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初步应用氨甲环酸治疗血液系统疾病患儿顽固性上尿路血尿的经验。

Preliminary experience with epsilon aminocaproic acid for treatment of intractable upper tract hematuria in children with hematological disorders.

机构信息

Department of Urology, Children's Healthcare of Atlanta/Emory University School of Medicine, Atlanta, Georgia, USA.

出版信息

J Urol. 2010 Sep;184(3):1152-7. doi: 10.1016/j.juro.2010.05.020. Epub 2010 Jul 21.

Abstract

PURPOSE

Gross, intractable hematuria is rare in children. Although the role of epsilon aminocaproic acid in the management of refractory hematuria is well established in the adult population, few data exist about its use in children for this indication. We present our initial experience with epsilon aminocaproic acid for the treatment of intractable hematuria after more conservative measures failed, and propose an algorithm for administration of epsilon aminocaproic acid in children.

MATERIALS AND METHODS

We reviewed the charts of all patients treated with epsilon aminocaproic acid for intractable gross hematuria at our institution during a period of 36 months. All patients underwent hematological evaluation and any underlying bleeding dyscrasias were addressed. All patients also underwent renal and bladder ultrasound, retrograde pyelogram and ureteroscopy. Demographic information, medical and surgical histories, and epsilon aminocaproic acid dosing and outcomes were recorded.

RESULTS

Three boys and 1 girl 11 to 17 years old were treated with epsilon aminocaproic acid. Three patients had sickle trait (1 with nutcracker phenomenon) and 1 had hemophilia A. Three patients required packed red blood cell transfusions to maintain hematocrit. Three renal angiograms were performed, all of which were nondiagnostic. Duration of hematuria ranged from 1 to 52 weeks before administration of epsilon aminocaproic acid. Endoscopic evaluation demonstrated hematuria localized to 1 ureteral orifice in all 4 patients. All patients received 100 mg/kg epsilon aminocaproic acid orally every 6 hours, which uniformly led to cessation of hematuria.

CONCLUSIONS

Epsilon aminocaproic acid is useful for the management of gross refractory hematuria when more conservative measures fail. Because of its potential side effects, it should be used cautiously.

摘要

目的

儿童中严重且顽固的血尿较为罕见。虽然在成人中,ε-氨基己酸在治疗难治性血尿方面的作用已得到充分证实,但关于其在该适应证下用于儿童的资料甚少。我们报告了在更保守的治疗措施失败后,使用ε-氨基己酸治疗难治性血尿的初步经验,并提出了儿童使用ε-氨基己酸的给药方案。

材料和方法

我们回顾了 36 个月内在我院接受ε-氨基己酸治疗严重肉眼血尿的所有患者的病历。所有患者均接受了血液学评估,并对任何潜在的出血性疾病进行了处理。所有患者还接受了肾脏和膀胱超声、逆行肾盂造影和输尿管镜检查。记录了人口统计学信息、医疗和手术史,以及ε-氨基己酸的剂量和结果。

结果

3 名男孩和 1 名 11 至 17 岁的女孩接受了ε-氨基己酸治疗。3 名患者有镰状细胞特征(1 名伴有胡桃夹现象),1 名患有血友病 A。3 名患者需要输注浓缩红细胞以维持血细胞比容。进行了 3 次肾血管造影,均无诊断意义。在给予ε-氨基己酸之前,血尿持续时间为 1 至 52 周不等。内镜检查显示 4 名患者均有血尿局限于 1 个输尿管口。所有患者均接受 100mg/kgε-氨基己酸口服,每 6 小时 1 次,均能迅速停止血尿。

结论

当更保守的治疗措施失败时,ε-氨基己酸可用于治疗严重且难治性血尿。由于其潜在的副作用,应谨慎使用。

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