No abstract

Hereditary deficiency of the alpha-1-antitrypsin occupies a leading position among the causes of chronic nonspecific lung diseases with emphysema formation. Deficiency of alpha-1-antitrypsin is a potentially fatal hereditary disease, under-diagnosed by physicians of various specialities. The authors familiarize pediatricians with this hereditary disease, difficulties of its diagnosis and treatment. There are presented own author`s data on the diagnosis and the experience of the enzyme replacement therapy of alpha-1-antitrypsin deficiency with the drug INN «Alpha-1 antitrypsin human» (Respikam).

The gold standard for diagnosing injuries in polytrauma is computed tomography (CT) of the whole body. This method of radiation diagnostics allows to quickly and non-invasively obtain high-resolution images for an accurate assessment of all trauma-related injuries. To date, there are different opinions on the optimal algorithm for conducting and evaluating CT in patients with polytrauma, but a unified standard of research methods for this category of patients has not been developed. This article presents the experience of various domestic and foreign hospitals providing medical care to patients with various types of combined and multiple injuries, as well as describes methodological recommendations for performing and analyzing CT of the whole body in patients with polytrauma, obtained on the basis of our own practical experience. In the article, we also reflected the peculiarities of the interpretation of the resulting traumatic injuries in the native, arterial, venous and delayed phases of scanning; clinical examples of CT studies in patients with polytrauma are given. We noted the particular importance of postprocessing processing of images obtained during CT examination. Here, we recommend performing CT of the whole body in polytrauma with the use of multiphase intravenous bolus contrasting, which allows a more detailed assessment of the changes in internal organs and soft tissues and diagnoses the presence and nature of bleeding. Comprehensive and detailed evaluation of CT images obtained in patients with polytrauma is aimed at helping clinical specialists choose the best treatment method, prioritizing and predicting patient management in each case.