Leroux C, Grasland M, Turmel N, Le Breton F, Chesnel C, Hentzen C, Amarenco G
GREEN GRC-01 UPMC (Group of Clinical Research in Neuro-urology), Sorbonne university, 75005 Paris, France.
GREEN GRC-01 UPMC (Group of Clinical Research in Neuro-urology), Sorbonne university, 75005 Paris, France.
Prog Urol. 2020 Mar;30(4):205-208. doi: 10.1016/j.purol.2019.10.008. Epub 2019 Nov 21.
Wolfram syndrome is a neurodegenerative disorder characterized by childhood onset diabetes mellitus, optic nerve atrophy, diabetes insipidus, hearing impairment, brainstem alteration and commonly bladder and bowel dysfunction.
We present here, 6 new cases of urinary dysfunction in this rare disease.
All patients had urinary retention with overactive bladder. The urodynamic assessment found overactive detrusor in 3 cases. Five out of six patients performed self-catheterization and were treated with anticholinergics or intradetrusor injection of botulinum toxin. The follow-up at 5 years found an alteration of the upper urinary tracts and a renal failure 3/6.
Urinary dysfunction is common in Wolfram syndrome, mainly characterized by overactive bladder and urinary retention. The urological risk is major requiring a systelmatic follow-up of these patients.
沃夫勒姆综合征是一种神经退行性疾病，其特征为儿童期发病的糖尿病、视神经萎缩、尿崩症、听力障碍、脑干改变，且通常伴有膀胱和肠道功能障碍。
我们在此报告6例这种罕见疾病中出现的排尿功能障碍新病例。
所有患者均有膀胱过度活动症伴尿潴留。尿动力学评估发现3例患者存在逼尿肌过度活动。6例患者中有5例进行了自我导尿，并接受了抗胆碱能药物治疗或膀胱逼尿肌内注射肉毒杆菌毒素。5年随访发现上尿路改变，6例中有3例出现肾衰竭。
排尿功能障碍在沃夫勒姆综合征中很常见，主要表现为膀胱过度活动症和尿潴留。泌尿系统风险很大，需要对这些患者进行系统随访。
4级。
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