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脾切除术在儿童纯合子镰状细胞病中的作用。

Role of splenectomy in homozygous sickle cell disease in childhood.

作者信息

Emond A M, Morais P, Venugopal S, Carpenter R G, Serjeant G R

出版信息

Lancet. 1984 Jan 14;1(8368):88-91. doi: 10.1016/s0140-6736(84)90014-x.

DOI:10.1016/s0140-6736(84)90014-x
PMID:6140433
Abstract

60 Jamaican children with homozygous sickle cell (SS) disease underwent splenectomy, 14 for prophylaxis against recurrent acute splenic sequestration and 46 for treatment of sustained hypersplenism. Age at operation varied from 9 months to 16 years. Patients were followed up for 1 month to 27 years (median 6 years), with a total of 369 years of patient-observation. None of the 3 patients who died, at ages 2 1/2, 6 1/2, and 21 years, had received prophylaxis against infection. Overwhelming sepsis was possible but not confirmed in the first two deaths which occurred 11 months and 2 1/2 years after operation; the third died from chronic renal failure 11 years after splenectomy. After operation, there were no confirmed cases of pneumococcal septicaemia or meningitis, and the commonest clinical event was the acute chest syndrome.

摘要

60名患有纯合子镰状细胞(SS)病的牙买加儿童接受了脾切除术,其中14例是为预防反复发生的急性脾滞留,46例是为治疗持续性脾功能亢进。手术年龄从9个月至16岁不等。对患者进行了1个月至27年的随访(中位时间为6年),患者观察总时长为369年。3例死亡患者年龄分别为2岁半、6岁半和21岁,均未接受过感染预防措施。前两例死亡分别发生在术后11个月和2年半,可能是暴发性败血症,但未得到确诊;第三例在脾切除术后11年死于慢性肾衰竭。术后,没有确诊的肺炎球菌败血症或脑膜炎病例,最常见的临床事件是急性胸综合征。

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Role of splenectomy in homozygous sickle cell disease in childhood.脾切除术在儿童纯合子镰状细胞病中的作用。
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Loss of NRF2 function exacerbates the pathophysiology of sickle cell disease in a transgenic mouse model.NRF2 功能丧失会加剧转基因小鼠模型中镰状细胞病的病理生理学。
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Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease.
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Cochrane Database Syst Rev. 2017 Nov 7;11(11):CD003425. doi: 10.1002/14651858.CD003425.pub4.
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Evolving locally appropriate models of care for indian sickle cell disease.为印度镰状细胞病发展适合当地情况的护理模式。
Indian J Med Res. 2016 Apr;143(4):405-13. doi: 10.4103/0971-5916.184282.
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Splenectomy and acute splenic sequestration crises in sickle cell disease.镰状细胞病中的脾切除术与急性脾滞留危象
Pediatr Surg Int. 1996 Dec;11(1):26-8. doi: 10.1007/BF00174580. Epub 2013 Sep 21.
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Splenic complications of sickle cell anemia and the role of splenectomy.镰状细胞贫血的脾脏并发症及脾切除术的作用。
ISRN Hematol. 2011;2011:864257. doi: 10.5402/2011/864257. Epub 2010 Oct 31.
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