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Martin Astbury
Thank you for your email about glucagon deficiency. There is very little information in this area, The theory is plausible. Measurement of glucose post-mortem is not reliable owing to post-mortem changes. Glucagon itself seems not to have been measured. I wonder whether there is a suitable stain for it in the liver post-mortem that might be applied? There are few researchers in this area. I have attached a reference list of four papers of which we are aware and that might give you some further information.
Cheryl Swann BSc
Research Administrator
Reference List
1. Chanoine JP, Rebuffat E, Kahn A, VanVliet G. Decreased growth hormone response to glucagon in infants after an apnea of infancy. J.Pediatr. 1998;132:452-454.
Reference ID: 4752
Reprint: In File
Abstract: We studied glucagon-induced growth hormone secretion in 9 patients with apnea of infancy and in 55 siblings of children who had died of sudden infant death syndrome, who were included as a comparison group. We observed a 33% decrease in growth hormone secretion in patients with apnea of infancy. However, linear growth remained normal. This finding could be related to either repeated episodes of hypoxia or to abnormal maturation of the autonomous nervous system
2. Chanoine JP, Rebuffat E, Kahn A, Bergmann P, VanVliet G. Glucose, growth hormone, cortisol, and insulin responses to glucagon injection in normal infants, aged 0.5-12 months. J.Clin.Endocrinol.Metab. 1995;80:3032-3035.
Reference ID: 3057
Reprint: Not in File
Abstract: We performed glucagon stimulation tests in 59 normally growing siblings of children who died from sudden infant death syndrome. These investigations were performed to exclude a possible metabolic disorder (found in 4 siblings) as an underlying cause of sudden infant death syndrome. The remaining 55 siblings (32 boys and 23 girls) provide control data for this age range. Testing was performed at 0800 h after a 15-h fast. The median age was 98 days (range, 13-349 days). Plasma glucose and serum cortisol, insulin, and GH were determined before and 30, 60, 90, 120, 150, and 180 min after im injection of 0.1 mg/kg glucagon. No side-effects were observed during the procedure. Asymptomatic hypoglycemia was noted in 11% of the infants at least once between 120-180 min. Basal and peak GH concentrations were greater than 10 micrograms/L in 31% and 80% of the infants, respectively. There was a significant negative correlation between age and basal GH concentration [Spearman's rank correlation coefficient (rs) = -0.37; P < 0.01]. There was a significant correlation between age and glucagon-stimulated cortisol at 120, 150, and 180 min (rs) = 0.41; P < 0.005), but not between age and changes in glucose levels. There was no significant correlation between age and basal cortisol or peak GH concentrations and no difference between boys and girls for any of the variables studied. In conclusion, the glucagon stimulation test is well tolerated in very young subjects. The peak GH response to glucagon injection is independent of age between 0.5-12 months. The age-related increase in the glucagon-stimulated cortisol response despite a similar decrease in glucose suggests the existence of a postnatal maturation in the response of the pituitary-adrenal axis to stress
3. Hawdon JM, Weddell A, Aynsley GA, Ward-Platt MP. Hormonal and metabolic response to hypoglycaemia in small for gestational age infants. Arch.Dis.Child 1993;68:269-273.
Reference ID: 4824
Reprint: In File
Abstract: Little is known of the ability of hypoglycaemic infants who are small for gestational age (SGA) to mount the coordinated hormonal and metabolic counterregulatory response that is seen in healthy older subjects during glycopenia. This response was studied in 22 SGA infants (birth weight < 10th centile) by measuring the blood concentrations of glucose, intermediary metabolites, and glucoregulatory hormones. Plasma non-esterified fatty acid and blood ketone body concentrations were low, even when blood glucose concentrations were low. Plasma insulin and glucagon varied widely (< 1.0-53.1 mU/l and 16.6-87.1 pmol/l, respectively). Concentrations of noradrenaline and glucagon were raised, but cortisol and adrenaline were lower than those found in hypoglycaemic adults. There was no relationship between the concentration of any hormone and blood glucose concentration. We postulate that hypoglycaemia and the failure to mobilise alternative fuels in some SGA infants is secondary both to a poorly coordinated counterregulatory hormone response and to a peripheral insensitivity to the actions of the hormones. Those infants, who fail to mount a counterregulatory response, should be identified by accurate and reliable blood glucose monitoring, and an adequate exogenous supply of energy, either enteral or parenteral, should be ensured
4. Hawdon JM, Aynsley GA, Bartlett K, Ward-Platt MP. The role of pancreatic insulin secretion in neonatal glucoregulation. II. Infants with disordered blood glucose homoeostasis. Arch.Dis.Child 1993;68:280-285.
Reference ID: 4822
Reprint: In File
Abstract: Some neonates, such as those who are preterm or small for dates, become hypoglycaemic or hyperglycaemic. These disorders represent a failure of neonatal metabolic adaptation, but the underlying mechanisms are unclear. Data from studies of hypoglycaemic and hyperglycaemic infants were reviewed in the light of new data from studies of healthy neonates. Data from 28 neonates, who had disordered blood glucose homoeostasis, were analysed to determine the interrelationships between circulating concentrations of glucose, intermediary metabolites, glucagon and insulin, and glucose production rates. Blood glucose concentrations ranged from 2.5 to 26.1 mmol/l, and glucose production rates from 0 to 19.2 mg/kg/min. Blood glucose concentrations were positively related to intravenous glucose infusion rates and to glucose production rates. A negative relationship existed between plasma glucagon and blood glucose concentrations, but there was a wide variation in plasma insulin levels at all blood glucose concentrations. No relationship between either plasma insulin or glucagon concentration and glucose production rate was shown. It is concluded that in neonates with disordered blood glucose homoeostasis, blood glucose concentration is influenced by the rate of administration of glucose, with less precise internal control mechanisms than older subjects. This emphasises the importance of blood glucose monitoring and careful prescribing of exogenous glucose by clinicians caring for such infants
Sarah Kenyon
FSID
14 Halkin Street
London SW1X 7DP
Telephone: 020 7235 0965
Fax: 020 7823 1986
http://www.sids.org.uk/fsid/
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