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Table 1 Main characteristics of the MPS patients enrolled in the present study

From: Cardiac involvement in MPS patients: incidence and response to therapy in an Italian multicentre study

MPS subtype

Number of pts (male; female)

Clinical phenotype

Age at diagnosis (years)§

Time between diagnosis and start of ERT (years)§

Age at start of ERT (years)§

MPS I

9 (6 M; 3F)

6 non-neuropathic, 3 neuropathic

2.5 ± 2#

1.5 ± 1.8

6.2 ± 6

MPS II

16 (15 M; 1F)

6 non-neuropathic, 11 neuropathic

4.6 ± 3*

4.6 ± 8

10.5 ± 10*

MPS IIIA

8 (5 M; 3F)

1 mild, 7 severe

6.27 ± 5

–

–

MPS IIIB

5 (2 M;3F)

5 severe

4.28 ± 3

–

–

MPS IIID

1 (1 M; 0F)

1 severe

5.48

–

–

MPS IVA

12 (4 M; 8F)

5 slowly progressing,7 rapidly progressing

3.3 ± 2

9.5 ± 9.9

12.8 ± 10

MPS IVB

2 (2 M; 0F)

2 slowly progressing

10 ± 3

–

–

MPS VI

7 (2 M; 5F)

2 slowly progressing, 5 rapidly progressing

2 ± 0.7

4.7 ± 3

6.7 ± 3

  1. #One patient, diagnosticated at fourteen years of age, was excluded from the average. *One patient, diagnosticated at forty-four years of age, was excluded from the average; §data expressed as mean ± standard deviation