Clinical evaluation of chemokine and enzymatic biomarkers of Gaucher disease
Autor: | Timothy M. Cox, J. Paul Schofield, Ian G. McFarlane, Patrick Deegan, Mary Teresa Moran, Rolf G. Boot, Johannes M. F. G. Aerts |
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Přispěvatelé: | AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, Medical Biochemistry, ACS - Amsterdam Cardiovascular Sciences |
Jazyk: | angličtina |
Rok vydání: | 2005 |
Předmět: |
Adult
Male medicine.medical_specialty Chemokine Adolescent Imiglucerase Enzyme-Linked Immunosorbent Assay Spleen Severity of Illness Index Gastroenterology Internal medicine medicine Humans Platelet Molecular Biology Aged Aged 80 and over chemistry.chemical_classification Gaucher Disease biology Platelet Count business.industry Chitinases CCL18 Cell Biology Hematology Enzyme replacement therapy Clinical Enzyme Tests Middle Aged Enzymes Enzyme medicine.anatomical_structure chemistry Case-Control Studies Chemokines CC Splenomegaly Immunology biology.protein Glucosylceramidase Molecular Medicine Biomarker (medicine) Female Chemokines Drug Monitoring business Biomarkers Hepatomegaly medicine.drug |
Zdroj: | Blood cells, molecules & diseases, 35(2), 259-267. Academic Press Inc. |
ISSN: | 1079-9796 |
DOI: | 10.1016/j.bcmd.2005.05.005 |
Popis: | Purpose: Gaucher disease is an exemplary orphan disorder. Enzyme replacement therapy with imiglucerase is effective, but very expensive. To improve the assessment of severity of disease and responses to this costly treatment, we have evaluated several enzymatic biomarkers and a newly-described chemokine. Subjects and methods: We studied 48 untreated adults with Type I Gaucher disease: 20 patients were studied after the introduction of enzyme replacement. Disease activity was monitored by serial measurement of platelet count, visceral volumes (spleen and liver) by magnetic resonance imaging, serum activities of total acid phosphatase, angiotensin-converting enzyme (ACE) and the lysosomal chitinase, chitotriosidase. Pulmonary and activation-regulated chemokine (PARC/CCL 18) was also determined in serurn by ELISA. Results: Serum PARC concentrations were elevated 10-40-fold in patients with Gaucher disease compared with 67 healthy controls, without overlap (P |
Databáze: | OpenAIRE |
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