| ICD-10-CM-2023 > E00-E89 > E70-E88 > E71 |
E70-E88 | Metabolic disorders | |
Excl1.: | androgen insensitivity syndrome (E34.5-) congenital adrenal hyperplasia (E25.0) hemolytic anemias attributable to enzyme disorders (D55.-) Marfan's syndrome (Q87.4) 5-alpha-reductase deficiency (E29.1) | |
Excl2.: | Ehlers-Danlos syndromes (Q79.6-) | |
E71 | Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism | |
E71.0 | Maple-syrup-urine disease | |
E71.1 | Other disorders of branched-chain amino-acid metabolism | |
E71.11 | Branched-chain organic acidurias | |
E71.110 | Isovaleric acidemia | |
E71.111 | 3-methylglutaconic aciduria | |
E71.118 | Other branched-chain organic acidurias | |
E71.12 | Disorders of propionate metabolism | |
E71.120 | Methylmalonic acidemia | |
E71.121 | Propionic acidemia | |
E71.128 | Other disorders of propionate metabolism | |
E71.19 | Other disorders of branched-chain amino-acid metabolism | |
Info: | Hyperleucine-isoleucinemia Hypervalinemia | |
E71.2 | Disorder of branched-chain amino-acid metabolism, unspecified | |
E71.3 | Disorders of fatty-acid metabolism | |
Excl1.: | peroxisomal disorders (E71.5) Refsum's disease (G60.1) Schilder's disease (G37.0) | |
Excl2.: | carnitine deficiency due to inborn error of metabolism (E71.42) | |
E71.30 | Disorder of fatty-acid metabolism, unspecified | |
E71.31 | Disorders of fatty-acid oxidation | |
E71.310 | Long chain/very long chain acyl CoA dehydrogenase deficiency | |
Info: | LCAD VLCAD | |
E71.311 | Medium chain acyl CoA dehydrogenase deficiency | |
Info: | MCAD | |
E71.312 | Short chain acyl CoA dehydrogenase deficiency | |
Info: | SCAD | |
E71.313 | Glutaric aciduria type II | |
Info: | Glutaric aciduria type II A Glutaric aciduria type II B Glutaric aciduria type II C | |
Excl1.: | glutaric aciduria (type 1) NOS (E72.3) | |
E71.314 | Muscle carnitine palmitoyltransferase deficiency | |
E71.318 | Other disorders of fatty-acid oxidation | |
E71.32 | Disorders of ketone metabolism | |
E71.39 | Other disorders of fatty-acid metabolism | |
E71.4 | Disorders of carnitine metabolism | |
Excl1.: | Muscle carnitine palmitoyltransferase deficiency (E71.314) | |
E71.40 | Disorder of carnitine metabolism, unspecified | |
E71.41 | Primary carnitine deficiency | |
E71.42 | Carnitine deficiency due to inborn errors of metabolism | |
Use add'l | associated inborn error or metabolism | |
E71.43 | Iatrogenic carnitine deficiency | |
Info: | Carnitine deficiency due to hemodialysis Carnitine deficiency due to Valproic acid therapy | |
E71.44 | Other secondary carnitine deficiency | |
E71.440 | Ruvalcaba-Myhre-Smith syndrome | |
E71.448 | Other secondary carnitine deficiency | |
E71.5 | Peroxisomal disorders | |
Excl1.: | Schilder's disease (G37.0) | |
E71.50 | Peroxisomal disorder, unspecified | |
E71.51 | Disorders of peroxisome biogenesis | |
Info: | Group 1 peroxisomal disorders | |
Excl1.: | Refsum's disease (G60.1) | |
E71.510 | Zellweger syndrome | |
E71.511 | Neonatal adrenoleukodystrophy | |
Excl1.: | X-linked adrenoleukodystrophy (E71.42-) | |
E71.518 | Other disorders of peroxisome biogenesis | |
E71.52 | X-linked adrenoleukodystrophy | |
E71.520 | Childhood cerebral X-linked adrenoleukodystrophy | |
E71.521 | Adolescent X-linked adrenoleukodystrophy | |
E71.522 | Adrenomyeloneuropathy | |
E71.528 | Other X-linked adrenoleukodystrophy | |
Info: | Addison only phenotype adrenoleukodystrophy Addison-Schilder adrenoleukodystrophy | |
E71.529 | X-linked adrenoleukodystrophy, unspecified type | |
E71.53 | Other group 2 peroxisomal disorders | |
E71.54 | Other peroxisomal disorders | |
E71.540 | Rhizomelic chondrodysplasia punctata | |
Excl1.: | chondrodysplasia punctata NOS (Q77.3) | |
E71.541 | Zellweger-like syndrome | |
E71.542 | Other group 3 peroxisomal disorders | |
E71.548 | Other peroxisomal disorders | |
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