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Hemolytic uremic syndrome, atypical, childhood

MedGen UID:
430731
Concept ID:
CN036211
Disease or Syndrome
Synonym: Atypical childhood HUS

Disease characteristics

Excerpted from the GeneReview: Genetic Atypical Hemolytic-Uremic Syndrome
Hemolytic-uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs. The onset of atypical HUS (aHUS) ranges from the neonatal period to adulthood. Genetic aHUS accounts for an estimated 60% of all aHUS. Individuals with genetic aHUS frequently experience relapse even after complete recovery following the presenting episode; 60% of genetic aHUS progresses to end-stage renal disease (ESRD). [from GeneReviews]
Authors:
Marina Noris  |  Elena Bresin  |  Caterina Mele, et. al.   view full author information

Professional guidelines

PubMed

Schröder-Braunstein J, Kirschfink M
Mol Immunol 2019 Oct;114:299-311. Epub 2019 Aug 14 doi: 10.1016/j.molimm.2019.08.002. PMID: 31421540
Westra D, Wetzels JF, Volokhina EB, van den Heuvel LP, van de Kar NC
Neth J Med 2012 Apr;70(3):121-9. PMID: 22516576
Scheiring J, Andreoli SP, Zimmerhackl LB
Pediatr Nephrol 2008 Oct;23(10):1749-60. Epub 2008 Aug 13 doi: 10.1007/s00467-008-0935-6. PMID: 18704506Free PMC Article

Recent clinical studies

Etiology

Schaefer F, Ardissino G, Ariceta G, Fakhouri F, Scully M, Isbel N, Lommelé Å, Kupelian V, Gasteyger C, Greenbaum LA, Johnson S, Ogawa M, Licht C, Vande Walle J, Frémeaux-Bacchi V; Global aHUS Registry
Kidney Int 2018 Aug;94(2):408-418. Epub 2018 Jun 19 doi: 10.1016/j.kint.2018.02.029. PMID: 29907460
Fidan K, Göknar N, Gülhan B, Melek E, Yıldırım ZY, Baskın E, Hayran M, Gülleroglu K, Özçakar ZB, Ozaltin F, Soylemezoglu O
Pediatr Nephrol 2018 Aug;33(8):1395-1403. Epub 2018 Apr 2 doi: 10.1007/s00467-018-3933-3. PMID: 29610995
Vaisbich MH
J Bras Nefrol 2014 Apr-Jun;36(2):208-20. doi: 10.5935/0101-2800.20140032. PMID: 25055362
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
Proesmans W
Kidney Blood Press Res 1996;19(3-4):205-8. doi: 10.1159/000174075. PMID: 8887262

Diagnosis

Donadelli R, Sinha A, Bagga A, Noris M, Remuzzi G
Semin Nephrol 2023 Jul;43(4):151436. Epub 2023 Nov 10 doi: 10.1016/j.semnephrol.2023.151436. PMID: 37949684
Schaefer F, Ardissino G, Ariceta G, Fakhouri F, Scully M, Isbel N, Lommelé Å, Kupelian V, Gasteyger C, Greenbaum LA, Johnson S, Ogawa M, Licht C, Vande Walle J, Frémeaux-Bacchi V; Global aHUS Registry
Kidney Int 2018 Aug;94(2):408-418. Epub 2018 Jun 19 doi: 10.1016/j.kint.2018.02.029. PMID: 29907460
Sperati CJ, Moliterno AR
Hematol Oncol Clin North Am 2015 Jun;29(3):541-59. Epub 2015 Apr 4 doi: 10.1016/j.hoc.2015.02.002. PMID: 26043391
Vaisbich MH
J Bras Nefrol 2014 Apr-Jun;36(2):208-20. doi: 10.5935/0101-2800.20140032. PMID: 25055362
Vehaskari VM
Adv Chronic Kidney Dis 2011 Sep;18(5):317-23. doi: 10.1053/j.ackd.2011.07.001. PMID: 21896372

Therapy

Riedl Khursigara M, Matsuda-Abedini M, Radhakrishnan S, Hladunewich MA, Lemaire M, Teoh CW, Noone D, Licht C
Adv Chronic Kidney Dis 2022 May;29(3):231-242. doi: 10.1053/j.ackd.2022.04.003. PMID: 36084970
Fidan K, Göknar N, Gülhan B, Melek E, Yıldırım ZY, Baskın E, Hayran M, Gülleroglu K, Özçakar ZB, Ozaltin F, Soylemezoglu O
Pediatr Nephrol 2018 Aug;33(8):1395-1403. Epub 2018 Apr 2 doi: 10.1007/s00467-018-3933-3. PMID: 29610995
Ito N, Hataya H, Saida K, Amano Y, Hidaka Y, Motoyoshi Y, Ohta T, Yoshida Y, Terano C, Iwasa T, Kubota W, Takada H, Hara T, Fujimura Y, Ito S
Clin Exp Nephrol 2016 Apr;20(2):265-72. Epub 2015 Jul 9 doi: 10.1007/s10157-015-1142-y. PMID: 26156042
Verhave JC, Wetzels JF, van de Kar NC
Nephrol Dial Transplant 2014 Sep;29 Suppl 4:iv131-41. doi: 10.1093/ndt/gfu235. PMID: 25165180
Scheiring J, Andreoli SP, Zimmerhackl LB
Pediatr Nephrol 2008 Oct;23(10):1749-60. Epub 2008 Aug 13 doi: 10.1007/s00467-008-0935-6. PMID: 18704506Free PMC Article

Prognosis

Schaefer F, Ardissino G, Ariceta G, Fakhouri F, Scully M, Isbel N, Lommelé Å, Kupelian V, Gasteyger C, Greenbaum LA, Johnson S, Ogawa M, Licht C, Vande Walle J, Frémeaux-Bacchi V; Global aHUS Registry
Kidney Int 2018 Aug;94(2):408-418. Epub 2018 Jun 19 doi: 10.1016/j.kint.2018.02.029. PMID: 29907460
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
Vehaskari VM
Adv Chronic Kidney Dis 2011 Sep;18(5):317-23. doi: 10.1053/j.ackd.2011.07.001. PMID: 21896372
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
Proesmans W
Kidney Blood Press Res 1996;19(3-4):205-8. doi: 10.1159/000174075. PMID: 8887262

Clinical prediction guides

Schaefer F, Ardissino G, Ariceta G, Fakhouri F, Scully M, Isbel N, Lommelé Å, Kupelian V, Gasteyger C, Greenbaum LA, Johnson S, Ogawa M, Licht C, Vande Walle J, Frémeaux-Bacchi V; Global aHUS Registry
Kidney Int 2018 Aug;94(2):408-418. Epub 2018 Jun 19 doi: 10.1016/j.kint.2018.02.029. PMID: 29907460
Ito N, Hataya H, Saida K, Amano Y, Hidaka Y, Motoyoshi Y, Ohta T, Yoshida Y, Terano C, Iwasa T, Kubota W, Takada H, Hara T, Fujimura Y, Ito S
Clin Exp Nephrol 2016 Apr;20(2):265-72. Epub 2015 Jul 9 doi: 10.1007/s10157-015-1142-y. PMID: 26156042
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
Ruggenenti P, Remuzzi G
J Nephrol 1998 Nov-Dec;11(6):300-10. PMID: 10048496
Proesmans W
Kidney Blood Press Res 1996;19(3-4):205-8. doi: 10.1159/000174075. PMID: 8887262

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