Open AccessIs ghrelin a biomarker of early-onset scoliosis in children with Prader–Willi syndrome?
Author(s) -
Dibia Liz Pacoricona Alfaro,
Gwénaëlle Diene,
Graziella Pinto,
JeanPierre Salles,
Isabelle Gennero,
Sandy Faye,
Catherine Molinas,
Marion Valette,
Catherine Arnaud,
Maïthé Tauber
Publication year - 2021
Publication title -
orphanet journal of rare diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.274
H-Index - 110
ISSN - 1750-1172
DOI - 10.1186/s13023-021-01930-1
Subject(s) - ghrelin , medicine , scoliosis , cobb angle , idiopathic scoliosis , biomarker , endocrinology , surgery , hormone , chemistry , biochemistry
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.