Voprosy Prakticheskoi Pediatrii最新文献

{"title":"Effectiveness of neonatal screening for cystic fibrosis in the Russian Federation","authors":"V.D.Sherman V.D.Sherman, S. S.I.Kutsev, V.L.Izhevskaya V.L.Izhevskaya, E.I.Kondratyeva E.I.Kondratyeva","doi":"10.20953/1817-7646-2022-3-12-19","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-3-12-19","url":null,"abstract":"Mass neonatal screening (NS) for cystic fibrosis (CF) has been performed in the Russian Federation since 2006–2007. It includes measurement of serum immunoreactive trypsinogen (IRT) in the first week of life and repeated testing on day 21–28 in case of positive first test. The sweat test is used to confirm the diagnosis. Objective. To evaluate the effectiveness of neonatal screening for cystic fibrosis in the Russian Federation. Materials and methods. We collected information on key parameters of NS for cystic fibrosis for 2019 from regional specialists in medical genetics. Results. We collected information form 50 regions of the Russian Federation, where a total of 1,009,832 newborns had been examined. The number of newborns with elevated IRT was 9,138 (0.9%); 1388 (0.1%) newborns tested positive at reexamination. CF was diagnosed in 104 newborns. Two pathogenic variants in the CFTR gene were detected in 78 infants with CF (75%); one pathogenic variant, in 20 infants (19%); no pathogenic variants, in 3 infants (2.9%). The NS protocol was not completed in 395 patients (4.3%). False negative screening results were observed in 8 newborns (7.1%), including two with meconium ileus. The sensitivity of screening was approximately 93%. One hundred and four infants tested positive at reexamination had no final diagnosis. The mean age at examination by a CF specialist was 41 (±21) days. More than two-thirds of newborns (73%) were diagnosed in outpatient departments, while the remaining patients (27%) were diagnosed in hospitals. We obtained mean cut-off values for IRT. Conclusion. Dynamic analysis of NS performance is a promising approach for its optimization. Key words: cystic fibrosis, neonatal screening, immunoreactive trypsinogen, sweat test, false positive result","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67744557","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of respiratory tract infections on the costs of cystic fibrosis therapy","authors":"V. Shadrina, V. Sherman, E. Furman","doi":"10.20953/1817-7646-2022-3-40-46","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-3-40-46","url":null,"abstract":"Cystic fibrosis (CF) is a severe progressive disease in which the control of respiratory infection determines the disease prognosis. Objective. To assess the impact of different respiratory tract infections on the costs of antibiotic therapy in children with CF, depending on their age. Patients and methods. Patients were divided into three groups (0–3 years, 4–10 years, and 11–18 years) for the costeffectiveness analysis; mean body weight was calculated for each group. Current regulatory documents were used to evaluate antibacterial therapy. The State Register of Medicines was used to calculate the costs of each drug. Results. We found that the highest costs were associated with inhaled antibiotics used for the eradication therapy in patients with P. aeruginosa and Achromobacter spp. infections (up to 1,152,000 RUB per year), chronic P. aeruginosa and Achromobacter spp. infections (up to 2,304,000 RUB per year), and infections caused by Burkholderia cepacia complex (up to 750,178 RUB per year). In case of intravenous antibiotics, the highest costs were associated with the treatment of infections caused by Burkholderia cepacia complex (up to 790,236 RUB per year). Conclusion. Patients with CF require regular microbiological monitoring to ensure timely detection of pathogenic microorganisms and respiratory tract infections. Early initiation of adequate antibacterial therapy in accordance with clinical guidelines is necessary to prevent chronic infections. Key words: antibacterial drugs, children, cystic fibrosis, therapy cost","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67745026","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ectopic ureter in a patient with a non-duplex hypoplastic kidney","authors":"G. I. Kuzovleva, V. V. Rostovskaya, O. Staroverov, E. Ayryan, N.A. Khvatynets, G. A. Korolev","doi":"10.20953/1817-7646-2022-4-151-154","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-4-151-154","url":null,"abstract":"Ectopic ureter (EU) is a rare urinary tract malformation often found in patients with a duplex kidney. Abnormal location of the ureteral orifice in a non-duplex kidney is observed in 20–25% cases of all ectopias. The paraurethral EU is described in very few publications. An 8-year-old female patient complained of periodic abdominal pain, inflammatory changes in the urinary sediment, and periodic urinary incontinence. The child had not been examined earlier. Ultrasonography findings: left kidney size 41 × 20mm; the ureter was dilated along its entire length and has bends; the diameters of its upper and thirds were 16 mm and up to 23 mm, respectively. Cystography showed no evidence of vesicoureteral reflux. Contrast-enhanced computed tomography findings: the orifice of the left ureter was not visualized; there was a significant decrease in the function of the left kidney and dilation of the left ureter. Findings of cystoscopy under anesthesia: the orifice of the left ureter was in the bladder; the bladder neck was not visualized. During the examination of the area between the urethra and the vagina, we found the orifice of the left ureter located to the left of the midline. The patient underwent laparoscopic left-sided nephroureterectomy. Six months postoperatively, the patient had no urinary incontinence and no urinary syndrome. Static renal scintigraphy showed that the index of integral uptake on the right was 108; the total volume of the functioning parenchyma was not reduced. Key words: ectopic ureter, nephroureterectomy, children.","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67745595","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bilateral chondromesenchymal hamartoma of the chest wall in children","authors":"O. Pikin, D. A. Morozov, O. Topilin, M. Airapetyan, O. Sukhodolskaya","doi":"10.20953/1817-7646-2022-4-118-124","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-4-118-124","url":null,"abstract":"Chondromesenchymal hamartoma (CMH) of the chest wall is a rare benign tumor arising from the ribs and affecting mainly infants and young children. Intrathoracic tension syndrome and scoliotic deformities are the leading clinical manifestations in such patients. At the same time, tumor removal, which always includes rib resection, leads to chest wall deformities. The issue of the optimal method of thoracoplasty for such lesions remains unresolved. Our study presents the 15th case of bilateral chondromesenchymal hamartoma of the chest wall among those described in the world literature. Surgical intervention to remove tumor consisted of three stages due to the bilateral lesion and ongoing tumor growth in the postoperative period. Xenopericardial plates and an artificial rib were used as materials for thoracoplasty. Key words: chest wall, children, artificial rib, xenopericardium, thoracoplasty, chondromesenchymal hamartoma","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67745740","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Horner's syndrome a child after surgery for spontaneous pneumothorax: a case report","authors":"A.A. Burovnikova, G. Rumyantseva, A. Yusufov, Y. Brevdo, S. V. Trukhachev, V. V. Svetlov, A. Kazakov","doi":"10.20953/1817-7646-2022-4-126-129","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-4-126-129","url":null,"abstract":"In this article we present a case of Horner syndrome in a teenager occurring with thoracoscopic treatment for spontaneous pneumothorax. Horner's syndrome is a rare pathological condition caused by a lesion of the sympathetic nervous system responsible for the innervation of the eye. A brief review of the available literature data on the occurrence of this complication in spontaneous pneumothorax, its etiopathogenesis, and clinical manifestations is presented. The anamnesis, clinical picture, instrumental studies, stages of operation of this clinical case are described in detail. It is assumed that Horner's syndrome is associated with tissue damage near the stellate ganglion and nerve fibers during physical pleurodesis by argon-plasma coagulation. Key words: children, pleurodesis, Horner syndrome, spontaneous pneumothorax","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67745881","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"New possibilities of anti-inflammatory therapy in the complex treatment of community-acquired pneumonia","authors":"D. Vasilková, L. Ulyanova, N. Esakova, A.Y. Pishchalnikov, O. Abramovskikh","doi":"10.20953/1817-7646-2022-4-25-31","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-4-25-31","url":null,"abstract":"Pneumonia is one of the most urgent and serious pathologies in pediatrics, the pathogenesis of which is determined by the pathogen-induced acute inflammatory response in the bronchial mucosa and lung tissue. In this regard, along with etiotropic medications, anti-inflammatory agents play an important role in the treatment of this group of patients. Objective. To evaluate the experience of using the anti-inflammatory drug ammonium glycyrrhizinate in the complex treatment of children with community-acquired pneumonia. Patients and methods. Fifty children aged 3 to 7 years diagnosed with community-acquired pneumonia participated in the observational program. Patients were divided into two groups: the control group (n = 30), which represented patients who received standard therapy, and the study group (n = 30), which represented patients who received ammonium glycyrrhizinate (AG) for 10 days in addition to standard therapy. Daily physical examination, scoring of symptoms (cough, sputum production) and adverse events were performed in the groups. Results. Patients in the study group, in comparison with the control group, showed a significantly more pronounced positive dynamics against the background of AG therapy, which was expressed in a considerable decrease in the intensity of daytime/nighttime cough already from the 3rd day of treatment. It allowed 50–60% of children to achieve complete relief of cough symptoms by day 5–6, and the remaining proportion of patients had its minimal manifestations (1 or less scores), which did not affect daytime activity or sleep (p < 0.05). The use of AG in patients in the study group reduced the presence of daytime/nighttime cough symptoms by 25% compared with the control group (p < 0.05). On the 4th day of observation, there were statistically significant differences in the incidence of fever; it was absent in a significant proportion (97%) of patients in the study group (p < 0.05). By the 7th day of treatment against the background of AG therapy, pulmonary function was restored in all patients in the study group, which was characterized by the absence of sputum production. Conclusion. Inclusion of ammonium glycyrrhizinate in the treatment of children with community-acquired pneumonia provides a rapid regression of the severity of the main respiratory symptoms of pneumonia (daytime/nighttime cough, sputum production) and a significant reduction in the duration of their presence and relief, which considerably accelerates the rate of recovery and improvement in the overall health of children, as well as the prognosis of the disease in general. Key words: pneumonia, children, rehabilitation, ammonium glycyrrhizinate","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67746541","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Features of clinical and laboratory changes in adolescents in the dynamics of novel coronavirus infection","authors":"O. Obukhova, T. Ryabichenko, G. Skosyreva, O. Gorbenko, E. Timofeeva, T. V. Kartseva, A.O. Izyumov","doi":"10.20953/1817-7646-2022-5-52-59","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-5-52-59","url":null,"abstract":"The relevance of research on a novel coronavirus infection is associated with an increase in the incidence among children since 2021, which may be due to the accumulation of mutations in the virus genome and its evolution towards increased contagiousness, replicative ability, and evasion of immune protection. While there are many studies in adults, data analyzing the clinical course of the disease in pediatric patients infected with SARS-CoV-2 are limited, particularly regarding adolescents. Objective. To study the clinical and laboratory features of the course of a novel coronavirus infection in hospitalized adolescents in Novosibirsk during the first, second and third waves of the pandemic. Мaterials and methods. A retrospective analysis of case histories of 125 children treated at Novosibirsk Children's Clinical Hospital No 6 with a confirmed diagnosis of coronavirus infection during three pandemic waves was carried out (June–August 2020, October–December 2020, June–August 2021). Based on these time intervals, three groups of adolescents admitted to the hospital during the first, second, and third waves of coronavirus infection were formed. SARS-CoV-2 RNA in nasopharyngeal and oropharyngeal scrapings was determined using the PCR-RT method. Biochemical and general clinical studies were performed in accordance with the guidelines of the Ministry of Health of the Russian Federation. Statistical processing was carried out using the Satistika 7.0 software package (StatSoft, USA). Differences between the groups were assessed using the Z-test and the Mann–Whitney U test. Differences between the compared series were considered statistically significant with a probability level of 95%. Results. It was shown that during three pandemic waves (June 2020 – August 2021), more than half of the hospitalized children were adolescents. At the same time, regardless of the pandemic wave, intoxication, catarrhal and intestinal syndromes predominated in hospitalized adolescents. CNS injury symptoms were significantly less frequent in the first wave, as were skin rashes. Cough in the third wave was observed in 100% of hospitalized adolescents. The average values of the parameters of complete blood count, as well as CRP, D-dimer and ferritin had no statistically significant differences in different pandemic waves, but there was a significant variation in individual values within the groups in each wave. Key words: adolescents, coronavirus infection, COVID-19, clinical manifestations, laboratory parameters","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67747075","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Expression of HNP1 gene in children with atopic dermatitis","authors":"O. Svitich, V. Soboleva, N. Abramova, K. Gelezhe, A. Kudryavtseva","doi":"10.20953/1817-7646-2022-6-31-36","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-6-31-36","url":null,"abstract":"The expression of innate immune genes, defensins, changes in atopic dermatitis (AD). There has been much research on β-defensins, whereas the role of α-defensins in AD has not been adequately examined. Objective. To evaluate the expression of HNP1 gene in affected and visually unaffected skin areas in patients with different clinical and morphological forms of AD. Patients and methods. The participants in this study (n = 103) were patients admitted to the Maternity and Childhood Center of Sechenov University in 2020–2022. Two skin scraping samples were obtained from each of the patients with AD (n = 89), from skin areas affected and not affected by AD; one skin scraping sample was obtained from control group participants (n = 14) with no skin diseases. To determine the expression of HNP1 in skin cells, a real-time reverse transcription polymerase chain reaction followed by statistical analysis was performed. Results. A significant increase in the expression of HNP1 gene was demonstrated in a more severe course of AD with the development of lichenoid skin changes. The elevated expression level of this antimicrobial peptide indicates the development of inflammatory reactions involving neutrophils in the inflammation area, possibly associated with colonization of the affected skin by opportunistic bacterial pathogens. Conclusion. The α-defensin HNP1 plays an important role in disease progression and can subsequently be used in the treatment of AD as a highly effective alternative approach. Key words: atopic dermatitis, children, neutrophil peptide 1, HNP1","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67748052","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hypovitaminosis D in children with congenital heart defects and its correction","authors":"E.M. Savova, Anna Y Zavarina, V. Shvedunova, N. Putyato","doi":"10.20953/1817-7646-2022-6-79-88","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-6-79-88","url":null,"abstract":"Hypovitaminosis D currently affects nearly half of the world's population. Children with congenital heart defects (CHD) are particularly prone to vitamin D deficiency. Mean level of 25-hydroxyvitamin D in children with CHD is significantly lower than that in healthy children; cyanotic heart disease is usually associated with severe vitamin D deficiency. Low levels of vitamin D in these children can be caused by surgery with artificial circulation and, therefore, predispose to complications at different stages of the postoperative period. Another problem often overlooked after surgery for CHD is hypocalcemia, which cannot be addressed without adequate vitamin D intake and can lead to early osteoporosis in children. The majority of complications, in children with CHD (including those affecting the cardiovascular system) can be prevented by timely detection of hypovitaminosis D and its treatment; however, optimal therapeutic doses for these patients have not yet been determined. It is still debatable whether children with CHD require higher doses of vitamin D than healthy children. It is also unclear whether vitamin D level should be normalized before surgery and how long should it take to reach normal vitamin D level in serum. Key words: vitamin D, hypovitaminosis D, children, deficiency, insufficiency, congenital heart disease","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67748867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between postural disorders and malocclusion and methods of their detection","authors":"F. Ayupova, T. Murashkina, T. Gayvoronskaya, E. Badeeva, A. Arutyunov, Yu. A. Vasiliev, T.V. Ponomarenko, A.R. Gabbasov","doi":"10.20953/1817-7646-2022-6-89-96","DOIUrl":"https://doi.org/10.20953/1817-7646-2022-6-89-96","url":null,"abstract":"Postural disorders and malocclusion are highly prevalent in children. Malocclusion is found in 90% of children with postural disorders. This problem requires particular attention to identify optimal methods for early diagnosis of and timely treatment for postural disorders and malocclusion, as well as comprehensive health improvement of children. We analyzed the literature data on the types of postural disorders, currently availably methods of its diagnosis, prevention, and treatment. We performed our search using СyberLeninka, E-library, and MEDLINE/PubMed databases with the following key words: malocclusion, diagnosis, treatment, postural disorders, pathological changes in the maxillofacial region, prevention. We selected 57 publications exploring the association between postural disorders and malocclusion and describing main types of postural disorders, their causes, as well as current methods of their detection, prevention, and treatment. We identified difficulties hindering widespread use of the existing methods for the diagnosis of postural disorders. We believe that it is very important to improve available diagnostic methods and create new ones; we discuss the possibility of their development and implementation into routine clinical practice. Key words: malocclusion, diagnosis, treatment, postural disorders, pathological changes in the maxillofacial region, prevention","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67749771","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}