Document Detail


Recurrent Spontaneous Hypoglycaemia Causes Loss of Neurogenic and Neuroglycopaenic Signs in Infants with Congenital Hyperinsulinism.
MedLine Citation:
PMID:  21981106     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
H T Christesen; K Brusgaard; K Hussain
Related Documents :
1853716 - Association of neonatal thrombocytopenia and maternal anti-hla antibodies.
16014826 - A serosurvey to identify the window of vulnerability to wild-type measles among infants...
18332766 - Association of hiv and malaria with mother-to-child transmission, birth outcomes, and c...
2214076 - Transmission of hiv-1 infections from mothers to infants in haiti. impact on childhood ...
14626336 - Safety and tolerability of methacholine challenge in infants with recurrent wheeze.
12281546 - Socio-demographic correlates of divorce in new zealand.
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.
Affiliation:
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.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Body Mass Index, Sexual Difficulties and Sexual Satisfaction among people in regular heterosexual re...
Next Document:  New Cyclopentenyl-Linked [NPN] Ligands and Their Coordination Chemistry with Zirconium: Synthesis of...