{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,5,29]],"date-time":"2023-05-29T12:53:20Z","timestamp":1685364800192},"reference-count":26,"publisher":"Springer Science and Business Media LLC","issue":"8","license":[{"start":{"date-parts":[[2018,7,11]],"date-time":"2018-07-11T00:00:00Z","timestamp":1531267200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Internist"],"published-print":{"date-parts":[[2018,8]]},"DOI":"10.1007\/s00108-018-0455-9","type":"journal-article","created":{"date-parts":[[2018,7,11]],"date-time":"2018-07-11T05:42:00Z","timestamp":1531287720000},"page":"799-804","update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Humangenetik beim atypischen h\u00e4molytisch-ur\u00e4mischen Syndrom \u2013 Rolle in Diagnostik und Therapie"],"prefix":"10.1007","volume":"59","author":[{"given":"M.","family":"Knoop","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Haller","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Menne","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2018,7,11]]},"reference":[{"issue":"3","key":"455_CR1","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1016\/j.kint.2016.10.005","volume":"91","author":"TH Goodship","year":"2017","unstructured":"Goodship TH et\u00a0al (2017) Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a\u00a0\u201cKidney Disease: Improving Global Outcomes\u201d (KDIGO) Controversies Conference. Kidney Int 91(3):539\u2013551","journal-title":"Kidney Int"},{"issue":"7","key":"455_CR2","doi-asserted-by":"publisher","first-page":"654","DOI":"10.1056\/NEJMra1312353","volume":"371","author":"JN George","year":"2014","unstructured":"George JN, Nester CM (2014) Syndromes of thrombotic microangiopathy. N\u00a0Engl J Med 371(7):654\u2013666","journal-title":"N Engl J Med"},{"issue":"1","key":"455_CR3","first-page":"110","volume":"46","author":"RA Thompson","year":"1981","unstructured":"Thompson RA, Winterborn MH (1981) Hypocomplementaemia due to a\u00a0genetic deficiency of beta 1H globulin. Clin Exp Immunol 46(1):110\u2013119","journal-title":"Clin Exp Immunol"},{"issue":"1","key":"455_CR4","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1007\/s00281-017-0663-8","volume":"40","author":"EKS Wong","year":"2018","unstructured":"Wong EKS, Kavanagh D (2018) Diseases of complement dysregulation\u2014an overview. Semin Immunopathol 40(1):49\u201364","journal-title":"Semin Immunopathol"},{"issue":"1","key":"455_CR5","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1111\/j.1365-2141.2009.07916.x","volume":"148","author":"CM Taylor","year":"2010","unstructured":"Taylor CM et\u00a0al (2010) Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom. Br J Haematol 148(1):37\u201347","journal-title":"Br J Haematol"},{"issue":"1","key":"455_CR6","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1016\/j.molimm.2015.03.246","volume":"67","author":"CM Nester","year":"2015","unstructured":"Nester CM et\u00a0al (2015) Atypical aHUS: State of the art. Mol Immunol 67(1):31\u201342","journal-title":"Mol Immunol"},{"issue":"6","key":"455_CR7","doi-asserted-by":"publisher","first-page":"1249","DOI":"10.1084\/jem.20070301","volume":"204","author":"MC Pickering","year":"2007","unstructured":"Pickering MC et\u00a0al (2007) Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domains. J\u00a0Exp Med 204(6):1249\u20131256","journal-title":"J Exp Med"},{"issue":"3","key":"455_CR8","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1016\/j.molimm.2013.06.001","volume":"56","author":"C Skerka","year":"2013","unstructured":"Skerka C et\u00a0al (2013) Complement factor H related proteins (CFHRs). Mol Immunol 56(3):170\u2013180","journal-title":"Mol Immunol"},{"issue":"5","key":"455_CR9","doi-asserted-by":"publisher","first-page":"1151","DOI":"10.1038\/ki.2013.373","volume":"85","author":"A Sinha","year":"2014","unstructured":"Sinha A et al (2014) Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody-associated hemolytic uremic syndrome in children. Kidney Int 85(5):1151\u20131160","journal-title":"Kidney Int"},{"issue":"6","key":"455_CR10","doi-asserted-by":"publisher","first-page":"508","DOI":"10.1016\/j.semnephrol.2013.08.003","volume":"33","author":"D Kavanagh","year":"2013","unstructured":"Kavanagh D, Goodship TH, Richards A (2013) Atypical hemolytic uremic syndrome. Semin Nephrol 33(6):508\u2013530","journal-title":"Semin Nephrol"},{"issue":"4","key":"455_CR11","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1038\/ki.2009.472","volume":"77","author":"F Bienaime","year":"2010","unstructured":"Bienaime F et al (2010) Mutations in components of complement influence the outcome of Factor I\u2011associated atypical hemolytic uremic syndrome. Kidney Int 77(4):339\u2013349","journal-title":"Kidney Int"},{"issue":"1\u20133","key":"455_CR12","doi-asserted-by":"publisher","first-page":"111","DOI":"10.1016\/j.molimm.2006.07.004","volume":"44","author":"A Richards","year":"2007","unstructured":"Richards A et\u00a0al (2007) Implications of the initial mutations in membrane cofactor protein (MCP; CD46) leading to atypical hemolytic uremic syndrome. Mol Immunol 44(1\u20133):111\u2013122","journal-title":"Mol Immunol"},{"issue":"15","key":"455_CR13","doi-asserted-by":"publisher","first-page":"2359","DOI":"10.1182\/blood-2014-10-609073","volume":"125","author":"EC Schramm","year":"2015","unstructured":"Schramm EC et\u00a0al (2015) Mapping interactions between complement C3 and regulators using mutations in atypical hemolytic uremic syndrome. Blood 125(15):2359\u20132369","journal-title":"Blood"},{"issue":"4","key":"455_CR14","doi-asserted-by":"publisher","first-page":"633","DOI":"10.1053\/j.ajkd.2014.01.434","volume":"64","author":"G Ardissino","year":"2014","unstructured":"Ardissino G et\u00a0al (2014) Discontinuation of eculizumab maintenance treatment for atypical hemolytic uremic syndrome: a\u00a0report of 10 cases. Am J Kidney Dis 64(4):633\u2013637","journal-title":"Am J Kidney Dis"},{"issue":"3","key":"455_CR15","doi-asserted-by":"publisher","first-page":"368","DOI":"10.1182\/blood-2017-02-770214","volume":"130","author":"SA Merrill","year":"2017","unstructured":"Merrill SA et\u00a0al (2017) Eculizumab cessation in atypical hemolytic uremic syndrome. Blood 130(3):368\u2013372","journal-title":"Blood"},{"key":"455_CR16","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfx196","author":"KL Wijnsma","year":"2017","unstructured":"Wijnsma KL et\u00a0al (2017) Safety and effectiveness of restrictive eculizumab treatment in atypical haemolytic uremic syndrome. Nephrol Dial Transplant. \n                    https:\/\/doi.org\/10.1093\/ndt\/gfx196","journal-title":"Nephrol Dial Transplant"},{"issue":"8","key":"455_CR17","doi-asserted-by":"publisher","first-page":"1694","DOI":"10.1111\/j.1600-6143.2008.02297.x","volume":"8","author":"M Quintrec Le","year":"2008","unstructured":"Le Quintrec M et al (2008) Complement mutation-associated de novo thrombotic microangiopathy following kidney transplantation. Am J Transplant 8(8):1694\u20131701","journal-title":"Am J Transplant"},{"issue":"3","key":"455_CR18","doi-asserted-by":"publisher","first-page":"663","DOI":"10.1111\/ajt.12077","volume":"13","author":"M Quintrec Le","year":"2013","unstructured":"Le Quintrec M et\u00a0al (2013) Complement genes strongly predict recurrence and graft outcome in adult renal transplant recipients with atypical hemolytic and uremic syndrome. Am J Transplant 13(3):663\u2013675","journal-title":"Am J Transplant"},{"key":"455_CR19","first-page":"262","volume":"6","author":"NS Merle","year":"2015","unstructured":"Merle NS et\u00a0al (2015) Complement system part I\u2014molecular mechanisms of activation and regulation. Front Immunol 6:262","journal-title":"Front Immunol"},{"issue":"2","key":"455_CR20","doi-asserted-by":"publisher","first-page":"178","DOI":"10.1111\/1744-9987.12641","volume":"22","author":"V Krishnappa","year":"2018","unstructured":"Krishnappa V et al (2018) Atypical hemolytic uremic syndrome: a meta-analysis of case reports confirms the prevalence of genetic mutations and the shift of treatment regimens. Ther Apher Dial 22(2):178\u2013188","journal-title":"Ther Apher Dial"},{"issue":"21","key":"455_CR21","doi-asserted-by":"publisher","first-page":"2847","DOI":"10.1182\/blood-2016-11-709865","volume":"129","author":"TS Jokiranta","year":"2017","unstructured":"Jokiranta TS (2017) HUS and atypical HUS. Blood 129(21):2847\u20132856","journal-title":"Blood"},{"issue":"17","key":"455_CR22","doi-asserted-by":"publisher","first-page":"1676","DOI":"10.1056\/NEJMra0902814","volume":"361","author":"M Noris","year":"2009","unstructured":"Noris M, Remuzzi G (2009) Atypical hemolytic-uremic syndrome. N\u00a0Engl J Med 361(17):1676\u20131687","journal-title":"N Engl J Med"},{"issue":"Suppl 4","key":"455_CR23","doi-asserted-by":"publisher","first-page":"iv131","DOI":"10.1093\/ndt\/gfu235","volume":"29","author":"JC Verhave","year":"2014","unstructured":"Verhave JC, Wetzels JF, van de Kar NC (2014) Novel aspects of atypical haemolytic uraemic syndrome and the role of eculizumab. Nephrol Dial Transplant 29(Suppl 4):iv131\u2013iv41","journal-title":"Nephrol Dial Transplant"},{"issue":"4","key":"455_CR24","doi-asserted-by":"publisher","first-page":"554","DOI":"10.2215\/CJN.04760512","volume":"8","author":"V Fremeaux-Bacchi","year":"2013","unstructured":"Fremeaux-Bacchi V et al (2013) Genetics and outcome of atypical hemolytic uremic syndrome: a\u00a0nationwide French series comparing children and adults. Clin J Am Soc Nephrol 8(4):554\u2013562","journal-title":"Clin J Am Soc Nephrol"},{"issue":"8","key":"455_CR25","doi-asserted-by":"publisher","first-page":"2392","DOI":"10.1681\/ASN.2006080811","volume":"18","author":"AL Sellier-Leclerc","year":"2007","unstructured":"Sellier-Leclerc AL et al (2007) Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome. J\u00a0Am Soc Nephrol 18(8):2392\u20132400","journal-title":"J Am Soc Nephrol"},{"key":"455_CR26","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1186\/1750-1172-6-60","volume":"6","author":"C Loirat","year":"2011","unstructured":"Loirat C, Fremeaux-Bacchi V (2011) Atypical hemolytic uremic syndrome. Orphanet J Rare Dis 6:60","journal-title":"Orphanet J Rare Dis"}],"container-title":["Der Internist"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00108-018-0455-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00108-018-0455-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00108-018-0455-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,7,10]],"date-time":"2019-07-10T19:33:09Z","timestamp":1562787189000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00108-018-0455-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,7,11]]},"references-count":26,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2018,8]]}},"alternative-id":["455"],"URL":"https:\/\/doi.org\/10.1007\/s00108-018-0455-9","relation":{},"ISSN":["0020-9554","1432-1289"],"issn-type":[{"value":"0020-9554","type":"print"},{"value":"1432-1289","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,7,11]]},"assertion":[{"value":"11 July 2018","order":1,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Einhaltung ethischer Richtlinien"}},{"value":"M.\u00a0Knoop gibt an, dass kein Interessenkonflikt besteht. H.\u00a0Haller und J.\u00a0Menne haben von der Firma Alexion, die Eculizumab zur Behandlung von Patienten mit aHUS herstellt, Beratungs- oder Vortragshonorare erhalten.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}},{"value":"Dieser Beitrag beinhaltet keine von den Autoren durchgef\u00fchrten Studien an Menschen oder Tieren.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics"}}]}}