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纯合子β地中海贫血:悉尼皇家亚历山德拉儿童医院脾切除患者的回顾

Homozygous beta-thalassaemia: a review of patients who had splenectomy at the Royal Alexandra Hospital for Children, Sydney.

作者信息

Hoe T S, Lammi A, Webster B

机构信息

Royal Alexandra Hospital for Children, Sydney, Australia.

出版信息

Singapore Med J. 1994 Feb;35(1):59-61.

PMID:8009282
Abstract

Twenty-four patients with homozygous beta-thalassaemia who had been splenectomised and currently on treatment were studied retrospectively. They were divided into two groups. Group A: who had splenectomy prior to commencement of any regular blood transfusion. The mean haemoglobin for this group rose from 5.5 gm/dl pre-splenectomy to 7.7 gm/dl post splenectomy (p < 0.001). Group B: who were on regular blood transfusion when they had their splenectomy and the mean blood transfusion requirement dropped from 317 ml/kg/yr to 230 ml/kg/yr of packed red cells following splenectomy (p < 0.001). Three patients who were on regular blood transfusion and desferrioxamine developed Yersinia enterocolitica infection. They presented with fever and signs of an acute abdomen. At laparotomy, 2 of the patients had acute appendicitis. All 3 appendices grew Yersinia enterocolitica and one patient also had a Yersinia enterocolitica septicaemia. If a patient develops fever and enteritis, desferrioxamine should be stopped temporarily and cotrimoxazole started as prophylaxis against systemic Yersiniosis. No cases of pneumoccocal sepsis was reported.

摘要

对24例已行脾切除术且正在接受治疗的纯合子β地中海贫血患者进行了回顾性研究。他们被分为两组。A组:在开始任何常规输血之前进行脾切除术。该组患者术前平均血红蛋白为5.5g/dl,脾切除术后升至7.7g/dl(p<0.001)。B组:在进行脾切除术时正在接受常规输血,脾切除术后红细胞输注量从317ml/kg/年降至230ml/kg/年(p<0.001)。3例接受常规输血和去铁胺治疗的患者发生小肠结肠炎耶尔森菌感染。他们出现发热和急腹症体征。剖腹探查时,2例患者患有急性阑尾炎。所有3例阑尾均培养出小肠结肠炎耶尔森菌,1例患者还患有小肠结肠炎耶尔森菌败血症。如果患者出现发热和肠炎,应暂时停用去铁胺,并开始使用复方新诺明预防全身性耶尔森菌病。未报告肺炎球菌败血症病例。

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