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      Congenital Hypoaldosteronism

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      Hormone Research in Paediatrics

      S. Karger AG

      Adrenal, Hypoaldosteronism, 18-Oxidation defect

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          Identical male twins suffering from congenital hypoaldosteronism due to a rare adrenal enzyme deficiency between corticosterone and aldosterone were followed-up from birth till their present age of 13 years. The symptoms of salt loss disappeared and normal growth rate resumed following treatment with DOCA and salt supplementation. Discontinuation of mineralocorticoid administration at the age of 7 years resulted during a 5-year period in a marked decline in their growth rate. Laboratory data revealed a persistent, albeit less pronounced, metabolic impairment. Mineralocorticoid administration was resumed and the twins entered normal puberty and increased their growth rate, emphasizing their need for continued mineralocorticoid administration to maintain adequate growth rate and development.

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          Author and article information

          Horm Res Paediatr
          Hormone Research in Paediatrics
          S. Karger AG
          25 November 2008
          : 11
          : 1
          : 22-28
          Department of Pediatrics and Institute of Endocrinology, Chaim Sheba Medical Center, Tel Hashomer
          179034 Horm Res 1979;11:22–28
          © 1979 S. Karger AG, Basel

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          Pages: 7
          Original Paper


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