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β地中海贫血脾切除术后的结果

Post Splenectomy Outcome in β-Thalassemia.

作者信息

Merchant Rashid H, Shah Ami R, Ahmad Javed, Karnik Alka, Rai Nooralam

机构信息

Department of Pediatrics, Nanavati Super Speciality Hospital, Vile Parle (W), Mumbai, 400056, Maharashtra, India.

Department of Radiology, Nanavati Super Speciality Hospital, Mumbai, India.

出版信息

Indian J Pediatr. 2015 Dec;82(12):1097-100. doi: 10.1007/s12098-015-1792-5. Epub 2015 Jun 23.

Abstract

OBJECTIVES

To evaluate changes in annual blood transfusion requirements and complications after splenectomy in patients with β-thalassemia.

METHODS

Forty post-splenectomy β-thalassemic patients aged 8-33 y, receiving regular blood transfusions and chelation therapy were included and non transfusion dependant patients were excluded from this retrospective cross-sectional study. Details about their surgery, transfusion requirements, and platelet levels were recorded on a standard proforma. All patients underwent a B-mode and color-coded duplex sonography of the hepatoportal system during the study period.

RESULTS

The average ferritin level in the year prior to the study was 4432 mcg/L (range 480-12,200 mcg/L). The annual blood transfusion requirement in the first year and 5 y post splenectomy [mean ± SD (138.41 ± 90.38 ml/kg/y); (116 ± 41.44 ml/kg/y)] were significantly different from requirements before splenectomy [(mean ± SD) 294.85 ± 226 ml/kg/y; p value <0.001]. There was a significant rise in platelet counts within 24 h post splenectomy with a mean rise of 4,51,000/mm(3) (p value < 0.001). During the follow up period, infections were noted in 50 % of patients, with malaria (18.75 %) being the most common. Doppler study of the portal system in one case showed portal vein thrombosis.

CONCLUSIONS

A significant sustained fall in annual blood transfusion requirement and a rise in platelet counts occurred post-splenectomy. Increase in annual blood transfusion requirement should be investigated to find the cause.

摘要

目的

评估β地中海贫血患者脾切除术后每年输血需求及并发症的变化。

方法

本回顾性横断面研究纳入了40例年龄在8至33岁之间、接受定期输血和螯合治疗的脾切除术后β地中海贫血患者,排除了非输血依赖患者。有关其手术、输血需求和血小板水平的详细信息记录在标准表格上。在研究期间,所有患者均接受了肝门静脉系统的B超和彩色编码双功超声检查。

结果

研究前一年的平均铁蛋白水平为4432 mcg/L(范围480 - 12,200 mcg/L)。脾切除术后第一年和第5年的年输血需求量[均值±标准差(138.41±90.38 ml/kg/年);(116±41.44 ml/kg/年)]与脾切除术前的需求量[(均值±标准差)294.85±226 ml/kg/年;p值<0.001]有显著差异。脾切除术后24小时内血小板计数显著升高,平均升高451,000/mm³(p值<0.001)。在随访期间,50%的患者出现感染,其中疟疾(18.75%)最为常见。1例门静脉系统多普勒研究显示门静脉血栓形成。

结论

脾切除术后每年输血需求量显著持续下降,血小板计数升高。应调查年输血需求量增加的原因。

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