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Recurrent Spontaneous Hypoglycaemia Causes Loss of Neurogenic and Neuroglycopaenic Signs in Infants with Congenital Hyperinsulinism.
MedLine Citation:
PMID:  21981106     Owner:  NLM     Status:  Publisher    
Objective:  Hypoglycaemia-associated autonomic failure (HAAF) with impaired neurogenic and neuroglycopaenic responses occurs in adults following recent, repeated hypoglycaemia. We aimed to evaluate whether HAAF also occurs in patients with infant-onset Congenital Hyperinsulinism (CHI). Design, patients:  A controlled fast was performed in a) seven CHI infants with initial symptomatic hypoglycaemia and three recent episodes of spontaneous recurrent hypoglycaemia each lasting less than 5 minutes and in b) seven infants with idiopathic ketotic hypoglycaemia for control. Measurements:  At the time of hypoglycaemia (blood glucose <3 mmol/L or clinical signs), blood was drawn for serum insulin, cortisol, glucagon, epinephrine and nor-epinephrine. Signs of hypoglycaemia were documented. In CHI patients, the ABCC8 and KCNJ11 genes were analyzed by DHPLC and/or direct bidirectional sequencing. Results:  Two CHI patients had a paternal ABCC8 mutation, five had no mutations. When repeated hypoglycaemia was provoked, all CHI patients exhibited a complete loss of clinical signs of hypoglycaemia, along with a global blunting of the counter-regulatory hormones cortisol, glucagon, growth hormone, epinephrine and nor-epinephrine responses (median values 256nmol/L, 23pmol/L, 5.6 mU/L, 390pmol/L and 2.9nmol/L, respectively), irrespective of mutational status. In the controls, hypoglycaemia was always clinically overt with normal counter-regulatory cortisol, glucagon, epinephrine and nor-epinephrine responses (530nmol/L, 60pmol/L, 920pmol/L, and 4.0nmol/L, respectively). Conclusion:  Recurrent hyperinsulinaemic hypoglycaemia even of short duration blunts the autonomic, neuroglycopaenic, and glucose counter-regulatory hormonal responses in patients with infant-onset CHI resulting in clinically silent hypoglycaemia. Tight, or continuous, glucose monitoring is therefore recommended, especially in conservatively treated patients.
H T Christesen; K Brusgaard; K Hussain
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-10-10
Journal Detail:
Title:  Clinical endocrinology     Volume:  -     ISSN:  1365-2265     ISO Abbreviation:  -     Publication Date:  2011 Oct 
Date Detail:
Created Date:  2011-10-10     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0346653     Medline TA:  Clin Endocrinol (Oxf)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Blackwell Publishing Ltd.
H.C. Andersen Children's Hospital Dept. of Clinical Genetics, Odense University Hospital, Denmark London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children NHS Trust, London, UK; The Institute of Child Health, London, UK.
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