caa a22 4500 477035329 CHVBK 20180405111303.0 cr unu---uuuuu 170330e19960701xx s 000 0 eng 10.1007/s002689900123 doi (NATIONALLICENCE)springer-10.1007/s002689900123 Primary Hyperparathyroidism in Multiple Endocrine Neoplasia Type IIa: Retrospective French Multicentric Study [Elektronische Daten] [Jean-Louis Kraimps, Anne Denizot, Bruno Carnaille, Jean-François Henry, Charles Proye, François Bacourt, Emile Sarfati, Jean-Louis Dupond, Brigitte Maes, Jean-Paul Travagli, Andrée Boneu, Patrick Roger, Chantal Houdent, Jacques Barbier, Elisabeth Modigliani] Abstract. Primary hyperparathyroidism (PHPT) in multiple endocrine neoplasia (MEN) type IIa is rare, occurring in 20% to 30% of the patients. The aim of this study was to evaluate clinical findings, surgical therapy, and outcome for 56 patients affected by PHPT among 249 MEN-IIa patients collected from 84 families assembled by the Groupe d'Etude des Tumeurs á Calcitonine (GETC, French Calcitonin Tumors Study Group). This retrospective study was based on cases registered by the GETC (20 participating centers) from 1969 to 1994. Characteristics of PHPT in 56 patients (31 women, 25 men) with MEN-IIa were reviewed. All but two underwent cervicotomy. The median age at diagnosis was 37.6 years. PHPT was found concomitantly with medullary thyroid carcinoma (MTC) or pheochromocytoma in 43 patients (77%). PHPT was asymptomatic in 68% of the patients. Serum calcium levels ranged from 2.20 to 3.70 mmol/L (median 2.82 mmol/L; normal 2.10-2.60 mmol/L). The number of parathyroid glands removed at surgery was 0 (: n = 2), 1 ( n = 24), 2 ( n = 5), > 2 ( n = 12), 4 ( n = 11). Pathology (initial surgery) consisted of 24 adenomas, 4 double adenomas, and 25 hyperplasia. Cure after initial surgery was obtained in 89%, including a 22% incidence of hypoparathyroidism. There were 6 cases (11%) with persistent PHPT. With a mean follow-up of 6.4 years, five patients (9%) had recurrent PHPT. The results indicate that MEN-IIa-related PHPT is generally associated with mild, often asymptomatic hypercalcemia. Despite recurrences encountered 5 to 15 years after the first cervicotomy, resection of only macroscopically enlarged glands generally appears sufficient. Subtotal or total parathyroidectomy with autotransplantation is associated with a high rate of hypoparathyroidism. 1996 by the Société Internationale de Chir, ugie Kraimps Jean-Louis GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Denizot Anne GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Carnaille Bruno GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Henry Jean-François GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Proye Charles GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Bacourt François GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Sarfati Emile GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Dupond Jean-Louis GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Maes Brigitte GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Travagli Jean-Paul GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Boneu Andrée GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Roger Patrick GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Houdent Chantal GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Barbier Jacques GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Modigliani Elisabeth GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut https://doi.org/10.1007/s002689900123 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s002689900123 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kraimps Jean-Louis GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Denizot Anne GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Carnaille Bruno GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Henry Jean-François GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Proye Charles GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Bacourt François GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Sarfati Emile GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Dupond Jean-Louis GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Maes Brigitte GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Travagli Jean-Paul GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Boneu Andrée GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Roger Patrick GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Houdent Chantal GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Barbier Jacques GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut NATIONALLICENCE 700 1- Modigliani Elisabeth GETC, Department of Endocrinology, Avicenne Hospital, 125 Route de Stalingrad, 93009 Bobigny, France, FR aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer