Adult patients with Laron syndrome tend to develop the metabolic syndrome

Abstract

BackgroundThe metabolic Syndrome is the name of a cluster of abnormal clinical and metabolic states, which constitute a risk factor for diabetes and cardiovascular disease. AimTo determine whether adult patients with Laron Syndrome with excessive obesity develop the characteristics of the Metabolic Syndrome. SubjectsOut of a cohort of adult patients with Laron Syndrome followed in our clinic, records of 23 patients (12 females, 11 males) were found to have sufficient data for analysis. MethodsThe degree of obesity was determined by the measurement of subscapular skinfold thickness (SSFT), BMI and total body DEXA. NAFLD was determined by liver ultrasonography, serum lipids including adiponectin leptin, insulin and glucose were assessed by radioimmunoassay. ResultsBoth female and male patients were markedly obese with 59% and 39% fat of the total body mass respectively, as were total and LDL cholesterol, triglycerides and adiponectin. Some had developed NAFLD. They also suffered from insulin resistance and glucose intolerance. Eleven patients (3 females, 8 males) developed diabetes. All had varying degrees of hypertension. Eight subjects (3 females, 5 males) suffered from cardiovascular disease. One female died at aged 53 years, and two males died at ages 75 and 78 years. ConclusionWith advancing age and increasing obesity, adult patients with Laron Syndrome developed the characteristics of Metabolic Syndrome including diabetes and cardiovascular disease.