Long-term follow-up of patients with congenital hyperinsulinism in Austria.
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.
Mercimek-Mahmutoglu
Saadet
S
Department of Pediatrics, Medical University Vienna, Vienna, Austria.
Rami
Birgit
B
Feucht
Martha
M
Herle
Marion
M
Rittinger
Olaf
O
Stoeckler-Ipsiroglu
Sylvia
S
Schober
Edith
E
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