Taghreed Mahmoud Ibraheem


Background: Gaucher disease (GD) is the most frequent lysosomal storage disorder. Bone and lung involvement are two major causes of morbidity in this disease. CD163 is expressed on cells of monocyte-macrophage lineage and its soluble form (sCD163) may be a valuable diagnostic biomarker for monitoring diseases with increased macrophage activation. Aim: We determined sCD163 levels in 30 children and adolescence with GD compared with 30 age- and sex-matched healthy controls and assessed relation to phenotypes its role as a potential marker for disease severity and complications. Materials and Methods: GD patients were recruited from the regular attendants of the Pediatric Hematology Clinic, Pediatric Hospital, Ain Shams University and studied stressing on disease duration and skeletal, pulmonary or neurological manifestations, enzyme replacement therapy, hematological profile, liver and kidney functions, plasma chitotriosidase activity and D-dimer. sCD163 was measured by enzyme linked immunosorbent assay. Abdominal ultrasonography was performed to assess the volume of liver and spleen and DXA was used for measurement of bone mineral density. Results: sCD163 levels were markedly elevated in patients compared with controls. D-dimer, chitotriosidase activity and sCD163 levels were significantly increased in type 3 GD patients compared with type 1. sCD163 was significantly elevated in GD patients with dysphagia, developmental delay, pulmonary hypertension or abnormal DXA findings (osteopenia/osteoporosis) than those without. Although splenectomized patients had higher sCD163 levels than non-splenectomized counterparts, yet, the difference was non-significant. sCD163 was positively correlated to age, disease duration, dose of ERT, D-dimer and chitotriosidase activity and on multiple regression analysis, pulmonary hypertension, D-dimer and chitotriosidase activity were independently related to sCD163. The cutoff value of sCD163 at 9400 ng/mL could differentiate GD patients with and without pulmonary hypertension with a sensitivity of 90% and specificity of 95%. Conclusions: We suggest that sCD163 is significantly elevated in GD, particularly type 3, and may be considered a biomarker for the clinical assessment of macrophage proliferation and activity that would help in risk prediction of bone and lung involvement and monitoring treatment response. However, the cutoff for sCD163 as a predictor of pulmonary hypertension should be validated in larger prospective studies before it could be incorporated into risk stratification to guide current therapeutic regimens.

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Other Titles مستوى سى دى 163 الذائب والمشتق من الخلايا البلعية فى المرضى المصابين بمرض جوشيه؛ وعلاقته بالأنماط الظاهرية ومضاعفات المرض وحدته
Issue Date 2014

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