Lloyd-Still J D, Bibus D M, Powers C A, Johnson S B, Holman R T
Department of Pediatrics, Rush Medical College, Chicago, IL 60612, USA.
Acta Paediatr. 1996 Dec;85(12):1426-32. doi: 10.1111/j.1651-2227.1996.tb13947.x.
Essential fatty acid (EFA) deficiency is a predisposing factor for pulmonary infection with Staphylococcus aureus and Pseudomonas aeruginosa, the two major pathogenic microorganisms in cystic fibrosis (CF).
The goal of this study was to investigate the essential fatty acid status of CF patients from infancy to 20 years old.
Plasma fatty acid profiles for phospholipid (PL) were determined for cord (n = 6), 4 months (n = 40), 16 months (n = 25), 3 y (n = 8), 5-10 y (n = 10), and 10-20 y (n = 10) aged CF patients and compared to their respective control; cord (n = 22), 1-36 months (n = 38) and adult (n = 100). Significance was established by Student's t-test (p < 0.05).
The plasma PL fatty acid profile for all CF patients, except cord, revealed consistent deficiency in omega 3 and omega 6 EFAs. These deficiencies were most marked at infancy and more pronounced for patients with meconium ileus.
EFA deficiency may contribute to the predisposition of CF infants to develop respiratory disease and to the excess cytotoxic activity found in bronchoalveolar lavage fluid at 2 months of age in the majority of screened infants.
必需脂肪酸（EFA）缺乏是金黄色葡萄球菌和铜绿假单胞菌肺部感染的一个诱发因素，这两种细菌是囊性纤维化（CF）的两大主要致病微生物。
本研究的目的是调查从婴儿期到20岁的CF患者的必需脂肪酸状况。
测定了脐带血（n = 6）、4个月（n = 40）、16个月（n = 25）、3岁（n = 8）、5 - 10岁（n = 10）和10 - 20岁（n = 10）的CF患者血浆磷脂（PL）脂肪酸谱，并与各自的对照组进行比较；脐带血（n = 22）、1 - 36个月（n = 38）和成年人（n = 100）。通过学生t检验确定显著性（p < 0.05）。
除脐带血外，所有CF患者的血浆PL脂肪酸谱均显示ω-3和ω-6必需脂肪酸持续缺乏。这些缺乏在婴儿期最为明显，胎粪性肠梗阻患者更为显著。
必需脂肪酸缺乏可能导致CF婴儿易患呼吸道疾病，以及在大多数筛查婴儿2个月时支气管肺泡灌洗液中发现的细胞毒性活性过高。
