Serum leptin levels in children and adolescents with spinal muscular atrophy types 2 and 3^✰ - 13/09/22

Doi : 10.1016/j.arcped.2022.08.015

S. Djordjevic ^a,^⁎ , V. Milic-Rasic ^b,^c, V. Brankovic ^b, A. Kosac ^b, I. Dejanovic-Djordjevic ^d, M. Bijelic ^a, T. Dimkic-Tomic ^e, Lj. Markovic-Denic ^c,^f, S. Kovacevic ^g, H. Petrovic ^h, S. Vitorovic ^c, Z. Dobric ^c, V. Zdravkovic ^c,^g
^a Department of Cardiology, University Children's Hospital, Belgrade 11000, Serbia
^b Clinic of Neurology and Psychiatry for Children and Youth, Belgrade 11000, Serbia
^c Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
^d Special Hospital for Treatment of Cerebrovascular Diseases “Saint Sava”, Belgrade 11000, Serbia
^e Clinic for Rehabilitation "Dr. Miroslav Zotovic", Belgrade 11000, Serbia
^f Institute of Epidemiology, Belgrade 11000, Serbia
^g Department of Endocrinology, University Children's Hospital, Belgrade 11000, Serbia
^h Department of Medical Genetics, University Children's Hospital, Belgrade 11000, Serbia

^⁎Corresponding author.

Sous presse. Épreuves corrigées par l'auteur. Disponible en ligne depuis le Tuesday 13 September 2022
Cet article a été publié dans un numéro de la revue, cliquez ici pour y accéder

Abstract

Aim

It has been reported that in patients with spinal muscular atrophy (SMA), lower levels of motor function are associated with hyperleptinemia. Additionally, hyperleptinemia has been found to be more frequent in underweight SMA patients. Therefore, we aimed to analyze serum leptin levels in patients with SMA.

Method

This was a cross-sectional study of pediatric patients (2–19 years old) with SMA types 2 and 3. The investigations included anthropometric measurements, assessment of pubertal status, motor function evaluation using the Hammersmith Functional Motor Scale – Expanded (HFMSE), and serum leptin levels.

Results

In total, 37 patients (22 with type 2 and 15 with type 3 SMA) were included in the study. The male-to-female ratio was 1:1.3 and 62.2% of patients were prepubertal. No statistically significant correlation was found between the HFMSE score and leptin levels, r_s(35) = 0.24, p = 0.15. There was, however, a strong positive relationship between the body mass index (BMI) z-score and leptin levels, r_s(35) = 0.87, p < 0.001.

Conclusion

Serum leptin levels do not seem to be a useful marker of disease severity in children and adolescents with types 2 and 3 SMA. As in the general pediatric population, leptin levels are strongly correlated with BMI, which is a surrogate measure of body fat.

Le texte complet de cet article est disponible en PDF.

Keywords : Spinal muscular atrophy, Leptin, Motor activity, Child, Adolescent

Plan

Introduction

Materials and methods

Patients

Anthropometric measurements and pubertal status

Assessment of motor function

^✰The present work was part of a broader study of cardiac and metabolic abnormalities in pediatric patients with SMA types 2 and 3 that formed the basis for the first author's doctoral thesis. Our previously published articles are referenced in the manuscript.