Partial muscle carnitine palmitoyltransferase-A deficiency
Journal Article
·
· JAMA, J. Am. Med. Assoc.; (United States)
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), Journal Name: JAMA, J. Am. Med. Assoc.; (United States) Vol. 257:1; ISSN JAMAA
- Country of Publication:
- United States
- Language:
- English
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Related Subjects
550601* -- Medicine-- Unsealed Radionuclides in Diagnostics
62 RADIOLOGY AND NUCLEAR MEDICINE
AMINO ACIDS
ANIMALS
BIOCHEMISTRY
CARBON 14 COMPOUNDS
CARBOXYLIC ACIDS
CARNITINE
CHEMISTRY
CHEMOTHERAPY
DISEASES
ELEMENTS
ENZYME ACTIVITY
ENZYMES
FEMALES
HYDROXY ACIDS
LABELLED COMPOUNDS
MAMMALS
MAN
METABOLIC DISEASES
MUSCLES
NONMETALS
ORGANIC ACIDS
ORGANIC COMPOUNDS
OXYGEN
PATIENTS
PRIMATES
RADIOASSAY
RESPIRATION
THERAPY
TRANSFERASES
VERTEBRATES
VITAMIN B GROUP
VITAMINS
WOMEN
62 RADIOLOGY AND NUCLEAR MEDICINE
AMINO ACIDS
ANIMALS
BIOCHEMISTRY
CARBON 14 COMPOUNDS
CARBOXYLIC ACIDS
CARNITINE
CHEMISTRY
CHEMOTHERAPY
DISEASES
ELEMENTS
ENZYME ACTIVITY
ENZYMES
FEMALES
HYDROXY ACIDS
LABELLED COMPOUNDS
MAMMALS
MAN
METABOLIC DISEASES
MUSCLES
NONMETALS
ORGANIC ACIDS
ORGANIC COMPOUNDS
OXYGEN
PATIENTS
PRIMATES
RADIOASSAY
RESPIRATION
THERAPY
TRANSFERASES
VERTEBRATES
VITAMIN B GROUP
VITAMINS
WOMEN