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Hemolytic uremic syndrome, atypical, susceptibility to

MedGen UID:
370773
•Concept ID:
C1969906
•
Finding
Synonym: HEMOLYTIC UREMIC SYNDROME, ATYPICAL, SUSCEPTIBILITY TO
 
OMIM®: 134371; 605336

Professional guidelines

PubMed

European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) Complement Guideline: Deficiencies, Diagnosis, and Management.
Brodszki N, Frazer-Abel A, Grumach AS, Kirschfink M, Litzman J, Perez E, Seppänen MRJ, Sullivan KE, Jolles S
J Clin Immunol 2020 May;40(4):576-591. Epub 2020 Feb 17 doi: 10.1007/s10875-020-00754-1. PMID: 32064578Free PMC Article
Atypical hemolytic uremic syndrome: Review of clinical presentation, diagnosis and management.
Sridharan M, Go RS, Willrich MAV
J Immunol Methods 2018 Oct;461:15-22. Epub 2018 Jul 19 doi: 10.1016/j.jim.2018.07.006. PMID: 30031798
Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome.
Picard C, Burtey S, Bornet C, Curti C, Montana M, Vanelle P
Pathol Biol (Paris) 2015 Jun;63(3):136-43. Epub 2015 Apr 3 doi: 10.1016/j.patbio.2015.03.001. PMID: 25845294

Recent clinical studies

Etiology

Verotoxin Receptor-Based Pathology and Therapies.
Lingwood C
Front Cell Infect Microbiol 2020;10:123. Epub 2020 Mar 31 doi: 10.3389/fcimb.2020.00123. PMID: 32296648Free PMC Article
Complement dysregulation in glomerulonephritis.
Kaartinen K, Safa A, Kotha S, Ratti G, Meri S
Semin Immunol 2019 Oct;45:101331. Epub 2019 Nov 9 doi: 10.1016/j.smim.2019.101331. PMID: 31711769
Eculizumab in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome: 10-year pharmacovigilance analysis.
Socié G, Caby-Tosi MP, Marantz JL, Cole A, Bedrosian CL, Gasteyger C, Mujeebuddin A, Hillmen P, Vande Walle J, Haller H
Br J Haematol 2019 Apr;185(2):297-310. Epub 2019 Feb 15 doi: 10.1111/bjh.15790. PMID: 30768680Free PMC Article
Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.
Goodship TH, Cook HT, Fakhouri F, Fervenza FC, Frémeaux-Bacchi V, Kavanagh D, Nester CM, Noris M, Pickering MC, Rodríguez de Córdoba S, Roumenina LT, Sethi S, Smith RJ; Conference Participants
Kidney Int 2017 Mar;91(3):539-551. Epub 2016 Dec 16 doi: 10.1016/j.kint.2016.10.005. PMID: 27989322
Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype.
Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, Daina E, Fenili C, Castelletti F, Sorosina A, Piras R, Donadelli R, Maranta R, van der Meer I, Conway EM, Zipfel PF, Goodship TH, Remuzzi G
Clin J Am Soc Nephrol 2010 Oct;5(10):1844-59. Epub 2010 Jul 1 doi: 10.2215/CJN.02210310. PMID: 20595690Free PMC Article

Diagnosis

European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) Complement Guideline: Deficiencies, Diagnosis, and Management.
Brodszki N, Frazer-Abel A, Grumach AS, Kirschfink M, Litzman J, Perez E, Seppänen MRJ, Sullivan KE, Jolles S
J Clin Immunol 2020 May;40(4):576-591. Epub 2020 Feb 17 doi: 10.1007/s10875-020-00754-1. PMID: 32064578Free PMC Article
CFHR Gene Variations Provide Insights in the Pathogenesis of the Kidney Diseases Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy.
Zipfel PF, Wiech T, Stea ED, Skerka C
J Am Soc Nephrol 2020 Feb;31(2):241-256. Epub 2020 Jan 24 doi: 10.1681/ASN.2019050515. PMID: 31980588Free PMC Article
Complement dysregulation in glomerulonephritis.
Kaartinen K, Safa A, Kotha S, Ratti G, Meri S
Semin Immunol 2019 Oct;45:101331. Epub 2019 Nov 9 doi: 10.1016/j.smim.2019.101331. PMID: 31711769
Atypical hemolytic uremic syndrome: Review of clinical presentation, diagnosis and management.
Sridharan M, Go RS, Willrich MAV
J Immunol Methods 2018 Oct;461:15-22. Epub 2018 Jul 19 doi: 10.1016/j.jim.2018.07.006. PMID: 30031798
Complement in hemolytic anemia.
Brodsky RA
Blood 2015 Nov 26;126(22):2459-65. Epub 2015 Nov 18 doi: 10.1182/blood-2015-06-640995. PMID: 26582375Free PMC Article

Therapy

Eculizumab in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome: 10-year pharmacovigilance analysis.
Socié G, Caby-Tosi MP, Marantz JL, Cole A, Bedrosian CL, Gasteyger C, Mujeebuddin A, Hillmen P, Vande Walle J, Haller H
Br J Haematol 2019 Apr;185(2):297-310. Epub 2019 Feb 15 doi: 10.1111/bjh.15790. PMID: 30768680Free PMC Article
Complement-driven anemia: more than just paroxysmal nocturnal hemoglobinuria.
Merrill SA, Brodsky RA
Hematology Am Soc Hematol Educ Program 2018 Nov 30;2018(1):371-376. doi: 10.1182/asheducation-2018.1.371. PMID: 30504334Free PMC Article
Atypical hemolytic uremic syndrome: Review of clinical presentation, diagnosis and management.
Sridharan M, Go RS, Willrich MAV
J Immunol Methods 2018 Oct;461:15-22. Epub 2018 Jul 19 doi: 10.1016/j.jim.2018.07.006. PMID: 30031798
Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.
Goodship TH, Cook HT, Fakhouri F, Fervenza FC, Frémeaux-Bacchi V, Kavanagh D, Nester CM, Noris M, Pickering MC, Rodríguez de Córdoba S, Roumenina LT, Sethi S, Smith RJ; Conference Participants
Kidney Int 2017 Mar;91(3):539-551. Epub 2016 Dec 16 doi: 10.1016/j.kint.2016.10.005. PMID: 27989322
Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome.
Picard C, Burtey S, Bornet C, Curti C, Montana M, Vanelle P
Pathol Biol (Paris) 2015 Jun;63(3):136-43. Epub 2015 Apr 3 doi: 10.1016/j.patbio.2015.03.001. PMID: 25845294

Prognosis

Complement dysregulation in glomerulonephritis.
Kaartinen K, Safa A, Kotha S, Ratti G, Meri S
Semin Immunol 2019 Oct;45:101331. Epub 2019 Nov 9 doi: 10.1016/j.smim.2019.101331. PMID: 31711769
Atypical hemolytic uremic syndrome: Review of clinical presentation, diagnosis and management.
Sridharan M, Go RS, Willrich MAV
J Immunol Methods 2018 Oct;461:15-22. Epub 2018 Jul 19 doi: 10.1016/j.jim.2018.07.006. PMID: 30031798
Atypical hemolytic uremic syndrome: Laboratory characteristics, complement-amplifying conditions, renal biopsy, and genetic mutations.
Hossain MA, Cheema A, Kalathil S, Bajwa R, Costanzo EJ, Cosentino J, Cheng J, Vachharajani T, Asif A
Saudi J Kidney Dis Transpl 2018 Mar-Apr;29(2):276-283. doi: 10.4103/1319-2442.229287. PMID: 29657193
Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults.
Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaimé F, Dragon-Durey MA, Ngo S, Moulin B, Servais A, Provot F, Rostaing L, Burtey S, Niaudet P, Deschênes G, Lebranchu Y, Zuber J, Loirat C
Clin J Am Soc Nephrol 2013 Apr;8(4):554-62. Epub 2013 Jan 10 doi: 10.2215/CJN.04760512. PMID: 23307876Free PMC Article
Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype.
Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, Daina E, Fenili C, Castelletti F, Sorosina A, Piras R, Donadelli R, Maranta R, van der Meer I, Conway EM, Zipfel PF, Goodship TH, Remuzzi G
Clin J Am Soc Nephrol 2010 Oct;5(10):1844-59. Epub 2010 Jul 1 doi: 10.2215/CJN.02210310. PMID: 20595690Free PMC Article

Clinical prediction guides

Atypical hemolytic uremic syndrome: Laboratory characteristics, complement-amplifying conditions, renal biopsy, and genetic mutations.
Hossain MA, Cheema A, Kalathil S, Bajwa R, Costanzo EJ, Cosentino J, Cheng J, Vachharajani T, Asif A
Saudi J Kidney Dis Transpl 2018 Mar-Apr;29(2):276-283. doi: 10.4103/1319-2442.229287. PMID: 29657193
Modified Ham test for atypical hemolytic uremic syndrome.
Gavriilaki E, Yuan X, Ye Z, Ambinder AJ, Shanbhag SP, Streiff MB, Kickler TS, Moliterno AR, Sperati CJ, Brodsky RA
Blood 2015 Jun 4;125(23):3637-46. Epub 2015 Apr 10 doi: 10.1182/blood-2015-02-629683. PMID: 25862562Free PMC Article
The endothelial glycocalyx as a potential modifier of the hemolytic uremic syndrome.
Boels MG, Lee DH, van den Berg BM, Dane MJ, van der Vlag J, Rabelink TJ
Eur J Intern Med 2013 Sep;24(6):503-9. Epub 2013 Jan 26 doi: 10.1016/j.ejim.2012.12.016. PMID: 23357408
Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype.
Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, Daina E, Fenili C, Castelletti F, Sorosina A, Piras R, Donadelli R, Maranta R, van der Meer I, Conway EM, Zipfel PF, Goodship TH, Remuzzi G
Clin J Am Soc Nephrol 2010 Oct;5(10):1844-59. Epub 2010 Jul 1 doi: 10.2215/CJN.02210310. PMID: 20595690Free PMC Article
Atypical haemolytic uraemic syndrome and mutations in complement regulator genes.
Dragon-Durey MA, Frémeaux-Bacchi V
Springer Semin Immunopathol 2005 Nov;27(3):359-74. Epub 2005 Nov 11 doi: 10.1007/s00281-005-0003-2. PMID: 16189652

Recent systematic reviews

End stage renal disease in patient with microscopic polyangiitis and atypical hemolytic-uremic syndrome arose 3 weeks after the third dose of anti-SARS-CoV2 vaccine mRNA-1273: A case report with literature revision.
Moronti V, Carubbi F, Sollima L, Piscitani L, Ferri C
Medicine (Baltimore) 2023 Dec 15;102(50):e36560. doi: 10.1097/MD.0000000000036560. PMID: 38115241Free PMC Article

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