Фторидная технология получения титановых порошков

Обоснована необходимость разработки новой технологии переработки титансодержащих концентратов. Выполнено исследование процесса фторирования рутилового концентрата элементным фтором. Описаны процессы, протекающие при электролитическом получении титановых порошков во фторидных расплавах TiF4. Для отмывки от примесей катодного осадка предложено две технологии – в смеси неорганических кислот и безводным HF; показаны преимущества отмывки HF; содержание примесей в порошке титана, полученном отмывкой в безводном HF несколько ниже, чем при "кислотной" отмывке. При промышленном осуществлении процесса HF рециркулирует в схеме, обеспечивая экологическую безопасность и низкую себестоимость Ti.The brief review of industrial technologies of processing the titanium containing concentrates has been carried out. The research of the fluorination process of rutile concentrates with element fluorine was carried out. The processes occurring during the electrolysis of titanic powders from fluoride fusions, from TiF4, are described. In order to wash off impurities of cathode deposit, two techniques were proposed - in a mixture of inorganic acids and anhydrous HF; advantages of washing off with HF are shown; the impurity content in the titanium powder obtained by washing off in anhydrous HF is slightly lower than at "acid" washing off. In the industrial process implementation HF recycles tin a circuit that ensures environmental safety of production and low production cost of obtained Ti

Внедрение организационных инноваций в обустройстве инфраструктуры нефтегазовой отрасли

Инфраструктура занимает одну из важнейших должностей в организации, при грамотной инфраструктуре: организация несёт значительно меньше производственных потерь. Мы рассмотрим возможность внедрения инноваций в организационную деятельность в обустройстве инфраструктуры нефтегазовой отрасли.Infrastructure occupies one of the most important positions in the organization, with a competent infrastructure: the organization suffers significantly less production losses. We will consider the possibility of introducing innovations in organizational activities in arranging the infrastructure of the oil and gas industry

Genetic landscape of pediatric acute liver failure of indeterminate origin.

BACKGROUND AIMS Pediatric acute liver failure (PALF) is a life-threatening condition. In Europe, main causes are viral infections (12-16%) and inherited metabolic diseases (14-28%). Yet, in up to 50% of cases the underlying etiology remains elusive, challenging clinical management, including liver transplantation. We systematically studied indeterminate PALF cases referred for genetic evaluation by whole-exome sequencing (WES), and analyzed phenotypic and biochemical markers, and the diagnostic yield of WES in this condition. METHODS With this international, multicenter observational study, patients (0-18 y) with indeterminate PALF were analyzed by WES. Data on the clinical and biochemical phenotype were retrieved and systematically analyzed. RESULTS In total, 260 indeterminate PALF patients from 19 countries were recruited between 2011 and 2022, of whom 59 had recurrent PALF (RALF). WES established a genetic diagnosis in 37% of cases (97/260). Diagnostic yield was highest in children with PALF in the first year of life (46%), and in children with RALF (64%). Thirty-six distinct disease genes were identified. Defects in NBAS (n=20), MPV17 (n=8) and DGUOK (n=7) were the most frequent findings. When categorizing, most frequent were mitochondrial diseases (45%), disorders of vesicular trafficking (28%) and cytosolic aminoacyl-tRNA synthetase deficiencies (10%). One-third of patients had a fatal outcome. Fifty-six patients received liver transplants. CONCLUSION This study elucidates a large contribution of genetic causes in PALF of indeterminate origin with an increasing spectrum of disease entities. The high proportion of diagnosed cases and potential treatment implications argue for exome or in future rapid genome sequencing in PALF diagnostics

Untersuchung zur Lagerungsfähigkeit von nasalen Schleimhautbiopsien zur Zilienfunktionsdiagnostik

Die Zilienfunktionsdiagnostik aus respiratorischen Epithelzellen zur Bestätigung oder zum Ausschluss einer primären Ziliendyskinesie ist auf wenige Zentren beschränkt. In dieser Studie wurde untersucht, wie sich ein Abstand von 24h zwischen Probenentnahme und Untersuchung auf die Probenqualität auswirkt und ob ein Probenversand in diesem Zeitraum möglich ist. Die Zilienschlagfrequenz nasal gewonnener Flimmerepithelzellen wurde photometrisch direkt und 24h nach Entnahme bestimmt. In 3 Versuchsanordnungen wurde untersucht, ob sich die Probenqualität im Vergleich zur Lagerung der Zellen im einfachen Medium durch den Zusatz von Penicillin/Streptomycin oder durch die Kühlung der Proben auf +4°C während der 24h verbessern lässt. Ein zeitl. Abstand von 24h reduziert den Anteil auswertbarer Proben auf 75% der initial verwertbaren Proben, hat aber bei nachweisbarer Flimmeraktivität keine Fehlbeurteilung des Patienten zur Folge, wenn die Probe mit Antibiotikazusatz versehen oder gekühlt wurde