Table 1.
Characteristics of Female Participants with HGPS Older than Age 12 Years
| Patient ID | Age at last Contact, years | Age at Menarche, years | Testing in Relation to Menarche, months | Tanner Stage for Presence of Sparse Pubic Hair | Tanner Stage for Presence of Breast Bud | Segmental height, cm | Fasting Weight, kg | BMI, kg/m2 | Percentage of body fat by DXA | Leptin, ng/mL | Albumin, g/dL (years)* | Bone age |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 15.8 | 15.4 | 12 Pre | 1 | 1 | 117.8 | 14.4 | 10.4 | 19.8 | 0.4 | 4.6 (12.6) | WNL |
| 2 | 15.2 | 15.6 | 12 Pre | 1 | 1 | 111.6 | 12.3 | 9.9 | 17.7 | 0.4 | 4.3 | WNL |
| 3 | 15.75 | 15.75 | 24 Pre | 1 | 1 | 107.8 | 13.8 | 11.9 | 23.1 | 0.4 | 4.1 (12.4) | Advanced by 0.4 years |
| 4 | 16.5 | 16.5 | 27 Pre | 1 | 1 | 112.1 | 12.9 | 10.3 | 20.4 | 0.4 | 4.1 | WNL |
| 5 | 13.8 | 13.3 | 4 Post | 1 | 2 | 121.5 | 16.3 | 11.0 | 19.5 | 0.4 | 4.2 | Advanced by 1.4 years |
| 6 | 17.4 | 14.0 | 6 Post | 1 | 1 | 107.4 | 12.1 | 10.5 | 15.8 | 0.5 | 4.3 | Advanced by 0.4 years |
| 7 | 17.3 | 14.25 | 10 Post | 2 | 1 | 114.3 | 12.4 | 9.5 | 19.4 | 0.5 | 4 | WNL |
| 8 | 20.3 | 14.4 | 22 Post | 1 | 1 | 116.2 | 14.3 | 10.6 | 9.3 | 0.5 | 3.9 | Delayed by 1.4 years |
| 9 | 19.2 | 12.0 | 66 Post | 2 | 2 | 104.2 | 13.0 | 12.0 | 19.4 | 0.4 | N/M | WNL |
| A | 13.6 | – | – | 1 | 1 | 109.6 | 13.4 | 11.2 | 21.1 | 0.4 | 4.3 | Advanced by 1.8 years |
| B | 12.8 | – | – | 1 | 1 | 104.6 | 11.4 | 10.4 | 17.6 | 0.4 | N/M | WNL |
| C | 15.25 | – | – | 1 | 1 | 103.8 | 10.3 | 9.6 | 19.8 | 0.4 | 4.1 (10.8) | Advanced by 2.0 years |
| D | 14.4 | – | – | 2 | 2 | 116.8 | 14.2 | 10.4 | 20.0 | 0.4 | 4.8 (13.5) | WNL |
| E | 12.8 | – | – | 1 | 1 | 102.5 | 11.5 | 10.9 | 18.7 | 0.4 | N/M | WNL |
| F | 17.5 | – | – | 2 | 1 | 113.3 | 12.1 | 9.4 | 15.6 | 0.4 | 4.5 (14) | Delayed by 0.1 years |
BMI, body mass index; DXA, dual-energy x-ray absorptiometry scan; HGPS, Hutchinson-Gilford progeria syndrome; N/M, not measured; post, after menarche; pre, before menarche; WNL, within normal limits for age.
*
When albumin was measured earlier than listed time of testing, age of measurement is listed with albumin value.