| Massive hepatomegaly, steatosis, and secondary plasma carnitine deficiency in an infant with cystic fibrosis. | |
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MedLine Citation:
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PMID: 2726354 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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W R Treem; C A Stanley |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Pediatrics Volume: 83 ISSN: 0031-4005 ISO Abbreviation: Pediatrics Publication Date: 1989 Jun |
Date Detail:
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Created Date: 1989-07-06 Completed Date: 1989-07-06 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 0376422 Medline TA: Pediatrics Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 993-7 Citation Subset: AIM; IM |
Affiliation:
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Department of Pediatrics, Hartford Hospital, CT 06115. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Carnitine
/
blood*,
deficiency*,
urine Combined Modality Therapy Cystic Fibrosis / complications*, diagnosis, pathology, therapy Fatty Liver / diagnosis, etiology*, pathology, therapy Hepatomegaly / diagnosis, etiology*, pathology, therapy Humans Infant Infant Food / adverse effects Male Soybean Proteins Soybeans Vegetable Proteins / adverse effects Vitamin D Deficiency / complications, diagnosis, etiology, pathology, therapy |
| Grant Support | |
ID/Acronym/Agency:
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NS 17742/NS/NINDS NIH HHS; RR 00240/RR/NCRR NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Soybean Proteins; 0/Vegetable Proteins; 541-15-1/Carnitine |
| Comments/Corrections | |
Comment In:
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Pediatrics. 1991 May;87(5):744
[PMID:
2020530
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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