Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-16-27
G. Makovetskaya, G. Poretskova, L. Mazur, O. A. Sedashkina, V. N. Barinov, S. N. Reshetova
Transition of an adolescent patient with chronic kidney disease (CKD) from pediatric to adult healthcare system is an urgent problem Worldwide. The purpose of this research was to generalize the idea of readiness of adolescents with CKD to transfer into adult nephrology healthcare system, to describe the challenges of such transition period and to determine the roles of participants in the process and their mutual interactions in order to develop and implement the working regional model for organizing the transition of adolescents with CKD within a clustered system of provision of specialized healthcare services. Materials and methods used: a single-center cross-sectional questionnaire surveying of adolescents and young adults with CKD regarding their readiness to transition in two observation groups was carried out on the basis of both adult and pediatric sections of the Samara Oblast Regional Clinical Hospital named after V.D. Seredavin (Samara, Russia) in Jan. 2021-March 2023. Group 1 consisted of young adults aged 18 to 22 y/o with CKD stages 2 to 3; and Group 2 included adolescents aged 13 to 18 y/o with CKD stages 2 to 5. The results were then evaluated with the TRxANSITION Index (formerly TRxANSITION Scale), PedsQL and the SF-36 questionnaire. Adolescents’ parents/caretakers took a survey on the independence of their children as well. Results: the data on the insufficient independence of adolescents were obtained, as follows: 66.7% [34.9-90.1%] (n=8) “never” took medication on their own, 50.0% [21.1-78.9%] (n=6) have never asked their physicians questions (p<0.001), 50.0% [21.1-78.9%] (n=6) “never” and 33.3% [9.9-65.1%] (n=4) “almost never” did they agree to meet with the physician (p=0.005). Adolescents experienced difficulties in both communicating and working together with a physician: 33.3% [9.9-65.1%] (n=4) and 25.0% [5.5-57.2%] (n=3) “difficult,” respectively; 50.0% [21.1-78.9%] (n=6) and 58.3% [27.7-84.8%] (n= 7) “almost difficult,” respectively (p<0.001). On the other hand, “almost everything” and “everything” do they know about their health status 47.1% [23.0-72.2%] (n=8) and 35.4% [14.2-61.7%] (n=6), respectively, of young adults with CKD vs. adolescents (p<0.001). Conclusion: structurization of the participants’ roles in the communication system “patient and his family members - medicine professionals - psychologists - social workers” contributes to the stabilization of an adolescent with CKD during the transition period.
{"title":"ADOLESCENT PATIENT WITH CHRONIC KIDNEY DISEASE: READINESS FOR TRANSFER FROM PEDIATRIC INTO ADULT NEPHROLOGY HEALTHCARE SYSTEM, CHALLENGES OF THE TRANSITION. BIBLIOGRAPHICAL REVIEW AND AUTHORS’ OWN OBSERVATIONS","authors":"G. Makovetskaya, G. Poretskova, L. Mazur, O. A. Sedashkina, V. N. Barinov, S. N. Reshetova","doi":"10.24110/0031-403x-2023-102-4-16-27","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-16-27","url":null,"abstract":"Transition of an adolescent patient with chronic kidney disease (CKD) from pediatric to adult healthcare system is an urgent problem Worldwide. The purpose of this research was to generalize the idea of readiness of adolescents with CKD to transfer into adult nephrology healthcare system, to describe the challenges of such transition period and to determine the roles of participants in the process and their mutual interactions in order to develop and implement the working regional model for organizing the transition of adolescents with CKD within a clustered system of provision of specialized healthcare services. Materials and methods used: a single-center cross-sectional questionnaire surveying of adolescents and young adults with CKD regarding their readiness to transition in two observation groups was carried out on the basis of both adult and pediatric sections of the Samara Oblast Regional Clinical Hospital named after V.D. Seredavin (Samara, Russia) in Jan. 2021-March 2023. Group 1 consisted of young adults aged 18 to 22 y/o with CKD stages 2 to 3; and Group 2 included adolescents aged 13 to 18 y/o with CKD stages 2 to 5. The results were then evaluated with the TRxANSITION Index (formerly TRxANSITION Scale), PedsQL and the SF-36 questionnaire. Adolescents’ parents/caretakers took a survey on the independence of their children as well. Results: the data on the insufficient independence of adolescents were obtained, as follows: 66.7% [34.9-90.1%] (n=8) “never” took medication on their own, 50.0% [21.1-78.9%] (n=6) have never asked their physicians questions (p<0.001), 50.0% [21.1-78.9%] (n=6) “never” and 33.3% [9.9-65.1%] (n=4) “almost never” did they agree to meet with the physician (p=0.005). Adolescents experienced difficulties in both communicating and working together with a physician: 33.3% [9.9-65.1%] (n=4) and 25.0% [5.5-57.2%] (n=3) “difficult,” respectively; 50.0% [21.1-78.9%] (n=6) and 58.3% [27.7-84.8%] (n= 7) “almost difficult,” respectively (p<0.001). On the other hand, “almost everything” and “everything” do they know about their health status 47.1% [23.0-72.2%] (n=8) and 35.4% [14.2-61.7%] (n=6), respectively, of young adults with CKD vs. adolescents (p<0.001). Conclusion: structurization of the participants’ roles in the communication system “patient and his family members - medicine professionals - psychologists - social workers” contributes to the stabilization of an adolescent with CKD during the transition period.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75373835","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}
Telemedicine technologies in pediatric patients with type 1 diabetes mellitus (T1D) is an effective approach that have begun to be used recently. The purpose of this research was to evaluate the possibility and effectiveness of remote observation and training of children with newly diagnosed T1D in selected Russia regions. Materials and methods used: a multicenter prospective open uncontrolled experimental clinical study involved 92 children aged 1 to 18 y/o with T1D receiving intensified insulin therapy who were divided into two groups: G1 of 60 patients with T1D manifestation of less than 12 weeks prior to the inclusion in the study, and G2 of 32 children with T1D manifestation lasting over 12 weeks prior to the inclusion with poor disease control (glycated hemoglobin at 8% and above) from troubled families. The main indicator for evaluating the effectiveness was the glycated hemoglobin (HbA1c) level. Results: median age was 9.2 (6.2-11.2) y/o, 47 boys/45 girls. A total of 401 remote consultations had been carried out in various forms. The HbA1c level decreased statistically significantly by the end of the study by 3.5% and 0.6% (p<0.001) in G1 and G2, respectively. Statistically significant (p<0.001) increase in the number of patients with HbA1c at the level of less than 7% was reached, with most of them (52%) reaching the target glycemic control parameters. Telemedicine technologies therefore represent the effective (in terms of glycemic control) addition to the traditional dispensary observation, which in its turn increases the availability of medical care for children with endocrine diseases.
{"title":"EVALUATION OF THE MEDICAL CARE MODEL FOR CHILDREN WITH TYPE 1 DIABETES MELLITUS CARRIED OUT USING MOBILE APP AND REMOTE CONSULTATIONS. TARGETED ASSISTANCE TO DISABLED PEDIATRIC PATIENTS WITH DIABETES MELLITUS AND THEIR FAMILIES UNDER THE «ALFA-ENDO» CHARITY PROGRAM FOR CHILDREN WITH ENDOCRINE DISEASES","authors":"D.N. Laptev, A.O. Emelyanov, O.B. Bezlepkina, A.V. Karpushkina, E.S. Demina, E.E. Petryaikina, Yа.V. Girsh, I.V. Gunbina, E.V. Kolbasina, D.Yu. Sorokin, V.A. Peterkova","doi":"10.24110/0031-403x-2023-102-4-159-165","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-159-165","url":null,"abstract":"Telemedicine technologies in pediatric patients with type 1 diabetes mellitus (T1D) is an effective approach that have begun to be used recently. The purpose of this research was to evaluate the possibility and effectiveness of remote observation and training of children with newly diagnosed T1D in selected Russia regions. Materials and methods used: a multicenter prospective open uncontrolled experimental clinical study involved 92 children aged 1 to 18 y/o with T1D receiving intensified insulin therapy who were divided into two groups: G1 of 60 patients with T1D manifestation of less than 12 weeks prior to the inclusion in the study, and G2 of 32 children with T1D manifestation lasting over 12 weeks prior to the inclusion with poor disease control (glycated hemoglobin at 8% and above) from troubled families. The main indicator for evaluating the effectiveness was the glycated hemoglobin (HbA1c) level. Results: median age was 9.2 (6.2-11.2) y/o, 47 boys/45 girls. A total of 401 remote consultations had been carried out in various forms. The HbA1c level decreased statistically significantly by the end of the study by 3.5% and 0.6% (p<0.001) in G1 and G2, respectively. Statistically significant (p<0.001) increase in the number of patients with HbA1c at the level of less than 7% was reached, with most of them (52%) reaching the target glycemic control parameters. Telemedicine technologies therefore represent the effective (in terms of glycemic control) addition to the traditional dispensary observation, which in its turn increases the availability of medical care for children with endocrine diseases.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135492800","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}
Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-217-218
A.R. Polishchuk, A. S. Romanova, M.Sh. Khaider, S. Balashov, V. Sevostyanov
For citation: A.R. Polishchuk, A.S. Romanova, M.Sh. Khaider, S.L. Balashov, V.K. Sevostyanov. Morbidity structure in 14-years-old residents of the Moscow City (Russia): checkups’ study. Pediatria n.a. G.N. Speransky. 2023; 102 (4): 217-218. DOI: 10.24110/0031-403X-2023-102-4-217-218.
{"title":"MORBIDITY STRUCTURE IN 14-YEARS-OLD RESIDENTS OF THE MOSCOW CITY (RUSSIA): CHECKUPS’ STUDY","authors":"A.R. Polishchuk, A. S. Romanova, M.Sh. Khaider, S. Balashov, V. Sevostyanov","doi":"10.24110/0031-403x-2023-102-4-217-218","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-217-218","url":null,"abstract":"For citation: A.R. Polishchuk, A.S. Romanova, M.Sh. Khaider, S.L. Balashov, V.K. Sevostyanov. Morbidity structure in 14-years-old residents of the Moscow City (Russia): checkups’ study. Pediatria n.a. G.N. Speransky. 2023; 102 (4): 217-218. DOI: 10.24110/0031-403X-2023-102-4-217-218.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88582330","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}
Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-219-225
S. A. Sher, V. Albitskiy, N. V. Ustinova
Article represents the results of historical and medical bibliographical research reflecting the issues of restoration and continuous improvement of pediatric sanatorium and resort healthcare system in USSR during the post-WWII period. The purpose of the research was to evaluate the state of sanatorium and resort recreational services for child population whose health indicators highly deteriorated during WWII. The healthcare authorities in USSR faced the task of developing effective measures for the speedy restoration of children's health. In solving this problem, the highest importance was given to the development of children's sanatorium and resort healthcare system. Based on the thorough research of both published and unpublished documents in the State Archive of the Russian Federation (GARF), articles published in journals, scientific digests, chapters from monograph works, etc., it was found that almost all pediatric sanatoriums, the “Pioneer Camps” and other healthcare institutions, especially those located in the Soviet southern resorts, were occupied by the Nazis during the WWII and were fully or partially destroyed due to hostilities, plus the survived ones have then been reorganized into evacuation facilities and temporary hospitals. That is the reason why the restoration of sanatorium and resort recreational facilities became one of the priority tasks in the post-WWII period. Despite huge growth in the number of pediatric sanatoriums and other children’s health-improving institutions in 1947 compared to 1946, a decrease in child morbidity rate, an increase in the medical staff count serving pediatric patients, the recreational system had showed certain defects. Negative trends were caused by the lack in financing, laborers and qualified staff as well as re-profiling of children's healthcare facilities into those for adults. Thanks to the timely and adequate governmental measures intent to the restoration of pediatric sanatorium and resort recreation services, the bed capacity reached the pre-WWII level by 1950; a network of specialized sanatoriums started to develop and therefore increase in the health-improving measures efficiency was noted then.
{"title":"SANATORIUM AND RESORT RECREATION SERVICES FOR CHILD POPULATION IN USSR IN POST-WWII PERIOD (1946 TO 1950)","authors":"S. A. Sher, V. Albitskiy, N. V. Ustinova","doi":"10.24110/0031-403x-2023-102-4-219-225","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-219-225","url":null,"abstract":"Article represents the results of historical and medical bibliographical research reflecting the issues of restoration and continuous improvement of pediatric sanatorium and resort healthcare system in USSR during the post-WWII period. The purpose of the research was to evaluate the state of sanatorium and resort recreational services for child population whose health indicators highly deteriorated during WWII. The healthcare authorities in USSR faced the task of developing effective measures for the speedy restoration of children's health. In solving this problem, the highest importance was given to the development of children's sanatorium and resort healthcare system. Based on the thorough research of both published and unpublished documents in the State Archive of the Russian Federation (GARF), articles published in journals, scientific digests, chapters from monograph works, etc., it was found that almost all pediatric sanatoriums, the “Pioneer Camps” and other healthcare institutions, especially those located in the Soviet southern resorts, were occupied by the Nazis during the WWII and were fully or partially destroyed due to hostilities, plus the survived ones have then been reorganized into evacuation facilities and temporary hospitals. That is the reason why the restoration of sanatorium and resort recreational facilities became one of the priority tasks in the post-WWII period. Despite huge growth in the number of pediatric sanatoriums and other children’s health-improving institutions in 1947 compared to 1946, a decrease in child morbidity rate, an increase in the medical staff count serving pediatric patients, the recreational system had showed certain defects. Negative trends were caused by the lack in financing, laborers and qualified staff as well as re-profiling of children's healthcare facilities into those for adults. Thanks to the timely and adequate governmental measures intent to the restoration of pediatric sanatorium and resort recreation services, the bed capacity reached the pre-WWII level by 1950; a network of specialized sanatoriums started to develop and therefore increase in the health-improving measures efficiency was noted then.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75875075","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}
Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-63-72
T. N. Budkina, S. Vyazankina, M. Lokhmatov, S. Makarova, N. Murashkin, A. Fisenko, D. S. Yasakov, V. Oldakovsky, A. Tupylenko, G. A. Korolev, A. Potapov, K. Kulikov, O. Ereshko, A. Galimova, I. Gordeeva
Number of children with eosinophilic esophagitis (EoE) observed has recently increased, therefore significant experience has been gained in the diagnosis and treatment of this disease. However, the systemic data from Russian pediatric centers’ registers that would summarize the signs of the disease in childhood has not been published as yet. The purpose of this research was to study the demographic, clinical, endoscopic and morphological features of EoE in children and to form the unified register of patients on the basis of a single yet multidisciplinary pediatric hospital. Materials and methods used: a single-center retrospective cohort study included 50 pediatric (under the age of 18 y/o) patients’ (13 (26%) girls/37 (74%) boys) records of those who were included in the National Medical Research Center for Children’s Health (Moscow, Russia) Register of EoE patients in July 2020-May 2023. Demographic indicators were assessed as well as the clinical manifestations, endoscopic and morphological changes and concomitant diseases. Results: the median age of children at the time of the diagnosis onset was 12 (8; 15) y/o. The age and the gender characteristics of patients were typical for the disease: boys aged 11 to 17 y/o had prevailed. The frequency of the EoE detection among children who had underwent esophagogastroduodenoscopy (EGD test) for various reasons was a single case per 253 EGD tests (0.4%). Among the clinical manifestations, dysphagia (44%) and abdominal pain (70%) were noted more often; GERD-like symptoms and poor weight gain were less common. The vast majority of children (84%) had concomitant allergic pathologies. EoE had proceeded comorbidly with other diseases in 60% of cases. Endoscopic manifestations of EoE were described and analyzed in detail. Thus, swelling of the mucous membrane, longitudinal striation and exudate had prevailed. Complicated course of EoE with the formation of esophageal stenosis was noted in 14%. There were no statistically significant differences between the age groups of children aged 1 to 10 y/o and 11 to 17 y/o in relation to clinical manifestations, endoscopic and morphological characteristics of EoE. Conclusion: thorough registration of pediatric patients with EoE does allow studying and summarizing of the features of this disease, in detail, with further provision of the useful information for clinical practice.
近年来，儿童嗜酸性粒细胞性食管炎(EoE)的数量有所增加，因此在该病的诊断和治疗方面获得了重要的经验。然而，俄罗斯儿科中心登记的系统数据尚未公布，这些数据将总结儿童时期疾病的迹象。本研究的目的是研究儿童EoE的人口学、临床、内镜和形态学特征，并在单一但多学科的儿科医院的基础上形成统一的患者登记。使用的材料和方法:单中心回顾性队列研究纳入了2020年7月至2023年5月在俄罗斯莫斯科国家儿童健康医学研究中心EoE患者登记册中登记的50例儿科(18岁以下)患者(13例(26%)女孩/37例(74%)男孩)的记录。评估人口统计学指标、临床表现、内镜及形态学改变及伴发疾病。结果:患儿诊断发病时的中位年龄为12岁(8岁;15) y / o。患者的年龄和性别特征是典型的疾病:11至17岁的男孩占主导地位。在因各种原因接受食管胃十二指肠镜检查(EGD试验)的儿童中，EoE的检出率为每253例EGD试验中1例(0.4%)。临床表现中以吞咽困难(44%)和腹痛(70%)较多;反流样症状和体重增加不佳的情况较少见。绝大多数儿童(84%)伴有过敏性疾病。在60%的病例中，EoE与其他疾病并存。详细描述并分析了EoE的内镜表现。可见粘膜肿胀、纵纹、渗出为主。合并食管狭窄的患者占14%。1 ~ 10岁和11 ~ 17岁患儿的临床表现、内镜及形态学特征比较，差异均无统计学意义。结论:对儿童EoE患者进行彻底的登记，确实可以详细地研究和总结该疾病的特征，并进一步为临床实践提供有用的信息。
{"title":"CLINICAL AND MORPHOLOGICAL CHARACTERISTICS OF EOSINOPHILIC ESOPHAGITIS IN CHILDREN: A COHORT STUDY","authors":"T. N. Budkina, S. Vyazankina, M. Lokhmatov, S. Makarova, N. Murashkin, A. Fisenko, D. S. Yasakov, V. Oldakovsky, A. Tupylenko, G. A. Korolev, A. Potapov, K. Kulikov, O. Ereshko, A. Galimova, I. Gordeeva","doi":"10.24110/0031-403x-2023-102-4-63-72","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-63-72","url":null,"abstract":"Number of children with eosinophilic esophagitis (EoE) observed has recently increased, therefore significant experience has been gained in the diagnosis and treatment of this disease. However, the systemic data from Russian pediatric centers’ registers that would summarize the signs of the disease in childhood has not been published as yet. The purpose of this research was to study the demographic, clinical, endoscopic and morphological features of EoE in children and to form the unified register of patients on the basis of a single yet multidisciplinary pediatric hospital. Materials and methods used: a single-center retrospective cohort study included 50 pediatric (under the age of 18 y/o) patients’ (13 (26%) girls/37 (74%) boys) records of those who were included in the National Medical Research Center for Children’s Health (Moscow, Russia) Register of EoE patients in July 2020-May 2023. Demographic indicators were assessed as well as the clinical manifestations, endoscopic and morphological changes and concomitant diseases. Results: the median age of children at the time of the diagnosis onset was 12 (8; 15) y/o. The age and the gender characteristics of patients were typical for the disease: boys aged 11 to 17 y/o had prevailed. The frequency of the EoE detection among children who had underwent esophagogastroduodenoscopy (EGD test) for various reasons was a single case per 253 EGD tests (0.4%). Among the clinical manifestations, dysphagia (44%) and abdominal pain (70%) were noted more often; GERD-like symptoms and poor weight gain were less common. The vast majority of children (84%) had concomitant allergic pathologies. EoE had proceeded comorbidly with other diseases in 60% of cases. Endoscopic manifestations of EoE were described and analyzed in detail. Thus, swelling of the mucous membrane, longitudinal striation and exudate had prevailed. Complicated course of EoE with the formation of esophageal stenosis was noted in 14%. There were no statistically significant differences between the age groups of children aged 1 to 10 y/o and 11 to 17 y/o in relation to clinical manifestations, endoscopic and morphological characteristics of EoE. Conclusion: thorough registration of pediatric patients with EoE does allow studying and summarizing of the features of this disease, in detail, with further provision of the useful information for clinical practice.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77228776","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}
Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-46-51
R. Nikolaeva, P. Ananin, T. Vashurina, O. Zrobok, A. Pushkov, K. Savostyanov, A. Fisenko, A. Tsygin
Nephrocalcinosis can result from some of acquired causes as well as being a part of the symptomatic complex of hereditary diseases. Despite monogenic causes of nephrocalcinosis being rare they nevertheless represent a significant disease burden with early onset of symptoms and a risk for the chronic kidney disease development. The purpose of this research was to describe the etiological characteristics and clinical picture of children with nephrocalcinosis as well as to assess its progression and impact on further decline in renal function. Methods used: a retrospective-prospective cohort study of 85 children with nephrocalcinosis was conducted on the basis of the National Medical Research Center for Children’s Health (Moscow, Russia) in 2012-2023. Results: the median age of the nephrocalcinosis detection was 1.5 [0.3; 4.4] y/o. Median observation duration was 2.5 [1.0; 5.0] years. For 37 (63%) there was a causative mutation responsible for the development of the disease associated with nephrocalcinosis diagnosed. Mutations in the CYP24A1, CLCN5, AGXT and HPRT1 genes had predominated. Further nephrocalcinosis progression had no significant effect on kidney function within a 2-year follow-up. Conclusion: nephrocalcinosis may be a part of the diseases with a high risk for progression and poor renal prognosis. Early diagnosis of the nephrocalcinosis cause is important for obtaining accurate prognostic information and timely therapy initiation.
肾钙质沉着症可以由一些获得性原因引起，也可以是遗传性疾病症状复合体的一部分。尽管肾钙化症的单基因原因很少见，但它们仍然是一种显著的疾病负担，具有早期发病症状和慢性肾脏疾病发展的风险。本研究的目的是描述儿童肾钙质沉着症的病因特征和临床表现，并评估其进展和对肾功能进一步下降的影响。方法:2012-2023年，在俄罗斯莫斯科国家儿童健康医学研究中心的基础上，对85例肾钙化症儿童进行回顾性-前瞻性队列研究。结果:肾钙质沉着症的中位年龄为1.5 [0.3];4.4] y / o。中位观察时间为2.5 [1.0];5.0)年。其中37例(63%)存在与肾钙质病诊断相关的疾病发展相关的致病突变。CYP24A1、CLCN5、AGXT和HPRT1基因突变占主导地位。在2年的随访中，肾钙化症的进一步进展对肾功能没有显著影响。结论:肾钙质沉着症可能是肾脏病进展危险高、预后差的疾病之一。早期诊断肾钙质沉着症的病因对于获得准确的预后信息和及时开始治疗是重要的。
{"title":"ETIOLOGICAL FACTORS AND CLINICAL MANIFESTATIONS OF NEPHROCALCINOSIS IN CHILDREN","authors":"R. Nikolaeva, P. Ananin, T. Vashurina, O. Zrobok, A. Pushkov, K. Savostyanov, A. Fisenko, A. Tsygin","doi":"10.24110/0031-403x-2023-102-4-46-51","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-46-51","url":null,"abstract":"Nephrocalcinosis can result from some of acquired causes as well as being a part of the symptomatic complex of hereditary diseases. Despite monogenic causes of nephrocalcinosis being rare they nevertheless represent a significant disease burden with early onset of symptoms and a risk for the chronic kidney disease development. The purpose of this research was to describe the etiological characteristics and clinical picture of children with nephrocalcinosis as well as to assess its progression and impact on further decline in renal function. Methods used: a retrospective-prospective cohort study of 85 children with nephrocalcinosis was conducted on the basis of the National Medical Research Center for Children’s Health (Moscow, Russia) in 2012-2023. Results: the median age of the nephrocalcinosis detection was 1.5 [0.3; 4.4] y/o. Median observation duration was 2.5 [1.0; 5.0] years. For 37 (63%) there was a causative mutation responsible for the development of the disease associated with nephrocalcinosis diagnosed. Mutations in the CYP24A1, CLCN5, AGXT and HPRT1 genes had predominated. Further nephrocalcinosis progression had no significant effect on kidney function within a 2-year follow-up. Conclusion: nephrocalcinosis may be a part of the diseases with a high risk for progression and poor renal prognosis. Early diagnosis of the nephrocalcinosis cause is important for obtaining accurate prognostic information and timely therapy initiation.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74532247","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}
Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-8-9
A.A. Baranov, L.S. Namazova-Baranova
ON JULY 18, 2023 PEDIATRIC PRACTITIONERS AND SCIENTISTS IN RUSSIA AND WORLDWIDE HAVE CELEBRATED THE ANNIVERSARY OF THE EDITORIAL BOARD MEMBER WITH THE JOURNAL «PEDIATRIA» NAMED AFTER G.N. SPERANSKY, AN OUTSTANDING PEDIATRICIAN OF OUR TIME, HONORED SCIENTIST OF THE RUSSIAN FEDERATION, LAUREATE OF THE RUSSIAN FEDERATION GOVERNMENT PRIZE IN SCIENCE AND TECHNOLOGY, PRESIDENT OF THE UNION OF PEDIATRICIANS OF RUSSIA, ACADEMICIAN OF THE RUSSIAN ACADEMY OF SCIENCES, PROFESSOR LEYLA SEYMUROVNA NAMAZOVA-BARANOVA
{"title":"ON JULY 18, 2023 PEDIATRIC PRACTITIONERS AND SCIENTISTS IN RUSSIA AND WORLDWIDE HAVE CELEBRATED THE ANNIVERSARY OF THE EDITORIAL BOARD MEMBER WITH THE JOURNAL «PEDIATRIA» NAMED AFTER G.N. SPERANSKY, AN OUTSTANDING PEDIATRICIAN OF OUR TIME, HONORED SCIENTIST OF THE RUSSIAN FEDERATION, LAUREATE OF THE RUSSIAN FEDERATION GOVERNMENT PRIZE IN SCIENCE AND TECHNOLOGY, PRESIDENT OF THE UNION OF PEDIATRICIANS OF RUSSIA, ACADEMICIAN OF THE RUSSIAN ACADEMY OF SCIENCES, PROFESSOR LEYLA SEYMUROVNA NAMAZOVA-…","authors":"A.A. Baranov, L.S. Namazova-Baranova","doi":"10.24110/0031-403x-2023-102-4-8-9","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-8-9","url":null,"abstract":"ON JULY 18, 2023 PEDIATRIC PRACTITIONERS AND SCIENTISTS IN RUSSIA AND WORLDWIDE HAVE CELEBRATED THE ANNIVERSARY OF THE EDITORIAL BOARD MEMBER WITH THE JOURNAL «PEDIATRIA» NAMED AFTER G.N. SPERANSKY, AN OUTSTANDING PEDIATRICIAN OF OUR TIME, HONORED SCIENTIST OF THE RUSSIAN FEDERATION, LAUREATE OF THE RUSSIAN FEDERATION GOVERNMENT PRIZE IN SCIENCE AND TECHNOLOGY, PRESIDENT OF THE UNION OF PEDIATRICIANS OF RUSSIA, ACADEMICIAN OF THE RUSSIAN ACADEMY OF SCIENCES, PROFESSOR LEYLA SEYMUROVNA NAMAZOVA-BARANOVA","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135492799","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}
Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-58-63
M. Aksenova, N. Konkova, O. Piruzeeva, N. Zaikova, T. Lepaeva, T. Nikishina, V. Obukhova, V. Dlin
Patients with a hemizygous mutation in the COL4A5 gene have a high risk of developing renal failure at a young age. Proteinuria reflects the progression of glomerulopathy in Alport syndrome (AS). In order to improve renal survival in AS patients with proteinuria are prescribed with angiotensin system inhibitors. There is a limited, insufficient number of studies demonstrating the effectiveness of angiotensin-converting enzyme inhibitors (ACEI) in children with AS; the therapeutic effect initiated prior to the development of proteinuria has not been studied as yet. The purpose of the research was to determine the effectiveness of ACEI therapy in the form of enalapril in the prevention of proteinuria in boys with X-linked AS (XLAS). Materials and methods used: boys aged 2 to 15 y/o with genetically confirmed XLAS were included in a single-center prospective cohort study. Enalapril was prescribed at doses of 1 to 3 mg/m2/day. Urinary protein excretion (Pr) and estimated glomerular filtration rate (eGFR) were studied at the time of inclusion and with a frequency of one time per six months for the three-year period or longer. The primary endpoint was the time of onset of proteinuria. Results: 72 boys in total, incl. 29 without proteinuria, who received enalapril therapy (Group 1, age 6.2±1.9 y/o, Pr 67±20 mg/m2/day, eGFR 118±15.2 ml /min/1.73 m2) and 43 children without therapy (Group 2, age 6.8±1.9 y/o, Pr 87±23 mg/m2/day, eGFR 119±17.3 ml/min/1.73 m2). The incidence of proteinuria (0.45 in G1 vs. 0.98 in G2, p<0.001), incl. up to the age of 10 y/o (0.42 vs 0.91, respectively, p<0.001) was statistically significantly lower in G1. Proteinuria developed later in the treatment group (10.2±2.9 vs. 4.9±1.4 years, p=0.300). Non-missense mutations in the COL4A5 gene, episodes of macrohematuria in records and lack of therapy were the risk factors for proteinuria; the lack of enalapril therapy was a predictor for proteinuria. The risk of proteinuria during therapy was reduced by 50% (RR=0.46 (95% CI 0.31; 0.68); ARR=0.53; NNT=1.9). Conclusion: initiation of enalapril therapy at the pre-proteinuria stage of nephropathy in boys with XLAS reduces the risk of proteinuria preventing its occurrence in every 50$ of cases when receiving treatment. Early initiation of therapy may be effective in preventing the progression of kidney disease in boys with XLAS.
{"title":"ANGIOTENSIN-CONVERTING ENZYME INHIBITORS IN PREVENTING PROTEINURIA IN BOYS WITH X-LINKED ALPORT SYNDROME: A SINGLE-CENTER COHORT NON-RANDOMIZED STUDY","authors":"M. Aksenova, N. Konkova, O. Piruzeeva, N. Zaikova, T. Lepaeva, T. Nikishina, V. Obukhova, V. Dlin","doi":"10.24110/0031-403x-2023-102-4-58-63","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-58-63","url":null,"abstract":"Patients with a hemizygous mutation in the COL4A5 gene have a high risk of developing renal failure at a young age. Proteinuria reflects the progression of glomerulopathy in Alport syndrome (AS). In order to improve renal survival in AS patients with proteinuria are prescribed with angiotensin system inhibitors. There is a limited, insufficient number of studies demonstrating the effectiveness of angiotensin-converting enzyme inhibitors (ACEI) in children with AS; the therapeutic effect initiated prior to the development of proteinuria has not been studied as yet. The purpose of the research was to determine the effectiveness of ACEI therapy in the form of enalapril in the prevention of proteinuria in boys with X-linked AS (XLAS). Materials and methods used: boys aged 2 to 15 y/o with genetically confirmed XLAS were included in a single-center prospective cohort study. Enalapril was prescribed at doses of 1 to 3 mg/m2/day. Urinary protein excretion (Pr) and estimated glomerular filtration rate (eGFR) were studied at the time of inclusion and with a frequency of one time per six months for the three-year period or longer. The primary endpoint was the time of onset of proteinuria. Results: 72 boys in total, incl. 29 without proteinuria, who received enalapril therapy (Group 1, age 6.2±1.9 y/o, Pr 67±20 mg/m2/day, eGFR 118±15.2 ml /min/1.73 m2) and 43 children without therapy (Group 2, age 6.8±1.9 y/o, Pr 87±23 mg/m2/day, eGFR 119±17.3 ml/min/1.73 m2). The incidence of proteinuria (0.45 in G1 vs. 0.98 in G2, p<0.001), incl. up to the age of 10 y/o (0.42 vs 0.91, respectively, p<0.001) was statistically significantly lower in G1. Proteinuria developed later in the treatment group (10.2±2.9 vs. 4.9±1.4 years, p=0.300). Non-missense mutations in the COL4A5 gene, episodes of macrohematuria in records and lack of therapy were the risk factors for proteinuria; the lack of enalapril therapy was a predictor for proteinuria. The risk of proteinuria during therapy was reduced by 50% (RR=0.46 (95% CI 0.31; 0.68); ARR=0.53; NNT=1.9). Conclusion: initiation of enalapril therapy at the pre-proteinuria stage of nephropathy in boys with XLAS reduces the risk of proteinuria preventing its occurrence in every 50$ of cases when receiving treatment. Early initiation of therapy may be effective in preventing the progression of kidney disease in boys with XLAS.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75963721","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}
Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-133-140
P. Svirin, L. Larina, I.N. Lavrentyevа
Part 2 of the Article represents published data dedicated to the thrombolytic therapy (TT) for thrombosis of various localizations in children. Options for dosing and methods of thrombolytic therapy are considered. Indications and contraindications for this method of treatment are described. information on thrombolytic therapy in newborns is provided as well. Part 1 of the Article is available at https://doi.org/10.24110/0031-403X-2023-102-3-145-150: P.V. Svirin, L.E. Larina, I.N. Lavrentyeva. Thrombolytic therapy in pediatric practice (Part 1). Pediatria n.a. G.N. Speransky. 2023; 102 (3): 145-150. DOI: 10.24110/0031-403X-2023-102-3-145-150.
{"title":"THROMBOLYTIC THERAPY IN PEDIATRIC PRACTICE: A BIBLIOGRAPHICAL REVIEW (PART 2)","authors":"P. Svirin, L. Larina, I.N. Lavrentyevа","doi":"10.24110/0031-403x-2023-102-4-133-140","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-133-140","url":null,"abstract":"Part 2 of the Article represents published data dedicated to the thrombolytic therapy (TT) for thrombosis of various localizations in children. Options for dosing and methods of thrombolytic therapy are considered. Indications and contraindications for this method of treatment are described. information on thrombolytic therapy in newborns is provided as well. Part 1 of the Article is available at https://doi.org/10.24110/0031-403X-2023-102-3-145-150: P.V. Svirin, L.E. Larina, I.N. Lavrentyeva. Thrombolytic therapy in pediatric practice (Part 1). Pediatria n.a. G.N. Speransky. 2023; 102 (3): 145-150. DOI: 10.24110/0031-403X-2023-102-3-145-150.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82256339","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}
Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-80-92
S. S. Nikitin, N. B. Guseva, R. Ignatiev
Article represents a bibliographical review on the problem of standards for conducting urodynamic research methods. The need for urodynamic studies is quite high and is due to the high prevalence of urination disorders in children. At the same time, the Russia urodynamic laboratories count is low and they aren’t available in all Russia regions. Plus, the number of scientific publications on urodynamics and the pediatric neurology in general is clearly insufficient. Performance of urodynamic studies in adult patients is standardized by the ICS guidelines. At the same time not all of the guidelines are acceptable in children, it is therefore necessary to approach each clinical situation individually when choosing methods for urodynamic research. This review discusses methods of urodynamic studies, such as: uroflowmetry, uroflowmetry with simultaneous recording of pelvic floor electromyography, cystometry, pressure-flow study and urethral profilometry. A brief historical background is presented as well as the indications for the methods of urodynamic research, the research techniques and the peculiarities of the results interpretation. In addition, an example of a conclusion is shown when conducting a comprehensive urodynamic study in a pediatric patient. The article is aimed at standardizing the conduct, the interpretation of the results and the formulation of conclusions when conducting urodynamic studies in children.
{"title":"STANDARDS FOR CONDUCTING AND INTERPRETING THE RESULTS OF URODYNAMIC STUDIES IN CHILDREN","authors":"S. S. Nikitin, N. B. Guseva, R. Ignatiev","doi":"10.24110/0031-403x-2023-102-4-80-92","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-80-92","url":null,"abstract":"Article represents a bibliographical review on the problem of standards for conducting urodynamic research methods. The need for urodynamic studies is quite high and is due to the high prevalence of urination disorders in children. At the same time, the Russia urodynamic laboratories count is low and they aren’t available in all Russia regions. Plus, the number of scientific publications on urodynamics and the pediatric neurology in general is clearly insufficient. Performance of urodynamic studies in adult patients is standardized by the ICS guidelines. At the same time not all of the guidelines are acceptable in children, it is therefore necessary to approach each clinical situation individually when choosing methods for urodynamic research. This review discusses methods of urodynamic studies, such as: uroflowmetry, uroflowmetry with simultaneous recording of pelvic floor electromyography, cystometry, pressure-flow study and urethral profilometry. A brief historical background is presented as well as the indications for the methods of urodynamic research, the research techniques and the peculiarities of the results interpretation. In addition, an example of a conclusion is shown when conducting a comprehensive urodynamic study in a pediatric patient. The article is aimed at standardizing the conduct, the interpretation of the results and the formulation of conclusions when conducting urodynamic studies in children.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84404454","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}
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