Fatty infiltration in the liver in medium chain acyl CoA dehydrogenase deficiency.
Fatty infiltration of the liver at postmortem examination has been recommended as a criterion for selection of infants who have died suddenly and unexpectedly for further biochemical investigation for disorders of fatty acid oxidation. We describe a boy with medium chain acyl CoA dehydrogenase deficiency who died four months after diagnosis and in whom only minimal hepatic fatty infiltration was found.
Losty
H C
HC
Department of Medical Biochemistry, University Hospital of Wales, Heath Park, Cardiff.
Lee
P
P
Alfaham
M
M
Gray
O P
OP
Leonard
J V
JV
D044944:Acyl-CoA Dehydrogenases / Q000172:deficiency*; D004040:Dietary Carbohydrates / Q000627:therapeutic use; D004827:Epilepsy / Q000209:etiology; D005234:Fatty Liver / Q000473:pathology*; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology*; D008297:Male
D004040:Dietary Carbohydrates; D044944:Acyl-CoA Dehydrogenases