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- Title
Hyperinsulinism in syndromal disorders.
- Authors
Meissner, T; Rabl, W; Mohnike, K; Scholl, S; Santer, R; Mayatepek, E
- Abstract
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.
- Subjects
NEONATAL diseases; INSULIN shock
- Publication
Acta Paediatrica, 2001, Vol 90, Issue 8, p856
- ISSN
0803-5253
- Publication type
Article
- DOI
10.1111/j.1651-2227.2001.tb02445.x