{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T14:38:37Z","timestamp":1761057517657},"reference-count":14,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2002,9,3]],"date-time":"2002-09-03T00:00:00Z","timestamp":1031011200000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2002,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Variable increases in chitotriosidase levels have been reported in Italian patients with \u03b2\u2010thalassemia major and intermedia. We measured plasma chitotriosidase levels in Israeli patients with \u03b2\u2010thalassemia to ascertain its use as a universal marker of disease and\/or response to therapy. Chitotriosidase levels in 39 adults (16\u201353 years; 30 with \u03b2\u2010thalassemia major, 9 with intermedia), and in 14 children (0.7\u201315 years; 12 with \u03b2\u2010thalassemia major, 2 with intermedia) were compared with other measures of disease, such as ferritin, hemoglobin, liver function tests, and genotype. Plasma chitotriosidase levels were normal (0.37 \u00b1 0.04 mU\/mL) in all children. Twelve adults (31%) had elevated levels (&gt;1.33 mU\/mL): 11 patients (37%) with thalassemia major and 1 patient (11%) with thalassemia intermedia. A significant correlation was only found between plasma chitotriosidase levels and ferritin levels, and with mean number of transfusions per year. The patient with the highest chitotriosidase (1,440 nmol\/mL\/hr) had the highest ferritin (5,175 \u03bcg\/L), required the most transfusions per year (40), and had abnormal liver tests. Normal chitotriosidase levels in the pediatric cohort and increased levels in only some adults may reflect status of iron overload in macrophages; thus there may be a role for monitoring chitotriosidase in patients with \u03b2\u2010thalassemia. Our results confirm results of the Italian cohort; however, in the latter, a more universal correlation was noted and chitotriosidase levels were much higher. Am. J. Hematol. 71:7\u201310, 2002. \u00a9 2002 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.10151","type":"journal-article","created":{"date-parts":[[2002,10,8]],"date-time":"2002-10-08T23:01:00Z","timestamp":1034118060000},"page":"7-10","source":"Crossref","is-referenced-by-count":18,"title":["Plasma chitotriosidase activity in patients with \u03b2\u2010thalassemia"],"prefix":"10.1002","volume":"71","author":[{"given":"G.","family":"Altarescu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Rudensky","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Abrahamov","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Goldfarb","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Rund","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Zimran","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Elstein","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2002,9,3]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.5.2198"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.44.26252"},{"key":"e_1_2_1_4_2","doi-asserted-by":"crossref","first-page":"1288","DOI":"10.1172\/JCI117084","article-title":"Marked elevation of plasma chitotriosidase activity. A novel hallmark of Gaucher disease","volume":"98","author":"Hollak CEM","year":"1994","journal-title":"J Clin Invest"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0950-3536(97)80042-X"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005367328003"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0009-9120(98)00030-7"},{"key":"e_1_2_1_8_2","first-page":"2","article-title":"Plasma chitotriosidase activity in patients with beta\u2010thalassemia","volume":"25","author":"Barone R","year":"1998","journal-title":"Blood Cell Mol Dis"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1159\/000046546"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0009-8981(01)00398-9"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF02436762"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF02682034"},{"key":"e_1_2_1_13_2","unstructured":"MeraitouM DimitriouE MichelakakisH.Iron overload and chitotriosidase activity in rat tissues. 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