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, rs(35) = 0.24, p = 0.15. There was, however, a strong positive relationship between the body mass index (BMI) z-score and leptin levels, rs(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.
Subject(s)
Muscular Atrophy, Spinal , Spinal Muscular Atrophies of Childhood , Adolescent , Adult , Child , Child, Preschool , Cross-Sectional Studies , Female , Humans , Leptin , Male , Severity of Illness Index , Spinal Muscular Atrophies of Childhood/diagnosis , Young AdultABSTRACT
Background: Adipose tissue is a dynamic endocrine organ, a highly active metabolic tissue, and an important source of cytokines. Inflammatory factors play an important role in visceral obesity associated with insulin resistance (IR), metabolic syndrome (MS), hypertension, non-alcoholic fatty liver disease (NAFLD), diabetes mellitus type 2 (DM2), endothelial dysfunction (ED) and atherosclerosis. Objectives: To examine corelation of siMS score, as a quantification method for metabolic syndrome (MS), with insulin resistance, glucoregulation parameters, as with other co-founding factors of MS, inflammation and thrombosis factors, microalbuminuria, uric acid, fatty liver index (FLI) and homocysteine. Methods: The study included 451 obese individuals with pre-metabolic syndrome (pre-MS) and MS (age 16-75, body mass index (BMI) > 25kg/m2) classified into two groups: I-age 10-30 (167 patients); II-age 31-75 (284 patients). International Diabetes Federation (IDF) classification was applied for diagnosing metabolic syndrome. Patients with less than three criteria indicated below were considered pre-metabolic syndrome. siMS risk score was used. Results: siMS score increased with age: I-3.03 ± 0.87, II-3.27 ± 0.90. siMS score correlated with associated factors of MS: hyperinsulinemia and IR, ALT, gama-GT, FLI, uric acid in both groups and CRP (p < 0.01) in group I. Correlations in II group: siMS score with PAI-1 (p = 0.01), microalbuminuria (p = 0.006), homocysteine ââ(p = 0.076). Conclusion: Correlation of siMS score with HOMA-IR confirmed that hyperinsulinism and insulin resistance are in the basis of MS. Correlation of siMS score with parameters of NAFLD, CRP, PAI-1, uric acid, microalbuminuria and homocysteine indicates that they are significant co-founding factors of MS. Correlation of siMS score with PAI-1, microalbuminuria, homocysteine, indicates higher risk for progression of endothelial dysfunction and atherosclerosis with age.
