A Guide for Adult Nephrologists and Hematologists to Managing Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy in Teens Transitioning to Young Adults

Magdalena Riedl Khursigara; Mina Matsuda-Abedini; Seetha Radhakrishnan; Michelle A Hladunewich; Mathieu Lemaire; Chia Wei Teoh; Damien Noone; Christoph Licht

doi:10.1053/j.ackd.2022.04.003

A Guide for Adult Nephrologists and Hematologists to Managing Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy in Teens Transitioning to Young Adults

Adv Chronic Kidney Dis. 2022 May;29(3):231-242. doi: 10.1053/j.ackd.2022.04.003.

Authors

Magdalena Riedl Khursigara¹, Mina Matsuda-Abedini², Seetha Radhakrishnan², Michelle A Hladunewich³, Mathieu Lemaire⁴, Chia Wei Teoh², Damien Noone², Christoph Licht⁵

Affiliations

¹ Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada.
² Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada; Department of Paediatrics, Temerty Faculty of Medicine, University of Toronto, Toronto, ON, Canada.
³ Division of Nephrology and Obstetric Medicine, Department of Medicine, Sunnybrook Health Sciences, Temerty Faculty of Medicine, University of Toronto, Toronto, ON, Canada.
⁴ Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada; Department of Paediatrics, Temerty Faculty of Medicine, University of Toronto, Toronto, ON, Canada; Cell Biology Program, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada.
⁵ Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada; Department of Paediatrics, Temerty Faculty of Medicine, University of Toronto, Toronto, ON, Canada; Cell Biology Program, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada. Electronic address: [email protected].

PMID: 36084970
DOI: 10.1053/j.ackd.2022.04.003

Abstract

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.

Keywords: Atypical hemolytic uremic syndrome; C3 glomerulopathy; Education; Transition; Transplantation.

Publication types

Review

MeSH terms

Adolescent
Adult
Antigen-Antibody Complex / therapeutic use
Atypical Hemolytic Uremic Syndrome* / diagnosis
Atypical Hemolytic Uremic Syndrome* / therapy
Child
Complement System Proteins
Glomerulonephritis, Membranoproliferative* / therapy
Humans
Kidney Diseases*
Nephrologists
Young Adult

Substances

Antigen-Antibody Complex
Complement System Proteins