Elevated serum proinsulin in beta cell nesidioblastosis. Report of a case in a newborn.
A newborn infant with severe hypoglycaemia and nesidioblastosis was subjected to subtotal pancreatectomy without any sign of improvement. In spite of very low plasma levels of glucose (i.e. less than 1 mmol/l) plasma insulin concentrations were high (i.e. greater than 700 pmol/l). Plasma proinsulin was considerably enhanced comprising 43% of the total insulin immunoreactivity. Plasma glucagon concentrations were normal. Postoperatively normal to subnormal plasma glucose levels could only be maintained by treatment with frequent meals, diazoxide and intramuscular injections of a long-acting glucagon preparation. With time, signs of mental retardation became obvious.
D007516:Adenoma, Islet Cell / Q000097:blood / Q000151:congenital* / Q000601:surgery; D001786:Blood Glucose / Q000032:analysis; D005260:Female; D005934:Glucagon / Q000097:blood; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000097:blood* / Q000601:surgery; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000097:blood / Q000151:congenital* / Q000601:surgery; D011384:Proinsulin / Q000097:blood*
D001786:Blood Glucose; D005934:Glucagon; D011384:Proinsulin