成人肾病学家和血液学家管理青少年过渡到年轻人的非典型溶血性尿毒症综合征和C3肾小球病指南

IF 2 3区 医学 Q2 UROLOGY & NEPHROLOGY Advances in chronic kidney disease Pub Date : 2022-05-01 DOI:10.1053/j.ackd.2022.04.003
Magdalena Riedl Khursigara , Mina Matsuda-Abedini , Seetha Radhakrishnan , Michelle A. Hladunewich , Mathieu Lemaire , Chia Wei Teoh , Damien Noone , Christoph Licht
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引用次数: 0

摘要

非典型溶血性尿毒症综合征和C3肾小球病/免疫复合物膜增生性肾小球肾炎是极为罕见的慢性补体介导疾病,大多数病例以儿童为表现。从儿科到成人肾内科医生的临床护理的转变——通常是非典型溶血性尿毒症综合征患者的原生肾脏或移植肾脏疾病得到控制,以及C3肾小球病变/免疫复合物膜增殖性肾小球肾炎患者的慢性进行性疾病,尽管治疗努力——确定了这些患者旅程中的一个具有挑战性的时刻。提高对这一患者群体脆弱性的认识;提供疾病病理生理学和管理方面的教育,包括使用新型高精度补体拮抗剂;在患者、家属和所有参与年龄差异的医疗团队成员之间建立持续的对话是不可避免的,以确保患者的最佳结果和这些患者安全过渡到成年。
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A Guide for Adult Nephrologists and Hematologists to Managing Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy in Teens Transitioning to Young Adults

Atypical hemolytic uremic syndrome and C3 glomerulopathy/immune complex membranoproliferative glomerulonephritis are ultra-rare chronic, complement-mediated diseases with childhood manifestation in a majority of cases. Transition of clinical care of patients from pediatric to adult nephrologists—typically with controlled disease in native or transplant kidneys in case of atypical hemolytic uremic syndrome and often with chronic progressive disease despite treatment efforts in case of C3 glomerulopathy/immune complex membranoproliferative glomerulonephritis—identifies a challenging juncture in the journey of these patients. Raising awareness for the vulnerability of this patient cohort; providing education on disease pathophysiology and management including the use of new, high-precision complement antagonists; and establishing an ongoing dialog of patients, families, and all members of the health care team involved on either side of the age divide will be inevitable to ensure optimal patient outcomes and a safe transition of these patients to adulthood.

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来源期刊
Advances in chronic kidney disease
Advances in chronic kidney disease 医学-泌尿学与肾脏学
自引率
3.40%
发文量
69
审稿时长
11.1 weeks
期刊介绍: The purpose of Advances Chronic Kidney Disease is to provide in-depth, scholarly review articles about the care and management of persons with early kidney disease and kidney failure, as well as those at risk for kidney disease. Emphasis is on articles related to the early identification of kidney disease; prevention or delay in progression of kidney disease
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