Abstract
Background: Surgery is the elective therapy for primary hyperparathyroidism (HPT) in patients with multiple endocrine neoplasia type 1 (MEN1). As MEN1 mutation carriers invariably will develop HPT up to 50 years, the annual periodic screening have allowed an early diagnosis of HPT in MEN1 syndrome, frequently before manifestation of clinical symptoms. Health-related quality of life (HR-QoL) both before after parathyroidectomy (PTx) has been poorly described. As the ideal timing to indicate PTx is yet controversial and undefined and, most recently, the value of less extensive surgeries has been considered for some groups, data on HR-QoL may be useful to clarify better these conflicting issues. Objectives: To investigate post-operative HR-QoL aspects of patients submitted to PTx for MEN1-related HPT (HPT/MEN1). Methods: Prospective analysis of HPT/MEN1 patients submitted to total PTx with immediate forearm autograft (TPTx+A) or subtotal parathyroidectomy (SPTx) in single institution. A psychologist applied Short Form 36 Health Survey Questionnaire immediately before and 6 months after surgery. Changes of both Physical Component Summary Score (PCS) and Mental Component Summary Score (MCS) were analyzed considering persistence of HPT, hypoparathyroidism and pancreatectomy after the PTx. Results: Of 19 patients (mean age 38.4±14.4, 7 men), 8 underwent TPTx+A and 11 had SPTx. Overall, median (interquartiles ranges) of pre-operative PCS and MCS were 73.8 (50.0-88.0) and 65.0 (44.5-83.2), respectively. At 6 months, median PCS was 71.2 (55.8-91.0) and median MCS was 80.2 (46.8-88.0). There were five (4, PTxT+A; 1, SPTx) patients with postoperative hypoparathyroidism, one with persistent HPT (SPTx) and 13 with normal parathyroid function (4, TPTx+A; 9, SPTx) six months after PTx. PCS worsened in four cases with hypoparathyroidism (80%) and in five euparathyroid (36%), but without statistical significance (p, 0.14). MCS worsened in four cases with hypoparathyroidism (80%), but in only one euparathyroid (7%) (p, 0.0061). The worst decrease of both PCS and MCS occurred in two patients submitted to pancreatectomy during the follow-up after parathyroidectomy (PCS decreased 79% in one case and 53% in the other; MCS decreased 75% and 37%, respectively). Conclusion: Hypoparathyroidism worsens the HR-QoL after PTx in HPT/MEN1, particularly in patients undergoing subsequent pancreatectomy.
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