{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T15:13:06Z","timestamp":1787238786916,"version":"build-2736575974"},"reference-count":15,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2009,7,8]],"date-time":"2009-07-08T00:00:00Z","timestamp":1247011200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2009,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of the immune system caused by inadequate induction of apoptosis via the Fas pathway, mainly characterized by generalized lymphadenopathy, splenomegaly, and autoimmune cytopenias, as well as increased risk of lymphoma. Although the clinical course of ALPS is highly variable, without treatment long\u2010term prognosis is unsatisfactory for most patients. ALPS has been treated with most of the existing immunosuppressive agents, with variable success. We hereby present a case of a child with ALPS whose greatly enlarged lymph nodes rapidly regressed upon initiation of rapamycin, a novel potential therapeutic agent in the treatment of ALPS. Pediatr Blood Cancer 2009;53:1117\u20131119. \u00a9 2009 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/pbc.22151","type":"journal-article","created":{"date-parts":[[2009,7,9]],"date-time":"2009-07-09T21:33:10Z","timestamp":1247175190000},"page":"1117-1119","source":"Crossref","is-referenced-by-count":17,"title":["Rapid regression of lymphadenopathy upon rapamycin treatment in a child with autoimmune lymphoproliferative syndrome"],"prefix":"10.1002","volume":"53","author":[{"given":"Jani\u0107 Mihailo","family":"Dragana","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bra\u0161anac \u010cedomir","family":"Dimitrije","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jankovi\u0107 Jovan","family":"Sr\u0111a","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dokmanovi\u0107 Bogdan","family":"Lidija","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krstovski Radmio","family":"Nada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kraguljac Kurtovi\u0107 Janko","family":"Nada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,7,8]]},"reference":[{"key":"e_1_2_4_2_2","doi-asserted-by":"publisher","DOI":"10.2174\/1381612033392107"},{"key":"e_1_2_4_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/s100380050029"},{"key":"e_1_2_4_4_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V89.4.1341"},{"key":"e_1_2_4_5_2","doi-asserted-by":"publisher","DOI":"10.1080\/10428190310001593166"},{"key":"e_1_2_4_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.09.025"},{"key":"e_1_2_4_7_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1701306"},{"key":"e_1_2_4_8_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-01-010124"},{"key":"e_1_2_4_9_2","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e31802d7503"},{"key":"e_1_2_4_10_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2002.03753.x"},{"key":"e_1_2_4_11_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2002.03357.x"},{"key":"e_1_2_4_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21007"},{"key":"e_1_2_4_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21965"},{"key":"e_1_2_4_14_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2009.07595.x"},{"key":"e_1_2_4_15_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.2436348100"},{"key":"e_1_2_4_16_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M504192200"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.22151","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.22151","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.22151","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,18]],"date-time":"2023-10-18T14:19:06Z","timestamp":1697638746000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.22151"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,7,8]]},"references-count":15,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2009,12]]}},"alternative-id":["10.1002\/pbc.22151"],"URL":"https:\/\/doi.org\/10.1002\/pbc.22151","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,7,8]]}}}