ABSTRACT
Laron syndrome (LS) is an autosomal recessive disease caused by deletions or mutations in the GH receptor gene leading to an inability of insulin-like growth factor I (IGF-I) generation. Among the major resulting body changes are dwarfism and obesity. The only effective treatment is daily administration of biosynthetic IGF-I. Body composition determination by DEXA (dual energy X-ray absorptiometry) of three girls with LS treated by IGF-I for 1, 3 and 11 1/2 years, respectively, revealed that concomitantly with the increase in growth there was a significant increase in body adipose tissue to double or triple the normal values. Due to the underdevelopment of the muscular and skeletal systems body mass index (BMI) did not accurately reflect the degree of obesity. In conclusion, IGF-I similar to insulin, exerts an adipogenic effect.
Subject(s)
Adiposity/drug effects , Insulin-Like Growth Factor I/therapeutic use , Laron Syndrome/metabolism , Child , Child, Preschool , Female , Humans , Insulin-Like Growth Factor I/pharmacology , Laron Syndrome/drug therapyABSTRACT
OBJECTIVE: To test the applicability of a bedside scoring method for screening for diabetic peripheral neuropathy (DPN) in patients with type 1 diabetes mellitus (DM) in an ambulatory clinic. The prevalence of DPN was estimated and its risk factors identified. METHODS: A total of 217 patients (102 males) with type 1 DM, median age 23.4 years (7.5-49 years) and median duration of DM 13.2 years (1-34 years) were evaluated for DPN using the bedside Neuropathy Disability Score (NDS). A score of 3-5 indicated mild DPN, 6-8 moderate DPN and 9-10 severe DPN. The presence of DPN was correlated with possible predictive factors. RESULTS: The NDS was reliable and highly reproducible. The overall prevalence of DPN was 17.1%: mild in 14.3%, moderate in 2.3%, and severe in 0.5% of patients. The prevalence and severity of DPN were significantly related to long-term glycemic control (p < 0.001), DM duration (p < 0.005), age (p = 0.005), and duration of pubertal DM duration (p = 0.03). The prevalence of DPN was significantly associated with the presence of retinopathy (p < 0.002) and overt proteinuria (p < 0.005). CONCLUSIONS: The NDS is a simple, reliable and reproducible screening method for use in the ambulatory clinic to identify the early signs of DPN, leading to early institution of intensive diabetes control measures and preventive foot care.
