Abstract
Gaucher disease is lysosomal storage disease caused by decreased activity of glukocerebrosidase. Due to the deficit of this acid hydrolase glucocerebroside is stored in many tissues, most of all in the bone marrow, spleen, liver.
Patients with m. Gaucher suffer with bleeding tendency, fatigue and bone pain due to trombocytopenia, anaemia and skeletal burden.
The most effectice therapy is so called enzyme replacement therapy (ERT) with imiglucerase (Cerezyme). In our article we describe the outcome of two women with twin pregnancy - one without treatment and the second on ERT during gravidity, delivery and lactation. we propose, that ERT is an effective and safe method of therapy, enabling to prevent the most frequent complications in this period.