目的探讨W BC H em oly ing E rror(溶血不良)患者红细胞膜脂质含量变化;并分析全自动血细胞计数仪计数白细胞时,引起白细胞计数假性增高原因。方法选用60例W BC H em oly in E rror EDTA-K2抗凝全血,并配制成一定浓度红细胞混悬液,利...目的探讨W BC H em oly ing E rror(溶血不良)患者红细胞膜脂质含量变化;并分析全自动血细胞计数仪计数白细胞时,引起白细胞计数假性增高原因。方法选用60例W BC H em oly in E rror EDTA-K2抗凝全血,并配制成一定浓度红细胞混悬液,利用全自动血细胞分析仪检测红细胞数,低渗制备红细胞膜,再用7150全自动生化分析仪测其磷脂、胆固醇含量,并将结果进行配对t检验。结果60例W BC H em oly ing E rror组中,胆固醇为(1.27±0.052 3)×1-0 13mm ol/RBC,磷脂为(0.756±0.026)×10-13mm ol/RBC,同时与正常对照组比较[胆固醇(0.96±0.051 8)×1-0 13mm ol/RBC,磷脂(0.997±0.033)×1-0 13mm ol/RBC],P<0.05,具有显著性差异。结论W BC H em oly ing E rror患者红细胞膜脂质发生明显变化,膜磷脂降低,膜胆固醇增高,特别是胆固醇/磷脂明显增高,从而佐证了白细胞计数假性增高原因。展开更多
Objective: To determine whether adipose and red blood cell membrane lipids,particularly long-chain polyunsaturated fatty acids such as docosahexaenoic acid and eicosapentaenoic acid,are significantly correlated with p...Objective: To determine whether adipose and red blood cell membrane lipids,particularly long-chain polyunsaturated fatty acids such as docosahexaenoic acid and eicosapentaenoic acid,are significantly correlated with phenotype in a family with autosomal dominant Stargardt macular dystrophy (gene locus STGD3). Amutation in the ELOVL4 gene is responsible for the macular dystrophy in this family,and its disease-causing mechanism may be its possible involvement in fatty acid elongation in the retina. Methods: The subjects in this study included 18 adult family members known to have a 2-base pair deletion in the ELOVL4 gene. Control subjects included 26 family members without the mutation. Each subject received a complete eye examination including fundus photographs,the results of which were used to grade the severity of macular dystrophy on a 3-tier scale. Red blood cell membrane and adipose tissue lipids were analyzed as an indication of short-term and long-term dietary fatty acid intake. Results: When adipose lipids were analyzed,there was a significant inverse relationship between phenotypic severity and the level of eicosapentaenoic acid (r=-0.54; P =.04). When red blood cell lipids were analyzed,there were significant inverse relationships between phenotypic severity and levels of eicosapentaenoic acid (r=-0.55; P=.02) and docosahexaenoic acid (r=-0.48; P=.04). Conclusions: These results indicate that the phenotypic diversity in this family may be related to differences in dietary fat intake as reflected by adipose and red blood cell lipids. Clinical Relevance: This study demonstrates that dietary factors can influence the severity of an inherited human macular dystrophy.展开更多
文摘目的探讨W BC H em oly ing E rror(溶血不良)患者红细胞膜脂质含量变化;并分析全自动血细胞计数仪计数白细胞时,引起白细胞计数假性增高原因。方法选用60例W BC H em oly in E rror EDTA-K2抗凝全血,并配制成一定浓度红细胞混悬液,利用全自动血细胞分析仪检测红细胞数,低渗制备红细胞膜,再用7150全自动生化分析仪测其磷脂、胆固醇含量,并将结果进行配对t检验。结果60例W BC H em oly ing E rror组中,胆固醇为(1.27±0.052 3)×1-0 13mm ol/RBC,磷脂为(0.756±0.026)×10-13mm ol/RBC,同时与正常对照组比较[胆固醇(0.96±0.051 8)×1-0 13mm ol/RBC,磷脂(0.997±0.033)×1-0 13mm ol/RBC],P<0.05,具有显著性差异。结论W BC H em oly ing E rror患者红细胞膜脂质发生明显变化,膜磷脂降低,膜胆固醇增高,特别是胆固醇/磷脂明显增高,从而佐证了白细胞计数假性增高原因。
文摘Objective: To determine whether adipose and red blood cell membrane lipids,particularly long-chain polyunsaturated fatty acids such as docosahexaenoic acid and eicosapentaenoic acid,are significantly correlated with phenotype in a family with autosomal dominant Stargardt macular dystrophy (gene locus STGD3). Amutation in the ELOVL4 gene is responsible for the macular dystrophy in this family,and its disease-causing mechanism may be its possible involvement in fatty acid elongation in the retina. Methods: The subjects in this study included 18 adult family members known to have a 2-base pair deletion in the ELOVL4 gene. Control subjects included 26 family members without the mutation. Each subject received a complete eye examination including fundus photographs,the results of which were used to grade the severity of macular dystrophy on a 3-tier scale. Red blood cell membrane and adipose tissue lipids were analyzed as an indication of short-term and long-term dietary fatty acid intake. Results: When adipose lipids were analyzed,there was a significant inverse relationship between phenotypic severity and the level of eicosapentaenoic acid (r=-0.54; P =.04). When red blood cell lipids were analyzed,there were significant inverse relationships between phenotypic severity and levels of eicosapentaenoic acid (r=-0.55; P=.02) and docosahexaenoic acid (r=-0.48; P=.04). Conclusions: These results indicate that the phenotypic diversity in this family may be related to differences in dietary fat intake as reflected by adipose and red blood cell lipids. Clinical Relevance: This study demonstrates that dietary factors can influence the severity of an inherited human macular dystrophy.
