Body composition in untreated adult patients with Laron syndrome (primary GH insensitivity).
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.


METHODS
Eleven LS patients (seven females and four males) aged 28-53 years were studied. Seven healthy males and six healthy females served as controls.


METHODS
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.
Laron
Zvi
Z
Endocrinology and Diabetes Research Unit, Schneider Children's Medical Centre, Petah Tikva, Israel. laronz@clalit.org.il
Ginsberg
Shira
S
Lilos
Pearl
P
Arbiv
Mira
M
Vaisman
Nahum
N
D015502:Absorptiometry, Photon; D000328:Adult; D001823:Body Composition; D015992:Body Mass Index; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000172:deficiency; D046150:Laron Syndrome / Q000503:physiopathology*; D008055:Lipids / Q000097:blood; D008297:Male; D008875:Middle Aged; D012737:Sex Factors; D018709:Statistics, Nonparametric
D008055:Lipids; D007334:Insulin-Like Growth Factor I