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西罗莫司成功治疗巨大先天性肝血管瘤合并系统性高血压

Successful Management of Massive Congenital Hepatic Hemangioma and Systemic Hypertension With Sirolimus.

机构信息

Department of Pediatrics, Chonnam National University Children's Hospital.

Department of Pediatrics, Chonnam National University Medical School.

出版信息

J Pediatr Hematol Oncol. 2022 Mar 1;44(2):e424-e427. doi: 10.1097/MPH.0000000000002146.

DOI:10.1097/MPH.0000000000002146
PMID:33735153
Abstract

Congenital hepatic hemangioma (CHH) is a common benign vascular tumor of the liver, seen in infancy. The clinical manifestations vary from incidental findings to life-threatening complications. The authors present here a case of an infant with massive CHH who developed systemic hypertension because of compression of the right renal artery by the CHH and did not respond to other lines of treatment. After sirolimus therapy, the CHH size decreased and antihypertensive drugs were no longer necessary. In a critical situation, if the embolization and/or steroids do not seem to control the situation, then adding sirolimus may be considered as secondary therapy with good additive effects.

摘要

先天性肝血管瘤(CHH)是一种常见的肝良性血管肿瘤,见于婴儿期。临床表现从偶然发现到危及生命的并发症不等。作者在此介绍了一例因 CHH 压迫右肾动脉而导致全身性高血压的巨大 CHH 婴儿病例,该患儿对其他治疗方法均无反应。西罗莫司治疗后,CHH 大小减小,不再需要抗高血压药物。在危急情况下,如果栓塞和/或类固醇似乎无法控制病情,那么加用西罗莫司可能是一种二线治疗方法,具有良好的附加效果。

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Sirolimus for vascular anomalies in the first year of life: a systematic review.
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Renomedullary exosomes produce antihypertensive effects in reversible two-kidney one-clip renovascular hypertensive mice.肾髓质外泌体在可复性两肾一夹型肾血管性高血压小鼠中产生降压作用。
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