The article presents modern ideas about the treatment of systemic lupus erythematosus (SLE). The details of the management of patients with SLE during immunosuppressive and genetically engineered therapy is given. The article also reflects the aspects of rehabilitation, prevention of exacerbations, and follow-up care of children with SLE. The criteria for assessing the quality of medical care for children with SLE are presented. The detailed information on systemic lupus erythematosus for patients with SLE and their parents is outlined specifically.

The review analyzes the role of chronic inflammatory diseases in the induction of endothelial dysfunction as a factor in the acceleration of atherosclerotic changes in the cardiovascular system. The importance of non-invasive techniques for evaluating endothelial dysfunction to detect preclinical atherosclerotic changes in children and adolescents with rheumatic was discussed. It is postulated that the detection of early signs of endothelial dysfunction in children and adolescents with rheumatic diseases, along with traditional risk factors, should become an integral part of the strategy for preventing adverse cardiovascular events in subsequent age periods.

Background. Retinal hemorrhages in infants are frequent pathologies, but their causes, clinical forms and functional outcomes are poorly studied. The study of hemorrhage localization in the eye structure, time frames of their resolution, and the long-term effects that may affect the development of vision is of particular interest.

Objective. Our aim was to analyze clinical forms, time frames of resolution and long-term clinical and functional outcomes of retinal hemorrhages in infants.

Methods. All children with retinal hemorrhages, who applied to a premature baby room in the MRI of ED n. a. Helmholtz at the age from 3 weeks to 3 months for 5 years (2011–2015), were examined using indirect ophthalmoscopy. In case of extensive lesions, the process dynamics was controlled by a pediatric digital retinal imaging system. Long-term clinical and functional outcomes of retinal hemorrhages were studied using ultrasound examination and spectral optical coherence tomography.

Results. Fundus hemorrhages were revealed in 108 (5.9%) of 1,825 infants on 142 eyes (34 children had bilateral hemorrhages). Hemorrhages were more frequent in children delivered vaginally (79 children, 73.2%), were unilateral (74 children, 63.5%), pre-retinal (108 eyes, 76.1%), of central localization (119 eyes, 83.8%). The time frames of hemorrhage resolution in 53 children (49.1%) were more than one month. Long-term outcomes of retinal hemorrhages were studied in 22 children (33 eyes) at the age of 2–5 years. Residual changes in the structure of the neuroepithelium and vitreoretinal interface with a decrement in visual acuity were noted in 7 children (9 eyes).

Conclusion. Retinal hemorrhages of newborns are characterized by clinical polymorphism, different time frames of resolution and outcomes, which requires a case follow-up.

Background. Etanercept is a biological drug most commonly used in patients with juvenile idiopathic arthritis (JIA). The results of its use are showed in local studies.

Objective. Our aim was to evaluate the efficacy and safety of the use of etanercept in children with non-systemic JIA, to determine the predictors of remission and the risk factors for the development of exacerbations.

Methods. In a retrospective cohort study, the results of etanercept treatment (remission, exacerbations, adverse events) in children with non-systemic JIA were analyzed. The minimum follow-up period was 6 months.

Results. The period of remission within 6–36 months occurred in 77/131 (58.8%), exacerbations developed in 18/129 (14.0%) patients. Predictors of achieving remission were the age of JIA onset < 8 years [relative risk (RR) 2.05; 95% confidence interval (CI) 1.27–3.23], the age of prescribing etanercept ≤ 10 years (RR 1.7, 95% CI 1.22–2.38), the time of the disease prior to etanercept prescription < 2.5 years (RR 2.4, 95% CI 1.4–4.4), the presence of HLA-B27 antigen (RR 2.15, 95% CI 0.98–4.75; p = 0.06). The risk of exacerbations was higher in children with polyarticular JIA (RR 2.7, 95% CI 0.9–8.2; p = 0.08), whereas methotrexate therapy reduced the risk of exacerbations (RR 0.32, 95% CI 0.1–1.15; p = 0.05). Etanercept was discontinued due to primary (improvement by the ACRpedi criteria after 3 months of therapy <30%) or secondary (loss of previously achieved ≥ 30% improvement) failure in 14/152 (9.2%) patients; de novo uveitis developed in 8/152 (5.3%) patients; reactions at the injection site — in 6/152 (4.0%) patients.

Conclusion. Therapy involving etanercept is more likely to induce remission in younger patients with JIA onset at the age of 8 years and a history of less than 2.5 years. A high risk of exacerbations was noted in patients with polyarticular JIA, and low one — in those receiving methotrexate as a part of combined therapy.

The article analyzes modern scientific data on the changes in breast milk composition during lactation. These changes affect both short-term (sleep, appetite) and long-term parameters (growth and development, temperament, behavior) with respect to the health of the child. This information will help to have a better idea of the benefits of breast milk and breastfeeding for a child and to strengthen a support and promotion of breastfeeding among mothers and health workers.

Background. Congenital syphilis is a severe intrauterine infection which, affecting most of the organs and systems of a child, can lead to his disability. However, it is extremely difficult to suspect congenital syphilis in a child in the absence of information about prior syphilis in his mother. The article presents a case of a monosymptomatic clinical picture of early congenital syphilis.

Description of the Clinical Case. At the age of 1 month, the child had hyperemia, isolated edema of the right forearm, and restriction of limb movements. He was examined by a pediatrician who suspected a fracture of the forearm bones. X-ray did not show any fracture. The situation was regarded as an allergic reaction; desensitizing therapy was prescribed during which the range of movements in the right arm slightly increased. At the age of 2 months, there was a decrease in the range of movements in the left arm. On admission, the condition was regarded as moderate one. No fever; no signs of intoxication. Skin and visible mucous membranes were not changed. Cardiopulmonary activity was satisfactory. The abdomen was palpable in all compartments, painless. The liver was enlarged 6 cm, of dense consistency. The spleen was enlarged 5 cm. Bowel and bladder functions were normal. Neurological status: the reaction to examination was adequate, emotional cry. Cranial nerves without pathology. Can see and hear. Restricted range of movements in the arms; passive movements were painful. Muscle tone in the arms was low, in the legs — closer to physiological one. Equal and brisk tendon reflexes from the arms and legs. Large fontanel 1.5[1]1.5 cm, not protruded. X-ray examination of the forearm bones showed a change in the structure of the epiphyses of both forearm bones that was common to syphilitic osteochondritis.

Conclusion. The article describes the complexity of diagnosis of early congenital syphilis in a child aged 2 months in the absence of a corresponding anamnesis in his mother. The need to rule out syphilitic infection in infants with motor impairments, born from unexamined mothers, has been emphasized.

Background. Non-compaction cardiomyopathy is a group of genetically heterogeneous, poorly studied myocardial diseases with a variety of clinical manifestations (from asymptomatic course to progressive systolic dysfunction with symptoms of chronic heart failure, arrhythmias, and thromboembolic complications). Considering the variety of genetic disorders associated with the development of noncompaction cardiomyopathy, genetic verification of the diagnosis is important for determining the prognosis and conducting genetic counselling of families with cases of the disease.

Description of the Clinical Case. The article presents two clinical observations of a severe course of non-compaction cardiomyopathy with remodeling of the heart cavities according to the dilated phenotype. In order to clarify the disease etiology, a molecular genetic study was conducted using the method of direct automatic sequencing with the analysis of targeted regions of 404 genes which mutations are described in hereditary diseases of the heart and blood vessels. After verifying the mutation (in the ACTC1 and MYBPC3 genes), we performed a search for the detected nucleotide substitution in the venous blood samples of parents and in one case — in the fetal DNA sample. The mode of inheritance has been determined; the probability of recurrence of the disease in siblings in subsequent pregnancies has been estimated.

Conclusion. The description of clinical cases shows the importance of genetic verification of the diagnosis in patients with non-compaction cardiomyopathy for determining the disease prognosis and developing an algorithm for monitoring relatives of a proband.

The article discusses issues related to the long-term administration of macrolide antibiotics for a bronchoectatic disorder of the pulmonary system in children. The relevant information about the results of long-term intake of macrolides in adults and children, the effective dose titration, possible toxic effects of therapy, including the development of cardiotoxic effect and ways to prevent it, has been enlightened in detail. It has been noted that a limited number of clinical studies involving children determine the need for studying the possibility to extrapolate adult treatment.