Block · Chapter 4
E70-E88Metabolic disorders (E70-E88)
366 codes · 17 categories
E70Disorders of aromatic amino-acid metabolism34 codes →
E70Disorders of aromatic amino-acid metabolismHeaderE70.0Classical phenylketonuriaBillableE70.1Other hyperphenylalaninemiasBillableE70.2Disorders of tyrosine metabolismHeaderE70.20Disorder of tyrosine metabolism, unspecifiedBillableE70.21TyrosinemiaBillableE70.29Other disorders of tyrosine metabolismBillableE70.3AlbinismHeaderE70.30Albinism, unspecifiedBillableE70.31Ocular albinismHeaderE70.310X-linked ocular albinismBillableE70.311Autosomal recessive ocular albinismBillableE70.318Other ocular albinismBillableE70.319Ocular albinism, unspecifiedBillableE70.32Oculocutaneous albinismHeaderE70.320Tyrosinase negative oculocutaneous albinismBillableE70.321Tyrosinase positive oculocutaneous albinismBillableE70.328Other oculocutaneous albinismBillableE70.329Oculocutaneous albinism, unspecifiedBillableE70.33Albinism with hematologic abnormalityHeaderE70.330Chediak-Higashi syndromeBillableE70.331Hermansky-Pudlak syndromeBillableE70.338Other albinism with hematologic abnormalityBillableE70.339Albinism with hematologic abnormality, unspecifiedBillableE70.39Other specified albinismBillableE70.4Disorders of histidine metabolismHeaderE70.40Disorders of histidine metabolism, unspecifiedBillableE70.41HistidinemiaBillableE70.49Other disorders of histidine metabolismBillableE70.5Disorders of tryptophan metabolismBillableE70.8Other disorders of aromatic amino-acid metabolismHeaderE70.81Aromatic L-amino acid decarboxylase deficiencyBillableE70.89Other disorders of aromatic amino-acid metabolismBillableE70.9Disorder of aromatic amino-acid metabolism, unspecifiedBillable
E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism50 codes →
E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolismHeaderE71.0Maple-syrup-urine diseaseBillableE71.1Other disorders of branched-chain amino-acid metabolismHeaderE71.11Branched-chain organic aciduriasHeaderE71.110Isovaleric acidemiaBillableE71.1113-methylglutaconic aciduriaBillableE71.118Other branched-chain organic aciduriasBillableE71.12Disorders of propionate metabolismHeaderE71.120Methylmalonic acidemiaBillableE71.121Propionic acidemiaBillableE71.128Other disorders of propionate metabolismBillableE71.19Other disorders of branched-chain amino-acid metabolismBillableE71.2Disorder of branched-chain amino-acid metabolism, unspecifiedBillableE71.3Disorders of fatty-acid metabolismHeaderE71.30Disorder of fatty-acid metabolism, unspecifiedBillableE71.31Disorders of fatty-acid oxidationHeaderE71.310Long chain/very long chain acyl CoA dehydrogenase deficiencyBillableE71.311Medium chain acyl CoA dehydrogenase deficiencyBillableE71.312Short chain acyl CoA dehydrogenase deficiencyBillableE71.313Glutaric aciduria type IIBillableE71.314Muscle carnitine palmitoyltransferase deficiencyBillableE71.318Other disorders of fatty-acid oxidationBillableE71.32Disorders of ketone metabolismBillableE71.39Other disorders of fatty-acid metabolismBillableE71.4Disorders of carnitine metabolismHeaderE71.40Disorder of carnitine metabolism, unspecifiedBillableE71.41Primary carnitine deficiencyBillableE71.42Carnitine deficiency due to inborn errors of metabolismBillableE71.43Iatrogenic carnitine deficiencyBillableE71.44Other secondary carnitine deficiencyHeaderE71.440Ruvalcaba-Myhre-Smith syndromeBillableE71.448Other secondary carnitine deficiencyBillableE71.5Peroxisomal disordersHeaderE71.50Peroxisomal disorder, unspecifiedBillableE71.51Disorders of peroxisome biogenesisHeaderE71.510Zellweger syndromeBillableE71.511Neonatal adrenoleukodystrophyBillableE71.518Other disorders of peroxisome biogenesisBillableE71.52X-linked adrenoleukodystrophyHeaderE71.520Childhood cerebral X-linked adrenoleukodystrophyBillableE71.521Adolescent X-linked adrenoleukodystrophyBillableE71.522AdrenomyeloneuropathyBillableE71.528Other X-linked adrenoleukodystrophyBillableE71.529X-linked adrenoleukodystrophy, unspecified typeBillableE71.53Other group 2 peroxisomal disordersBillableE71.54Other peroxisomal disordersHeaderE71.540Rhizomelic chondrodysplasia punctataBillableE71.541Zellweger-like syndromeBillableE71.542Other group 3 peroxisomal disordersBillableE71.548Other peroxisomal disordersBillable
E72Other disorders of amino-acid metabolism39 codes →
E72Other disorders of amino-acid metabolismHeaderE72.0Disorders of amino-acid transportHeaderE72.00Disorders of amino-acid transport, unspecifiedBillableE72.01CystinuriaBillableE72.02Hartnup's diseaseBillableE72.03Lowe's syndromeBillableE72.04CystinosisBillableE72.09Other disorders of amino-acid transportBillableE72.1Disorders of sulfur-bearing amino-acid metabolismHeaderE72.10Disorders of sulfur-bearing amino-acid metabolism, unspecifiedBillableE72.11HomocystinuriaBillableE72.12Methylenetetrahydrofolate reductase deficiencyBillableE72.19Other disorders of sulfur-bearing amino-acid metabolismBillableE72.2Disorders of urea cycle metabolismHeaderE72.20Disorder of urea cycle metabolism, unspecifiedBillableE72.21ArgininemiaBillableE72.22Arginosuccinic aciduriaBillableE72.23CitrullinemiaBillableE72.29Other disorders of urea cycle metabolismBillableE72.3Disorders of lysine and hydroxylysine metabolismBillableE72.4Disorders of ornithine metabolismBillableE72.5Disorders of glycine metabolismHeaderE72.50Disorder of glycine metabolism, unspecifiedBillableE72.51Non-ketotic hyperglycinemiaBillableE72.52TrimethylaminuriaBillableE72.53Primary hyperoxaluriaHeaderE72.530Primary hyperoxaluria, type 1BillableE72.538Other specified primary hyperoxaluriaBillableE72.539Primary hyperoxaluria, unspecifiedBillableE72.54Secondary hyperoxaluriaHeaderE72.540Dietary hyperoxaluriaBillableE72.541Enteric hyperoxaluriaBillableE72.548Other secondary hyperoxaluriaBillableE72.549Secondary hyperoxaluria, unspecifiedBillableE72.59Other disorders of glycine metabolismBillableE72.8Other specified disorders of amino-acid metabolismHeaderE72.81Disorders of gamma aminobutyric acid metabolismBillableE72.89Other specified disorders of amino-acid metabolismBillableE72.9Disorder of amino-acid metabolism, unspecifiedBillable
E74Other disorders of carbohydrate metabolism32 codes →
E74Other disorders of carbohydrate metabolismHeaderE74.0Glycogen storage diseaseHeaderE74.00Glycogen storage disease, unspecifiedBillableE74.01von Gierke diseaseBillableE74.02Pompe diseaseBillableE74.03Cori diseaseBillableE74.04McArdle diseaseBillableE74.05Lysosome-associated membrane protein 2 [LAMP2] deficiencyBillableE74.09Other glycogen storage diseaseBillableE74.1Disorders of fructose metabolismHeaderE74.10Disorder of fructose metabolism, unspecifiedBillableE74.11Essential fructosuriaBillableE74.12Hereditary fructose intoleranceBillableE74.19Other disorders of fructose metabolismBillableE74.2Disorders of galactose metabolismHeaderE74.20Disorders of galactose metabolism, unspecifiedBillableE74.21GalactosemiaBillableE74.29Other disorders of galactose metabolismBillableE74.3Other disorders of intestinal carbohydrate absorptionHeaderE74.31Sucrase-isomaltase deficiencyBillableE74.39Other disorders of intestinal carbohydrate absorptionBillableE74.4Disorders of pyruvate metabolism and gluconeogenesisBillableE74.8Other specified disorders of carbohydrate metabolismHeaderE74.81Disorders of glucose transport, not elsewhere classifiedHeaderE74.810Glucose transporter protein type 1 deficiencyBillableE74.818Other disorders of glucose transportBillableE74.819Disorders of glucose transport, unspecifiedBillableE74.82Disorders of citrate metabolismHeaderE74.820SLC13A5 Citrate Transporter DisorderBillableE74.829Other disorders of citrate metabolismBillableE74.89Other specified disorders of carbohydrate metabolismBillableE74.9Disorder of carbohydrate metabolism, unspecifiedBillable
E75Disorders of sphingolipid metabolism and other lipid storage disorders31 codes →
E75Disorders of sphingolipid metabolism and other lipid storage disordersHeaderE75.0GM2 gangliosidosisHeaderE75.00GM2 gangliosidosis, unspecifiedBillableE75.01Sandhoff diseaseBillableE75.02Tay-Sachs diseaseBillableE75.09Other GM2 gangliosidosisBillableE75.1Other and unspecified gangliosidosisHeaderE75.10Unspecified gangliosidosisBillableE75.11Mucolipidosis IVBillableE75.19Other gangliosidosisBillableE75.2Other sphingolipidosisHeaderE75.21Fabry (-Anderson) diseaseBillableE75.22Gaucher diseaseBillableE75.23Krabbe diseaseBillableE75.24Niemann-Pick diseaseHeaderE75.240Niemann-Pick disease type ABillableE75.241Niemann-Pick disease type BBillableE75.242Niemann-Pick disease type CBillableE75.243Niemann-Pick disease type DBillableE75.244Niemann-Pick disease type A/BBillableE75.248Other Niemann-Pick diseaseBillableE75.249Niemann-Pick disease, unspecifiedBillableE75.25Metachromatic leukodystrophyBillableE75.26Sulfatase deficiencyBillableE75.27Pelizaeus-Merzbacher diseaseBillableE75.28Canavan diseaseBillableE75.29Other sphingolipidosisBillableE75.3Sphingolipidosis, unspecifiedBillableE75.4Neuronal ceroid lipofuscinosisBillableE75.5Other lipid storage disordersBillableE75.6Lipid storage disorder, unspecifiedBillable
E76Disorders of glycosaminoglycan metabolism16 codes →
E76Disorders of glycosaminoglycan metabolismHeaderE76.0Mucopolysaccharidosis, type IHeaderE76.01Hurler's syndromeBillableE76.02Hurler-Scheie syndromeBillableE76.03Scheie's syndromeBillableE76.1Mucopolysaccharidosis, type IIBillableE76.2Other mucopolysaccharidosesHeaderE76.21Morquio mucopolysaccharidosesHeaderE76.210Morquio A mucopolysaccharidosesBillableE76.211Morquio B mucopolysaccharidosesBillableE76.219Morquio mucopolysaccharidoses, unspecifiedBillableE76.22Sanfilippo mucopolysaccharidosesBillableE76.29Other mucopolysaccharidosesBillableE76.3Mucopolysaccharidosis, unspecifiedBillableE76.8Other disorders of glucosaminoglycan metabolismBillableE76.9Glucosaminoglycan metabolism disorder, unspecifiedBillable
E78Disorders of lipoprotein metabolism and other lipidemias24 codes →
E78Disorders of lipoprotein metabolism and other lipidemiasHeaderE78.0Pure hypercholesterolemiaHeaderE78.00Pure hypercholesterolemia, unspecifiedBillableE78.01Familial hypercholesterolemiaHeaderE78.010Homozygous familial hypercholesterolemia [HoFH]BillableE78.011Heterozygous familial hypercholesterolemia [HeFH]BillableE78.019Familial hypercholesterolemia, unspecifiedBillableE78.1Pure hyperglyceridemiaBillableE78.2Mixed hyperlipidemiaBillableE78.3HyperchylomicronemiaBillableE78.4Other hyperlipidemiaHeaderE78.41Elevated Lipoprotein(a)BillableE78.49Other hyperlipidemiaBillableE78.5Hyperlipidemia, unspecifiedBillableE78.6Lipoprotein deficiencyBillableE78.7Disorders of bile acid and cholesterol metabolismHeaderE78.70Disorder of bile acid and cholesterol metabolism, unspecifiedBillableE78.71Barth syndromeBillableE78.72Smith-Lemli-Opitz syndromeBillableE78.79Other disorders of bile acid and cholesterol metabolismBillableE78.8Other disorders of lipoprotein metabolismHeaderE78.81Lipoid dermatoarthritisBillableE78.89Other lipoprotein metabolism disordersBillableE78.9Disorder of lipoprotein metabolism, unspecifiedBillable
E79Disorders of purine and pyrimidine metabolism9 codes →
E79Disorders of purine and pyrimidine metabolismHeaderE79.0Hyperuricemia without signs of inflammatory arthritis and tophaceous diseaseBillableE79.1Lesch-Nyhan syndromeBillableE79.2Myoadenylate deaminase deficiencyBillableE79.8Other disorders of purine and pyrimidine metabolismHeaderE79.81Aicardi-Goutières syndromeBillableE79.82Hereditary xanthinuriaBillableE79.89Other specified disorders of purine and pyrimidine metabolismBillableE79.9Disorder of purine and pyrimidine metabolism, unspecifiedBillable
E80Disorders of porphyrin and bilirubin metabolism12 codes →
E80Disorders of porphyrin and bilirubin metabolismHeaderE80.0Hereditary erythropoietic porphyriaBillableE80.1Porphyria cutanea tardaBillableE80.2Other and unspecified porphyriaHeaderE80.20Unspecified porphyriaBillableE80.21Acute intermittent (hepatic) porphyriaBillableE80.29Other porphyriaBillableE80.3Defects of catalase and peroxidaseBillableE80.4Gilbert syndromeBillableE80.5Crigler-Najjar syndromeBillableE80.6Other disorders of bilirubin metabolismBillableE80.7Disorder of bilirubin metabolism, unspecifiedBillable
E83Disorders of mineral metabolism40 codes →
E83Disorders of mineral metabolismHeaderE83.0Disorders of copper metabolismHeaderE83.00Disorder of copper metabolism, unspecifiedBillableE83.01Wilson's diseaseBillableE83.09Other disorders of copper metabolismBillableE83.1Disorders of iron metabolismHeaderE83.10Disorder of iron metabolism, unspecifiedBillableE83.11HemochromatosisHeaderE83.110Hereditary hemochromatosisBillableE83.111Hemochromatosis due to repeated red blood cell transfusionsBillableE83.118Other hemochromatosisBillableE83.119Hemochromatosis, unspecifiedBillableE83.19Other disorders of iron metabolismBillableE83.2Disorders of zinc metabolismBillableE83.3Disorders of phosphorus metabolism and phosphatasesHeaderE83.30Disorder of phosphorus metabolism, unspecifiedBillableE83.31Familial hypophosphatemiaBillableE83.32Hereditary vitamin D-dependent rickets (type 1) (type 2)BillableE83.39Other disorders of phosphorus metabolismBillableE83.4Disorders of magnesium metabolismHeaderE83.40Disorders of magnesium metabolism, unspecifiedBillableE83.41HypermagnesemiaBillableE83.42HypomagnesemiaBillableE83.49Other disorders of magnesium metabolismBillableE83.5Disorders of calcium metabolismHeaderE83.50Unspecified disorder of calcium metabolismBillableE83.51HypocalcemiaBillableE83.52HypercalcemiaBillableE83.59Other disorders of calcium metabolismBillableE83.8Other disorders of mineral metabolismHeaderE83.81Hungry bone syndromeBillableE83.82Disorders of pyrophosphate metabolismHeaderE83.820Generalized arterial calcification of infancy with unspecified genetic causalityBillableE83.821ENPP1 deficiency causing generalized arterial calcification of infancyBillableE83.822ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2BillableE83.823ABCC6 deficiency causing generalized arterial calcification of infancyBillableE83.824ABCC6 deficiency causing pseudoxanthoma elasticumBillableE83.825CD73 deficiency causing arterial calcificationBillableE83.89Other disorders of mineral metabolismBillableE83.9Disorder of mineral metabolism, unspecifiedBillable
E84Cystic fibrosis7 codes →
E84Cystic fibrosisHeaderE84.0Cystic fibrosis with pulmonary manifestationsBillableE84.1Cystic fibrosis with intestinal manifestationsHeaderE84.11Meconium ileus in cystic fibrosisBillableE84.19Cystic fibrosis with other intestinal manifestationsBillableE84.8Cystic fibrosis with other manifestationsBillableE84.9Cystic fibrosis, unspecifiedBillable
E85Amyloidosis11 codes →
E85AmyloidosisHeaderE85.0Non-neuropathic heredofamilial amyloidosisBillableE85.1Neuropathic heredofamilial amyloidosisBillableE85.2Heredofamilial amyloidosis, unspecifiedBillableE85.3Secondary systemic amyloidosisBillableE85.4Organ-limited amyloidosisBillableE85.8Other amyloidosisHeaderE85.81Light chain (AL) amyloidosisBillableE85.82Wild-type transthyretin-related (ATTR) amyloidosisBillableE85.89Other amyloidosisBillableE85.9Amyloidosis, unspecifiedBillable
E87Other disorders of fluid, electrolyte and acid-base balance17 codes →
E87Other disorders of fluid, electrolyte and acid-base balanceHeaderE87.0Hyperosmolality and hypernatremiaBillableE87.1Hypo-osmolality and hyponatremiaBillableE87.2AcidosisHeaderE87.20Acidosis, unspecifiedBillableE87.21Acute metabolic acidosisBillableE87.22Chronic metabolic acidosisBillableE87.29Other acidosisBillableE87.3AlkalosisBillableE87.4Mixed disorder of acid-base balanceBillableE87.5HyperkalemiaBillableE87.6HypokalemiaBillableE87.7Fluid overloadHeaderE87.70Fluid overload, unspecifiedBillableE87.71Transfusion associated circulatory overloadBillableE87.79Other fluid overloadBillableE87.8Other disorders of electrolyte and fluid balance, not elsewhere classifiedBillable
E88Other and unspecified metabolic disorders30 codes →
E88Other and unspecified metabolic disordersHeaderE88.0Disorders of plasma-protein metabolism, not elsewhere classifiedHeaderE88.01Alpha-1-antitrypsin deficiencyBillableE88.02Plasminogen deficiencyBillableE88.09Other disorders of plasma-protein metabolism, not elsewhere classifiedBillableE88.1Lipodystrophy, not elsewhere classifiedHeaderE88.10Lipodystrophy, unspecifiedBillableE88.11Partial lipodystrophyBillableE88.12Generalized lipodystrophyBillableE88.13Localized lipodystrophyBillableE88.14HIV-associated lipodystrophyBillableE88.19Other lipodystrophy, not elsewhere classifiedBillableE88.2Lipomatosis, not elsewhere classifiedBillableE88.3Tumor lysis syndromeBillableE88.4Mitochondrial metabolism disordersHeaderE88.40Mitochondrial metabolism disorder, unspecifiedBillableE88.41MELAS syndromeBillableE88.42MERRF syndromeBillableE88.43Disorders of mitochondrial tRNA synthetasesBillableE88.49Other mitochondrial metabolism disordersBillableE88.8Other specified metabolic disordersHeaderE88.81Metabolic syndrome and other insulin resistanceHeaderE88.810Metabolic syndromeBillableE88.811Insulin resistance syndrome, Type ABillableE88.818Other insulin resistanceBillableE88.819Insulin resistance, unspecifiedBillableE88.82Obesity due to disruption of MC4R pathwayBillableE88.89Other specified metabolic disordersBillableE88.9Metabolic disorder, unspecifiedBillableE88.AWasting disease (syndrome) due to underlying conditionBillable