Relevance. The hypoplastic left-heart syndrome at congenital heart disease has frequency of 261 cases out of 10,000 newborns. Children with hypoplastic left-heart syndrome can have comorbid congenital anomalies and acquired diseases that require treatment and are associated with high mortality risk.

Description of a clinical case. Female patient, 6 years 4 months, with the hypoplastic left-heart syndrome was admitted to our hospital with the diagnosis «Sensorineural hearing loss, speech delay» for performing of cochlear implantation. The council of physicians was convoked before the surgery to coordinate patient management. Members of surgical, anesthesiology, laboratory and instrumental examination departments were involved into preoperative assessment and planning due to the high level of surgical and anesthetic risk. The patient was dismissed from hospital on the 10th day after cochlear implantation. The patient has undergone the course of auditory-verbal therapy a month after. The child was dismissed wit state improvement.

Conclusion. The example of successfully performed surgery in the patient with hypoplastic left-heart syndrome and such severe comorbid pathology as sensorineural hearing loss is presented. Complete physical examination of the child in preoperative period and adequate preparation for the surgery were the key factors for patient successful management and further rehabilitation.

Relevance. Omalizumab is recommended for treatment of severe forms of recurrent urticaria in children since 12 years old. Though the Omalizumab efficiency and safety in children with recurrent urticaria have not been studied specifically.

Objective. To estimate results of the therapy including Omalizumab in adolescents with recurrent urticaria.

Methods. We have studied clinical records of day hospital patients aged from 12 to 17 with recurrent urticaria uncontrolled with second-generation antihistamines and/or immunosuppressive drugs at least for a month (in standard or higher dose), but receiving Omalizumab (300 mg once in 4 weeks subcutaneously). The main result of this therapy is disease control (urticaria activity score over 7 days, UAS7, equal to zero) at 3 and 6 months of therapy. Additional outcomes of therapy: number of significant recrudescence (use of glucocorticosteroids or emergency hospitalization) at 6th month of therapy and in 6 and 12 months after its end; end of concomitant treatment (antihistamines and/or immunosuppressive drugs) at 3 and 6 months and genetically engineered biological agent (GEBD); remission (UAS7 = 0) after 6 and 12 months after GEBD cessation; adverse effects of Omalizumab therapy (any medical cases connected with GEBD).

Results. The disease control at 3 months of treatment was reached in 12 (67%) patients out of 18 children with recurrent urticaria, at 6 months — at 13 (72%) patients. During Omalizumab therapy and at 6 months after therapy cessation there were no significant recrudescence, at 12 months after therapy cessation — at 1 (6%) adolescent. The concomitant treatment was ended after 3 months at 3 (17%) patients, after 6 months — at 10 (56%) patients. Remission was sustained at 11 (61%) out of 18 patients at 6 months and at 9 (60%) out of 15 patients at 12 months after the therapy end. Adverse effects of Omalizumab have not been noted.

Conclusion. Addition of Omalizumab to the therapy in adolescents with uncontrolled recurrent urticaria let us reach remission of disease in most cases by 6 months of treatment.

Rhythm and conduction disorders of the heart occupy one of the leading places in the structure of cardiovascular pathology in children. Supraventricular tachycardias means tachyarrhythmias, caused by abnormal myocardial excitation with the source of rhythm localization above the His bundle bifurcation — in the atria, atrioventricular junction (node), and also arrhythmias with circulation of the excitation wave between the atria and the ventricles with additional atrial compounds. The team of authors presents clinical recommendations developed on the principles of evidence-based medicine, including all stages of diagnosis and treatment of children with supraventricular tachycardias. The use of recommendations in clinical practice allows to selecte the best strategy for diagnosis and treatment of supraventricular tachycardia in a particular patient.

The clinical recommendations on management of children with Gitelman syndrome developed by the experts of the Union of pediatricians of Russia are presented in this article. Gitelman syndrome is a dysfunction of distal renal tubules with further development of hypomagnesemia, hypocalciuria and secondary aldosteronism which determine hypokalemia and metabolic alkalosis. Features of epidemiology, etiology and pathogenesis, disease progression, differential diagnostics and evidence-based treatment are presented.

The clinical recommendations on management of children with Liddle syndrome which is characterized by severe hypertension along with low activity levels of renin and aldosterone in blood plasma, hypokalemia and metabolic alkalosis, were developed by the experts of the Union of pediatricians of Russia. Aspects of epidemiology, etiology and pathogenesis, disease progression, differential diagnostics and evidence-based treatment are presented.

The priority of breastfeeding in the care of premature infants is not in doubt. For preterm babies, breastfeeding provides a dual effect, reducing the risk of both immediate and long-term effects associated with prematurity. In this regard, extraordinary efforts are being made at the international level to promote and support breast-feeding of newborns, including in intensive care units. Mother’s own milk is the first choice for feeding a premature baby. In recent years, the problems of quality and safety of expressed breast milk — both pasteurized and fresh — for newborn patients of intensive care units are discussed. The authors point out that the treatment procedures and storage conditions of expressed breast milk differ significantly; there are no common criteria for its microbiological safety, which limits the use of this unique product, especially in premature infants. In addition to the risk of bacterial infection, the risk of infection with cytomegalovirus (CMV) when using fresh milk from a CMV — seropositive mother is discussed for premature infants. The review examines the criteria for the risk of CMV infection in children and indications for selective treatment of breast milk for inactivation of a probable pathogen.

Background. As has been demonstrated by Russian and foreign experience the implementation of combined vaccines within national immunization schedule (NIS) reduces the overall number of injections, enhances vaccination commitment, and as consequence leads to higher vaccination coverage.

Objective.Pharmacoeconomic analysis of planned immunization of children aged from 3 to 18 months with combined diphtheria, pertussis, tetanus, poliomyelitis, haemophilus influenza b vaccine (DTaP/IPV/Hib) within Russian Federation NIS.

Methods. Modelling; estimation economic method: cost analysis, budget impact analysis. Results. Only health system costs on four planned pentavalent vaccines requires application of 1 013 955 985 roubles. From the government and society perspective the change-over to combined vaccine allows to retrench 1 825 385 700 roubles for children aged from 3 to 18 months for all horizon period.

Conclusion. According to budget impact analysis such change-over with controlling only health system costs will require extra expenses. According to government and society perspective (that means social and other expenditures not covered with health system budget) the implementation of scheme of 4-time combined vaccine injections for all cohort allows to reduce cost loading related to infectious disease burden. Present analysis indicated the advisability of pentavalent vaccine implementation for all children within Russian national immunization schedule.