[The place of scales and questionnaires in assessing the disease's severity and the long-term prophylaxis's prescribing in patients with hereditary angioedema].

Hereditary angioedema is a hereditary life-threatening disease characterized by recurrent angioedema of various strength and localization. To date, definite diseases severity criteria have not been developed. There are many different factors to consider not only the frequency of episodes, but also their duration, strength, influence on the patient's appearance, the severity of the pain syndrome. Disease related quality of life, the number of work disability days, and the patient's satisfaction with the prescribed treatment are important. In addition, there are no criteria for prescribing long-term prophylaxis, and no parameters for choosing a specific drug. The introduction of specific scales and questionnaires developed to assess such characteristics as disease activity, disease control, quality of life in patients with recurrent angioedema into clinical practice seems to be the best solution for both questions. The comprehensive understanding of the diseases severity in a particular patient can be carried out with the combined use of assessment tools.

[Pulmonary manifestations in adult patients with a defect in humoral immunity]. / Pul'monologicheskie proyavleniya u vzroslykh patsientov s defektom gumoral'nogo zvena immuniteta.

Primary immunodeficiencies (PIDs) are a group of congenital diseases of the immune system, which numbers more than 230 nosological entities associated with lost, decreased, or wrong function of its one or several components. Due to the common misconception that these are extremely rare diseases that occur only in children and lead to their death at an early age, PIDs are frequently ruled out by physicians of related specialties from the range of differential diagnosis. The most common forms of PIDs, such as humoral immunity defects, common variable immune deficiency, X-linked agammaglobulinemia, selective IgA deficiency, etc., are milder than other forms of PID, enabling patients to attain their adult age, and may even manifest in adulthood. Bronchopulmonary involvements are the most common manifestations of the disease in patients with a defect in humoral immunity. Thus, a therapist and a pulmonologist are mostly the first doctors who begin to treat these patients and play a key role in their fate, since only timely diagnosis and initiation of adequate therapy can preserve not only the patient's life, but also its quality, avoiding irreversible complications. Chest computed tomography changes play a large role in diagnosis. These are not specific for PID; however, there are a number of characteristic signs that permit this diagnosis to be presumed.

[A place of intravenous immunoglobulins in current clinical practice: Privigen is a novel 10% immunoglobulin]. / Mesto immunoglobulinov dlya vnutrivennogo vvedeniya v sovremennoi klinicheskoi praktike: Prividzhen - novyi 10% immunoglobulin.

Intravenous immunoglobulins (IVIGs) were initially designed to treat patients with primary immunodeficiencies (PID). Due to the multidirectional effect of IVIGs on the immune system, a range of nosological entities, in which these agents are successfully administered, is steadily expanding. As of now, IVIGs are successfully used in neurology, rheumatology, hematology, and oncology and they are essential drugs for many patients. In spite of the long experience with IVIGs, their mechanism of action remains unclear, numerous investigations for their clinical introduction are being continued. Therefore, there is a growing need to increase the production of the drugs, which gives rise to the emergence of novel medications, which differ in their composition and manufacture technologies, on the pharmacological market. The 10% intravenous immunoglobulin privigen, the safety and efficacy of which has been proven in foreign practice, is a novel drug on the Russian market.

Analysis of polymorphisms in T(H)2-associated genes in Russian patients with atopic bronchial asthma.

BACKGROUND: Bronchial asthma is a chronic respiratory disorder characterized by airway inflammation, airway hyperresponsiveness, and periodic reversible airway obstruction. Subtype 2 helperT cell (T(H)2) cytokines play an important role in the development of allergic airway inflammation in patients with bronchial asthma. OBJECTIVE: To investigate whether the single-nucleotide polymorphisms (SNPs) Ile75Val and Gln576Arg in the IL4RA gene, -33C>T in the IL4 gene, and Gly237Glu in the FCER1B gene contribute to the development and severity of atopic bronchial asthma in Russian patients from Moscow. METHODS: We analyzed DNA samples from 224 patients with atopic bronchial asthma and 172 healthy individuals. Genotyping was performed by primer extension followed by matrix-assisted laser desorption/ionization-time of flight mass spectrometry. RESULTS: We observed a moderate association between the Arg/Arg genotype of Gln576Arg and protection against asthma (odds ratio [OR], 0.16; P < .012) and a strong association between the T allele and TT genotype of -33C> and atopic bronchial asthma (OR, 1.91 and 4.65, respectively; P < .0001). Carriers of the C allele had a reduced risk of asthma (OR, 0.53; P < .0001). Furthermore, we found that the TT genotype of -33C>T correlated with higher concentrations of total serum immunoglobulin E and interleukin 4 than the CC and CT genotypes. CONCLUSION: We found an association between atopic bronchial asthma and the SNPs Gln576Arg in IL4RA and -33C>T in IL4. IL4RA and IL4 seem to be involved in the pathogenesis of asthma.

[M31R and R335C polymorphic variants of the IL8RA gene in Russian and Buryat patients with atopic bronchial asthma].

To test the M31R and R335C polymorphisms of the Il8RA gene for association with atopic bronchial asthma (BA), the allele and genotype frequency distributions of the polymorphisms were studied in Russian patients from Moscow and Buryat patients from Ulan-Ude. The study involved two Russian groups, one including 291 DNA samples of patients with atopic BA, and the other, 266 DNA samples of healthy people. The two Buryat groups included 124 and 152 DNA samples from patients with atopic BA and healthy people, respectively. The M31R polymorphism proved to be associated with atopic BA in Russians. Allele Arg and genotype Met/Arg suggested a higher risk of BA (OR= 4.45, P = 0.003 and OR = 4.58, P = 0.003, respectively), while allele Met and genotype Met/Metwere associated with a lower risk (OR = 0.22, P = 0.003 and OR = 0.22, P = 0.003, respectively). The R335C polymorphism was not associated with atopic BA in Russians and was in Buryats. Allele Arg and homozygous genotype Arg/Arg suggested a higher risk of the disease (OR = 3.06, P = 0.030 and OR = 3.20, P = 0.027, respectively), while allele Cys and genotype Arg/Cys suggested a lower risk (OR = 0.33, P = 0.030 and OR = 0.31, P = 0.027, respectively). The results support the role of the IL8RA gene in atopic BA.

[CARD15 and TLR4 genes polymorphisms in atopic bronchial asthma].

In order to investigate whether single nucleotide polymorphisms G(+2722)C and 3020insC in CARD15 gene and Asp299Gly in TLR4 gene contribute to atopic bronchial asthma we performed a comparative analysis of alleles and genotypes frequencies of these polymorphisms in Russian patients from Moscow. DNA samples from 283 patients with atopic bronchial asthma and 227 healthy donors were genotyped. There were associations neither of G(+2722)C and 3020insC in CARD15 gene and Asp299Gly in TLR4 gene with asthma nor of markers of CARD15 gene with asthma severity. Haplotype frequency analysis of CARD15 gene polymorphisms did not reveal significant difference between groups. However, a strong association was found between Asp299Gly and asthma severity. Allele Asp of this marker showed association with mild atopic bronchial asthma and allele Gl--with moderate/severe asthma = 0.47, 95% CI [0.24-0.93] i OR = 2.12, 95% CI [1.08-4.18] respectively).

[The actual approaches to the economic analysis of effectiveness of functioning of multi-profile curative preventive medical institution].

The article deals with the technique of economic analysis of effectiveness of functioning of multi-profile curative preventive medical institution in the conditions of transition to the payment according the completed case of treatment. The necessity of the measures targeted to prevent the financial losses under the new form of payment for hospital care is proved.

[Biochemical characteristics of strains from the Citrobacter and Hafnia genera and their laboratory identification]. / Nekotorye dannye izucheniia biokhimicheskoi kharakteristiki shtammov rodov Citrobacter i Hafnia i ikh identifikatsiia v laboratornoi pratike

A study of biochemical properties of 176 strains of coprocultures isolated from the patients and persons who sustained various acute enteric diseases, and also from contacts and persons examined for prophylaxis showed that they corresponded to the taxonomic definition of the Citrobacter and Hafnia genus. In connection with the similarity by biochemical properties between bacteria of Citrobacter and Salmonella genus and bacteria of Hafnia and Shigella genus it is of expedience in their identification and differentiation carried out in bacteriological laboratories in the diagnosis of enteric infections to use several biochemical tests.