Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant syndrome characterized by primary hyperparathyroidism, pituitary neoplasia and foregut lineage neuroendocrine tumours. It has also been associated with premature cardiovascular death. As diabetes is a risk factor for increased cardiovascular mortality we investigated the prevalence and clinical correlates of glycaemic abnormalities in a large MEN 1 kindred.Patients and design
The glycaemic status of 72 MEN 1 affected and 133 unaffected members of a single large MEN 1 pedigree was assessed. Fasting glucose results were categorized and compared using WHO criteria. Associations between glycaemic status and MEN 1 phenotype were assessed.Results
Thirteen (18·1%) patients with MEN 1 compared to 5 (3·8%) control patients were diabetic (P < 0·001). Six (8·3%) MEN 1 patients had impaired fasting glucose compared to 4 (3%) of controls (P < 0·05). Of patients with MEN 1, uncontrolled hypercalcaemia (P < 0·05) and elevated serum gastrin (P < 0·05) were more common amongst patients diagnosed with abnormal glycaemia than those with normoglycaemia. There was a nonsignificant trend for elevated chromogranin A, pancreatic polypeptide, gastric inhibitory polypeptide (but not glucagon) and history of bronchopulmonary carcinoid in MEN 1 patients with elevated glycaemia.Conclusions
Diabetes and impaired fasting glucose occur significantly more frequently amongst MEN 1 patients than controls and is associated with uncontrolled hyperparathyroidism and evidence of enteropancreatic hyperstimulation.