Table 1 Clinical characteristics of Tunisian MPS I patients
FAMILLES | I | II | III | IV | V | VI | VII | VIII | ||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
Patients | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 |
Age | 5 years 6 months (died) | 1 year 3 month (died) | 9 months | 9 years (died) | 10 years | 25 years(died) | 13 years | 10 years | 5 years (died) | 8 years (died) | 6 years | 5 years |
Age of onset | 2 months | 1 month half | 9 months | 7 months | 8 months | 9 months | 5 years | 1 year 6 months | 1 year | 5 years | 1 year | 9 months |
Sex | Male | Male | Female | Male | Male | Male | Female | Male | Female | Female | Female | Female |
Height (cm) | 103 (-1,5 DS) | 71 (+1DS) | 51(+1DS) | 107 (-3 DS) | 118 (-3 DS) | 120 (-3 DS) | 110 (-3 DS) | 115 (-3 DS) | 102 (-3DS) | 107 (-3DS) | 120(3DS) | 120(3DS) |
Weight (Kg) | 13 (-2 DS) | 12 (+2 DS) | 7(+1DS) | 19 (-1 DS) | 18 (- 3 DS) | 20 (-3DS) | 15 (- 1 DS) | 19 (-1 DS) | 18 (-1 DS) | 19 (-1DS) | 20(-3DS) | 18(-1DS) |
IDUA activity (μKat/kg) | 0,00 | 0,00 | 0,00 | 0,018 | 0,018 | 0,030 | nd | 0,050 | 0,020 | nd | 0.09 | 0.00 |