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":"16 1","pages":""},"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}
Pub Date : 2023-08-10DOI: 10.24110/0031-403x-2023-102-4-177-186
M. Shumikhina, O. Chugunova, A. Gurevich
Both autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease may occur at any age starting infancy and up to adulthood. Manifestations of autosomal dominant polycystic kidney disease usually occur in adult patients with a risk of development of end-stage renal failure after the fifth decade of life. However, an increase in the number of pediatric cases of a very-early onset of the disease, in utero or during the first 18 months of life, had been registered recently, that were characterized by severe clinical manifestations which in tis turn had mimic the autosomal recessive polycystic kidney disease. The three infant cases of autosomal dominant polycystic kidney disease are observed in the Article with perinatal period features, clinical manifestations of the disease, tests’ results and its interpretation given. Children with very-early onset of autosomal dominant polycystic kidney disease are in the high-risk group for nephromegaly, severe hypertension and the early end-stage renal failure.
{"title":"AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE WITH VERY-EARLY ONSET","authors":"M. Shumikhina, O. Chugunova, A. Gurevich","doi":"10.24110/0031-403x-2023-102-4-177-186","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-177-186","url":null,"abstract":"Both autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease may occur at any age starting infancy and up to adulthood. Manifestations of autosomal dominant polycystic kidney disease usually occur in adult patients with a risk of development of end-stage renal failure after the fifth decade of life. However, an increase in the number of pediatric cases of a very-early onset of the disease, in utero or during the first 18 months of life, had been registered recently, that were characterized by severe clinical manifestations which in tis turn had mimic the autosomal recessive polycystic kidney disease. The three infant cases of autosomal dominant polycystic kidney disease are observed in the Article with perinatal period features, clinical manifestations of the disease, tests’ results and its interpretation given. Children with very-early onset of autosomal dominant polycystic kidney disease are in the high-risk group for nephromegaly, severe hypertension and the early end-stage renal failure.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":"52 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77115660","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-2023-102-4-186-193
E. Orekhova, O. Chugunova, S. Blokh, M. Boyadjan, M. Yudin, P.V. Pavlova, O. I. Yaroshevskaya, P. Shumilov
Article describes a clinical case of a female adolescent with macrohematuria, nephrocalcinosis, nephrolithiasis and cholelithiasis, in which laboratory examination and imaging of parathyroid glands gave grounds to diagnose primary hyperparathyroidism due to parathyroid adenoma. Diagnosis was based on laboratory examination and later confirmed by instrumental methods: when hypercalcemia was detected, the necessary determination of parathyroid hormone level took place and when it increased, a mandatory biochemical analysis of urine was performed as well as required. Despite the rarity of primary hyperparathyroidism in childhood, alertness in relation to this pathology is necessary for pediatric practitioners of various specialties, including pediatric physicians and nephrologists, since this pathology is more likely in cases of nephrocalcinosis, nephrolithiasis and urolithiasis in children.
{"title":"PRIMARY HYPERPARATHYROIDISM AS A CAUSE OF NEPHROCALCINOSIS AND NEPHROLITHIASIS IN AN ADOLESCENT PATIENT: A CLINICAL CASE","authors":"E. Orekhova, O. Chugunova, S. Blokh, M. Boyadjan, M. Yudin, P.V. Pavlova, O. I. Yaroshevskaya, P. Shumilov","doi":"10.24110/0031-403x-2023-102-4-186-193","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-186-193","url":null,"abstract":"Article describes a clinical case of a female adolescent with macrohematuria, nephrocalcinosis, nephrolithiasis and cholelithiasis, in which laboratory examination and imaging of parathyroid glands gave grounds to diagnose primary hyperparathyroidism due to parathyroid adenoma. Diagnosis was based on laboratory examination and later confirmed by instrumental methods: when hypercalcemia was detected, the necessary determination of parathyroid hormone level took place and when it increased, a mandatory biochemical analysis of urine was performed as well as required. Despite the rarity of primary hyperparathyroidism in childhood, alertness in relation to this pathology is necessary for pediatric practitioners of various specialties, including pediatric physicians and nephrologists, since this pathology is more likely in cases of nephrocalcinosis, nephrolithiasis and urolithiasis in children.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77521949","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-2023-102-4-172-176
E. Vasichkina, N. Vashakmadze, S. Voronin, E. Zakharova, A. Koroteev, E. Krasilnikova, S. Kutsev, S. V. Moiseev, T. Pervunina, A. G. Rumyantsev, E. Serebryakova, E.A. Khvostikova
For citation: E.S. Vasichkina, N.D. Vashakmadze, S.V. Voronin, E.Yu. Zakharova, A.L. Koroteev, E.Yu. Krasilnikova, S.I. Kutsev, S.V. Moiseev, T.M. Pervunina, A.G. Rumyantsev, E.A. Serebryakova, E.A. Khvostikova. «Diagnosis and treatment of Fabry disease: prospects and challenges.» Resolution by the Council of Experts dated April 20, 2023. Moscow, Russia. Pediatria n.a. G.N. Speransky. 2023; 102 (4): 172-176. DOI: 10.24110/0031-403X-2023-102-4-172-176.
{"title":"«DIAGNOSIS AND TREATMENT OF FABRY DISEASE: PROSPECTS AND CHALLENGES.» RESOLUTION BY THE COUNCIL OF EXPERTS DATED APRIL 20, 2023. MOSCOW, RUSSIA","authors":"E. Vasichkina, N. Vashakmadze, S. Voronin, E. Zakharova, A. Koroteev, E. Krasilnikova, S. Kutsev, S. V. Moiseev, T. Pervunina, A. G. Rumyantsev, E. Serebryakova, E.A. Khvostikova","doi":"10.24110/0031-403x-2023-102-4-172-176","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-172-176","url":null,"abstract":"For citation: E.S. Vasichkina, N.D. Vashakmadze, S.V. Voronin, E.Yu. Zakharova, A.L. Koroteev, E.Yu. Krasilnikova, S.I. Kutsev, S.V. Moiseev, T.M. Pervunina, A.G. Rumyantsev, E.A. Serebryakova, E.A. Khvostikova. «Diagnosis and treatment of Fabry disease: prospects and challenges.» Resolution by the Council of Experts dated April 20, 2023. Moscow, Russia. Pediatria n.a. G.N. Speransky. 2023; 102 (4): 172-176. DOI: 10.24110/0031-403X-2023-102-4-172-176.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":"22 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75823886","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-2023-102-4-112-124
Y. Kozlov, S. Poloyan, A. A. Marchuk, A. Rozhanski, A. A. Byrgazov, K. Kovalkov, C. Ochirov, V. Kapuller, A. Narkevich
Thoracoscopic reconstruction of esophageal atresia (EA) has become a standard procedure in many surgical centers Worldwide. The existing bibliographical reviews and meta-analyses have failed to demonstrate any significant difference in the immediate outcome of mini-aggressive treatment of EA. Authors had performed this truly systematic review and meta-analysis searching for the new evidence and confirmation of success of thoracoscopy in the treatment of EA. Materials and methods used: the systematic bibliographical review was prepared in accordance with the PRISMA 2020 statement guidelines. Thus, the scientific publications starting 1990 were searched in three major databases as follows: MEDLINE, PubMed and eLIBRARY.RU. Results: the included studies were published between 2008 and 2022 representing a total of 1958 cases that included 1369 (69.92%) open-surgery interventions and 589 (30.08%) thoracoscopic ones. Authors have found no significant difference between thoracoscopy and thoracotomy excluding an increased risk for esophageal anastomotic leak in the open-surgery treatment group (OR, 0.68; 95% CI, 0.46; 0.99; p = 0.04). A meta-analysis demonstrated that the thoracoscopy group had shorter time spent on lung ventilation (MD, -1.83; 95% CI, -3.02; -0.63; p = 0.003), shorter period to start oral feeding (MD, -1.34; 95% CI, -2.13; -0.56; p = 0.0008) and shorter hospital stay (MD, -4.26; 95% CI, -7.03; -1.49; p = 0.003). The data on the long-term outcomes of both thoracoscopic and open-surgery interventions were reported in a limited - insufficient number of observed cases and had demonstrated that gastroesophageal reflux was more common in the thoracoscopy group (OR, 2.82; 95% CI, 1.72; 4.61; p < 0.0001). Conclusion: the meta-analysis had proved that, compared to open-surgery, thoracoscopy is associated with an almost comparable rate of postoperative complications coupled with the exception of anastomotic leaks. However, it is impossible to ignore the significant advantages of the modern method for treatment of EA which is characterized - among other things - by the more rapid recovery of patients after the surgical interventions.
{"title":"SYSTEMATIC BIBLIOGRAPHICAL REVIEW AND META-ANALYSIS OF THORACOSCOPIC VS. OPEN-SURGICAL TREATMENT OF ESOPHAGEAL ATRESIA","authors":"Y. Kozlov, S. Poloyan, A. A. Marchuk, A. Rozhanski, A. A. Byrgazov, K. Kovalkov, C. Ochirov, V. Kapuller, A. Narkevich","doi":"10.24110/0031-403x-2023-102-4-112-124","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-112-124","url":null,"abstract":"Thoracoscopic reconstruction of esophageal atresia (EA) has become a standard procedure in many surgical centers Worldwide. The existing bibliographical reviews and meta-analyses have failed to demonstrate any significant difference in the immediate outcome of mini-aggressive treatment of EA. Authors had performed this truly systematic review and meta-analysis searching for the new evidence and confirmation of success of thoracoscopy in the treatment of EA. Materials and methods used: the systematic bibliographical review was prepared in accordance with the PRISMA 2020 statement guidelines. Thus, the scientific publications starting 1990 were searched in three major databases as follows: MEDLINE, PubMed and eLIBRARY.RU. Results: the included studies were published between 2008 and 2022 representing a total of 1958 cases that included 1369 (69.92%) open-surgery interventions and 589 (30.08%) thoracoscopic ones. Authors have found no significant difference between thoracoscopy and thoracotomy excluding an increased risk for esophageal anastomotic leak in the open-surgery treatment group (OR, 0.68; 95% CI, 0.46; 0.99; p = 0.04). A meta-analysis demonstrated that the thoracoscopy group had shorter time spent on lung ventilation (MD, -1.83; 95% CI, -3.02; -0.63; p = 0.003), shorter period to start oral feeding (MD, -1.34; 95% CI, -2.13; -0.56; p = 0.0008) and shorter hospital stay (MD, -4.26; 95% CI, -7.03; -1.49; p = 0.003). The data on the long-term outcomes of both thoracoscopic and open-surgery interventions were reported in a limited - insufficient number of observed cases and had demonstrated that gastroesophageal reflux was more common in the thoracoscopy group (OR, 2.82; 95% CI, 1.72; 4.61; p < 0.0001). Conclusion: the meta-analysis had proved that, compared to open-surgery, thoracoscopy is associated with an almost comparable rate of postoperative complications coupled with the exception of anastomotic leaks. However, it is impossible to ignore the significant advantages of the modern method for treatment of EA which is characterized - among other things - by the more rapid recovery of patients after the surgical interventions.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":"70 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88624594","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-2023-102-4-92-100
A. Safina, O. Chugunova, S. Paunova, M. A. Daminova
Urinary tract infections are a common problem in premature newborns, but its diagnosis is of certain difficulties due to the non-specific clinical picture and laboratory parameters, which in its turn complicates timely treatment and can therefore lead to the development of unfavorable complications. The article provides a bibliographical review on the issue and discusses the modern approaches to the diagnosis and treatment of urinary tract infections in premature newborns.
{"title":"URINARY TRACT INFECTIONS IN PRETERM INFANTS: DIFFICULTIES IN DIAGNOSIS AND TREATMENT","authors":"A. Safina, O. Chugunova, S. Paunova, M. A. Daminova","doi":"10.24110/0031-403x-2023-102-4-92-100","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-92-100","url":null,"abstract":"Urinary tract infections are a common problem in premature newborns, but its diagnosis is of certain difficulties due to the non-specific clinical picture and laboratory parameters, which in its turn complicates timely treatment and can therefore lead to the development of unfavorable complications. The article provides a bibliographical review on the issue and discusses the modern approaches to the diagnosis and treatment of urinary tract infections in premature newborns.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":"19 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82525309","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-2023-102-4-73-79
L. Kiselnikova, V. Tsarev, M. Podporin, F.M. Balafendieva
Eruption of temporary teeth in children is accompanied by a wide range of undesirable local and general manifestations and the changes in the oral cavity microbiocenosis. The purpose of this research was to evaluate the clinical and microbiological effectiveness of the use of topical drugs in the syndrome of difficult temporary teeth eruption in children. Materials and methods used: 120 children aged 5 months to 2 years old randomly distributed into 3 groups of 40 with difficulties in the eruption of temporary teeth. For the studied period of eight days all of the children have been receiving drugs to relieve unwanted symptoms in case of difficult eruption of temporary teeth: Dantinorm Baby by BOIRON, France, CALGEL Teething Gel by GlaxoSmithKline Pharmaceuticals, UK/Poland, and Viburgel by Biologische Heilmittel Heel GmbH, Germany. The dental status was carried out according to the PMA index; the state of the oral cavity microbiocenosis was assessed as well. Results: the gums’ PMA index after the course of Dantinorm Baby corresponded to mild degree of gingivitis, whilst both CALGEL Teething Gel and Viburgel had corresponded to the moderate degree. Against the background of the drug use the ratio between cariogenic and periodontopathogenic groups of the microbiota shifted towards the predominance of stabilizing species such as S. salivarius, S. sanguis, Corynebacterium spp. and Veillonella parvula in the group 1 versus the use of the drugs in the groups 2 and 3. Conclusions: clinical efficacy of the Dantinorm Baby drug in difficult temporary teeth eruption was higher, was accompanied by stabilization of oral microbiocenosis in children.
{"title":"CLINICAL AND MICROBIOLOGICAL EFFICACY OF THE TREATMENT OF DIFFICULT TEMPORARY TEETH ERUPTION IN CHILDREN","authors":"L. Kiselnikova, V. Tsarev, M. Podporin, F.M. Balafendieva","doi":"10.24110/0031-403x-2023-102-4-73-79","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-73-79","url":null,"abstract":"Eruption of temporary teeth in children is accompanied by a wide range of undesirable local and general manifestations and the changes in the oral cavity microbiocenosis. The purpose of this research was to evaluate the clinical and microbiological effectiveness of the use of topical drugs in the syndrome of difficult temporary teeth eruption in children. Materials and methods used: 120 children aged 5 months to 2 years old randomly distributed into 3 groups of 40 with difficulties in the eruption of temporary teeth. For the studied period of eight days all of the children have been receiving drugs to relieve unwanted symptoms in case of difficult eruption of temporary teeth: Dantinorm Baby by BOIRON, France, CALGEL Teething Gel by GlaxoSmithKline Pharmaceuticals, UK/Poland, and Viburgel by Biologische Heilmittel Heel GmbH, Germany. The dental status was carried out according to the PMA index; the state of the oral cavity microbiocenosis was assessed as well. Results: the gums’ PMA index after the course of Dantinorm Baby corresponded to mild degree of gingivitis, whilst both CALGEL Teething Gel and Viburgel had corresponded to the moderate degree. Against the background of the drug use the ratio between cariogenic and periodontopathogenic groups of the microbiota shifted towards the predominance of stabilizing species such as S. salivarius, S. sanguis, Corynebacterium spp. and Veillonella parvula in the group 1 versus the use of the drugs in the groups 2 and 3. Conclusions: clinical efficacy of the Dantinorm Baby drug in difficult temporary teeth eruption was higher, was accompanied by stabilization of oral microbiocenosis in children.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":"203 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72799518","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-2023-102-4-52-58
M. Aksenova, N. Konkova, O. Piruzeeva, N. Zaikova, T. Lepaeva, T. Nikishina, V. Obukhova, V. Dlin
Males with X-linked Alport syndrome (XLAS) have a poor prognosis with a risk of developing kidney failure at a young age. Renal survival in glomerulopathies is determined by genetic and non-genetic risk factors with the role of the latter in Alport syndrome not being studied enough as yet. The purpose of this research was to determine the risk factors for poor nephrological prognosis in boys with XLAS. Materials and methods used: 89 boys aged 3 to 14 y/o with XLAS were included in a single-center cohort retrospective study. Blood pressure (BP), proteinuria (Pr, mg/m2/day), estimated glomerular filtration rate according to Bedside Schwartz Formula (eGFR, ml/min/1.73 m2 with norm≥90 ml/min/1.73 m2) and the data on therapy with angiotensin-converting enzyme inhibitors (ACEI) were monitored one time per 6 to 12 months for 4 [3; 6] years. BP≥90% was defined as uncontrolled (UHBP); proteinuria criterion Pr>100 mg/m2/day; the research endpoint was the time to reach eGFR<60 ml/min/1.73 m2. Results: 69 children had eGFR≥60 ml/min/1.73 m2 in dynamics (Group 1), 20 had reached the endpoint (Group 2). Age at inclusion, incidence of non-missense COL4A5 variants, birth weight, incidence of low body weight for gestational age, gross hematuria and age at onset of hematuria were not statistically significantly different between groups; UHBP (0.2 for G1 vs. 0.65 for G2; p<0.001), Pr (0.61 vs. 0.95, respectively; p=0.004), decrease in eGFR (0.06 vs. 0.45; p<0.001) were statistically significantly more common in G2. Boys from G2 were less likely to receive ACEI therapy (0.97 vs. 0.65; p=0.012); age of onset and doses of ACEI for ramipril were not statistically significantly different between the groups. Non-missense variants of COL4A5 (p=0.007), NBP (p<0.001), persistence of Pr>250 mg/m2/day (p<0.001), no ACEI therapy (p<0.001) and its onset at the stage of proteinuria (p<0.001) were the risk factors for reaching the endpoint. Persistence of Pr, UHBP, initiation of ACEI therapy at the proteinuria stage independently affected the progression of the disease. Conclusion: UHBP, proteinuria, lack of therapy/appointment of ACEI at the proteinuria stage of the disease are the risk factors for further poor nephrological prognosis in boys with XLAS.
{"title":"RISK FACTORS FOR POOR NEPHROLOGICAL PROGNOSIS IN BOYS WITH X-LINKED ALPORT SYNDROME: A SINGLE CENTER COHORT STUDY","authors":"M. Aksenova, N. Konkova, O. Piruzeeva, N. Zaikova, T. Lepaeva, T. Nikishina, V. Obukhova, V. Dlin","doi":"10.24110/0031-403x-2023-102-4-52-58","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-4-52-58","url":null,"abstract":"Males with X-linked Alport syndrome (XLAS) have a poor prognosis with a risk of developing kidney failure at a young age. Renal survival in glomerulopathies is determined by genetic and non-genetic risk factors with the role of the latter in Alport syndrome not being studied enough as yet. The purpose of this research was to determine the risk factors for poor nephrological prognosis in boys with XLAS. Materials and methods used: 89 boys aged 3 to 14 y/o with XLAS were included in a single-center cohort retrospective study. Blood pressure (BP), proteinuria (Pr, mg/m2/day), estimated glomerular filtration rate according to Bedside Schwartz Formula (eGFR, ml/min/1.73 m2 with norm≥90 ml/min/1.73 m2) and the data on therapy with angiotensin-converting enzyme inhibitors (ACEI) were monitored one time per 6 to 12 months for 4 [3; 6] years. BP≥90% was defined as uncontrolled (UHBP); proteinuria criterion Pr>100 mg/m2/day; the research endpoint was the time to reach eGFR<60 ml/min/1.73 m2. Results: 69 children had eGFR≥60 ml/min/1.73 m2 in dynamics (Group 1), 20 had reached the endpoint (Group 2). Age at inclusion, incidence of non-missense COL4A5 variants, birth weight, incidence of low body weight for gestational age, gross hematuria and age at onset of hematuria were not statistically significantly different between groups; UHBP (0.2 for G1 vs. 0.65 for G2; p<0.001), Pr (0.61 vs. 0.95, respectively; p=0.004), decrease in eGFR (0.06 vs. 0.45; p<0.001) were statistically significantly more common in G2. Boys from G2 were less likely to receive ACEI therapy (0.97 vs. 0.65; p=0.012); age of onset and doses of ACEI for ramipril were not statistically significantly different between the groups. Non-missense variants of COL4A5 (p=0.007), NBP (p<0.001), persistence of Pr>250 mg/m2/day (p<0.001), no ACEI therapy (p<0.001) and its onset at the stage of proteinuria (p<0.001) were the risk factors for reaching the endpoint. Persistence of Pr, UHBP, initiation of ACEI therapy at the proteinuria stage independently affected the progression of the disease. Conclusion: UHBP, proteinuria, lack of therapy/appointment of ACEI at the proteinuria stage of the disease are the risk factors for further poor nephrological prognosis in boys with XLAS.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":"43 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90976240","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 : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-131-136
E.I. Chechev, N. S. Grachev, N. V. Babaskina, I. N. Vorozhtsov, E. Konopleva
Authors analyze bibliographical data on the treatment of osteosarcoma and Ewing's sarcoma of the maxillofacial region in children and adolescents. The role of surgical treatment in the primary lesion of this localization is analyzed in detail as well as the complex therapeutic approaches.
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Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-174-182
Y. Samoilova, M. Kovarenko, O. Oleynik, D. Kudlay, M. Matveeva, D. Podchinenova, I. Novikova, S. Romanenko
Obesity is the most common reason for the insulin resistance (IR) though obesity in children is not always associated with IR. The markers based on fasting glucose and/or insulin levels and alternative parameters with conventional lipid-based indexes are used in assessing the IR in clinical practice. The purpose of the research was to assess the relationship between alternative IR surrogate biomarkers as follows: triglycerides (TG) to high-density lipoprotein cholesterol (HDL-C), TG/HDL-C ratio; single-point insulin sensitivity estimator (SPISE) index; triglyceride-glucose index (TyG), with generally accepted indices based on the insulin/glucose ratios, such as HOMA-IR, QUICKI, Caro, in a cohort of obese children and adolescents. Materials and methods used: a single-stage multicenter comparative study was conducted involving 127 patients aged 6 to 17 y/o with simple obesity of varying degrees. The following indices were calculated: HOMA-IR = fasting insulin (mU/ml) x fasting plasma glucose (FPG) (mmol/L) / 22.5; QUICKI = 1/Log (Fasting Insulin, µU/ml) + Log (Fasting Glucose, mg/dl); Caro = fasting plasma glucose (mmol/l) / fasting insulin (μU/ml); TG/HDL-C = TG (mmol/L) / HDL-C (mmol/L); triglyceride-glucose index TyG = Ln [fasting TG (mg/dl) x fasting plasma glucose (mg/dl)/2], where Ln is the logarithm; SPISE = 600 × HDL-C0.185/(TG0.2 x BMI1.338). Results: 75 (59%) boys and 52 (41%) girls were included in the study; the median age was 12.9 [10.4; 14.9] y/o. Pre-pubertal development was recorded in 25 (19.7%), pubertal - in 102 (80.3%). The prevalence of impaired glucose tolerance (IGT) was 5.5%. In children with IGT, the SPISE index was lower than in children with normal glucose tolerance (3.69 [3.49; 4.37] vs. 4.8 [3.99; 5.55], p=0.021) with no statistically significant differences between these groups in other markers. In the IGT subgroup, a high negative correlation was found between SDS BMI and SPISE (R=-0.97). In the general SDS group, BMI correlated with insulin (R=+0.24), TG (R=+0.2), HOMA (R=+0.24), Caro (R=-0.26), TG/HDL-C (R=+0.28) and SPISE (R=-0.65). The SPISE index was lower in girls than in boys (4.94 [3.63; 5.73] vs. 4.07 [3.59; 5.16], p = 0.045). No statistically significant differences were recorded for other gender-related markers. A negative correlation was also found between the obesity degree and SPISE in the subgroups of girls (R=-0.49) and boys (R=-0.70) as well as among pre-pubertal children of both genders (R=-0.61). The maximum correlation between SDS BMI and SPISE was recorded in the subgroup of teenagers of both genders (R=-0.88). In the general group, TyG was weakly correlated with HOMA-IR (R=+0.26) and QUICKI (R=-0.24), while SPISE showed a weak association with all studied insulin indices; no statistically significant correlation between insulin indices and TG/HDL-C was found. In the subgroup of girls, no significant relationships between the evaluated markers were found, in the subgroup of boys, TyG weakly correl
{"title":"SURROGATE BIOMARKERS OF INSULIN RESISTANCE IN OBESE CHILDREN AND ADOLESCENTS","authors":"Y. Samoilova, M. Kovarenko, O. Oleynik, D. Kudlay, M. Matveeva, D. Podchinenova, I. Novikova, S. Romanenko","doi":"10.24110/0031-403x-2023-102-3-174-182","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-174-182","url":null,"abstract":"Obesity is the most common reason for the insulin resistance (IR) though obesity in children is not always associated with IR. The markers based on fasting glucose and/or insulin levels and alternative parameters with conventional lipid-based indexes are used in assessing the IR in clinical practice. The purpose of the research was to assess the relationship between alternative IR surrogate biomarkers as follows: triglycerides (TG) to high-density lipoprotein cholesterol (HDL-C), TG/HDL-C ratio; single-point insulin sensitivity estimator (SPISE) index; triglyceride-glucose index (TyG), with generally accepted indices based on the insulin/glucose ratios, such as HOMA-IR, QUICKI, Caro, in a cohort of obese children and adolescents. Materials and methods used: a single-stage multicenter comparative study was conducted involving 127 patients aged 6 to 17 y/o with simple obesity of varying degrees. The following indices were calculated: HOMA-IR = fasting insulin (mU/ml) x fasting plasma glucose (FPG) (mmol/L) / 22.5; QUICKI = 1/Log (Fasting Insulin, µU/ml) + Log (Fasting Glucose, mg/dl); Caro = fasting plasma glucose (mmol/l) / fasting insulin (μU/ml); TG/HDL-C = TG (mmol/L) / HDL-C (mmol/L); triglyceride-glucose index TyG = Ln [fasting TG (mg/dl) x fasting plasma glucose (mg/dl)/2], where Ln is the logarithm; SPISE = 600 × HDL-C0.185/(TG0.2 x BMI1.338). Results: 75 (59%) boys and 52 (41%) girls were included in the study; the median age was 12.9 [10.4; 14.9] y/o. Pre-pubertal development was recorded in 25 (19.7%), pubertal - in 102 (80.3%). The prevalence of impaired glucose tolerance (IGT) was 5.5%. In children with IGT, the SPISE index was lower than in children with normal glucose tolerance (3.69 [3.49; 4.37] vs. 4.8 [3.99; 5.55], p=0.021) with no statistically significant differences between these groups in other markers. In the IGT subgroup, a high negative correlation was found between SDS BMI and SPISE (R=-0.97). In the general SDS group, BMI correlated with insulin (R=+0.24), TG (R=+0.2), HOMA (R=+0.24), Caro (R=-0.26), TG/HDL-C (R=+0.28) and SPISE (R=-0.65). The SPISE index was lower in girls than in boys (4.94 [3.63; 5.73] vs. 4.07 [3.59; 5.16], p = 0.045). No statistically significant differences were recorded for other gender-related markers. A negative correlation was also found between the obesity degree and SPISE in the subgroups of girls (R=-0.49) and boys (R=-0.70) as well as among pre-pubertal children of both genders (R=-0.61). The maximum correlation between SDS BMI and SPISE was recorded in the subgroup of teenagers of both genders (R=-0.88). In the general group, TyG was weakly correlated with HOMA-IR (R=+0.26) and QUICKI (R=-0.24), while SPISE showed a weak association with all studied insulin indices; no statistically significant correlation between insulin indices and TG/HDL-C was found. In the subgroup of girls, no significant relationships between the evaluated markers were found, in the subgroup of boys, TyG weakly correl","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":"14 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91213386","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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