1,721,078 research outputs found
(Editor) Abstracts delle comunicazioni al XXII Congresso Nazionale della Società Italiana di Fisiopatologia Chirurgica (SIFIPAC).
Clinical Features, Treatment, and Surveillance of Hyperparathyroidism-Jaw Tumor Syndrome: An Up-to-Date and Review of the Literature
Hyperparathyroidism-jaw tumor (HPT-JT) syndrome is an autosomal dominant disorder characterized by parathyroid tumors in association with fibro-osseous jaw tumors and uterine and renal lesions. HPT-JT syndrome is caused by germline mutations of the cell division cycle 73 (CDC73) gene that encodes the parafibromin, a 531-amino acid protein with antiproliferative activity. Primary hyperparathyroidism is the main finding of HPT-JT syndrome, usually caused by a single-gland parathyroid involvement (80% of cases), at variance with other variants of hereditary hyperparathyroidism, in which a multiglandular involvement is more frequent. Moreover, parathyroid carcinoma may occur in approximately 20% of cases. Surgery is the treatment of choice for primary hyperparathyroidism, but the extent of surgery remains controversial, varying between bilateral neck and focused exploration, with subtotal or limited parathyroidectomy. Recently, more limited approaches and parathyroid excisions have been suggested in order to decrease the risk of permanent hypoparathyroidism, the main surgical morbidity following more extensive surgical approaches. Ossifying fibromas of the mandible or maxilla may present only in a minority of cases and, even if benign, they should be surgically treated to avoid tumor growth and subsequent functional limitations. Benign and malignant uterine involvement (including leiomyomas, endometrial hyperplasia, adenomyosis, multiple adenomyomatous polyps, and adenosarcomas) is the second most common clinical feature of the syndrome, affecting more than 50% of CDC73-carrier women. Genetic testing should be performed in all family members of affected individuals, in young patients undergoing surgery for primary hyperparathyroidism, or in presence of other associated tumors, allowing early diagnosis and prompt treatment with more tailored surgery. Moreover, CDC73 mutation carriers should be also periodically screened for primary hyperparathyroidism and the other associated tumors. The present review was aimed to summarize the main clinical features of HPT-JT syndrome, focusing on genetic screening and surgical treatment, and to revise the available literature
Ringraziamento a FREGO MAURO in: IACOBONE M., BARZON L., PORZIONATO A., MASI G., MACCHI V., MARINO F, VIEL G., FAVIA G. Parafibromin expression, single-gland involvement, and limited parathyroidectomy in familial isolated hyperparathyroidism.
Parathyroid surgery: an evidence-based volume—outcomes analysis: European Society of Endocrine Surgeons (ESES) positional statement
Background: The interest in correlation between hospital and surgeon practice volume and postoperative outcomes has grown considerably over the last decades; it has been suggested that surgery is likely to be associated with higher cure rates, lower morbidity and more favourable results in cost-effectiveness when performed in a high-volume setting. The aim of this paper is to undertake an evidence-based literature review of the relationship between surgical volume and clinical outcomes in parathyroidectomy for primary hyperparathyroidism. We used accepted quality markers to identify the relationship between volume and outcome with a view to defining a reproducible minimal surgical volume-related standard of care in parathyroid surgery. Methods: A peer review literature analysis of volume and outcomes in parathyroid surgery was carried out and assessed from an evidence-based perspective. Results were discussed at the 2019 Conference of the European Society of Endocrine Surgeons devoted to “Volumes, Outcomes and Quality Standards in Endocrine Surgery”. Results: Literature reports no prospective randomised studies; thus, a low level of evidence may be achieved. Conclusions: Parathyroid surgery is at increased risk of failures, morbidity and need for reoperations and cost when performed in low-volume settings; thus, it should be concentrated in dedicated settings, with adequate annual volume and expertise. Acceptable results may be achieved moving parathyroid surgery cases away from low-volume settings ( 40 parathyroidectomies/year). © 2019, Springer-Verlag GmbH Germany, part of Springer Nature
Ruolo della surrenectomia monolaterale nella iperplasia nodulare surrenalica bilaterale ACTH indipendente (AIMAH)
Il carcinoma surrenalico con infiltrazione cavale: nostra esperienza e revisione della letteratura
AN INTERACTIVE TRAINING MODEL AND QUALITY ASSURANCE IN A SCHOOL OF OCCUPATIONAL MEDICINE
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