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Long-term follow-up of patients with congenital hyperinsulinism in Austria
Saadet Mercimek-Mahmutoglu1, Birgit Rami, Martha Feucht
1Department of Pediatrics, Medical University Vienna, Vienna, Austria.
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.
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