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Vol 11, No 4 (2020)

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Editorial
Character of feeding newborns in perinatal center. Problems of first week
Berezkina E.N., Ivanov D.O., Novikova V.P., Zavyalova A.N., Gostimskii A.V., Susanina A.M., Lisovskii O.V.
Abstract

The key to shaping the health of the future generation are the start timing of breastfeeding. In a single-center observational study in the Department of physiology of the newborn Perinatal Center, the nature of breastfeeding and problems in breast-feeding in mothers of healthy newborns in the first days after birth were evaluated. An analysis of the promotion and support of breastfeeding in 103 pairs (mother – infant) during hospital stay in accordance with WHO recommendations was conducted. Women were polled on socio-demographic issues (age, marital status, education, method of delivery), gestational age of infants, antropometry, Apgar score and infant feeding method. 71% of newborn were exclusively breastfed. Problems with breastfeeding were found in 41.7% of women. The most often meet: hypogalactia (17.48%) and cracked nipples (8.74%). All women received advice on how to eliminate their causes. The importance of providing permanent specialized professional support in breastfeeding to overcome the difficulties of matters after discharge from the hospital has been proved.

Pediatrician (St. Petersburg). 2020;11(4):5-13
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Original studies
Clinical and morphofunctional features of the cardiovascular system in children with remheld syndrome
Lymarenko M.P., Iskovich D.V.
Abstract

The article is devoted to an urgent problem of pediatrics – Remheld syndrome. The aim of the present work was to study the clinical and morphofunctional features of the cardiovascular system in children with Remkheld’s syndrome. 23 children from 10 to 18 years old with Remkheld’s syndrome were examined. Research methods. Study of blood biochemical parameters (MV-CPK, ASLO titer, C-reactive protein), identification of persistent viral infection markers, ECG, daily monitoring of ECG and blood pressure by Holter, echocardiography, ultrasound of the vessels of the head and neck, ultrasound of the thyroid gland, ultrasound organs of the gastrointestinal tract. In the anamnesis, 78,3% of children had chronic gastroduodenitis, 21,7% had gastroesophageal reflux disease, and 8,7% had a hiatal hernia. All patients complained of cardialgia, interruptions in the work of the heart, a feeling of lack of air, dizziness upon admission. The appearance of noted complaints was associated with eating. Results. A study of the state of the cardiovascular system showed the presence of sinus tachycardia in 60,9% of patients, sinus bradycardia in 34,8%, single supraventricular extrasystole in 13,0%, paroxysmal supraventricular tachycardia in 4,3%, and 4,3% – autonomic sinus node dysfunction, in 13,0% – congenital heart disease (patent ductus arteriosus, bicuspid aortic valve), in 8,7% – mitral valve anterior prolapse, in 4,3% – a patent foramen ovale, in 95,7% of children – vegetative-vascular dysfunction. Conclusion. Children and adolescents with diseases of the gastrointestinal tract, when cardiac complaints appear, need an in-depth examination of the cardiovascular system.

Pediatrician (St. Petersburg). 2020;11(4):15-19
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Cortisol and laboratory indicators of systemic inflammation in case of bacterial purulent meningitis and viral encephalitis in children
Alekseeva L.A., Bessonova T.V., Makarenkova E.V., Zhirkov A.A., Monakhova N.E., Vilnits A.A., Gorelik E.Y.
Abstract

Pediatric bacterial purulent meningitis (BPM) and viral encephalitis (VE) are significant medical and social problems due to their course severity, high frequency of death cases, and formation of neurologic deficiency at the disease outcome. Activation of hormonal regulation and severity syndrome of systemic inflammatory response are important factors to evaluate the character of BPM and VE course. Objective. To study the level of cortisol and laboratory indicators of systemic inflammation in children with various variants of BPM and VE course depending on the period of the disease (acute period, reconvalescence) to specify their role in the pathogenesis of acute neuroinfections. Object and methods. There were investigated hematological indicators, the level of cortisol, C-reactive protein in blood serum of 60 children, 39 of them had BPM and 21 ones – VE. The comparison group included 14 children aged from 1 to 14 years old who were undergoing rehabilitation care due to neurologic problems at the Federal State-Financed Institution Pediatric Research and Clinical Center for Infectious Diseases under the Federal Medical Biological Agency. Results. The patients were divided into some subgroups according to the severity of their condition at the moment of hospitalization, i.e. urgent condition or critical condition requiring organ replacement therapy. The maximum increase of cortisol level and laboratory markers of systemic inflammation during the acute period was revealed in case of BPM in comparison with VE with a subsequent normalization to the stage of reconvalescence. The level of cortisol during the acute period of BPM was reliably higher in the subgroup with urgent conditions, whereas in case of VE – in the subgroup with critical conditions. There were no significant differences in the laboratory indicators of systemic inflammation response among the subgroups. There was established a correlation interrelation of cortisol level and the content of granulocytes and blood lymphocytes. Conclusion. There were identified characteristic features of cortisol content in children with bacterial and viral neuroinfections depending on the course of the disease.

Pediatrician (St. Petersburg). 2020;11(4):21-28
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Non-invasive prenatal screening: experience in the Republic of Tyva
Gritsinskaya V.L., Sanchat N.O., Badarchy M.S.
Abstract

Diagnosis of congenital developmental anomalies and chromosomal abnormalities of the intrauterine fetus in early pregnancy can reduce infant mortality rates. The purpose of research: to evaluate the first results of the non-invasive prenatal test (NIPT) in the Republic of Tyva. Materials and methods. A retrospective analysis of the reports of the Federal State Statistics Service for the Republic of Tyva, the Ministry of Health and the Tyva Perinatal Center was carried out; monitoring data of congenital developmental anomalies in the republican medical genetic center. Results. Analysis of infant mortality in the Republic of Tuva for the period from 2000 to 2019 showed that with a steady decline in indicators (29.9–7.3‰), its level significantly exceeds the national average. One of the leading causes of infant mortality in the republic is congenital malformations, the frequency of which among newborns in Tuva is 12.6–33.4‰. Diseases caused by chromosome abnormalities make up from 4.3% to 1.2% in the structure of congenital malformations. In recent years, the number of women who underwent fetal pathology screening in the first trimester of pregnancy has been increasing in the republic (56.2–62.1%). According to combined screening, the detection of major chromosomal aneuploidies (Down, Edwards and Patau syndromes) is 2.1–6.6%. In all cases of positive screening, NIPT was performed, which has a higher specificity and sensitivity; the presence of chromosomal aberrations in the fetus was confirmed in 98.1–99.5% of cases. Conclusion. The introduction of widespread use of NIPT in the republic will increase the efficiency of detecting chromosomal abnormalities, reduce the number of invasive diagnostic interventions, avoid unjustified termination of pregnancy and reduce infant mortality.

Pediatrician (St. Petersburg). 2020;11(4):29-33
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Reviews
Congenital sodium diarrhea
Ivanov D.O., Novikova V.P.
Abstract

Congenital sodium diarrhea (P78.3 according to ICD 10) is a rare autosomal recessive disease, clinically and genetically heterogeneous. The sodium absorption defect is caused by disruption of the intestinal sodium-proton “pump” in the non-syndromic form and in the epithelial sodium channels in the syndromic form. Mutations in 3 genes, SPINT2 (localization 19q13.2; OMIM code 270420) – syndromic form; GUCY2C (localization 12q12.3; OMIM code 601330) and SLC9A3 (localization 5p15.33; OMIM code 616868) – non-syndromic form, can cause congenital sodium diarrhea. The frequency of the disease is unknown, since it is rare, so far only 50 cases have been described. The classic non-syndromic form of congenital sodium diarrhea is manifested by polyhydramnios, severe secretory diarrhea, severe metabolic acidosis, alkaline pH of feces >7.5 and hyponatremia. The syndrome of congenital sodium diarrhea is also manifested by choanal and/or anal atresia, hypertelorism and erosion of the cornea. Typical laboratory data include metabolic acidosis and alkaline pH of feces (fecal pH >7.5), low Na+ concentrations. The concentration of Na+ in the stool is increased. Prenatal ultrasound diagnosis allows you to identify gidroamnion and expansion of intestinal loops, starting from the third trimester of pregnancy. The diagnosis is confirmed by genetic studies. Treatment: complete parenteral nutrition with correction of water-salt metabolism. The forecast is unfavorable.

Pediatrician (St. Petersburg). 2020;11(4):35-42
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Modern methods for diagnostics of liver fibrosis in children
Efremova N.A., Goryacheva L.G., Karabak I.A.
Abstract

This review is dedicated to nowaday methods of liver fibrosis diagnostic in children. The article presents various types of puncture biopsy of the liver. Implementation of the immunohistochemical method in the morphological analysis of biopsy samples allows us to expand our understanding of the pathogenesis of chronic liver diseases, the role of the concomitant infectious agent in the progression of the disease and its outcomes. Instrumental methods for visualizing fibrosis with an assessment of their diagnostic significance are observed in the article. Ultrasound is a screening method among instrumental examination. Computed and magnetic resonance imaging are obligate imaging methods for suspected fibrosis, but they are do not allow to verify its stage. The advantages and disadvantages of various types of elastography are presented. Promising directions of fibrosis diagnostics are associated with scintigraphy, acoustic structural quantitative analysis. Special attention is paid to serum markers for assessing the stage of liver fibrosis in children; data about the role of several fibrosis markers, such as hyaluronic acid, type IV collagen, transforming growth factor β1, as well as APRI, FIB-4, FibroTest indices in children are presented. Further study of the pathogenetic aspects of fibrogenesis, exploration of new non-invasive techniques for differentiation the intermediate stages of liver fibrosis, and the development of its prognostic criteria are required.

Pediatrician (St. Petersburg). 2020;11(4):43-54
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Opinions, Hypotheses, discussion issues
The association of neuropsychiatric disorders and endocrine parameters in hashimoto thyroiditis
Sobolevskaia P.A., Andreev B.V., Gvozdetckii A.N., Dolina A.A., Stepochkina A.M., Stroev Y.I., Utekhin V.J., Fedotkina T.V., Churilov L.P.
Abstract

Hashimoto thyroiditis is the most common thyroid disease. This form of pathology has a diverse clinical picture, including neuropsychiatric disorders. There are frequent cases of comorbidity of autoimmune thyroiditis and psychiatric forms of pathology, along with such a nosological entity as Hashimoto’s encephalopathy (aka: Steroid-responsive encephalopathy of autoimmune thyroiditis), characterized by an increased level of antithyroid autoantibodies and various mental disorders, with still unclear pathogenesis. The question arises, how to regard patients with psychiatric disorders and Hashimoto thyroiditis — either as patients having autoimmune thyroiditis, comorbid with psychiatric forms of pathology, or as patients with Hashimoto’s encephalopathy? We studied groups of patients with autoimmune thyroiditis free from any psychiatric disorders, autoimmune thyroiditis comorbid with psychiatric forms of pathology, and a group of healthy donors similar as regards to their age and sex. We also studied medical history, clinical manifestations of the disease, instrumental data and the serum levels of thyrotropin, thyroid hormones, various antithyroid autoantibodies, and prolactin. We analyzed the correlation of laboratory and instrumental parameters and clinical data in all groups of patients. There was a significant relationship (p < 0,05) between various psychiatric symptoms and a decreased level of free thyroxine, an increased level of thyroid stimulating hormone (TSH), an increased level of prolactin and an increased volume of a thyroid gland. A significant relationship (p < 0,05) was also found between various symptoms of hypothyroidism and a decreased level of free triiodothyronine (FT3), an increased level of antibodies to thyroglobulin (anti-TG Ab), and an increased level of antibodies to thyroid peroxidase (anti-TPO Ab).

Pediatrician (St. Petersburg). 2020;11(4):55-68
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Clinical observation
Case of severe heart and kidney damage in a young man with systemic red lupus
Timofeev E.V., Golubeva O.R., Isakov V.A., Vyutrikh E.V.
Abstract

Systemic autoimmune diseases remain a complex problem for specialists of different profiles. Despite some progress in their diagnosis and treatment, the incidence of these diseases is now steadily increasing. This is primarily due to polyorganic lesion, which requires a balanced approach to the choice of treatment of the patient, taking into account the functional condition of different organs and systems. Thus in particular systemic lupus erythematosus, which occupies a leading place in the structure of rheumatological pathology, often occurs with kidneys damage, cardiovascular system, hematological disorders. Most studies are based on the systemic lupus erythematosus in women, while the male sex is recognized as a factor of adverse outcomes and severe course of the disease. The article covers the peculiarities of the course of systemic lupus erythematosus in men, considered the variants of heart affection within the systemic process, and also presents a clinical case of systemic lupus erythematosus of high activity in a young man with terminal kidney affection, pancarditis with formation of severe mitral insufficiency, pancytopenia. The aspects of the management of a patient with severe valve disease of the heart in need of renal replacement therapy against the background of drug immunosuppression, the possibility of correction of valve disease. The combination of terminal renal failure and heart failure in a young man in the clinical case cited is an example of the multidiscplinar problem of treating systemic lupus erythematosus.

Pediatrician (St. Petersburg). 2020;11(4):69-75
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Clinical psychology
Features of experience of crisis associated with foot amputation by the elderly patients
Kut’kova A.K., Zemlianykh M.V.
Abstract

The article is devoted to the study of the features of experiencing a crisis associated with amputation of the foot by the elderly patients. The aim of the research was to study the characteristics of elderly patients experiencing a crisis associated with amputation of the foot. We studied 31 patients with the threat of foot amputation at the age of 59–73 years, who were hospitalized at the stage of reconstructive operations. The second group consisted of 32 patients aged 59–73 years who underwent amputation of the foot at least 7 days ago undergoing postoperative rehabilitation in the in-patient department of the hospital. Research methods: empirical (conversation, questioning, testing). A comparison of the indicators obtained during an empirical study revealed that elderly people with the risk of foot amputation are more likely to experience negative emotional experiences than elderly people with an amputated foot, use non-adaptive coping strategies, and have higher severity of dysfunctional believes, note dissatisfaction with one’s own body and correlate negative emotional experiences with the lower limbs, especially the feet. Compared to patients with the threat of foot amputation, patients with amputated foot are more likely to experience positive emotions, note less pronounced pain, use such psychological defense as intellectualization, note less dissatisfaction with their own body and project the entire spectrum of emotions in the head area. The results of testing and verifying the effectiveness of the program of psychological support for optimizing the psycho-emotional state of patients with the threat of foot amputation are presented.

Pediatrician (St. Petersburg). 2020;11(4):77-84
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Postgraduate medical education
Training of pathologists in the digital macroscopic photography
Khramtsov A.I., Nasyrov R.A., Khramtsova G.F.
Abstract

The pathology practice environment varies per healthcare setting. However, anatomic pathology is a visual applied science discipline and incorporation of high-quality images into a surgical pathology report is essential. Each specimen received for morphological examination is unique and variation in the description can exist between prosectors and they experience. That is why gross descriptions supported with digital photographs can eliminate the insufficiency of macroscopic examination. To form and strengthen pathologists’ competencies in digital macroscopic photography a problem-based learning approach is used for training. A problem-based learning ensures the strength of the acquired knowledge since it is obtained in an independent activity. The article discusses what type of problems a pathologist should solve when taking a macroscopic photograph of a surgical specimen. An analysis of literature on modern equipment for digital macroscopic photography was performed. Recommendations for step-by-step photographing, and schematic mapping for surgical specimen triaging are provided. An option is proposed for actively developing professional competencies including creation of digital photo archives of surgical gross specimens, as well as study sections and discussions by professionals at forums such as society meetings. It was concluded that pathologists’ competency in digital macroscopic photography is necessary to maintain a high standard of medical care.

Pediatrician (St. Petersburg). 2020;11(4):85-90
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Anniversaries
Mikhail stepanovich maslov – outstanding scientist, pediatrician, teacher (towards 135th anniversary)
Savenkova N.D., Ivanov D.O.
Abstract

The editorial presents medical, scientific, pedagogical activity academician of the USSR Academy of Medical Sciences, Honored Scientist of the RSFSR Mikhail Stepanovich Maslov (1885–1961). Mikhail Stepanovich Maslov Founder and Head of the Pediatrics Department of the Leningrad pediatric medical Institute (1930–1961). Academician of the USSR Academy of Medical Sciences Michael Stepanovich Maslov has made a great contribution in the development in domestic pediatrics. The head of the Department of faculty Pediatrics of LPMI, аcademician M.S. Maslov went down in history as a great scientist, one of the founders of the Russian scientific pediatric school, as a teacher who brought up several generations of pediatricians and scientific personnel, as a doctor who restored the health of hundreds of thousands of sick children. Academician M.S. Maslov is the author of more than 200 scientific works, including 16 monographs and 8 textbooks. Under the guidance of academician M.S. Maslov, 36 dissertations of the candidate of medical Sciences were completed and defended, 10 dissertations of doctors of science were prepared with scientific advice. The great scientist, teacher, doctor and public figure Mikhail Stepanovich Maslov is the pride of Russian pediatric science, education and healthcare.

Pediatrician (St. Petersburg). 2020;11(4):91-98
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In memoriam of Evgeny Iosifovich Schwartz
Gorbunova V.N.
Abstract

E.I. Schwartz stood at the foundation of Russian molecular genetics: he was the first to apply the novel method of polymerase chain reaction (PCR) in this country. E.I. Schwartz graduated from the LPMI at 1967. His area of research belonged at first to the realm of the metabolic manifestations of hereditary diseases. Since 1985, he began working at the Laboratory of Molecular Genetics, Leningrad Institute of Nuclear Physics (LINP). Shortly thereafter, E.I. Schwartz began to reproduce and implement the PCR, recently proposed by K. Mullis. Coincidentally, the seemingly purely fundamental studies of thermostable polymerase, which turned out to be the key PCR enzyme, had been independently conducted in LINP and contributed to the success of E.I. Schwartz undertaking. He also initiated the development of the first Russian thermocyclers. He implemented PCR in fruitful long-term research projects on the molecular epidemiology of monogenic diseases: phenylketonuria, familial hypercholesterolemia, hereditary disorders of hemostasis, etc. E.I. Schwartz optimized the methods of isolating nucleic acids from dried blood spots and other sources, “suboptimal” for a laboratory specialist, but ideal for screening and forensic practice. Another field of his research was to elucidate the role of polymorphic alleles in multifactorial diseases. E.I. Schwartz enthusiastically propagated the new molecular approaches among clinicians and laboratory geneticists: he established close and fruitful collaborations between specialists of the vastly divergent backgrounds. In 1989 E.I. Schwartz founded the Department of Medical Genetics of LPMI, which was one of the first such departments in U.S.S.R. In 2001, he founded the Department of Molecular Genetic Technologies in I.P. Pavlov`s St. Petersburg State Medical University. In 2003, E.I. Schwartz has passed away, leaving behind him the good memory of numerous students and successors of his work

Pediatrician (St. Petersburg). 2020;11(4):99-105
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