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Robert E. Regel was the first to comprehensively analyze the diversity of cultivated barley in Russia and identify more than fifty new subvarieties. Some of them he published in two articles: «Barleys with smooth awns» (1908) and «The most important varieties and races of barleys in Russia» (1910). The present study was carried out because no one has yet conducted a search for authentic herbarium specimens and lectotypified the Hordeum taxa described by R.E. Regel. We have found that the main collection of the Herbarium of cultivated plants of the world, their wild relatives and weeds (WIR) contains the authentic herbarium specimens of nine barley taxa described by R.E. Regel, namely Hordeum distichon var. erectum subvar. anglicum R. Regel, H. distichon var. nigricans subvar. vilmorenianum R. Regel, H. distichon var. nutans subvar. europeicum R. Regel, H. distichon var. nutans subvar. turkestanicum R. Regel, H. distichon var. nutans subvar. сolchicum R. Regel, H. vulgare var. nigrum subvar. taganrocense R. Regel, H. vulgare var. pallidum. subvar. Lapponicum R. Regel, H. vulgare var. pallidum subvar. praecox R. Regel, and H. vulgare var. ricotense subvar. kiarchanum R. Regel. Since the available herbarium sheets contain later reproductions of the collection specimens cited in the protolog, these herbarium specimens were chosen by us as neotypes.

Introduction. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal disease of the blood system characterized by intravascular hemolysis, bone marrow dysfunction and an increased risk of thrombotic and organ complications.Aim — to provide relevant clinical recommendations for the provision of medical care to adults and children with PNH.Basic information. Experts from the National Hematological Society association which is focused on the promotion of hematology, transfusiology and bone marrow transplantation along with experts from the public organization, National Society of Pediatric Hematologists and Oncologists, have developed current clinical recommendations for providing medical care to adults and children with PNH. The recommendations address in detail the issues of etiology, pathogenesis, epidemiology, and clinical manifestations of the disease. Special attention is paid to the diagnosis, differential diagnosis, and treatment of PNH based on the principles of evidence.

This review surveys the current state of taxonomy, origin, and utilization prospects for naked barley. The cultivated barley Hordeum vulgare L. incorporates the covered and naked barley groups. Naked barleys are divided into six-row naked barley (convar. сoeleste (L.) A. Trof.) and two-row naked barley (convar. nudum (L.) A. Trof.). The groups include botanical varieties differing in the structural features of spikes, awns, floret and spikelet glumes, and the color of kernels. The centers of morphogenesis for naked barley are scrutinized employing archeological and paleoethnobotanical data, and the diversity of its forms. Hypotheses on the centers of its origin are discussed using DNA marker data. The main areas of its cultivation are shown, along with possible reasons for such a predominating or exclusive distribution of naked barley in highland areas. Inheritance of nakedness and mechanisms of its manifestation are considered in the context of new data in genetics. The biochemical composition of barley grain in protein, some essential and nonessential amino acids, β-glucans, vitamins, and antioxidants is described. Naked barley is shown to be a valuable source of unique combinations of soluble and insoluble dietary fibers and polysaccharides. The parameters limiting wider distribution of naked barley over the world are emphasized, and breeding efforts that could mitigate them are proposed. Pathogen-resistant naked barley accessions are identified to serve as promising sources for increasing grain yield and quality. Main stages and trends of naked barley breeding are considered and the importance of the VIR global germplasm collection as the richest repository of genetic material for the development of breeding is shown.В обзоре освещены вопросы современного состояния систематики, происхождения и перспектив использования голозерного ячменя. Представлены положения систематики культурного ячменя Hordeum vulgare L., в которой выделяются группы многорядного (сonvar. coeleste (L.) A. Trof.) и двурядного (сonvar. nudum (L.) A. Trof.) голозерного ячменя. Группы включают разновидности согласно особенностям строения колоса, остей, цветковых и колосковых чешуй и цвету зерновок. Отражены вопросы очагов формообразования голозерного ячменя, по данным археологии, палеоэтноботаники, разнообразию форм и сортов, а также рассмотрены вопросы происхождения голозерного ячменя по результатам ДНК-маркирования. Приведены основные зоны возделывания и возможные причины такого преимущественного или исключительного распространения голозерных ячменей в районах высокогорья. Обсуждается вопрос наследования признака голозерности ячменя и механизмов его проявления в соответствии с новыми данными в области генетики. Представлены характеристики биохимического состава зерна по белку, некоторым заменимым и незаменимым аминокислотам, β-глюканам, витаминам, антиоксидантам. Показано, что голозерный ячмень – ценный источник уникальных комбинаций растворимых и нерастворимых пищевых волокон и полисахаридов. Подчеркнуто значение параметров голозерного ячменя, которые ограничивают широкое распространение этой культуры в мире, и выделены направления селекционной работы для устранения этих недостатков. Приведены примеры образцов голозерного ячменя, устойчивых к вредным организмам, которые являются перспективными источниками для повышения урожайности и качества зерна, а также сохранения экологической чистоты и безопасности продукции. Отражены этапы и направления селекционных работ с голозерным ячменем и показано значение мировой коллекции ВИР как основного источника генетического материала для развития селекции.

Significant expansion of telemedicine technologies was made possible by the adoption of the necessary legal regulation and initiation of the national program “Healthcare”. National research centers were assigned a mission to provide advisory and methodological support to the regional hospitals. The manuscript describes the experience of the National Research Center for Hematology in application of telemedicine technologies in order to improve the quality of specialized medical care. Progressive increase in the number of requests for telemedicine consultations was observed during the last 5 years, also due to the activities aimed at expansion of geographical coverage of telemedicine technologies and involvement of the regional doctors. In 2019 1380 requests were received from 80 regions of the Russian Federation. The largest number of requests came from the hospitals of the Central (28%) and Siberian (25%) Federal Districts. Distribution of consultations by aim, disease, regions of origin is presented in the manuscript. Telemedicine consultations significantly contribute to the implementation of precise diagnostics and monitoring of patients with blood disorders, shortening of the time of diagnosis, timely treatment initiation, help to organize correct patient referrals, ultimately reducing the risks of treatment failure, complications and lethal outcomes. Analysis of the data accumulated in the consulting National research center allows to assess the quality and effectiveness of medical care in the regional hospitals.

Gaucher disease (GD) is an autosomal recessive Lysosomal storage disease caused by a deficiency of the enzyme glucocerebrosidase that is involved in glycosphingolipid degradation. The involvement of the osteoarticular system is one of the main manifestations of the disease and occurs in 70-100% of patients. However, the rarity of GD, nonspecific and heterogeneous clinical manifestations, and radiation semiotics similar to that of a number of other diseases may make differential diagnosis difficult. Objective : to present the radiation semiotics of involvement of the osteoarticular system in GD type I. To determine the role of MRI in diagnosing and estimating the severity of osteoarticular involvement in patients with GD type I. Material and methods . The data of X-ray and magnetic resonance images of the femurs along with the hip and knee joints were retrospectively analyzed in 86 patients diagnosed with GD type I. Studies were performed prior to specific therapy. The specific involvement of bone marrow in the pathological process was evaluated. Changes in the bones and joints, paraarticular structures, and soft tissues were determined. Results. The patients with GD type I were shown to have a spectrum of osteoarticular changes. It was ascertained that bone involvement in GD might be reversible and irreversible. MRI is a sensitive technique for diagnosing reversible changes in the osseous system in GD. Conclusion. The radiation semiotics of osteoarticular involvement in patients with GD type I is quite typical, and at the same time extremely variable. MRI is the gold standard method for the current diagnosis of osseous involvement in GD.

Differential diagnosis of bone involvement in patients with Gaucher disease can be challenging. Other diseases with similar radiological signs should be ruled out. Here we present a clinical case of tuberculous sacroiliitis in the patient with type I Gaucher disease. Advanced radiological methods of examination are described. Our case report proves the necessity of an individual approach to the management of such cohort of patients. Keywords: Gaucher disease, tuberculosis of bones and joints, differential diagnosis, comprehensive treatment.

Gaucher disease (GD) is an inherited enzymatic defect resulting from a deficiency of acid [3-glucosidase, a lysosomal enzyme involved in the degradation of cell metabolic products. The major clinical manifestations of GD are hepatosplenomegaly, cytopenia, and bony involvement varying from asymptomatic osteopenia to severest osteoporosis and ischemic necrosis to develop irreversible orthopedic defects. Timely enzyme replacement therapy with recombinant glucosidase makes it possible to arrest disease progression and to prevent damage to the vital organs. However, GD in adult patients is frequently diagnosed in the presence of occurring osteoarticular lesions (arthrosis deformans, abnormal fractures). In these instances, besides enzyme replacement therapy, high-quality orthopedic care is required. The description of the case history of a patient undergoing splenectomy in childhood is given as a clinical example of severe osteoarticular lesion in GD and complex differential diagnosis with the intercurrent disease extrapulmonary tuberculosis.