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Table 2 Patient baseline characteristics

From: A retrospective study of morbidity and mortality of chronic acid sphingomyelinase deficiency in Germany

Parameter

Overall (N = 33)

ASMD type B (n = 24)

ASMD type A/B (n = 9)

Age at the first evidence of ASMD (years)

 Numbera

32

24

8

 Mean (SD)

7.0 (11.8)

9.0 (13.1)

1.2 (1.2)

 Median [IQR]

2.0 [1.0–5.0]

3.0 [2.0–15.0]

1.0 [1.0–1.0]

 Range

0–58.0

0–58.0

0–4.0

Age at diagnosis (years)

 Numbera

32

24

8

 Mean (SD)

11.5 (14.3)

14.8 (15.2)

1.5 (1.1)

 Median [IQR]

4.0 [2.0–18.0]

8.0 [3.0–20.0]

1.0 [1.0–2.0]

 Range

1.0–58.0

1.0–58.0

1.0–4.0

Time between the first symptom onset and diagnosis of ASMD (months)

 Numbera

29

22

7

 Mean (SD)

58.7 (94.1)

76.1 (102.4)

4.3 (4.9)

 Median [IQR]

9.0 [1.0–89.0]

21.0 [4.0–120.0]

1.0 [0–9.0]

 Range

0–383

0–383

0–11

Sex [n(%)]

 Male

15 (45.5)

12 (50.0)

3 (33.3)

 Female

18 (54.5)

12 (50.0)

6 (66.7)

  1. aPatient with ASMD type A/B (n = 1) was excluded from the analysis due to significant missing variables
  2. ASMD, acid sphingomyelinase deficiency; IQR, interquartile range; n, number of patients; SD, standard deviation