Enzyme replacement therapy in a @entity1 with XXXX .

@entity1 @entity12842 @entity3265 @entity535 @entity12527 @entity7717 @entity2841 @entity369
In a @entity1 with @entity12842 , enzyme replacement therapy such as whole blood exchange transfusion or erythrocyte transfusion was performed, and its effect was confirmed in vitro as well as in vivo. The @entity1 has been treated with the restriction of protein intake, oral administration of an essential @entity535 mixture, and @entity7717 or @entity12527 . With these treatments, his plasma @entity3265 levels were controlled. On the other hand, plasma and @entity2841 concentrations of @entity369 were not so well controlled. With whole blood exchange transfusion and erythrocyte transfusion, plasma @entity369 concentrations and plasma @entity3265 levels were controlled. These effects have continued for about 3 months. Furthermore, the effect of exchanged erythrocytes on the blood @entity369 levels was also confirmed by in vitro experimentation that mixes @entity369 with erythrocytes in the medium RPMI 1640. @entity369 concentration of the medium did not decrease when @entity369 was mixed with erythrocytes of the @entity1 , but when @entity369 was mixed with erythrocytes of mother or normal controls, it decreased rapidly. In calculation, 10(7) of erythrocytes of the mother or normal controls was presumed to metabolize 4-5 nmole of @entity369 per day. From these results, the erythrocyte exchange transfusion is considered to be effective for the control of clinical and biochemical abnormalities in this disorder.
