Long-term prognosis of patients with pediatric pheochromocytoma

A third of patients with paraganglial tumors, pheochromocytoma, and paraganglioma, carry germline mutations in one of the susceptibility genes, RET, VHL, NF1, SDHAF2, SDHA, SDHB, SDHC, SDHD, TMEM127, and MAX. Despite increasing importance, data for long-term prognosis are scarce in pediatric presentations. The European-American-Pheochromocytoma-Paraganglioma-Registry, with a total of 2001 patients with confirmed paraganglial tumors, was the platform for this study. Molecular genetic and phenotypic classification and assessment of gene-specific long-term outcome with second and/or malignant paraganglial tumors and life expectancy were performed in patients diagnosed at <18 years. Of 177 eligible registrants, 80% had mutations, 49% VHL, 15% SDHB, 10% SDHD, 4%NF1, and one patient each in RET, SDHA, and SDHC. A second primary paraganglial tumor developed in 38% with increasing frequency over time, reaching 50% at 30 years after initial diagnosis. Their prevalence was associated with hereditary disease (P=0.001), particularly in VHL and SDHD mutation carriers (VHL vs others, P=0.001 and SDHD vs others, P=0.042). A total of 16 (9%) patients with hereditary disease had malignant tumors, ten at initial diagnosis and another six during follow-up. The highest prevalence was associated with SDHB (SDHB vs others, P<0.001). Eight patients died (5%), all of whom had germline mutations. Mean life expectancy was 62 years with hereditary disease. Hereditary disease and the underlying germline mutation define the long-term prognosis of pediatric patients in terms of prevalence and time of second primaries, malignant transformation, and survival. Based on these data, gene-adjusted, specific surveillance guidelines can help effective preventive medicine.publishersversionPeer reviewe

Экспериментальное исследование износа бандажей электровозов ВЛ11м с различными профилями

Експериментальне дослідження зносу бандажів електровозів ВЛ11М з різними профілями / М. Л. Коротенко, О. М. Заболотний, Л. О. Недужа, Н. М. Нестеренко, Д. О. Ягода // Вісн. Дніпропетр. нац. ун-ту залізн. трансп. ім. акад. В. Лазаряна. — Дніпропетровськ, 2005. — Вип. 8. — С. 53—57.UA: Проведені експериментальні дослідження зносу бандажів електровоза в гірських умовах Львівської залізниці. Розглядались періоди експлуатації електровоза з різними профілями коліс.RU: Проведены экспериментальные исследования износа бандажей электровоза в горных условиях Львовской дороги. Рассматривались периоды эксплуатации электровоза с различными профилями колес.EN: The experimental research of the bandage wear has fun conducted concerning electric locomotive performance under the mountain conditions of the Lvov railway. The operation periods of an electric locomotive have been considered as for the various wheel profile structures

Показатели динамики электровоза ЧС4 и прочности несущих конструкций рам тележек производства ХК «Лугансктепловоз»

Показники динаміки електровоза ЧС4 та міцності несучих конструкцій рам візків виробництва ХК "Луганськтепловоз" / І. Є. Батюшин, О. М. Бондарєв, В. Л. Горобець, О. М. Заболотний, Д. О. Ягода // Вісн. Дніпропетр. нац. ун-ту залізн. трансп. ім. акад. В. Лазаряна. — Дніпропетровськ, 2007. — Вип. 19. — С. 152—160.UA: В статті наведено результати динамічних ходових міцносних випробувань електровозу ЧС4 з візками виробництва ХК «Луганськтепловоз». На підставі отриманих результатів випробувань зроблено висновки про відповідність електровоза нормативним показникам при його експлуатації у складі пасажирських поїздів зі швидкостями до 140 км/год. включно.RU: В статье приведены результаты динамических ходовых прочностных испытаний электровоза ЧС-4 с рамами тележек производства ХК «Лугансктепловоз». Полученные результаты испытаний показали, что модернизированный электровоз соответствует Нормативным показателям.EN: Article is devoted to the results of dynamic tests, tests on the line and tests for durability of electric locomotive СS4 with bogies frames produced by HK «Luganskteplovoz». The received results of tests have shown, that the modernized electric locomotive corresponds to Normative parameters.Укрзалізниц

Показатели динамики и тягово-энергетические показатели электровоза ВЛ40У

Показники динаміки та тягово-енергетичні показники електровоза ВЛ40У / С. В. Пилипенко, О. М. Бондарєв, В. Л. Горобець, О. І. Гілевич и [и др.] // Вісн. Днiпропетр. нац. ун-ту залізн. трансп. iм. акад. В. Лазаряна. Дніпропетровськ, 2008. — Вип. 21. — С. 16—25.UK: В статті наведено результати експериментального визначення показників динаміки та тягово-енергетичних показників електровоза ВЛ40у. Встановлена придатність щодо експлуатації вказаних електровозів в пасажирському русі з пасажирськими поїздами до 15 вагонів включно.RU: В статье приведены результаты экспериментально установленных показателей динамики и тягово-энергетических показателей електровоза ВЛ40у. На основании полученных результатов определена пригодность электровозов подобного типа для эксплуатации в пассажирском движении с поездами до 15 пассажирских вагонов включительно.EN: In the article the results of dynamic running and traction-energy tests of the electric locomotive VL40U are presented. In accordance with the test results a conclusion about the suitability of electric locomotive of such a type for operation with trains containing up to 15 passenger coaches inclusive is made.Укрзалізниц

Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2:an international retrospective population-based study

Background The prevention of medullary thyroid cancer in patients with multiple endocrine neoplasia type 2 syndrome has demonstrated the ability of molecular diagnosis and prophylactic surgery to improve patient outcomes. However, the other major neoplasia associated with multiple endocrine neoplasia type 2, phaeochromocytoma, is not as well characterised in terms of occurrence and treatment outcomes. In this study, we aimed to systematically characterise the outcomes of management of phaeochromocytoma associated with multiple endocrine neoplasia type 2.Methods This multinational observational retrospective population-based study compiled data on patients with multiple endocrine neoplasia type 2 from 30 academic medical centres across Europe, the Americas, and Asia. Patients were included if they were carriers of germline pathogenic mutations of the RET gene, or were first-degree relatives with histologically proven medullary thyroid cancer and phaeochromocytoma. We gathered clinical information about patients' RET genotype, type of treatment for phaeochromocytoma (ie, unilateral or bilateral operations as adrenalectomy or adrenal-sparing surgery, and as open or endoscopic operations), and postoperative outcomes (adrenal function, malignancy, and death). The type of surgery was decided by each investigator and the timing of surgery was patient driven. The primary aim of our analysis was to compare disease-free survival after either adrenal-sparing surgery or adrenalectomy.Findings 1210 patients with multiple endocrine neoplasia type 2 were included in our database, 563 of whom had phaeochromocytoma. Treatment was adrenalectomy in 438 (79%) of 552 operated patients, and adrenal-sparing surgery in 114 (21%). Phaeochromocytoma recurrence occurred in four (3%) of 153 of the operated glands after adrenal-sparing surgery after 6-13 years, compared with 11 (2%) of 717 glands operated by adrenalectomy (p=0.57). Postoperative adrenal insufficiency or steroid dependency developed in 292 (86%) of 339 patients with bilateral phaeochromocytoma who underwent surgery. However, 47 (57%) of 82 patients with bilateral phaeochromocytoma who underwent adrenal-sparing surgery did not become steroid dependent.Interpretation The treatment of multiple endocrine neoplasia type 2-related phaeochromocytoma continues to rely on adrenalectomies with their associated Addisonian-like complications and consequent lifelong dependency on steroids. Adrenal-sparing surgery, a highly successful treatment option in experienced centres, should be the surgical approach of choice to reduce these complications.</p

Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based study

Background The prevention of medullary thyroid cancer in patients with multiple endocrine neoplasia type 2 syndrome has demonstrated the ability of molecular diagnosis and prophylactic surgery to improve patient outcomes. However, the other major neoplasia associated with multiple endocrine neoplasia type 2, phaeochromocytoma, is not as well characterised in terms of occurrence and treatment outcomes. In this study, we aimed to systematically characterise the outcomes of management of phaeochromocytoma associated with multiple endocrine neoplasia type 2. Methods This multinational observational retrospective population-based study compiled data on patients with multiple endocrine neoplasia type 2 from 30 academic medical centres across Europe, the Americas, and Asia. Patients were included if they were carriers of germline pathogenic mutations of the RET gene, or were first-degree relatives with histologically proven medullary thyroid cancer and phaeochromocytoma. We gathered clinical information about patients’ RET genotype, type of treatment for phaeochromocytoma (ie, unilateral or bilateral operations as adrenalectomy or adrenal-sparing surgery, and as open or endoscopic operations), and postoperative outcomes (adrenal function, malignancy, and death). The type of surgery was decided by each investigator and the timing of surgery was patient driven. The primary aim of our analysis was to compare disease-free survival after either adrenal-sparing surgery or adrenalectomy. Findings 1210 patients with multiple endocrine neoplasia type 2 were included in our database, 563 of whom had phaeochromocytoma. Treatment was adrenalectomy in 438 (79%) of 552 operated patients, and adrenal-sparing surgery in 114 (21%). Phaeochromocytoma recurrence occurred in four (3%) of 153 of the operated glands after adrenal-sparing surgery after 6-13 years, compared with 11 (2%) of 717 glands operated by adrenalectomy (p=0.57). Postoperative adrenal insufficiency or steroid dependency developed in 292 (86%) of 339 patients with bilateral phaeochromocytoma who underwent surgery. However, 47 (57%) of 82 patients with bilateral phaeochromocytoma who underwent adrenal-sparing surgery did not become steroid dependent. Interpretation The treatment of multiple endocrine neoplasia type 2-related phaeochromocytoma continues to rely on adrenalectomies with their associated Addisonian-like complications and consequent lifelong dependency on steroids. Adrenal-sparing surgery, a highly successful treatment option in experienced centres, should be the surgical approach of choice to reduce these complications