title: Effect of diet intervention and oral zinc supplementation on metabolic control in Berardinelli-Seip syndrome.
abstract: OBJECTIVE
  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.
authors.affiliation: Universidade Federal do Rio Grande do Norte, Natal, Brazil.
authors.forename: Erika
authors.initials: E
authors.lastname: Dantas de Medeiros Rocha
authors.forename: Lúcia
authors.initials: L
authors.lastname: Dantas Leite
authors.forename: Maria
authors.initials: M
authors.lastname: de Fátima Paiva Baracho
authors.forename: Maria
authors.initials: M
authors.lastname: Goretti do Nascimento Santos
authors.forename: Dina Maria
authors.initials: DM
authors.lastname: de Araújo
authors.forename: Mardone
authors.initials: M
authors.lastname: Cavalcante França
authors.forename: Carlos Antônio Bruno
authors.initials: CA
authors.lastname: da Silva
authors.forename: Maria
authors.initials: M
authors.lastname: das Graças Almeida
authors.forename: Adriana Augusto
authors.initials: AA
authors.lastname: Rezende
authors.forename: Júlio Sérgio
authors.initials: JS
authors.lastname: Marchini
authors.forename: José
authors.initials: J
authors.lastname: Brandão-Neto
mesh_terms: D000273:Adipose Tissue / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D003924:Diabetes Mellitus, Type 2 / Q000378:metabolism; D019587:Dietary Supplements; D002149:Energy Intake; D005215:Fasting; D005247:Feeding Behavior; D005260:Female; D006442:Glycated Hemoglobin / Q000378:metabolism; D006529:Hepatomegaly / Q000503:physiopathology; D006801:Humans; D007328:Insulin / Q000097:blood; D007333:Insulin Resistance; D020738:Leptin / Q000097:blood; D052497:Lipodystrophy, Congenital Generalized / Q000178:diet therapy*; D008297:Male; D055815:Young Adult; D015032:Zinc / Q000008:administration & dosage* / Q000097:blood
chemical_list: D006442:Glycated Hemoglobin; D007328:Insulin; D020738:Leptin; D015032:Zinc