Endocrine Abstracts

Primary hyperparathyroidism (PHPT) is commonly the first and most frequent clinical manifestation in MEN1 occurring in up to 95% of patients. Clinical course differs to that of sporadic PHPTH in terms of complications, surgical management and recurrence rates. This study aims to review the challenges of PHPT in MEN1. Retrospective analysis of patients attending a dedicated MEN1 clinic, under long-term review was carried out (n = 26). 85% had PHPT (n = 22; male:female 9:13; age 49.9: range 28-70 years); the most common manifestation. It was the initial presentation in 17 of these 26 patients. Average age at diagnosis of PHPT was 31 years. Calcium was 2.86 mmol/l and PTH 106 pg/mL at diagnosis. Fourteen patients had DEXA scan; all had either osteopenia (43%) or osteoporosis (57%). Renal calculi occurred in 22.7%. All patients underwent parathyroidectomy. Main surgical technique used in the first operation was subtotal parathyroidectomy (3 or 3.5 gland removal) (20 of 22 patients). Limited parathyroidectomy (2 gland removal) was employed in 2 patients. 9% and 54.5% had persistent and recurrent disease, respectively, after first surgery. Recurrence was on average 13 years after first surgery. Rate of remission after first surgery was low at 36.3%. 5 patients required a second surgery (3 achieved remission) and 1 patient required a third operation (now in remission). 31.8% developed hypoparathyroidism post-operatively. The experience in NI of MEN1-related PHPT is similar to other studies. This is a multi-glandular disease of which management is challenging. Recurrence rates are much higher compared with sporadic disease (54.5% vs 1-15%). Effects on bones are more severe in MEN1 (57% vs 40% in sporadic PHPT). Renal effects are similar. The timing of surgery requires multi-disciplinary involvement with close and long term follow up at a dedicated clinic to allow optimal outcomes.