{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,8,8]],"date-time":"2024-08-08T23:13:33Z","timestamp":1723158813317},"reference-count":0,"publisher":"National Library of Serbia","issue":"3-4","license":[{"start":{"date-parts":[[2012,1,1]],"date-time":"2012-01-01T00:00:00Z","timestamp":1325376000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Srp Arh Celok Lek","SRP ARH CELOK LEK","SRP ARK CELOK LEK","SRPSKI ARHIV CELOKUP"],"published-print":{"date-parts":[[2012]]},"abstract":"<jats:p>Introduction. Haemophagocytic lymphohistiocytosis (HLH) is a disorder\n   characterised by long-standing fever, splenomegaly and bicytopoenia or\n   pancytopoenia. Lymphadenopathy, jaundice and neurological symptoms may also\n   occur. HLH may ensue in various forms of innate or acquired immunodeficiency\n   with impaired cytotoxic lymphocyte function resulting in excessive macrophage\n   activation. Objective. To describe and analyse clinical characteristics of\n   patients treated for HLH at the University Children?s Hospital of Belgrade\n   from August 2000 to August 2010. Methods. Retrospective analysis of medical\n   records. Results. Diagnosis of HLH was established in 13 children (five boys\n   and eight girls) aged from one month to 14 years. In six children HLH was\n   secondary (to visceral leishmaniasis in two, Ebstein-Barr virus infection in\n   one, Langerhans? cell histiocytosis in one and systemic juvenile rheumatoid\n   arthritis in two). Of the remaining seven patients, genes for perforin and\n   syntaxin 11 were examined in two and no mutations were found. Of the\n   remaining seven patients, genes encoding perforin and syntaxin 11 were\n   analyzed in two, but no mutations were found. All children had fever,\n   splenomegaly, cytopoenias, hyperferritinaemia and hypertriglyceridaemia, but\n   haemophagocytosis was seen in only six (46.1%). Six children were cured (four\n   with secondary HLH and two with primary HLH). Two children are undergoing\n   treatment, while five succumbed (three before treatment could be administered\n   and two due to complications). In four of the six cured children, HLH arose\n   in the first year of life. Cure rate in those who underwent haematopoietic\n   stem cell trans- plantation was 2\/3. Conclusion. Results underscore the\n   importance of timely diagnosis and treatment of HLH, warranting that in all\n   children with fever, splenomegaly and\/or cytopoenias, with or without\n   haemophagocytosis, HLH be actively sought.<\/jats:p>","DOI":"10.2298\/sarh1204191k","type":"journal-article","created":{"date-parts":[[2012,5,9]],"date-time":"2012-05-09T13:23:11Z","timestamp":1336569791000},"page":"191-197","source":"Crossref","is-referenced-by-count":3,"title":["Clinical characteristics and disease course in children with haemophagocytic lymphohistiocytosis treated at the University children\u2019s hospital in Belgrade"],"prefix":"10.2298","volume":"140","author":[{"given":"Lidija","family":"Krivokapic-Dokmanovic","sequence":"first","affiliation":[{"name":"Medicinski fakultet, Beograd + Univerzitetska de\u010dja klinika, Beograd"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nada","family":"Krstovski","sequence":"additional","affiliation":[{"name":"Medicinski fakultet, Beograd + Univerzitetska de\u010dja klinika, Beograd"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Srdja","family":"Jankovic","sequence":"additional","affiliation":[{"name":"Univerzitetska de\u010dja klinika, Beograd"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jelena","family":"Lazic","sequence":"additional","affiliation":[{"name":"Univerzitetska de\u010dja klinika, Beograd"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nedeljko","family":"Radlovic","sequence":"additional","affiliation":[{"name":"Medicinski fakultet, Beograd + Univerzitetska de\u010dja klinika, Beograd"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dragana","family":"Janic","sequence":"additional","affiliation":[{"name":"Medicinski fakultet, Beograd + Univerzitetska de\u010dja klinika, Beograd"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1078","container-title":["Srpski arhiv za celokupno lekarstvo"],"original-title":[],"language":"en","deposited":{"date-parts":[[2023,5,29]],"date-time":"2023-05-29T10:31:44Z","timestamp":1685356304000},"score":1,"resource":{"primary":{"URL":"https:\/\/doiserbia.nb.rs\/Article.aspx?ID=0370-81791204191K"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012]]},"references-count":0,"journal-issue":{"issue":"3-4","published-print":{"date-parts":[[2012]]}},"URL":"https:\/\/doi.org\/10.2298\/sarh1204191k","relation":{},"ISSN":["0370-8179","2406-0895"],"issn-type":[{"value":"0370-8179","type":"print"},{"value":"2406-0895","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012]]}}}