Farrell P M, Bieri J G, Fratantoni J F, Wood R E, di Sant'Agnese P A
J Clin Invest. 1977 Jul;60(1):233-41. doi: 10.1172/JCI108760.
The role of vitamin E in human nutrition was studied by investigation of patients with cystic fibrosis (CF) and associated pancreatic insufficiency. Vitamin E status was assessed by measurement of the plasma concentration of the principal circulating isomer, alpha-tocopherol. Results of such determinations in 52 CF patients with pancreatogenic steatorrhea revealed that all were deficient in the vitamin. The extent of decreased plasma tocopherol varied markedly but correlated with indices of intestinal malabsorption, such as the serum carotene concentration and percentage of dietary fat absorbed. Supplementation with 5-10 times the recommended daily allowance of vitamin E in a water-miscible form increased the plasma alpha-tocopherol concentrations to normal in all 19 CF patients so evaluated. Studies on the effects of vitamin E deficiency focused on possible hematologic alterations. An improved technique was developed to measure erythrocyte hemolysis in vitro in the presence of hydrogen peroxide. While erythrocyte suspensions from control subjects demonstrated resistance to hemolysis during a 3-h incubation, all samples from tocopherol-deficient CF patients showed abnormal oxidant susceptibility, evidenced by greater than 5% hemoglobin release. The degree of peroxide-induced hemolysis was related to the plasma alpha-tocopherol concentration in an inverse, sigmoidal manner. The possibility of in vivo hemolysis was assessed by measuring the survival of (51)Cr-labeled erythrocytes in 19 vitamin-E deficient patients. A moderate but statistically significant decrease in the mean (51)Cr erythrocyte half-life value was found in this group. Measurement of erythrocyte survival before and after supplementation of 6 patients with vitamin E demonstrated that the shortened erythrocyte lifespan could be corrected to normal with this treatment. Other hematologic indices in deficient subjects, however, were normal and did not change upon supplementation with vitamin E. It is concluded that CF is invariably associated with vitamin E deficiency, provided that the patient in question has pancreatic achylia and is not taking supplementary doses of tocopherol. Concomitant hematologic effects consistent with mild hemolysis, but not anemia, occur and may be reversed with vitamin E therapy. Patients with CF should be given daily doses of a water-miscible form of vitamin E to correct the deficiency.
通过对囊性纤维化（CF）及相关胰腺功能不全患者的调查，研究了维生素E在人体营养中的作用。通过测量主要循环异构体α-生育酚的血浆浓度来评估维生素E状态。对52例患有胰腺性脂肪泻的CF患者进行此类测定的结果显示，所有患者均缺乏该维生素。血浆生育酚降低的程度差异显著，但与肠道吸收不良指标相关，如血清胡萝卜素浓度和膳食脂肪吸收百分比。以水溶性形式补充推荐每日摄入量5 - 10倍的维生素E后，所有19例接受评估的CF患者血浆α-生育酚浓度均恢复正常。对维生素E缺乏影响的研究集中在可能的血液学改变上。开发了一种改进技术，用于在过氧化氢存在下体外测量红细胞溶血。虽然来自对照受试者的红细胞悬液在3小时孵育期间表现出抗溶血能力，但来自缺乏生育酚的CF患者的所有样本均显示出异常的氧化敏感性，血红蛋白释放超过5%即可证明。过氧化物诱导的溶血程度与血浆α-生育酚浓度呈反比的S形关系。通过测量19例维生素E缺乏患者中（51）Cr标记红细胞的存活情况来评估体内溶血的可能性。该组患者的平均（51）Cr红细胞半衰期值出现中度但具有统计学意义的下降。对6例补充维生素E前后的患者进行红细胞存活测量表明，这种治疗可将缩短的红细胞寿命纠正至正常。然而，缺乏维生素E的受试者的其他血液学指标正常，补充维生素E后也未发生变化。得出结论，只要所讨论的患者存在胰腺无酸症且未服用补充剂量的生育酚，CF总是与维生素E缺乏相关。会出现与轻度溶血一致但非贫血的伴随血液学效应，维生素E治疗可能会使其逆转。CF患者应每日服用水溶性形式的维生素E以纠正缺乏。
