Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

Maple-syrup-urine disease

E71.0

Other disorders of branched-chain amino-acid metabolism

E71.1

Branched-chain organic acidurias

E71.11

Isovaleric acidemia

E71.110

3-methylglutaconic aciduria

E71.111

Other branched-chain organic acidurias

E71.118

Disorders of propionate metabolism

E71.12

Methylmalonic acidemia

E71.120

Propionic acidemia

E71.121

Other disorders of propionate metabolism

E71.128

Other disorders of branched-chain amino-acid metabolism

E71.19

Disorder of branched-chain amino-acid metabolism, unspecified

E71.2

Disorders of fatty-acid metabolism

E71.3

Disorder of fatty-acid metabolism, unspecified

E71.30

Disorders of fatty-acid oxidation

E71.31

Long chain/very long chain acyl CoA dehydrogenase deficiency

E71.310

Medium chain acyl CoA dehydrogenase deficiency

E71.311

Short chain acyl CoA dehydrogenase deficiency

E71.312

Glutaric aciduria type II

E71.313

Muscle carnitine palmitoyltransferase deficiency

E71.314

Other disorders of fatty-acid oxidation

E71.318

Disorders of ketone metabolism

E71.32

Other disorders of fatty-acid metabolism

E71.39

Disorders of carnitine metabolism

E71.4

Disorder of carnitine metabolism, unspecified

E71.40

Primary carnitine deficiency

E71.41

Carnitine deficiency due to inborn errors of metabolism

E71.42

Iatrogenic carnitine deficiency

E71.43

Other secondary carnitine deficiency

E71.44

Ruvalcaba-Myhre-Smith syndrome

E71.440

Other secondary carnitine deficiency

E71.448

Peroxisomal disorders

E71.5

Peroxisomal disorder, unspecified

E71.50

Disorders of peroxisome biogenesis

E71.51

Zellweger syndrome

E71.510

Neonatal adrenoleukodystrophy

E71.511

Other disorders of peroxisome biogenesis

E71.518

X-linked adrenoleukodystrophy

E71.52

Childhood cerebral X-linked adrenoleukodystrophy

E71.520

Adolescent X-linked adrenoleukodystrophy

E71.521

Adrenomyeloneuropathy

E71.522

Other X-linked adrenoleukodystrophy

E71.528

X-linked adrenoleukodystrophy, unspecified type

E71.529

Other group 2 peroxisomal disorders

E71.53

Other peroxisomal disorders

E71.54

Rhizomelic chondrodysplasia punctata

E71.540

Zellweger-like syndrome

E71.541

Other group 3 peroxisomal disorders

E71.542

Other peroxisomal disorders

E71.548