| Literature DB >> 10472533 |
R Parini1, F Invernizzi, F Menni, B Garavaglia, D Melotti, M Rimoldi, S Salera, C Tosetto, F Taroni.
Abstract
The results of a medium-chain triglyceride loading test in a patient with severe carnitine-acylcarnitine translocase deficiency clearly demonstrated impaired in vivo utilization of medium-chain triglycerides. The loading test was performed at the ages of 7 and 36 months. The diet was adjusted accordingly. The clinical course has been favourable and the child is now in very good condition at age 4 years. We conclude that the utilization of medium-chain triglycerides is only partial in carnitine-acylcarnitine translocase deficiency and cannot reasonably be considered an optimal source of energy for these patients. Careful adjustment of dietetic treatment may help to improve prognosis.Entities:
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Year: 1999 PMID： 10472533 DOI： 10.1023/a:1005548201355
Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982