Table 1.

Characteristics of the patients

Variables	n (%)	Median (IQR)
Gender
Male	18 (53)
Female	16 (47)
Genotype
N370S/N370S	5 (15)
N370S/other	18 (53)
Other/other	11 (32)
Previous spleen removal
Yes	10 (29)
No	24 (71)
Age, at therapy start (years)		27.8 (13.2–37.3)
Age, at reduction (years)		41.8 (29.2–50.5)
Duration of therapy, at reduction (years)		14.1 (10.5–15.6)
ERT dose (IU/kg/month)
Before reduction		55.5 (48–63)
After reduction		15.8 (15–30)
% variation		35.5 (28–47)