Characterization of fat distribution in Prader–Willi syndrome: Relationships with adipocytokines and influence of growth hormone treatment

Abstract

Marked anthropometric changes are seen in Prader-Willi syndrome (PWS). Emaciation is observed during infancy, whereas severe obesity is found in older children and adults. Growth hormone (GH) treatment modifies the anthropometric changes in PWS patients. In this study, we examined changes in the body composition of 51 PWS patients (age range, 6-54 years; median, 16.5 years), with a focus on the amount of abdominal visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT), VAT/SAT ratio, and serum levels of adipocytokines (adiponectin, leptin, and resistin). The relationships between VAT, SAT, and adipocytokines, and lipid abnormalities and type 2 diabetes in 24 patients with obese PWS were also evaluated. With increasing age, SAT and VAT both increased markedly, but in 18 patients receiving GH treatment, VAT remained low at ≤30 cm(2) . In the GH-completed patients (n = 19), VAT and SAT increased with age to levels similar to those in non-GH-treated patients (n = 14). In the obese group, adiponectin decreased as VAT increased (r = -0.35, P = 0.11). Leptin (r = 0.67, P < 0.001) and resistin (r = 0.45, P = 0.04) showed positive correlations with SAT. Total cholesterol, low-density lipoprotein, and triglyceride levels correlated negatively with adiponectin (r = -0.59, r = -0.56, r = -0.56, respectively, P < 0.05) and hemoglobin A1c (r = -0.42, P = 0.08). To maintain lower VAT and prevent cardiovascular disease risk factors, GH treatment may be advisable even in adult patients with PWS.

Dietary Energy Intake, Body Composition and Resting Energy Expenditure in Prepubertal Children with Prader-Willi Syndrome before and during Growth Hormone Treatment: A Randomized Controlled Trial

“…It could also be explained by an altered adipocyte function during GH treatment. Wölfing et al [44] showed that GH treatment stimulates secretion of adiponectin in cultured adipocytes, and a Japanese study observed a negative relation between the amount of visceral adipose tissue and adiponectin levels in patients with PWS [45]. However, clinical studies in girls with Turner syndrome and in short children born small for gestational age showed no significant relation between GH treatment and adiponectin [46,47].…”

Obesity and Metabolic Syndrome in Adults with Prader-Willi Syndrome

“…In adult GH deficiency, continued GH treatment has already been reported to maintain body composition, improve lipid abnormalities, and reduce the risk of CVD 36) . Considering PWS, Tanaka et al 37) reported that during GH treatment, although scAT increased, VAT remained low. However, after indications for GH in PWS were no longer met and GH treatment had been discontinued, the degree of obesity was similar to that of the group never treated with GH.…”

Energy Homeostasis in children with Prader- Willi syndrome

“…The adiposity was low among adult PWS patients while continuing growth hormone therapy, but it increased with age to similar levels to untreated patients once growth hormone was ceased (Tanaka, Abe et al 2013). In obese subgroup of adult PWS patients, adiponectin, which is cardio-protective (Bluher and Mantzoros 2015), was low as visceral adiposity increased, and was negatively correlated with total cholesterol, low-density lipoprotein and triglyceride (Tanaka, Abe et al 2013). During childhood the metabolic phenotype in PWS is more favourable compared to obese controls (Haqq, Muehlbauer et al , Brambilla, Crino et al 2011).…”

“…In an adult PWS study, both visceral and subcutaneous adipose tissue increased with increasing age (Tanaka, Abe et al 2013). The adiposity was low among adult PWS patients while continuing growth hormone therapy, but it increased with age to similar levels to untreated patients once growth hormone was ceased (Tanaka, Abe et al 2013). In obese subgroup of adult PWS patients, adiponectin, which is cardio-protective (Bluher and Mantzoros 2015), was low as visceral adiposity increased, and was negatively correlated with total cholesterol, low-density lipoprotein and triglyceride (Tanaka, Abe et al 2013).…”

“…Another study on PWS patients with mean age of 28 years showed they had higher high-sensitivity C-reactive protein, less exercise capacity, and greater microvascular dysfunction, that are associated with coronary artery disease (Patel, Harmer et al 2007). In an adult PWS study, both visceral and subcutaneous adipose tissue increased with increasing age (Tanaka, Abe et al 2013). The adiposity was low among adult PWS patients while continuing growth hormone therapy, but it increased with age to similar levels to untreated patients once growth hormone was ceased (Tanaka, Abe et al 2013).…”