Introduction and aim: Primary hyperparathyroidism (PHP) is the most frequent endocrine abnormality in MEN1 affecting about 100% of cases and inducing severe bone and kidney complications. Although surgery represents the only curative approach in MEN1 PHP, novel drugs are now available to effectively control PHP. The objective of this study was to show the recent changes in clinical presentation and therapeutic approach in MEN1-related PHP occurred in the last years.
Patients and methods: The study population included 27 consecutive patients with MEN1 related-PHP in follow-up between 1990 and 2010. The differences in clinical presentation of PHP and type and outcome of the therapeutic approaches for PHP between patients diagnosed before 2008 (13) and those diagnosed in 20082010 (14) were investigated.
Results: In the subgroup before 2008, 31% were asymptomatic, while 69% presented nephrolithiasis and/or osteoporosis; in the subgroup 20082010 64% were asymptomatic while 36% presented nephrolithiasis and/or osteoporosis.
Total parathyroidectomy was performed in 31% of cases diagnosed before 2008 and was never performed in those diagnosed in 20082010, while partial parathyroidectomy was performed in 46% of patients diagnosed before 2008 and in 14% of those diagnosed in 20082010. Medical therapy consistent with cinacalcet was administered to 7 patients diagnosed before 2008 (54%), while it was administered to 12 patients diagnosed in 20082010 (86%).
In all of patients treated with cinacalcet, serum calcium levels were normalized and PTH levels significantly decreased. No patient treated with total parathyroidectomy relapsed, however, all of them developed hypoparathyroidism. All but one of the patients undergone partial parathyroidectomy relapsed after surgery.
Conclusions: Clinical presentation of PHP is recently changed in patients with PHP. Based on the attenuation in the severity of clinical manifestations and on availability of an effective medical therapy, the therapeutic management of these patients is suggested to switch from surgery to medical therapy.
30 Apr - 04 May 2011
European Society of Endocrinology