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囊性纤维化中必需脂肪酸代谢缺陷

Defective essential-fatty-acid metabolism in cystic fibrosis.

作者信息

Rivers J P, Hassam A G

出版信息

Lancet. 1975 Oct 4;2(7936):642-3. doi: 10.1016/s0140-6736(75)90121-x.

Abstract

Cystic fibrosis (C.F.) is characterised by low serum levels of essential fatty acids (E.F.A.). However, the fatty-acid pattern does not totally resemble that of dietary E.F.A. deficiency. The differences suggest a reduction in the desaturation of E.F.S. It is not known whether this defect is the primary lesion in C.F. or is the result of tissue damage in the disease. It is proposed that C.F. patients might have increased linoleic-acid requirements, and possibly specific requirements for its desaturation products.

摘要

囊性纤维化(C.F.)的特征是血清中必需脂肪酸(E.F.A.)水平较低。然而,脂肪酸模式并不完全类似于饮食中E.F.A.缺乏的情况。这些差异表明E.F.S.的去饱和作用有所降低。目前尚不清楚这种缺陷是C.F.的原发性病变还是该疾病组织损伤的结果。有人提出,C.F.患者可能对亚油酸的需求量增加,并且可能对其去饱和产物有特定需求。

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