Elevated serum proinsulin in beta cell nesidioblastosis. Report of a case in a newborn.
Scand J Gastroenterol Suppl, 1979;53:49-52.
Kühl C, Hvorslev V, Tygstrup I, Lindkaer Jensen S
PMID: 225788
Abstract
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.
MeSH terms
Adenoma, Islet Cell; Blood Glucose; Female; Glucagon; Humans; Infant, Newborn; Infant, Newborn, Diseases; Pancreatectomy; Pancreatic Neoplasms; Proinsulin
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