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[重型地中海贫血的部分脾切除术。附19例报告]

[Partial splenectomy in thalassemia major. Apropos of 19 cases].

作者信息

Kehila M, Khelif A, Kharrat H, Ennabli S, Abderrahim T

机构信息

Service de Chirurgie, C.H.U., Tunisie.

出版信息

J Chir (Paris). 1994 Feb;131(2):99-103.

PMID:8207104
Abstract

Nineteen cases of B Thalassemia have benefited from partial splenectomy at the General Surgery Service of Farhat Hached Hospital in Sousse (Tunisia). The partial splenectomy indication was to reduce hypersplenism, thus transfusion needs, to suppress splenic pain and to conserve a splenic remnant, which preserves patients' immunity. The operation was in reality a subtotal splenectomy keeping the lower pole in all cases. We had no per-operatory complication. The preoperatory bleeding was not more serious than in total splenectomy. In all the patients, we noticed reduction of about half the transfusion need, except one who had also a chronic deficit in glyco-six phospho-dehydrogenase. The average hemoglobin rate increased from 60 g/L in the pre-operatory to 80 g/L after the operation. Consequently, this reduction of transfusion needs results in the decrease of the hemochromatosis, which is one of the main complications of hypertransfused thalassemia.

摘要

在突尼斯苏塞的法哈特·哈谢德医院普通外科,19例β地中海贫血患者接受了部分脾切除术并从中受益。部分脾切除术的指征是减轻脾功能亢进,从而减少输血需求,抑制脾脏疼痛,并保留脾脏残余部分,以维持患者的免疫力。实际上,该手术在所有病例中都是保留脾下极的次全脾切除术。我们没有术中并发症。术前出血情况并不比全脾切除术更严重。在所有患者中,除了一名患有糖六磷酸脱氢酶慢性缺乏症的患者外,我们发现输血需求减少了约一半。平均血红蛋白水平从术前的60g/L升至术后的80g/L。因此,输血需求的减少导致血色素沉着症的减轻,而血色素沉着症是多次输血的地中海贫血的主要并发症之一。

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