bluuebunny/crossref_metadata_2025 huggingface dataset:
Uso de implantes dentais em pacientes com bruxismo
Authors: Garcia Menandro dos Santos , Furuyama Ricardo Jun , Favarete Dalter Silva , Fares Nasser Hussein
Published: March 2021

Dentition with severe wear in patients stricken by bruxism is a challenge due to loss of dental structure and occlusal vertical dimension and the temporomandibular implications. Bruxism is presented as a contraindication in cases of dental implants. This systematic review was carried out by examining the electronic literature available at PubMed and Google Scholar from 2010 to 2020 in Portuguese, English and Spanish languages. The review details the relationship between bruxism and dental implant failure in contrast to temporomandibular disorders, presenting the following conclusions: a) bruxism is a contributing factor to the occurrence of technical / biological complications of the dental implant and plays an important role in the failure of the dental implant; b) the anamnesis must be detailed to confirm sleep or day bruxism; c) the control of bruxism through the use of occlusal stabilization device may provide relief in the implants region and it is highly recommended. The aim of this literature review was to summarize the influences of different potential risk factors on the incidence of late dental implant failure. The protocol for this systematic review was prepared and implemented based on PRISMA guideline – Preferred Reporting Items for Systematic Reviews and Meta-Analyses.
O impacto da Covid-19 na Odontologia – revisão sistemática
Authors: de Lira Ana de Lourdes Sá , de Sousa Francisca Janiele
Published: March 2021

This study aimed to review, qualify and summarize the existing evidence on associations and risk factors for Covid-19 during dental treatment and dentists’ knowledge about these risks. After being registered with PROSPERO, the systematic review was carried out in accordance with the PRISMA guidelines, with searches in electronic databases, in specific journals, manual searches and Google Scholar, without language restrictions, from December 2019 to August 2020. Two questions were asked to be investigated: did patients seek dental care in the emergency room and do dentists know the symptoms and the mode of transmission of Covid-19, feeling qualified to attend them? Only 6 articles were included because they met all defined inclusion criteria. It was concluded that endodontic urgencies and emergencies with symptomatic irreversible pulpitis are the most common, consisting of a much higher proportion of dental emergencies during Covid-19 epidemic compared with dental trauma and no-urgent care. Although dentists are aware of the symptoms, mode of transmission, infection controls, and measures in dental clinics, they do not feel confident about the correct measures to be taken to prevent Covid-19 for their staff and patients.
Remoção cirúrgica de terceiro molar ectópico em seio maxilar – relato de caso
Authors: de Oliveira Iuri Francisco , Carvalho Marcelo Magalhães , Aguiar Felipe Muniz
Published: March 2021

The maxillary sinus is a pneumatic space that, when inside it contains foreign bodies, can trigger infections or inflammations of different severities, causing the appearance of cysts, tumors or the development of chronic sinusitis. Imaging is essential for the correct diagnosis of the case, as well as surgical planning for the removal of the foreign body. The present work aims to describe a clinical-surgical case of a patient with an ectopic third molar in the maxillary sinus, emphasizing the clinical, radiographic characteristics, therapeutic conduct and surgical technique, as well as showing the importance of theoretical and practical knowledge regarding the subject. A 49-year-old female patient attended the university’s dental clinic for surgical removal using the Caldwell-Luc technique of the ectopic upper third molar in the left maxillary sinus. The surgical removal of the ectopic element in the maxillary sinus was effective in the case presented, preventing the patient from future pathologies that could affect the sinus, as well as the symptomatological reduction of sinusitis.
Correção funcional e estética de sequela de fratura fronto-naso-órbito-etmoidal – relato de caso
Authors: Varela Dimas Albertiny Barradas de Sousa , Fialho Priscila Vital , de Carvalho Mariana Mendes , Salviano Victor Hugo Moraes , Moreira Carlos Vinicius , Seixas Alexandre Martins
Published: March 2021

Fronto-naso-orbito-etmoidal fractures, known as FNOE fractures, are the result of blunt trauma of high energy in the middle and upper third of the face. Presenting an approximate frequency of 5% to 15% of facial trauma in children and adults, respectively, FNOE fractures are found more commonly after motor vehicle accidents, physical aggression, falls or cycling accidents. The diagnosis and treatment of FNOE fractures are difficult to perform and, for this reason, the performance of a thorough clinical examination associated with a good imaging evaluation is of great importance in these traumas, as incorrect diagnosis and inadequate or late treatment generally result in aesthetic and functional. The treatment of this type of fracture must be defined after the identification of the extension, type of fracture, and affected structures in order to restore function and shape of the middle face. This study aimed to report a case of sequelae of FNOE fracture where there were aesthetic and respiratory complaints, treated with coronal access and subsequent reduction of nasal bones fracture and aesthetic correction of the nasal dorsum and glabella with the use of polymethylmethacrylate cement (PMMA). It was concluded that the early diagnosis of FNOE fractures is of great importance to avoid sequelae, with coronal access being a good access option for the surgical correction of these sequelae and the biocompatibility and handling characteristics, as well as the low cost of cement PMMA is a good option for grafting in corrections of craniofacial deformities.
Implicações clínicas do uso do L-PRF em cirurgias orais
Authors: Girundi Ana Luíza Gonçalves , Girundi Francisco Mauro da Silva , Guimarães Rodrigo Porto , Santos Vinícius Rodrigues , da Silva Wander José
Published: March 2021

The autologous blood concentrate L-PRF is responsible for the healing response in surgical procedures. However, studies results are inconclusive. Therefore, the aim of this article was to collect existing literature’s evidence concerning the clinical implications of L-PRF use in oral surgeries such as maxillary sinus lift, alveolus filling after dental extractions, and dental implants. Pubmed database was assessed and randomized clinical trials between 2006 and 2019 considering the clinical implications of L-PRF use in oral surgeries were included. A hundred and seventy articles were identified and fifteen were selected by eligibility. Although some studies had diverged, the great majority evidenced improved healing process, osseointegration and reduced post-operation pain because of L-PRF use, what was attributed to the morphological and biological characteristics of the fibrin membrane that has a three-dimensional matrix due to lack of anticoagulant during its formation, and stores growth factors, leukocytes, cytokines, and bone cells. In addition, this fibrin matrix attracts undifferentiated stem cells that will become osteoblasts. In conclusion, the greater cicatricial response, less postoperative discomfort for the patient, increased bone formation and implant-bone contact (BIC), and reduced osseointegration period are benefits associated with the L-PRF membrane use, that make L-PRF to be considered a good choice in the field of tissue regeneration.
Laminados cerâmicos – relato de caso
Authors: Montenegro Gil , Marques Erica Camargo da Silva , Marçal Ricardo Lellis , Pinto Tarcísio
Published: March 2021

The demand for aesthetics is increasing regardless of social class, age or gender. With the evolution of ceramic materials, resin cements, acid etching techniques, and silanization agents, ceramic laminates are now one of the best alternatives for rehabilitation of anterior teeth that require aesthetic restorative treatments. Given this evolution, this case report aims to demonstrate how to optimize the steps of laminates manufacturing and provide an excellent aesthetic and functional result after restoration of anterior teeth with veneers.
Reabilitação de paciente com rebordo atrofiado – relato de caso
Authors: Queiroz Marina Oliveira , Gusmão João Milton Rocha , Gusmão Cristiane Aguiar , de Menezes Lucília Fonseca
Published: March 2021

The aim of this article is to report the clinical case of rehabilitation of a patient with atypical ridge due to cleft lip and palate (CLP) through total removable prosthesis in the Unified Health System. To perform the treatment clinical and laboratory sessions were performed from the initial examination until the prosthesis installation. The patient showed significant improvement in facial aesthetics and had the masticatory function restored, which reflected on his quality of life.
Coágulo sanguíneo: uma opção NÃO viável para a preservação alveolar
Authors: Sagnori Renata Silveira , de Sá Bruno Costa Martins , Nóia Claudio Ferreira
Published: March 2021

The loss of dental elements leads to an intense remodeling of the alveolar process, resulting in important dimensional changes, which often make it difficult to install dental implants. Considering this, the objective of the present study was to evaluate the changes in height and thickness that occurred in dental alveoli after extraction. For this, 10 patients were evaluated and submitted to extraction of dental elements and followed up in the postoperative period in which there was natural healing. Cone beam computed tomography were obtained preoperatively and 3 months postoperatively and were used as a basis for assessing loss of height and thickness that occurred. Pearson correlation test with a significance level of 5% was used to assess dimensional changes. The results showed that vertical loss (average of 2.99 mm) was greater than the horizontal loss (average of 2.52 mm). The statistical analysis pointed out the existence of a very good and significant correlation between these bone losses and also revealed that the higher the loss of height, the greater the loss of thickness. In 8 of 10 patients in the sample, some grafting was necessary prior to the installation of the implants, thus increasing morbidity and time of treatment. Considering this, it was concluded that the clot was not a viable alternative for alveolar preservation aiming posterior installation of dental implants.
Utilização do Blue®m no tratamento do líquen plano oral – relato de caso
Authors: Sukekava Flavia , Carmo Filho Luiz Carlos , Luiz Julia Helena , Luiz Jaques
Published: March 2021

Oral lichen planus (OLP) is an autoimmune disease, one of the most prevalent in the mouth, which mainly affects women, leukoderma, between the third and sixth decades of life. Of unknown etiology, it may present with bilateral/symmetrical lesions, occurring throughout the mouth. The clinical characteristics range from white keratotic lesions to painful erosions and ulcerations, with reticular, atrophic, papular, erosive, bullous, and erythematous forms, depending on the time of evolution and location. Oxygen-releasing products are gaining ground in the dental market with the promise of aid in wound healing. The purpose of this case report was to associate these products in the treatment of a patient with OLP. The leukoderma female patient, 56 years old, sought dental care with a complaint of bleeding gums for years. On clinical examination, spontaneous gingival bleeding was observed in the anterior teeth region, excellent plaque control, and erythematous lesions in the cheek mucosa and anterior gingiva. Basic periodontal treatment and mouthwash with Blue®m was proposed for 2 weeks. In the reassessment, improvement was reported for 2 weeks and return to the bleeding condition. At this moment, an incisional biopsy was performed, and fragments were sent for laboratorial analysis, which found structures characteristic of OLP. The patient was instructed to maintain the standard of oral hygiene, to keep the mouthwash twice a day, associated with Blue®m oral gel over the lesions. After one-year follow-up, the patient continues to have no lesion return, no side effects, with significant improvement in quality of life.
TUBERCULOSIS UNDER CONDITIONS OF NOVEL CORONAVIRUS INFECTION
Authors:   V.A. Almazov National Medical Research Centre of the Ministry of Healthcare of Russia, Saint Petersburg, Russia, Starshinova A.A. , Malkova A.M. ,   Saint Petersburg State University, Saint Petersburg, Russia, Starshinova A.Ya. ,   Saint Petersburg State Pediatric Medical University, Saint Petersburg, Russia, Karev V.E. ,   V.A. Almazov National Medical Research Centre of the Ministry of Healthcare of Russia, Saint Petersburg, Russia,   Russian Scientific and Research Institute for Hematology and Transfusiology of the Russian Federal Biomedical Agency, Saint Petersburg, Russia, Kudlay D.A. ,   I.M. Sechenov First Moscow State Medical University, Moscow, Russia,   National Research Center Institute of Immunology of the Russian Federal Biomedical Agency, Moscow, Russia, Dovgalyuk I.F. ,   Saint Petersburg Scientific Research Institute of Phthisiopulmonology, Saint Petersburg, Russia
Published: March 2021

Tuberculosis (TB) remains an important medical and social problem up to today. In recent years, great strides have been made in the fight against tuberculosis infection (TI) in reducing TB morbidity and mortality, especially in the Russian Federation. Emergence of new COVID-19 infection in the world has led to the need for long-term isolation of the population, on the one hand, and the need to resort to personal protective equipment, on the other. The spread of COVID-19 may have an impact on the increase in TB morbidity, despite the decrease in the incidence rate, which is related to objective difficulties in detecting TB and complexity of the structure of clinical manifestations of the disease against the background of coronavirus infection and severe complications after COVID-19. The use of immunosuppressive therapy in the treatment of COVID-19 may cause immunosuppression, which will affect the results of TI immunodiagnostics using skin tests. At the same time, IGRA tests can be useful in these conditions, since they are of great diagnostic value. Equally important is the reduction of patient visits to treatment facilities in the context of a pandemic or a high incidence of COVID-19.
FORMATION OF MALE FERTILITY IN ONTOGENESIS
Authors:   National Medical Research Center for Children’s Health, Moscow, Russia, Yatsyk S.P. ,   Russian Medical Academy for Continuous Professional Education, Moscow, Russia, Tarzyan A.O. ,   National Medical Research Center for Children’s Health, Moscow, Russia, Gusev A.A. ,   National Medical Research Center for Children’s Health, Moscow, Russia
Published: March 2021

The main human species mission is fertility, which is an important point of the concept of health in general and of reproductive health in particular. Endocrinologists, obstetrician-gynaecologists, urologists and paediatricians are focused in this field of medicine. A decline in male fertility of up to 30–35% results in infertility which becomes serious concerns growing for human population. Many of the problems that lead to impaired male fertility are rooted in childhood, or rather, in prenatal ontogenesis. The penetration of vascular-bed substances into the cavity of the seminiferous tubules depends on the maturity and functional status of the structures constituting the blood-testicular barrier (BTB). A breach of BTB results in damage to the sperm epithelium and, as a consequence, in different grades of impairment of spermatogenesis. Among the diseases that predispose men to infertility varicocele predominates (up to 30%). One of the reasons for infertility is the impaired integrity of BTB when the process of spermatogenesis changes, sometimes in combination with infection, trauma, and toxic effects. Given the unfavorable prognosis of the consequences of these diseases in children, their careful and timely diagnosis is important. Fertility estimation places special emphasis on the state of BTB, which can be determined by the content of claudine-11, antisperm antibodies, inhibin B, and other markers.
HUMORAL AND CELLULAR RESPONSE TO SARS-CoV-2 IN COVID-19 PATIENTS
Authors:   National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia, Rumyantsev A.G.
Published: March 2021

The study of the immune response to SARS-CoV-2 is crucial for the prognosis and control of coronavirus infection, diagnosis and formation of individual and population immunity, the development of indications and evaluation of the effectiveness of vaccinations, and, ultimately, the scientific prediction of the course of a pandemic. One year after the infection debuted in numerous immunological studies in COVID-19 patients, kinetics, duration and evolution of immune memory in humans due to infection are not well predictable, as data obtained represent the initial effector phase of the immune response, and the responses after recovery from infection cannot be used for long-term prediction. The paper presents an analysis of the results of studies of immune response and immune memory to SARS-CoV-2, including all three branches of adaptive immunity: immunoglobulins, memory B-cells, CD8+ and CD4+ T-cells in sick and cured patients in the dynamic period of 6–8 months after the onset of the disease.
MODERN POSSIBILITIES OF SUBSTITUTION THERAPY AND HEMOPHILIA A PREVENTION IN CHILDREN
Authors:   National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia, Zharkov P.A.
Published: March 2021

Currently, the prophylactic use of factor VIII concentrate is the «gold standard» for treatment of an uncomplicated severe hemophilia A without inhibitors. However, there are a number of difficulties associated with frequent intravenous injections to maintain the activity of factor VIII above 1% in children and adolescents, which cannot but affect the adherence of patients to this type of treatment. The article discusses modern approaches to extend the half-life of recombinant factor VIII allowing to reduce the frequency of infusions and increase the residual activity of the deficient factor. On the example of efmoroctocog alpha, the first recombinant factor VIII concentrate registered in our country with a prolonged half-life, effectiveness and safety data of this class of drugs approved for use in children is presented.
RISK FACTORS FOR REDUCING VITAMIN D SUPPLY IN ADOLESCENTS WITH ARTERIAL HYPERTENSION AND OBESITY
Authors:   Bashkir State Medical University, Ufa, Republic of Bashkortostan, Russia, Zeid S.S.K. , Yakovleva L.V. ,   Bashkir State Medical University, Ufa, Republic of Bashkortostan, Russia
Published: March 2021

The aim of the study is to determine the contribution of external and internal factors, such as the season, body mass index (BMI), age, sex, degree of AH, the formation of insufficiency and/or deficiency of 25 (OH)D in adolescents with arterial hypertension (AH). Materials and methods: the core group consisted of 87 adolescents with primary AH, which then were divided into two subgroups depending on BMI (Subgroup 1 – overweight and obese, Subgroup 2 – with normal body weight). The control group consisted of 23 adolescents with the phenomenon of «white coat hypertension» (WHT). The level of 25(OH)D in the blood serum was determined by immunoextraction with further with further quantitative determination by enzyme immunoassay using StarFax 2100 analyzer (USA). A DIA source Immuno assays S.A. test system. (Belgium) was used. All calculations were carried out according to the instruction in which the norm was 25(OH)D>/=30–100 ng/ml; insufficiency – 10–29 ng/ml; deficiency – > 10 ng/ml. Results: the level of 25(OH)D in peripheral blood fluctuated in a wide range – from 5,1 to 50,2 ng/ml, the median level of 25(OH)D was 17,8 ng/ml [12,2; 23,5]. According to the results of the study, only 4 (3,6%) children had a normal vitamin D level, 95 (86,4%) had vitamin D insufficiency and 11 (10%) had a deficiency. The median values of 25(OH)D in children of the comparison group were statistically significantly higher than in children with AH of the 1st and 2nd subgroups – 23,3 ng/ml [20,8; 26,0], 14,9 ng/ml [10,8; 19,5] and 17,1 ng/ml [11,8; 23,7], respectively, p=0,001. Conclusion: according to the results of the multivariate analysis, 25(OH)D in the observed adolescents depends on many factors, such as age (6,7%), gender differences (5,7%), etc. The seasons have the greatest influence on its level (62,3%) and BMI (21,4%), the least – AH (3,9%).
STRUCTURE OF HEREDITARY AND CONGENITAL PATHOLOGY IN A MULTIDISCIPLINARY SPECIALIZED PEDIATRIC CLINIC: PERSONALIZED APPROACH TO DIAGNOSTICS
Authors:   Prof. V.F. Voino-Yasenetsky Scientific and Practical Center for Specialized Medical Care for Children of the Moscow Department of Healthcare, Moscow, Russia, Kozhanova T.V. ,   Pirogov Russian National Research Medical University, Moscow, Russia, Zhilina S.S. ,   Prof. V.F. Voino-Yasenetsky Scientific and Practical Center for Specialized Medical Care for Children of the Moscow Department of Healthcare, Moscow, Russia,   Pirogov Russian National Research Medical University, Moscow, Russia, Mescheryakova T.I. ,   Prof. V.F. Voino-Yasenetsky Scientific and Practical Center for Specialized Medical Care for Children of the Moscow Department of Healthcare, Moscow, Russia, Lukash E.N. ,   Prof. V.F. Voino-Yasenetsky Scientific and Practical Center for Specialized Medical Care for Children of the Moscow Department of Healthcare, Moscow, Russia, Markova S.I. ,   Prof. V.F. Voino-Yasenetsky Scientific and Practical Center for Specialized Medical Care for Children of the Moscow Department of Healthcare, Moscow, Russia, Zavadenko N.N. ,   Pirogov Russian National Research Medical University, Moscow, Russia, Prityko A.G. ,   Prof. V.F. Voino-Yasenetsky Scientific and Practical Center for Specialized Medical Care for Children of the Moscow Department of Healthcare, Moscow, Russia,   Pirogov Russian National Research Medical University, Moscow, Russia
Published: March 2021

Hereditary and congenital pathologies make up a significant proportion in the structure of the general morbidity and mortality of the child population. In this regard, the problem of personalized medical care in multidisciplinary clinical centers seems very urgent. In the Scientific-Practical Center of specialized medical care for children the medical genetic service successfully functions since 1995. In 2017–2019, patients were consulted by geneticists, which accounted for 10,6% of all discharged patients; karyotype studies were performed in 463 (20,4%) consulted patients, chromosomal pathology was revealed in 28 (6,04%) of all karyotyped patients. DNA research was performed in 188 patients: exome sequencing – in 112; sequencing of exons of the SCN1A gene – in 39; search for mutations in the PHOX2B gene – in 17; search for frequent mutations in the FGFR1,2,3 genes – in 15 (the technique was introduced since September 2018); sequencing of SLC2A1 gene – in 5 patients. The high demand for medical genetic counseling and laboratory genetic diagnosis of hereditary and congenital diseases confirms the need for independent medical genetic units in multidisciplinary clinical hospitals, that should interact with medical genetic services at regional and federal levels.
INTERRELATION OF rs9939609 POLYMORPHISM OF THE FTO GENE WITH CLINICAL AND METABOLIC PARAMETERS IN GIRLS WITH DIFFERENT FAT DEPOSITION TOPOGRAPHY: A PILOT STUDY
Authors:   Research Center for Family Health and Human Reproduction Problems, Irkutsk, Russia, Bairova T.A. , Sheneman E.A. ,   Research Center for Family Health and Human Reproduction Problems, Irkutsk, Russia, Ievleva K.D. ,   Research Center for Family Health and Human Reproduction Problems, Irkutsk, Russia, Rychkova L.V. ,   Research Center for Family Health and Human Reproduction Problems, Irkutsk, Russia
Published: March 2021

Objective of the research: to assess the relationship between rs9939609 FTO carriage and clinical and metabolic parameters in adolescent girls with different types of fat deposition to identify the A allele as a possible marker of abdominal fat deposition. Materials and methods: 254 adolescent girls (14–17 years): 101 girls (39%) with a body mass index (BMI) in the range of ±1 SDS included in the control group [age 16 (15–17) years], 153 girls with SDS>1 were included in the main group [age 15 (15–16) years], including 95 (62%) girls with overweight (SDS BMI from +1,0 to +2,0) [age 15 (14,5–17) years] and 58 (37%) obese girls (SDS BMI>2) [age 15 (14–16) years]. Study design: singlecenter prospective complete open controlled. Anthropometric studies using standard methods (body weight, height, BMI, waist circumference, waist-to-height ratio (WHtR), thigh circumference, fat fold thickness above the biceps, triceps muscles, on the chest front surface, at the scapula lower angle, in the abdomen area and mid-thigh area). Adipose tissue percentage (% of fat mass) was determined by the bioelectrical resistance method using a professional scale – a TANITATBF-410MA fat analyzer (Japan). Laboratory studies included determination of lipid spectrum (triglycerides, total cholesterol, high-density lipoprotein cholesterol), fasting plasma glucose, glycated hemoglobin, oral glucose tolerance test, insulin with calculation of the insulin resistance index HOMA-IR, and molecular genetic studies (rs9939609FTO). Results: the frequency of the A-allele rs9939609 FTO in the adolescent samples WHtR<0,5 and WHtR>0,5 (56,28%, χ2=10,66, p=0,0011) is higher than in the group of adolescents with normal weight (40,10%). Comparing the frequencies of alleles and genotypes between groups with WHtR<0,5 and WHtR>0,5 did not revealed any statistically significant differences (χ2=0,92, p=0,63; χ2=0,34, p=0,56). In carriers of the AA genotype with an abdominal type of fat deposition (WHtR>0,5) in comparison with carriers of a similar genotype of a gluteofemoral type of fat deposition (WHtR<0,5), the BMI SDS is higher (2,75±0,66 vs. 1,54±0,46, p=0,01) and HOMA-IR (6,55±8,09 vs. 2,59±1,34, p=0,04). Conclusion: the prevalence of the A allele rs9939609 FTO in the group of overweight and obese adolescent girls is higher than in the group of adolescents with normal weight. In adolescent girls, the A allele rs9939609 FTO is associated with excess fat accumulation (SDS BMI) and insulin resistance (HOMA-IR).
CHRONIC ATYPICAL NEUTROPHILIC DERMATOSIS WITH LIPODYSTROPHY AND FEVER (CANDLE SYNDROME) IN PEDIATRIC PRACTICE
Authors:   I.M. Sechenov First Moscow State Medical University, Moscow, Russia, Sukhovjova O.G. , Ivanova I.A. ,   Center for Family Planning and Reproduction, Moscow, Russia, Kalugina N.A. ,   Branch No. 3 of the Moscow City Children's Polyclinic No. 150 of the Moscow Department of Healthcare, Moscow, Russia, Zholobova E.S. ,   I.M. Sechenov First Moscow State Medical University, Moscow, Russia
Published: March 2021

The purpose of this publication is to describe the CANDLE syndrome (its etiology, pathogenesis, clinical manifestations) and present a clinical observation. CANDLE syndrome is a rare genetically inherited disease caused by impaired assembly by of the protease, which leads to the accumulation of abnormal (ubiquitinated) proteins in B-lymphocytes, fibroblasts, macrophages and some other cells of the body. As a result, an excessive activation of interferon synthesis occurs and a systemic inflammatory reaction develops with symptoms such as febrile fever, skin syndrome, represented by nodules and maculopapular rash, hepatosplenomegaly, lipodystrophy, amyotrophy, delayed physical development, as well as high laboratory activity. The article describes a clinical observation of a female patient with typical symptoms of CANDLE syndrome. However, due to the rarity of the disease and lack of publications describing it, it has not been possible to diagnose the child for a long time.
EXPERIENCE IN THE USE OF ALLERGEN-SPECIFIC IMMUNOTHERAPY IN TREATMENT OF PERENNIAL ALLERGIC RHINITIS AND CONCOMITANT ATOPIC DERMATITIS
Authors:   Department of Internal Medicine No. 2 of the Medical Academy named after S.I. Georgievsky with the V.I. Vernadsky Crimean Federal University (Vernadsky CFU), Simferopol, Crimea, Ukraine, Znamenskaya L.K. , Shadchneva N.A. ,   Department of Internal Medicine No. 2 of the Medical Academy named after S.I. Georgievsky with the V.I. Vernadsky Crimean Federal University (Vernadsky CFU), Simferopol, Crimea, Ukraine, Panevskaya G.N. ,   Department of Internal Medicine No. 2 of the Medical Academy named after S.I. Georgievsky with the V.I. Vernadsky Crimean Federal University (Vernadsky CFU), Simferopol, Crimea, Ukraine
Published: March 2021

Allergen-specific immunotherapy (ASIT) is currently recognized as the only treatment that can affect all links in the pathological process of allergic diseases. Airway hyperresponsiveness is significantly reduced in case of allergy respiratory manifestations, which reduces symptoms severity and the need for drugs. The article describes a clinical observation of the ASIT positive effect in persistent atopic dermatitis (AtD) with concomitant respiratory allergy and complete atopic triad development. The authors propose to consider ASIT as a preventive therapy for bronchial asthma and allergic rhinitis if patient has AtD manifestations with confirmed sensitization to aeroallergens.
HEINER SYNDROME AS A RARE FORM OF ALLERGY TO COW’S MILK PROTEINS DURING THE BABY’S FIRST YEAR OF LIFE
Authors:   Tyumen State Medical University, Tyumen, Russia, Richkova O.A. ,   Tyumen Regional Clinical Hospital No. 1, Tyumen, Russia, Khorosheva E.Yu. ,   Tyumen State Medical University, Tyumen, Russia,   Tyumen Regional Clinical Hospital No. 1, Tyumen, Russia, Kuzmicheva K.P. ,   Tyumen State Medical University, Tyumen, Russia,   Tyumen Regional Clinical Hospital No. 1, Tyumen, Russia, Gorohova N.E. ,   Tyumen Regional Clinical Hospital No. 1, Tyumen, Russia, Malinina E.I. ,   Tyumen State Medical University, Tyumen, Russia, Sheshegova M.M. ,   Tyumen State Medical University, Tyumen, Russia
Published: March 2021

The problem of food allergy is now receiving considerable attention in paediatrics, due not only to its increasing prevalence but also to the difficulties of differential diagnosis of the disease. Cow's milk protein (CMP) is a leading food allergen in young children. The skin and gastrointestinal tract (GIT) most often become shock organs in case of CMP allergy, and the lungs are much less likely to be affected. One of the variants of lung tissue damage is Heiner syndrome – an extremely rare pathology caused by non-IgE-dependent hypersensitivity to CMP, which occurs mainly in infants and toddlers. This clinical case described 8-month-old baby suffering from recurrent broncho-obstructive syndrome resistant to standard therapy, and repeated pneumonia. Differential diagnosis was run with congenital pulmonary airway malformation, cystic fibrosis, primary immunodeficiencies, volume rendering of tracheobronchial tree, organic pathology of GIT, gastroesophageal reflux disease. All of the above mentioned conditions are excluded during laboratory and instrumental examination. Taking into account the burdened allergic anamnesis, familial predisposition to allergies, the presence of CMP in the child's diet, a rare form of CMP allergy with damage to lung tissue – Heiner syndrome was supposed. After correcting the child's nutrition and transferring him to feeding with an adapted milk formula based on highly hydrolyzed CMP, a positive dynamics of a pronounced degree was noted. Elimination diet is the main therapeutic approach for Heiner syndrome. The description of this clinical observation will help to increase the level of knowledge about rare forms of allergic pathology in children, timely diagnosis and improve the quality of medical care for such patients.
OVERLAP NEUROLOGICAL SYNDROMES ASSOCIATED WITH ANTIBODIES TO GLUTAMIC ACID DECARBOXYLASE: CLINICAL OBSERVATION
Authors:   Pirogov Russian National Research Medical University, Moscow, Russia, Druzhinina E.S. ,   "Practical Neurology" Medical Center, Moscow, Russia, Bembeeva R.T. ,   Pirogov Russian National Research Medical University, Moscow, Russia, Druzhinin D.S. ,   Department of Nervous Diseases with Medical Genetics and Neurosurgery of the Yaroslavl State Medical University, Yaroslavl, Russia, Azizova U.M. ,   Pirogov Russian National Research Medical University, Moscow, Russia, Nikitin S.S. ,   "Practical Neurology" Medical Center, Moscow, Russia, Maslak A.A. ,   "European Medical Center" (EMC), Moscow, Russia
Published: March 2021

The article presents a description of an overlap syndrome, rare in pediatric practice, associated with antibodies to glutamic acid decarboxylase in a 4-year-old child, which was initially regarded as a variant of Guillain–Barré syndrome due to the similarity of clinical manifestations.
FIRST TIME EXPERIENCE OF COLD ATMOSPHERIC PLASMA USE IN MICROBIAL ECZEMA TREATMENT IN A 3-YEAR-OLD CHILD
Authors:   Pirogov Russian National Research Medical University, Moscow, Russia, Korotkiy V.N. , Shemshuk M.I. ,   Pirogov Russian National Research Medical University, Moscow, Russia, Rumyantsev A.G. ,   Pirogov Russian National Research Medical University, Moscow, Russia,   National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia
Published: March 2021

Microbial eczema (ME) is a chronic inflammatory process prone to recurrence, the treatment of which is associated with a number of difficulties due to the ever increasing antibiotic resistance of the microorganisms that cause it. Cold atmospheric plasma (CAP), like ultraviolet irradiation, has a pronounced bactericidal effect, but unlike the latter, it does not have undesirable effects on healthy cells. The article presents a clinical observation of the successful use of CAP in the treatment of severe ME in a 3-year-old child.
MULTISYSTEM INFLAMMATORY SYNDROME IN CHILDREN ASSOCIATED WITH COVID-19 IN THE 6-YEAR-OLD PATIENT WITH MANIFESTATION OF TYPE 1 DIABETES MELLITUS WITH DIABETIC KETOACIDOSIS
Authors:   Morozov Children’s City Clinical Hospital, Moscow, Russia, Tikhonovich Yu.V. ,   I.M. Sechenov First Moscow State Medical University, Moscow, Russia, Rtishchev A.Yu. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia,   Pirogov Russian National Research Medical University, Moscow, Russia,   People’s Friendship University of Russia, Moscow, Russia, Glazyrina A.A. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia,   People’s Friendship University of Russia, Moscow, Russia, Ovsyannikov D.Yu. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia,   People’s Friendship University of Russia, Moscow, Russia, Timofeev A.V. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia,   Pirogov Russian National Research Medical University, Moscow, Russia, Rybkina I.G. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia, Gariaeva I.V. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia, Novikova Yu.Yu. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia,   People’s Friendship University of Russia, Moscow, Russia, Romanova V.V. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia, Vorontsova I.G. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia,   Pirogov Russian National Research Medical University, Moscow, Russia, Petryaykina E.E. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia,   Pirogov Russian National Research Medical University, Moscow, Russia,   People’s Friendship University of Russia, Moscow, Russia
Published: March 2021

For the first time in the domestic literature, the article presents a clinical observation of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 in the 6-year-old patient with manifestation of type 1 diabetes mellitus (T1DM) in the form of diabetic ketoacidosis. Anamnestic, clinical and laboratory data are presented on the basis of which two life-threatening diseases was diagnosed, as well as tactics of therapy, which made it possible to achieve a positive result. This clinical observation is compared with observations of foreign colleagues. Possible pathogenetic mechanisms of MIS-C and T1DM comorbidity are discussed.
HEREDITARY ANGIOEDEMA – A PROBLEM AT THE INTERSECTION OF IMMUNOLOGY AND ALLERGOLOGY: ANALYSIS OF LITERATURE DATA AND DESCRIPTION OF A SERIES OF 34 CASES
Authors:   Saint Petersburg State Pediatric Medical University, Saint Petersburg, Russia, Guseva M.N. ,   Academician I.P. Pavlov First Saint Petersburg State Medical University, Saint Petersburg, Russia, Zinina E.I. ,   Saint Petersburg State Pediatric Medical University, Saint Petersburg, Russia, Suspitsyn E.N. ,   Saint Petersburg State Pediatric Medical University, Saint Petersburg, Russia,   N.N. Petrov National Medical Research Center for Oncology, Saint Petersburg, Russia, Kostik M.M. ,   V.A. Almazov National Medical Research Centre of the Ministry of Healthcare of Russia, Saint Petersburg, Russia
Published: March 2021

Hereditary angioedema (HAE) is a rare autosomal dominant disease characterized by isolated recurrent angioedema (AE) of various localizations: skin, the submucosa in the gastrointestinale tract, the respiratory tract. The characteristic features of edema in HAE are the absence of itching, skin flushing, accompanying urticaria, as well as the absence of pronounced effect of systemic corticosteroid therapy, systemic antihistamines therapy and adrenaline. The aim of our study was to present a descriptive characteristics of the group of patients with HAE and to access efficacy and safety of treatment. Materials and methods: the retrospective multicenter continuous nonrandomized uncontrolled study included data on 34 patients: 19 men (56%) and 15 women (44%) with HAE. The diagnosis of HAE was based on clinical and laboratorial data, according to the clinical guidelines of the Russian Association of allergologists and clinical immunologists on HAE (2014). Results The age range of patients at the time of inclusion in the study was 5–82 years, median – 38,8 (20,6; 46,9) years. Median age of first HAE episode was 12,5 (5,8; 16,3) years, ranged from 2 to 40, 22/28 (79%) of the patients manifested before 18 years. HAE was diagnosed at the median age of 30 years (17,5; 44,8) years, in 14,0 (9,0; 25,0) years after onset of the clinical manifestations. Most of the observations described have a positive family history of HAE (91%), in total 9 families were described. In 67% of families the disease manifested itself in two generations and in 33% of families – in three generations. At the onset of HAE peripheral edema was in 29/31 (94%) of the patients, and 22/31 (71%) had edema confined to the distal extremities. 7/31 (29%) patients had peripheral edema combined with edema of other locations. Abdominal attacks were in 6/31 (19%), head, neck and upper airways edema were in 7/31 (23%). The frequency of swelling of head, neck, upper respiratory tract increased statistically significantly from 23% (7/31) to 35% (7/20, p=0,003). 24 patients have recieved Icatibant treatment. No deaths were recorded against the background of this treatment. Conclusions HAE is diagnosed with significant time delays. Icatibant treatment is safe and effective.
FEATURES OF FOOD ANAPHYLAXIS IN CHILDREN ARISING FROM ALTERNATIVE ROUTES OF ALLERGEN INTAKE
Authors:   Research and Clinical Institute for Pediatrics named after Academician Yuri Veltischev of the Pirogov Russian National Research Medical University, Moscow, Russia, Esakova N.V. , Pampura A.N. ,   Research and Clinical Institute for Pediatrics named after Academician Yuri Veltischev of the Pirogov Russian National Research Medical University, Moscow, Russia
Published: March 2021

Objective of the study: to determine the frequency, spectrum of allergens, symptoms, and the severity of food anaphylaxis in children, occurred during transdermal or inhalation routes of allergen intake. Materials and methods of the research: the study included 110 children (a total of 227 episodes of anaphylaxis) aged 5 months to 18 years with a history of episodes of systemic reactions to food allergens that meet the clinical criteria for anaphylaxis. The collection of patient data was carried out by filling out a questionnaire. Allergological examination included determination of the concentration of SiGe antibodies to the putative triggers of food anaphylaxis (ImmunoCAP Phadia AB, Sweden). Results: 18 patients (16,4%) had systemic reactions after skin contact with an allergen or inhalation of trigger, the frequency of these reactions among the total number of episodes of anaphylaxis was 6,6% and 3,9%, respectively. The dominant triggers in the development of anaphylaxis during skin contact were fish/seafood allergens (46%) and cow's milk (33%), with inhalation intake – fish/seafood allergens (89%), the average level of sensitization to the latter was 100 kUA/l (95% CI: 22,59–100). The concentration of sIgE to triggers did not correlate with the severity of anaphylaxis (p>0,05, Rs=0,18). The proportion of severe reactions after inhaled injection of the trigger was slightly higher (33%), compared to anaphylaxis during skin contact (20%) (p>0,05). Patients with anaphylactic reactions at the same time both with transdermal and inhalation routes of allergen, were characterized by their severe course. Conclusion: food anaphylaxis in children resulting from skin contact with or inhalation of the trigger is most frequently developed in fish/seafood and is characterized by high sensitization to the allergen. Up to 1/3 of these systemic reactions are severe, and it is always important to consider the possibility of death.
PILOT STUDY OF TOLL-LIKE RECEPTOR LEVEL 2 AND THYMIC STROMAL LYMPHOPOIETIN IN CHILDREN WITH ATOPIC DERMATITIS
Authors:   Bashkir State Medical University, Ufa, Republic of Bashkortostan, Russia, Zaynullina O.N. , Pechkurov D.V. ,   Samara State Medical University, Samara, Russia, Hismatullina Z.R. ,   Bashkir State Medical University, Ufa, Republic of Bashkortostan, Russia, Gankovskaya L.V. ,   Pirogov Russian National Research Medical University, Moscow, Russia
Published: March 2021

The aim of this study is to assess the level of Toll-like receptor level 2 (TLR-2) and thymic stromal lymphopoietin (TSLP) in the blood serum of children and to establish the relationship between these parameters and the severity of clinical manifestations of atopic dermatitis (AD) and the degree of microbial contamination of the gut. Research materials and methods: 68 children aged from 3 months to 6 years with an AD and 31 conditionally healthy children of the corresponding age were examined. Determination of the level of TLR-2 and TSLP in blood serum was carried out by enzyme immunoassay, and the composition of the intestinal microflora was assessed by MALDITOF mass spectrometry. Results: in children with AD, the median serum TLR-2 level was 2,88 [1,60; 4,91] ng/ml, TSLP – 6,01 [1,11; 9,5] pg/ml, which is statistically significantly higher than in conventionally healthy children (p<0,0001). The highest levels of TLR-2 and TSLP were found in erythematoussquamous with lichenification of the AD form – Me=6,10 [3,55; 10,62] ng/ml and Ме=12,73 [5,81; 22,70] pg/ml respectively. In children with moderate severity of AD, TLR-2 and TSLP values were statistically significantly higher (p<0,0001) compared to mild AD (Me=5,58 [2,29; 8,18] ng/ml versus 0,72 [0,22; 2,02] ng/ml and 7,48 [4,25; 12,95] pg/ml versus 0,64 [0,39; 0,75] pg/ml, respectively). The level of TLR-2 and TSLP increased with the degree of intestinal dysbiosis: Me=6,27 [4,14; 8,02] ng/ml at grade 3 dysbiosis versus 1,25 [0,46; 2,15] ng/ml at the 1st degree of dysbiosis and 16,28 [10,01; 22,6] pg/ml at the 3rd degree of dysbiosis versus 0,63 [1,33; 0,70] pg/ml at the 1st degree of dysbiosis, respectively (p<0,0001). Results: the obtained data on the activation of TLR-2 and TSLP in AD emphasize the systemic nature of the inflammatory response; however, the causal relationship between the level of TLR-2 and TSLP and AD activity requires further verification.
IMPORTANCE OF ANTIBODIES TO 21-HYDROXYLASE FOR THE DIAGNOSIS, DIFFERENTIAL DIAGNOSIS AND PROGNOSIS OF AUTOIMMUNE CHRONIC PRIMARY ADRENAL INSUFFICIENCY
Authors:   National Medical Research Center for Endocrinology of the Ministry of Healthcare of Russia, Moscow, Russia, Sozaeva L.S. , Makazan N.V. ,   National Medical Research Center for Endocrinology of the Ministry of Healthcare of Russia, Moscow, Russia, Nikankina L.V. ,   National Medical Research Center for Endocrinology of the Ministry of Healthcare of Russia, Moscow, Russia, Malysheva N.M. ,   National Medical Research Center for Endocrinology of the Ministry of Healthcare of Russia, Moscow, Russia, Kareva M.A. ,   National Medical Research Center for Endocrinology of the Ministry of Healthcare of Russia, Moscow, Russia, Orlova E.M. ,   National Medical Research Center for Endocrinology of the Ministry of Healthcare of Russia, Moscow, Russia, Peterkova V.A. ,   National Medical Research Center for Endocrinology of the Ministry of Healthcare of Russia, Moscow, Russia
Published: March 2021

21-hydroxylase (21-OH) is the main antigen of the adrenal cortex, so the determination of antibodies (Ab) to 21-OH can help in the diagnosis and prognosis of chronic primary adrenal insufficiency (CPAI). Purpose of the study: evaluation of the relevance of Ab to 21-OH for the diagnosis and prediction of autoimmune CPAI. Materials and methods of research: the study consisted of three blocks: 1) assessment of the specificity and sensitivity, as well as the prognostic potential of Ab to 21-OH in patients with polyglandular autoimmune syndrome (APS) – individuals with APS type 1 with and without CPAI (n=106); 2) assessment of the dynamics of the level of Ab to 21-OH – patients with autoimmune CPAI were included (n=41); 3) assessment of the significance of Ab data for the differential diagnosis of various forms of CPAI, including patients with CPAI and APS type 1 exclusion (n=30). The study of Ab to 21-hydroxylase was performed using enzymelinked immunosorbent assay (BioVendor kits, Czech Republic). Results: statistically significant differences were obtained in the frequency of detection of Ab to 21-OH in patients with or without PCNI (p<0,001). The sensitivity of the method was 96%, specificity was 75%, a positive predictive value was 90%, and the negative predictive value was 89%. In 83% of patients, the level of Ab decreased with time (median size decreases – 20,4%/year). An inverse relationship was also found between the level of Ab and the duration of the course of CPAI (R=–0,460, p<0,001). In a group of 30 patients with CPAI and with exclusion of APS type 1, 21 were found to have Ab to 21-OH, only one of them had a monogenic non-autoimmune cause of CPAI (a mutation in the MC2R gene). Monogenic forms of CPAI were found in another 7 patients (mutations were found in the DAX1 and ABCD1 genes), among them an increase in Ab to 21-OH was not detected. Conclusion: determination of Ab to 21-OH is a specific and sensitive method for the diagnosis of autoimmune CPAI. An increase in Ab to 21-OH is a risk marker of autoimmune CPAI development.