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Vol 11, No 5 (2020)

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Original studies

Small heart anomalies as cardiac manifestations of hereditary connective tissue disorders

Timofeev E.V., Malev E.G., Zemtsovsky E.V.

Abstract

Introduction. Small heart anomalies (SHA) are the morphological basis for functional changes in cardiac activity and can exacerbate the course of organic heart lesions. The most studied SHA include false chords of the left ventricle (FCLV) and mitral valve prolapse. Prevalence, association with external signs of dysembryogenesis, as well as the predictive value of SHA are not sufficiently studied.

Materials and methods. We examined 611 people between the ages of 18 and 23 (average age 20.3 ± 1.6 years), including 257 boys and 354 girls. All of the surveyed performed phenotypic, anthropometric and echocardiographic examinations. To identify the SHA links to heart rhythm disorders, the 205 surveyed performed Holter’s ECG monitoring.

Results. SHA identified in 90% of the individuals surveyed: atrial septum aneurysm (24%), tricuspid valve prolapse (23.4%), asymmetry of the aortic valve (20.6%), additional papillary muscles (39.4%) and FCLV (75,1%). Correlation analysis showed the presence of links between these SHA and bone signs of dysembryogenesis (chest deformities, arachnodactyllia, dolistennomely and high palate), as well as heart rhythm disorders (supraventricular and ventricular extrasystoles, rhythm driver migration and episodes of AV-blockade 1 degree). Patients with marfanoid habitus have a higher average number of SHA (2.1 ± 1.4 vs 0.9 ± 0.7, p < 0.005).

Conclusions. SHA are identified in the vast majority of healthy people. Bone signs of dysembryogenesis are associated with significant SHA and can serve as a marker for the involvement of the heart in the dysplastic process. Patients with SHA have significant cardiac arrhythmias.

Pediatrician (St. Petersburg). 2020;11(5):5-12
pages 5-12 views

Metapneumovirus infection in children

Sharipova E.V., Babachenko I.V., Orlova E.D.

Abstract

Objective: to study the clinical features of metapneumovirus infection in children of different ages in a hospital.

Materials and methods. A retrospective analysis of medical records of 142 patients aged 1 month to 14 years inclusive who were hospitalized in the period from January 2012 to April 2019. Metapneumovirus infection was confirmed by hMPV nucleic acid isolation by PCR in nasopharyngeal smears.

Results. Metapneumovirus infection is detected among hospitalized children with acute respiratory viral infections in 4,4% of cases. In the age structure, 72,2% are children under 4 years old, and the maximum incidence rate is among children aged 3 years of life. The leading clinical symptoms are cough in 93,0% of cases and rhinitis in 96,5% of cases.
In 88,2% of children, the disease proceeds with an increase in temperature >38 °C, including in 34,6% – 39,5 °C and above. Symptoms of gastrointestinal dysfunction in the form of vomiting and diarrhea develop in 26,1% and 22,5% of children, respectively. 78,2% of patients requiring hospitalization suffer hMPV infection with damage to the lower respiratory tract, including in the form of bronchitis in 85,6% of cases and pneumonia in 14,4% of cases. The disease is complicated by the development of bronchial obstructive syndrome in 38,7% and acute respiratory failure in 22,3% of cases. ARF and BOS are significantly more likely to develop in children of the first 3 years of life – 71,0% versus 29,0% in children of the older age group (p = 0.038) and 69,8% against 30,2% (p = 0.007), respectively. In a clinical blood test for hMPV infection, leukopenia and leukocytosis are detected only in 3,5% and 12,7% of cases, respectively, as well as an increase in ESR in 23,9% of children. The level of CRP in the 93,0% of children was less than 20 mg/l.

Conclusions. Virological confirmation of metapneumovirus infection in hospitalized children with lower respiratory tract infections contributes to the formation of an adequate therapeutic tactic.

Pediatrician (St. Petersburg). 2020;11(5):13-19
pages 13-19 views

Analysis of the prevalence of mental and speech disorders in children in the Srednekolymsky Ulus of Yakutia (based on the results of a screening examination)

Pozdnyak V.V., Grechanyy S.V.

Abstract

The article is devoted to the analysis of mental, including speech, disorders among children in the villages of the Srednekolymsky ulus of the Republic of Sakha (Yakutia). The purpose of the survey was to analyze the diagnosed mental, including speech, disorders among the children of the villages of the Srednekolymsky ulus of the Republic of Sakha (Yakutia). 501 children aged 2 to 18 years were examined. Clinically significant disorders were detected in 187 children (37.4%). The structure of mental disorders is described: the first place is occupied by specific speech and language disorders (F80), the second is mental retardation (F83) and the third is mild cognitive impairment (F06.7). The data on the prevalence of mental disorders in various age groups are presented: most often, mental disorders occur in adolescence (51.0% of all examined children). Less disturbances are diagnosed in the early age group and the first period of childhood – 22.0% of all children examined. In the group of preschool age, speech disorders are in the lead, in the group of primary school age – mental retardation, speech disorders and mild cognitive impairment. In adolescence, a wide range of mental disorders is diagnosed, including adaptive reactions and eating disorders. Sexual differences in the nosological specificity of mental disorders were revealed: mental disorders prevail in boys. Speech disorders, stuttering, and behavioral disorders are significantly more common among boys. Among girls, adaptation disorders, emotionally labile disorders and eating disorders are significantly more common.

Pediatrician (St. Petersburg). 2020;11(5):21-29
pages 21-29 views

Recurrent hiatal hernias. To operate or not?

Vasilevsky D.I., Bechvaya G.T., Ahmatov A.M., Korolkov A.Y., Smirnov A.A., Kiriltseva M.M., Davletbaeva L.I.

Abstract

The recurrence of a hiatal hernia after surgical treatment is the most serious and far from resolved problem in this area of surgery. The validity and effectiveness of surgical treatment of recurrent hiatal hernia of the diaphragm remains the subject of clinical research and scientific discussion. The main problems of such interventions are the difficulty of eliminating the anatomical or functional factors that underlie the failure of the primary operation. The stated provisions determine the need for further searches for a solution of this problem. In the period from 2015 to 2020, 61 patients with recurrent hernia of the gastrointestinal orifice of the diaphragm underwent surgical treatment. Indications for the operation were gastroesophageal reflux refractory to drug therapy or anatomical changes that carry the risk of developing life-threatening conditions. In 58 (95.1%) cases, surgery was performed laparoscopically, in 3 (4.9%) — through left-side thoracotomy. In 54 (88.5%) cases, complete restoration of normal anatomy with closure of the hiatal opening with prosthetic material is performed. In 7 (11.5%) cases, when the esophagus was shortened, the fundoplication cuff was created in the chest cavity, and the hiatal opening was performed only with its own tissues. Complications occurred in 11 (18.0%) cases (7 – pneumothorax, 2 – bleeding, 2 – perforation of a hollow organ). Long-term results (12-48 months) were evaluated in 57 (93.4% of operated) people. Repeated recurrence of hiatal hernia of the diaphragm was detected in 6 (10.5%) cases. In 44 (77.2%) cases, the natural anatomical position of the abdominal organs was documented. In 7 (12.3%) patients, with a fundoplication cuff formed in the chest, its initial position was ascertained.

Pediatrician (St. Petersburg). 2020;11(5):31-35
pages 31-35 views

Reviews

Comparative aetio-epidemiological analysis of lung tuberculosis versus sarcoidosis: classical and new concepts

Nikolaev A.V., Utekhin V.J., Churilov L.P.

Abstract

The review presents data on two similar granulomatous inflammatory diseases: tuberculosis and sarcoidosis of the lungs, which together cover about 5% of all pulmonary pathology, albeit occur with different incidence (20 : 1). Despite the established aetiology of tuberculosis, the disease has not disappeared and nowadays has even acquired a new urgency: It is getting out of control due to growing poverty, the comorbidity with HIV infection, increasing cases of drug resistance of Mycobacteria, insufficient effectiveness and the growing costs of its treatment. Against the background of the expansion of anthropogenic influences and other environmental impacts on the immune system, the incidence of lung sarcoidosis is also increasing, while patients are initially often misdiagnosed with tuberculosis, with resulting unjustified anti-tuberculosis chemotherapy, leading to chronization of the disease with frequent relapses and, accordingly, to an increase in disability and mortality rates. In recent years, clinical manifestations of sarcoidosis due to a variety of trigger aetiological factors with adjuvant-like action (from Mycobacteria to xenobiotics) – are considered by a number of authors as a variant of autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA). The article emphasizes the similarity of two granulomatous inflammatory diseases and the concept of two variants of the body’s response to similar or even identical aetiological factors within different human reactivity (possibly on a different mosaic/permissive background). In brief the newest data on experimental models of sarcoidosis are reviewed as well as the role of autophagy disorders and opposite macrophageal polarization in tuberculosis versus sarcoidosis. Authors coined the original hypothesis of the possible therapeutic effectiveness of Rapamycin in sarcoidosis and for the first time posed a question of equivocal character of comorbidity between these granulomatoses and COVID-19 infection.

Pediatrician (St. Petersburg). 2020;11(5):37-50
pages 37-50 views

Compression of the сeliac trunk syndrome. History of the problem

Khamid Z.M., Vasilevsky D.I., Ignashov A.M., Korolkov A.Y., Balandov S.G.

Abstract

The article presents the main stages of the development of ideas about a fairly rare pathology in clinical practice – the syndrome of compression of the ventral trunk. Until the mid-nineteenth century, reports of the clinical picture of chronic abdominal ischemia were more descriptive. Serial pathoanatomic studies, comparison of anatomical features revealed at autopsy with clinical manifestations accompanying these changes, contributed to the isolation of chronic ischemic disease of the digestive system in a separate nosological form. The discovery of angiography contributed to the significant development of vascular surgery and the isolation of the ventral trunk compression syndrome as an independent disease, since it allowed for a lifetime comparison of the detected changes in blood vessels with the clinical manifestations of the disease. The term “ventral trunk compression syndrome” was first introduced by P. Harjola when describing a case of abdominal pain in a patient with compression of the ventral trunk by the neuroganglionic tissue of the ventral plexus. Later, J. Dunbar confirmed the Association of clinical manifestations of chronic abdominal pain with compression of the ventral trunk by the median arched ligament of the diaphragm. In English-language literature, the disease was named “Dunbar syndrome”. The effectiveness of eliminating the symptoms of this disease by surgical dissection of compression structures has been proven. Further study of this problem developed in parallel with the development of surgery in General in accordance with the emergence and introduction of new surgical technologies. The purpose of numerous studies was to study the etiopathogenetic mechanisms of the occurrence of a variety of clinical manifestations of this syndrome and improve the results of its treatment by improving known surgical techniques and introducing new ones.

Pediatrician (St. Petersburg). 2020;11(5):51-56
pages 51-56 views

Clinical observation

Giant hepatic hemangiomas in newborns, review literature. Two cases of giant congenital hepatic hemangiomas

Podkamenev A.V., Syrtsova A.R., Ti R.A., Kuzminykh S.V., Kondratiev G.V., Myznikova I.V., Kostylev A.A.

Abstract

Hemangiomas are the most frequent tumors of childhood age. In the first year of life their prevalence is 10–12%. Among premature children, the prevalence of hemangiomas correlates with the degree of prematurity. Liver hemangiomas are a wide range of benign vascular formations that can acquire both malignant course and capable of spontaneous regression. Small-sized hemangiomas generally do not require specific treatment and proceed asymptomally. Large-sized formations can cause life-threatening conditions such as severe thrombocytopenia with coagulopathy (Kazabah–Merrit syndrome), anaemia, tumor hemorrhage, spontaneous and traumatic tumor breaks, heart failure associated with intra-tumor bypass of blood flow, abdominal compression syndrome, severe hypothyroidism. Diagnosis of this pathology is carried out on the basis of a set of data of medical imaging, laboratory diagnostics and clinical picture. This review presents key points of general classification, the most characteristic diagnostic signs, as well as basic algorithms of treatment of liver hemangiomas in newborns and children of the first year of life. There are also described 2 clinical cases of newborns with giant liver hemangiomas, which were examined and treated in the pathology department of newborns and infants of Perinatal Center of St. Petersburg State Pediatric Medical University with a description of the peculiarities of the course of each of them.

Pediatrician (St. Petersburg). 2020;11(5):57-65
pages 57-65 views

Niemann–Pick disease in a child with congenital cytomegalovirus infection

Bem E.V., Fedorova L.A., Sajkova M.Y., Reutskaia O.G.

Abstract

In this publication, we present own clinical observation demonstrating the early diagnosis of Niemann–Pick disease, type C (NPС) in a child with congenital cytomegalovirus infection (сCMV). Niemann–Pick disease is a rare orphan inherited disease with an autosomal recessive type of inheritance that belongs to lysosomal accumulation diseases. There are 4 clinical phenotypes of Niemann–Pick disease (A, B, C, D), among which types A and B are associated with a mutation of the lysosomal sphingomyelinase (SMPD1) gene, and types C and D are associated with mutations in the NPC1 or NPC2 genes responsible for intracellular transport of cholesterol and lipids. The disease is a form of glycosphingolipidosis, which is accompanied by progressive degenerative processes in the target organs. The incidence of NPС is approximately 1 case per 100,000 live births. During the newborn period, NPС may occur under the guise of the other inherited or acquired diseases, and sometimes be combined with them. In particular, the manifest form of neonatal cytomegalovirus infection has similar target organs for damage to the NPС: the liver, the spleen, and the Central nervous system. The multi-system nature of clinical manifestations requires a differential diagnosis and clarification as early as possible. The polymorphism of clinical manifestations and the lack of diagnostic tests for mass screening make it is difficult to diagnose the disease. In the given clinical case, the diagnosis of NPС in a child with congenital cytomegаlovirus infection was established using DNA diagnostics of NPC1 gene mutation at the age of 2.5 months of life, which allowed determining the long-term management and medical observation.

Pediatrician (St. Petersburg). 2020;11(5):67-72
pages 67-72 views

Psychogenic dysuria

Shishkov V.V., Kokurenkova P.A., Ilyichev A.B., Grechanyy S.V., Pozdnyak V.V., Khutoryanskaya Y.V.

Abstract

The causes of dysuria can be different, moreover, cases of psychogenic dysuria are not casuistry in general medical practice. Such patients complain of increased urination (pollakiuria), urinary incontinence (enuresis), urinary retention, or loss of urination. As a rule, for the first time, such patients turn to a pediatrician (therapist), then to a urologist who, after a comprehensive examination, excludes any organic violation of the genitourinary system. Psychogenic dysuria can be noted in the structure of general neurosis, be a somatoform disorder, or be a neurotic (usually conversion) monosymptom. Hysterical (conversion) disorders, by definition, are not simulative, but manipulative, representing either a way to draw attention to your person, which is typical for females, or being a kind of unconscious way out of a hopeless – from the patient’s point of view – situation, as it was in this case. For the same reasons, the treatment of patients with hysteria remains one of the most difficult problems, requiring the involvement of a specialized specialist – a psychotherapist or psychiatrist. Integrative psychotherapy (rational, behavioral and suggestive) is quite effective here, however, long-term treatment is usually required to achieve a good effect. Which consists of consultation with confrontational and paradoxical techniques, and the way out of such a confrontation is usually the same behavioral therapy and 5–10 sessions of hypnotherapy. The given clinical example is of scientific and practical interest, since the full effect was achieved after the first consultation and one session of hypnotic suggestive therapy.

Pediatrician (St. Petersburg). 2020;11(5):73-79
pages 73-79 views

Clinical psychology

On the norm in human mental development

Averin V.A.

Abstract

Various approaches and models of the concept of “mental health norm” are analysed: medical (biological), psychological, social normative, based on the principles of mechanistic determinism. As opposed to them, A.B. Cholmogorova advocates biopsychosocial models, which are based on a systemic approach. B.S. Bratus’ singles out the “statistical adaptation approach” and the “adoption of negative criteria of the norm” opposing it, as well as the “cultural relativistic and existential” approaches and the “descriptive criteria of mental health” opposing them. Searching for optimal criteria for the norms of mental development the HUMAN BEING is found to be in the centre of consideration as a result of cultural development in all its diversity: as an individual, personality, subject of activity, the integration of the variety of properties of which is expressed in individuality. The development of a human being within culture leads to the formation of a cultural consciousness, the main criteria of which are freedom, independence, creativity, self sufficiency and conscience. They can be considered as criteria for the normal course of a person’s mental development. An attempt to formulate psychological tools to achieve aims of the mental development of a human being has been made. At the level of individual-personality this role belongs to the directedness of the personality, expressing the relation of an individual to the aims of the activity. At the level of the subject of activity to the individual style of activity. The interaction of these key factors of the development of a human being ensures the internal unity of all levels of the organization of a human being, resulting in the concept of the “integrity of human being”, which fully expresses the psychological content of individuality the bearer of cultural consciousness.

Pediatrician (St. Petersburg). 2020;11(5):81-90
pages 81-90 views

Secondary correction of infertility and oncophobia by means of psychological intervention (clinical case description)

Salekhov S.A.

Abstract

The analysis of psychological correction for post-traumatic stress disorder and progressive chronic psychological stress was carried out. Strategy of the first consultation was based on the principle of A.A. Ukhtomsky, the theory of functional systems by P.K. Anokhin, A. Maslow’s pyramid of needs, taking into account the hierarchy of emotional stress and cognitive function in the structure of PS, social immobilization and the characteristics of the energy supply of the central nervous system. An algorithm for psychological counseling was developed: 1. Provocation to ensure an intense emotional reaction of suppressed aggression to a stimulus (NLP) under the control of the psychological and physiological state (NLP calibration, lie verification and profiling). 2. Inhibition of the activity of the limbic system and zeroing of emotional stress with the use of pattern interrupt (short-term confusion), followed by breathing control under conditions of transient hypoxia and overload of the representative systems. 3. Conducting resource-relaxation trance (Ericksonian hypnosis). 4. Post-hypnotic suggestion, cognitive analysis and embedding of new patterns of behavior and response to stress. 5. Teaching the method of zeroing emotional stress and reaction to external stimuli. Within 3 weeks after a single consultation, emotional stress disappeared, relations in the professional sphere normalized (transferred to a more prestigious job with a slight increase in salary), friendly relations with a neighbor-friend were restored, and mutual understanding was reached with parents. At the same time, against the background of regression of uterine hypertonicity, the painful formation in the pelvic cavity, perceived as an oncological pathology, disappeared, and the woman got pregnant.

Pediatrician (St. Petersburg). 2020;11(5):91-96
pages 91-96 views

History of medicine

The history of emergence and development of medical genetics in Russia

Gorbunova V.N., Polishchuk A.M.

Abstract

The early years of human genetics in Russia were distinguished by a pronounced interest in eugenics, pioneering methodological achievements in human cytogenetics, systematic studies of the contribution of heredity to the development of multifactorial diseases, the physical and mental human characteristics. The vast scientific infrastructure in genetics that had developed by the mid-1930s was demolished in a series of political decisions beginning with the campaigns to eradicate eugenic ideas and ending with the notorious 1948 VASKhNIL session, which entailed a complete ban on the genetics. For almost 25 years, key to the development of this science throughout the world, its progress in Russia was first completely frozen, and then retarded profoundly. The revival of medical genetics went in different ways, by virtue of many prominent specialists in various fields of human genetics, formed before the era of repression. New scientific schools were founded by them and their disciples. Despite the enormous gap in the level of domestic and world genetics, which lagged even after the ban was lifted, a series of important works in the field of radiation mutagenesis, population genetics, cytogenetics, as well as a number of world class achievements in the field of biochemical genetics and the study of metabolic diseases, should be noted. Gradually, as the state recognized the importance of genetics for medicine, an extensive medical genetic service arose in the country’s healthcare system. The conditional point of the final “rehabilitation” of medical genetics can be recognized in the emergence of departments of this discipline in medical schools throughout the country in the late 1980s. Despite the gap, still persisting between the world’s level of medical genetic science and the current state of it in our country, this gap has significantly reduced nowadays.

Pediatrician (St. Petersburg). 2020;11(5):97-110
pages 97-110 views

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