The article is dedicated to prevention of pneumococcal infections, which remain among the most common diseases in infants. The authors analyze international approaches to vaccination effectiveness assessment in light of predicting the expected effect of cohort immunization, given introduction of vaccination against this infection to the Russian National Immunization Calendar. The article presents a discussion of difficulties in interpreting epidemiological, immunological and clinical data in the absence of studies on direct
comparison of drugs. The authors demonstrate the need in multicomponent approach to immunization program effectiveness analysis, which would include optimization of epidemiological vigilance, adoption of the standard definition of a case, registration of results of prelicensure clinical trials of vaccines and postlicensure follow-up in similar groups of patients.

The article present current published data on the incidence rate, pathogenesis, clinical manifestations, diagnosis and treatment of
life-threatening conditions of the cardiovascular system at type I mucopolysaccharidosis (MPS). Mucopolysaccharidosis is a rare
genetically determined disease with chronic progressive course and wide range of clinical manifestations. Specific relevance of the
disease is justified by its severe incapacitating potential, development of a large number of complications of various organs and systems (up to failure), difficulty diagnosing and high risk of fatal outcomes as early as in childhood. Prevalence and severity of cardiovascular disorders in such patients is very high. Currently, there are two methods of treating MPS patients based on pathophysiology of this disease: transplantation of hemopoietic stem cells, which is used only in certain patients, and enzyme-replacement therapy. Early diagnosis and timely launched treatment help to improve condition of patients, attenuate the disease course, including termination of progression of cardiac disorders.

Abdominal pains are the most common manifestation of functional or organic pathology of the gastrointestinal tract; however, in girls
they are often caused by various diseases of pelvic organs. The article presents the main nosological forms of gynecological diseases in female children and adolescents, the main symptom whereof is abdominal pains. The authors discuss methods of differential diagnosis and demonstrate that failures of diagnosis and management and treatment tactics are caused by insufficient knowledge of anatomy and physiology of the reproductive system, neglect of gynecological anamnesis, untimely diagnosis of apoplexies of ovaries and complicated functional cysts, lack of oncological suspicion when encountering tumors and tumor-like ovarian neoplasms.

Prevalence of diseases associated with human papilloma virus (HPV) is high. The burden of this infection is so heavy all over the world, that the WHO recommended all countries to introduce vaccination against HPV into National Immunization Calendars. The article presents data on HPV prevalence in the Russian Federation in whole and the Khanty-Mansi Autonomous Okrug (Yugra) in particular, measures of timely identification of this disease and prevention of cervical cancer development. The program of school vaccination of female adolescent aged 12 years started operation in 2014 in Yugra. The author emphasizes the need in continuing with this program and introducing vaccination for male adolescents. According to the authors, introduction of preventive cohort vaccination against HPV into the regional and the National Calendars will reduce risk of development of oncological pathologies, such as cervical cancer and other HPV associated diseases, among the population.

Nowadays, parents are anxious about vaccination, as they fear both side effects of vaccines and large numbers of injections. The need in simultaneous administration of 4–5 injections became irrelevant due to development of combined vaccines; it resulted in improved vaccination coverage of children and higher trust of patients in vaccination in whole. The article presents experience of using a hexavaccine at the vaccinal prevention unit for children with health deviations of the Scientific Center of Children’s Health, as well as experience of concurrent immunization of children with a hexavaccine and a 10-valent vaccine against pneumococcal infection. The authors demonstrate that immunization with combined vaccines is safe for children, including premature infants, and reasonable when vaccination schedule has been broken. Moreover, it helps to reduce governmental expenses on immunization. Parents become more loyal vaccination due to the smaller number of injections and absence of adverse reactions.

Numerous scientific studies have proved an important role of microbiota in maintaining adequate functioning of the macroorganism and thrown light upon the various issues associated with functional disturbances. Thus, one of the most common causes of intestinal dysbiosis in children is antibiotic therapy, in the setting whereof antibiotic-associated diarrhea (AAD) associated with, but not limited to, pathological activity of Clostridium difficile byproducts — enterotoxin (toxin A), cytotoxin (toxin B) and intestinal peristalsis-inhibiting protein — may develop. However, there are only few published research data on AAD prevalence in children all over the world, which is why it appears impossible to assess the large scale of the problem. That is why it is absolutely necessary to continuously upgrade perception of the role of normal microbiota, etiological factors inducing pathological alterations thereof and approaches to correction of dysbiotic disorders by various medical specialists. Analysis of the current scientific literature demonstrated that AAD prevalence in children varies from 6.2 to 80% depending on the country. The most common (23%) cause of AAD is associated with amoxicillin/clavulanate intake. Moreover, there are data indicating that the AAD development risk is the highest in under-2 children.
At the same time, there are data on the protective effect of probiotics, especially of the complex drugs containing a balanced
combination of bifidus and lactic bacteria, on microbiota.

Infantile hemangiomas (IHs) are the most common tumors detectable in infants, including 5–10% of the neonates. Despite the spontaneous involution characteristic of the uncomplicated course of the disease, IHs require therapy in order to prevent disfigurement, functional disorders and cankering. Effective experience of treating IHs with non-selective-blocker propranol was described in 2008. More than 200 articles on effectiveness and safety of -blockers for IH treatment and assessment of potential side effects have been published since then. The article presents published data on the assessment of effectiveness of treating difficult to access hemangiomas with propranol on the basis of the applicable standards of medical care rendering. Special attention is given to the side effects induced by administration of this drug. According to the results of the published scientific works, -blockers appear to be rather effective for IH treatment and feature a high safety profile. However, it is necessary to continue clinical research and establish a sufficient evidence basis in order to definitively confirm this hypothesis.

Support of long-term breast feeding is a pressing issue of neonatology. It is known that the unique composition of breast milk ensures proper physical and neuropsychic development of infants, as it contains all the necessary nutrients in the sufficient amount and optimal proportion. The authors gave specific attention to provision of premature infants, especially with very low and extremely low birth weight, with breast milk. However, it is very difficult to launch and maintain breast feeding in this very category of patients. There are many reasons impeding adequate provision of premature infants with breast milk. The main problem on the part of the mother is hypogalactia, which may be caused by preterm labor stress, lack of confidence in successful lactation, temporary medical contraindications and, therefore, deviant formation of the lactation dominant, motivation towards prolonged breast feeding etc. On the part of the child: severe condition, no or weak sucking reflex, often — prolonged parenteral and tube feeding, need in supplementary feeding. The article presents published data on various methods of maintaining breast feeding at the stage of hospital developmental care of premature infants and experience of breast feeding support accumulated at the Scientific Center of Children’s Health, which proves that simultaneous support and follow-up of the child’s mother and her family in whole by several specialists (neonatologist/pediatrician, psychologist, breast physician, dietician and recreation therapist) not only at the stages of labor and development care, but also after discharge from hospital are required to ensure rational and prolonged breast feeding of premature infants and normal growth and development thereof.

This literature review is dedicated to the issue of clinical and laboratory diagnosis of acute pharyngitis in children and adolescents. Relevance of the topic is justified by omnipresent (up to 70% of patients with acute pharyngitis) unreasonable prescription of antibacterial drugs. Differential diagnosis of acute pharyngitis is aimed at identifying patients with the infection induced by group A-hemolytic streptococcus requiring antimicrobial therapy. The article presents current recommendations on microbial diagnosis of streptococcal tonsillitis and a potential of using express tests for rapid diagnosis verification.

Aim. Hereditary spherocytosis (HS) is the most commonly encountered erythrocyte membranopathy. Frequency of occurrence of the disease makes one case per 2000−5000 newborns. Hereditary spherocytosis often causes a complex of clinical signs, including hemolytic crises in patients. At the same time many patients have asymptomatic HS. Differential diagnosis of HS is quite complex and in modern workload conditions the clinical doctors need a simpler diagnostics procedure. Patients and methods. Participants included 13 adults with verified hereditary spheroсytosis and 42 children with identified hereditary spherocytosis, 311 adults without hematological disorders, 42 children without hematological disorders. Verification of hereditary spherocytosis diagnosis was carried out using flow cytometry test (eosine-5 maleimid-binding), Deich`s method of determination of erythrocyte osmotic resistance and Sodium Dodecyl Sulfate-Poly Acrylamide Gel Electrophoresis. In this study we have assessed diagnostic value of hematological parameters provided by the hematological analyzer Beckman Coulter Cellular Analysis System DxH800 for identifying the degree of erythropoiesis disorder in patients with hereditary spherocytosis at the stage of reticulocytes maturation. According to our data, the ratio RET/IRF and calculated parameter MCV-MSCV can be used as the screening tests for hereditary spherocytosis. Results. Evaluation of the erythrocytes and reticulocytes parameters at the hematological analyzer identified the significant difference in estimate index MCV-MSCV (p < 0,0001, sensitivity 100%, specificity 100%, area under the ROC-curve 1,0) and RET/IRF (p < 0,0001, sensitivity 96,3%, specificity 94,1, area under the ROC-curve 0,97) between group of patients with HS and control group. We also evaluated the usability of eosine-5 maleimide binding in flow cytometry for verification of this membranopathy. For unify the test results we offer to use estimate indicator S (sample`s MFI / control`s mean MFI), cut level for positive cases of hereditary spherocytosis S < 0,84 (p = 0,0001, sensitivity 98,2%, specificity 99,2%, area under the ROC-curve 0,99). Conclusion. We recommend the hematological analyzer evaluation as the screening option for the identification of HS in patients and determine the estimated parameters for the values of the patients MCV-MSCV and RET/IRF. The most informative verifying test to prove hereditary spherocytosis is the flow cytometry test using eosine-5 maleimid. It is the laboratory test that proves a high degree of sensitivity and specificity for hereditary spherocytosis. Sodium Dodecyl Sulfate-PolyAcrylamide Gel Electrophoresis of red blood cells membranes proteins is useful for specify molecular deficiency in each hereditary spherocytosis case.

Relevance. Vertebral canal revision and decompression in patients with kyphotic deformities of thoracic and lumbar spine is performed not only in the setting of severe neurological symptoms, but also in the presence of risk of neurological deficiency associated with vertebral canal stenosis at the peak of vertebral deformity. There is no common method of measuring vertebral canal loss at the moment; this significantly complicates identification of indications to vertebral canal revision and decompression. The study was aimed at identifying indications to vertebral canal revision and decompression at operative treatment of long kyphotic deformities of thoracic and lumbar spine based on the method of measuring the relative vertebral canal’s stenotic area at the peak of deformity. Patients and methods. Operative treatment was performed in 108 study subjects with kyphotic deformities of thoracic and lumbar spine. The authors developed an original method of measuring the relative vertebral canal’s stenotic area in order to identify indications to vertebral canal revision and decompression. Measurement results were compared with vertebral canal stenosis thresholds for specific localizations. Results. Vertebral canal stenosis was revealed in 43 out of 108 patients (39.81%). Conclusion. The authors developed the first highly accurate method of measuring the relative vertebral canal’s stenotic area, which takes into consideration not only the anteroposterior dimension of the vertebral canal, but also narrowing thereof along its cross-sectional area. Exceedance of the vertebral canal loss threshold is an absolute indication to vertebral canal revision and decompression.

Congenital muscular dystrophies (CMDs) are an extremely heterogenous group of neuromuscular diseases. The article presents the general information on clinical and pathogenetic aspects of CMD diagnosis with emphasis on one of the most common forms of nervous system disturbances — merosin-deficient congenital muscular dystrophy (CMD1A). The authors describe clinical pattern of CMD1A, pathogenesis, epidemiological data, approaches to instrumental, morphological and molecular genetic diagnosis, as well as symptomatic treatment and prenatal diagnosis in detail. As long as there are few genetically confirmed clinical cases of CMD1A described in the publications written in Russian, the authors present their 3-year-long follow-up of a child with genetically confirmed diagnosis of CMD1A (mutations c.2049_2050delAG and c.6993-2A > C of gene LAMA2 [compound heterozygous state]). The article presents a detailed discussion of the described case, comparison with results of recent foreign and Russian observational studies of CMD1A in children. It also contains information on differential diagnosis of CMD1A in the structure of other congenital myodystrophies and optimization of molecular genetic diagnosis.

This review presents current data on the potential of enteroscopy — a new dynamically developing method of diagnosing jejunal diseases. Currently, two complementary methods of enteroscopy are being used — video capsule endoscopy and balloon enteroscopy. The article presents a discussion of optical systems for enteroscopy, indications for and advantages and disadvantages of methods of enteroscopy. Video capsule endoscopy is intended for initial visual assessment of jejunal mucosal condition. Depending on the position of the viewing system within the capsule, specialists distinguish between frontal and lateral systems. Balloon enteroscopy complements trials in terms of microsurgical aspects (biopsy sampling, elimination of polyps, extraction of foreign bodies, including the video capsule if it gets trapped in the stenotic portion of the intestinal lumen).

Diagnosis of hearing level in small children with conductive hearing loss associated with congenital craniofacial abnormalities, particularly with agenesis of external ear and external auditory meatus is a pressing issue. Conventional methods of assessing hearing in the first years of life, i. e. registration of brainstem auditory evoked responses to acoustic stimuli in the event of air conduction, does not give an indication of the auditory analyzer’s condition due to potential conductive hearing loss in these patients. This study was aimed at assessing potential of diagnosing the auditory analyzer’s function with registering brainstem auditory evoked responses (BAERs) to acoustic stimuli transmitted by means of a bone vibrator. The study involved 17 children aged 3–10 years with normal hearing. We compared parameters of registering brainstem auditory evoked responses (peak V) depending on the type of stimulus transmission (air/bone) in children with normal hearing. The data on thresholds of the BAERs registered to acoustic stimuli in the event of air and bone conduction obtained in this study are comparable; hearing thresholds in the event of acoustic stimulation by means of a bone vibrator correlates with the results of the BAERs registered to the stimuli transmitted by means of air conduction earphones (r = 0.9). High correlation of thresholds of BAERs to the stimuli transmitted by means of a bone vibrator with thresholds of BAERs registered when air conduction earphones were used helps to assess auditory analyzer’s condition in patients with any form of conductive hearing loss.

The article presents a case of late diagnosis of cutaneomucosal lymphonodular syndrome (Kawasaki syndrome). The child featured fever, mucosal lesion (conjunctivitis, stomatitis), rash, thick edemas on arms and feet, arthritis and coronaritis. Initial therapy proved ineffective. Pathogenetic therapy, which proved to be rather effective, was prescribed after diagnosis was confirmed. The authors present a case of successful use of normal human immunoglobulin for intravenous injections in the dose of 2 g/kg of body weight per course in combination with acetylsalicylic acid in the dose of 80 mg/kg per day. Body temperature decreased down to subfebrile figures and foot pain attenuated as early as after 1 day of treatment. Fever, rash, stomatitis and conjunctivitis terminated, edemas of limbs and arthritic manifestations attenuated considerably and laboratory parameters of disease activity normalized after 1 week (ESR and CRP). Inflammation of coronary arteries terminated after 3 weeks. No adverse events in the setting of immunoglobulin therapy were observed.

The article presents a short review of the current notion of the leading role methotrexate in treatment of most clinical variants of
juvenile idiopathic arthritis. The authors demonstrate that methotrexate is a “gold standard” of antirheumatic therapy and the key disease-modifying drug of pediatric rheumatology. The emphasis is laid on safety profile and advantages of parenteral administration thereof. The article presents follow-up of a clinical case involving use of dosage form of methotrexate in the dose of 15 mg/m per week for subcutaneous administration in a patient with polyarticular juvenile idiopathic arthritis featuring insufficient effectiveness and low tolerance of the oral drug. Prescription of methotrexate for subcutaneous administration helped to terminate progression of the disease and pain syndrome and improve function of the affected joints and the patient’s quality of life.

The article presents a clinical case of high effectiveness of adalimumab — human monoclonal antibodies to tumor necrosis factor — in a patient with juvenile ankylosing spondyloarthritis refractory to therapy with classic immunosuppressive drugs and secondary to virus hepatitis B in the integrative phase. Joint pains and intensity of exudative alterations in interphalangeal and wrist joints attenuated as early as after as the first adalimumab injection; the authors achieved a 50% improvement in indices BASDAI (2.4) and ASDAI (1.1). Acute inflammatory alterations terminated, motion range of the affected joints completely recovered, general laboratory parameters of disease activity (erythrocyte sedimentation rate, C-reactive protein and white blood cell count) normalized and inactive disease stage was registered after 6 months. Adverse events or virus hepatitis B exacerbations in the setting of adalimumab therapy were not observed. No osteochondral destruction progression was observed at computed and magnetic resonance imaging of joints 6 months after the therapy beginning. Emotional condition and quality of life of the child and his family improved considerably.

The article is dedicated to a pressing issue of pediatrics — diagnosis and treatment of an autosomal-recessive disease from the group of organic acidemias — methylmalonic acidemia. Despite the attained progress in diagnosis of this disease based on the molecular genetic research and an expanding arsenal of therapeutic and nutritive instruments, results of treating children remain unsatisfactory. Metabolic crises in patients with organic acid metabolic disorders are accompanied by a severe affection of the central nervous system and associated with high mortality (up to 50% and more depending on the genetic subtype of the disease). Development of a metabolic crisis requires emergency medical care, including a wide range of therapeutic approaches, nutritional support recommendations and use of replacement renal technologies. The article present current data on pathophysiology, clinical laboratory description of the disease and metabolic crises, as well as the acute methylmalonic acidemia attack (crisis) management protocol, use of which helps to achieve a significant increase in survivability of pediatric patients.

Relevance. Pancreatic pathology is often observed in children, especially in adolescents. Timely adequate therapy helps to reduce duration of hospitalization and, possibly, prevent future relapses. The study was aimed at examining peculiarities of the course of chronic pancreatitis in children and determine effectiveness of comprehensive treatment using trimebutine maleate. Patients and methods. 100 children aged 7–18 years with chronic pancreatitis were examined. The control group was comprised of 30 virtually healthy children of the similar age. Results. According to the anamnestic data, eating disorder (68.0%), pancreatic exacerbation of chronic noncalculolis cholecystitis or sphincter of Oddi dysfunction (65.0 and 50.0%, respectively) are significant risk factors of chronic pancreatitis in children; obesity (40.0%) and hypodynamia (35.0%) are less common risk factors. The main clinical manifestations of chronic pancreatitis are “girth half” spastic abdominal pain attacks (78.0%), nausea regardless of food intake (65.0%) and bile emesis not attenuating the patient’s condition (45.0%). The study established moderate manifestations of exocrine pancreatic failure. The vasointestinal polypeptide level was low at erosive lesion of gastroduodenal zone’s mucosa (2.15 ± 0.3 ng/l) and cardiac esophageal sphincter failure (2.29 ± 0.2 ng/l). Regression of clinical manifestations was more rapid in the group of children taking a motility regulator — trimebutine maleate — than in the group of children, who did not take this drug. Conclusion. It is necessary to identify and control risk factors in order to prevent development of chronic pancreatitis. Introduction of a motility regulator — trimebutine maleate — into the comprehensive therapeutic program is effective and helps to reduce duration of hospitalization.

The issues of diagnosing and treating urinary tract infections and their role in development of renal injury are being actively discussed by scientists and practicing pediatricians. The article presents the most recent data on etiological factors, pathogenesis and clinical manifestations of this disease. It provides recommendations on diagnosis and management of patients depending on their age. The article presents a discussion of antibacterial therapy course duration and indications for anti-relapse treatment. The study demonstrates that intravenous antibacterial therapy must be launched immediately in neonates in the event of pyretic fever; empirical antibacterial therapy must be launched immediately in older children after diagnosis of the urinary tract infection has been confirmed; subsequently, treatment ought to be corrected depending on the results of a bacteriological trial, sensitivity to antibiotics and effectiveness of the prescribed antibiotic. Along with normalization of urination rhythm and water intake schedule, antibacterial preventive therapy might be considered, if effective, in the event of recurrent nature of the urinary tract infection.

Inflammation of palatine tonsils (acute and chronic tonsillitis) is a pressing issue of pediatrics. Several pathogenetic links of chronic
tonsillitis development have not been identified yet despite high prevalence of the disease. The article presents current data on
formation of biofilms at chronic tonsillites and immunological peculiarities of the Pirogoff-Waldeyer’s tonsillar ring’s lymphoid tissue in children. Authors emphasize the fact that prescription of antibacterial therapy at inflammation of palatine tonsils must be clearly justified due to the risk of development of microflora resistance, as well as of disturbed natural balance of mouth cavity microbiota. Differential diagnosis of tonsillitis and rare Marshall syndrome is important for determining appropriate management tactics. The authors demonstrated effectiveness of using a complex phytopreparation for acute and chronic tonsillites in children.