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纯合子镰状细胞病的脾切除术后病程

Postsplenectomy course in homozygous sickle cell disease.

作者信息

Wright J G, Hambleton I R, Thomas P W, Duncan N D, Venugopal S, Serjeant G R

机构信息

MRC Laboratories (Jamaica) and the Division of Paediatric Surgery, University of the West Indies, Kingston, Jamaica.

出版信息

J Pediatr. 1999 Mar;134(3):304-9. doi: 10.1016/s0022-3476(99)70454-3.

DOI:10.1016/s0022-3476(99)70454-3
PMID:10064666
Abstract

OBJECTIVE

To determine whether children with homozygous sickle cell (SS) disease and splenectomy are at greater risk of death, overwhelming septicemia, or other complications.

METHODS

A total of 130 patients with SS treated by splenectomy (46 recurrent acute splenic sequestration, 84 chronic hypersplenism) over a 22.5-year period at the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica, were compared with a control group matched for sex, age, and duration of follow-up in a retrospective review. Deaths and bacteremias were examined over the whole study period. Painful crises, acute chest syndromes, and febrile episodes were compared in the 90 patients completing 5 years of postsplenectomy follow-up.

FINDINGS

Mortality and bacteremic episodes did not differ between the splenectomy and control groups. Painful crises were more common in the splenectomy group than in the control group (P =.01) but did not differ between splenectomy indications. Acute chest syndrome was more common in the splenectomy group than in the control group (P <.01) and was more common in the acute splenic sequestration group than in the hypersplenism group (P =.01). Febrile events did not differ between the groups or between the indications for splenectomy.

CONCLUSION

Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons.

摘要

目的

确定纯合子镰状细胞(SS)病患儿及行脾切除术者是否有更高的死亡、暴发性败血症或其他并发症风险。

方法

在牙买加金斯敦西印度大学医院镰状细胞诊所,对22.5年间接受脾切除术的130例SS患者(46例复发性急性脾滞留、84例慢性脾功能亢进)与按性别、年龄和随访时间匹配的对照组进行回顾性比较。在整个研究期间检查死亡情况和菌血症。对完成脾切除术后5年随访的90例患者的疼痛性危象、急性胸部综合征和发热发作进行比较。

结果

脾切除术组和对照组的死亡率和菌血症发作情况无差异。疼痛性危象在脾切除术组比对照组更常见(P = 0.01),但在脾切除指征之间无差异。急性胸部综合征在脾切除术组比对照组更常见(P < 0.01),在急性脾滞留组比脾功能亢进组更常见(P = 0.01)。发热事件在两组之间或脾切除指征之间无差异。

结论

脾切除术不会增加SS病患者的死亡或菌血症疾病风险,如有其他指征,不应因此而推迟手术。

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