Very‐long‐chain acyl‐coenzyme A dehydrogenase (VLCAD) deficiency: monitoring of treatment by carnitine/acylcarnitine analysis in blood spots

2000 ◽  
Vol 89 (4) ◽  
pp. 492-495 ◽  
Author(s):  
U Spiekerkötter ◽  
B Schwahn ◽  
H Korall ◽  
FK Trefz ◽  
BS Andresen ◽  
...  
Keyword(s):  
Coenzyme A ◽  
Blood Spots ◽  
Chain Acyl ◽  
Acylcarnitine Analysis ◽  
Acyl Coenzyme A ◽  
Vlcad Deficiency ◽  
Long Chain

Diagnostic assessment and long-term follow-up of 13 patients with Very Long-Chain Acyl-Coenzyme A dehydrogenase (VLCAD) deficiency

2009 ◽  
Vol 19 (5) ◽  
pp. 324-329 ◽  
Author(s):  
Pascal Laforêt ◽  
Cécile Acquaviva-Bourdain ◽  
Odile Rigal ◽  
Michèle Brivet ◽  
Isabelle Penisson-Besnier ◽  
...  
Keyword(s):  
Coenzyme A ◽  
Diagnostic Assessment ◽  
Chain Acyl ◽  
Long Term Follow Up ◽  
Acyl Coenzyme A ◽  
Vlcad Deficiency ◽  
Long Chain

scholarly journals Anesthesia management in a patient with very long-chain acyl-Coenzyme A dehydrogenase deficiency

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Haruyuki Yuasa ◽  
Yukio Onoda ◽  
Atsuhiro Kitaura ◽  
Takashi Mino ◽  
Shota Tsukimoto ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Body Temperature ◽  
Alternative Energy ◽  
Coenzyme A ◽  
Case Presentation ◽  
Energy Needs ◽  
Chain Acyl ◽  
Acyl Coenzyme A ◽  
Vlcad Deficiency ◽  
Long Chain

Abstract Background In a patient with very long-chain acyl-Coenzyme A dehydrogenase (VLCAD) deficiency, metabolism of fatty acids is impaired and a supply of alternative energy is limited when glucose level is insufficient on starvation. Case presentation A 37-year-old woman with VLCAD deficiency was diagnosed with an ovarian cyst and was scheduled for laparoscopic ovarian cystectomy. Glucose was administered intravenously with the start of fasting. Anesthesia was induced with remifentanil, midazolam, and thiamylal, maintained with desflurane and remifentanil. Body temperature was maintained at 36.2–36.7 °C. During anesthesia, hypoglycemia did not occur, creatine kinase levels were in the normal range, and myoglobinuria was not detected. No shivering was observed after extubation. Conclusions Glucose was administered to avoid perioperative hypoglycemia. Body temperature was controlled to avoid shivering, which would otherwise increase skeletal muscle energy needs. Blood creatine kinase did not increase, and myoglobinuria was not detected; thus, rhabdomyolysis was unlikely to develop.

scholarly journals Mechanism of the control of (Na+ + K+)-ATPase by long-chain acyl coenzyme A

1989 ◽  
Vol 264 (5) ◽  
pp. 2605-2608
Author(s):  
W H Huang ◽  
Y Wang ◽  
A Askari
Keyword(s):  
Coenzyme A ◽  
Chain Acyl ◽  
Acyl Coenzyme A ◽  
Long Chain

ANALYSIS OF LONG-CHAIN ACYL-COENZYME A ESTERS

2012 ◽  
pp. 109-131 ◽  
Author(s):  
Tine Bækdal ◽  
Charlotte Karlskov Schjerling ◽  
Jan Krogh Hansen ◽  
Jens Knudsen
Keyword(s):  
Coenzyme A ◽  
Chain Acyl ◽  
Acyl Coenzyme A ◽  
Coenzyme A Esters ◽  
Long Chain

Very long-chain acyl coenzyme A dehydrogenase deficiency presenting with exercise-induced myoglobinuria

Neurology ◽  
1994 ◽  
Vol 44 (3, Part 1) ◽  
pp. 467-467 ◽  
Author(s):  
I. Ogilvie ◽  
M. Pourfarzam ◽  
S. Jackson ◽  
C. Stockdale ◽  
K. Bartlett ◽  
...  
Keyword(s):  
Dehydrogenase Deficiency ◽  
Coenzyme A ◽  
Chain Acyl ◽  
Exercise Induced ◽  
Acyl Coenzyme A ◽  
Long Chain
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