Journal of trace elements in medicine and biology : organ of the Society for Minerals and Trace Elements (GMS) · 2012
Kinetics of zinc status and zinc deficiency in Berardinelli-Seip syndrome.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Santos Maria Goretti do Nascimento, Baracho Maria de Fátima Paiva, Vale Sancha Helena de Lima, Leite Lúcia Dantas, Rocha Erika Dantas de Medeiros, de Brito Naira Josele Neves, França Mardone Cavalcante, Almeida Maria das Graças, Chiquetti Samanta Cristina, Marchini Júlio Sérgio, Brandão-Neto José
Аннотация
Berardinelli-Seip syndrome (BSS) is a very rare disorder characterized by near-complete absence of adipose tissue from birth or early infancy, hypoleptinemia, hypertriglyceridemia, insulin resistance, diabetes mellitus, and other clinical signals. It is caused by mutations in AGPAT2 or Gng3lg. We evaluated 10 BSS patients and 10 healthy subjects. A single dose of 382.43 μmol zinc was administered intravenously before and after 3 months of oral zinc supplementation. Blood samples were collected from the contralateral arm at 0, 30, 60, 90, and 120 min after zinc injection. Plasma and serum were obtained to measure hematological and biochemical parameters. Urine was collected to measure creatinine, protein, and zinc. Basal serum zinc levels were similar in controls and BSS patients. However, serum zinc profiles were significant reduced in BSS patients in comparison with controls. The change in total-body zinc clearance was more significant in BSS patients, indicating that these patients had suboptimum zinc deficiency.