Pediatria. Journal named after G.N. Speransky最新文献

{"title":"ROBOT-ASSISTED INTERVENTIONS IN PEDIATRIC ABDOMINAL SURGERY: RUSSIA’S INITIAL EXPERIENCE","authors":"Y. Kozlov, S. Poloyan, E. V. Sapukhin, A.S. Strashinskiy, M. V. Makarochkina, A. A. Marchuk, A.P. Rozhanskiy, A. A. Byrgazov, S.A. Muravyov, A. N. Narkevich","doi":"10.24110/0031-403x-2024-103-3-111-121","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-111-121","url":null,"abstract":"Purpose of this research was to apply the possibilities of robot-assisted surgery technology in children with abdominal pathology in terms of assessing feasibility and evaluating postoperative results. Material and methods used: the scientific study was carried out at the Irkutsk Oblast Regional Children's Clinical Hospital (Irkutsk, Russia) in Dec. 2022-June 2023. During this period, 11 robot-assisted procedures were performed on the abdominal organs. The study was conducted within the framework of the Clinical Trials Program for Medical Products approved by the Federal Service for Surveillance in Healthcare of the Russian Federation. All surgical procedures were implemented according to the same principles and patterns that the Authors have gathered previously with the Versius Surgical Robot. In all cases, the three robotic ports were used: a single 12-mm trocar for optics (intervened at the umbilicus) and two 5-mm trocars for robotic arms. Additionally, another 5-mm laparoport was installed for the assistant surgeon to work during the robotic intervention. During the study, patients' perioperative parameters related to data charts, surgical details and both early and long-term outcomes were recorded. Results: the total number of patients involved in robotic interventions on the abdominal organs was 11, 4 (36,4%) boys/7 (63,6%) girls. The mean age at the time of surgery was 10,9±5.1 y/o (Me 12.0 [7.0; 15.0] y/o), mean weight at the time of surgery was 39.7±18.0 kg (Me 43.0 [24.0; 53.8] kg). The distribution of reasons for surgical interventions was as follows: 3 (27,3%) patients with Nissen fundoplication, 3 (27,3%) patients with cholecystectomy, 2 (18,2%) patients with Heller esophagomyotomy with Dor fundoplication, 2 (18,2%) patients with splenic cyst fenestration and a single (9%) patient with enucleation of ovarian teratoma. The mean duration was 100±52.4 min (Me 70.0 [65.0; 122.5] min). None of the operations were accompanied by any intraoperative complications. There were also no conversions into laparoscopic and/or open surgeries. In the long-term period after the operations, the number of significant complications in the form of recurrence of symptoms of the initial disease did not increase, patients showed no digestive problems during the entire follow-up period as well. Conclusion: Russia’s initial experience with robotic-assisted abdominal surgery confirmed that robotics can be safely and effectively used in children. Fundoplication, esophagomyotomy and cholecystectomy are by far the most commonly performed basic interventions in pediatric surgery. Obviously, this is the first stage in the development of robotic abdominal surgery in children hence it would further involve a more complex procedure.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"12 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141340761","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":"PHYSIOLOGICAL BASIS OF CARDIONEUROABLATION AND ITS CLINICAL APPLICATION IN CARDIAC ARRHYTHMIAS","authors":"I. A. Kovalyov, V. M. Solovyov, L. V. Egorov","doi":"10.24110/0031-403x-2024-103-3-159-165","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-159-165","url":null,"abstract":"Increased tone of the parasympathetic part of the autonomic nervous system, affecting the pacemaker cells, may cause sinus bradycardia, develop long pauses of heart rhythm, paroxysmal or permanent atrioventricular block followed by reflex syncope. The usual reflex syncope treatment is conservative and includes avoiding situations that trigger syncope (prolonged standing, sight of blood etc.), tilt-training, adequate drinking regime, wearing compression underwear, sleeping with the head of the bed elevated and in some cases stimulating neuro- and vegetotropic therapy. Some patients are refractory to conventional methods of treatment and retain severe symptoms that worsens quality of their life seriously. These patients may be required a pacemaker implantation subsequently. Cardioneuroablation is an alternative treatment in patients with overactive parasympathetic nervous system that is based on radiofrequency catheter ablation of the major parasympathetic autonomic ganglia around the heart.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"25 10","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141341296","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":"CLINICAL CASE OF PARANEOPLASTIC NEUROLOGICAL SYNDROME IN A CHILD WITH NEUROBLASTOMA","authors":"T. Kadricheva, S. O. Falaleeva, I. Demyanova, M.V. Borisova, E. V. Shishkina, A.V. Krasitskiy, V.E. Kazakova, L.S. Karpova, E.O. Yashina, E.A. Lyamina","doi":"10.24110/0031-403x-2024-103-3-166-171","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-166-171","url":null,"abstract":"Paraneoplastic syndromes are of particular difficulties in establishing a timely diagnosis of a malignant tumor esp. in cases when the decease development is occurred chronologically much earlier than the appearance of its specific symptoms and the neoplasm growth’s first signs. And the clinical manifestation can be represented solely by an isolated clinic of paraneoplastic syndrome, which in its turn is much alike the symptoms of the paraneoplastic neurological syndrome (PNS). Authors represent a clinical case of a severe PNS coupled with the central nervous system (CNS) lesion, mainly the cerebellum, which was manifested clinically by ataxia, tremor, myoclonus, dysartria, cognitive and mental impairment and psychotic disorders, which in its turn had delayed the timely diagnosis of neuroblastoma (left-sided intermixed adrenal ganglioneuroblastoma with metastatic tumors in ipsilateral lymph nodes and paranephric fat, deletion 1q, deletion 1p36 and MYCN amplification - all negative and INSS stage 2B) in a 5 y/o male patient. No ‘classical’ opsoclonus-myoclonus was a matter of diagnosis due to the opsoclonus absence during the whole disease course. This clinical case observation publication is having a practical purpose of pinning attention to paraneoplastic syndromes among practitioners of wide spectrum: neurologists, infectionists and pediatricians.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"104 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141342143","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":"CLINICAL CASE OF COL2A1-ASSOCIATED PROGRESSIVE PSEUDORHEUMATOID DYSPLASIA","authors":"I. Vorontsova, O. Shchagina, D. Korostin, A. Glazyrina, A. A. Sautenko, E. Demina, E. Petryaykina, V. Kenis, A. Tyulpakov","doi":"10.24110/0031-403x-2024-103-3-190-198","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-190-198","url":null,"abstract":"Modern medical scientific sources offer descriptions of cases of hereditary bone dysplasias occurring under the guise of rheumatoid arthritis (RA). These include the progressive pseudorheumatoid dysplasia (PPRD), an autosomal recessive disease caused by defects in the CCN6 gene, as well as autosomal dominant forms of pseudorheumatoid dysplasia associated with variants in the COL2A1 gene. Though there are no references to pseudorheumatoid dysplasia case observations in available domestic sources. This Article represents a discussion over the bibliographic sources on PPRD and a clinical case observation of a patient initially observed for RA but diagnosed subsequently with an autosomal dominant form of bone dysplasia caused by a de novo variant in the COL2A1 gene.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"32 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141344021","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":"SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS, MACROPHAGE ACTIVATION SYNDROME AND CARDIOPULMONARY INVOLVEMENT IN RHEUMATIC DISEASES: A RHEUMATOLOGIST’S MUST-KNOWS","authors":"K. Belozerov, M. Kostik","doi":"10.24110/0031-403x-2024-103-3-146-158","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-146-158","url":null,"abstract":"Systemic juvenile idiopathic arthritis (sJIA) is the most severe variant of JIA that can be characterized by a life-threatening course. sJIA manifests with fever, arthralgia/arthritis, hepatosplenomegaly, leukocytosis and systemic inflammation markers. Macrophage activation syndrome (MAS) is the most severe complication of this disease. This Article represents up-to-date information on pathogeneses of sJIA and MAS, describes approaches to diagnosis, treatment of both uncomplicated and complicated forms of sJIA and refractory variants of the MAS course. Current data regarding the monitoring of sJIA therapy effectiveness and safety is presented as well. Predictors for the most recently discovered, i.e. relatively new “target organs” in patients with sJIA and cardiopulmonary disorders in particular are given.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"91 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141342320","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":"HERPESVIRUS INFECTIONS REACTIVATION IN ONCOLOGICAL PEDIATRIC PATIENTS","authors":"S. Lapaeva, Y. Toshina, Y. Dinikina","doi":"10.24110/0031-403x-2024-103-3-31-41","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-31-41","url":null,"abstract":"Herpesvirus infection (HVI) reactivation in children with cancer is a common complication during the period of postcytostatic immunosuppression and occurs mostly after courses of high-dose chemotherapy (HDCT) with hematopoietic stem cell transplantation (HSCT). Clinical manifestations can range from asymptomatic viral reactivation and chemo-induced cytopenia worsening to fatal outcomes of the disease coupled with multiorgan involvement. The purpose of the research was to analyze cases of HVI reactivation in oncological pediatric patients in order to identify the risk factors for HVI development, features of its clinical manifestations and the effectiveness of antiviral therapy. Materials and methods used: a single-center retrospective cohort study was provided. The register of HVI reactivation cases in patients receiving antitumor therapy at the V.A. Almazov National Medical Research Centre of the Ministry of Healthcare of Russia (Saint Petersburg, Russia) in Jan. 2017-Jul. 2021 was analyzed. Routine monitoring of HVI reactivation (CMV, HHV-6A/B, EBV) included determination of viral DNA in patients’ biological materials by PCR using the AmpliSens test system on a Rotor-Gene device (amplifier). Results: 40 cases of HVI reactivation were registered, 22 (55%) were associated with cytomegalovirus infection (CMV), 4 (10%) with human herpes virus 6 (HHV-6A/B), 3 (7.5%) with Epstein-Barr virus (EBV) and 11 (27.5%) mixed. DNAemia was registered in 55%, organ damage in 45%. More often, reactivation of HVI developed after allogeneic HSCT (alloHSCT) (42,5%), autologous HSCT (autoHSCT) (40%). A statistically significant decrease of HVI reactivation cases was observed in patients after alloHSCT without TCRaβ depletion (53% v. 5,5%, p=0.036). Antiviral therapy was carried out in all patients. The first-line drug most often was ganciclovir (60%). Second line antiviral therapy was required in 10% of cases and those were the only CMV infection cases. Conclusion: a statistically significant decrease of HVI reactivation cases was observed in patients without TCRaβ depletion in alloHSCT. The clinical signs usually are not specific and require routine laboratory monitoring. Adequate antiviral therapy allowed achieving infectious control over HVI in most of the cases.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"33 35","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141340157","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":"COMPARATIVE ANALYSIS OF EARLY AND LATE ADMINISTRATION OF THE RITUXIMAB BIOSIMILAR BCD020 FOR SYSTEMIC LUPUS ERYTHEMATOSUS IN CHILDREN. RESULTS OF A SINGLE-CENTER RETROSPECTIVE COHORT STUDY","authors":"E. Kalashnikova, E. Isupova, E. Gaidar, V. Masalova, K. Belozerov, L. Sorokina, M. Kaneva, R. Raupov, E.D. Sysoeva, T. L. Kornishina, O. Kalashnikova, V. Chasnyk, M. Kostik","doi":"10.24110/0031-403x-2024-103-3-48-55","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-48-55","url":null,"abstract":"Juvenile systemic lupus erythematosus (jSLE) is a severe life-threatening disease with an immunoinflammatory pathogenesis and possible involvement of several organs or organ systems, both sequentially and simultaneously. Early initiation of biological therapy may improve disease outcomes. The purpose of this research was to evaluate the benefits of early initiation of genetically engineered biological therapy for jSLE with the Rituximab biosimilar BCD020. Materials and methods used: a single-center retrospective cohort study performed at the Saint Petersburg State Pediatric Medical University (Saint Petersburg, Russia) in 2012-2022 that included information on 36 patients with early Rituximab prescription (ERP; within less than 6 months from the jSLE diagnosis) and late Rituximab prescription (LRP; after over one year). The main characteristics of the disease were compared at the onset, at the time of initiation of Rituximab therapy and at 12 months after that. Results: the main baseline differences between the groups were statistically significantly higher disease activity according to the SLEDAI scale (p=0.003) and a higher incidence of macrophage activation syndrome (p=0.096), a higher average daily dose of glucocorticosteroids (GCS) in the ERP group (p=0.027). At the end of the study there were no statistically significant differences in the main outcomes of SLE between the compared groups. The main advantages of ERP were a statistically significantly shorter time to achieve a low daily dose of GCS (<0.2 mg/kg) - 1.2 (0.9; 1.4) years compared to 2.8 (2.3; 4 .0) years (p<0.001) and a higher likelihood of achieving a low dose of corticosteroids (RR=57.8 [95% CI: 7.2; 463.2], p<0.001) and achieving remission (SLEDAI=0); RR=37.6 [95% CI: 4.45; 333.3], p<0.001). During the research there were no statistically significant differences in the incidence of adverse events, including severe adverse events, between the compared groups of patients. Conclusion: ERP makes it possible to improve the control over the activity of jSLE and minimize the volume of GCS drugs with similar treatment outcomes and safety profile. Further research is required in order to determine the indications for biological therapy for jSLE.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"12 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141343729","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":"CLINICAL CASE OF SHORT BOWEL SYNDROME IN A CHILD TREATED WITH TEDUGLUTIDE DRUG WITH POSITIVE OUTCOME","authors":"D.A. Poluboyarinova, M. O. Revnova, N. Volkova, T. V. Mishkina","doi":"10.24110/0031-403x-2024-103-3-198-202","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-198-202","url":null,"abstract":"Authors represent a clinical case of short bowel syndrome, pathological condition caused by a decrease in the absorptive surface of the small intestine, which arose in a child as a result of surgical interventions for small intestinal atresia type IV (multiple atresias). The use of the Teduglutide drug experience is described in a child with severe manifestations of short bowel syndrome.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"9 8","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141340678","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":"CLINICAL CASE OF PYODERMA GANGRENOSUM IN A FEMALE ADOLESCENT","authors":"V. Udzhukhu, L. Ilyenko, E.R. Gumennaya, A. S. Botkina, S. V. Kukalo","doi":"10.24110/0031-403x-2024-103-3-185-189","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-185-189","url":null,"abstract":"Pyoderma gangrenosum (PG) is the most severe type of pyoderma and is extremely rare among children and adolescents. Authors represent a clinical case of PG in a 14 years old female adolescent characterized by multiple sharply painful ulcerative defects with a pronounced tendency to peripheral growth. PG had arisen and proceeded further during the postoperative period against the background of iron deficiency anemia, chronic nonspecific nonulcerative colitis and a dysfunctional state of humoral immunity. Making a diagnosis, conducting differential diagnostics, identifying provoking factors, searching for concomitant somatic pathology, using a set of therapeutic measures made possible eliminating the PG clinical manifestations completely though development of the preventive measures and determination of the prognosis of the disease are difficult clinical tasks for pediatric practitioners nowadays.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"20 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141343295","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":"THERAPEUTIC MONITORING OF BEDAQUILINE DRUG USE IN THE TREATMENT OF CHILDREN WITH MULTIDRUG-RESISTANT RESPIRATORY TUBERCULOSIS","authors":"V.A. Aksenova, A. Kazakov, N. Klevno, A. Pakhlavonova, V. A. Romanenko, A. V. Karasev, O.A. Shevlyakova, E. A. Sokolskaya","doi":"10.24110/0031-403x-2024-103-3-133-138","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-3-133-138","url":null,"abstract":"Use of Bedaquiline in children is studied relatively rarely therefore, according to the WHO experts, it is necessary to further conduct the research on the effectiveness and safety of including of the Bedaquiline drug in chemotherapy regimens in children as well as to obtain the new data on the characteristics of its metabolism in routine clinical practice. The purpose of this research was to evaluate the therapeutic monitoring performance of the Bedaquiline drug use in treatment of pediatric patients with multidrug-resistant tuberculosis (TB). Materials and methods used: pharmacokinetic (PK) parameters were determined in 10 patients of both genders aged 6 to 17 y/o who had received Bedaquiline drug as part of complex therapy in the treatment regimen for multidrug-resistant TB (MDR-TB). All patients had not received anti-tuberculosis drugs previously. Results: during the period of initial daily administration (saturation) of the Bedaquiline drug, Cmax was 3901 ng/ml, Cmin 165 ng/ml, Me 1672 (781.7-2562.3; 95% CI) ng/ml. During the period of maintenance dose intake (3 times per week) prior to taking the drug, Cmax was 987 ng/ml, Cmin 251 ng/ml, Me 504 (340.2-667.7; 95% CI) ng/ml, two hours after Bedaquiline intake Cmax was 2387 ng/ml, Cmin 576 ng/ml, Me 1429 (1005.9-1852.1; 95% CI) ng/ml. Conclusion: the data obtained coincide with the bibliographical data on the Bedaquiline PK in children aged 5 to 8 y/o issues.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"21 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141338808","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}