Table 1.
Age of onset (years) | Range | 1-18 |
---|---|---|
Mean ± SD | 9.92 ± 0.37 | |
Median ± IR | 10 ± 4 | |
Age at presentation (years) | Range | 1.0 -19 |
Mean ± SD | 10.96 ± 0.45 | |
Median ± IR | 10 ± 4 | |
Sex (M/F) | 43/34 | |
Consanguinity No. (%) | 56/71 (78.9%) | |
Number of families | 50 | |
Residency (73)- No. (%) | Upper Egypt | 25 (34.2%) |
Lower Egypt | 30 (41.1%) | |
Cairo | 12 (16.4%) | |
Non-Egyptian | 6 (8.2%) | |
Type of presentation No. (%) | Hepatic | 35 (45.5%) |
Neurological | 6 (7.8%) | |
Combined | 9 (11.7%) | |
Asymptomatic | 27 (35.1%) |
N.B. Two adult patients with ages of 30 and 37 years (cousins of one of the children) were excluded when calculating the age ranges and mean.
M: male, F: female, SD: standard deviation, IR: interquartial range