Document Detail

Massive hepatomegaly, steatosis, and secondary plasma carnitine deficiency in an infant with cystic fibrosis.
MedLine Citation:
PMID:  2726354     Owner:  NLM     Status:  MEDLINE    
Hepatomegaly and steatosis are common findings in children with cystic fibrosis and are most often attributed to malnutrition. An infant fed a carnitine-free soy formula is described. Massive hepatomegaly and steatosis developed in the baby at a time of severe viral respiratory illness, prolonged fasting, hypoglycemia, and hypoketonuria. The infant was found to have secondary plasma carnitine deficiency and excessive loss of carnitine in the urine as part of a more generalized renal tubular dysfunction accompanying vitamin D deficiency and secondary hyperparathyroidism. With correction of the metabolic abnormalities and institution of a high carnitine diet, the hepatomegaly disappeared, plasma carnitine returned to normal levels, and the renal carnitine loss ceased. The findings suggest that secondary carnitine deficiency may play a role in fatty infiltration of the liver in patients with cystic fibrosis, especially during times of severe fasting stress.
W R Treem; C A Stanley
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Pediatrics     Volume:  83     ISSN:  0031-4005     ISO Abbreviation:  Pediatrics     Publication Date:  1989 Jun 
Date Detail:
Created Date:  1989-07-06     Completed Date:  1989-07-06     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  993-7     Citation Subset:  AIM; IM    
Department of Pediatrics, Hartford Hospital, CT 06115.
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MeSH Terms
Carnitine / blood*,  deficiency*,  urine
Combined Modality Therapy
Cystic Fibrosis / complications*,  diagnosis,  pathology,  therapy
Fatty Liver / diagnosis,  etiology*,  pathology,  therapy
Hepatomegaly / diagnosis,  etiology*,  pathology,  therapy
Infant Food / adverse effects
Soybean Proteins
Vegetable Proteins / adverse effects
Vitamin D Deficiency / complications,  diagnosis,  etiology,  pathology,  therapy
Grant Support
Reg. No./Substance:
0/Soybean Proteins; 0/Vegetable Proteins; 541-15-1/Carnitine
Comment In:
Pediatrics. 1991 May;87(5):744   [PMID:  2020530 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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