Screening for multiple endocrine neoplasia syndrome (type 1) in patients with primary hyperparathyroidism.
In 63 consecutive patients with primary hyperparathyroidism (HPT) a prospective screening study was undertaken for coexistent multiple endocrine neoplasma-(MEN)-syndrome type 1. The screening consisted of a clinical examination, a radiological examination of the sella turcica with skeletal tomography (and in equivocal cases computed tomography), visual field examination by perimetry and a hormonal evaluation including measurements of the serum levels of prolactin, gastrin, pancreatic polypeptide (PP) and subunits of human chorionic gonadotrophin (HCG-alpha and -beta). Clinical examination did not reveal any signs of endocrine disease suggestive of a MEN-1 syndrome. In only one case there was a radiological abnormality of the sella turcica; this patient had an empty sella syndrome and a raised serum prolactin value. All other prolactin values were within the normal range. In 41% of the patients raised serum gastrin levels were found; these tended to normalize after parathyroidectomy. As a group, patients with raised gastrin values were older than the others and generally they had hypo- or achlorhydria. The serum PP levels were raised in 28% of the patients but there was no clinical evidence of a pancreatic tumour in any of these cases, and the serum HCG-alpha and -beta levels were within the normal range in all patients but two. We conclude that the incidence of MEN-1 syndrome in unselected patients with primary HPT must be low, and that investigations for this syndrome are justified only in HPT patients with specific symptoms or with a positive family history.
D000328:Adult; D000368:Aged; D006063:Chorionic Gonadotropin / Q000097:blood; D005260:Female; D005755:Gastrins / Q000097:blood; D006801:Humans; D006961:Hyperparathyroidism / Q000097:blood / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D010191:Pancreatic Polypeptide / Q000097:blood; D011388:Prolactin / Q000097:blood; D011859:Radiography; D012658:Sella Turcica / Q000000981:diagnostic imaging
D006063:Chorionic Gonadotropin; D005755:Gastrins; D010191:Pancreatic Polypeptide; D011388:Prolactin