ABSTRACT
BACKGROUND/AIMS: Berardinelli-Seip syndrome (BSS), also termed congenital generalized lipodystrophy or congenital generalized lipoatropic diabetes, is a rare autosomal recessive disease characterized by the nearly complete absence of metabolically active adipose tissue from birth, extreme insulin resistance, diabetes mellitus, and hepatomegaly. The aim of this study was to evaluate the effect of diet intervention and oral zinc supplementation on the metabolic control of BSS patients. METHODS: During a 3-month period, 10 BSS patients received individualized diets and oral zinc supplementation. Food intake, clinical laboratory parameters, serum zinc and leptin, and plasma C-peptide concentrations were evaluated at the beginning of the study and after 3 months. RESULTS: At the beginning of the study, all patients had elevated energy, protein, total fat, carbohydrate, calcium, iron, and zinc intakes. After 3 months, all of these parameters had decreased. Total fiber intakes remained low before and after diet intervention and oral zinc supplementation, and plasma levels of fasting glucose remained high. In contrast, glycated hemoglobin decreased significantly. Plasma leptin, C-peptide, and serum zinc levels increased during venous zinc tolerance testing, but there were no significant differences between the 2 curves obtained before and after diet intervention and oral zinc supplementation. CONCLUSIONS: Diet intervention and oral zinc supplementation were effective at controlling energy consumption, macronutrients, and glycated hemoglobin. Zinc likely acts as an adjunct therapy, thereby improving the effectiveness of leptin.
Subject(s)
Dietary Supplements , Lipodystrophy, Congenital Generalized/diet therapy , Zinc/administration & dosage , Adipose Tissue/metabolism , Adolescent , Adult , Diabetes Mellitus, Type 2/metabolism , Energy Intake , Fasting , Feeding Behavior , Female , Glycated Hemoglobin/metabolism , Hepatomegaly/physiopathology , Humans , Insulin/blood , Insulin Resistance , Leptin/blood , Male , Young Adult , Zinc/bloodABSTRACT
The purpose of this study was to identify the effect of oral zinc supplementation in patients with type 1 diabetes (T1DM) on metabolic control and zinc blood concentrations. The sample consisted of 20 patients with T1DM and a control group (n=17). Metabolic control was evaluated by glycemia at fast, 24 h glycosuria, and HbA1c. Zinc concentrations were measured in plasma and erythrocytes. After the first collection of biological material, oral zinc supplementation was initiated and continued for 4 mo in T1MD patients (T1). Daily dosages were established based on Dietary Recommended Intakes (DRIs), considering zinc intake based on data from other studies previously performed with this population. All analyses were repeated after supplementation (T2). Metabolic control was unsatisfactory, with an HbA1c increase at T2. There was no difference in zinc concentrations in plasma and erythrocytes between patients with T1DM and control. Zinc concentrations in plasma were within the normal range in T1MD before and after supplementation and the control. Zinc concentrations in erythrocyte presented lower than normal values for all groups. A zinc increase in erythrocyte after supplementation was observed in T1DM patients, although without statistical significance. More studies are needed to confirm oral zinc supplementation as nutritional management in diabetes.
