{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T13:46:17Z","timestamp":1791812777762,"version":"4.3.4"},"reference-count":18,"publisher":"The Endocrine Society","issue":"3","license":[{"start":{"date-parts":[[2020,1,8]],"date-time":"2020-01-08T00:00:00Z","timestamp":1578441600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,3,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Introduction<\/jats:title>\n                  <jats:p>Carney Complex (CNC) is a rare multiple endocrine and nonendocrine neoplasia syndrome. Manifestations and genotype-phenotype correlations have been described by retrospective studies, but no prospective study evaluating the occurrence of the different manifestations has been available so far.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>This multicenter national prospective study included patients with CNC, primary pigmented nodular adrenal disease (PPNAD), or a pathogenic PRKAR1A mutation; after a full initial workup, participants were followed for 3 years with annual standardized evaluation.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>The cohort included 70 patients (50 female\/20 male, mean age 35.4 \u00b1 16.7 years, 81% carrying PRKAR1A mutation). The initial investigations allowed identification of several manifestations. At the end of the 3-year follow-up, the newly diagnosed manifestations of the disease were subclinical acromegaly in 6 patients, bilateral testicular calcifications in 1 patient, and cardiac myxomas in 2 patients. Recurrences of cardiac myxomas were diagnosed in 4 patients during the 3-year follow-up study period. Asymptomatic abnormalities of the corticotroph and somatotroph axis that did not meet criteria of PPNAD and acromegaly were observed in 11.4% and 30% of the patients, respectively. Patients carrying the PRKAR1A c.709-7del6 mutation had a mild phenotype.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>This study underlines the importance of a systematic follow-up of the CNC manifestations, especially a biannual screening for cardiac myxoma. By contrast, regular screening for the other manifestations after a first extensive workup could be spread out, leading to a lighter and more acceptable follow-up schedule for patients. These are important results for recommendations for long-term management of CNC patients.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/clinem\/dgaa002","type":"journal-article","created":{"date-parts":[[2020,1,8]],"date-time":"2020-01-08T08:14:02Z","timestamp":1578471242000},"page":"e436-e446","source":"Crossref","is-referenced-by-count":60,"title":["Frequency and Incidence of Carney Complex Manifestations: A Prospective Multicenter Study With a Three-Year Follow-Up"],"prefix":"10.1210","volume":"105","author":[{"given":"St\u00e9phanie","family":"Espiard","sequence":"first","affiliation":[{"name":"INSERM U1016, CNRS UMR8104, Institut Cochin, Universit\u00e9 Paris Descartes, Paris"},{"name":"Service d\u2019Endocrinologie, Centre de r\u00e9f\u00e9rence des maladies rares de la surr\u00e9nale, Assistance Publique H\u00f4pitaux de Paris, H\u00f4pital Cochin, Paris, France"},{"name":"Service d\u2019endocrinologie, diab\u00e9tologie, m\u00e9tabolisme et nutrition, CHR-U de Lille, H\u00f4pital Huriez, Lille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-Christine","family":"Vantyghem","sequence":"additional","affiliation":[{"name":"Service d\u2019endocrinologie, diab\u00e9tologie, m\u00e9tabolisme et nutrition, CHR-U de Lille, H\u00f4pital Huriez, Lille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Assi\u00e9","sequence":"additional","affiliation":[{"name":"INSERM U1016, CNRS UMR8104, Institut Cochin, Universit\u00e9 Paris Descartes, Paris"},{"name":"Service d\u2019Endocrinologie, Centre de r\u00e9f\u00e9rence des maladies rares de la surr\u00e9nale, Assistance Publique H\u00f4pitaux de Paris, H\u00f4pital Cochin, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Cardot-Bauters","sequence":"additional","affiliation":[{"name":"Service d\u2019endocrinologie, diab\u00e9tologie, m\u00e9tabolisme et nutrition, CHR-U de Lille, H\u00f4pital 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