OBJECTIVE: To quantify body adiposity and its distribution in untreated adult patients with Laron syndrome (LS; primary GH insensitivity) caused by molecular defects of the GH receptor gene or postreceptor pathways and characterized by dwarfism, obesity, insulin resistance and hyperlipidaemia. PATIENTS: Eleven LS patients (seven females and four males) aged 28-53 years were studied. Seven healthy males and six healthy females served as controls. MEASUREMENTS: Body composition of the total body trunk, upper and lower extremities was determined using dual-energy X-ray absorptiometry (DEXA). Statistical analysis using an analysis of variance (anova) and Mann-Whitney nonparametric methods was performed separately in males and females. RESULTS: Percentage body fat in the LS patients was much higher (P < 0.01) than that in the control population and the female LS patients were significantly more obese (59% total body fat) than the male patients (39% total body fat) (P < 0.002). It was also evident that in these types of patients with markedly increased body fat and decreased muscle and bone mass, body mass index (BMI) does not accurately reflect the body composition. CONCLUSIONS: Lifelong congenital IGF-I deficiency leads to extreme adiposity.
OBJECTIVE: To quantify body adiposity and its distribution in untreated adult patients with Laron syndrome (LS; primary GH insensitivity) caused by molecular defects of the GH receptor gene or postreceptor pathways and characterized by dwarfism, obesity, insulin resistance and hyperlipidaemia. PATIENTS: Eleven LS patients (seven females and four males) aged 28-53 years were studied. Seven healthy males and six healthy females served as controls. MEASUREMENTS: Body composition of the total body trunk, upper and lower extremities was determined using dual-energy X-ray absorptiometry (DEXA). Statistical analysis using an analysis of variance (anova) and Mann-Whitney nonparametric methods was performed separately in males and females. RESULTS: Percentage body fat in the LS patients was much higher (P < 0.01) than that in the control population and the female LS patients were significantly more obese (59% total body fat) than the male patients (39% total body fat) (P < 0.002). It was also evident that in these types of patients with markedly increased body fat and decreased muscle and bone mass, body mass index (BMI) does not accurately reflect the body composition. CONCLUSIONS: Lifelong congenital IGF-I deficiency leads to extreme adiposity.
Authors: Darlene E Berryman; Edward O List; Amanda J Palmer; Min-Yu Chung; Jacob Wright-Piekarski; Ellen Lubbers; Patrick O'Connor; Shigeru Okada; John J Kopchick Journal: J Gerontol A Biol Sci Med Sci Date: 2009-11-09 Impact factor: 6.053
Authors: Edward O List; Lucila Sackmann-Sala; Darlene E Berryman; Kevin Funk; Bruce Kelder; Elahu S Gosney; Shigeru Okada; Juan Ding; Diana Cruz-Topete; John J Kopchick Journal: Endocr Rev Date: 2010-12-01 Impact factor: 19.871
Authors: Edward O List; Darlene E Berryman; Kevin Funk; Elahu S Gosney; Adam Jara; Bruce Kelder; Xinyue Wang; Laura Kutz; Katie Troike; Nicholas Lozier; Vincent Mikula; Ellen R Lubbers; Han Zhang; Clare Vesel; Riia K Junnila; Stuart J Frank; Michal M Masternak; Andrzej Bartke; John J Kopchick Journal: Mol Endocrinol Date: 2013-01-24
Authors: Veronique Groleau; Joan I Schall; Virginia A Stallings; Christina A Bergqvist Journal: Dev Med Child Neurol Date: 2014-04-20 Impact factor: 5.449