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Body composition in untreated adult patients with Laron syndrome (primary GH insensitivity)
Author(s) -
Laron Zvi,
Ginsberg Shira,
Lilos Pearl,
Arbiv Mira,
Vaisman Nahum
Publication year - 2006
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/j.1365-2265.2006.02558.x
Subject(s) - medicine , endocrinology , body mass index , obesity , analysis of variance , trunk , lean body mass , insulin resistance , population , biology , body weight , ecology , environmental health
Summary 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.