Table 1 Patient characteristics of the overall CGL cohort (N = 43)
Characteristic | Overall cohort |
|---|---|
Age at CGL diagnosis, years | |
n(available) | 43 |
Mean (± SD) | 5.1 (± 8.9) |
Median (range) | 1.0 (at birth–37) |
Sex, n/n(available) (%) | |
Female | 37/43 (86) |
Male | 6/43 (14) |
Country/State, n/n(available) (%) | |
Egypt | 1/43 (2) |
Iraq | 1/43 (2) |
Kingdom of Bahrain | 1/43 (2) |
Libya | 3/43 (7) |
Oman | 13/43 (30) |
Palestine* | 1/43 (2) |
Saudi Arabia | 20/43 (47) |
Tunisia | 1/43 (2) |
United Arab Emirates (UAE) | 2/43 (5) |
Parental consanguinity, n/n(available) (%) | 40/43 (93) |
Family history of lipodystrophy, n/n(available) (%) | 29/43 (67) |
Height (cm) | |
n(available) | 32 |
Mean (± SD) | 92 (± 40) |
Median (range) | 79 (45–174) |
Weight (kg) | |
n(available) | 33 |
Mean (± SD) | 18.2 (± 19.9) |
Median (range) | 9.5 (2.4–68.0) |
Body mass index (kg/m2) | |
n(available) | 30 |
Mean (± SD) | 16.5 (± 3.7) |
Median (range) | 16.6 (9.9–25.0) |
CGL subtype** | |
CGL1 (AGPAT2) | 14/42 (33) |
CGL2 (BSCL2) | 18/42 (43) |
CGL4 (CAVIN1) | 10/42 (24) |
Physical features, n/n(available) (%) | |
Acromegaloid features | 25/39 (64) |
Acanthosis nigricans | 20/39 (51) |
Hirsutism†| 7/37 (19) |
Organ system abnormalities, n/n(available) (%) | |
At least one abnormality | 35/43 (81) |
Bones and/or joints | 8/43 (19) |
Cardiovascular system | 16/43 (37) |
Liver | 30/43 (70) |
 Hepatomegaly | 27/43 (63) |
Pancreas | 4/43 (9) |
Renal system | 10/43 (23) |
Reproductive system‡ | 7/9 (78) |
Spleen | 14/43 (33) |
 Splenomegaly | 14/43 (33) |
Medication history, n/n(available) (%) | |
Anticoagulant | 1/43 (2) |
Antidiabetic medication | 9/43 (21) |
Cardiovascular medication | 7/43 (16) |
Lipid-lowering medication/treatment | 10/43 (23) |