{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T06:21:11Z","timestamp":1790144471390,"version":"4.0.1"},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2015,4,25]],"date-time":"2015-04-25T00:00:00Z","timestamp":1429920000000},"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":["Pediatr Nephrol"],"published-print":{"date-parts":[[2015,7]]},"DOI":"10.1007\/s00467-015-3102-x","type":"journal-article","created":{"date-parts":[[2015,4,24]],"date-time":"2015-04-24T07:14:41Z","timestamp":1429859681000},"page":"1197-1202","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":35,"title":["Renal involvement in the immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) disorder"],"prefix":"10.1007","volume":"30","author":[{"given":"Yuri","family":"Sheikine","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Craig B.","family":"Woda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pui Y.","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Talal A.","family":"Chatila","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sevgi","family":"Keles","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis-Marie","family":"Charbonnier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Birgitta","family":"Schmidt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seymour","family":"Rosen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nancy M.","family":"Rodig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2015,4,25]]},"reference":[{"key":"3102_CR1","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1038\/nrendo.2010.40","volume":"6","author":"AW Michels","year":"2010","unstructured":"Michels AW, Gottlieb PA (2010) Autoimmune polyglandular syndromes. Nat Rev Endocrinol 6:270\u2013277","journal-title":"Nat Rev Endocrinol"},{"key":"3102_CR2","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1016\/S0022-3476(82)80573-8","volume":"100","author":"BR Powell","year":"1982","unstructured":"Powell BR, Buist NR, Stenzel P (1982) An X-linked syndrome of diarrhea, polyendocrinopathy, and fatal infection in infancy. J Pediatr 100:731\u2013737","journal-title":"J Pediatr"},{"key":"3102_CR3","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1111\/j.1365-3083.2011.02574.x","volume":"74","author":"YF An","year":"2011","unstructured":"An YF, Xu F, Wang M, Zhang ZY, Zhao XD (2011) Clinical and molecular characteristics of immunodysregulation, polyendocrinopathy, enteropathy, X-linked syndrome in China. Scand J Immunol 74:304\u2013309","journal-title":"Scand J Immunol"},{"key":"3102_CR4","doi-asserted-by":"crossref","first-page":"R75","DOI":"10.1172\/JCI11679","volume":"106","author":"TA Chatila","year":"2000","unstructured":"Chatila TA, Blaeser F, Ho N, Lederman HM, Voulgaropoulos C, Helms C, Bowcock AM (2000) JM2, encoding a fork head-related protein, is mutated in X-linked autoimmunity-allergic disregulation syndrome. J Clin Invest 106:R75\u201381","journal-title":"J Clin Invest"},{"key":"3102_CR5","doi-asserted-by":"crossref","first-page":"1799","DOI":"10.1007\/s00467-007-0532-0","volume":"22","author":"A Moudgil","year":"2007","unstructured":"Moudgil A, Perriello P, Loechelt B, Przygodzki R, Fitzerald W, Kamani N (2007) Immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome: an unusual cause of proteinuria in infancy. Pediatr Nephrol 22:1799\u20131802","journal-title":"Pediatr Nephrol"},{"key":"3102_CR6","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/j.jpeds.2005.04.017","volume":"147","author":"L Bindl","year":"2005","unstructured":"Bindl L, Torgerson T, Perroni L, Youssef N, Ochs HD, Goulet O, Ruemmele FM (2005) Successful use of the new immune-suppressor sirolimus in IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome). J Pediatr 147:256\u2013259","journal-title":"J Pediatr"},{"key":"3102_CR7","doi-asserted-by":"crossref","first-page":"98","DOI":"10.2350\/06-07-0130.1","volume":"10","author":"ML Heltzer","year":"2007","unstructured":"Heltzer ML, Choi JK, Ochs HD, Sullivan KE, Torgerson TR, Ernst LM (2007) A potential screening tool for IPEX syndrome. Pediatr Dev Pathol 10:98\u2013105","journal-title":"Pediatr Dev Pathol"},{"key":"3102_CR8","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1016\/j.jaci.2010.05.021","volume":"126","author":"LM Burroughs","year":"2010","unstructured":"Burroughs LM, Torgerson TR, Storb R, Carpenter PA, Rawlings DJ, Sanders J, Scharenberg AM, Skoda-Smith S, Englund J, Ochs HD, Woolfrey AE (2010) Stable hematopoietic cell engraftment after low-intensity nonmyeloablative conditioning in patients with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. J Allergy Clin Immunol 126:1000\u20131005","journal-title":"J Allergy Clin Immunol"},{"key":"3102_CR9","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1038\/modpathol.2008.161","volume":"22","author":"N Patey-Mariaud de Serre","year":"2009","unstructured":"Patey-Mariaud de Serre N, Canioni D, Ganousse S, Rieux-Laucat F, Goulet O, Ruemmele F, Brousse N (2009) Digestive histopathological presentation of IPEX syndrome. Mod Pathol 22:95\u2013102","journal-title":"Mod Pathol"},{"key":"3102_CR10","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1136\/jmg.38.12.874","volume":"38","author":"I Kobayashi","year":"2001","unstructured":"Kobayashi I, Shiari R, Yamada M, Kawamura N, Okano M, Yara A, Iguchi A, Ishikawa N, Ariga T, Sakiyama Y, Ochs HD, Kobayashi K (2001) Novel mutations of FOXP3 in two Japanese patients with immune dysregulation, polyendocrinopathy, enteropathy, X linked syndrome (IPEX). J Med Genet 38:874\u2013876","journal-title":"J Med Genet"},{"key":"3102_CR11","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1053\/j.gastro.2010.06.006","volume":"139","author":"N Moes","year":"2010","unstructured":"Moes N, Rieux-Laucat F, Begue B, Verdier J, Neven B, Patey N, Torgerson TT, Picard C, Stolzenberg MC, Ruemmele C, Rings EH, Casanova JL, Piloquet H, Biver A, Breton A, Ochs HD, Hermine O, Fischer A, Goulet O, Cerf-Bensussan N, Ruemmele FM (2010) Reduced expression of FOXP3 and regulatory T-cell function in severe forms of early-onset autoimmune enteropathy. Gastroenterology 139:770\u2013778","journal-title":"Gastroenterology"},{"key":"3102_CR12","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.clim.2011.06.006","volume":"141","author":"K Otsubo","year":"2011","unstructured":"Otsubo K, Kanegane H, Kamachi Y, Kobayashi I, Tsuge I, Imaizumi M, Sasahara Y, Hayakawa A, Nozu K, Iijima K, Ito S, Horikawa R, Nagai Y, Takatsu K, Mori H, Ochs HD, Miyawaki T (2011) Identification of FOXP3-negative regulatory T-like (CD4(+)CD25(+)CD127(low)) cells in patients with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. Clin Immunol 141:111\u2013120","journal-title":"Clin Immunol"},{"key":"3102_CR13","doi-asserted-by":"crossref","first-page":"1181","DOI":"10.1007\/s00467-009-1119-8","volume":"24","author":"Y Hashimura","year":"2009","unstructured":"Hashimura Y, Nozu K, Kanegane H, Miyawaki T, Hayakawa A, Yoshikawa N, Nakanishi K, Takemoto M, Iijima K, Matsuo M (2009) Minimal change nephrotic syndrome associated with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. Pediatr Nephrol 24:1181\u20131186","journal-title":"Pediatr Nephrol"},{"key":"3102_CR14","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/j.jaut.2010.06.017","volume":"35","author":"M Tsuda","year":"2010","unstructured":"Tsuda M, Torgerson TR, Selmi C, Gambineri E, Carneiro-Sampaio M, Mannurita SC, Leung PS, Norman GL, Gershwin ME (2010) The spectrum of autoantibodies in IPEX syndrome is broad and includes anti-mitochondrial autoantibodies. J Autoimmun 35:265\u2013268","journal-title":"J Autoimmun"},{"key":"3102_CR15","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1136\/jmg.39.8.537","volume":"39","author":"RS Wildin","year":"2002","unstructured":"Wildin RS, Smyk-Pearson S, Filipovich AH (2002) Clinical and molecular features of the immunodysregulation, polyendocrinopathy, enteropathy, X linked (IPEX) syndrome. J Med Genet 39:537\u2013545","journal-title":"J Med Genet"}],"container-title":["Pediatric Nephrology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-015-3102-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00467-015-3102-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-015-3102-x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,5,28]],"date-time":"2019-05-28T21:07:09Z","timestamp":1559077629000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00467-015-3102-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,4,25]]},"references-count":15,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2015,7]]}},"alternative-id":["3102"],"URL":"https:\/\/doi.org\/10.1007\/s00467-015-3102-x","relation":{},"ISSN":["0931-041X","1432-198X"],"issn-type":[{"value":"0931-041X","type":"print"},{"value":"1432-198X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,4,25]]}}}