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除了肾功能丧失:常染色体显性遗传性多囊肾病的患者护理。

Beyond Loss of Kidney Function: Patient Care in Autosomal Dominant Polycystic Kidney Disease.

机构信息

Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota.

Section of Nephrology, Yale University School of Medicine, New Haven, Connecticut.

出版信息

Kidney360. 2023 Dec 1;4(12):1806-1815. doi: 10.34067/KID.0000000000000296. Epub 2023 Nov 27.

Abstract

Patients with autosomal dominant polycystic kidney disease benefit from specialized care over their lifetimes, starting with diagnosis of the condition with ongoing discussion of both the renal course and extra-renal issues. Both renal and extra-renal issues may continue to cause major morbidity even after successful kidney transplant or initiation of RRT, and extra-renal disease aspects should always be considered as part of routine management. In this review, we will focus on updates in pain/depression screening, cardiac manifestations, liver and pancreatic cysts, kidney stone management, and genetic counseling. In some instances, we have shared our current clinical practice rather than an evidence-based guideline. We anticipate more standardization of care after the release of the Kidney Disease Improving Global Outcomes guidelines for management in autosomal dominant polycystic kidney disease later this year.

摘要

常染色体显性遗传性多囊肾病患者受益于终身的专业护理,从诊断开始,持续讨论肾脏病程和肾脏以外的问题。即使在成功进行肾移植或开始肾脏替代治疗后,肾脏和肾脏以外的问题仍可能导致严重的发病率,并且应始终将肾脏以外疾病的各个方面视为常规管理的一部分。在本次综述中,我们将重点介绍疼痛/抑郁筛查、心脏表现、肝脏和胰腺囊肿、肾结石管理以及遗传咨询方面的最新进展。在某些情况下,我们分享的是目前的临床实践,而不是循证指南。我们预计今年晚些时候,肾脏病预后质量倡议发布常染色体显性遗传性多囊肾病管理指南后,护理将更加标准化。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b150/10758524/c640b53dbf2b/kidney360-4-1806-g001.jpg

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