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Table 1 Population characteristics and disease history

From: Prospective study of the natural history of chronic acid sphingomyelinase deficiency in children and adults: eleven years of observation

Parameter Pediatric cohort (age < 18 at baseline)
N = 30
Adult cohort (age ≥ 18 at baseline)
N = 29
Age range at baseline year 7–17 18–64
Age distribution n (years) BL
N = 30
Yr1
N = 28
Final
N = 15
BL
N = 29
Yr1
N = 22
Final
N = 17
 6–11 12 7 0 0 0 0
 12–17 18 19 1 0 0 0
 18–23 0 2 10 10 9 2
 24–30 0 0 4 3 2 2
 31–40 0 0 0 9 7 4
 41–50 0 0 0 5 3 7
  > 50 0 0 0 2 1 2
Sex, n (%)       
 Male 20 (66.7)    11 (37.9)   
 Female 10 (33.3)    18 (62.1)   
Ethnicity, n (%)       
 Caucasian 27 (90.0)    27 (93.1)   
 Black 0    1 (3.4)   
 Hispanic 0    0   
 Asian 0    0   
 Other 3 (10.0)    1 (3.4)   
Age at symptom onset       
 Mean (SD), years 2.52 (2.8)    7.94 (8.8)   
 Median (range) 2.00 (0.1,14.0)    4.00 (0.9, 30)   
Age at diagnosis       
 Mean (SD), years 5.23 (3.6)    15.10 (13.5)   
 Median (range) 3.63 (1, 14.3)    11.00 (2, 45.1)   
Splenectomy n (%)       
 Partial 0    1 ( 3.4)   
 Total 0    4 ( 13.8)