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We present to your attention a case of mature teratoma of the pharynx and nasal cavity in a newborn, which caused severe obstruction of the upper respiratory tract and required emergency action. The article describes the diagnostic algorithm, the choice of optimal treatment tactics, which allowed rehabilitate the child in infancy.

Laryngeal cysts in children are relatively rare, occupying the 4-5th place in the structure of congenital malformations. The paper presents the combined experience of two Russian pediatric otorhinolaryngological clinics traditionally involved in the rehabilitation of patients with congenital and acquired pathology of the larynx., Objective: To analyze the features of the clinic, diagnosis and treatment of laryngeal cysts in children., Material and Methods: The study included 68 children with laryngeal cysts aged from 3 days to 16 years (on average 39.5±37.0 months, Me=15.5 months). The cyst was localized in the vestibular region of the larynx in 15 (22.1%) patients, in the vocal region - in 15 (22.1%) patients, and in the sub-vocal region - in 38 (55.9%) patients. Data on the presence of a history of tracheal intubation were available in 35 (89.7%) children, including 35 (92.1%) of 38 children with a subfold cyst. 11 patients were admitted with a previously applied tracheostomy., Results: The main reasons for going to the clinic were signs of laryngeal stenosis (stridor, signs of obstruction of the upper airways) in 60.3% of patients, dysphonia - in 33.8%, and in 5.9%, the detection of a cyst became an accidental finding. To eliminate the cyst, the method of laser marsupialization was used in 10 patients, coagulatory ablation - in 2 patients, in the remaining 56 patients, decortication was performed with microinstruments, followed by laser treatment of the cyst bed. In the follow-up, children were traced from 6 months to 7 years. We did not observe a recurrence of a cyst in any case., Conclusion: Currently, the lining department is the "favorite" localization of the cyst in childhood. Subclavian cysts are more common in preterm infants who need tracheal intubation. A necessary condition for radical elimination is the resection of the cyst walls.

Aim: To optimize the current algorithms of diagnosis and treatment of children with congenital laryngeal paralysis., Method: A retrospective study of case histories of patients with congenital laryngeal paralysis who were examined and treated at St. Petersburg State Pediatric Medical University., Results: For the period from 1995 to 2015, 30 children with various forms of congenital laryngeal paralysis were under medical supervision at the Clinic for Otorhinolaryngology at St. Petersburg State Medical University., Conclusion: In cases of stage 1-2 laryngeal stenosis in children older than 3 years 6 months, the chordarythenoidotomy may be the procedure of choice. In cases of stage 2 laryngeal stenosis, in children with tracheostomy, we consider laryngotracheoplasty with the insertion of costal autograft in the back wall of the larynx at the age of 4 years as the optimal choice.

The number of large deletions of mitochondrial DNA in whole peripheral blood of the former Mayak PA workers occupationally exposed to prolonged γ-radiation has been determined in the long term period after irradiation (mean cumulative dose 135.40 ± 22.03 cGy, age range at the time of blood sampling 67-76 years) and compared with the number of deletions in groups of "young" (19-33 years) and "adult" (66-73 years) individuals who had no contact with radiation sources. Samples of the total DNA from the peripheral blood were obtained from the Radiobiological Human Tissue Repository of the Southern Urals Biophysics Institute (Ozyorsk, Chelyabinsk region) and used for carrying out a long-distance PCR. The analysis of the data showed a statistically significant increase in the number of large deletions in the peripheral blood of "adult" donors of the control group as compared with the control group of "young" donors (51.6 and 14.3%, respec- tively). No statistically significant difference in the number of large deletions in the group of former Mayak PA workers occupationally subjected to prolonged exposure to γ-radiation as compared with the control do- nors of similar age was found (53.6 and 43.8% respectively).

Radiobiological Human Tissue repository was established in order to obtain and store biological material from Mayak PA workers occupationally exposed to ionizing (α- and/or γ-) radiation in a wide dose range, from the residents exposed to long term radiation due to radiation accidents and transfer of the samples to scientists for the purpose of studying the effects of radiation for people and their offspring. The accumulated biomaterial is the informational and research potential that form the basis for the work of the scientists in different spheres of biology and medicine. The repository comprises 5 sections: tumor and non-tumor tissues obtained in the course of autopsies, biopsies, surgeries, samples of blood and its components, of DNA, induced sputum, saliva, and other from people exposed or unexposed (control) to radiation. The biomaterial is stored in formalin, in paraffin blocks, slides, as well as in the freezers under low temperatures. All the information on the samples and the registrants (medical, dosimetry, demographic, and occupational data) was obtained and entered into the electronic database. A constantly updated website of the repository was developed in order to provide a possibility to get acquainted with the material and proceed with application for biosamples for scientists from Russia and abroad. Some data obtained in the course of scientific research works on the basis of the biomaterial from the Repository are briefly introduced in the review.

Long-term post-radiation changes in the level of microsatellite DNA polymorphism in peripheral blood of the male "Mayak" employees (Ozyorsk, Russia), who had been exposed to prolonged gamma-irradiation during professional activities, were studied. DNA samples were obtained from the Radiobiology Repository of Human Tissue (Southern-Urals Biophysics Institute FMBA) and used as templates for arbitrarily primed PCR. Comparative analysis of the obtained samples of DNA fragments showed a significant increase in the number of high-molecular fragments and reduction in the number of amplified low molecular weight DNA fragments in comparison with the control. However, a direct correlation of the level of DNA polymorphism with the accumulated total dose of radiation was not found. The study of the polymorphism of microsatellite DNA repeats can be used for qualitative assessment of the levels of genetic variability.

The objective of this research was quantitative assessment of serum and membrane regulatory proteins in blood from nuclear workers as markers of radiation-induced alterations in immune homeostasis in the late period after protracted exposure of nuclear workers with different doses. The effector and regulatory lymphocytes were measured using a flow cytofluorometer in workers from the main facilities of the Mayak PA (aged ∼60 y up to 80 y) in the late period after combined exposure to external gamma-rays and internal alpha-radiation from incorporated 239Pu. The control group included non-occupationally exposed members of the Ozyorsk population matched by gender and age to the group of Mayak workers. Thirty serum proteins involved in regulation of immune homeostasis, such as growth factors, multifunctional interleukins, pro- and anti-inflammatory cytokines, and their receptors, were measured using ELISA in blood serum specimens from the Radiobiology Human Tissue Repository. The dosimetry estimates were obtained using Doses-2005. The correlation analysis revealed a statistically significant direct relationship of T-killers and plutonium body burden and a decreasing level of T-helpers with accumulated external dose in exposed individuals. There were differences in expression of membrane markers in young regulatory cells (double null T-lymphocytes, NKT-lymphocytes, regulatory T-cells, and an increase of activated forms of T-lymphocytes), which indicated an active role of regulatory cells in maintaining immune homeostasis in terms of protracted exposure. The assessment of regulatory proteins in blood indicated that growth factors (EGF, TGF-β1, PDGF), multifunctional interleukins (IL-17A, IL-18), and pro-inflammatory cytokines (IL-1β and INF-γ) could be potential markers of radiation-induced alterations in protein status. An imbalance of pro- and antiinflammatory proteins in blood and variations of protein profiles at the lower exposure levels (gamma-ray dose <1 Gy, plutonium body burden <0.74 kBq) in the late period after protracted exposure were less pronounced than at the higher exposure levels, which was probably explained by compensatory-adaptive responses in the late period among senile individuals with polypathology.

Genome variability and changes in immune homeostasis, induced in man in the course of long-term industrial contact with ionizing radiation (IR) sources were studied by using unique biomaterials stored in the Radiobiological Repository for Human Tissues at the Southern Urals Biophysics Institute, FMBA. The biomaterials, peripheral blood samples and blood DNA were obtained from the "Mayak" PA employers occupationally exposed to prolonged external gamma-radiation and/or internal alpha-radiation from incorporated 239Pu in a wide range of accumulated doses. A significant increase in the polymorphism of microsatellite-associated peripheral blood DNA repeats was revealed in a group of persons with accumulated doses of external gamma-radiation above 2.0 Gy, as well as in the descendants of parents with preconceptive doses of higher than 2.0 Gy. In persons whose parents had a preconceptive dose above 2.0 Gy, an increase in the gene p53 mutation rate was observed, and descendants of persons with dose of 3.0 Gy and higher showed mtDNA heteroplasmy, regardless of the sex of an exposed parent. Changes in the expression of membrane markers for the effector and regulatory T-lymphocytes depending on radiation type and dose load were determined. The growth factor level variations (TGF-beta1, EGF, HGF, FGF) in peripheral blood serum in persons exposed to radiation from gamma- or alpha-sources, allow us to consider them as biomarkers of radiation-induced disturbances in immune homeostasis. The concentration changes of TGF-beta1, apoptosis proteins (p53, TPA-cyk, sAPO-1/Fas), and the adhesion molecule sCD27 in the case of cardiovascular diseases in the serum of both irradiated and non-irradiated "Mayak" PA employers point to the information value of these immune response characteristics as specific biomarkers of cardiac disorders. It is proposed that the revealed changes in immune homeostasis and in the variability of somatic cell genome may provoke development of tumors and cardiovascular diseases in man in delayed periods after prolonged exposure to IR.

The mutations in mitochondrial DNA (mtDNA) arise at a higher frequency than in nuclear DNA, and their appearance in peripheral blood can be considered as a sensitive marker to estimate the level of genotoxic load. For revealing the presence of mutations in mtDNA of peripheral blood, we used the method of temporal temperature gradient gel electrophoresis (TTGE). The samples of whole blood DNA from four donor groups were used. Group I contained 10 young (23-26 years) donors and Group II 12 elderly (65-74 years) donors. Group III was formed from patients with breast cancer (12 women) past sessions of radio-chemotherapies (RCHT). Group IV was made of professionals of a nucleus plant occupationally exposed to chronic gamma-irradiation. PCR was carried out on four coding sequences and on one hypervariable sequence of the D-loop (DloopI) of mtDNA. PCR products were tested with TTGE. Most mutations were revealed in the DloopI. Heteroplasmy in the region of DloopI was registered in the blood of each donor of Group III 7 days after the RCHT session. Also, mutations in mtDNA Dloop1 were found in 6 of 13 individuals of Group IV. The blood of this donor group was taken 16 to 28 years after prolonged irradiations in a dose range of 250-350 cGy. In the elderly donor group, the same results were observed in 3 of 12 individuals. The results show that the method of TTGE can be used in mass analyses to assess the effects of radiation and other genotoxic agents in man by detection of unknown mutations in peripheral blood mtDNA.

A repository of bio-specimens that includes organs from 700 deceased workers employed at the first nuclear weapons facility "Mayak" and donations of blood, buccal cells, and tissues removed at the time of surgery and/or biopsy from the members of the Mayak cohort undergoing medical treatment or diagnostic procedures has been established at the Southern Ural Biophysics Institute, in Ozyorsk, Russian Federation. The autopsied tissues include formaline-preserved organs, paraffin blocks, and histology slides. For all, occupational, dosimetry, and detailed medical information is available. For 359 individuals, information on malignant tumors, i.e., lung (171), stomach (51), liver (28), and intestine (19), as well as 32 cases of leukemia, are available. External gamma exposures are known for 95% of the 700 autopsies, of whom 560 were exposed to protracted doses exceeding 0.5 Gy, with known maximum annual doses ranging from 0.01-0.5 Gy for about 46%, and annual doses exceeding 0.5 Gy for 48%. Plutonium body burden is known for 73%, of which 40% had body burden greater than 1.5 kBq, and 15% of individuals had body burdens greater than 11.85 kBq. Newly collected specimens include frozen lymphocytes, EBV-immortalized B-cells, frozen erythrocytes, and DNA as well as frozen tumors. Donations were obtained to date from more than 1,600 individuals. For these donors external doses of exposure exceeded 0.5 Gy for 83%, and plutonium body burden exceeded 1.48 kBq for 30%. A Web site describing the Repository that also includes forms for tissue requests can be accessed at http://www.subi.ru/RHTR.

Intravenous injection of plutonium dioxide with 1-2 microns particle sizes in amount of 92.5, 46.3 and 23.2 kBq/kg of body mass increased the yield of chromosome aberrations in bone marrow cells of rats by 3.7, 2.3 and 1.7 times, correspondingly, in comparison with the spontaneous level. The model of chromosome aberration dependence on dose of radionuclide was developed based on the experimental results.

Frequency of chromosome aberrations in the liver cells of hepatectomized Wistar rats aged 3, 9, 12 and 18 months was studied. Changes in the number of chromosomes and damage of their structure at the metaphase stage of the first division hepatocytes are analyzed. The content of aneuploid cells was 1-3% of the total number of metaphases studied and did not change with the age of animals. Frequency of chromosomal aberrations increased with aging from 1.3 to 6.9 in rats aged 3 and 18 months, respectively, mainly due to chromatid-type aberrations.

Intravenous injection of polymeric 239Pu(IV) nitrate (166.5, 55.5 and 18.5 kBq/kg body mass) to Wistar rats was shown to produce biphase changes in the frequency of hepatocyte chromosome aberrations. The increase in the structural damages to chromosomes at later times of observation was a pronounced function of radiation dose. The absence of such a dependence at early times was evidently due to the elimination of damaged liver parenchyma cells.