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土耳其的镰状细胞贫血。97例病例评估(含父母检查结果)。

Sickle-cell anaemia in Turkey. Evaluation of 97 cases (with parents' findings).

作者信息

Ozsoylu S, Altinöz N

出版信息

Scand J Haematol. 1977 Jul;19(1):85-92.

PMID:882846
Abstract

The haematological and clinical data in 97 sickle-cell anaemia cases and haematological findings of their parents are reported. In spite of the low Hb values of the patients, they tolerated their anaemia and very rarely required blood transfusions. The Hb F levels of the patients were in general higher than African origin SS anaemia patients but lower than the Shiite Saudi Arabians. However, in most of the cases the concentration of Hb F did not seem to influence the Hb concentration of the patients. Serum iron was found unexpectedly decreased in 22% of the patients. Osmotic fragility was found decreased in 100% of the patients and in 83.5% of the parents. The prevalence of G-6-PD deficiency was 21.2% in male patients and 15.6% in the parents.

摘要

报告了97例镰状细胞贫血患者的血液学和临床数据及其父母的血液学检查结果。尽管患者血红蛋白值较低,但他们能够耐受贫血,很少需要输血。患者的胎儿血红蛋白(Hb F)水平总体上高于非洲裔SS贫血患者,但低于什叶派沙特阿拉伯人。然而,在大多数情况下,Hb F浓度似乎并未影响患者的血红蛋白浓度。出乎意料的是,22%的患者血清铁降低。100%的患者和83.5%的父母红细胞渗透脆性降低。男性患者中葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症的患病率为21.2%,父母中的患病率为15.6%。

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