{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T20:23:24Z","timestamp":1773174204169,"version":"3.50.1"},"reference-count":16,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,11,17]],"date-time":"2020-11-17T00:00:00Z","timestamp":1605571200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2020,11,17]],"date-time":"2020-11-17T00:00:00Z","timestamp":1605571200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Nephrol"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n<jats:title>Background<\/jats:title>\n<jats:p>Pregnancy-related Atypical Haemolytic Uremic Syndrome (P-aHUS) is a rare condition affecting genetically predisposed women during pregnancy. It is often difficult to diagnose and has a significant impact on maternal and foetal outcomes. It is characterised by microangiopathic haemolytic anaemia and kidney injury from thrombotic microangiopathy.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Case presentation<\/jats:title>\n<jats:p>A 27-year-old female of Lebanese descent presented at 36\u2009weeks\u2019 gestation with foetal death in-utero (FDIU) with placental abruption on a background of previously normal antenatal visits. She was coagulopathic and anaemic with anuric acute kidney injury, requiring emergency Caesarean section, intubation and dialysis. Her coagulopathy rapidly resolved, however, her anaemia and renal dysfunction persisted. A diagnosis of P-aHUS was made, and she was empirically treated with Eculizumab. Her ADAMTS13 level was normal, effectively excluding thrombotic thrombocytopenic purpura. Within 2\u2009weeks of treatment her haematological parameters improved, and her renal function began to recover and within 2\u2009months she became dialysis independent.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusion<\/jats:title>\n<jats:p>This case highlights the challenges of a timely diagnosis of P-aHUS from other pregnancy-related diseases. Although our patient is dialysis-independent, her risk of relapse remains high with subsequent pregnancies. Currently we are awaiting her genetic sequencing to complete her assessment for underlying mutations and are determining the safest approach to a future planned pregnancy.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s12882-020-02100-4","type":"journal-article","created":{"date-parts":[[2020,11,17]],"date-time":"2020-11-17T09:04:53Z","timestamp":1605603893000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["A life-threatening case of pregnancy-related atypical Haemolytic uremic syndrome and successful treatment with Eculizumab"],"prefix":"10.1186","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0752-4651","authenticated-orcid":false,"given":"Prianka","family":"Puri","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anida","family":"Hanxhiu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel V.","family":"O\u2019Hara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Danny","family":"Hsu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mirna","family":"Vucak-Dzumhur","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,11,17]]},"reference":[{"issue":"12","key":"2100_CR1","doi-asserted-by":"publisher","first-page":"2071","DOI":"10.2215\/CJN.09450912","volume":"7","author":"P August","year":"2012","unstructured":"August P. Obstetric nephrology: pregnancy and the kidney--inextricably linked. Clin J Am Soc Nephrol CJASN. 2012;7(12):2071\u20132.","journal-title":"Clin J Am Soc Nephrol CJASN"},{"issue":"4","key":"2100_CR2","first-page":"239","volume":"17","author":"K Richani","year":"2005","unstructured":"Richani K, Soto E, Romero R, Espinoza J, Chaiworapongsa T, Nien JK, et al. Normal pregnancy is characterized by systemic activation of the complement system. J Matern-Fetal Neonatal Med Off J Eur Assoc Perinat Med Fed Asia Ocean Perinat Soc Int Soc Perinat Obstet. 2005;17(4):239\u201345.","journal-title":"J Matern-Fetal Neonatal Med Off J Eur Assoc Perinat Med Fed Asia Ocean Perinat Soc Int Soc Perinat Obstet"},{"issue":"5","key":"2100_CR3","doi-asserted-by":"publisher","first-page":"859","DOI":"10.1681\/ASN.2009070706","volume":"21","author":"F Fakhouri","year":"2010","unstructured":"Fakhouri F, Roumenina L, Provot F, Sall\u00e9e M, Caillard S, Couzi L, et al. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J Am Soc Nephrol JASN. 2010;21(5):859\u201367.","journal-title":"J Am Soc Nephrol JASN"},{"key":"2100_CR4","unstructured":"Eculizumab in pregnancy: a narrative overview. - Abstract - Europe PMC [Internet]. [cited 2020 Apr 26]. Available from: https:\/\/europepmc.org\/article\/med\/30159857."},{"key":"2100_CR5","doi-asserted-by":"crossref","unstructured":"Stefanovic V. The Extended Use of Eculizumab in Pregnancy and Complement Activation\u2212Associated Diseases Affecting Maternal, Fetal andNeonatal Kidneys-The Future Is Now? J Clin Med. 2019;8(3).","DOI":"10.3390\/jcm8030407"},{"key":"2100_CR6","doi-asserted-by":"publisher","unstructured":"Regal JF, Gilbert JS, Burwick RM. The complement system and adverse pregnancy outcomes. Mol Immunol. 2015;67(1):56-70. https:\/\/doi.org\/10.1016\/j.molimm.2015.02.030. Epub 2015 Mar 21. PMID: 25802092; PMCID: PMC4447554.","DOI":"10.1016\/j.molimm.2015.02.030"},{"issue":"8","key":"2100_CR7","doi-asserted-by":"publisher","first-page":"1237","DOI":"10.2215\/CJN.00280117","volume":"12","author":"A Bruel","year":"2017","unstructured":"Bruel A, Kavanagh D, Noris M, Delmas Y, Wong EKS, Bresin E, et al. Hemolytic uremic syndrome in pregnancy and postpartum. Clin J Am Soc Nephrol CJASN. 2017;12(8):1237\u201347.","journal-title":"Clin J Am Soc Nephrol CJASN"},{"issue":"47","key":"2100_CR8","first-page":"795","volume":"112","author":"F Bergmann","year":"2015","unstructured":"Bergmann F, Rath W. The differential diagnosis of thrombocytopenia in pregnancy. Dtsch \u00c4rztebl Int. 2015;112(47):795\u2013802.","journal-title":"Dtsch \u00c4rztebl Int"},{"issue":"11","key":"2100_CR9","doi-asserted-by":"publisher","first-page":"1032","DOI":"10.1056\/NEJMoa1502950","volume":"373","author":"RJ Kelly","year":"2015","unstructured":"Kelly RJ, H\u00f6chsmann B, Szer J, Kulasekararaj A, de Guibert S, R\u00f6th A, et al. Eculizumab in pregnant patients with paroxysmal nocturnal Hemoglobinuria. N Engl J Med. 2015 Sep 10;373(11):1032\u20139.","journal-title":"N Engl J Med"},{"issue":"12","key":"2100_CR10","first-page":"2122","volume":"31","author":"A Servais","year":"2016","unstructured":"Servais A, Devillard N, Fr\u00e9meaux-Bacchi V, Hummel A, Salomon L, Contin-Bordes C, et al. Atypical haemolytic uraemic syndrome and pregnancy: outcome with ongoing eculizumab. Nephrol Dial Transplant Off Publ Eur Dial Transpl Assoc - Eur Ren Assoc. 2016;31(12):2122\u201330.","journal-title":"Nephrol Dial Transplant Off Publ Eur Dial Transpl Assoc - Eur Ren Assoc"},{"issue":"2","key":"2100_CR11","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1016\/j.kint.2017.06.022","volume":"93","author":"A Huerta","year":"2018","unstructured":"Huerta A, Arjona E, Portoles J, Lopez-Sanchez P, Rabasco C, Espinosa M, et al. A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome. Kidney Int. 2018;93(2):450\u20139.","journal-title":"Kidney Int"},{"issue":"21","key":"2100_CR12","doi-asserted-by":"publisher","first-page":"3253","DOI":"10.1182\/blood-2014-09-600411","volume":"125","author":"R Cofiell","year":"2015","unstructured":"Cofiell R, Kukreja A, Bedard K, Yan Y, Mickle AP, Ogawa M, et al. Eculizumab reduces complement activation, inflammation, endothelial damage, thrombosis, and renal injury markers in aHUS. Blood. 2015 May 21;125(21):3253\u201362.","journal-title":"Blood."},{"key":"2100_CR13","unstructured":"Health AGD of. Pharmaceutical Benefits Scheme (PBS) Australian Government Department of Health. culizumab for aHUS: 24 month predicted versus actual analysis, published 2017. Viewed April 26 2020. http:\/\/www.pbs.gov.au\/info\/industry\/listing\/participants\/public-release-docs\/2017-09\/eculizumabfor-ahus."},{"issue":"4","key":"2100_CR14","doi-asserted-by":"publisher","first-page":"554","DOI":"10.2215\/CJN.04760512","volume":"8","author":"V Fremeaux-Bacchi","year":"2013","unstructured":"Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaim\u00e9 F, Dragon-Durey M-A, 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 CJASN. 2013;8(4):554\u201362.","journal-title":"Clin J Am Soc Nephrol CJASN."},{"issue":"1","key":"2100_CR15","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1038\/nrneph.2010.155","volume":"7","author":"J Zuber","year":"2011","unstructured":"Zuber J, Le Quintrec M, Sberro-Soussan R, Loirat C, Fr\u00e9meaux-Bacchi V, Legendre C. New insights into postrenal transplant hemolytic uremic syndrome. Nat Rev Nephrol. 2011 Jan;7(1):23\u201335.","journal-title":"Nat Rev Nephrol"},{"issue":"1","key":"2100_CR16","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1681\/ASN.2013050453","volume":"25","author":"F Bu","year":"2014","unstructured":"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 JASN. 2014;25(1):55\u201364.","journal-title":"J Am Soc Nephrol JASN"}],"container-title":["BMC Nephrology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12882-020-02100-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s12882-020-02100-4\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12882-020-02100-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,11,17]],"date-time":"2020-11-17T09:10:40Z","timestamp":1605604240000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcnephrol.biomedcentral.com\/articles\/10.1186\/s12882-020-02100-4"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,11,17]]},"references-count":16,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,12]]}},"alternative-id":["2100"],"URL":"https:\/\/doi.org\/10.1186\/s12882-020-02100-4","relation":{},"ISSN":["1471-2369"],"issn-type":[{"value":"1471-2369","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,11,17]]},"assertion":[{"value":"25 June 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"12 October 2020","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 November 2020","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The study was approved by Westmead Hospitals Health Human Research Ethics Committee. Consent for participation and obtained both verbally and on attached consent form from the participant.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Patient consent was obtained prior to publication and patient completed a written consent form (can be supplied). All Authors contested to publication. All patient information was deidentified.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"There were no financial supports or relationships that may pose conflict of interest for any authors involved in the drafting of this manuscript. No Authors have and conflicting interests to declare.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"488"}}