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ICD-10-CM/PCS MS-DRGv33 Definitions Manual Previous Page [Alt-p] Up a level [Alt-u] Next Page [Alt-n]
MDC 10 Endocrine, Nutritional & Metabolic Diseases & Disorders
Inborn Errors of Metabolism
Inborn Errors of Metabolism


C965Multifocal and unisystemic Langerhans-cell histiocytosis
C966Unifocal Langerhans-cell histiocytosis
D813Adenosine deaminase [ADA] deficiency
D815Purine nucleoside phosphorylase [PNP] deficiency
D81810Biotinidase deficiency
D841Defects in the complement system
E700Classical phenylketonuria
E701Other hyperphenylalaninemias
E7020Disorder of tyrosine metabolism, unspecified
E7029Other disorders of tyrosine metabolism
E7030Albinism, unspecified
E70310X-linked ocular albinism
E70311Autosomal recessive ocular albinism
E70318Other ocular albinism
E70319Ocular albinism, unspecified
E70320Tyrosinase negative oculocutaneous albinism
E70321Tyrosinase positive oculocutaneous albinism
E70328Other oculocutaneous albinism
E70329Oculocutaneous albinism, unspecified
E70330Chediak-Higashi syndrome
E70331Hermansky-Pudlak syndrome
E70338Other albinism with hematologic abnormality
E70339Albinism with hematologic abnormality, unspecified
E7039Other specified albinism
E7040Disorders of histidine metabolism, unspecified
E7049Other disorders of histidine metabolism
E705Disorders of tryptophan metabolism
E708Other disorders of aromatic amino-acid metabolism
E709Disorder of aromatic amino-acid metabolism, unspecified
E710Maple-syrup-urine disease
E71110Isovaleric acidemia
E711113-methylglutaconic aciduria
E71118Other branched-chain organic acidurias
E71120Methylmalonic acidemia
E71121Propionic acidemia
E71128Other disorders of propionate metabolism
E7119Other disorders of branched-chain amino-acid metabolism
E712Disorder of branched-chain amino-acid metabolism, unspecified
E7130Disorder of fatty-acid metabolism, unspecified
E71310Long chain/very long chain acyl CoA dehydrogenase deficiency
E71311Medium chain acyl CoA dehydrogenase deficiency
E71312Short chain acyl CoA dehydrogenase deficiency
E71313Glutaric aciduria type II
E71314Muscle carnitine palmitoyltransferase deficiency
E71318Other disorders of fatty-acid oxidation
E7132Disorders of ketone metabolism
E7139Other disorders of fatty-acid metabolism
E7140Disorder of carnitine metabolism, unspecified
E7141Primary carnitine deficiency
E7142Carnitine deficiency due to inborn errors of metabolism
E7143Iatrogenic carnitine deficiency
E71440Ruvalcaba-Myhre-Smith syndrome
E71448Other secondary carnitine deficiency
E7150Peroxisomal disorder, unspecified
E71510Zellweger syndrome
E71511Neonatal adrenoleukodystrophy
E71518Other disorders of peroxisome biogenesis
E71520Childhood cerebral X-linked adrenoleukodystrophy
E71521Adolescent X-linked adrenoleukodystrophy
E71528Other X-linked adrenoleukodystrophy
E71529X-linked adrenoleukodystrophy, unspecified type
E7153Other group 2 peroxisomal disorders
E71540Rhizomelic chondrodysplasia punctata
E71541Zellweger-like syndrome
E71542Other group 3 peroxisomal disorders
E71548Other peroxisomal disorders
E7200Disorders of amino-acid transport, unspecified
E7202Hartnup's disease
E7203Lowe's syndrome
E7209Other disorders of amino-acid transport
E7210Disorders of sulfur-bearing amino-acid metabolism, unspecified
E7212Methylenetetrahydrofolate reductase deficiency
E7219Other disorders of sulfur-bearing amino-acid metabolism
E7220Disorder of urea cycle metabolism, unspecified
E7222Arginosuccinic aciduria
E7229Other disorders of urea cycle metabolism
E723Disorders of lysine and hydroxylysine metabolism
E724Disorders of ornithine metabolism
E7250Disorder of glycine metabolism, unspecified
E7251Non-ketotic hyperglycinemia
E7259Other disorders of glycine metabolism
E728Other specified disorders of amino-acid metabolism
E729Disorder of amino-acid metabolism, unspecified
E7400Glycogen storage disease, unspecified
E7401von Gierke disease
E7402Pompe disease
E7403Cori disease
E7404McArdle disease
E7409Other glycogen storage disease
E7420Disorders of galactose metabolism, unspecified
E7429Other disorders of galactose metabolism
E744Disorders of pyruvate metabolism and gluconeogenesis
E748Other specified disorders of carbohydrate metabolism
E749Disorder of carbohydrate metabolism, unspecified
E7521Fabry (-Anderson) disease
E7522Gaucher disease
E75240Niemann-Pick disease type A
E75241Niemann-Pick disease type B
E75242Niemann-Pick disease type C
E75243Niemann-Pick disease type D
E75248Other Niemann-Pick disease
E75249Niemann-Pick disease, unspecified
E753Sphingolipidosis, unspecified
E755Other lipid storage disorders
E756Lipid storage disorder, unspecified
E7601Hurler's syndrome
E7602Hurler-Scheie syndrome
E7603Scheie's syndrome
E761Mucopolysaccharidosis, type II
E76210Morquio A mucopolysaccharidoses
E76211Morquio B mucopolysaccharidoses
E76219Morquio mucopolysaccharidoses, unspecified
E7622Sanfilippo mucopolysaccharidoses
E7629Other mucopolysaccharidoses
E763Mucopolysaccharidosis, unspecified
E768Other disorders of glucosaminoglycan metabolism
E769Glucosaminoglycan metabolism disorder, unspecified
E770Defects in post-translational modification of lysosomal enzymes
E771Defects in glycoprotein degradation
E778Other disorders of glycoprotein metabolism
E779Disorder of glycoprotein metabolism, unspecified
E780Pure hypercholesterolemia
E781Pure hyperglyceridemia
E782Mixed hyperlipidemia
E784Other hyperlipidemia
E785Hyperlipidemia, unspecified
E786Lipoprotein deficiency
E7870Disorder of bile acid and cholesterol metabolism, unspecified
E7879Other disorders of bile acid and cholesterol metabolism
E7881Lipoid dermatoarthritis
E7889Other lipoprotein metabolism disorders
E789Disorder of lipoprotein metabolism, unspecified
E791Lesch-Nyhan syndrome
E792Myoadenylate deaminase deficiency
E798Other disorders of purine and pyrimidine metabolism
E799Disorder of purine and pyrimidine metabolism, unspecified
E800Hereditary erythropoietic porphyria
E801Porphyria cutanea tarda
E8020Unspecified porphyria
E8021Acute intermittent (hepatic) porphyria
E8029Other porphyria
E803Defects of catalase and peroxidase
E8300Disorder of copper metabolism, unspecified
E8301Wilson's disease
E8309Other disorders of copper metabolism
E8310Disorder of iron metabolism, unspecified
E83110Hereditary hemochromatosis
E83111Hemochromatosis due to repeated red blood cell transfusions
E83118Other hemochromatosis
E83119Hemochromatosis, unspecified
E8319Other disorders of iron metabolism
E8330Disorder of phosphorus metabolism, unspecified
E8331Familial hypophosphatemia
E8332Hereditary vitamin D-dependent rickets (type 1) (type 2)
E8339Other disorders of phosphorus metabolism
E8389Other disorders of mineral metabolism
E839Disorder of mineral metabolism, unspecified
E8801Alpha-1-antitrypsin deficiency
E881Lipodystrophy, not elsewhere classified
E882Lipomatosis, not elsewhere classified
E8840Mitochondrial metabolism disorder, unspecified
E8841MELAS syndrome
E8842MERRF syndrome
E8849Other mitochondrial metabolism disorders
E8881Metabolic syndrome
E8889Other specified metabolic disorders
E889Metabolic disorder, unspecified
H49811Kearns-Sayre syndrome, right eye
H49812Kearns-Sayre syndrome, left eye
H49813Kearns-Sayre syndrome, bilateral
H49819Kearns-Sayre syndrome, unspecified eye

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