Strandvik B
Department of Paediatrics, Karolinska Institute, Huddinge University Hospital, Stockholm, Sweden.
Acta Paediatr Scand Suppl. 1989;363:58-63; discussion 63-5. doi: 10.1111/apa.1989.78.s363.58.
Studies in our laboratory have supported the hypothesis, that the basic defect in cystic fibrosis increases the metabolism of essential fatty acids and thereby gradually gives rise to essential fatty acid deficiency, which is a well documented finding in most cases with this disease. Both the increased metabolism--giving high liberation of arachidonic acid and its metabolic products, i.e. different eicosanoids--and the subsequent essential fatty acid deficiency will cause gastrointestinal symptoms and the sequence of this development will mirror the natural history of the disease. Clinical data and results from animal research are discussed in relation to gastrointestinal symptoms and signs of cystic fibrosis.
我们实验室的研究支持了这样一种假说,即囊性纤维化的基本缺陷会增加必需脂肪酸的代谢,从而逐渐导致必需脂肪酸缺乏,这在大多数该疾病病例中是一个有充分记录的发现。代谢增加——导致花生四烯酸及其代谢产物(即不同的类二十烷酸)大量释放——以及随后的必需脂肪酸缺乏都会引起胃肠道症状,而这一发展过程将反映该疾病的自然病史。本文讨论了与囊性纤维化的胃肠道症状和体征相关的临床数据及动物研究结果。