Table 1.
Endocrine status according to transition status from pediatric to adult care.
All (n = 95) | Transition (n = 31) | Without transition (n = 64) | P | |
---|---|---|---|---|
Age at diagnosis (year) | 7.5 ± 8.0 (0.0; 40.0) | 5.7 ± 6.1 | 14.9 ± 12.2 | <0.001 |
Age at multidisciplinary assessments in adult nutrition department (year) | 24.7 ± 8.2 (16.1; 58.7) | 19.9 ± 3.1 | 27.1 ± 8.9 | <0.001 |
Female gender (%) | 51 (53.7) | 17 (54.8) | 34 (53.1) | 0.88 |
Genotype | ||||
Deletion (%) | 50 (52.6) | 17 (54.8) | 33 (51.6) | 0.58 |
Uniparental disomy (%) | 39 (41.1) | 14 (45.2) | 25 (39.1) | |
Imprinting defects (%) | 2 (2.1) | 0 (0) | 2 (3.1) | |
Somatotropic axis | ||||
During childhood | ||||
GH stimulation test (%) | 44 (46.3) | 23 (74.2) | 21 (32.8) | 0.001 |
GH deficiency (%) | 41/44 (93.2) | 20/23 (87.0) | 21/21 (100) | 0.99 |
GH treatment (%) | 34 (35.8) | 19 (61.3) | 15 (23.4) | <0.0001 |
After completing growth | ||||
GH stimulation test (%) | 16 (16.8) | 7 (22.6) | 9 (14.1) | 0.43 |
GH deficiency (%) | 8/16 (50.0) | 2/7 (28.6) | 6/9 (66.7) | 0.17 |
GH treatment (%) | 14 (14.7) | 8 (25.8) | 6 (9.4) | 0.08 |
Gonadotropic axis | ||||
Before transition | ||||
Sex-hormone therapy (%) | 20 (21.1) | 16 (51.6) | 4 (6.3) | <0.0001 |
After transition | ||||
Screening (%)§ | 90 (94.7) | 30 (96.8) | 60 (93.8) | 1 |
Hypogonadism (%) | 82/90 (91.1) | 27/30 (90.0) | 55/60 (91.7) | 1 |
Sex-hormone therapy (%) | 54 (56.8) | 19 (61.3) | 35 (54.7) | 0.89 |
Corticotropic axis in adulthood | ||||
Screening (%)* | 36 (37.9) | 12 (38.7) | 24 (37.5) | 1 |
Central adrenal insufficiency (%) | 3/36 (8.3) | 1/12 (8.3) | 2/24 (8.3) | 1 |
Thyrotropic axis in adulthood | ||||
Screening (%) | 91 (95.8) | 31 (100.0) | 60 (93.8) | 0.59 |
Hypothyroidism (%) | 29/91 (31.2) | 10/31 (32.3) | 19/60 (31.7) | 0.99 |
l-Thyroxine treatment (%) | 22 (23.2) | 8 (25.8) | 14 (21.9) | 0.99 |
Categorical data are presented as numbers and percentages. Continuous variables are presented as the means ± s.d., minimum and maximum. Nonparametric Wilcoxon and X 2 tests (or Fischer’s exact test for small sample size) were used for comparisons of continuous or non-continuous variables, respectively.
*Cortisol level at 08:00 h <138 nmol/L or >500 nmol/L or dynamic test performed; §measurement of estradiol or testosterone and gonadotrophins levels at baseline.