SOCIAL PEDIATRICS AND HEALTH CARE
The article is devoted to the important course of the medicine development at the beginning of the 20th century — mother and infant protection. The contribution of pediatricians and obstetricians to prevention of high infant mortality in Moscow is represented. The activities of G.N. Speransky and other doctors in public benevolent institutions are reported.
PROFESSIONAL DEVELOPMENT
The lecture analyzes modern knowledge about etiology, key mechanisms of pathogenesis, clinical manifestations, types, diagnostic methods and treatment strategy of chordoma (tumor from notochordal cells). To assess the retention of the lecture material, a case problem and multiple-choice test questions are given.
REVIEW
Infantile cerebral palsy (ICP) is the main cause of childhood disability and is characterized by a non-progressive lesion and/or impaired development of the brain in a foetus or newborn. Magnetic resonance imaging (MRI) is a modern non-invasive method with extensive capabilities for diagnosing brain damage in ICP. The review focuses on anatomical structural MR patterns of brain damage in ICP and gives the present-day classification of MR changes in this disease. The role of MRI in determining the duration of brain damage in ICP has been considered. Data on the ratio of ICP phenotypes to pathological MR findings has been presented. Neuroimaging prognostic biomarkers are discussed. It is emphasized that many questions regarding the prognostic significance of MR findings remain unresolved; prospects are associated with the use of new MRI modalities such as functional and diffusiontensor MRI.
The review presents an analysis of the mechanisms of iron effect on the brain development. The importance of iron deficiency in the perinatal period is considered as a risk factor for the development of neuropsychiatric disorders in children with autism spectrum disorders (ASDs). Possible causes of sideropenia are discussed; data on haematological and biochemical parameters characterizing iron metabolism in children with ASDs are presented. The demand for studying the role of iron metabolism imbalance in the development of neuropsychiatric disorders in order to clarify pathogenetic mechanisms of ASDs and to determine methods for their correction is emphasized.
ORIGINAL ARTICLES
Background. An important goal of treating patients with juvenile idiopathic arthritis (JIA) is to achieve the best quality of life associated with health. Objective. Our aim was to assess the impact of methotrexate plus etanercept therapy on the quality of life of patients with early and late JIA. Methods. The prospective study included patients with early and late JIA without systemic manifestations. The patients’ quality of life was assessed with the help of questionnaires for children and parents: the Pediatric Quality of Life Inventory (PedsQL) Generic Core Scale, the Pediatric Quality of Life Inventory (PedsQL) Rheumatology Module, and the Health Utilities Index Mark 3 (HUI3). The quality of life was assessed prior to the therapy and after one, six, and 12 months of treatment. Results. 150 children with JIA aged 5.1 (2.0; 17.7) years; 50 children aged 4.0 (2.3–11.4) years in the group of etanercept monotherapy, 50 children aged 5.0 (3.2–9.0) years in the group of methotrexate monotherapy, and 50 children aged 9.9 (6.4–13.0) years in the group of methotrexate plus etanercept combination therapy. All groups showed low scores on all questionnaires before treatment, compared to healthy children. In the course of therapy, there was a tendency for score increase to almost 1.0 according to the HUI3 questionnaire in all groups. After one year of etanercept therapy, the parameters of the quality of life of children with early JIA did not differ from healthy children; the score increased from 56 to 90 p = 0.942 according to the physical functioning scale and from 60 to 85 p = 0.889 according to the emotional functioning scale. In the 2nd group, there was a tendency for score increase, but a statistically significant difference was found across all scales of the questionnaire. After 12 months of etanercept plus methotrexate combination therapy in patients with late JIA, the questionnaire analysis showed that the responses of healthy children and children with JIA differed with probability p = 0.001 for the physical functioning scale, p = 0.001 for the social functioning scale, p = 0.001 for role functioning, and p = 0.001 for the total score. The score increase from 60 to 85 p = 0.789 was noted for emotional functioning scales. Conclusion. The use of questionnaires to assess the quality of life in children with severe chronic diseases can significantly improve the efficacy of treatment and ensure its control.
Background. Cryptosporidium protozoa are the leading causative agent of diarrhea and cause of death in children under 5 years of age. The role of cryptosporidia in the development and course of acute intestinal infections (AII) in children in Russia remains unstudied. Objective. Our aim was to study the prevalence and clinical laboratory features of cryptosporidium-associated aII in children under 5 years of age. Methods. A cross-sectional study (conducted in March-June 2017) included children admitted to hospital with symptoms of AII (fever, loose watery stools, weakness, decreased appetite and/or vomiting) by the ambulance service. On admission, stool samples were collected from all patients. Cryptosporidium oocysts were determined by microscopic examination of faecal smears stained according to Tsil-Nielsen after preliminary concentration by a modified formalin-ether technique. The presence of intestinal pathogens was determined by a bacteriological technique and using a polymerase chain reaction. Results. The study included 107 children with AII (girls — 51%). Cryptosporidia were detected in 28 (26%) patients, in 93% of cases — together with bacterial and/or viral pathogens. The etiological structure of cryptosporidium-associated AII and AII in cryptosporidiosis negative children (n = 79) did not differ. On admission, children with cryptosporidium-associated AII had a higher blood leukocyte count — 13.0_109/L (9.2; 16.0) versus 8.3_109/L (6.1; 11.2) in children without cryptosporidiosis (p < 0.001). It has been also found that antibiotics were more often used in the treatment of children with cryptosporidium-associated AII — in 21 (75%) versus 39 (49%) in the comparison group (p = 0.026). Conclusion. Cryptosporidia are detected in every fourth child with AII under 5 years of age. Patients with cryptosporidia are distinguished by a higher level of blood leukocytes upon admission and a more frequent prescription of antibiotics than in the group of cryptosporidiosis negative patients.
CLINICAL OBSERVATIONS
Background. Infections are the main cause of death for patients with autoimmune rheumatic diseases. In adult patients with rheumatoid arthritis (RA), mortality caused by respiratory infections is 2–5 times higher than in the population. One of the frequent infectious complications in the course of treatment with tocilizumab, the first-choice drug for treating systemic juvenile idiopathic arthritis (sJIA), is pneumonia characterized by a poor clinical picture, normal values of laboratory indices of the disease activity (ESR, C-reactive protein) with pronounced changes in the lungs revealed by computed tomography. In case of acute respiratory infection in children with systemic JIA, immunosuppressants and genetically engineered biological preparations (GEBP) are discontinued. This often leads to an exacerbation of the underlying disease and the progression of a pathological process. At present, vaccination against pneumococcal infection in Russia is not included in the standard for managing patients with rheumatic diseases. Studies of the safety and efficacy of vaccination with 13-valent pneumococcal conjugate vaccine (PCV) in patients with sJIA receiving genetically engineered biological preparations were not conducted. Clinical Case Description. The article shares the experience of vaccination of a girl aged 9 years with a 13-valent PCV that was conducted in the course of a scientific investigation, which studied the efficacy and safety of vaccination of children with systemic JIA prior to prescription of GEBP tocilizumab. Vaccination did not cause a deterioration in the course of the main disease (1 month), led to a reduction in the incidence of acute respiratory infections (from 4 to 1 time within 6 months before and after vaccination), and discontinuation of antibacterial drugs within 6 months after vaccination. Conclusion. The safety of a 13-valent PCV in a child with sJIA and a decrease of the incidence of respiratory diseases after vaccination, their complications, and the use of antibacterial drugs have been shown.
Background. Insufficient efficacy or intolerance of the first TNF-α inhibitor in patients with juvenile idiopathic arthritis (JIA) is an indication for the appointment of a second inhibitor. Golimumab is a new TNF-α inhibitor registered for treating JIA under pediatric indications. Clinical Case Description. At an early age, the patient had an onset of polyarticular JIA. Due to the aggressive and rapidly progressive course, failure of therapy with nonsteroidal anti-inflammatory drugs, methotrexate and glucocorticosteroids for intra-articular administration, infliximab was prescribed to the patient, with a positive effect. Subsequently, the patient developed a secondary resistance to infliximab, inflammatory changes in the joints relapsed; thus, a second TNF-α inhibitor (golimumab) was prescribed. In the course of therapy, pain and signs of arthritis in the patient were reversed, and the range of motion in the affected joints increased. After one year of therapy, JIA remission was ascertained. At the same time, the child was not administered oral glucocorticosteroids. The duration of remission of the joint syndrome was 5 years. Adverse events were not serious and did not constitute a basis for drug discontinuation. Conclusion. Switching to a second TNF-α inhibitor (golimumab) was effective in a patient with a secondary resistance to the first TNF-α inhibitor.
Background. The prevalence of congenital malformations of the vessels or angiodysplasia ranges from 1:50,000 to 1:5,000,000. Congenital angiodysplasia is a consequence of impaired formation and development of the vascular system in embryogenesis. The aetiology of angiodysplasia remains poorly studied, and the diagnosis involves significant difficulties in some cases. Clinical Case Description. The observation of a rare case of a combined malformation of vessels and thymic aplasia in a female infant is presented. Angiodysplasia included the syndrome of congenital generalised phlebectasia (synonym: congenital telangiectatic marbled skin) combined with multiple vascular malformations with predominant vascular lesions of the brain, lungs, heart, kidneys, and mesentery. Clinically, the disease was characterised by a generalised change in the skin in the form of livedo reticularis accompanied by the development of severe pneumonia, persistent urinary syndrome, neurological symptoms (convulsive seizures, motor disorders), and progressive heart failure. The diagnosis was confirmed in the course of a pathological study. Conclusion. The presented case allows expanding the notion of the variety of clinical manifestations of congenital angiodysplasia, as well as its possible combinations with other malformations.
A DOCTOR’S AID
The article presents the modern knowledge of the structural and functional peculiarities of the skin in children. Information on the etiopathogenesis of a debut atopic dermatitis has been also given. The results of our own observations with an analysis of the clinical efficacy and safety of cosmetics based on highly purified lanolin and cotton extract in infants have been presented.
EXPERT OPINION
The results of a questionnaire survey of mothers on the use of infant formulas at the obstetric hospital have been presented. Possible ways to improve the situation with the use of infant formulas in term infants at the obstetric hospital have been discussed. A high frequency of the use of formulas in healthy newborns at the obstetric hospital as well as the practice of issuing formulas as a gift on discharge from the obstetric hospital have been revealed. The literature data has been given on the negative effect of both unreasonable use of formulas and the issuance of formulas as a gift on discharge on the preservation of breastfeeding. It is expected that restricting the distribution of milk formulas without proper indications will lead to an increase in the prevalence of breastfeeding.
The problem of dosing drugs at an early age is conditioned by specific metabolism of medicinal products (MP) in the child's body. Currently, there are a few clinical trials on the study of physiological characteristics in different periods of childhood and systematised data. It is still relevant to understand the characteristic differences that affect the bioavailability, distribution and excretion of MP, especially in children over one month of life. The results of such studies are necessary in order to formulate the recommendations for use of MP in children taking into account their age and compensate for the lack of data from direct clinical trials in pediatrics. The possibility of using a dose calculation method regarding the fat content of the body in different periods of childhood and the chemical properties of the substance has been discussed.
IN MEMORY OF
ISSN 1682-5535 (Online)