Abstract:
UNLABELLED:Essential fatty acid deficiency is well known in cystic fibrosis patients, but its pathogenesis remains unclear. It might be related to protein-energy malnutrition which is a common feature of cystic fibrosis or to some specific defects in fatty acid metabolism. To avoid the deleterious effects of protein-energy malnutrition, this study assesses the plasma phospholipid fatty acid pattern in well nourished young cystic fibrosis subjects. Sixteen cystic fibrosis subjects aged 6.6-20.0 years were studied and compared to 16 healthy controls matched for gender, age and nutritional status. Plasma phospholipids were separated by thin layer chromatography and phospholipid fatty acid pattern was determined by gas liquid chromatography. Anthropometry and dual-energy X-ray absorptiometry showed that lean body mass, fat-free mass and fat mass were similar in the two groups. Nutritional inquiry showed higher ingestion of macronutrients by cystic fibrosis subjects than by controls. Plasma phospholipid palmitoleic acid and eicosatrienoic acid were higher, and by contrast linoleic acid and docosahexaenoic acid were lower in cystic fibrosis subjects than in controls. The ratio linoleic acid/arachidonic acid was lower and the ratio eicosatrienoic acid/arachidonic acid was higher in cystic fibrosis subjects than in controls. CONCLUSION:Essential fatty acid deficiency is present in young cystic fibrosis subjects in the absence of protein-energy malnutrition. It means that this deficiency is probably related to specific defects in fatty acid metabolism.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Roulet M,Frascarolo P,Rappaz I,Pilet Mdoi
10.1007/s004310050750subject
Has Abstractpub_date
1997-12-01 00:00:00pages
952-6issue
12eissn
0340-6199issn
1432-1076journal_volume
156pub_type
杂志文章abstract::Severe neonatal centronuclear myopathy is inherited as an X-linked condition characterized by primary asphyxia, extreme muscular hypotonia and absent spontaneous movements. We report seven cases from three families to point out the importance of diagnosis with regard to prognosis, outcome and genetic counselling. In h...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02072056
更新日期:1990-12-01 00:00:00
abstract::Mucocutaneous bleeding is common in childhood and may be the result of primary hemostatic disorders such as vascular abnormalities, von Willebrand disease, thrombocytopenia, and platelet dysfunction. A detailed bleeding history and physical examination are essential to distinguish between normal and abnormal bleeding ...
journal_title:European journal of pediatrics
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abstract::In a five-year-old girl with congenital hepatic fibrosis raised erythrocyte sedimentation rate and serum gammaglobulins were noted for 3 years. Needle liver biopsy showed histological features of cholangitis and Klebsiella oxytoca was cultured from a liver biopsy specimen. Antibiotic treatment resulted in normalizatio...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01377359
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journal_title:European journal of pediatrics
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更新日期:1980-09-01 00:00:00
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01955190
更新日期:1996-06-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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更新日期:1980-03-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章,随机对照试验
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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更新日期:1980-09-01 00:00:00
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
pub_type: 杂志文章,随机对照试验
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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更新日期:1999-05-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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pub_type: 杂志文章
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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pub_type: 杂志文章,多中心研究
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journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
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