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镰状细胞病和地中海贫血患儿的脾切除术

Splenectomy in children with sickle cell disease and thalassemia.

作者信息

al-Salem A H, Khwaja M S, al-Fadel M, Grant C, al Awami B

出版信息

Indian J Pediatr. 1989 Nov-Dec;56(6):747-52. doi: 10.1007/BF02724458.

DOI:10.1007/BF02724458
PMID:2638677
Abstract

A number of Saudi children (31) with sickle cell disease and thalassemia underwent splenectomy: 12 for frequent blood transfusions, 15 for chronic hypersplenism (most of whom were also the recipients of periodic blood transfusion) and 4 for splenic abscess. The mean age of splenectomy was 8.8 years (8 months-18 years). Eight patients had sickle cell disease, 14 beta-thalassemia and 9 had sickle cell thalassemia. All patients received prophylaxis against pneumococcal infection. There was one postoperative death most probably due to sepsis. Sixteen of those who required frequent preoperative blood transfusions needed no more transfusions, while in 7 the need for transfusions decreased significantly (p less than 0.05). For those with hypersplenism, there was a significant postoperative increase in total hemoglobin (P less than 0.001), RBC (P less than 0.001) and platelet counts (p less than 0.02); and a substantial decrease in reticulocyte counts (p less than 0.05). The common post splenectomy complications were chest infection and a brief episode of pyrexia, but without undue morbidity. The study establishes a definite place for splenectomy in a selected population of children with sickle cell disease and thalassemia.

摘要

一些患有镰状细胞病和地中海贫血的沙特儿童(31名)接受了脾切除术:12名因频繁输血,15名因慢性脾功能亢进(其中大多数也是定期输血的受者),4名因脾脓肿。脾切除术的平均年龄为8.8岁(8个月至18岁)。8例患有镰状细胞病,14例患有β地中海贫血,9例患有镰状细胞-地中海贫血。所有患者均接受了预防肺炎球菌感染的治疗。有1例术后死亡,很可能是由于败血症。16名术前需要频繁输血的患者术后不再需要输血,7名患者输血需求显著减少(p<0.05)。对于脾功能亢进患者,术后总血红蛋白(P<0.001)、红细胞(P<0.001)和血小板计数显著增加(p<0.02);网织红细胞计数大幅下降(p<0.05)。脾切除术后常见的并发症是胸部感染和短暂的发热,但发病率不高。该研究确定了脾切除术在特定镰状细胞病和地中海贫血儿童群体中的明确地位。

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