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临床医生、患者和研究人员在 Alport 综合征中的合作的重要性。

The importance of clinician, patient and researcher collaborations in Alport syndrome.

机构信息

Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, MN, USA.

Department of Medicine, Royal Melbourne Hospital, Victoria, Australia.

出版信息

Pediatr Nephrol. 2020 May;35(5):733-742. doi: 10.1007/s00467-019-04241-7. Epub 2019 May 1.

Abstract

Alport syndrome is caused by mutations in the genes COL4A3, COL4A4 or COL4A5 and is characterised by progressive glomerular disease, sensorineural hearing loss and ocular defects. Occurring in less than 1:5000, Alport syndrome is a rare genetic disorder but still accounts for > 1% of the prevalent population receiving renal replacement therapy. There is also increasing awareness about the risk of chronic kidney disease in individuals with heterozygous mutations in Alport syndrome genes. The mainstay of current therapy is the use of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers, yet potential new therapies are now entering clinical trials. The 2017 International Workshop on Alport Syndrome in Glasgow was a pre-conference workshop ahead of the 50th anniversary meeting of the European Society for Pediatric Nephrology. It focussed on updates in clinical practice, genetics and basic science and also incorporated patient perspectives. More than 80 international experts including clinicians, geneticists, researchers from academia and industry, and patient representatives took part in panel discussions and breakout groups. This report summarises the workshop proceedings and the relevant contemporary literature. It highlights the unique clinician, patient and researcher collaborations achieved by regular engagement between the groups.

摘要

Alport 综合征是由 COL4A3、COL4A4 或 COL4A5 基因突变引起的,其特征是进行性肾小球疾病、感觉神经性听力损失和眼部缺陷。发病率低于 1/5000,Alport 综合征是一种罕见的遗传性疾病,但仍占接受肾脏替代治疗的流行人群的>1%。人们也越来越意识到杂合突变的 Alport 综合征基因个体存在慢性肾脏病的风险。目前治疗的主要方法是使用血管紧张素转换酶抑制剂和血管紧张素受体阻滞剂,但潜在的新疗法现在正在进入临床试验。2017 年在格拉斯哥举行的 Alport 综合征国际研讨会是在欧洲儿科肾脏病学会成立 50 周年会议之前举行的会前研讨会。它侧重于临床实践、遗传学和基础科学的最新进展,还纳入了患者的观点。包括临床医生、遗传学家、学术界和工业界的研究人员以及患者代表在内的 80 多名国际专家参加了小组讨论和分组讨论。本报告总结了研讨会的会议记录和相关的当代文献。它强调了通过小组之间的定期互动实现的独特的临床医生、患者和研究人员合作。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f3da/7096363/a8cbf85270c8/467_2019_4241_Fig1_HTML.jpg

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