Premature Adrenarche in Children with Prader-Willi Syndrome Treated with Recombinant Human Growth Hormone Seems to Not Influence the Course of Central Puberty and the Efficacy and Safety of the Therapy
Abstract
:1. Introduction
2. Material and Methods
3. Data Analysis
4. Results
Case Report of PA Followed by CPP
5. Comparison Group 1 vs. Group 2
6. Discussion
7. Conclusions
Author Contributions
Funding
Conflicts of Interest
References
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Patient | Sex | Gestational Age at Birth (Weeks of Pregnancy) | Birth Weight SDS/ Birth Length SDS | Apgar Score (1st Minute of Life) | Age of PWS Diagnosis (Years) | Type of Genetic Diagnosis |
---|---|---|---|---|---|---|
1 | M | 38 | −2.35/2.19 | 6 | 0.2 | abnormal methylation pattern of SNRPN |
2 | F | 39 | −1.66/1.77 | 10 | 0.2 | DEL15 |
3 | F | 38 | −2.73/0.51 | 9 | 0.3 | DEL15 |
4 | F | 38 | −2.22/1.57 | 8 | 0.4 | DEL15 |
5 | M | 41 | −1.23/1.13 | 7 | 0.6 | DEL15 |
6 | F | 38 | −0.88/3.23 | 10 | 1.1 | DEL15 |
7 | F | 40 | −3.96/0.31 | 5 | 1.1 | DEL15 |
8 | M | 36 | −2.13/2.50 | 8 | 1.5 | UPD 15 |
9 | F | 36 | −2.59/0.81 | 5 | 2.5 | DEL15 |
10 | F | 38 | −2.70/1.41 | 10 | 2.8 | abnormal methylation pattern of SNRPN |
11 | F | 42 | −2.00/1.92 | 7 | 3.3 | DEL15 |
Patient | Age at rhGH Start (Years) | rhGH Dose (IU/kg/Week) | IGF1 SD at rhGH Start | Age of Adrenarche (Years) | BA | DHEAS (ng/mL) (90–720) | Androstenedione (ng/dL) (8–50) | Testosterone (pg/mL) (30–100) |
---|---|---|---|---|---|---|---|---|
1 | 2.8 | 0.38 | −0.85 | 7.5 | 8.0 | 1325 | ND | 248.0 |
2 | 1.2 | 0.60 | −1.05 | 6.2 | 4.5 | 612 | 39.2 | ND |
3 | 1.1 | 0.42 | −0.58 | 5.8 | 6.8 | ND | ND | ND |
4 | 3.1 | 0.44 | −0.79 | 7.4 | 7.0 | 482 | 51.7 | ND |
5 | 5.3 | 0.80 | −1.04 | 8.4 | 10.0 | 1102 | 48.8 | 29.0 |
6 | 3.4 | 0.42 | −1.33 | 6.5 | 6.0 | 1369 | 137.0 | ND |
7 | 12.3 | 0.68 | −0.61 | 6.8 | 8.0 | 701 | 40.0 | 129.0 |
8 | 4.3 | 0.41 | −0.30 | 7.0 | 6.5 | 1111 | 53.9 | 41.8 |
9 | 3.7 | 0.38 | −0.53 | 6.8 | 7.0 | 2068 | 94.0 | ND |
10 | 3.9 | 0.86 | −0.80 | 6.8 | 8.8 | 1742 | 164.0 | 72.0 |
11 | 8.1 | 0.49 | 0.30 | 6.8 | 6.0 | 1772 | 132.0 | 95.8 |
Patient | Age (Years) | Glucose (mg/dL) 0’ | Glucose (mg/dL) 120’ | Insulin (uIU/mL) 0’ | Insulin (uIU/mL) 120’ | HOMA-IR | Cholesterol (mg/dL) | LDL (mg/dL) | HDL (mg/dL) | Triglycerides (mg/dL) |
---|---|---|---|---|---|---|---|---|---|---|
1 | 6.2 | 91 | 98 | 9.2 | 105 | 2.1 | 223 | 168 | 39 | ND |
2 | 3.4 | 67 | 67 | 6.4 | 5.8 | 1.1 | 181 | 126 | 43 | 62 |
4 | 8.9 | 86 | 95 | 7.7 | 28.9 | 1.6 | 173 | 103 | 55 | 77 |
5 | 13.6 | 94 | 101 | 9.9 | 24.4 | 2.3 | 170 | 81 | 75 | 68 |
6 | 11.0 | 73 | 143 | 10.4 | 44.0 | 1.9 | 152 | 78 | 64 | 62 |
7 | 17.5 | 80 | 104 | 11.2 | 83.3 | 2.2 | 208 | 136 | 42 | 152 |
8 | 7.6 | 75 | 113 | 31.3 | 10.2 | 5.8 | 116 | 51 | 58 | 36 |
9 | 7.5 | 65 | 181 | 8.2 | 61.9 | 1.3 | 193 | 118 | 61 | 70 |
10 | 7.3 | 78 | 133 | 15.9 | 72.1 | 3.1 | 143 | 83 | 44 | 83 |
11 | 17.7 | 89 | 126 | 16.6 | 121.0 | 3.6 | 184 | 118 | 50 | 83 |
Group | Number of Patients | Age at rhGH Start (Years), p < 0.05 | rhGH Dose (IU/kg/Week; mg/kg/Day) | IGF1 SD at rhGH Start, p < 0.05 | IGF1 SD GH1 | IGF1 SD GH2 | BA/CA | Adrenarche Age (Years) | Central Puberty Age (Years) |
---|---|---|---|---|---|---|---|---|---|
1 | 11 | 4.5 ± 3.2 | 0.53 ± 0.17; 0.025 ± 0.008 | −0.7 ± 0.4 | 1.7 ± 1.6 | 1.7 ± 1.7 | 1.0 ± 0.2 | 6.9 ± 0.7 | 12.0 ± 2.2 * |
2 | 14 | 7.1 ± 2.9 | 0.50 ± 0.14; 0.024 ± 0.007 | −1.1 ± 0.5 | 1.1 ± 1.5 | 1.3 ± 1.5 | 1.0 ± 0.1 | 10.5 ± 1.3 | 11.9 ± 1.5 ** |
Group | Height SD rhGH Start, p = 0.15 | Height SD GH1 | Height SD GH2 | GV GH1 | GV GH2 | BMI SDS rhGH Start, p < 0.05 | BMI SDS GH1 | BMI SDS GH2 | HOMA-IR |
---|---|---|---|---|---|---|---|---|---|
1 | −1.7 ± 1.3 | −1.1 ± 1.2 | −0.6 ± 1.8 | 9.0 ± 1.8 | 6.1 ± 2.0 | 0.8 ± 1.2 | −0.6 ± 1.1 | 0.6 ± 1.1 | 2.5 ± 1.4 |
2 | −2.5 ± 1.4 | −1.3 ± 1.1 | −1.2 ± 0.9 | 9.6 ± 2.0 | 5.9 ± 1.0 | 1.8 ± 1.5 | −0.1 ± 1.2 | 1.1 ± 1.3 | 3.0 ± 2.0 |
Group | rhGH Total Time (Years) | Age at the Last Visit (Years) | Height SD | BMI SD, p = 0.12 |
---|---|---|---|---|
1 | 10.2 ± 2.7 | 14.5 ± 4.0 | −1.1 ± 1.5 | 0.4 ± 1.2 |
2 | 8.5 ± 3.1 | 16.8 ± 2.0 | −1.5 ± 1.0 | 1.2 ± 1.4 |
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Lecka-Ambroziak, A.; Wysocka-Mincewicz, M.; Marszałek-Dziuba, K.; Rudzka-Kocjan, A.; Szalecki, M. Premature Adrenarche in Children with Prader-Willi Syndrome Treated with Recombinant Human Growth Hormone Seems to Not Influence the Course of Central Puberty and the Efficacy and Safety of the Therapy. Life 2020, 10, 237. https://doi.org/10.3390/life10100237
Lecka-Ambroziak A, Wysocka-Mincewicz M, Marszałek-Dziuba K, Rudzka-Kocjan A, Szalecki M. Premature Adrenarche in Children with Prader-Willi Syndrome Treated with Recombinant Human Growth Hormone Seems to Not Influence the Course of Central Puberty and the Efficacy and Safety of the Therapy. Life. 2020; 10(10):237. https://doi.org/10.3390/life10100237
Chicago/Turabian StyleLecka-Ambroziak, Agnieszka, Marta Wysocka-Mincewicz, Kamila Marszałek-Dziuba, Agnieszka Rudzka-Kocjan, and Mieczysław Szalecki. 2020. "Premature Adrenarche in Children with Prader-Willi Syndrome Treated with Recombinant Human Growth Hormone Seems to Not Influence the Course of Central Puberty and the Efficacy and Safety of the Therapy" Life 10, no. 10: 237. https://doi.org/10.3390/life10100237