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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
biochemistry, genetics and molecular biology
Carnitine deficiency and l-carnitine supplementation in lysinuric protein intolerance
Metabolism: Clinical and Experimental, Volume 57, No. 4, Year 2008
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Description
The aim of the study was to investigate the prevalence and mechanisms of development of carnitine deficiency in patients with lysinuric protein intolerance (LPI). In our cohort of 37 Finnish patients with LPI, 8 (8-52 years of age) have been diagnosed with hypocarnitinemia. Their free and total serum carnitine levels, acyl carnitine profiles, renal function, diet, and medication were compared with the data from 8 age- and sex-matched patients with LPI not treated with carnitine supplementation. In patients with LPI, hypocarnitinemia was strongly associated with female sex, renal insufficiency, and the use of ammonia-scavenging drugs. Of the 8 hypocarnitinemic patients, 3 complained of muscle weakness, and their symptoms disappeared during carnitine supplementation. Oral lysine supplementation did not correct hypocarnitinemia in our patients. The patients with LPI are at considerable risk for carnitine deficiency. Supplementation of hypocarnitinemic LPI patients with oral l-carnitine improved serum total carnitine values, but the ratio of free and total carnitine remained subnormal in all supplemented patients except one. Furthermore, decreased ratio of free and total serum carnitine was common even in LPI patients with normal total serum carnitine concentration. © 2008 Elsevier Inc. All rights reserved.
Authors & Co-Authors
Tanner, Laura M.
Finland, Turku
Turun Yliopisto
Näntö-Salonen, Kirsti
Finland, Turku
Turun Yliopisto
Rashed, Mohamed S.
Saudi Arabia, Riyadh
King Faisal Specialist Hospital and Research Centre
Kotilainen, Soile
Finland, Turku
Lääketieteellinen Tiedekunta
Aalto, Maija
Finland, Turku
Turun Yliopistollinen Keskussairaala
Venetoklis, Jaana
Finland, Turku
Turun Yliopistollinen Keskussairaala
Niinikoski, Harri
Finland, Turku
Turun Yliopisto
Huoponen, Kirsi A.
Finland, Turku
Turun Yliopisto
Simell, Olli
Finland, Turku
Turun Yliopisto
Statistics
Citations: 17
Authors: 9
Affiliations: 4
Identifiers
Doi:
10.1016/j.metabol.2007.11.019
ISSN:
00260495
Research Areas
Health System And Policy
Study Design
Cross Sectional Study
Cohort Study
Participants Gender
Female