{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T18:12:20Z","timestamp":1793211140304,"version":"4.5.0"},"reference-count":46,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2014,5,16]],"date-time":"2014-05-16T00:00:00Z","timestamp":1400198400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2014,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Gaucher disease (GD) is caused by deficiency of the enzyme glucocerebrosidase catalysing the regular lysosomal degradation of glucosylceramide. In the common non\u2010neuropathic variant of GD, glucosylceramide\u2010laden macrophages (Gaucher cells) accumulate in various tissues. Gaucher cells secrete chitotriosidase, an active chitinase, resulting in increased plasma chitotriosidase levels, which can be sensitively monitored by an enzyme activity assay. Plasma chitotriosidase is a rough estimate of body burden of Gaucher cells. Non\u2010neuronopathic GD is presently treated by enzyme replacement therapy (ERT) and substrate reduction therapy (SRT). We addressed the question whether plasma chitotriosidase acts as (predictive) marker of clinical manifestations in non\u2010neuronopathic GD patients receiving treatment. Reductions in plasma chitotriosidase during therapy correlated with corrections in liver and spleen volumes and showed positive trends with improvements in haemoglobin and platelet count and bone marrow composition. The occurrence of long\u2010term complications and associated conditions such as multiple myeloma, bone complications, Parkinson's disease, hepatocellular carcinoma and pulmonary hypertension positively correlated with the plasma chitotriosidase level pre\u2010therapy, the average plasma chitotriosidase during 3 years of ERT and the residual plasma chitotriosidase after 2 years of ERT. In summary, plasma chitotriosidase is a valuable marker in the assessment and follow\u2010up of GD patients.<\/jats:p>","DOI":"10.1007\/s10545-014-9711-x","type":"journal-article","created":{"date-parts":[[2014,5,15]],"date-time":"2014-05-15T17:57:01Z","timestamp":1400176621000},"page":"991-1001","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":66,"title":["Value of plasma chitotriosidase to assess non\u2010neuronopathic Gaucher disease severity and progression in the era of enzyme replacement therapy"],"prefix":"10.1002","volume":"37","author":[{"given":"L.","family":"van Dussen","sequence":"first","affiliation":[{"name":"Department of Endocrinology and Metabolism, Academic Medical Center University of Amsterdam PO box 22660 Amsterdam 1100 DD The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E. 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