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

S. Özsoylu, Necdet ALTINÖZd
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Abstract

The haematological and clinical data in 97 sickle-cell anaemia cases and haemato-logical 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.
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土耳其的镰状细胞贫血症。97例病例评价(附家长结果)。
报告了97例镰状细胞贫血的血液学和临床资料及其父母的血液学结果。尽管患者的Hb值较低,但他们能耐受贫血,很少需要输血。患者的Hb F水平普遍高于非洲血统的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的浓度似乎并不影响患者的Hb浓度。22%的患者血清铁意外下降。100%的患者和83.5%的家长的渗透脆弱性都有所下降。G-6-PD缺乏症在男性患者中患病率为21.2%,在父母中患病率为15.6%。
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