Bolezn' Goshe u detey: osnovnye aspekty


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Abstract

The article presents data on the epidemiology, pathogenesis, modern classification, and major clinical manifestations of Gaucher disease (GD) in children. The criteria of differential diagnosis with other diseases are discussed. It is shown that pathogenetic enzyme replacement therapy is only effective treatment of GD, which stops the main clinical manifestations of the disease, improving quality of life in patients with GD without causing side effects. The monitoring of condition of patients on therapy is recommended in accordance with the requirements of the Joint International Group on Gaucher disease. The main errors of diagnosis and management of these patients, as well as unwarranted use of drugs for the treatment of G, are discussed.

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O. S Gundobina

References

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