title: Long-term follow-up of patients with congenital hyperinsulinism in Austria.
abstract: OBJECTIVE
  To assess the neurological and clinical long-term outcome of patients diagnosed with congenital hyperinsulinism (CHI) in Austria.
  
  
  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.
  
  
  CONCLUSIONS
  Despite early diagnosis and intensive treatment, 31% of the patients presented with mental retardation.
authors: Department of Pediatrics, Medical University Vienna, Vienna, Austria.
authors: Saadet
authors: S
authors: Mercimek-Mahmutoglu
authors: Birgit
authors: B
authors: Rami
authors: Martha
authors: M
authors: Feucht
authors: Marion
authors: M
authors: Herle
authors: Olaf
authors: O
authors: Rittinger
authors: Sylvia
authors: S
authors: Stoeckler-Ipsiroglu
authors: Edith
authors: E
authors: Schober
mesh_terms: D000293:Adolescent; D000328:Adult; D001317:Austria; D002648:Child; D002675:Child, Preschool; D044903:Congenital Hyperinsulinism / Q000150:complications / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000209:etiology; D008297:Male; D010180:Pancreatectomy; D012189:Retrospective Studies; D013997:Time Factors