Open AccessPostoperative Coma in a Child with Carnitine Palmitoyltransferase I Deficiency
Author(s) -
Pekka T. Neuvonen,
A. A. Van Den Berg
Publication year - 2001
Publication title -
anesthesia and analgesia/anesthesia and analgesia
Language(s) - English
Resource type - Journals
eISSN - 1526-7598
pISSN - 0003-2999
DOI - 10.1097/00000539-200103000-00017
Subject(s) - medicine , carnitine , carnitine o palmitoyltransferase , sickle cell trait , encephalopathy , coma (optics) , endocrinology , rhabdomyolysis , beta oxidation , metabolism , disease , physics , optics
We describe prolonged drowsiness proceeding to hepatic encephalopathy in a child with sickle cell trait and carnitine palmitoyltransferase deficiency. The latter defect of fatty acid metabolism is associated with striated muscle dysfunction and hepatic failure.