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. 2024 Dec;106(6):1038-1050.
doi: 10.1016/j.kint.2024.09.012. Epub 2024 Oct 10.

Outcomes from the International Society of Nephrology Hemolytic Uremic Syndromes International Forum

Collaborators, Affiliations

Outcomes from the International Society of Nephrology Hemolytic Uremic Syndromes International Forum

David Kavanagh et al. Kidney Int. 2024 Dec.

Abstract

Hemolytic uremic syndromes (HUSs) are a heterogeneous group of conditions, only some of which are mediated by complement (complement-mediated HUS). We report the outcome of the 2023 International Society of Nephrology HUS International Forum where a global panel of experts considered the current state of the art, identified areas of uncertainty, and proposed optimal solutions. Areas of uncertainty and areas for future research included the nomenclature of HUS, novel complement testing strategies, identification of biomarkers, genetic predisposition to atypical HUS, optimal dosing and withdrawal strategies for C5 inhibitors, treatment of kidney transplant recipients, disparity of access to treatment, and the next generation of complement inhibitors in complement-mediated HUS. The current rationale for optimal patient management is described.

Keywords: C5; acute kidney injury; complement; hemolytic uremic syndrome; thrombotic microangiopathy.

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Figures

Figure 1:
Figure 1:. Classification thrombotic microangiopathies
Abbreviations: AAV, ANCA (anti-neutrophil cytoplasmic antibody) associated vasculitis; ADAMTS13, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13; ACTN4, Actinin 4; aHUS, atypical haemolytic uraemic syndrome; C3G, C3 glomerulopathy; CAPS, catastrophic antiphospholipid syndrome; EXOSC3, exosome component 3; DGKE, gene encoding diacylglycerol kinase Ɛ; FH, factor H; FSGS, focal segmental glomerulosclerosis; HELLP, syndrome of haemolysis, elevated liver enzymes and low platelets; HIV, human immunodeficiency virus; HUS, haemolytic uraemic syndrome; HSD11B2, 1-beta-hydroxysteroid dehydrogenase; IgAN, IgA nephropathy; INF2, Inverted formin 2; MN, membranous nephropathy; MPGN, membranoproliferative glomerulonephritis; MTHFD1, methylenetetrahydrofolate dehydrogenase 1; MMACHC, methylmalonic aciduria and homocystinuria cblC type; MTR, 5-methyltetrahydrofolate-homocysteine S-methyltransferase; NPHS2, Podocin; SLE, systemic lupus erythematosus; SRC, scleroderma renal crisis; STEC, shiga toxin producing Escherichia coli; TMA, thrombotic microangiopathy; TTP, thrombotic thrombocytopenic purpura; TSEN2, tRNA splicing endonuclease subunit 2; WT1, WT1 transcription factor. Modified with permission from

References

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