Edit

E71.0
Maple-syrup-urine disease 

E71.1
Other disorders of branched-chain amino-acid metabolism 
Hyperleucine-isoleucinaemia 
Hypervalinaemia 
Isovaleric acidaemia 
Methylmalonic acidaemia 
Propionic acidaemia 

E71.2
Disorder of branched-chain amino-acid metabolism, unspecified 

E71.3
Disorders of fatty-acid metabolism 
Adrenoleukodystrophy [Addison-Schilder] 
Muscle carnitine palmityltransferase deficiency 
Excludes: Refsum's disease (G60.1) 
Schilder's disease (G37.0) 
Zellweger's syndrome (Q87.8) 



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