Specific code 2014 ICD-9-CM Diagnosis Code 271.8
Other specified disorders of carbohydrate transport and metabolism
  • Billable Code
  • Short description: Dis carbohydr metab NEC.
  • ICD-9-CM 271.8 will be replaced by an equivalent ICD-10-CM code (or codes) when the United States transitions from ICD-9-CM to ICD-10-CM on October 1, 2015.
  • ICD-9-CM 271.8 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Disease Synonyms
  • Acquired monosaccharide malabsorption
  • Adult fucosidosis
  • alpha, alpha-Trehalase deficiency
  • Antifreeze oxalosis
  • Beta-D-mannosidosis
  • Carbohydrate deficiency glycoprotein syndrome
  • Carbohydrate deficient glycoprotein syndrome
  • Carbohydrate-deficient glycoprotein syndrome
  • Carbohydrate-deficient glycoprotein syndrome type II
  • Carbohydrate-deficient glycoprotein syndrome type III
  • Deficiency of alpha-mannosidase
  • Deficiency, pyruvate carboxylase
  • Deficiency, pyruvate deshydrogenase
  • Disorder of carbohydrate absorption
  • Disorder of glucose metabolism
  • Disorder of glycerate metabolism
  • Disorder of glycoprotein metabolism
  • Disorders of pyruvate metabolism and gluconeogenesis
  • Dysglycemia
  • Enteric hyperoxaluria
  • Essential benign pentosuria
  • Essential pentosuria
  • Fucosidosis
  • Glycoprotein metabolism disorder
  • Hyperglycemic disorder
  • Hyperoxaluria
  • Impaired glucose tolerance associated with genetic syndrome
  • Impaired intestinal epithelial cell transport of carbohydrates
  • Infantile fucosidosis
  • Insulin resistance - type A
  • Insulin resistance - type B
  • Juvenile fucosidosis
  • Lactate dehydrogenase deficiency
  • Mannosidosis
  • Mannosidosis, type I
  • Mannosidosis, type II
  • Nonglucosuric melituria
  • Oxalosis
  • Primary hyperoxaluria
  • Primary hyperoxaluria, type I
  • Primary hyperoxaluria, type II
  • Pyruvate carboxylase deficiency
  • Pyruvate dehydrogenase complex deficiency
  • Pyruvate dehydrogenase deficiency
  • Pyruvate metabolism and gluconeogenesis disorder
  • Secondary oxalosis
  • Sialidosis
  • Syndrome of carbohydrate intolerance
  • Xylosuria
Clinical Information
  • Either of two genetic disorders characterized by urinary excretion of large amounts of oxalate, with nephrolithiasis, nephrocalcinosis, early onset of renal failure, and often a generalized deposit of calcium oxalate, resulting from a defect in glyoxalate metabolism.
  • Lysosomal storage disease caused by defective alpha-l-fucosidase and accumulation of fucose containing glycoconjugates; clinical symptoms include psychomotor deterioration, growth retardation, hepatosplenomegaly, cardiomegaly, and seizures.
  • Lysosomal storage disease due to defective alpha-mannosidase with resultant oligosaccharide accumulation.
  • Lysosome storage disease due to alpha-l-fucosidase (e.c. 3.2.1.51) deficiency in leukocytes manifested by abnormal accumulation in tissues and urinary excretion of partially catabolized oligosaccharides, glycoasparagines, and glycolipids with alpha-linked fucose at the nonreducing end of the glycogen chain. The phenotype is variable and may include delayed growth and mental development, progressive neurological deterioration, hurler-like (mucopolysaccharidosis i-h) coarse facies, recurrent infections, visceromegaly, skeletal abnormalities, joint contractures, deafness, and angiokeratoma corporis diffusum. Several types are recognized by different researchers. The form exhibiting a longer survival, mild neurological manifestations, and angiokeratoma is sometimes referred to as fucosidosis type ii. In a different scheme, three different types are recognized according to their age of onset. Types i and ii are the most severe and have their onsets at 10 and 18 months, respectively with life expectancy of 6 years. Type iii represents a juvenile form which is marked by a milder form of psychomotor retardation and a slower deterioration of neurological activities. Hurler-like (gargyloid) facies occur mainly in types i and ii and is less commonly in type iii.
Applies To
  • Essential benign pentosuria
  • Fucosidosis
  • Glycolic aciduria
  • Hyperoxaluria (primary)
  • Mannosidosis
  • Oxalosis
  • Xylosuria
  • Xylulosuria
Convert to ICD-10-CM: 271.8 converts approximately to:
  • 2014 ICD-10-CM E72.52 Trimethylaminuria
    Or:
  • 2014 ICD-10-CM E72.53 Hyperoxaluria
    Or:
  • 2014 ICD-10-CM E74.4 Disorders of pyruvate metabolism and gluconeogenesis
    Or:
  • 2014 ICD-10-CM E74.8 Other specified disorders of carbohydrate metabolism
    Or:
  • 2014 ICD-10-CM E77.1 Defects in glycoprotein degradation
ICD-9-CM Volume 2 Index entries containing back-references to 271.8:
  • Absence (organ or part) (complete or partial)
    • alpha-fucosidase 271.8
    • fucosidase 271.8
  • Aciduria 791.9
    • glycolic 271.8
  • Bird's disease (oxaluria) 271.8
  • Carbohydrate-deficient glycoprotein syndrome (CDGS) 271.8
  • CDGS (carbohydrate-deficient glycoprotein syndrome) 271.8
  • Deficiency, deficient
    • alpha-fucosidase 271.8
    • alpha-mannosidase 271.8
    • fucosidase 271.8
    • mannosidase 271.8
    • phosphoenolpyruvate carboxykinase 271.8
    • phosphomannomutase 271.8
    • phosphomannose isomerase 271.8
    • phosphomannosyl mutase 271.8
    • pyruvate carboxylase 271.8
    • pyruvate dehydrogenase 271.8
  • Diathesis
    • oxalic 271.8
  • Disease, diseased - see also Syndrome
    • Bird's (oxaluria) 271.8
  • Disorder - see also Disease
    • congenital
      • glycosylation (CDG) 271.8
    • infant sialic acid storage 271.8
    • transport, carbohydrate 271.9
      • specified type NEC 271.8
  • Fucosidosis 271.8
  • Hyperoxaluria (primary) 271.8
  • L-xyloketosuria 271.8
  • Malabsorption 579.9
    • monosaccharide 271.8
  • Mannoheptulosuria 271.8
  • Mannosidosis 271.8
  • Oxalosis 271.8
  • Oxaluria 271.8
  • Pentosuria (benign) (essential) 271.8
  • Syndrome - see also Disease
    • carbohydrate-deficient glycoprotein (CDGS) 271.8
  • Xyloketosuria 271.8
  • Xylosuria 271.8
  • Xylulosuria 271.8
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 271.8 is one of thousands of ICD-9-CM codes used in healthcare. Although ICD-9-CM and CPT codes are largely numeric, they differ in that CPT codes describe medical procedures and services. Can't find a code? Start at the root of ICD-9-CM, check the 2014 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.