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      Total Deficiency of Growth Hormone and Prolactin, and Partial Deficiency of Thyroid Stimulating Hormone in Two Dutch Families: a New Variant of Hereditary Pituitary Deficiency

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          Four out of 10 children in two unrelated families presented with a total pituitary growth hormone (GH) and prolactin deficiency and a partial thyrotropin (TSH) deficiency. The GH gene was intact in family I. The pituitaries, visualized by magnetic resonance imaging, were normal. All children responded well to GH and L-thyroxine therapy. Baseline plasma somatostatin and its peak response to arginine infusion were elevated in family I and they had a milder TSH deficiency than family II. Plasma insulin showed a poor response to arginine infusion. This hereditary combination of pituitary deficiencies suggests a deficiency of a common positive transcription factor.

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          Author and article information

          Hormone Research in Paediatrics
          S. Karger AG
          02 December 2008
          : 32
          : 5-6
          : 170-177
          aDepartment of Pediatrics, Division of Endocrinology, University Hospital for Children and Youth ‘Het Wilhelmina Kinderziekenhuis’, State University Utrecht, The Netherlands; bDepartment of Pediatrics, Division of Endocrinology, State University Groningen, The Netherlands; cEndocrine Laboratory, Bergweg Hospital, Rotterdam, The Netherlands dDepartment of Endocrinology, State University, Utrecht, The Netherlands
          181284 Horm Res 1989;32:170–177
          © 1989 S. Karger AG, Basel

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          Pages: 8
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