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. 2011 Jun 17;11:56. doi: 10.1186/1471-2431-11-56

Table 1.

Demographic data and type of presentation of the studied group

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