Multiple endocrine neoplasia type I.
During the 13-year period 1970-1983 only 7 cases of multiple endocrine neoplasia type I (MEN I) were seen at Groote Schuur Hospital, suggesting that the associated gene is rare in this area. Only 1 of these patients was black. Endocrine associations were as follows: hyperparathyroidism--6 cases, pituitary hypersecretion--6 cases (3 each involving growth hormone and prolactin), and pancreatic hypersecretion--3 cases (2 of gastrinoma and 1 of insulinoma). The presenting features were predictably diverse and depended on the component which manifested first. There was little difficulty in reaching a diagnosis on routine investigation. All patients with hyperparathyroidism underwent a 3 1/2-gland parathyroidectomy as the first treatment procedure, normocalcaemia being achieved in 5 cases, but persistent hypercalcaemia in the 6th suggested a supernumerary gland. A pituitary adenoma was removed in 4 cases, but persistent prolactinaemia necessitated bromocriptine therapy in 3. Successful distal pancreatectomy was undertaken in a patient with insulinoma and a patient with gastrinoma, and a further patient with gastrinoma awaits surgery. The overall prognosis in cases of MEN I appears to depend on the most aggressive component, often the pancreatic lesion; our patients have run a surprisingly benign course with only 1 late death, from hypertensive heart disease.
Beukes
E
E
Dent
D M
DM
De Villiers
J C
JC
Miller
J L
JL
D000328:Adult; D005260:Female; D006801:Humans; D006961:Hyperparathyroidism / Q000150:complications; D006965:Hyperplasia; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000150:complications / Q000601:surgery; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000601:surgery; D010280:Parathyroid Glands / Q000473:pathology / Q000601:surgery; D010911:Pituitary Neoplasms / Q000378:metabolism / Q000601:surgery; D015043:Zollinger-Ellison Syndrome