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Hyperinsulinism in syndromal disorders.

AbstractUNLABELLED:
Analysis of a German database comprising a total of 54 patients with neonatal manifestations of persistent hyperinsulinism revealed 5 patients in whom hyperinsulinism was associated with additional clinical symptoms, suggesting an underlying syndromal disorder. Three of the patients presented with a similar yet unknown clinical entity characterized by severe psychomotor retardation, chronic pulmonary disease, hypothyroidism and congenital heart defects. A fourth patient was affected by severe congenital central hypoventilation syndrome. The fifth patient presented with Beckwith-Wiedemann syndrome, with unusually severe and persistent hyperinsulinism requiring subtotal pancreatectomy.
CONCLUSION:
Our results indicate that, in addition to the well-known biochemical pathways, more complex pathophysiological mechanisms can result in persistent hyperinsulinism that presents clinically with a disease involving multiple organs.
AuthorsT Meissner, W Rabl, K Mohnike, S Scholl, R Santer, E Mayatepek
JournalActa paediatrica (Oslo, Norway : 1992) (Acta Paediatr) Vol. 90 Issue 8 Pg. 856-9 (Aug 2001) ISSN: 0803-5253 [Print] Norway
PMID11529530 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Topics
  • Female
  • Humans
  • Hyperinsulinism (drug therapy, etiology)
  • Infant
  • Infant, Newborn
  • Male
  • Syndrome

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