The article presents the results of historical and medical research reflecting the issues of reforming children’s health care after the World War II. The aim of the study is to present the problems of reforming children’s health care in the USSR in the post-war decade (1946–1956). The study relevance is defined by necessity to examine the historical experience of reforming children’s health care during the most difficult for our country post-war period so we can learn crucial lessons from the past and use them further for the successful implementation of the federal project “Development of children’s health care, including the creation of modern infrastructure for medical care provision for children”. Published and unpublished documents of the USSR Ministry of Health and the USSR Academy of Medical Sciences, stored in the State Archive of the Russian Federation, as well as publications from medical journals, scientific collections, monographs were studied to cover this issue. The analysis of this study has shown that the most significant part in the state health reform started in the first post-war years was the merger of hospitals and clinics for adults, maternity hospitals with women’s consultations, children’s hospitals with children’s consultations and outpatients’ clinics. The post-war reform has contributed to the improvement of the quality of inpatient and outpatient medical care, to the increasing role of the hospital as the organizing and leading center of all united facilities. The integration of children’s hospitals with consultations and outpatients’ clinic, as well as the organization of the primary pediatric care according to the system of medical and preventive care provision for children aged from birth to 16 years by single pediatrician, have shown succession in the work of primary care doctors and hospitals. The increase in the health workers qualifications, the development of specialized types of medical care, the improvement in the quality of medical and preventive care were observed. The reform of children’s health care contributed to the diseases’ timely diagnosis and early patients’ hospitalization, strengthening the connection of the primary care doctor and nurse with the child’s family, and decreasing of child morbidity and mortality.

The results of modern studies on breast milk jaundice pathogenesis, that is common form of neonatal jaundice, are presented. The major risk factors and diagnostic methods (excluding other forms of jaundice syndrome at recurrent and lingering jaundice in newborns) were defined. The main therapy approaches for breast milk jaundice were presented. Issues of phototherapy indications were discussed. The feasibility of maintaining breastfeeding in case of breast milk jaundice development was proved. This condition requires the attention of pediatricians despite the relatively favorable course and outcomes. The need to continue studies on pathogenetic therapy of such patients was noted.

This review summarizes stages of intestinal microbiota development in infant and immune responses modulation associated to these stages. The leading role of breastfeeding in the optimal microbiota and associated immune responses development during the first half of child’s life is presented. The biological feasibility of supplemental feeding implementation at the second window of opportunity (4–6 months) is justified, as well as role of supplementation products (including cereal) in adult microbiota development.

Atopic dermatitis (AD) is a widespread chronic inflammatory skin disease that has a significant impact on various aspects of patient's life. This review presents modern view on AD pathophysiology, its correlation with other concomitant diseases, and covers practical aspects of external anti-inflammatory therapy implementation. The analysis of clinical studies has shown the significant role of calcineurin inhibitors in the effective treatment of AD in children from its first manifestations at early age.

The use of magnetic resonance imaging in morphometry, as quantitative assessment of brain parameters (thickness, surface area, volume), allows to detect changes in many neuropsichiatric conditions that were previously considered intact. This article provides data on neuroimaging brain morphometry and effective use of this method in neurosciences.

Background. Campylobacteriosis is the leading cause of bloody diarrhea worldwide. Infants have high risk severe campylobacteriosis as well as development of complications affecting the child growth and development. Objective. The aim of the study is to evaluate the long-term effects of campylobacteriosis in infants. Methods. A multicenter, prospective, dynamic, open, observational study included 80 children aged from 3 months to 2 years. The follow-up period after campylobacteriosis was 12 months. Patients were divided into three groups according to the feeding type: 1 — acidified milk formulas, 2 — non-acidified milk formula without a probiotic, 3 — breastfeeding. Physical examination data and anthropometric indicators were evaluated: height, body weight and z-indices. Moreover, qualitative and quantitative indicators of microbiocenosis were evaluated on the 21st day from the disease onset. Results. Functional gastrointestinal disorders (FGID) were diagnosed in 17 children (42.50%) at the follow-up period after campylobacteriosis: functional constipation (n = 11; 27.5%), functional diarrhea (n = 3; 7.50%), infantile colic (n = 2; 5.00%), cyclic vomiting syndrome (n = 1; 2.50%). Disorders of intestinal microbiocenosis were characterized by Bifidobacterium spp. and B. thetaiotaomicron decrease in all groups. Patients with FGID after campylobacteriosis had profound decrease in the level of Bifidobacterium spp., F. prausnitzii and B. thetaiotaomicron, and slower rate of body weight gain. The more profound decrease in body weight gain rates was observed in patients with initially low indicators. 10 (25.00%) patients after campylobacteriosis had no slowdown in body weight gain. 5 of them were administered with acidified milk formula during the convalescence period. 7 children (17.5%) had a decrease in weight-to-age ratio relevant to body weight deficiency, while 6 children (15.0%) moreover had malnutrition in BMI-to-age index significantly more frequently (p = 0.0050) with non-acidified milk formula without a probiotic after completion of the follow-up. Conclusion. The inclusion of children on formula feeding in the complex of therapeutic measures (with acidified milk formulas with probiotics) contributes to the improvement of qualitative and quantitative indicators of microbiocenosis and the rapid restoration of the body weight gain and growth rates.

Background. Alport syndrome is a systemic, hereditary, progressive disease characterized by ultrastructural changes in the glomerular basement membrane caused by pathogenic variants of type IV collagen genes. The use of angiotensin-converting enzyme inhibitors (ACEI) for nephroprotection is effective at the microhematuria and/or albuminuria stage. Treatment tactics in case of nephrotic syndrome development in such patients remains the subject of discussion. Clinical case description. The patient was diagnosed with proteinuria at the neonatal period and hematuria at the age of one month. The hereditary nephritis was diagnosed at the age of 6 years; the ACEI was administered, however, the proteinuria continued to increase. The diagnosis was confirmed at the age of 8.5 years via the puncture nephrobiopsy: collagenopathy, type IV, focal segmental glomerular sclerosis. Moreover, chronic bilateral sensorineural hearing loss and bilateral myopic astigmatism were diagnosed. Ciclosporin A (125 mg/day) was additionally prescribed. The increase in the cystatin C, urea, uric acid, cholesterol levels in blood was mentioned after 14 months of treatment. These parameters decreased after reducing cyclosporine A dose to 100 mg/day, however, proteinuria has increased. Angiotensin II receptor blocker (candesartan 8 mg/day) was prescribed to enhance nephroprotective therapy at the age of 10 years 2 months. Another increase of the immunodepressant dose was performed at the age of 11, it led to decrease in the estimated glomerular filtration rate and increase of creatinine, cystatin C, urea, cholesterol, uric acid, and potassium levels in the blood. These changes were considered as cyclosporine-dependent. The dose of cyclosporine A was reduced to 125 mg/day, and to 100 mg/day from the age of 14. There was no progression of chronic kidney disease at the follow-up at the age of 15.5 years. Conclusion. Nephroprotective treatment of a child with Alport syndrome initiated after the development of nephrotic syndrome did not stop the chronic kidney disease progression. Whereas relatively high doses of ciclosporin A have reduced proteinuria but led to nephrotoxicity and cyclosporin dependence.

Background. The management of children with systemic lupus erythematosus (SLE) is usually associated with lifelong systemic glucocorticoids administration and, thereby, high risk of serious side effects, including steroid-induced diabetes. The belimumab (B-lymphocyte stimulator inhibitor) administration significantly reduces the glucocorticoids dose, the risk and severity of steroid therapy complications. Clinical case description. The patient was diagnosed with SLE at the age of 16 years. Therapy with hydroxychloroquine and oral glucocorticoid at a high dose (methylprednisolone 56 mg per day) was initiated. Steroid-induced diabetes was diagnosed 1 month after the therapy start; avascular necrosis sites were revealed in 2 months. Mycophenolate mofetil made it possible to achieve the disease activity control. However, the belimumab was prescribed 5 months after diagnosis verification due to continuous insulin requirement and avascular necrosis progression. Conclusion. Belimumab is the only genetically engineered biologic drug approved for the treatment of children with SLE. As a result of its use, it was possible to stabilize the patient's condition quickly (within 3 months), to reduce significantly the dose of oral glucocorticoid, methylprednisolone (from 24 to 8 mg/day), to achieve remission of steroidinduced diabetes with further insulin withdrawal, and also to relieve avascular necrosis clinical symptoms.

Background. Hurler syndrome (mucopolysaccharidosis, type I) is a rare hereditary disease with chronic course. The main methods for Hurler syndrome management are hematopoietic stem cell transplantation (HSCT) and enzyme replacement therapy (ERT). In recent years, combination treatment (ERT administration both before and after HSCT) has shown its efficacy in case of disease progression. Clinical case description. The presented clinical cases demonstrate the efficacy of ERT administration in patients with Hurler syndrome after HSCT: in the first clinical case due to the decrease in alpha-iduronidase activity 2 years after HSCT, in the second clinical case due to the aggravation of the patient's condition (cardiovascular and respiratory systems, hepatomegaly, although the level of enzyme and glycosaminoglycans in the patient's urine remained within normal values). Conclusion. Combination treatment including ERT not only before HSCT, but also in case of clinical state worsening after HSCT, plays significant role in stabilizing the patient's condition, preventing rapid progression of symptoms and development of life-threatening complications (especially cardiovascular ones).

Hereditary amino acid metabolism disorders (aminoacidopathies) are clinically and genetically heterogeneous group of hereditary metabolic diseases caused by enzymes deficiency involved in amino acid metabolism, that finally leads to progressive damage of central nervous system, liver, kidneys, and other organs and systems. Hereditary urea cycle disorders occur because of enzyme deficiency leading to impaired urea synthesis and hyperammoniemia in patients. The age of disease onset and clinical manifestations severity range from milder, intermittent forms to severe, manifesting in the first hours of life. Expanded neonatal screening (implemented in Russian Federation at 01.01.2023) allows to diagnose diseases from these groups in the first days of life, to prescribe timely pathogenetic therapy. Altogether it helps to prevent the development of disease severe complications. Raising awareness about hereditary aminoacidopathies and urea cycle disorders among pediatricians, neonatologists, neurologists, gastroenterologists, ophthalmologists is a topical issue of modern pediatrics.

Acid sphingomyelinase deficiency is a rare hereditary disease caused by enzyme deficiency due to mutations in the SMPD1 gene. Decreased enzyme activity leads to accumulation of sphingomyelin in lysosomes. This disease is characterized by wide diversity of clinical manifestations: from infantile neurovisceral form (Niemann-Pick disease type A) with severe rapidly progressive neurodegeneration to chronic visceral form (Niemann-Pick disease type B) with almost no central nervous system involvement. There is also intermediate phenotype: chronic neurovisceral form (Niemann-Pick disease type A/B) that includes manifestations two other forms, both visceral and neurological. The disease course is most commonly progressive and multi-system due to sphingomyelin accumulation in cells of the monocyte-macrophage system, as well as in other cells such as hepatocytes.

Providing medical care based on a patient-centered medicine model supposes close collaboration with patients and their families based on partnership. The development of action plan aimed on implementation of comfortable conditions for the child during surgery and anesthesia, considering his needs and age characteristics, is a topical issue in pediatrics. This article provides an organizational model for anesthesia in child-friendly environment and covers all the main stages of the patient's stay in the hospital.