Endocrine Abstracts

The brain is dependent on a continuous supply of glucose as its source of energy, the rate-limiting step being the rate of transport across the blood-brain barrier. Glucose deprivation of the brain causes neuroglycopenia and rapid malfunction. This is demonstrated by the development of abnormal neurophysiological function with slowing of neural transmission and electroencephalographic changes, and by the progressive impairment of cognitive function, affecting several domains. ...

Non-islet cell tumour hypoglycaemia (NICTH) is a syndrome associated with overproduction of pro-IGF-II, a 14-18 kDa product of incomplete proteolytic cleavage of IGF-II pro-hormone, usually secreted by large mesenchymal tumours. The excess of pro-IGF-II in NICTH leads to a reduction in circulating insulin, GH, IGF-I, IGFBP-3 and acid-labile subunit (ALS) levels and to an increase in IGF binding protein-2 (IGFBP-2). IGFs and IGFBP-3 are found primarily in binary complexes of ap...

Hyperinsulinism in Infancy (HI) is a potentially-lethal condition of neonates and during early childhood. For many years the pathophysiology of this disorder was unknown. Recent advances in genetics, histopathology and molecule physiology have now revealed the causes of HI in a large cohort of patients. This review focuses upon the relationship between the basis of HI and current treatment options. From defects in ion channel subunit genes to lesions in the control of pancreat...