Severe urological involvement in junctional epidermolysis bullosa justifying bladder enlargement by sigmoidocystoplasty and Mitrofanoff appendicovesicostomy

IF 1.3 4区 医学 Q3 PEDIATRICS Archives De Pediatrie Pub Date : 2025-05-01 Epub Date: 2025-03-18 DOI:10.1016/j.arcped.2025.01.002
Marielle Ehilé-Kacou , Pierre Yves Mure , Delphine Demede , Alice Phan , Justine Bacchetta , Valeska Bidault , Bruno Ranchin
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Abstract

The urological complications of junctional epidermolysis bullosa (JEB) are difficult to manage because of the fragility of the skin and mucosa, and are not the subject of any recommendations. We report on 2 cases of chronic lower urinary tract dysfunction causing chronic kidney disease, treated by bladder enlargement with sigmoidocystoplasty and Mitrofanoff appendicovesicostomy after failure of medical treatment and suprapubic catheterisation several times a day. Follow-up was favourable in both children, with kidney function preservation and significant improvement of quality of life.
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大疱性结缔组织表皮松解严重累及泌尿系统,证明乙状结肠膀胱成形术和米特罗凡诺夫阑尾膀胱造口术可扩大膀胱。
交界性表皮松解症(JEB)的泌尿系统并发症很难处理,因为皮肤和粘膜很脆弱,而且没有任何建议。我们报告了两例慢性下尿路功能障碍导致慢性肾病的病例,在药物治疗和每天数次耻骨上导尿失败后,通过乙状结肠膀胱成形术和米特罗法诺夫阑尾造口术扩大膀胱。两名患儿的后续治疗效果良好,肾功能得以保留,生活质量显著提高。
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来源期刊
Archives De Pediatrie
Archives De Pediatrie 医学-小儿科
CiteScore
2.80
自引率
5.60%
发文量
106
审稿时长
24.1 weeks
期刊介绍: Archives de Pédiatrie publishes in English original Research papers, Review articles, Short communications, Practice guidelines, Editorials and Letters in all fields relevant to pediatrics. Eight issues of Archives de Pédiatrie are released annually, as well as supplementary and special editions to complete these regular issues. All manuscripts submitted to the journal are subjected to peer review by international experts, and must: Be written in excellent English, clear and easy to understand, precise and concise; Bring new, interesting, valid information - and improve clinical care or guide future research; Be solely the work of the author(s) stated; Not have been previously published elsewhere and not be under consideration by another journal; Be in accordance with the journal''s Guide for Authors'' instructions: manuscripts that fail to comply with these rules may be returned to the authors without being reviewed. Under no circumstances does the journal guarantee publication before the editorial board makes its final decision. Archives de Pédiatrie is the official publication of the French Society of Pediatrics.
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