bluuebunny/crossref_metadata_2025_split huggingface dataset:
The clinical course of cutaneous mastocytosis in a prospective group of 163 children according to electronic parent survey
Authors: Potapenko V. G. , Baykov V. V. , Boychenko E. G. , Zaslavsky D. V. , Krasnogorskaya O. L. , Krivolapov Yu. A. , Lisukova E. V. , Seminiv S. Ya. , Skoryukova K. A. , Sydikov A. A. , Kulibaba T. G. 
Published: July 2021

Introduction. Mastocytosis occurs due to clonal mast cells proliferation and may have different clinical course. As the disease only rarely occurs in pediatric population there are very few clinical observations describing its symptoms, prognosis and therapy response in pediatric cohorts.Aim of the study — characterization of gender, sympthoms, prognosis and therapy in children with mastocytosis using data obtained by electronic parent survey.Methods. The study data was collected from November 2014 till August 2020 from parents referring for consultation of hematologist via “Vkontakte” social network. All parents completed two surveys. The first one was performed right after the referral, the second one was performed from May 2020 to August 2020. The data on age at symptoms onset, family history, symptoms, rash distribution, serum tryptase concentrations, sunlight sensitivity and clinical course was obtained and analyzed.Results. The data on 163 children was obtained, 86 (52.7 %) of them were boys and 77(47.3 %) were girls. The median age was 6 (0.5—22) years. At the end of survey the median observation time was 61.5 (2—276) months. The most common mastocytosis clinical variant was urticaria pigmentosa (n = 129; 79.1 %), mastocytoma in 18 (11 %) and in 15 (9.9 %) an unknown variant was observed. The diagnosis was based on skin biopsy in 19 patients (11.6 %). Characteristic symptoms were seen in 123 (75.4 %) children. The main complaints were skin reaction triggered by various factors (n = 89; 72.3 %) and itching (n = 78; 63.4 %). Most patients took antihistamines (n = 64; 61 %) and ketotifen (n = 22; 21 %). The second survey was conducted in 139 (85.2 %) pts. At the time of survey symptoms progression was seen in 4 (2.9 %) patients, while in 42 (30.2 %) cases stabilization, and in 93 (66.9 %) cases improvement were observed.Conclusion. The symptoms onset mostly occurs in the first year of life. Most frequents symptoms are itching and rash in response to various triggers. Antihistamine drugs led to symptoms mitigation. The clinical course is benign in most children.
Von Willebrand factor multimeric assay: novel diagnostics capabilities
Authors: Poletaev A. V. , Seregina E. A. , Pshonkin A. V. , Karamyan N. A. , Fedorova D. V. , Plyasunova S. A. , Zharkov P. A. 
Published: July 2021

Introduction. Distinguishing between Von Willebrand disease (vWD) types often requires multimer gel analysis. The current techniques for vWF multimer structure are manual, complicated, non-standardized and time consuming. The aim of this study was to evaluate diagnostic capabilities of new automated vWF multimer screening assay.Materials and methods. Children with vWD, acquired von Willebrand Syndrome (aVWS) and 8 healthy donors as a control group were enrolled in this study. Von Willebrand factor antigen (vWF Ag); ristocetin cofactor activity (VWF:Rco); vWF collagen binding (VWF:CB); ristocetin-induced platelet aggregation (RIPA); factor VIII clotting activity (FVIII:C) and vWF factor VIII binding activity (vWF:FVIIIb) were performed to evaluate vWD. Multimer analysis was carried out using the commercial HYDRAGEL 5 von Willebrand Multimers kit on semi-automatic gel electrophoresis instrument HYDRASYS (SEBIA).Results. The samples from control group had 9—12 bands of vWF multimers with the same distribution as control plasma. Patients with type I vWD had the proportional decrease in the intensity of the bands with preservation of the normal distribution of the band. Patients with type III vWD reveal the complete absence of the multimer bands on the gel. Multimer analysis in type IIA shows the absence of high molecular weight multimer bands. In other patients the distribution of vWF multimers was normal against the changes in functional properties of vWF (types IIM, N). Most of the children with aVWS also revealed normal distribution of vWF multimers, however, in some patients, the slight decrease in large multimeric forms was observed visually on the gel.Conclusion. Multimer analysis allows to visualize the multimer distribution in various types of von Willebrand disease. The method is easy to perform and can be useful for distinguishing between the subtypes of vWD. But only the full test panel including genetic tests would allow the differentiantion of vWD types with high precision.
Treatment of severe infectious complications caused by multidrug-resistant Klebsiella pneumoniae in children with malignant neoplasms of the hematopoietic system: experience of the Research Institute of Pediatric Oncology and Hematology at N.N. Blokhin Russian Cancer Research Center
Authors: Sidorova N. V. , Machneva E. B. , Valiev T. T. , Kostareva I. O. , Aliev T. Z. , Elfimova A. Yu. , Lozovan Yu. V. , Pavlova T. Yu. , Korkina Yu. S. , Gorbunova T. V. , Grigorievskaya Z. V. , Petukhova I. N. , Epifanova N. Yu. , Kirgizov K. I. 
Published: July 2021

Introduction. So far there has been no clear protocol on the treatment of bacterial infections in hematopoietic cancer patients undergoing polychemotherapy (PCT) and hematopoietic stem cell transplantation (HSCT). Guidelines available from antibiotic therapy panels such as EMBT, NCCN, ECIL, Sepsis-3 often fail to cover the entire spectrum of clinical risk factors of severe complications caused specifically by multiresistant Klebsiella pneumoniae.The aim of the study — is to showcase the clinical experience of demonstration of the experience of the Research Institute of Pediatric Oncology and Hematology at N.N. Blokhin Russian Cancer Research Center with respect to adjusting antibacterial therapy for the spectrum of microorganisms found in the patient before the onset of antitumor therapy, and for the multiresistant microorganism findings in patients with blood cancers and febrile neutropenia (FN) undergoing PCT and HSCT.Materials and methods. The study involved five patients undergoing either PCT or HSCT for hematopoietic cancers at Research Institute of Pediatric Oncology and Hematology in October 2019 — October 2020, multiresistant Klebsiella pneumonia colonies found in each case. Results. Five patients with hematopoietic cancers and induced bone marrow aplasia were found to have multiresistant Klebsiella pneumoniae colonies on top of post-PCT/HSCT immunosuppression. Given high risk of death, these patients need early antibacterial therapy with reserve antibiotics outside standard empirical antibacterial treatment protocols should they develop FN. The Center's practices have shown that baseline protocols are often inadequate to the severity of these patients' conditions in a certain timeframe.Conclusions. To sum up the Center's limited experience, the finding is that additional research is required into the factors of risk of severe multiresistant Klebsiella pneumoniae infections in patients undergoing PCT and HSCT; algorithms must be developed for the treatment of patients in such a critical condition.
Predictive biomarkers of inhibitors immune checkpoints therapy in malignant tumors
Authors: Kiselevsky M. V. , Samoylenko I. V. , Zharkova O. V. , Ziganshina N. V. , Petkevich A. A. , Sitdikova S. M. , Suleymanova A. M. , Sagoyan G. B. , Efimova M. M. , Kirgizov K. I. , Varfolomeeva S. R. 
Published: July 2021

Immune checkpoint inhibitors (ICT) therapy is a successful immunotherapy (IT) strategy that is quite effective in a number of patients with non-small cell lung cancer, melanoma, bladder cancer, breast cancer and others. Nevertheless, there is a need in predictive markers for ICT therapy for personalized IT as far as there is a large group of patients, the proportion of which varies depending on the tumor, who do not have a clinical response to such therapy. The review summarizes the theoretical aspects and results of clinical trials dedicated to various clinical efficiency predictor using modern databases. As a result of the analysis it is established that the main candidates for the role of such markers are tumor infiltrating lymphocytes and their subpopulations, peripheral blood lymphocytes (PBL) and their subpopulations. PD1 (programmed death receptor 1) and PDL1 (programmed death receptor ligand 1) expression in tumor tissue can also be important for predicting IT efficiency. The most promising predictive biomarker meaning the most clinically relevant is a combination of the PBL subpopulations study and PD1 and PDL1 expression on the tumor cells.PubMed, Scopus, Web of Science, eLibrary, Russian Science Citation Index databases were searched for the available appropriate literature reports. The authors included 82 in the given review.
The latest trends in improving CAR-T cell therapy: from leukemia to solid malignant tumors
Authors: Ershov A. V. , Demyanov G. V. , Nasrullaeva D. A. , Radkevich E. R. , Dolgikh V. T. , Sidorova N. V. , Valiev T. T. , Efimova M. M. , Machneva E. B. , Kirgizov K. I. , Kiselevsky M. V. , Manasova Z. Sh. 
Published: July 2021

CAR-Т cell therapy with the use of cytotoxic lymphocytes with chimeric antigen receptors occupies an important place among modern approaches to the cancer treatment. This therapy has established itself as an effective method of the treatment of CD19+ acute lymphoblastic leukemia. Nevertheless, the recurrences of the illness are not uncommon; the treatment of solid tumors with genetically engineered lymphocytes shows modest results and it is accompanied by the high toxicity. One thing, however, is certain: CAR-Т cell therapy has great potential in the treatment of cancer and further improving of the structure and functions of genetically engineered lymphocytes with chimeric Т cell receptors help greatly increase the efficiency of antitumor treatment.The review includes the current data on the structure of chimeric lymphocytes of different generations and the trends in improving CAR-Т cell therapy. It includes also the fundamental platform for formation of ideology of use CAR-Т cells for the treatment of solid malignant tumors.
Disinfection Measures during COVID-19 for Dental Operatories
Authors: Motwani Nidhi , Ikhar Anuja , Chandak Manoj , Gondivkar Shailesh 
Published: July 2021

Background:
In the dental health-care setups, the environmental object and surfaces are expected to be infected by the COVID virus wherein definite procedures are performed. Consequently, these objects and surfaces, particularly where COVID-19 patients are being treated, must be appropriately cleaned and sanitized to stop further spread.

Objective:
The present article is intended for providing protocols about the cleaning and disinfection of objects and surfaces in the circumstances of COVID-19 for Dental operatories.

Methods:
Studies evaluating the surface characteristics of the virus as well as effective disinfection measures have been documented.

Results:
Various chemicals in different concentrations have a virocidal effect. The devices introduced include used UV radiation and ozone.

Conclusion:
These changes in dental clinical practice are needed to save humanity by preventing further transmission of disease.
Investigating the Knowledge of Sexually Transmitted Diseases in University Students of Iran
Authors: Vakilian Katayon 
Published: July 2021

Background:
Unprotected sex, multi partnership, no or inconsistent use of the condom can be mentioned as risk behaviors putting youth at high risk to Sexually Transmitted Diseases (STDs). The present study aimed to investigate the knowledge of sexually transmitted diseases in the university students of Shahroud in Iran.

Methods:
This cross-sectional study was conducted in Shahroud city of Iran. 1500 female and male students in the age bracket of 18-24 were included in the study. Multistage sampling was employed. After stating the objective of the study, the questionnaire was distributed to students during the end of lecture time upon the agreement of the education officials and collected after 15 minutes. To ensure that the information provided will be kept confidential, the students were asked not to write down their names and fields of study. Data were described using descriptive statistics by SPSS software, version 20.

Results:
The answers showed that the female and male students have heard more about HIV (94.9% vs. 93.1%), gonorrhea (47.2% vs. 50.2%), genital herpes (45.6% vs. 33.1%), and genital warts (31.4% vs. 18.1%), in the order of frequency. 35.9% of females had no knowledge about the symptoms of diseases in women and 53% of males had no knowledge about the symptoms of the diseases in men. 26.6% of female students and 16% of male students knew regarding at least three symptoms of diseases.

Conclusion:
The present study showed that the university students' knowledge is far distant from the desired situation. This study succeeded in identifying the educational needs of the youth.
Routing and Treatment of Patients with Hematogenous Vertebral Osteomyelitis on the Tyumen Region Example
Authors: Bazarov A. Yu. 
Published: July 2021

Background. Currently, various algorithms of tactical classifications and treatment regimens for hematogenous vertebral osteomyelitis (HVO) are proposed. However, all proposed approaches are designed for the treatment of patients in specialized multidisciplinary clinics departments, although the initial treatment often occurs in municipal polyclinics, private medical centers and district hospitals. The question is what the appropriate scope of diagnostic and therapeutic measures in institutions with different material and resource bases and how to ensure continuity according to the existing three-level system of medical care remains open. The aim of this studyis to determine the probable routing and treatment options for patients with HVO on the example of the Tyumen region, depending on the existing three-level system of providing medical care to the population.Object and Methods.The current Orders of the Ministry of Health of the Russian Federation and the Resolutions of the Government of the Tyumen Region were studied, on which basis a three-level system of providing medical care to the population was organized and organizations providing emergency and elective medical care to patients with HVO were identified. The volume of medical and diagnostic measures in patients with HVO in institutions of various levels, as well as their routing to the regional clinical hospital No. 2 in case of impossibility or inexpediency of providing specialized medical care at the place of primary treatment, is analyzed on the example of the Tyumen region. The clinical material is presented by 267 patients with HVO who were treated in the Tyumen regional clinical hospital No. 2 from 2006 to 2019, 78.7% of whom were treated in the profile of traumatology and orthopedics, 18.7% — neurosurgery and 2.6% — surgery. Results.The list of medical institutions that provide emergency and planned care in traumatology and orthopedics, neurosurgery in the Tyumen region is defined, depending on the level of the institution and the available material and resource base. A block diagram is proposed that determines the scope of medical and diagnostic measures in institutions of I-III levels and specialized departments of multidisciplinary clinical hospitals. The principle of determining the patient profile (surgical, orthopedic, neurosurgical) depending on the type and severity of the inflammatory lesion according to the classification E. Pola (2017) is proposed.Conclusion. The need for effective use of medical and diagnostic resources starting from level I is determined by clear routing of the patient to ensure the continuity of medical care. A full-fledged diagnosis is possible in institutions not lower than the third level with the mandatory determination of the lesion type and the severity of the inflammatory process. HVO treatment is advisable in level III institutions, specialized departments of regional clinical hospitals and federal institutions.
Complications of Acute Acetabular Fractures Surgical Treatment: Systematic Review
Authors: Kolesnik A. I. , Zagorodniy N. V. , Ochkurenko A. A. , Lazarev A. F. , Solod E. I. , Donchenko S. V. , Solodilov I. M. , Ivanov D. A. , Ovcharenko A. V. , Surikov V. V. 
Published: July 2021

Background.The combination of classical anterior and posterior approaches, as well as their modifications, is recognized as the most effective in acetabular fractures surgical treatment. The use of classical and modified approaches is accompanied by serious intra- and postoperative complications associated primarily with the duration of surgery, significant blood loss, tissue trauma, surgical site infection. The aim of review — to determine the main and most frequent complications associated with surgical approaches to the acetabulum. material and methods. Access to literature sources is carried out in the information systems and databases PubMed/Medline, Embase, Scopus, Сochran Library, eLibrary, Wiley Online Library. Keywords: acetabular fractures, surgical treatment, approach to the acetabulum, total hip replacement.Results. The most common intraoperative complications are incorrect fragments reduction, sciatic nerve injury, less often — intraarticular implant position, damage to the superior gluteal artery and other vessels, among the early postoperative complications - wound infection, both superficial and deep, less often — venous thrombosis. Late postoperative complications are mainly represented by the development of heterotopic ossification, post-traumatic coxarthrosis, less often revealed aseptic necrosis of the femoral head, residual protrusion and secondary femoral head lesion. Conclusion. The main approach to the acetabulum are the ileo-inguinal and the Kocher-Langenbeck approach, as well as their combination.Complications were more common using two approaches, especially in cases of simultaneous use. The main intraoperative complications are the sciatic nerve and the superior gluteal artery injury with posterior approaches, the femoral lateral cutaneous nerve with expanded ilio-femoral approach, and the obturator nerve with anterior approaches.
Efficiency of Surgical Debridement and Implant Retaining in Treatment of Early Postoperative and Acute Hematogenous Periprosthetic Infections of Hip
Authors: Tryapichnikov A. S. , Ermakov A. M. , Silantieva T. A. , Burtsev A. V. 
Published: July 2021

Background. Periprosthetic infection is a devastating complication that influences the duration of treatment and patients life quality. Debridement, Antibiotics and Implant Retention (DAIR) is considered as least invasive surgery patients with stable implant, except cases of chronical periprosthetic infection. The aim of this study was to evaluate efficiency of surgical debridement and implant retaining in control over infection in group patients with early postoperative and acute hematogenous periprosthetic infections. Materials and Methods.We performed retrospective monocentral cohort study of treatment early postoperative and acute hematogenous periprosthetic infections of hip in 26 patients. The group included cases with stable implants and period between manifestation of infection and DAIR no more than 4 weeks. We have classified infection as early postoperative in 22 patients (84,2%) and as acute hematogenous in 4 cases (15,8%). Results.At mean follow-up 42,8±2,3 mounts five patients underwent removal of implant due to reinfection. We performed successful twostage revision for four of them and had to perform resection arthroplasty in one case. Thus, DAIR protocol was successful in 80,8(%) cases. The mean Harris Hip Score significantly improved compared to preoperative values from 59,2±2,5 to 80,5±1,3 at the last follow-up (p = 0,0002, Z-3,7). Conclusions.The efficiency of DAIR according to our data was 80,8%. These results allow to consider DAIR as a method of treatment of patients with early postoperative and acute hematogenous periprosthetic infections. Exchange of modular components can decrease the reinfection rate.
Upper Limb Pathology in Children with Mucopolysaccharidoses
Authors: Kenis V. M. , Melchenko E. V. , Markova T. V. , Al Kaissi Ali , Sapogovskiy A. V. , Agranovich O. V. , Petrova E. V. 
Published: July 2021

Background. Despite the success in the treatment of children with mucopolysaccharidoses (MPS) as a result of the widespread of enzyme replacement therapy and hematopoietic stem cells transplantation, orthopedic manifestations continue to be a significant problem, while the pathology of the upper limbs in children with MPS is not sufficiently represented in the literature. The aim of this studywas to analyze orthopedic and neurological manifestations in the upper extremities of children with mucopolysaccharidosis based on a sequential case series. Materials and Methods. We carried out a comprehensive analysis of clinical and radiological involvement of the upper extremities in 49 patients with MPS. Results. The most common complaints reated to the upper extremities were difficulties in the daily activities (dressing, self-care, playing), impairment of the fine motor skills, and muscle weakness. The most frequent clinical manifestations related to the upper extremities were limited active shoulder abduction, impaired hand grip, flexion contractures of the elbow joint, ulnar deviation of the hand. All patients with MPS types I, II, and VI had limited active and passive extension and flexion of the metacarpophalangeal and interphalangeal joints. In patients with MPS IV, hypermobility of the hand joints prevailed. We noticed minimal presence of typical clinical manifestations related to compression of the median nerve secific for carpal tunnel syndrome. The majority of patients showed a decrease in tendon and periosteal reflexes. The most pronounced decrease in muscle strength was observed in to extensors (elbow, fingers) and shoulder abductors, which may contribute to the predominant formation of a flexion pattern of contractures. On radiographs of the hand, the “melting sugar” symptom and shortening of the metacarpal bones were observed in most patients. Conclusion.Clinical and radiological manifestations related to the upper extremities take place in all types of the mPS, and lead to functional disorders that complicate daily life and self-care. Upper limb pathology in children with MPS requires earlier detection and more active treatment after comprehensive risk assessment.