Chitotriosidase

Chitotriosidase

Clinical significance

Chitotriosidase - an enzyme synthesized by activated macrophages. Determination of serum chitotrosidase activity is an additional test performed in the diagnosis of Gaucher disease. In this disease, a more than 1,000-fold increase in this enzyme compared to reference values is characteristic. Gaucher disease belongs to the group of sphingolipidoses. The pathomechanism involves a deficit in the activity of beta-glucocerebrosidase, whichóra under physiological conditions cleaves glucose from the cerebroside molecule. Symptoms depend on the degree of residual activity of the enzyme, with different cells and tissues showing different sensitivity to its deficiency. The most sensitive are monocytes/macrophages, whichóre physiologically metabolizing large amounts of glucosylceramide derived from the cell membranes of decaying blood cells. Clinically, there are two clinical types of Gaucher's disease: type 1 - non-neuropathic form, characterized by enlargement of the liver and spleen and/or painful lytic bone lesions; types 2 and 3 - neuronopathic form, characterized by rów also hepatosplenomegaly, hematological and bone lesions and central nervous system involvement of varying degrees of severity.

Patient Preparation

Material: Serum