E71 |
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Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism  |
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E71.0 |
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Maple-syrup-urine disease |
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E71.1 |
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Other disorders of branched-chain amino-acid metabolism |
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E71.11 |
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Branched-chain organic acidurias |
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E71.110 |
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Isovaleric acidemia |
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E71.111 |
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3-methylglutaconic aciduria |
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E71.118 |
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Other branched-chain organic acidurias |
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E71.12 |
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Disorders of propionate metabolism |
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E71.120 |
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Methylmalonic acidemia |
| | |
E71.121 |
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Propionic acidemia |
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E71.128 |
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Other disorders of propionate metabolism |
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E71.19 |
 |
Other disorders of branched-chain amino-acid metabolism |
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Hyperleucine-isoleucinemia | Hypervalinemia |
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| |
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E71.2 |
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Disorder of branched-chain amino-acid metabolism, unspecified |
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E71.3 |
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Disorders of fatty-acid metabolism |
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Excludes1 |
peroxisomal disorders (E71.5) Refsum's disease (G60.1) Schilder's disease (G37.0)
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Excludes2 |
carnitine deficiency due to inborn error of metabolism (E71.42)
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|
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E71.30 |
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Disorder of fatty-acid metabolism, unspecified |
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E71.31 |
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Disorders of fatty-acid oxidation |
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E71.310 |
 |
Long chain/very long chain acyl CoA dehydrogenase deficiency |
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LCAD deficiency | VLCAD deficiency |
|
| | |
E71.311 |
 |
Medium chain acyl CoA dehydrogenase deficiency |
| |
|
| | |
E71.312 |
 |
Short chain acyl CoA dehydrogenase deficiency |
| |
|
| | |
E71.313 |
 |
Glutaric aciduria type II |
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Glutaric aciduria type II A | Glutaric aciduria type II B | Glutaric aciduria type II C |
Excludes1 |
glutaric aciduria (type 1) NOS (E72.3)
|
|
| | |
E71.314 |
|
Muscle carnitine palmitoyltransferase deficiency |
| | |
E71.318 |
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Other disorders of fatty-acid oxidation |
| | | |
E71.32 |
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Disorders of ketone metabolism |
| | |
E71.39 |
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Other disorders of fatty-acid metabolism |
| |
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E71.4 |
 |
Disorders of carnitine metabolism |
| |
Excludes1 |
Muscle carnitine palmitoyltransferase deficiency (E71.314)
|
|
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E71.40 |
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Disorder of carnitine metabolism, unspecified |
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E71.41 |
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Primary carnitine deficiency |
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E71.42 |
 |
Carnitine deficiency due to inborn errors of metabolism |
| |
CodeAlso |
associated inborn error or metabolism
|
|
| | |
E71.43 |
 |
Iatrogenic carnitine deficiency |
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Carnitine deficiency due to hemodialysis | Carnitine deficiency due to Valproic acid therapy |
|
| | |
E71.44 |
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Other secondary carnitine deficiency |
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E71.440 |
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Ruvalcaba-Myhre-Smith syndrome |
| | |
E71.448 |
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Other secondary carnitine deficiency |
| | |
| |
E71.5 |
 |
Peroxisomal disorders |
| |
Excludes1 |
Schilder's disease (G37.0)
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|
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E71.50 |
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Peroxisomal disorder, unspecified |
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E71.51 |
 |
Disorders of peroxisome biogenesis |
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Group 1 peroxisomal disorders |
Excludes1 |
Refsum's disease (G60.1)
|
|
| |
E71.510 |
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Zellweger syndrome |
| | |
E71.511 |
 |
Neonatal adrenoleukodystrophy |
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Excludes1 |
X-linked adrenoleukodystrophy (E71.42-)
|
|
| | |
E71.518 |
|
Other disorders of peroxisome biogenesis |
| | | |
E71.52 |
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X-linked adrenoleukodystrophy |
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E71.520 |
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Childhood cerebral X-linked adrenoleukodystrophy |
| | |
E71.521 |
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Adolescent X-linked adrenoleukodystrophy |
| | |
E71.522 |
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Adrenomyeloneuropathy |
| | |
E71.528 |
 |
Other X-linked adrenoleukodystrophy |
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Addison only phenotype adrenoleukodystrophy | Addison-Schilder adrenoleukodystrophy |
|
| | |
E71.529 |
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X-linked adrenoleukodystrophy, unspecified type |
| | | |
E71.53 |
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Other group 2 peroxisomal disorders |
| | |
E71.54 |
|
Other peroxisomal disorders |
| |
E71.540 |
 |
Rhizomelic chondrodysplasia punctata |
| |
Excludes1 |
chondrodysplasia punctata NOS (Q77.3)
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|
| | |
E71.541 |
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Zellweger-like syndrome |
| | |
E71.542 |
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Other group 3 peroxisomal disorders |
| | |
E71.548 |
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Other peroxisomal disorders |
| | |