Table 1

Demographic and baseline characteristics of the 2 groups

30 U/kg/infusion (n = 15)60 U/kg/infusion (n = 16)
Mean age, y, ± SD at entry (range) 36.3 ± 12.2 (19-74) 36.0 ± 12.2 (19-58) 
Male:female 7:8 8:8 
Ashkenazi Jewish:non-Jewish 6:9 4:12 
N370S homozygous:other genotype 7:8 6:10 
Mean spleen volume, mL (range) 2134 (886-4901) 2117 (914-5418) 
Mean spleen volume, MN* (range) 15 (8-35) 17 (8-54) 
Mean liver volume, mL (range) 2880 (2282-5096) 2481 (1758-3297) 
Mean liver volume, MN* (range) 1.7 (1.4-2.9) 1.6 (0.9-2.6) 
Mean hemoglobin: g/dL (range) 12.2 (7.9-14.6) 11.4 (5.5-16.0) 
Mean platelets, /mm3 (range) 75 320 (28 000-163 00065 038 (28 000-134 000
Mean chitotriosidase, nmol/mL/h (range) 28 158 (7791-50 024) 24 702 (4639-66 628) 
30 U/kg/infusion (n = 15)60 U/kg/infusion (n = 16)
Mean age, y, ± SD at entry (range) 36.3 ± 12.2 (19-74) 36.0 ± 12.2 (19-58) 
Male:female 7:8 8:8 
Ashkenazi Jewish:non-Jewish 6:9 4:12 
N370S homozygous:other genotype 7:8 6:10 
Mean spleen volume, mL (range) 2134 (886-4901) 2117 (914-5418) 
Mean spleen volume, MN* (range) 15 (8-35) 17 (8-54) 
Mean liver volume, mL (range) 2880 (2282-5096) 2481 (1758-3297) 
Mean liver volume, MN* (range) 1.7 (1.4-2.9) 1.6 (0.9-2.6) 
Mean hemoglobin: g/dL (range) 12.2 (7.9-14.6) 11.4 (5.5-16.0) 
Mean platelets, /mm3 (range) 75 320 (28 000-163 00065 038 (28 000-134 000
Mean chitotriosidase, nmol/mL/h (range) 28 158 (7791-50 024) 24 702 (4639-66 628) 
*

MN indicates multiple of normal organ size: spleen 0.2 body weight, liver 2.5 body weight.

One patient was allowed to enter with platelet counts > 120 000/mm3.

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