307 research outputs found

    Clinical Features, Treatment, and Surveillance of Hyperparathyroidism-Jaw Tumor Syndrome: An Up-to-Date and Review of the Literature

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    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

    The "false" nonrecurrent inferior laryngeal nerve

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    BACKGROUND: Communicating branches between the cervical sympathetic system and the inferior laryngeal nerve (ILN) have been described. They usually originate from the middle cervical sympathetic ganglion (MCSG). These branches (sympathetic-inferior laryngeal anastomotic branch [SILAB]), usually thin, sometimes have the same diameter as the ILN. In this study we prospectively evaluated the frequency of this condition and its implications during surgical neck exploration. METHODS: From November 1998 to October 1999, 791 patients underwent surgical neck exploration, and 1253 ILNs were dissected: 656 on the right side (52.3%) and 597 on the left side (47.7%). RESULTS: On the right side, a nonrecurrent ILN was found in 3 cases (0.46%), and a large SILAB was found in 10 cases (1.5%). The SILAB originated from the superior cervical sympathetic ganglion in 2 cases and directly from the sympathetic trunk above the MCSG in 8 cases. No anomalous branch was found on the left side. CONCLUSIONS: The SILAB may originate not only from the MCSG but also from the superior cervical sympathetic ganglion or directly from the sympathetic trunk. When the SILAB is as large as the ILN, it could be mistaken for a nonrecurrent ILN. The awareness of this anatomic condition during neck dissection may help to avoid injuries of the genuine ILN running in the usual pathway

    Parathyroid surgery: an evidence-based volume—outcomes analysis: European Society of Endocrine Surgeons (ESES) positional statement

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    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

    Christine de Pizan and Her Treatise on Fortifications (1410)

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    Christine de Pizan/ Cristina da Pizzano (1365–1430) is well known as poet and as author of The Book of the City of Ladies (1405), in which she describes female leaders in history. Instead it’s quite unknown her book Livre des fais d’armes et de chevalrie, written around 1410, translated in English in 1489 and also in 1932 (The Book of fayttes of armes and of chyualrye). Apart from editorial events, this Treatise is important especially because it’s the first to re-cognize the role of technology of medieval warfare and it’s an important source for gunpower weapon technology, as well as for strategy and tactics. Based on Vegetius’s theory on fortifications, she describes—in the second part—new forms and features of fortresses, also introducing parade grounds along the walls, which was an advanced system of defense for the XV century. She has been the first to talk about artillery and its importance to organize (also geometrically) fortresses in a Treatise, while other important authors, such as L. B. Alberti or Filarete, completely ignored this item in their books. The importance of the Book has been recognized by Italian military historians since the XIX century, for example Carlo Promis and Ignazio Calvi

    Optimum Planning of Electricity Production

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    The problem of planning the production of a pool of power plants has been deeply investigated. Maintenance management and load allocation problems have been assumed as crucial aspects for achieving maximum plant profitability. A production-planning approach has been developed, and genetic algorithm techniques have been adopted to implement the developed approach. Life consumption of gas turbines' hot-section components has been considered as a key element required in simulating plants' behaviors. As a result, a deterioration model has been developed and included into the planning algorithm. The developed approach takes market scenarios, as well as actual statuses and performances of plant components into account. The plants' physical models are developed on a modular approach basis and provide the operating parameters required by the planning algorithm. Neural network techniques have been applied to speed up the simulation. Economic implications related to maintenance strategies, including postponement or anticipation of maintenance interventions, are investigated and the results obtained by the numerical simulation are presented and widely discussed
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