Pediatric nephrology (Berlin, Germany) · 2026
IPNA clinical practice recommendations for the management of aHUS and secondary TMA in children.
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Riedl Khursigara Magdalena, Alconcher Laura F, Alhasan Khalid, Awan Atif, Bagga Arvind, Bashir Admani, Boyer Olivia, Bresolin Nilzete Liberato, Coccia Paula A, Durkan Anne, Greenbaum Larry A, Kang Hee Gyung, Haffner Dieter, Iijima Kazumoto, Kavanagh David, Khandelwal Priyanka, Loos Sebastian, Ma Alison Lap-Tak, McCulloch Mignon, Palma Lilian Monteiro Pereira, Pape Lars, Raina Rupesh, Safouh Hesham, Topaloglu Rezan, van de Kar Nicole C A J, Van de Walle Johan, Oh Jun, Licht Christoph
Аннотация
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) caused by complement-mediated injury of the vascular endothelium. We present Clinical Practice Recommendations (CPRs) and Practice Points (CPPs) for the diagnosis and management of aHUS and the related spectrum of secondary thrombotic microangiopathies (TMAs) in children, based on current evidence and expert consensus, developed by a panel of international experts including pediatric nephrologists associated with the International Pediatric Nephrology Association (IPNA), kidney pathologists, geneticists, and adult nephrologists. The guidance focuses exclusively on pediatric populations, in alignment with IPNA's scope, and does not address diagnoses exclusive to the adult age group.
METHODS: Six structured clinical questions were formulated using the PICO (Population, Intervention, Comparator, Outcomes) framework, guiding comprehensive literature reviews. Recommendations were developed through expert consensus using a modified Delphi process. Two predefined levels of agreement-agreement and strong agreement-were used, with consensus on either level requiring ≥ 80% concurrence among panel members.
RESULTS: A total of 51 CPRs for the diagnosis and management of genetic aHUS, autoimmune aHUS, aHUS in the setting of kidney transplantation, and secondary TMA are presented. Additionally, 9 CPPs provide guidance for the management of secondary TMA. Importantly, all guidance is contextualized to also reflect the distinct epidemiology and healthcare landscapes of less well-resourced countries.
CONCLUSION: These recommendations, based on global consensus, aim to standardize and provide guidance for the diagnostic work-up and treatment of aHUS and secondary TMAs in children worldwide, thus ultimately improving patient outcomes. In addition, this guidance supports implementation efforts in resource-limited settings and provides direction for future research.
METHODS: Six structured clinical questions were formulated using the PICO (Population, Intervention, Comparator, Outcomes) framework, guiding comprehensive literature reviews. Recommendations were developed through expert consensus using a modified Delphi process. Two predefined levels of agreement-agreement and strong agreement-were used, with consensus on either level requiring ≥ 80% concurrence among panel members.
RESULTS: A total of 51 CPRs for the diagnosis and management of genetic aHUS, autoimmune aHUS, aHUS in the setting of kidney transplantation, and secondary TMA are presented. Additionally, 9 CPPs provide guidance for the management of secondary TMA. Importantly, all guidance is contextualized to also reflect the distinct epidemiology and healthcare landscapes of less well-resourced countries.
CONCLUSION: These recommendations, based on global consensus, aim to standardize and provide guidance for the diagnostic work-up and treatment of aHUS and secondary TMAs in children worldwide, thus ultimately improving patient outcomes. In addition, this guidance supports implementation efforts in resource-limited settings and provides direction for future research.