Sirolimus in the Treatment of 5 Cases of Blue Rubber Bleb Nevus Syndrome in Children and Literature Review

Wang Yuesheng; Zhang Jing; Xue Fumin; Yu Zhidan; Li Xiaoqin

doi:10.13407/j.cnki.jpp.1672-108X.2024.02.010

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Sirolimus in the Treatment of 5 Cases of Blue Rubber Bleb Nevus Syndrome in Children and Literature Review

更新时间：2024-03-04

- Sirolimus in the Treatment of 5 Cases of Blue Rubber Bleb Nevus Syndrome in Children and Literature Review
- Vol. 30, Issue 2, Pages: 36-40(2024)
- 作者机构：
  
  郑州大学附属儿童医院,河南省儿童医院,郑州儿童医院,郑州市儿童消化疾病重点实验室,郑州,450018
- 作者简介：
- 基金信息：
- DOI：10.13407/j.cnki.jpp.1672-108X.2024.02.010
  CLC：
- Published：2024，
  
  Published Online：10 February 2024，
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Wang Yuesheng, Zhang Jing, Xue Fumin, et al. Sirolimus in the Treatment of 5 Cases of Blue Rubber Bleb Nevus Syndrome in Children and Literature Review. [J]. 30(2):36-40(2024)
DOI：

Wang Yuesheng, Zhang Jing, Xue Fumin, et al. Sirolimus in the Treatment of 5 Cases of Blue Rubber Bleb Nevus Syndrome in Children and Literature Review. [J]. 30(2):36-40(2024) DOI： 10.13407/j.cnki.jpp.1672-108X.2024.02.010.

摘要

目的:探讨儿童蓝色橡皮疱痣综合征(BRBNS)的临床特征及应用西罗莫司治疗的临床疗效。方法:收集我院2019 年1 月至2022 年4 月收治的5 例儿童BRBNS 的临床资料。通过PubMed、中国知网、万方数据库检索建库至2022 年4 月收录的文献

分析BRBNS 的临床特征、诊断、治疗及预后情况。结果:5 例BRBNS 均有皮肤静脉畸形及胃肠道病变;伴随重度小细胞低色素性贫血

表现为面色苍白、生长缓慢;内镜对BRBNS 诊断率达100%

病变分布在胃、十二指肠、空肠、回肠、结肠等。治疗上需多次输血、口服普萘洛尔、局部介入或硬化处理病变。多数3~6 个月复发

改用西罗莫司后症状好转。西罗莫司起始日剂量为 0. 5~1. 0 mg/ m2

根据血药浓度调整剂量增加至每日2. 0 mg/ m2

达到目标浓度5. 0~10. 0 ng/ mL

维持血红蛋白至稳定水平。结论:儿童BRBNS 临床症状较重且易反复

西罗莫司可作为安全有效的治疗药物。

Abstract

Objective: To probe into the clinical characteristics of blue rubber bleb nevus syndrome (BRBNS) in children and the clinical efficacy of sirolimus. Methods: Clinical data of 5 children with BRBNS from Jan. 2019 to Apr. 2022 were extracted. Literature were retrieved through PubMed

CNKI and Wanfang database to analyze the clinical characteristics

diagnosis

treatment and prognosis of BRBNS until Apr. 2022. Results: Five cases of BRBNS had cutaneous venous malformations and gastrointestinal lesions

accompanied by severe small cell hypochromic anemia

characterized by pale complexion and slow growth. Endoscopic diagnosis of BRBNS was 100%

with lesions distributed in the stomach

duodenum

jejunum

ileum

and colon. Treatment required multiple blood transfusions

oral propranolol

local intervention or sclerotic treatment of the lesion. Most of the lesions recurred from 3 to 6 months

the symptoms were improved after using sirolimus. The initial dose of sirolimus was 0. 5 to 1. 0 mg/ m2 per day

the dose was adjusted to 2 mg/ m2 per day according to the blood concentration to reach the target concentration of 5. 0 to 10. 0 ng/ mL

the hemoglobin was maintained to a stable level. Conclusion: The clinical symptoms of BRBNS in children are severe and easy to recur

sirolimus can be used as a safe and effective treatment for BRBNS in children.

关键词

西罗莫司蓝色橡皮疱痣综合征临床特征儿童

Keywords

sirolimusblue rubber bleb nevus syndromeclinical characteristicschildren

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