Multiple endocrine neoplasia type 1 (MEN 1) is associated with an increased prevalence of diabetes mellitus and impaired fasting glucose.
OBJECTIVE
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.


METHODS
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.
McCallum
Roland W
RW
Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, Tasmania, Australia.
Parameswaran
Venkat
V
Burgess
John R
JR
D000328:Adult; D001786:Blood Glucose / Q000032:analysis*; D002318:Cardiovascular Diseases / Q000209:etiology; D003920:Diabetes Mellitus / Q000175:diagnosis*; D004812:Epidemiologic Methods; D005215:Fasting / Q000097:blood; D005260:Female; D005951:Glucose Tolerance Test; D006306:Health Surveys; D006706:Homeostasis; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000097:blood / Q000150:complications* / Q000235:genetics; D010375:Pedigree; D010641:Phenotype; D012307:Risk Factors
D001786:Blood Glucose