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重型β地中海贫血中的脾切除术、铁过载和肝硬化

Splenectomy, iron overload and liver cirrhosis in beta-thalassemia major.

作者信息

Okon E, Levij I S, Rachmilewitz E A

出版信息

Acta Haematol. 1976;56(3):142-50. doi: 10.1159/000207931.

Abstract

In order to evaluate the interrelations of splenectomy, iron overload and cirrhosis, histologic specimens of liver and spleen were examined and correlated in 12 children with beta-thalassemia major. All patients had received blood transfusions since infancy. Correlations seemed to exist between splenic hemosiderosis and splenic weight, and between the latter and the age at time of splenectomy. All liver samples showed varying hemosiderosis, not correlated with the number of transfusions or the children's age. Irregular liver cirrhosis existed in three children, 7, 8, and 14 years after splenectomy. No cirrhosis existed in any of the children where the spleen was in situ. Splenectomy in children with thalassemia may carry the long-term risk of liver cirrhosis.

摘要

为了评估脾切除术、铁过载和肝硬化之间的相互关系,对12例重型β地中海贫血患儿的肝脏和脾脏组织标本进行了检查并相互关联分析。所有患者自婴儿期起就接受输血治疗。脾铁沉积与脾脏重量之间以及脾脏重量与脾切除时的年龄之间似乎存在关联。所有肝脏样本均显示出不同程度的铁沉积,这与输血次数或患儿年龄无关。脾切除术后7年、8年和14年,有3名儿童出现了不规则肝硬化。脾脏未切除的患儿均未出现肝硬化。地中海贫血患儿进行脾切除术可能存在肝硬化的长期风险。

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