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Atypical hemolytic-uremic syndrome with anti-factor H antibodies

MedGen UID:
1825998
•Concept ID:
C5680286
•
Disease or Syndrome
Synonyms: aHUS with anti-factor H antibodies; aHUS with neutralizing autoantibodies against factor H; Atypical hemolytic uremic syndrome with anti-factor H antibodies; atypical hemolytic-uremic syndrome with anti-factor H antibodies; Atypical HUS with anti-factor H antibodies; atypical HUS with anti-factor H antibodies; D-HUS with anti-factor H antibodies; hemolytic-uremic syndrome without diarrhea with anti-factor H antibodies; hemolytic-uremic syndrome without diarrhoea with anti-factor H antibodies
 
Monarch Initiative: MONDO:0019739
Orphanet: ORPHA93581

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAtypical hemolytic-uremic syndrome with anti-factor H antibodies

Professional guidelines

PubMed

Hemolytic uremic syndrome in a developing country: Consensus guidelines.
Bagga A, Khandelwal P, Mishra K, Thergaonkar R, Vasudevan A, Sharma J, Patnaik SK, Sinha A, Sethi S, Hari P, Dragon-Durey MA; Indian Society of Pediatric Nephrology
Pediatr Nephrol 2019 Aug;34(8):1465-1482. Epub 2019 Apr 15 doi: 10.1007/s00467-019-04233-7. PMID: 30989342
An international consensus approach to the management of atypical hemolytic uremic syndrome in children.
Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V; HUS International
Pediatr Nephrol 2016 Jan;31(1):15-39. Epub 2015 Apr 11 doi: 10.1007/s00467-015-3076-8. PMID: 25859752

Recent clinical studies

Etiology

Anti-factor B antibodies in atypical hemolytic uremic syndrome.
Khandelwal P, Nambiar S, Saini R, Saini S, Coshic P, Sinha A, Hari P, Palanichamy JK, Bagga A
Pediatr Nephrol 2024 Jun;39(6):1909-1916. Epub 2024 Jan 22 doi: 10.1007/s00467-024-06284-x. PMID: 38252289
Atypical hemolytic uremic syndrome: A monocentric adult Tunisian study and review of literature.
Kherder-Elfekih R, Hajji M, Fatma LB, Jebali H, Mami I, Smaoui W, Krid M, Rais L, Beji S, Zouaghi K, Sellami-Kallel M
Saudi J Kidney Dis Transpl 2018 Mar-Apr;29(2):297-302. doi: 10.4103/1319-2442.229263. PMID: 29657196
An international consensus approach to the management of atypical hemolytic uremic syndrome in children.
Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V; HUS International
Pediatr Nephrol 2016 Jan;31(1):15-39. Epub 2015 Apr 11 doi: 10.1007/s00467-015-3076-8. PMID: 25859752
Atypical hemolytic uremic syndrome.
Loirat C, Frémeaux-Bacchi V
Orphanet J Rare Dis 2011 Sep 8;6:60. doi: 10.1186/1750-1172-6-60. PMID: 21902819Free PMC Article
Complement and the atypical hemolytic uremic syndrome in children.
Loirat C, Noris M, Fremeaux-Bacchi V
Pediatr Nephrol 2008 Nov;23(11):1957-72. Epub 2008 Jul 2 doi: 10.1007/s00467-008-0872-4. PMID: 18594873Free PMC Article

Diagnosis

Constipation and hemolytic uremic syndrome.
Crawford B, Strebeck P, Saccente S
Pediatr Nephrol 2024 Feb;39(2):603-607. Epub 2023 Jul 20 doi: 10.1007/s00467-023-06093-8. PMID: 37474629
Anti-factor H antibody and its role in atypical hemolytic uremic syndrome.
Raina R, Mangat G, Hong G, Shah R, Nair N, Abboud B, Bagga S, Sethi SK
Front Immunol 2022;13:931210. Epub 2022 Aug 23 doi: 10.3389/fimmu.2022.931210. PMID: 36091034Free PMC Article
An international consensus approach to the management of atypical hemolytic uremic syndrome in children.
Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V; HUS International
Pediatr Nephrol 2016 Jan;31(1):15-39. Epub 2015 Apr 11 doi: 10.1007/s00467-015-3076-8. PMID: 25859752
Anti-factor H autoantibodies assay.
Dragon-Durey MA, Blanc C, Roumenina LT, Poulain N, Ngo S, Bordereau P, Frémeaux-Bacchi V
Methods Mol Biol 2014;1100:249-56. doi: 10.1007/978-1-62703-724-2_20. PMID: 24218265
Atypical hemolytic uremic syndrome.
Loirat C, Frémeaux-Bacchi V
Orphanet J Rare Dis 2011 Sep 8;6:60. doi: 10.1186/1750-1172-6-60. PMID: 21902819Free PMC Article

Therapy

Anti-factor B antibodies in atypical hemolytic uremic syndrome.
Khandelwal P, Nambiar S, Saini R, Saini S, Coshic P, Sinha A, Hari P, Palanichamy JK, Bagga A
Pediatr Nephrol 2024 Jun;39(6):1909-1916. Epub 2024 Jan 22 doi: 10.1007/s00467-024-06284-x. PMID: 38252289
An international consensus approach to the management of atypical hemolytic uremic syndrome in children.
Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V; HUS International
Pediatr Nephrol 2016 Jan;31(1):15-39. Epub 2015 Apr 11 doi: 10.1007/s00467-015-3076-8. PMID: 25859752
Eculizumab in anti-factor h antibodies associated with atypical hemolytic uremic syndrome.
Diamante Chiodini B, Davin JC, Corazza F, Khaldi K, Dahan K, Ismaili K, Adams B
Pediatrics 2014 Jun;133(6):e1764-8. doi: 10.1542/peds.2013-1594. PMID: 24843055
Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome.
Dragon-Durey MA, Sethi SK, Bagga A, Blanc C, Blouin J, Ranchin B, André JL, Takagi N, Cheong HI, Hari P, Le Quintrec M, Niaudet P, Loirat C, Fridman WH, Frémeaux-Bacchi V
J Am Soc Nephrol 2010 Dec;21(12):2180-7. Epub 2010 Nov 4 doi: 10.1681/ASN.2010030315. PMID: 21051740Free PMC Article
Anti-factor H autoantibody-associated hemolytic uremic syndrome: review of literature of the autoimmune form of HUS.
Dragon-Durey MA, Blanc C, Garnier A, Hofer J, Sethi SK, Zimmerhackl LB
Semin Thromb Hemost 2010 Sep;36(6):633-40. Epub 2010 Sep 23 doi: 10.1055/s-0030-1262885. PMID: 20865640

Prognosis

Constipation and hemolytic uremic syndrome.
Crawford B, Strebeck P, Saccente S
Pediatr Nephrol 2024 Feb;39(2):603-607. Epub 2023 Jul 20 doi: 10.1007/s00467-023-06093-8. PMID: 37474629
Atypical hemolytic uremic syndrome: A monocentric adult Tunisian study and review of literature.
Kherder-Elfekih R, Hajji M, Fatma LB, Jebali H, Mami I, Smaoui W, Krid M, Rais L, Beji S, Zouaghi K, Sellami-Kallel M
Saudi J Kidney Dis Transpl 2018 Mar-Apr;29(2):297-302. doi: 10.4103/1319-2442.229263. PMID: 29657196
An international consensus approach to the management of atypical hemolytic uremic syndrome in children.
Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V; HUS International
Pediatr Nephrol 2016 Jan;31(1):15-39. Epub 2015 Apr 11 doi: 10.1007/s00467-015-3076-8. PMID: 25859752
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
Atypical hemolytic uremic syndrome.
Loirat C, Frémeaux-Bacchi V
Orphanet J Rare Dis 2011 Sep 8;6:60. doi: 10.1186/1750-1172-6-60. PMID: 21902819Free PMC Article

Clinical prediction guides

Anti-factor H antibody associated hemolytic uremic syndrome following SARS-CoV-2 infection.
Khandelwal P, Krishnasamy S, Govindarajan S, Kumar M, Marik B, Sinha A, Hari P, Bagga A
Pediatr Nephrol 2022 Sep;37(9):2151-2156. Epub 2022 Jan 28 doi: 10.1007/s00467-021-05390-4. PMID: 35089377Free PMC Article
IgM Autoantibodies to Complement Factor H in Atypical Hemolytic Uremic Syndrome.
Cugno M, Berra S, Depetri F, Tedeschi S, Griffini S, Grovetti E, Caccia S, Cresseri D, Messa P, Testa S, Giglio F, Peyvandi F, Ardissino G
J Am Soc Nephrol 2021 May 3;32(5):1227-1235. Epub 2021 Mar 12 doi: 10.1681/ASN.2020081224. PMID: 33712527Free PMC Article
Atypical hemolytic uremic syndrome: A monocentric adult Tunisian study and review of literature.
Kherder-Elfekih R, Hajji M, Fatma LB, Jebali H, Mami I, Smaoui W, Krid M, Rais L, Beji S, Zouaghi K, Sellami-Kallel M
Saudi J Kidney Dis Transpl 2018 Mar-Apr;29(2):297-302. doi: 10.4103/1319-2442.229263. PMID: 29657196
An international consensus approach to the management of atypical hemolytic uremic syndrome in children.
Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V; HUS International
Pediatr Nephrol 2016 Jan;31(1):15-39. Epub 2015 Apr 11 doi: 10.1007/s00467-015-3076-8. PMID: 25859752
Complement and the atypical hemolytic uremic syndrome in children.
Loirat C, Noris M, Fremeaux-Bacchi V
Pediatr Nephrol 2008 Nov;23(11):1957-72. Epub 2008 Jul 2 doi: 10.1007/s00467-008-0872-4. PMID: 18594873Free PMC Article

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