Peretti N, Marcil V, Drouin E, Levy E
Department of Nutrition, CHU-Sainte-Justine, Université de Montréal, Montréal, Québec, Canada.
Nutr Metab (Lond). 2005 May 3;2(1):11. doi: 10.1186/1743-7075-2-11.
Transport mechanisms, whereby alimentary lipids are digested and packaged into small emulsion particles that enter intestinal cells to be translocated to the plasma in the form of chylomicrons, are impaired in cystic fibrosis. The purpose of this paper is to focus on defects that are related to intraluminal and intracellular events in this life-limiting genetic disorder. Specific evidence is presented to highlight the relationship between fat malabsorption and essential fatty acid deficiency commonly found in patients with cystic fibrosis that are often related to the genotype. Given the interdependency of pulmonary disease, pancreatic insufficiency and nutritional status, greater attention should be paid to the optimal correction of fat malabsorption and essential fatty acid deficiency in order to improve the quality of life and extend the life span of patients with cystic fibrosis.
在囊性纤维化中，消化道脂质被消化并包装成小的乳糜微粒进入肠细胞，然后以乳糜微粒的形式转运到血浆中的转运机制受到损害。本文旨在关注这种危及生命的遗传性疾病中与肠腔内和细胞内事件相关的缺陷。文中给出了具体证据，以突出囊性纤维化患者中常见的脂肪吸收不良与必需脂肪酸缺乏之间的关系，而这通常与基因型有关。鉴于肺部疾病、胰腺功能不全和营养状况之间的相互依存关系，应更加重视脂肪吸收不良和必需脂肪酸缺乏的最佳纠正，以提高囊性纤维化患者的生活质量并延长其寿命。
