The chronic diarrhea is pretty relevant problem in children due to its incidence, wide range of causes, heterogeneity of pathogenetic mechanisms and clinical manifestations. Meantime there is no common algorithm for screening of such patients with chronic diarrhea. This usually aggravates the diagnosis, timely treatment, and finally decreases patient quality of life. This review provides updated data on etiology, pathogenesis, clinical manifestations and diagnostic approaches of chronic diarrhea in children. Issues of structure and management of different diseases manifested in children with chronic diarrhea are considered.

Background. Upper limb prosthetics can be required in many conditions, and congenital as well. The situation in Russian healthcare system with rehabilitation of children with upper limb prosthetics has not been studied before.

Objective. The aim of the study was to study current situation and challenges in rehabilitation of children in need of upper limb prosthetics due to congenital diseases.

Methods. The simultaneous qualitative research was performed — in depth semi-structured interview of parents whose children had got active prosthetics of upper limbs due to congenital diseases in the period from October 2017 to April 2018. There were 8 open-ended questions and 13 closed-ended question in the interview about availability and timeliness of rehabilitation, complexity and personality of the approach, observance of the phasing and continuity principles, and also involvement into rehabilitation process. The interview was performed after obtaining of the active prosthetic.

Results. 20 parents of children aged from 2 to 17 years from 8 regions of Russian Federation have undergone the survey. Only 75% of children undergone rehabilitation treatment over the years (before prosthetic positioning), and 93% of them only during the first phase (in health resort institution). Active prosthetic was delayed in 90% of cases. The complex and individual approach to rehabilitation was disrupted in 75% of cases. The child and his family were not actively involved in rehabilitation process in all cases. All respondents have mentioned low availability of rehabilitation. Most of the parents (80%) mentioned as well that they have to pay their own money to cover all the expenses.

Conclusion. The interview of parents whose children are in need of active prosthetic revealed that one in four child did not get rehabilitation treatment. In cases when rehabilitation had been carried out many imperfection in its organization were identified. Low awareness about rehabilitation itself among parents and low interest in changing of the existing rehabilitation system were revealed.

Background. Patients with juvenile idiopathic arthritis (JIA) can have low levels of antibodies to vaccine antigens due to immunologic features of the main disease, disruptions in vaccination schedule and immunosuppressive drugs administration

Objective. The aim of the study was to examine the status of postvaccinal immunity and determine the factors associated with preservation of protective level of antibodies in patients with JIA.

Methods. This cross-sectional study included patients with JIA at the age from 2 to 17 years old vaccinated under the age of two (before JIA) against measles, rubella, parotitis, hepatitis B and diphtheria. Levels of IgG to vaccine antigens were measured by enzyme immunoassay. The minimum protective level of anti-measles IgG was esteemed as 0.18 IU/ml, antibodies to rubella — 10 IU/ml, for parotitis — COI > 1.0, for hepatitis B — 10 mIU/ml, antibodies to diphtheria — 0.09 IU/ml.

Results. The study included 90 patients with JIA (71% of girls) at the age (median) 11.3 (7.5; 14.9) years. The age of JIA manifestation was 6.0 (4.0; 8.0) years, disease duration — 4.0 (2.0; 7.3) years. Glucocorticosteroids administration in anamnesis or at study entry was recorded in 24/88 (27%) patients, methotrexate — 81/88 (92%), genetically engineered biologic drugs — 54/89 (61%). Protective level of antibodies to measles virus was revealed in 45 (50%) children with JIA, to rubella virus — in 88 (98%), to parotitis — in 68 (76%), to hepatitis B — in 49 (54%), to diphtherial anatoxin — in 45 (50%). The decrease of postvaccinal immunity level was associated with JIA duration and glucocorticosteroids administration (against diphtheria) duration, as well as drop-out immunization (against measles).

Conclusion. Major part of children with JIA have no protection against measles, parotitis, hepatitis B or diphtheria. High risk of progression of such vaccine-preventable diseases in these children demands development of individual programs of immunization.

Background. The PEWS (Pediatric Early Warning Score) is always used for prognosis of critical incidents in children at hospital. However, recording of various vital deviations may be advantageous. In this regard, it is relevant to study their prognostic value for risk of mortality estimation.

Objective. The aim of the study was to estimate the frequency of vitals deviations from reference ranges in patients with low and high risk of mortality.

Methods. We have studied medical records of patients aged from 1 month to 17 years old hospitalized in intensive care unit (ICU). Mortality prediction was counted with PIM3 score. Low and high risk of mortality groups were created due to the results of cluster analysis. The low risk group was made up of patients with mortality prediction rate < 37%, high risk group > 57%. Heart rate, respiratory rate, oxygen saturation in arterial blood, body temperature, systolic and diastolic blood pressure and deviations of all these values from reference ranges on presentation to ICU and further daily (every 2 hours) during the first 5 days (or less in case if the patient was transferred from the ICU earlier) were analysed.

Results. Medical records of 66 patients (including 10 (15%) with a high risk of mortality) have been studied. The low and high risk groups were comparable by sex, age, spectrum of diseases, length of patient stay in the ICU. The lethal outcome in ICU was registered in two cases: both patients with high risk of mortality (> 50%) due to PIM3 score. The frequency of vitals deviations from reference ranges on presentation to ICU and in the following 5 days (except for HR on the first day) in risk groups did not differ statistically. Conclusion. Registration of only vitals deviations from reference ranges in the child during first 5 days in ICU does not allow us to predict high risk of mortality measured by PIM3 score.

Background. Hypophosphatasia is rare hereditary disease caused by deficiency of the tissue-nonspecific alkaline phosphatase isozyme. It manifests with bone and teeth mineralisation defects, electrolyte imbalance, respiratory disorders, convulsive syndrome, physical developmental delay, nephrocalcinosis. The rarity of this disease, clinical polymorphism, non-specificity of complains and signs are the major reasons of hypophosphatasia late diagnosis. The enzyme replacement therapy with recombinant alkaline phosphatase (asfotase alfa) can be used for treatment of severe forms of disease.

Clinical Case Description. The girl (1 y 4 m) was routinely admitted with complains of physical developmental delay and psychomotor retardation, deformation of lower limbs, chest, gait abnormality, teeth loss and with diagnosis «protein-calorie malnutrition». Rickets-like changes in skeleton, myopathic syndrome, early normal teeth loss, hepatomegaly were revealed. Reduced activity of alkaline phosphatase in blood serum (33 u/l; reference range 156–369 u/l) was revealed. The infantile hypophosphatasia has been diagnosed. Due to molecular genetic testing of ALPL gene we revealed pathogenic variants c.526G>A (p.Ala176Thr) and c.1375G>A (p.Val459Met) in compound heterozygous state. The asphotase alpha therapy was initiated at the age of 1 y 10 m, the dose was 2 mg/kg subcutaneously 3 times a week. The results of 6 months of the therapy are the following: significant increase in the activity of alkaline phosphatase (maximum 4400 u/l), body weight (+ 2 kg), growth (+ 6 cm), reduction of bone deformation, normalisation of muscle tone and gait, exercise tolerance. The patient tolerated the drug administration well. Rarely there were hyperemia zones up to 4 cm in diameter with moderate induration at the injection site but they spontaneous disappeared in 2–3 days though.

Conclusion. Patients with rickets-like diseases and low alkaline phosphatase activity requires performing of molecular genetic testing to confirm hypophosphatasia. Timely diagnosis and early initiation of enzyme replacement therapy can significantly improve the quality of life of children with hypophosphatasia.

Background. Gravitational erythema is rare pathologic condition that is characterized by abnormal vascular response on venous pressure changes. It is benign disease, and it is included in differential diagnosis of vasculitis, vasculopathies, neuropathies and does not require treatment. Compression garments may be required in some cases.

Clinical Case Description. The case of gravitational erythema in 13 years old patient of is presented. The disease was presented with erythematous mosaic rash on the lower limbs skin. It has appeared in 4-6 minutes after verticalisation and mostly on the lower legs. The rash was accompanied with some bluish spots disappearing after pressure. The elements of rush were itchy, there was pain in distal parts of the feet. The rash was reversed after walking and changing of limb position. Positive compression test was mentioned.

Conclusion. Differential diagnosis of gravitational erythema with other clinically similar conditions remains relevant. The rare diagnosis of gravitational erythema and low awareness of doctors about it cause misdiagnosis, costly continuous examination and ineffective treatment.

Background. Pulmonary cysts is relatively rare disease for pediatrics and it is 3.5-5.5% of all non-specific lung diseases. There are congenital and acquired pulmonary cysts. Pulmonary cysts in right lung are slightly more common than in the left one. Understanding the nature of cavitary lesion is one of the most difficult problem in pulmonology. Basic diagnostics of cysts is radiological methods.

Clinical Case Description. The child (11 y 10 m) was diagnosed with pulmonary cyst in the right lung with local pneumothorax and alongside with congenital developmental brain malformation and symptomatic epilepsy. The first episode of severe acute right-sided pneumonia was diagnosed at the age of 11 y 7 m, he was on hospital stay at place of residence for two weeks. The big cavitary lesion was revealed in right lung. The pulmonary cyst was suspected. One month after the child was hospitalized again in the in intensive care unit with worsening course due to acute respiratory infection. Taking into account big cavitary lesion in right lung he was sent to Moscow for further examination and treatment. The diagnosis of right pulmonary cyst was confirmed. The thoracoscopic lobectomy was performed at the age of 11 y 10 m. The laparoscopic gastrostomy and fundoplication were performed due to gastroesophageal reflux and questionable hiatal hernia one month later. Post-surgery period was without any complications. Spontaneous stool was on the second day, activization of the patient was on the third day. According to check-up X-rays: no diaphragm defects, it is located correctly. The patient was discharged stable to the place of residence for further observation by pediatrician and surgeon.

Conclusion. Right pulmonary cyst in the child remained unrecognized for a long time due to mild symptoms. It was revealed only when the child was admitted for the hospital treatment at the place of residence with acute bilateral pneumonia. Timely diagnosis and treatment of such lung pathology are important both for prognosis the disease course and sustaining of patient's quality of life.

Foreign practices of cost-effectiveness analysis of revaccination against pertussis in preschool children, teenagers and adults are presented. The economic viability of the second revaccination against pertussis at preschool/early-school age is shown: it can reduce both direct and indirect expenses associated with pertussis not only in revaccinated groups but also in infants due to increasing of population immunity. International guidelines on the implementation of revaccination against pertussis through replacing of exhausted diphtheria tetanus modified vaccine on DTaP within the framework of the national vaccination schedule.

Pyodermas are the most frequent reason for visiting dermatologists among children. The immunity to development of pyodermas is based on skin barrier the part of which is cutaneous microbiome. The microbiome composition is unique and depends on age, localization on various body regions, environment. The change of its qualitative and quantitative composition leads to the development of purulent dermal diseases characterized by clinical diversity and severe course and development of complications (without adequate treatment). In this regard the choice of the correct therapy remains relevant. The results of studies covering the effectiveness of various topic antibacterial agents are analysed. Practical aspects of pyodermia therapy in children using local therapy are illustrated.

Mucopolysaccharidosis type II (MPS II), or Hunter syndrome, is the hereditary lysosomal storage disease caused by pathological variants in IDS gene. Such variants lead to iduronate-2-sulfatase enzyme deficiency and glycosaminoglycan catabolism disorder. Major clinical signs are central nervous system lesion, disorders of musculoskeletal system, cardiovascular and respiratory systems pathologies, hepatosplenomegaly, hearing impairment. Enzyme replacement therapy (ERT) makes it possible to adjust metabolic processes in lysosomes of many organs and tissues, to improve clinical signs due to partial restoring of the damaged enzyme function. Cardiovascular pathology is the main cause of death in patients with MPS. In this regard we have studied efficiency of ERT with idursulfase and its effects on the cardiovascular system in 55 patients with MPS II. It has been shown that ERT started from an early age can significantly improve children's condition, reduce or event prevent cardiac involvement. Treatment gaps from 1 to 7 months due to economic or organizational factors in 12 patients caused worsening course of the disease.