Vol 9, No 1 (2018)

Articles
Peculiarities of the construction of the placentary complex and the main causes of perinatal losses
Ivanova L.A., Titkova E.V.
Abstract

The article presents the analysis of the massometric parameters of the fetoplacental complex (weight of the placenta of full-term newborns, placental-fetal coefficient (PPC), linear mass of the umbilical cord (LMU). Indicators that alarm the neonatologist doctor as possible markers of perinatal losses are: a decrease in the placenta mass, an increase in PPC, high LMU (a consequence of ascending bacterial infection (funiculitis) and the development of circulatory umbilical edema in acute right ventricular failure, for example, in viral atrial myocarditis). A histological study of newborn children of the main and control group was carried out to identify the main types of histological structure of the placenta: normal structure, ascending infection of the placenta, hematogenous infection of the placenta, chronic placental insufficiency, acute placental insufficiency. revealed a different frequency of occurrence of these conditions in patients of the main and control groups. The main causes of perinatal losses are analyzed. It is determined that more than half of cases of perinatal fetal death is infectious pathology: viral, bacterial and mixed viral-bacterial. About a quarter of cases of perinatal death is caused by acute heart failure with placental abruption and a complex of cardiovascular and respiratory disorders in premature infants. In 18% of cases, congenital malformations of the fetus lead to perinatal death. CRF causes perinatal fetal death in approximately 10% of cases.

Pediatrician (St. Petersburg). 2018;9(1):5-10
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Features of species composition of intestinal bifidobacterium and microbial metabolism profile in children of the first half of life depending on the way of delivery
Bulatova E.M., Shabalov A.M., Bogdanova N.M., Shilov A.I., Oganesiyan E.G., Kuritsyna N.S.
Abstract

Changes of intestinal microbiome in first year of life, related with cesarean section, are the most unfavorable and have long-term negative immunological and metabolic effects. The aim of the study. Evaluate the species composition of Bifidobacterium, range of Short-chain Fatty Acids (SCFA) in feces in children, who were born by natural way or with cesarean section, for clarification their diagnostic value in the process of formation of microbiocenosis in children. Materials and methods. Exa mined 60 children in the age of 4-6 months. 48 children were born in natural way and 12 were born by cesarean section. All of them were full-term and were on breastfeeding. Functional disorders of digestion were noted in 49 children (81,7%). Researchs that were conducted: microbiological examination of feces, typing of Bifidobacterium (PCR), definition of SCFA (C2 – acetic acid, C3 – propionic acid, C4 – butyric acid, C5 – valeric acid, C6 – caproic acid) content in feces. Results. III-grade dysbiotic changes in intestines were found significantly less in natural-way born (NW) children in comparison with cesarean section (CS) born children (14,58 и 33,33%; p < 0,05). In natural-way born children, infant strains of Bifidobacterium were not detected just in 12.5% but after cesarean section in 25% (p < 0,05). One infant strain of Bifidobacterium was in 41,67% of NW born children and in 25% CS born children (p > 0,05). Established, that content of acetic acid was significantly higher in NW born children (1,59 ± 0,75 и 1,04 ± 0,56; p < 0,05). Conclusion. In CS born children, impoverishment of the intestines by infantile species of Bifidobacterium, lower content of acetic acid, were detected significantly more often. It shows decrease of activity and amount of obligatory anaerobic bacteria (Bifidum-, Lacto-) in intestines, increased activity of opportunistic flora. That can be used in practical healthcare with diagnostic purpose.

Pediatrician (St. Petersburg). 2018;9(1):11-16
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The benefits of primary prevention of colorectal cancer around the age of 20 compared to secondary prevention after the age of 50
Shemerovskii K.A., Shabanov P.D., Mitreikin V.P., Seliverstov P.V., Iurov A.Y.
Abstract

The major advantages of primary prevention of colorectal cancer in young adults (around 20 years) compared with se condary prevention in adults (after 50 years). Primary prevention is aimed at screening and eliminating one of the major proven risk factors for colon cancer – chronic constipation and its predecessor – colorectal bradyarrhythmia – as slower circadian rhythm of defecation. The main advantages of primary prevention of colorectal cancer are: non-invasiveness, safety, efficiency and timeliness. Screening of colorectal bradyarrhythmia at 2501 operating physician is allowed to diagnose this arrhythmia in 44% of the surveyed entities. There is discovered the new fundamental dependence between the regularity of circadian rhythm of defecation and an acrophase of this rhythm. The implementation of the morning acrophase of the circadian rhythm of defecation is the key mechanism of the regularity of this rhythm. Repeated screening of colorectal bradyarrhythmia in individuals around the age of 20 years (a month after informing students about the law of circadian rhythm in the brain and intestine) showed that 53% of the students began to observe the morning routine of bowel movements. This led to a transition of moderate stage of colorectal bradyarrhythmia severity (3-4 times a week) in an easy stage (5-6 times a week). It’s proved the possibility of primary (behavioral) prevention of colorectal bradyarrhythmia (colorectal coprostasis) as a risk factor for colorectal cancer. Secondary prevention of colon cancer is associated with the invasiveness of colonoscopy, its possible complications (bleeding, perforation of bowel and fatal outcomes), the high cost of special equipment and late in the approach to screening for colorectal cancer (after the appearance of blood in the stool or after 50 years).

Pediatrician (St. Petersburg). 2018;9(1):17-22
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Forensic medical assessment of morphological changes in the myocardium, affecting its contractile capacity in cases of death from alcoholic cardiomyopathy
Sokolova O.V., Yagmurov O.D., Nasyrov R.A.
Abstract

A retrospective analysis of acts of forensic medical autopsies from the archive of BSME and a histological study of myocardial tissue in 180 cases (87 women and 93 men) were carried out with statistical processing of the obtained results for the purpose of studying and assessing the morphological changes in the main components of the histohematological barrier of myocardium, affecting the contractility of the cardiac muscle in cases of the death from alcoholic cardiomyopathy. As a result of the study, it was found that the occurrence of metabolic disturbances due to the toxic effects of ethanol and its metabolites contribute to the development of hypoxia of the heart muscle with the development of dystrophic and irreversible necrobiotic processes in it, which in its turn play a direct role in the formation of excitability processes, contractile function of myofibrils with the development of fatal rhythm disorders. The morphological changes in the contractile apparatus of the myocardium were discovered in the study in polarized light and can be used to diagnose alcohol damage to the heart. During the study of deaths from alcoholic cardiomyopathy in forensic medicine, it is recommended to use methods of polarization microscopy.

Pediatrician (St. Petersburg). 2018;9(1):23-28
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Amblyopia and associated problems
Kononova N.E., Somov E.E.
Abstract

Both earlier and now amblyopia associated with strabismus, remains a significant problem in pediatric ophthalmology, as evidenced by the large number of works, dedicated to this question, and not very high efficiency of treatment of patients with this disease. In our view, the current definition of amblyopia is very vague, as not clearly indicate its origin and distinctive clinical features. Different views on the essence of amblyopia has caused the emergence of several different classifications. So, E.S. Avetisov (1968), for example, proposes to assess the severity of amblyopia according to the reduction of central vision, and E.E. Somov (1997) – saving the character of his vision. And it is, as you know, is an integral indicator of the functional status of the visual analyzer. This is a really important point, because, as a rule, an essential companion of amblyopia of any origin are violations detected in binocular vision. Thus, good results of held pleoptic, that physicians usually evaluate the treatment outcomes of children with strabismus, should not be considered as final. We evaluated the functional status of the organ of vision of 91 children with amblyopia, associated with strabismus. In addition to the standard for such cases methods, we also used others – new or in our modification: the determine the of the nature of vision with Bagolini glasses, indication of the nature of visual fixation, of critical flicker fusion frequency (CFFF), evaluation the nature of visual fixation, optic coherence tomography (OCT) of the macular zone of the retina. Identified in the visual status of examined children of this age specialties allowed to develop for those who have a central visual fixation, adequate therapeutic algorithms, presented below.

Pediatrician (St. Petersburg). 2018;9(1):29-36
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Developmental Features of Corpus Callosum in Children Revealed by MRI
Zykin P.A., Yalfimof A.N., Aleksandrov T.A., Krasnoshchekova E.I., Tkachenko L.A., Sereda V.M., Nasyrov R.A.
Abstract

With the wide use of magnetic resonance imaging (MRI) in clinical practice, more attention is paid to corpus callosum hypoplasia in children with various central nervous system diseases, including the cerebral palsy. We compared the areas of corpus callosum segments on the mid-sagittal MR images of the children with cerebral palsy and a control group; full-term infants and preterm infants. During the postnatal development, overall callosum area naturally increases, but single segments change differently in both absolute and relative values. In the control group, the relative area of the splenium grew insignificantly, whereas for the genu, it was stable. Children with cerebral palsy also show age-specific growth, but the area of corpus callosum is smaller compared with equal age children in the control group. We found an increased relative area of corpus callosum genu and a decrease of anterior body and splenium in the cerebral palsy group compared with the control group. The kCC index shows higher values in the control group than in any age subgroup of children with cerebral palsy. Mean values for the control group steadily increase with age, whereas in the cerebral palsy group, they remain the same. For every age-specific group, the difference of kCC was statistically significant. Mean kCC index values depend on gestational age and are statistically lower in preterm infants compared with full-term infants. Selective corpus callosum hypoplasia found in the current research could be due to Wallerian degeneration or a decreased number of axons in some of its segments. This could be explained by the disruption of neurogenesis in certain cortical areas. The morphometric index kCC can be used to detect deviations in the corpus callosum structure associated with prematurity and cerebral palsy.

Pediatrician (St. Petersburg). 2018;9(1):37-48
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The features of hemodynamic support of exercise stress in patients with neurocirculatory dystonia
Zotov D.D., Isakov V.A.
Abstract

Neurocirculatory dystonia occupies a special place among the most common cardiovascular diseases. On the one hand, it is almost the most often diagnosed cardiologic ambulatory disease in young patients, on the other hand, its clinical-nosological framework and diagnostic criteria have remain not completely defined just as decades ago. One of the characteristic manifestations of neurocirculatory dystonia is a reduction in physical capacity of patients, due both to the lack of strong-willed qualities and detraining, and disorders of oxygen and hemodynamic support of physical activity. In this study, it was shown that one of the factors limiting exercise tolerance in patients with neurocirculatory dystonia, is the lack of growth of parameters of cardiac output at all levels of exercise stress. These features of hemodynamic support of physical activity were most pronounced in patients with low tolerance to the load, while in the group with high physical ability they were in limits of statistically non-significant trends. The recovery period in patients neurocirculatory dystonia was extended: heart rate, in contrast to the healthy persons, did not return to initial values even for 10 minutes. Throughout the recovery period, patients remained observed at the peak of the load relative reduction in stroke and cardiac indexes. Perhaps one of the causes of hemodynamic dysadaptation in NDC patients to physical activity and reduced physical performance is a violation of their orthostatic reactions, the lack of mechanisms that ensure adequate venous return and cardiac output. Hyper-reactivity of the cardiovascular system, manifested in a consistent trend of tachycardia and increased peripheral vascular tone, may play a compensatory role.

Pediatrician (St. Petersburg). 2018;9(1):49-53
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The organization of medical-social work among children
Orel V.I., Kim A.V., Sereda V.M., Bezhenar S.I., Roslova Z.A., Rubezhov A.L., Buldakova T.I., Rukavishnikov A.S., Libova E.B., Sharafutdinova L.L., Gureva N.A.
Abstract

At the heart of modern conceptual approaches to the development of children’s health, especially the primary care, along with the other is the principle of the evaluation and analysis of environmental factors influencing the health of the child population, and as a consequence, organizational search technologies, having a maximum socialization and effectively integrated into such an environment. It is a complex medical-social approach to monitoring the lives and health of every child allows to achieve the most positive results. In everyday practice a direct part of the pediatric service in addressing social issues arise most often in cases of a clear threat to the life or health of the child. Early diagnosis of adverse society, medical support, social adaptation of the child or are not conducted or are conducted in a very limited extent. However, according to the number of researchers in recent years, the proportion of children, which they can and should be attributed to the categories of social risk, increases and amounts according to various sources 10-25% [5, 8]. First of all, the children in difficult life situations, children from large and dysfunctional families, children with disabilities, children in care, young mothers, children and adolescents are prone to a variety of offences, vagrancy. In modern conditions improvement of the system of medical support of the child population should be provided not only on ways to improve the efficiency and quality of diagnostic and therapeutic aid, but also intensive development of health and social care. So in the decree of the President of the Russian Federation from June 1, 2012 No 761 “On the National strategy of actions in interests of children for 2012-2017” include the need for the development and implementation of modern and efficient technologies of medical and social support children and adolescents.

Pediatrician (St. Petersburg). 2018;9(1):54-60
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Regional features of early neonatal mortality (data on the Belgorod region for the period 2012-2015)
Procenko E.A., Gurova M.M., Podsvirova E.V., Volobueva S.V., Romanova T.A., Popova V.S., Burtseva Y.V.
Abstract

Early neonatal mortality occupies one of the leading places in the structure of infant mortality, which determines the medical and social significance of this problem. The aim of the study was to study regional features of the structure and causes of early neonatal mortality. The study was carried out on the basis of data from the Department of Pediatric Pathology of the Belgorod Regional Pathoanatomical Bureau for the period from January 2012 to December 2015. It was found that early neonatal mortality decreased from 2.9‰ in 2012 to 2.5‰ in 2015. It was shown the presence of the two main peaks of mortality occurring on the first and third days of a child's life. It was established that the main proportion of children with early neonatal death was children with extremely low body weight (34.7%). These children were born in the result of premature birth – in 60% of cases in 2012 with the growth of the indicator to 71.4% in 2015. The main causes of early neonatal mortality were: 1) respiratory disorders – respiratory distress syndrome of newborns, pneumonia, and aspiration of meconium – 51%; 2) congenital anomalies – multiple congenital malformations and congenital heart defects – 12.5%; 3) non-traumatic intraventricular hemorrhages – 13.5%. 61.2% of pregnant women had pathological conditions during pregnancy. Among the pathological conditions of pregnant women, most often identified chronic feto-placental insufficiency and intrauterine infections with infection of the placenta (moderate and severe) in 78% of cases. Among the pathogens of intrauterine infections dominated chlamydia (more than a third of cases), fungi of the genus Candida, ureaplasma and mycoplasma.

Pediatrician (St. Petersburg). 2018;9(1):61-67
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Algorithm for detecting suicidal risk in adolescents
Kononchuk V.V., Davydov A.T., Tyukavin A.I.
Abstract

Studied that in general educational establishments, military commissariats during the initial staging of teenagers on the military account, upon applicants in Universities to identify suicidal risk guidance documents are not provided and are not carried out, and a statistic of children's and teen suicides in Russia exceeds the world average in 2,7 times. Found that adolescents with a high level of mental adaptation indicators of suicidal risk, and the level of mental adaptation increased the proportion of adolescents with suicidal risk. In adolescents with the right-side brain lateralization of suicidal risk indicators identified in 3 times more frequent than in adolescents with left-side lateralization. In patients with right-sided brain laterali zation indicators of suicidal risk only for those individuals who were originally in a group “maladaptive”, as well as persons who were des adaptations to the dynamics of this group of groups with higher mental adaptation, even assuming that this exclusion was staging with a subsequent improvement. For adolescents with a left-handed lateralization peculiarity is not typical. It is shown that in groups with low levels of mental adaptation increases significantly the proportion of persons with the presence of suicidal risk. The algorithm of early objective risk assessment of suicide mass surveys and prediction of suicidal behavior in teenagers. Using this algorithm, for the first time, you can diagnose the deterioration of mental status in dynamics on dynamic reduce the level of mental adaptation and promptly implement their correction to prevent suicidal behavior. The algorithm provides for active participation of surveyed adolescents and their parents (guardians) with observance of all legal provisions in the diagnosis level of mental adaptation and measures to prevent suicides.

Pediatrician (St. Petersburg). 2018;9(1):68-71
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Import Substitution Possibilities in Palliative Medicine
Gavschuk M.V., Gostimskii A.V., Bagaturiya G.O., Lisovskii O.V., Zavyalova A.N., Karpatskii I.V., Kosulin A.V., Gostimskiy I.A., Aladjeva E.E.
Abstract

Nutrition is an important problem of palliative care. If oral feeding is not possible, percutaneous endoscopic gastrostomy (PEG) is the method of choice. The wide application of the procedure is limited by the cost of single-use sets produced in other countries. The aim of the study was to review methodic of the PEG and to find an opportunity to reduce its cost. A reusable device was developed for the application of the PEG with use of a Pétzzer catheter. Approbation of the device and methodic was carried out in experiment with 10 rabbits. In 2 cases animals died on the 5th and 6th day after the operation because of acute pneumonia and enterocolitis, complications from the operating wound and gastrostomy were absent. Remaining 8 rabbits were withdrawn from the experiment on the 10-13 day after the operation. In 3 cases purulent infection of the postoperative wound and formation of abscesses of abdominal cavity were revealed, while the gastrostomic fistula was without any signs of failure. In 5 cases were no complications. The gastrostomic fistula was placed next to laparotomic wound and was not complicated in all cases. All the described complications are considered to features of laparotomy and postoperative period in animals. The constructed analogue of the PEG allow significantly reduce costs and increase the economic efficiency of minimally invasive gastrostomy, reduce dependence on foreign materials. Encouraging results obtained in animal experiments allow testing of the technique in clinical settings.

Pediatrician (St. Petersburg). 2018;9(1):72-76
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Functional dyspepsia and chronic gastritis
Uspenskiy Y.P., Baryshnikova N.V.
Abstract

Diseases of upper parts of digestive tract are extremely widespread pathology and have not only the medical, but also social importance since often occur at young working-age people. It is actual to separate the items: functional dyspepsia (FD) and a dyspepsia associated with Helicobacter pylori because it is important for optimization of patient’s treatment. The review of modern foreign and Russian references about treatment of dyspeptic patients depending on existence or absence of an infection of Helicobacter pylori is presented in article (recommendation of V Maastricht consensus, Kyoto consensus, Russian gastroenterological association, Science society of Russian gastroenterologists). At diagnosis of a functional dyspepsia, it is recommended to be guided by the Roman criteria of the IV revision. In with document not only importance of an exception of organic pathology of digestive tract as the reasons of a secondary dyspepsia, but also need of inspection for the purpose of verification of Helicobacter pylori for an exception of an etiological role of a microorganism in development of a dyspepsia is emphasized. Allocation of two clinical options of a functional dyspepsia is also proved from the point of view of approaches to therapy of this disease. Algorithms of treatment of patients with various options of a dyspepsia are presented in article. These algorithms include different schemes of eradication therapy in case of dyspepsia associated with Helicobacter pylori and prokinetics/acid suppressors in case of functional dyspepsia.

Pediatrician (St. Petersburg). 2018;9(1):77-83
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Treatment of high-risk patients with severe aortic stenosis by the use of transcatheter aortic valve implantation
Sizov A.V., Sergeev A.S., Shloydo E.A., Suchov V.K., Zvereva V.V.
Abstract

Aortic stenosis is the most common disease from the group of acquired heart diseases. The frequency and degree of damage of the aortic valve increases with age. The prevalence of this disease increases due to the general extension in life expectancy of the population. Aortic valve stenosis caused by calcification is the pathology with progressive course characterized by a poor prognosis at the stage of expanded symptoms. Five-year survival of patients with developed clinical picture of aortic stenosis without surgery, according to some estimates, is reduced to 15%, which is the worst figure, even in comparison with most potentially incurable cancer. In the treatment of aortic stenosis aortic valve replacement is the “gold standard” and gives positive results in all age groups of patients. Only one third of patients are sent for aortic valve replacement owing to high risk caused by the age, severity of the defect, reduced contractility of the left ventricle, pulmonary hypertension and other comorbidities. Aortic valve replacement in patients of the older age groups with concomitant diseases is associated with high level of hospital mortality. One of the attempts to reduce the frequency of complications and mortality in this group of patients is the implementation of a less traumatic surgery – transcatheter aortic valve implantation. At present our clinic has successfully performed more than 70 operations of transcatheter aortic valve implantation. Transcatheter aortic valve implantation is a good alternative to standard aortic valve replacement in patients with high risk of open-heart surgery.

Pediatrician (St. Petersburg). 2018;9(1):84-89
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Neuroendocrine tumors of the pancreas: from theory to practical recommendations
Morgoshiia T.S.
Abstract

The article shows that a significant proportion of neuroendocrine tumors of the pancreas are non-functional, i.e., not secreting into the blood a variety of gastrointestinal hormones and polypeptides and as a result, not accompanied by typical clinical manifestations. Unfortunately, often the scan search starts in the detection of distant metastases of neuroendocrine tumors and development of cancer cachexia. Noted that the diagnosis of neuroendocrine tumors of the pancreas is a highly complex task, the solution of which depends on the choice of treatment and its remote results. Currently, the only radical method of treatment of hormonally functioning tumors of the pancreas is surgical. It is indicated that symptomatic therapy can be considered as a stage of preoperative preparation of the patient. The indication for surgical treatment in this situation, along with the ineffectiveness of conservative treatment is the size of detectable formations of 2 cm or more, which is a risk factor of distant metastases. Stressed that according to modern views of patients with nonfunctioning neuroendocrine tumors of the pancreas are subject to surgical treatment. Given that in 70-92% of cases they are malignant, most often used distal or extensive pancreatoduodenal resection. Currently, noted that the large size of the tumors are not a contraindication to surgery and if the tumor dormant, to 1 cm, the possibility of a short observation. Any tumors found in the pancreas, needs to be taken out of the context, or if it is possible – is widely excised. The article examines the rational and integrated use of modern Arsenal of surgical and therapeutic methods, which enable significantly prolong life, improve its quality in patients with metastatic forms of neuroendocrine tumors of the pancreas.

Pediatrician (St. Petersburg). 2018;9(1):90-99
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Congenital chicken pox: a clinical case of a child at the age of 6 days
Timchenko V.N., Kaplina T.A., Bannova S.L., Subbotina M.D., Sukhovetskaya V.F., Bulina O.V.
Abstract

Chicken pox can be both acquired and congenital. The information is presented in the form of a diagram on the incidence of chickenpox in St. Petersburg, which shows that among children, the most common are children aged 3 to 6 years. A brief etiology of the varicella virus, a clinical picture, describing the typical and atypical forms of the disease. Due to the airborne pathway and the high infectivity of the infection, there is a risk of contracting seronegative pregnant women, which is 50%. It has been established that for 1,000 deliveries there are from 1 to 5 cases of chicken pox. In addition, the flow of chicken pox and lethality depends on the possibility of transmitting from mother to child protective specific maternal antibodies. Features of infection in pregnant women and fetus, the severity of intrauterine infection, depending on the timing of infection are described. The characteristics of varicella and neonatal chickenpox syndrome, as well as nonspecific clinical manifestations of the infectious process in the newborn are presented. We describe our own clinical observation of congenital varicella in a 6-day-old child, born from a mother who fell ill four days before the birth. The inclusion in the complex therapy of children with congenital chickenpox recombinant interferon-α2β with antioxidants (viferon rectal suppositories) contributes to the rapid positive dynamics of clinical symptoms, easier flow, prevents the layering of acute respiratory infections, reduces the material costs of treatment.

Pediatrician (St. Petersburg). 2018;9(1):100-105
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Diagnostics and treatment the deficiency of biotinidase in practice of the children’s neurologist
Poteshkina O.V., Artyushkina J.N., Shchugareva L.M., Povzun A.A., Savelyeva E.A., Ivanov D.V.
Abstract

Biotinidase deficiency is a hereditary metabolic disease from the group of organic acidurias with an autosomal recessive type of inheritance. The disease is caused by mutations in the BTD gene, which encodes an enzyme biotinidase. Deficiency of biotinidase leads to insufficiency of intracellular Biotin, which is the coenzyme of four carboxylases involved in gluconeogenesis, leucine metabolism and biosynthesis of fatty acids. At infringement of function of carboxylase accumulate substrates that are toxic to the human body. Deficiency of biotinidase is manifested primarily by neurocutaneous disorders. The Central nervous system is particularly vulnerable, since the activity of biotinidase in the human brain is very low and therefore, for the normal functioning of neurons, it is necessary to receive enough biotin through the blood-brain barrier. With its deficiency, neurological disorders for a certain period may be the only sign of the disease. Symptoms can be successfully cured or prevented by the introduction of pharmacological doses of biotin. The article presents two clinical observations of young children with biotinidase deficiency, the main manifestation of which in the first clinical case were neurological disorders, in the second – respiratory disorders. To confirm the diagnosis, an enzyme diagnosis was carried out, which revealed a low level of biotinidase. The rapid and pronounced efficacy of biotin therapy with cessation of attacks, the possibility of cancellation of anticonvulsants, regression of neurological, skin and respiratory disorders.

Pediatrician (St. Petersburg). 2018;9(1):106-111
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Surgical treatment of congenital kyphosis caused by multiple vertebral malformations
Kosulin A.V., Elyakin D.V., Okhlopkova E.I., Pridatko O.G., Klybanskaya Y.V., Dvoretskiy V.S.
Abstract

Clinical case of successful operative treatment of congenital kyphosis is reported. Deformation was caused by multiple lower thoracic and lumbar vertebral body abnormalities. Associated spinal abnormalities were outspread absence of laminae, hydromyelia, and diastematomyelia. Preoperative computed tomography analysis revealed absence of instrumentable bony structures within malformed lower thoracic and lumbar spine. As instrumented fusion procedure was technically impossible anterior fusion without internal fixation was performed. Autogenous rib was used as a bone graft. Bone graft was extended with calcium sulphas (osteoconductive media), demineralized allograft bone matrix (osteoinductive media), and patient’s bone marrow aspirate (osteogenic media). Resulting combined bone grafting material possessed osteoconductivity, osteoinductivity, ostegenicity, and mechanical strength. At 18 months post operation follow-up computed tomography revealed solid bony fusion formation. Some neurologic improvement also was noticed. Congenital kyphosis caused by multiple outspread vertebral malformations remains insufficiently studied clinical problem. Affected children need individual multidisciplinary surgical approach, meticulous surgical planning, and regular neurological, imaging, and orthopedic follow-up until the end of bone growth. Absence of instrumentable bony structures in desired spinal arthrodesis does not preclude successful fusion. Combination of several heterogenous bone grafting materials with different properties provides fusion in difficult clinical setting.

Pediatrician (St. Petersburg). 2018;9(1):112-117
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New principles of diagnosis and classification of the Ehlers-Danlos syndrome
Arsentev V.G., Kadurina T.I., Abbakumova L.N.
Abstract

Ehlers-Danlos syndrome (EDS) is a heterogeneous group of monogenic diseases caused by a violation of collagen metabolism, the structure and function of myomatrix and the synthesis of proteoglycans. This pathology is characteri zed by hyperelasticity of the skin, subcutaneous globules, overextension of the joints, tissue vulnerability and hemorrhagic syndrome. EDS is one of the seven hereditary connective tissue disorders for which international diagnostic criteria are met. More than 30 years ago, the so-called Berlin nosology of hereditary connective tissue disorders was first compiled and approved (1986). For a long time, doctors used the “Villefranche Nosology” classification of EDS, adopted in 1998 and divided the disease into 6 types. The new criteria were published by the International Committee of Experts in 2017. In the clinical classification of EDS, 13 types with different inheritance, clinical features and biochemical defects are described. In most cases, it is inherited by an autosomal dominant type. True prevalence is unknown due to the complexity of verification and a large number of light forms, the frequency of diagnosed cases is 1 : 5000 births, severe forms are rare (1 : 100 000). Diagnosis of this syndromeis also based on the diagnostic criteria of the international classification. The lecture presents new data on classification diagnostic criteria of EDS, polymorphism of the clinical picture, genetic heterogeneity, the main principles of treatment of the disease. The new classification criteria take into account, in the main, the features of the clinical picture, they did not simplify the diagnosis, but they increased the specificity and increased the significance of the clinical and anamnestic features. The scope of the examination is determined by the presence of leading clinical signs. The genealogical examination and molecular genetic methods of diagnostics are of great importance.

Pediatrician (St. Petersburg). 2018;9(1):118-125
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