RESUMO
The widespread introduction of genetic testing in recent years has made it possible to determine that more than a third of cases of pheochromocytomas and paragangliomas (PPPGs) are caused by germline mutations. Despite the variety of catecholamine-producing tumors manifestations, there is a sufficient number of clinical and laboratory landmarks that suggest a hereditary genesis of the disease and even a specific syndrome. These include a family history, age of patient, presence of concomitant conditions, and symptoms of the disease. Considering that each of the mutations is associated with certain diseases that often determine tactics of treatment and examination of a patient, e.g. high risk of various malignancies. Awareness of the practitioner on the peculiarities of the course of family forms of PPPGs will allow improving the tactics of managing these patients.The article provides up-to-date information on the prevalence of hereditary PPPGs. The modern views on the pathogenesis of the disease induced by different mutations are presented. The main hereditary syndromes associated with PPPGs are described, including multiple endocrine neoplasia syndrome type 2A and 2B, type 1 neurofibromatosis, von Hippel-Lindau syndrome, hereditary paraganglioma syndrome, as well as clinical and laboratory features of the tumor in these conditions. The main positions on the necessity of genetic screening in patients with PPPGs are given.
Assuntos
Neoplasias das Glândulas Suprarrenais , Neoplasia Endócrina Múltipla Tipo 2a , Paraganglioma , Feocromocitoma , Doença de von Hippel-Lindau , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/genética , Humanos , Neoplasia Endócrina Múltipla Tipo 2a/diagnóstico , Neoplasia Endócrina Múltipla Tipo 2a/genética , Paraganglioma/diagnóstico , Paraganglioma/genética , Feocromocitoma/genética , Doença de von Hippel-Lindau/diagnóstico , Doença de von Hippel-Lindau/genéticaRESUMO
Xenon is known as an anesthetic in many ways closer to the ideal. In recent years, despite a number of objective reasons limiting its widespread use, the search continues for understanding how xenon influences on central hemodynamic parameters and regional blood flow. Analysis of several dozen of modern articles revealed many differences between the earlier and present data, emphasizing the prospect offurther research. According to Russian authors Xe has a positive inotropic effect on the myocardium and increases cardiac output. Foreign researchers in animal experiments notice vasoconstrictor properties of Xe with increased vascular resistance in the systemic and pulmonary circulation. The place of Xe as an anesthetic in the anesthetist's arsenal yet to be seen.
