title: Glucose metabolism and hypoglycaemia in SIDS.
abstract: Once a child is born its survival depends on the maturation of the blood glucose homeostatic control mechanisms. When this fails or where there is an inborn error of metabolism the infant is susceptible to potentially fatal hypoglycaemic episodes. A variety of environmental stresses, either singly or in combination, such as inappropriate or low caloric intake, acute infections of childhood, endotoxaemia, fever, xenobiotic exposure, oxidative stress or anaphylaxis, can greatly exacerbate the deficiency of the normal homeostatic compensatory mechanism and result in the onset of hypoglycaemia. Various inborn errors have been found in infants who died of SIDS. Our approach to this problem has been to use the six microsomal glucose-6-phosphatase proteins as a model system to study defects in carbohydrate metabolism in cases of SIDS. Initial studies determined the ontogeny of the glucose-6-phosphatase proteins and showed that intact microsomes isolated from unfrozen liver samples can be used to study glucose-6-phosphatase in cases of SIDS that were presumably due to the low concentrations of liver lipid peroxidation. More recently we have used a combination of techniques to demonstrate the abnormalities of glucose-6-phosphatase in cases of SIDS. Classic gross pathology and histology have now clearly defined the various subgroups of sudden and unexpected deaths of infancy. This now enables us to develop new molecular approaches to predict and prevent hypoglycaemia in infants who are at risk of SIDS.
authors.affiliation: Department of Obstetrics & Gynaecology, Ninewells Hospital, Dundee.
authors.forename: A
authors.initials: A
authors.lastname: Burchell
authors.forename: H
authors.initials: H
authors.lastname: Lyall
authors.forename: A
authors.initials: A
authors.lastname: Busuttil
authors.forename: E
authors.initials: E
authors.lastname: Bell
authors.forename: R
authors.initials: R
authors.lastname: Hume
mesh_terms: D001786:Blood Glucose / Q000378:metabolism*; D005952:Glucose-6-Phosphatase / Q000378:metabolism; D006801:Humans; D007003:Hypoglycemia / Q000150:complications*; D007223:Infant; D007231:Infant, Newborn; D008862:Microsomes, Liver / Q000201:enzymology; D013398:Sudden Infant Death / Q000097:blood / Q000209:etiology*
chemical_list: D001786:Blood Glucose; D005952:Glucose-6-Phosphatase