Advanced Search
Korean J Pediatr 2012 February;55(2) :58-62.
Transient carnitine transport defect with cholestatic jaundice: report of one case in a premature baby
Hyun-Seok Cho (Cho HS)1, Young Kwang Choo (Choo YK)1, Hong Jin Lee (Lee HJ)2, hyeon-Soo Lee (Lee hS)
Department of Pediatrics, Kangwon National University School of Medicine, Chuncheon, Korea
1Department of Pediatrics, Kangwon National University Hospital, Chuncheon, Korea
2Department of Pediatrics, Hallym University College of Medicine, Chuncheon, Korea
Corresponding Author: hyeon-Soo Lee ,Tel: +82-033-258-2318, Fax: +82-33-258-2418, Email:
Copyright © 2012 by The Korean Pediatric Society
Carnitine (-hydroxy--trimethylaminobutyric acid) is involved in the transport of long-chain fatty acids into the mitochondrial matrix and the removal of potentially toxic acylcarnitine esters. Transient carnitine transport defect is a rare condition in newborns reported in 1/90,000 live births. In this paper, we describe a case of transient carnitine transport defect found in a premature baby who had prolonged cholestatic jaundice and poor weight gain, and who responded dramatically to oral carnitine supplementation.
Keywords: Transient carnitine transport defect | Cholestatic jaundice | Poor weight gain | Prematurity
PDF Links  PDF Links
Full text via DOI  Full text via DOI
  via Pubmed
Full text via PMC  Full text via PMC
via Pubreader  via PubReader
Download Citation  Download Citation
Supplementary Material  Supplementary Material
Register for e-submission
Register here to access the e-submission system of Korean J Pediatr for authors and reviewers.
Manuscript Submission
To submit a manuscript, please visit the Korean J Pediatr e-submission management system at, read the Instructions for Authors, and log into the Korean J Pediatr e-submission system. For assistance with manuscript submission, please contact:
Free archive
Anyone may access any past or current articles without logging in.
Korean Pediatric Society Office
#1606, Seocho World Officetel, 19 Seoun-ro, Seocho-gu, Seoul 137-070, Korea
TEL : +82-2-3473-7305    FAX : +82-2-3473-7307   E-mail:
BrowseCurrent IssueFor Authors and ReviewersAbout
Copyright© The Korean Pediatric Society. All right reserved.