Partial muscle carnitine palmitoyltransferase-A deficiency
After initiation of ibuprofen therapy, a 45-year-old woman developed muscle weakness and tenderness with rhabdomyolysis, culminating in respiratory failure. A muscle biopsy specimen showed a vacuolar myopathy, and markedly decreased muscle carnitine content and carnitine palmitoyltransferase activity. Following recovery, muscle carnitine content was normal but carnitine palmitoyltransferase activity was still abnormally low. The ratio of palmitoyl-coenzyme A plus carnitine to palmitoylcarnitine oxidation by muscle mitochondria isolated from the patient was markedly decreased. The authors conclude that transiently decreased muscle carnitine content interacted with partial deficiency of carnitine palmitoyltransferase-A to produce rhabdomyolysis and respiratory failure and that ibuprofen may have precipitated the clinical event.
- Research Organization:
- Veterans Administration Medical Center, Cleveland, OH
- OSTI ID:
- 6796553
- Journal Information:
- JAMA, J. Am. Med. Assoc.; (United States), Vol. 257:1
- Country of Publication:
- United States
- Language:
- English
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CARNITINE
BIOCHEMISTRY
MUSCLES
ENZYME ACTIVITY
TRANSFERASES
RADIOASSAY
CARBON 14 COMPOUNDS
CHEMOTHERAPY
METABOLIC DISEASES
OXYGEN
PATIENTS
RESPIRATION
WOMEN
AMINO ACIDS
ANIMALS
CARBOXYLIC ACIDS
CHEMISTRY
DISEASES
ELEMENTS
ENZYMES
FEMALES
HYDROXY ACIDS
LABELLED COMPOUNDS
MAMMALS
MAN
NONMETALS
ORGANIC ACIDS
ORGANIC COMPOUNDS
PRIMATES
THERAPY
VERTEBRATES
VITAMIN B GROUP
VITAMINS
550601* - Medicine- Unsealed Radionuclides in Diagnostics