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镰状细胞病的血管闭塞性危象发作

Vaso-occlusive crisis episodes in sickle cell disease.

作者信息

Adhikary P K, Hara S, Dwivedi C, Davis J W, Weaver C, Pavuluri S R

出版信息

J Med. 1986;17(3-4):227-40.

PMID:3473166
Abstract

Seventeen adult males and females with Hb-SS, Hb-SC (1) and Hb-S Thal (1) hemoglobinopathies were continuously studied for 3 years. Various hematological and biochemical parameters were measured in the venous blood of the subjects for blood gases, CBC profile, blood chemistry (SMA-18), fibrinogen, alpha-HBD and myoglobin levels, percent sickling, blood viscosity, oxygen affinity of whole blood, osmofragility of red blood cells and calcium and zinc contents in plasma and in RBC. The results were compared between those subjects who encountered more frequent vaso-occlusive crisis episodes (frequent sicklers) and those with fewer crisis episodes (infrequent sicklers), along with parameters between crisis and non-crisis states of frequent sicklers. Our studies showed that percent sickling, P50 for O2, CBC profile, PO2, serum calcium, ALP, LDH, alpha-HBD level, zinc and calcium levels in plasma and in RBC varied between crisis and non-crisis states of frequent sicklers and also between frequent sicklers and infrequent sicklers. A logical explanation of such variations may help in understanding the etiology of vaso-occlusive crisis episodes in sickle cell disease.

摘要

对17名患有血红蛋白病Hb-SS、Hb-SC(1例)和Hb-S地中海贫血(1例)的成年男性和女性进行了为期3年的持续研究。测量了受试者静脉血中的各种血液学和生化参数,包括血气、全血细胞计数、血液化学(SMA-18)、纤维蛋白原、α-羟丁酸脱氢酶和肌红蛋白水平、镰变百分比、血液粘度、全血的氧亲和力、红细胞渗透脆性以及血浆和红细胞中的钙和锌含量。比较了那些经历更频繁血管闭塞性危机发作的受试者(频繁镰变者)和发作次数较少的受试者(不频繁镰变者)的结果,以及频繁镰变者危机状态和非危机状态之间的参数。我们的研究表明,频繁镰变者危机状态和非危机状态之间以及频繁镰变者和不频繁镰变者之间,镰变百分比、O2的P50、全血细胞计数、PO2、血清钙、碱性磷酸酶、乳酸脱氢酶、α-羟丁酸脱氢酶水平、血浆和红细胞中的锌和钙水平存在差异。对这些差异进行合理的解释可能有助于理解镰状细胞病血管闭塞性危机发作的病因。

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