Primary hyperparathyroidism in familial multiple endocrine neoplasia type I. Long-term follow-up of serum calcium levels after parathyroidectomy

Abstract

Serum calcium levels were analyzed after one or more explorations for primary hyperparathyroidism in familial multiple endocrine neoplasia type I. These data covered all 85 operations (performed in many hospitals) on 61 of 62 members from 14 kindreds. After 61 initial operations, there were high rates of persistent or recurrent hypercalcemia (54 percent) and chronic hypocalcemia (10 percent). These rates contrast with lower postoperative rates of hypercalcemia (4 to 16 percent) or chronic hypocalcemia (1 to 8 percent) in large series of primary hyperparathyroidism. Persistent or recurrent hypercalcemia after initial exploration decreased only modestly in patients who underwent surgery after 1975 versus before 1975 (46 versus 63 percent). The rate for long-term remission of hypercalcemia after initial parathyroidectomy was higher after a diagnosis of parathyroid hyperplasia was made (as opposed to adenoma) (57 versus 30 percent, p less than 0.05) and after removal of three or more glands (as opposed to removal of two and a half or less) (70 versus 34 percent, p less than 0.01). Following 24 reoperations, there were also high rates of persistent or recurrent hypercalcemia (46 percent) and chronic hypocalcemia (25 percent). After surgery in unselected patients with primary hyperparathyroidism, recurrent hypercalcemia (as opposed to persistent hypercalcemia) is distinctly uncommon; however, it was frequent in familial multiple endocrine neoplasia type I, with total recurrences increasing from 21 percent at five years to 41 percent at 10 years in patients who showed a normocalcemic interval after surgery. The data indicate that the occurrence of persistent or recurrent hypercalcemia after parathyroidectomy in familial multiple endocrine neoplasia type I remains frequent. Although recurrent hypercalcemia may be characteristic of the response to any technique of parathyroidectomy in familial multiple endocrine neoplasia type I and not preventable, persistent hypercalcemia can be decreased by preoperative recognition of the specific familial cause, involvement of an experienced surgical team, and histologic confirmation of the identification of three or more parathyroid glands.