Skip to main content
Erschienen in: Clinical and Experimental Nephrology 4/2016

15.07.2016 | Guideline

Clinical guides for atypical hemolytic uremic syndrome in Japan

verfasst von: Hideki Kato, Masaomi Nangaku, Hiroshi Hataya, Toshihiro Sawai, Akira Ashida, Rika Fujimaru, Yoshihiko Hidaka, Shinya Kaname, Shoichi Maruyama, Takashi Yasuda, Yoko Yoshida, Shuichi Ito, Motoshi Hattori, Yoshitaka Miyakawa, Yoshihiro Fujimura, Hirokazu Okada, Shoji Kagami, The Joint Committee for the Revision of Clinical Guides of Atypical Hemolytic Uremic Syndrome in Japan

Erschienen in: Clinical and Experimental Nephrology | Ausgabe 4/2016

Einloggen, um Zugang zu erhalten

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. In 2013, we developed diagnostic criteria to enable early diagnosis and timely initiation of appropriate treatment for aHUS. Recent clinical and molecular findings have resulted in several proposed classifications and definitions of thrombotic microangiopathy and aHUS. Based on recent advances in this field and the emerging international consensus to exclude secondary TMAs from the definition of aHUS, we have redefined aHUS and proposed diagnostic algorithms, differential diagnosis, and therapeutic strategies for aHUS.
Literatur
1.
Zurück zum Zitat Thompson RA, Winterborn MH. Hypocomplementaemia due to a genetic deficiency of beta 1H globulin. Clin Exp Immunol. 1981;46(1):110–9.PubMedPubMedCentral Thompson RA, Winterborn MH. Hypocomplementaemia due to a genetic deficiency of beta 1H globulin. Clin Exp Immunol. 1981;46(1):110–9.PubMedPubMedCentral
4.
Zurück zum Zitat Sawai T, Nangaku M, Ashida A, Fujimaru R, Hataya H, Hidaka Y, et al. Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society. Clin Exp Nephrol. 2014;18(1):4–9. doi:10.1007/s10157-013-0911-8.CrossRefPubMed Sawai T, Nangaku M, Ashida A, Fujimaru R, Hataya H, Hidaka Y, et al. Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society. Clin Exp Nephrol. 2014;18(1):4–9. doi:10.​1007/​s10157-013-0911-8.CrossRefPubMed
5.
Zurück zum Zitat Sawai T, Nangaku M, Ashida A, Fujimaru R, Hataya H, Hidaka Y, et al. Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society. Pediatr Int. 2014;56(1):1–5. doi:10.1111/ped.12274.CrossRefPubMed Sawai T, Nangaku M, Ashida A, Fujimaru R, Hataya H, Hidaka Y, et al. Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society. Pediatr Int. 2014;56(1):1–5. doi:10.​1111/​ped.​12274.CrossRefPubMed
8.
Zurück zum Zitat Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016;31(1):15–39. doi:10.1007/s00467-015-3076-8.CrossRefPubMed Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016;31(1):15–39. doi:10.​1007/​s00467-015-3076-8.CrossRefPubMed
11.
Zurück zum Zitat Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaime F, Dragon-Durey MA, Ngo S, et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol. 2013;8(4):554–62. doi:10.2215/CJN.04760512.CrossRefPubMedPubMedCentral Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaime F, Dragon-Durey MA, Ngo S, et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol. 2013;8(4):554–62. doi:10.​2215/​CJN.​04760512.CrossRefPubMedPubMedCentral
12.
Zurück zum Zitat Sheerin NS, Kavanagh D, Goodship TH, Johnson S. A national specialized service in England for atypical haemolytic uraemic syndrome-the first year’s experience. QJM. 2015;109(1):27–33. doi:10.1093/qjmed/hcv082.CrossRefPubMed Sheerin NS, Kavanagh D, Goodship TH, Johnson S. A national specialized service in England for atypical haemolytic uraemic syndrome-the first year’s experience. QJM. 2015;109(1):27–33. doi:10.​1093/​qjmed/​hcv082.CrossRefPubMed
13.
Zurück zum Zitat Hofer J, Janecke AR, Zimmerhackl LB, Riedl M, Rosales A, Giner T, et al. Complement factor H-related protein 1 deficiency and factor H antibodies in pediatric patients with atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol. 2013;8(3):407–15. doi:10.2215/CJN.01260212.CrossRefPubMed Hofer J, Janecke AR, Zimmerhackl LB, Riedl M, Rosales A, Giner T, et al. Complement factor H-related protein 1 deficiency and factor H antibodies in pediatric patients with atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol. 2013;8(3):407–15. doi:10.​2215/​CJN.​01260212.CrossRefPubMed
15.
Zurück zum Zitat Bu F, Maga T, Meyer NC, Wang K, Thomas CP, Nester CM, et al. Comprehensive genetic analysis of complement and coagulation genes in atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2014;25(1):55–64. doi:10.1681/ASN.2013050453.CrossRefPubMed Bu F, Maga T, Meyer NC, Wang K, Thomas CP, Nester CM, et al. Comprehensive genetic analysis of complement and coagulation genes in atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2014;25(1):55–64. doi:10.​1681/​ASN.​2013050453.CrossRefPubMed
16.
Zurück zum Zitat Johnson S, Stojanovic J, Ariceta G, Bitzan M, Besbas N, Frieling M, et al. An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatr Nephrol. 2014;29(10):1967–78. doi:10.1007/s00467-014-2817-4.CrossRefPubMed Johnson S, Stojanovic J, Ariceta G, Bitzan M, Besbas N, Frieling M, et al. An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatr Nephrol. 2014;29(10):1967–78. doi:10.​1007/​s00467-014-2817-4.CrossRefPubMed
17.
Zurück zum Zitat Uemura O, Honda M, Matsuyama T, Ishikura K, Hataya H, Yata N, et al. Age, gender, and body length effects on reference serum creatinine levels determined by an enzymatic method in Japanese children: a multicenter study. Clin Exp Nephrol. 2011;15(5):694–9. doi:10.1007/s10157-011-0452-y.CrossRefPubMed Uemura O, Honda M, Matsuyama T, Ishikura K, Hataya H, Yata N, et al. Age, gender, and body length effects on reference serum creatinine levels determined by an enzymatic method in Japanese children: a multicenter study. Clin Exp Nephrol. 2011;15(5):694–9. doi:10.​1007/​s10157-011-0452-y.CrossRefPubMed
18.
Zurück zum Zitat KDIGO. Clinical practice guideline for acute kidney injury. Kidney Int Suppl. 2012;2:1–138.CrossRef KDIGO. Clinical practice guideline for acute kidney injury. Kidney Int Suppl. 2012;2:1–138.CrossRef
19.
Zurück zum Zitat Kato H, Yoshida Y, Nangaku M. Pathogenesis of complement-mediated and coagulation-mediated atypical hemolytic uremic syndrome. Nihon Jinzo Gakkai Shi. 2014;56(7):1058–66. Kato H, Yoshida Y, Nangaku M. Pathogenesis of complement-mediated and coagulation-mediated atypical hemolytic uremic syndrome. Nihon Jinzo Gakkai Shi. 2014;56(7):1058–66.
21.
Zurück zum Zitat Fujimura Y, Isonishi A. Pathophysiology of thrombotic thrombocytopenic purpura. Nihon Jinzo Gakkai Shi. 2014;56(7):1043–51. Fujimura Y, Isonishi A. Pathophysiology of thrombotic thrombocytopenic purpura. Nihon Jinzo Gakkai Shi. 2014;56(7):1043–51.
22.
Zurück zum Zitat Shah N, Rutherford C, Matevosyan K, Shen YM, Sarode R. Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP). Br J Haematol. 2013;163(4):514–9. doi:10.1111/bjh.12569.CrossRefPubMed Shah N, Rutherford C, Matevosyan K, Shen YM, Sarode R. Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP). Br J Haematol. 2013;163(4):514–9. doi:10.​1111/​bjh.​12569.CrossRefPubMed
23.
Zurück zum Zitat Hattori M. Pathogenesis and clinical features of HUS and aHUS. Nihon Jinzo Gakkai Shi. 2014;56(7):1052–7. Hattori M. Pathogenesis and clinical features of HUS and aHUS. Nihon Jinzo Gakkai Shi. 2014;56(7):1052–7.
25.
Zurück zum Zitat von Vigier RO, Seibel K, Bianchetti MG. Positive Coombs test in pneumococcus-associated hemolytic uremic syndrome. A review of the literature. Nephron. 1999;82(2):183–4.CrossRef von Vigier RO, Seibel K, Bianchetti MG. Positive Coombs test in pneumococcus-associated hemolytic uremic syndrome. A review of the literature. Nephron. 1999;82(2):183–4.CrossRef
27.
Zurück zum Zitat Shimizu M, Yokoyama T, Sakashita N, Sato A, Ueno K, Akita C, et al. Thomsen-Friedenreich antigen exposure as a cause of Streptococcus pyogenes-associated hemolytic-uremic syndrome. Clin Nephrol. 2012;78(4):328–31.CrossRefPubMed Shimizu M, Yokoyama T, Sakashita N, Sato A, Ueno K, Akita C, et al. Thomsen-Friedenreich antigen exposure as a cause of Streptococcus pyogenes-associated hemolytic-uremic syndrome. Clin Nephrol. 2012;78(4):328–31.CrossRefPubMed
30.
Zurück zum Zitat Matsui K, Yasuda T. Drug/transplant-induced atypical hemolytic uremic syndrome. Nihon Jinzo Gakkai Shi. 2014;56(7):1067–74. Matsui K, Yasuda T. Drug/transplant-induced atypical hemolytic uremic syndrome. Nihon Jinzo Gakkai Shi. 2014;56(7):1067–74.
31.
Zurück zum Zitat Swisher KK, Doan JT, Vesely SK, Kwaan HC, Kim B, Lammle B, et al. Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports. Haematologica. 2007;92(7):936–43.CrossRefPubMed Swisher KK, Doan JT, Vesely SK, Kwaan HC, Kim B, Lammle B, et al. Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports. Haematologica. 2007;92(7):936–43.CrossRefPubMed
33.
Zurück zum Zitat Matsumoto M. Pathophysiology and management of transplantation associated TMA. Rinsho Ketsueki. 2013;54(10):1958–65.PubMed Matsumoto M. Pathophysiology and management of transplantation associated TMA. Rinsho Ketsueki. 2013;54(10):1958–65.PubMed
37.
Zurück zum Zitat Roumenina LT, Roquigny R, Blanc C, Poulain N, Ngo S, Dragon-Durey MA, et al. Functional evaluation of factor H genetic and acquired abnormalities: application for atypical hemolytic uremic syndrome (aHUS). Methods Mol Biol. 2014;1100:237–47. doi:10.1007/978-1-62703-724-2_19.CrossRefPubMed Roumenina LT, Roquigny R, Blanc C, Poulain N, Ngo S, Dragon-Durey MA, et al. Functional evaluation of factor H genetic and acquired abnormalities: application for atypical hemolytic uremic syndrome (aHUS). Methods Mol Biol. 2014;1100:237–47. doi:10.​1007/​978-1-62703-724-2_​19.CrossRefPubMed
38.
Zurück zum Zitat Yoshida Y, Miyata T, Matsumoto M, Shirotani-Ikejima H, Uchida Y, Ohyama Y, et al. A novel quantitative hemolytic assay coupled with restriction fragment length polymorphisms analysis enabled early diagnosis of atypical hemolytic uremic syndrome and identified unique predisposing mutations in Japan. PLoS One. 2015;10(5):e0124655. doi:10.1371/journal.pone.0124655.CrossRefPubMedPubMedCentral Yoshida Y, Miyata T, Matsumoto M, Shirotani-Ikejima H, Uchida Y, Ohyama Y, et al. A novel quantitative hemolytic assay coupled with restriction fragment length polymorphisms analysis enabled early diagnosis of atypical hemolytic uremic syndrome and identified unique predisposing mutations in Japan. PLoS One. 2015;10(5):e0124655. doi:10.​1371/​journal.​pone.​0124655.CrossRefPubMedPubMedCentral
39.
Zurück zum Zitat Sakai N, Wada T. Therapeutic strategy and thrombotic microangiopathy. Nihon Jinzo Gakkai Shi. 2014;56(7):1082–9. Sakai N, Wada T. Therapeutic strategy and thrombotic microangiopathy. Nihon Jinzo Gakkai Shi. 2014;56(7):1082–9.
40.
41.
Zurück zum Zitat Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368(23):2169–81. doi:10.1056/NEJMoa1208981.CrossRefPubMed Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368(23):2169–81. doi:10.​1056/​NEJMoa1208981.CrossRefPubMed
43.
Metadaten
Titel
Clinical guides for atypical hemolytic uremic syndrome in Japan
verfasst von
Hideki Kato
Masaomi Nangaku
Hiroshi Hataya
Toshihiro Sawai
Akira Ashida
Rika Fujimaru
Yoshihiko Hidaka
Shinya Kaname
Shoichi Maruyama
Takashi Yasuda
Yoko Yoshida
Shuichi Ito
Motoshi Hattori
Yoshitaka Miyakawa
Yoshihiro Fujimura
Hirokazu Okada
Shoji Kagami
The Joint Committee for the Revision of Clinical Guides of Atypical Hemolytic Uremic Syndrome in Japan
Publikationsdatum
15.07.2016
Verlag
Springer Japan
Erschienen in
Clinical and Experimental Nephrology / Ausgabe 4/2016
Print ISSN: 1342-1751
Elektronische ISSN: 1437-7799
DOI
https://doi.org/10.1007/s10157-016-1276-6

Weitere Artikel der Ausgabe 4/2016

Clinical and Experimental Nephrology 4/2016 Zur Ausgabe

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.