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Licensed Unlicensed Requires Authentication Published by De Gruyter October 22, 2020

Long-term Follow-up of Patients with Congenital Hyperinsulinism in Austria

  • Birgit Rami EMAIL logo , Saadet Mercimek-Mahmutoglu , Martha Feucht , Marion Herle , Olaf Rittinger , Sylvia Stoeckler-Ipsiroglu and Edith Schober

Abstract

Aim: To assess the neurological and clinical long-term outcome of patients diagnosed with congenital hyperinsulinism (CHI) in Austria. Patients and Methods: Fourteen patients diagnosed with CHI (1978-2000) were investigated retrospectively by reviewing hospital records. Thirteen of them were evaluated with either a questionnaire or clinical, neurological and biochemical investigations (age at evaluation 4.2- 25.5 years) in a follow-up study in the year 2004. Results: Fifty percent of the patients needed a pancreatectomy. The prevalence of mental retardation was 31%, of epilepsy 15% and of pancreatic insufficiency 14%. None of our patients had developed diabetes mellitus. Additionally the prevalence of obesity was 43% in patients after pancreatectomy. Sixty-nine percent of the patients had no further treatment at the time of follow-up. Conclusion: Despite early diagnosis and intensive treatment, 31% of the patients presented with mental retardation.

Published Online: 2020-10-22
Published in Print: 2008-06-01

© 2020 by Walter de Gruyter Berlin/Boston

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