tendon (sheath) 272.7
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