Abstract
Extrarenal Manifestations of Atypical Hemolytic Uremic Syndrome: A Systematic Review and Meta-Analysis: PUB231
Journal of the American Society of Nephrology, Vol.34(11S), pp.1112-1112
11/2023
DOI: 10.1681/ASN.20233411S11112c
Abstract
Background:
Atypical Hemolytic Uremic Syndrome (aHUS) is a chronic thrombotic microangiopathy (TMA) resulting from abnormal alternative complement pathway activation. Although renal vasculature is commonly targeted, aHUS can involve many organ systems. Our review investigates the incidence and clinical outcomes of aHUS patients with extrarenal manifestations.
Methods:
The literature search was conducted through PubMed/Medline, Embase, Web of Science Core Collection, and CINAHL. The search criteria comprised terms such as ‘aHUs', ‘extrarenal’, and various individual organ systems. Clinical features specific to the patient were collected, including extrarenal involvement, laboratory values, genetic abnormalities, and adverse outcomes. The meta-analysis was completed using SPSS version 22.
Results:
In total, 47 studies were included encompassing a sample size of 890 aHUS patients. The most widely reported genetic abnormalities were in factor H (CFH) mutations [12% (84/700 patients); n=19 studies] and anti-FH IgG antibodies [27.1% (102/376 patients); n=10 studies]. The most commonly occurring extrarenal manifestations across the included studies were related to the central nervous system [28% (240/858 patients), n=32 studies], with seizure being the most-common CNS symptom. This was followed by the gastrointestinal system [31% (230/741 patients); n=25 studies] and the cardiovascular system [16% (97/607); n=23]. The proportion of aHUS patients with end-stage renal disease was reported across 11 studies in 13.2% (61/463 patients) of this subpopulation, and the mortality rate was 8.9% (56/632 patients) across 27 studies.
Conclusions:
Extrarenal manifestations were present in approximately 20-30% of aHUS patients, depending on age and organ system. Our findings indicate neurologic and gastrointestinal involvement to be the most incident. Studies infrequently report genetic findings and are limited in population size, warranting larger multi-centered cohort investigations.
Details
- Title: Subtitle
- Extrarenal Manifestations of Atypical Hemolytic Uremic Syndrome: A Systematic Review and Meta-Analysis: PUB231
- Creators
- Kush Doshi - Akron General Medical CenterAbdel Rahem S. Yusuf - Northeast Ohio Medical UniversitySidharth K. Sethi - Medanta The MedicityOlivia Boyer - Structure Federative de Recherche Necker, Paris, FranceCarla M. Nester - University of IowaPranjal Sharma - Northeast Ohio Medical UniversityChristoph Licht - Hospital for Sick ChildrenRupesh Raina - Akron General Medical Center
- Resource Type
- Abstract
- Publication Details
- Journal of the American Society of Nephrology, Vol.34(11S), pp.1112-1112
- DOI
- 10.1681/ASN.20233411S11112c
- ISSN
- 1046-6673
- eISSN
- 1533-3450
- Language
- English
- Date published
- 11/2023
- Academic Unit
- Internal Medicine; Stead Family Department of Pediatrics; Nephrology, Dialysis and Transplantation
- Record Identifier
- 9984648571102771
Metrics
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