{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T10:46:29Z","timestamp":1793357189525,"version":"4.5.0"},"reference-count":17,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2017,9,20]],"date-time":"2017-09-20T00:00:00Z","timestamp":1505865600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Transfusion"],"published-print":{"date-parts":[[2017,12]]},"abstract":"<jats:sec><jats:title>BACKGROUND<\/jats:title><jats:p>Congenital thrombotic thrombocytopenic purpura (TTP) may not be recognized until organ failure related to the microvascular thrombosis occurs. Kidney failure may be the initial presenting clinical feature. Kidney transplantation has been contraindicated because of the assumption that the continuing microvascular thrombosis will cause inevitable graft failure.<\/jats:p><\/jats:sec><jats:sec><jats:title>CASE REPORT<\/jats:title><jats:p>We report a 48\u2010year\u2010old nulliparous woman who presented with end\u2010stage kidney disease that was attributed to hypertension. Her past history included a thromboembolic stroke at age 32, for which she was placed on permanent anticoagulation. Immediately after living unrelated\u2010donor kidney transplant, she developed severe hemolysis and acute decline in urine output for which she received red blood cell and platelet transfusions and an infusion of eculizumab (1200 mg). She promptly responded and was discharged on her fifth postoperative day with a serum creatinine level of 1.0 mg\/dL. Two weeks later, thrombocytopenia and hemolysis recurred. By this time, undetectable ADAMTS13 activity (&lt;5%) with no demonstrable inhibitor had been reported. She responded rapidly to plasma infusions. Genetic analysis confirmed the diagnosis of congenital TTP, documenting known pathogenic mutations in each of the <jats:italic>ADAMTS13<\/jats:italic> genes. She continued to receive twice\u2010monthly infusions for 4 months. Surveillance kidney biopsies at 6 and 12 months posttransplant demonstrated no evidence of thrombotic microangiopathy or graft rejection. After 2 years of follow\u2010up her creatinine remains stable at 1.0 mg\/dL (estimated glomerular filtration rate, 65 mL\/min\/1.73 m<jats:sup>2<\/jats:sup>).<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSION<\/jats:title><jats:p>Our experience suggests that kidney transplantation may be an appropriate management for carefully selected patients with congenital TTP who develop end\u2010stage renal disease.<\/jats:p><\/jats:sec>","DOI":"10.1111\/trf.14326","type":"journal-article","created":{"date-parts":[[2017,9,21]],"date-time":"2017-09-21T03:02:42Z","timestamp":1505962962000},"page":"3058-3062","source":"Crossref","is-referenced-by-count":10,"title":["Successful kidney transplantation in a patient with congenital thrombotic thrombocytopenic purpura (Upshaw\u2010Schulman syndrome)"],"prefix":"10.1111","volume":"57","author":[{"given":"Hasan","family":"Fattah","sequence":"first","affiliation":[{"name":"Division of Nephrology Richmond Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dhiren","family":"Kumar","sequence":"additional","affiliation":[{"name":"Division of Nephrology Richmond Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James N.","family":"George","sequence":"additional","affiliation":[{"name":"Departments of Epidemiology &amp; Biostatistics and Medicine University of Oklahoma Health Sciences Center Oklahoma City Oklahoma"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. Davis","family":"Massey","sequence":"additional","affiliation":[{"name":"Department of Pathology Virginia Commonwealth University Richmond Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne L.","family":"King","sequence":"additional","affiliation":[{"name":"Division of Nephrology Richmond Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth D.","family":"Friedman","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology Medical College of Wisconsin Milwaukee Wisconsin."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gaurav","family":"Gupta","sequence":"additional","affiliation":[{"name":"Division of Nephrology Richmond Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,9,20]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21481"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.5482\/HAMO-13-04-0026"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24612"},{"key":"e_1_2_8_5_1","first-page":"731","article-title":"The kidney in thrombotic thrombocytopenic purpura","volume":"98","author":"Tsai HM.","year":"2007","journal-title":"Minerva Med"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2369-14-156"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7933.2004.00623.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/jca.21251"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2002.tb00099.x"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2013050453"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2011.04341.x"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-02-408914"},{"key":"e_1_2_8_13_1","first-page":"214","article-title":"von Willebrand factor and factor VIII in renal transplant recipients under immunosuppression with cyclosporine and steroids. Sequential measurements over 4 months in 17 patients","volume":"34","author":"Huser B","year":"1990","journal-title":"Clin Nephrol"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOT.0000000000000090"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.ajkd.2015.06.032"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrneph.2012.195"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12185-015-1933-7"},{"key":"e_1_2_8_18_1","doi-asserted-by":"crossref","unstructured":"ScullyMK KentoucheK RiceL et al. Pharmacodynamic profile of a recombinant ADAMTS13 (BAX930) in hereditary thrombotic thrombocytopenic purpura (Upshaw\u2010Schulman Syndrome [USS]). American Society of Hematology (ASH)2016; San Diego CA.","DOI":"10.1182\/blood.V128.22.135.135"}],"container-title":["Transfusion"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Ftrf.14326","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/trf.14326","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T05:53:57Z","timestamp":1694757237000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/trf.14326"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,9,20]]},"references-count":17,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2017,12]]}},"alternative-id":["10.1111\/trf.14326"],"URL":"https:\/\/doi.org\/10.1111\/trf.14326","archive":["Portico"],"relation":{},"ISSN":["0041-1132","1537-2995"],"issn-type":[{"value":"0041-1132","type":"print"},{"value":"1537-2995","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,9,20]]}}}