Abstract
BackgroundAdolescents 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.MethodsWe 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.ResultsEOS 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.ConclusionsOur 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.
Highlights
Ghrelin, a 28-amino acid peptide hormone, was first described in 1999 in its acylated form (AG)
Our study focuses on early-onset scoliosis (EOS) in Prader–Willi syndrome (PWS) and investigates (i) whether ghrelin levels at diagnosis of scoliosis differ between PWS children with and without EOS, and correlate with scoliosis severity and (ii) whether ghrelin concentrations in the first year of life before growth hormone (GH) treatment are associated with the later EOS development
Key results Our study shows that children with PWS have a significantly lower Acylated ghrelin (AG)/unacylated ghrelin (UAG) ratio at the time of their EOS diagnosis than age- and body mass index (BMI)-matched PWS children without scoliosis, with a tendency for lower AG, suggesting that ghrelin may be a biomarker of scoliosis in PWS
Summary
A 28-amino acid peptide hormone, was first described in 1999 in its acylated form (AG). It is the endogenous ligand of the GHSR1a receptor [1]. Recent studies have documented increased ghrelin levels in adolescent girls with idiopathic scoliosis [9,10,11]. Scoliosis is a deformity in the three spinal axes with an angle on the frontal plane (Cobb angle) ≥ 10°. It most frequently affects peri-pubertal girls with a lean constitution and, in this case, is called adolescent idiopathic scoliosis (AIS) [12]. 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
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