Document Detail

Long-term follow-up of 114 patients with congenital hyperinsulinism.
MedLine Citation:
PMID:  12824865     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: The term congenital hyperinsulinism (CHI) comprises a group of different genetic disorders with the common finding of recurrent episodes of hyperinsulinemic hypoglycemia. OBJECTIVE: To evaluate the clinical presentation, diagnostic criteria, treatment and long-term follow-up in a large cohort of CHI patients. PATIENTS: The data from 114 patients from different hospitals were obtained by a detailed questionnaire. Patients presented neonatally (65%), during infancy (28%) or during childhood (7%). RESULTS: In 20 of 74 (27%) patients with neonatal onset birth weight was greatly increased (group with standard deviation scores (SDS) >2.0) with a mean SDS of 3.2. Twenty-nine percent of neonatal-onset vs 69% of infancy/childhood-onset patients responded to diazoxide and diet or to a carbohydrate-enriched diet alone. Therefore, we observed a high rate of pancreatic surgery performed in the neonatal-onset group (70%) compared with the infancy/childhood-onset group (28%). Partial (3%), subtotal (37%) or near total (15%) pancreatectomy was performed. After pancreatic surgery there appeared a high risk of persistent hypoglycemia (40%). Immediately post-surgery or with a latency of several Years insulin-dependent diabetes mellitus was observed in operated patients (27%). General outcome was poor with a high degree of psychomotor or mental retardation (44%) or epilepsy (25%). An unfavorable outcome correlated with infancy-onset manifestation (chi(2)=6.1, P=0.01). CONCLUSIONS: The high degree of developmental delay, in particular in infancy-onset patients emphasizes the need for a change in treatment strategies to improve the unfavorable outcome. Evaluation of treatment alternatives should take the high risk of developing diabetes mellitus into account.
Thomas Meissner; Udo Wendel; Peter Burgard; Silvia Schaetzle; Ertan Mayatepek
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  European journal of endocrinology / European Federation of Endocrine Societies     Volume:  149     ISSN:  0804-4643     ISO Abbreviation:  Eur. J. Endocrinol.     Publication Date:  2003 Jul 
Date Detail:
Created Date:  2003-06-25     Completed Date:  2003-08-14     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9423848     Medline TA:  Eur J Endocrinol     Country:  England    
Other Details:
Languages:  eng     Pagination:  43-51     Citation Subset:  IM    
Clinic of General Pediatrics, University Children's Hospital, Dusseldorf, Germany.
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MeSH Terms
Age of Onset
Child, Preschool
Developmental Disabilities / epidemiology*,  physiopathology
Diabetes Mellitus, Type 1 / epidemiology*,  physiopathology
Diazoxide / therapeutic use
Dietary Carbohydrates / therapeutic use
Follow-Up Studies
Gestational Age
Hyperinsulinism / congenital,  drug therapy,  epidemiology*,  surgery*
Infant, Newborn
Islets of Langerhans / physiopathology,  surgery
Retrospective Studies
Risk Factors
Treatment Outcome
Vasodilator Agents / therapeutic use
Reg. No./Substance:
0/Dietary Carbohydrates; 0/Vasodilator Agents; 364-98-7/Diazoxide

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