Early-onset growth hormone treatment in Prader–Willi syndrome attenuates transition to severe obesity
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Aneta Kodytková
,
Shenali Anne Amaratunga
,
Eva El-Lababidi
,
Ivana Čermáková
,
Jana Černá
,
Marcela Dvořáková
Abstract
Objectives
Subsequent to early life feeding issues, children with Prader–Willi syndrome (PWS) develop hyperphagia and severe obesity. Growth hormone (GH) therapy has been approved in PWS to improve growth, body composition, and BMI. We aimed to clarify the role of age at GH therapy onset on growth and BMI trajectories in children with PWS.
Methods
We analyzed height and BMI in 114 patients (58 boys) from REPAR – Czech national GH registry. From them, 69 started GH therapy prior to 2 y/o (age 0.8 ± 0.4 years; mean ± SD; early-onset group [EO]), and 45 later (age 7.1 ± 4.1 years; late-onset group [LO]).
Results
Height-SDS before therapy was similar in all (EO: −1.9 ± 1.2 [mean ± SD]; LO: −1.7 ± 1.1). After the first year of GH therapy, height-SDS in the EO group increased to −1.0 ± 1.2, in the LO group to −0.9 ± 1.1. After 5 years, height fully normalized in all (−0.1 ± 1.1 SDS). The LO children were already obese at treatment initiation (BMI-SDS: 2.9 ± 2.2), and their BMI-SDS decreased after 1 year of GH therapy by 0.9 (p=0.003). The weight in EO children was below average before GH treatment (BMI-SDS: −0.9 ± 1.2) and their BMI-SDS increased to the overweight range of 1.3 ± 2.2 (p<0.001) within the oncoming 3 years. Albeit BMI-SDS was around the obesity limit in most children after 5 years on GH therapy, the highest lifetime BMI-SDS was lower in EO (2.2 ± 2.6) than in LO (3.7 ± 2.2; p<0.001).
Conclusions
GH treatment in PWS normalizes body height. After 5 years of GH therapy, BMI-SDS in EO and LO groups are similar; however, the EO group is exposed to lower maximal BMI-SDS values.
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Research ethics: This study was approved by the Multicentric Ethics Committee at the Olomouc University Hospital, Palacky University (date of approval: 11th November 2013; 138/13 MEK 24). The research was conducted ethically in accordance with the World Medical Association Declaration of Helsinki.
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Informed consent: Patients and their legal guardians gave their written consent to collect and to further analyze their anonymized data on GH therapy within the REPAR database.
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Author contributions: The authors have accepted responsibility for the entire content of this manuscript and approved its submission.
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Use of Large Language Models, AI and Machine Learning Tools: None declared.
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Conflict of interest: The authors state no conflict of interest.
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Research funding: None declared.
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Data availability: Not applicable.
References
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Articles in the same Issue
- Frontmatter
- Review
- A recent update on childhood obesity: aetiology, treatment and complications
- Original Articles
- Chronotype, sleep, and glycemic control in children and adolescents with type 1 diabetes: a case-control study
- Determinants of childhood and adolescent obesity and it’s effect on metabolism in South Indian population
- Evaluation of continuous glucose monitoring and nutritional status in glycogen storage diseases
- Retrospective assessment of hepatic involvement in patients with inherited metabolic disorders: nine-year single-center experience
- Relationships among biological sex, body composition, and bone mineral density in young persons with and without diabetes
- The clinical characteristics of 10 cases and adult height of six cases of rare familial male-limited precocious puberty
- Optimal timing of repeat thyroid fine-needle aspiration biopsy
- Medium-chain acyl-CoA dehydrogenase deficiency in North Macedonia – ten years experience
- The effect of antenatal steroids on metabolic bone disease of prematurity
- Prader-Willi syndrome gene expression profiling of obese and non-obese patients reveals transcriptional changes in CLEC4D and ANXA3
- Early-onset growth hormone treatment in Prader–Willi syndrome attenuates transition to severe obesity
- Case Reports
- Neonatal severe hyperparathyroidism with inactivating calcium sensing receptor (CaSR) mutation (p.I81K)
- Clinical manifestations and molecular genetics of seven patients with Niemann–Pick type-C: a case series with a novel variant
- Expanding the genotypic spectrum of 3β-hydroxy-δ5-C27-steroid dehydrogenase (HSD3B7) deficiency: novel mutations and clinical outcomes
Articles in the same Issue
- Frontmatter
- Review
- A recent update on childhood obesity: aetiology, treatment and complications
- Original Articles
- Chronotype, sleep, and glycemic control in children and adolescents with type 1 diabetes: a case-control study
- Determinants of childhood and adolescent obesity and it’s effect on metabolism in South Indian population
- Evaluation of continuous glucose monitoring and nutritional status in glycogen storage diseases
- Retrospective assessment of hepatic involvement in patients with inherited metabolic disorders: nine-year single-center experience
- Relationships among biological sex, body composition, and bone mineral density in young persons with and without diabetes
- The clinical characteristics of 10 cases and adult height of six cases of rare familial male-limited precocious puberty
- Optimal timing of repeat thyroid fine-needle aspiration biopsy
- Medium-chain acyl-CoA dehydrogenase deficiency in North Macedonia – ten years experience
- The effect of antenatal steroids on metabolic bone disease of prematurity
- Prader-Willi syndrome gene expression profiling of obese and non-obese patients reveals transcriptional changes in CLEC4D and ANXA3
- Early-onset growth hormone treatment in Prader–Willi syndrome attenuates transition to severe obesity
- Case Reports
- Neonatal severe hyperparathyroidism with inactivating calcium sensing receptor (CaSR) mutation (p.I81K)
- Clinical manifestations and molecular genetics of seven patients with Niemann–Pick type-C: a case series with a novel variant
- Expanding the genotypic spectrum of 3β-hydroxy-δ5-C27-steroid dehydrogenase (HSD3B7) deficiency: novel mutations and clinical outcomes
