دورية أكاديمية
Serum IGF1 and linear growth in children with congenital leptin deficiency before and after leptin substitution
| العنوان: | Serum IGF1 and linear growth in children with congenital leptin deficiency before and after leptin substitution |
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| المؤلفون: | Beghini, Marianna, Brandt, Stephanie, Körber, Ingrid, Kohlsdorf, Katja, Vollbach, Heike, Lennerz, Belinda, Denzer, Christian, Shalitin, Shlomit, Santini, Ferruccio, Blum, Werner F., von Schnurbein, Julia, Wabitsch, Martin |
| بيانات النشر: | Universität Ulm |
| سنة النشر: | 2023 |
| المجموعة: | OPARU (OPen Access Repository of Ulm University) |
| مصطلحات موضوعية: | Endocrine system and metabolic diseases, Obesity, BINDING-PROTEIN (IGFBP)-1, EARLY-ONSET OBESITY, FACTOR-I, MISSENSE MUTATION, WEIGHT-LOSS, REFERENCE INTERVALS, POTENT STIMULATOR, FACTOR (IGF)-I, BONE TURNOVER, INSULIN, DDC 610 / Medicine & health |
| الوصف: | Background Evidence from in vitro and rodent studies suggests that leptin, a key signal of long-term energy reserves, promotes IGF1 synthesis and linear growth. This effect of leptin has not been fully investigated in humans. The aim of our study was to investigate the effect of leptin substitution on growth factors and linear growth in children with congenital leptin deficiency (CLD). Methods In this cohort study we included eight pediatric patients (six males), age 0.9–14.8 years, who were diagnosed with CLD and received leptin substitution at our University Medical Center. We calculated standard deviation scores (SDS) for serum levels of IGF1 and IGFBP3, IGF1/IGFBP3 molar ratio, and height at baseline (T0) and 12 months (T12) after the initiation of substitution with metreleptin. Results All patients had severe obesity (BMI-SDS mean ± SD: 4.14 ± 1.51) at T0 and significant BMI-SDS reduction to 2.47 ± 1.05 at T12. At T0, all patients were taller than the mid-parental median, yet had low IGF1 and IGF1/IGFBP3 molar ratios (IGF1-SDSx¯\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\overline x$$\end{document}T0: −1.58 ± 0.92, IGF1/IGFBP3 molar ratio-SDSx¯\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\overline x$$\end{document}T0: −1.58 ± 0.88). At T12, IGF1-SDS increased significantly (∆T0–12: 1.63 ± 1.40, p = 0.01), and IGFBP3-SDS and IGF1/IGFBP3 molar ratio-SDS showed a trend toward an increase. In the three children within the childhood growth period (post-infancy, pre-puberty) height-SDS increased (∆height-SDST0–12: 0.57 ± 0.06, p = 0.003) despite substantial weight loss. Conclusions These results in CLD patients are contrary to observations ... |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/pdf |
| اللغة: | English |
| العلاقة: | https://www.nature.com/articles/s41366-021-00809-2#Sec15Test; http://dx.doi.org/10.18725/OPARU-50885Test; https://oparu.uni-ulm.de/xmlui/123456789/50961Test; http://nbn-resolving.de/urn:nbn:de:bsz:289-oparu-50961-0Test |
| DOI: | 10.18725/OPARU-50885 |
| الإتاحة: | https://doi.org/10.18725/OPARU-50885Test https://oparu.uni-ulm.de/xmlui/123456789/50961Test http://nbn-resolving.de/urn:nbn:de:bsz:289-oparu-50961-0Test |
| حقوق: | CC BY 4.0 International ; https://creativecommons.org/licenses/by/4.0Test/ |
| رقم الانضمام: | edsbas.D2BB7623 |
| قاعدة البيانات: | BASE |
| DOI: | 10.18725/OPARU-50885 |
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