Is ghrelin a biomarker of early-onset scoliosis in children with Prader–Willi syndrome?
Abstract Background Adolescents with idiopathic scoliosis display high ghrelin levels. As hyperghrelinemia is found in patients with PWS and early-onset scoliosis (EOS) is highly prevalent in these patients, our aims were to explore (1) whether ghrelin levels differ between those with and without EO...
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| Format: | Article |
| Language: | English |
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BMC
2021-07-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13023-021-01930-1 |
| _version_ | 1818678392027873280 |
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| author | Dibia Liz Pacoricona Alfaro Gwenaelle Diene Graziella Pinto Jean-Pierre Salles Isabelle Gennero Sandy Faye Catherine Molinas Marion Valette Catherine Arnaud Maithé Tauber |
| author_facet | Dibia Liz Pacoricona Alfaro Gwenaelle Diene Graziella Pinto Jean-Pierre Salles Isabelle Gennero Sandy Faye Catherine Molinas Marion Valette Catherine Arnaud Maithé Tauber |
| author_sort | Dibia Liz Pacoricona Alfaro |
| collection | DOAJ |
| description | Abstract Background Adolescents with idiopathic scoliosis display high ghrelin levels. As hyperghrelinemia is found in patients with PWS and early-onset scoliosis (EOS) is highly prevalent in these patients, our aims were to explore (1) whether ghrelin levels differ between those with and without EOS and correlate with scoliosis severity, and (2) whether ghrelin levels in the first year of life are associated with the later development of EOS. Methods We used a case control study design for the first question and a longitudinal design for the second. Patients with PWS having plasma ghrelin measurements recorded between 2013 and 2018 in our database were selected and 30 children < 10 years old with EOS and 30 age- and BMI-matched controls without EOS were included. The Cobb angle at diagnosis was recorded. In addition, 37 infants with a ghrelin measurement in the first year of life were followed until 4 years of age and assessed for EOS. Total ghrelin (TG), acylated (AG) and unacylated ghrelin (UAG), and the AG/UAG ratio were analyzed. Results EOS children had an AG/UAG ratio statistically significantly lower than controls. The Cobb angle was positively correlated with TG and UAG. TG and AG in the first year of life were higher in infants who later develop EOS without reaching a statistically significant difference. Conclusions Our results suggest that ghrelin may play a role in the pathophysiology of EOS in PWS. Higher ghrelinemia in the first year of life required careful follow-up for EOS. |
| first_indexed | 2024-12-17T09:14:32Z |
| format | Article |
| id | doaj.art-85c4c50f7a6a445abadf58f6047bda63 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-17T09:14:32Z |
| publishDate | 2021-07-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-85c4c50f7a6a445abadf58f6047bda632022-12-21T21:55:02ZengBMCOrphanet Journal of Rare Diseases1750-11722021-07-011611910.1186/s13023-021-01930-1Is ghrelin a biomarker of early-onset scoliosis in children with Prader–Willi syndrome?Dibia Liz Pacoricona Alfaro0Gwenaelle Diene1Graziella Pinto2Jean-Pierre Salles3Isabelle Gennero4Sandy Faye5Catherine Molinas6Marion Valette7Catherine Arnaud8Maithé Tauber9CERPOP, Toulouse University, Inserm - Paul Sabatier UniversityCERPOP, Toulouse University, Inserm - Paul Sabatier UniversityPediatric Endocrinology, Diabetology and Gynecology Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Necker-Enfants Malades HospitalEndocrinology, Obesity, Bone Diseases, Genetics and Gynecology Unit, Children’s Hospital, University Hospital Center of ToulouseToulouse Institute of Infectious and Inflammatory Diseases (Infinity), Inserm UMR1291 - CNRS UMR5051, University Toulouse IIIEndocrinology, Obesity, Bone Diseases, Genetics and Gynecology Unit, Children’s Hospital, University Hospital Center of ToulouseEndocrinology, Obesity, Bone Diseases, Genetics and Gynecology Unit, Children’s Hospital, University Hospital Center of ToulouseCERPOP, Toulouse University, Inserm - Paul Sabatier UniversityCERPOP, Toulouse University, Inserm - Paul Sabatier UniversityEndocrinology, Obesity, Bone Diseases, Genetics and Gynecology Unit, Children’s Hospital, University Hospital Center of ToulouseAbstract Background Adolescents with idiopathic scoliosis display high ghrelin levels. As hyperghrelinemia is found in patients with PWS and early-onset scoliosis (EOS) is highly prevalent in these patients, our aims were to explore (1) whether ghrelin levels differ between those with and without EOS and correlate with scoliosis severity, and (2) whether ghrelin levels in the first year of life are associated with the later development of EOS. Methods We used a case control study design for the first question and a longitudinal design for the second. Patients with PWS having plasma ghrelin measurements recorded between 2013 and 2018 in our database were selected and 30 children < 10 years old with EOS and 30 age- and BMI-matched controls without EOS were included. The Cobb angle at diagnosis was recorded. In addition, 37 infants with a ghrelin measurement in the first year of life were followed until 4 years of age and assessed for EOS. Total ghrelin (TG), acylated (AG) and unacylated ghrelin (UAG), and the AG/UAG ratio were analyzed. Results EOS children had an AG/UAG ratio statistically significantly lower than controls. The Cobb angle was positively correlated with TG and UAG. TG and AG in the first year of life were higher in infants who later develop EOS without reaching a statistically significant difference. Conclusions Our results suggest that ghrelin may play a role in the pathophysiology of EOS in PWS. Higher ghrelinemia in the first year of life required careful follow-up for EOS.https://doi.org/10.1186/s13023-021-01930-1Prader–Willi syndromeEarly-onset scoliosisTotal ghrelinAcylated ghrelinUnacylated ghrelinAG/UAG ratio |
| spellingShingle | Dibia Liz Pacoricona Alfaro Gwenaelle Diene Graziella Pinto Jean-Pierre Salles Isabelle Gennero Sandy Faye Catherine Molinas Marion Valette Catherine Arnaud Maithé Tauber Is ghrelin a biomarker of early-onset scoliosis in children with Prader–Willi syndrome? Orphanet Journal of Rare Diseases Prader–Willi syndrome Early-onset scoliosis Total ghrelin Acylated ghrelin Unacylated ghrelin AG/UAG ratio |
| title | Is ghrelin a biomarker of early-onset scoliosis in children with Prader–Willi syndrome? |
| title_full | Is ghrelin a biomarker of early-onset scoliosis in children with Prader–Willi syndrome? |
| title_fullStr | Is ghrelin a biomarker of early-onset scoliosis in children with Prader–Willi syndrome? |
| title_full_unstemmed | Is ghrelin a biomarker of early-onset scoliosis in children with Prader–Willi syndrome? |
| title_short | Is ghrelin a biomarker of early-onset scoliosis in children with Prader–Willi syndrome? |
| title_sort | is ghrelin a biomarker of early onset scoliosis in children with prader willi syndrome |
| topic | Prader–Willi syndrome Early-onset scoliosis Total ghrelin Acylated ghrelin Unacylated ghrelin AG/UAG ratio |
| url | https://doi.org/10.1186/s13023-021-01930-1 |
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