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Myocardial effects of a low-protein diet in experimental kidney dysfunction 低蛋白饮食对实验性肾功能障碍的心肌影响
Pub Date : 2022-11-25 DOI: 10.36485/1561-6274-2022-26-4-110-118
O. Beresneva, M. Parastaeva, G. Ivanova, M. Zaraiski, S. Orlova, A. Kucher
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引用次数: 1
Fetuin A is assessing of developing bone mineral disorders and cardiovascular calcifi cation formation risk marker in patients with stage 5 chronic kidney disease 胎儿素A评估5期慢性肾病患者发生骨矿物质障碍和心血管钙化形成的风险标志物
Pub Date : 2022-11-24 DOI: 10.36485/1561-6274-2022-26-4-105-109
A. T. Makhieva, A. Mambetova
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引用次数: 0
The effectiveness of Cyclosporine A use in children with Alport syndrome: single center study 环孢素A在儿童Alport综合征中的应用效果:单中心研究
Pub Date : 2022-11-24 DOI: 10.36485/1561-6274-2022-26-4-66-73
K. V. Shebalkina, E. Petrosyan, P. Shumilov
BACKGROUND: Alport syndrome is a non-immune genetically determined glomerulopathy caused by mutation of genes encoding α3-5 chains of collagen type IV of the basement membranes. It manifests with hematuria and/or proteinuria, progressive renal functions decrease, often in combination with hearing and vision pathology. According to world statistics the incidence of Alport syndrome is less than 1:5000 people. THE AIM: We analyzed the effectiveness of combined Cyclosporine A and nephroprotective therapy in children with Alport syndrome in comparison with nephroprotectors only. PATIENTS AND METHODS: 35 patients were enrolled in retrospective controlled comparative non-randomized single-center longitudinal study: 9 girls (26 %) and 26 boys (74 %). The median age Me was 8,7 [5,4; 13,7] years old. The patients were divided into 2 groups. Group 1 (n=25) – patients receiving Cyclosporine A and nephroprotective therapy, group 2 (n=10) – patients receiving nephroprotective therapy only. The groups did not differ statistically significantly. The observation period was 24 months. The effectiveness of therapy was assessed by reducing proteinuria. RESULTS: In group 1, the level of proteinuria decreased significantly, especially in the first 6 months. Despite gradual increase in the level of proteinuria in this group, by 24 months of follow-up, there was statistically significant difference compared to baseline (1872.0 [1195.0; 2531.0] vs 805.0 [306.0; 1504.0]; p=0.0005). Use of nephroprotectors did not change significantly the dynamics of proteinuria. In general, after 2 years, the level of proteinuria remained practically the same (1812.0 [1508.0; 2093.0] vs 1080.0 [147.0; 3141.0]; p = 0.11). Glomerular filtration rate in two groups did not change significantly during the observation period: in group 1 – 133 [108; 146] vs 123 [106; 131]; p=0.1 and in group 2 – 124 [64; 133] vs 81 [40; 102]; p=0.18. CONCLUSION: The relative safety and efficacy of combined use of Cyclosporine A in low doses and nephroprotectors was shown in children with Alport syndrome with nephrotic proteinuria and glomerular filtration rate > 60 ml/min/1.73m2, if monocomponent nephroprotective therapy was ineffective.
背景:Alport综合征是一种非免疫遗传决定的肾小球病变,由编码基底膜α3-5型胶原链的基因突变引起。表现为血尿和/或蛋白尿,进行性肾功能下降,常伴有听觉和视觉病变。据世界统计,阿尔波特综合征的发病率不到50 000人。目的:我们分析环孢素A联合肾保护治疗儿童Alport综合征的有效性,并与单独使用肾保护药物进行比较。患者和方法:35例患者纳入回顾性对照比较非随机单中心纵向研究:9例女孩(26%)和26例男孩(74%)。中位年龄为8、7岁[5、4岁;[13,7]岁。患者分为两组。组1 (n=25) -接受环孢素A和肾保护治疗的患者,组2 (n=10) -仅接受肾保护治疗的患者。两组间差异无统计学意义。观察期24个月。通过减少蛋白尿来评估治疗的有效性。结果:1组患者蛋白尿水平明显下降,且以治疗前6个月下降最为明显。尽管该组蛋白尿水平逐渐升高,但随访24个月后,与基线相比仍有统计学差异(1872.0 [1195.0;2531.0] vs 805.0 [306.0;1504.0);p = 0.0005)。肾保护剂的使用并没有显著改变蛋白尿的动态。总的来说,2年后,蛋白尿水平基本保持不变(1812.0 [1508.0;2093.0] vs 1080.0 [147.0;3141.0);P = 0.11)。两组患者的肾小球滤过率在观察期内无明显变化:1 ~ 133组[108;[146] vs [106;131);P =0.1, 2 ~ 124组[64;[133] vs . 81 [40;102);p = 0.18。结论:在单组份肾保护治疗无效的Alport综合征肾病蛋白尿、肾小球滤过率> 60 ml/min/1.73m2患儿中,低剂量环孢素A与肾保护药物联合应用具有相对安全性和有效性。
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引用次数: 0
Factors of proteolysis in blood and urine as prognostic markers of progression of Alport syndrome in children 血液和尿液中蛋白水解因子作为儿童Alport综合征进展的预后标志物
Pub Date : 2022-11-24 DOI: 10.36485/1561-6274-2022-26-4-80-88
Z. Bashirova, I. Osmanov
BACKGROUND. Alport syndrome is a rare hereditary kidney disease that causes progressive renal failure. There are significant differences in the progression of the disease between patients with Alport syndrome. Identifying patients with a high risk of rapid progression in order to optimally balance benefits and risks for prescribing therapy has become particularly important at this time. In this study, we wanted to assess whether the factors of proteolysis in blood and urine are associated with the nature of the course and to assess their prognostic value for children with Alport syndrome. THE AIM: To determine the level in blood serum and urinary excretion of MMP-2, MMP-3 and MMP-9 and their inhibitors TIMP-1 and 2, PAI-I, to show the relationship of their changes with the character of the course of Alport syndrome in children as an additional criterion for progression. PATIENTS AND METHODS. The study included 32 children with Alport syndrome. The level of MMP-2, MMP-3 and MMP-9 and their inhibitors TIMP-1 and 2, PAI-I, in blood serum and urine was determined by ELISA. A decrease in eGFR of ≥ 30 % at 2 years from baseline was chosen to indicate a progressive course of Alport syndrome. RESULTS. 28.1 % of children with Alport syndrome had a progressive course of the dis ease, 71.9 % had a slowly progressive course. The frequency of a decrease in MMP-9 and an increase in TIMP-1 both in blood (88.9 versus 43.5 % and 77.8 versus 21.7 %; p = 0.044 and 0.006, respectively) and in urine (100 versus 47, 8 % and 88.9 versus 30.4 %; 0.012 and 0.005, respectively) were statistically significantly more often detected in children with Alport syndrome with a progressive course of the disease than in a slowly progressive course. CONCLUSION. Type 9 matrix metalloproteinase and type 1 tissue matrix metalloproteinase inhibitor can be considered as risk factors for the progression of Alport syndrome in children.
背景。阿尔波特综合征是一种罕见的遗传性肾脏疾病,可导致进行性肾衰竭。阿尔波特综合征患者的病情进展有显著差异。识别快速进展的高风险患者,以最佳地平衡处方治疗的益处和风险,在这个时候变得尤为重要。在这项研究中,我们想要评估血液和尿液中的蛋白水解因素是否与病程的性质有关,并评估其对Alport综合征儿童的预后价值。目的:测定血清和尿中MMP-2、MMP-3和MMP-9及其抑制剂TIMP-1和2 pai -1的水平,以显示其变化与儿童Alport综合征病程特征的关系,作为进展的附加标准。患者和方法。该研究包括32名患有阿尔波特综合症的儿童。ELISA法检测血清和尿液中MMP-2、MMP-3、MMP-9及其抑制剂TIMP-1、2 pai -1的水平。2年eGFR较基线下降≥30%被选为Alport综合征进展性病程的指标。结果。28.1%患儿病程进展,71.9%患儿病程缓慢进展。血液中MMP-9降低和TIMP-1升高的频率(88.9比43.5%和77.8比21.7%;P分别= 0.044和0.006)和尿(100比47.8%和88.9比30.4%;分别为0.012和0.005),在病程进展的Alport综合征患儿中检出率明显高于病程缓慢的Alport综合征患儿。结论。9型基质金属蛋白酶和1型组织基质金属蛋白酶抑制剂可被认为是儿童Alport综合征进展的危险因素。
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引用次数: 0
Functional state of the kidneys in the neonatal period in children with very low and extremely low body weight 极低和极低体重儿童新生儿期肾脏功能状态的研究
Pub Date : 2022-11-24 DOI: 10.36485/1561-6274-2022-26-4-89-96
N. Korotaeva, T. L. Nastausheva, L. I. Ippolitova
BACKGROUND. Preterm birth is still associated with an increased risk of neonatal morbidity and mortality in the early neonatal period. There is strong evidence demonstrating an association between a decrease in the number of nephrons in preterm infants and an increase in blood pressure, the risk of developing chronic kidney disease, which undoubtedly negatively affects the quality of life. THE AIM: to assess the functional state of the kidneys in children with very low (VLBW) and extremely low body weight (ELBW) in the first 8 weeks of postnatal life. PATIENTS AND METHODS. The study involved 134 newborns less than 37 weeks of gestation, who were divided into three groups depending on birth weight. The levels of protein and fluid intake, serum creatinine concentration, GFR according to Schwartz were taken into account as evaluation parameters. The Python programming language, t-tests, ShapiroWilk and d'Agostino tests were used as statistical methods. A threshold level of 0.05 was chosen to interpret the value of p tests for normality testing. RESULTS. There were no differences in the amount of protein received by preterm infants in the study groups both in the first week and subsequent 2–8 weeks of life. The average level of incoming fluid in the first week of postnatal life increased from 1 to 7 days in all study groups. There was a trend towards a more significant decrease in serum creatinine in children born with a larger birth weight. Analyzing the level of glomerular filtration rate in the studied groups, there is a clear picture of a progressive increase in the rate with age. CONCLUSION. The values of diuresis, creatinine level and GFR in premature babies with birth weight less than 1500 grams in the first 2 months of life have been established, which can be used in practice for comparison in the study of various pathologies.
背景。早产仍与新生儿早期发病和死亡风险增加有关。有强有力的证据表明,早产儿肾单位数量减少与血压升高、患慢性肾脏疾病的风险增加之间存在关联,这无疑会对生活质量产生负面影响。目的:评估极低(VLBW)和极低体重(ELBW)儿童在出生后前8周的肾脏功能状态。患者和方法。这项研究涉及134名怀孕不到37周的新生儿,根据出生体重将他们分成三组。蛋白质和液体摄入量水平,血清肌酐浓度,GFR根据Schwartz被考虑作为评价参数。统计学方法采用Python编程语言、t检验、ShapiroWilk检验和d’agostino检验。选择0.05的阈值水平来解释p检验的正态性检验值。结果。在第一周和随后的2-8周内,研究组早产儿接受的蛋白质量没有差异。在所有研究组中,出生后第一周的平均液体水平从1天增加到7天。在出生体重较大的儿童中,血清肌酐有更显著下降的趋势。通过分析实验组的肾小球滤过率水平,可以清楚地看到随着年龄的增长,肾小球滤过率呈进行性增加的趋势。结论。已确定出生体重小于1500克的早产儿在出生后2个月的利尿、肌酐水平和GFR值,可在实践中用于各种病理研究的比较。
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引用次数: 0
The rate of chronic kidney disease progression in advanced stages and the dynamics of the uremic syndrome parameters 慢性肾脏疾病晚期进展率和尿毒症综合征参数的动态变化
Pub Date : 2022-11-24 DOI: 10.36485/1561-6274-2022-26-4-50-65
D. Sadovskaya, K. A. Vishnevsky, I. N. Konakova, N. Bakulina
Background. The current practice of patients with advanced CKD stages management is not optimal, as it leads to the risk of an emergency dialysis start with an unfavorable prognosis, does not utilize all the possibilities of nephroprotective therapy and does not provide optimal correction of the most important uremic syndromes before starting dialysis, which worsens the per spectives of long-term patient-oriented dialysis treatment. THE AIM. The obtained features of the standard practice will provide the possibility to assemble group carefully matched with intensive management group to compare outcomes in future prospective study and to assess the significance of the proposed program components of the intensive management of patients with advanced stages of chronic kidney disease in the "transition center". PATIENTS AND METHODS. A group with regular (at least 6 visits per year) follow-up of 540 patients with baseline CKD3B was retrospectively formed from the city nephrology center database (which included 7696 patients with CKD3 and higher) and was traced to the need for renal replacement therapy or to death. As part of the follow-up, patients underwent regular clinical and laboratory evaluation and received nephroprotective therapy, which were recorded in the database. RESULTS. The dynamics of an accelerating decrease in eGFR (according to CKD-EPICr) from median of -2.76 (-3.26÷-2.36) to -4.34 (-5.01÷-3.46) and further to -6.01 (-7.11÷-5.23) ml/min/1.73 m2/ year for the stages of CKD3B→CKD4→CKD5 in parallel with the dynamics of blood levels of hemoglobin (and iron), phosphate (and calcium), albumin, as well as proteinuria is described – factors that turned out to be significant in the multiple regression model with a dependent variable – the rate of eGFR reduction (the significance of the model F=2.864; p=0.015). CONCLUSION. The obtained detailed description of the progression of CKD in a typical regional population under standard management conditions will provide the possiblity to form a group from a cohort of regular monitoring in a nephrocenter, carefully compared with an intensive management group in the prototype of a transition center based on a large inpatient dialysis center to assess the significance of the components of the proposed control and interventions program.
背景。目前对晚期CKD患者的管理并不理想,因为它会导致紧急透析开始的风险和不良预后,没有利用肾保护治疗的所有可能性,也没有在开始透析前提供最重要的尿毒症综合征的最佳纠正,这使长期以患者为导向的透析治疗的前景恶化。的目标。所获得的标准实践的特征将提供可能性,将组与强化管理组仔细匹配,以比较未来前瞻性研究的结果,并评估在“过渡中心”对晚期慢性肾脏疾病患者进行强化管理的拟议方案组成部分的意义。患者和方法。对540例基线CKD3B患者(包括7696例CKD3及以上患者)进行定期(每年至少6次)随访的一组回顾性研究,追踪到需要肾脏替代治疗或死亡。作为随访的一部分,患者接受了定期的临床和实验室评估,并接受了肾保护治疗,这些都被记录在数据库中。结果。在CKD3B→CKD4→CKD5阶段,eGFR加速下降的动态(根据CKD-EPICr)从中位数-2.76 (-3.26÷-2.36)到-4.34 (-5.01÷-3.46),并进一步到-6.01 (-7.11÷-5.23) ml/min/1.73 m2/年,与血液中血红蛋白(和铁)、磷酸盐(和钙)、白蛋白、以及蛋白尿——在具有因变量的多重回归模型中被证明是显著的因素——eGFR降低率(模型显著性F=2.864;p = 0.015)。结论。在标准管理条件下获得的典型区域人群CKD进展的详细描述将提供从肾中心定期监测队列中形成一个组的可能性,并与基于大型住院透析中心的过渡中心原型中的强化管理组进行仔细比较,以评估所提议的控制和干预计划组成部分的重要性。
{"title":"The rate of chronic kidney disease progression in advanced stages and the dynamics of the uremic syndrome parameters","authors":"D. Sadovskaya, K. A. Vishnevsky, I. N. Konakova, N. Bakulina","doi":"10.36485/1561-6274-2022-26-4-50-65","DOIUrl":"https://doi.org/10.36485/1561-6274-2022-26-4-50-65","url":null,"abstract":"Background. The current practice of patients with advanced CKD stages management is not optimal, as it leads to the risk of an emergency dialysis start with an unfavorable prognosis, does not utilize all the possibilities of nephroprotective therapy and does not provide optimal correction of the most important uremic syndromes before starting dialysis, which worsens the per spectives of long-term patient-oriented dialysis treatment. THE AIM. The obtained features of the standard practice will provide the possibility to assemble group carefully matched with intensive management group to compare outcomes in future prospective study and to assess the significance of the proposed program components of the intensive management of patients with advanced stages of chronic kidney disease in the \"transition center\". PATIENTS AND METHODS. A group with regular (at least 6 visits per year) follow-up of 540 patients with baseline CKD3B was retrospectively formed from the city nephrology center database (which included 7696 patients with CKD3 and higher) and was traced to the need for renal replacement therapy or to death. As part of the follow-up, patients underwent regular clinical and laboratory evaluation and received nephroprotective therapy, which were recorded in the database. RESULTS. The dynamics of an accelerating decrease in eGFR (according to CKD-EPICr) from median of -2.76 (-3.26÷-2.36) to -4.34 (-5.01÷-3.46) and further to -6.01 (-7.11÷-5.23) ml/min/1.73 m2/ year for the stages of CKD3B→CKD4→CKD5 in parallel with the dynamics of blood levels of hemoglobin (and iron), phosphate (and calcium), albumin, as well as proteinuria is described – factors that turned out to be significant in the multiple regression model with a dependent variable – the rate of eGFR reduction (the significance of the model F=2.864; p=0.015). CONCLUSION. The obtained detailed description of the progression of CKD in a typical regional population under standard management conditions will provide the possiblity to form a group from a cohort of regular monitoring in a nephrocenter, carefully compared with an intensive management group in the prototype of a transition center based on a large inpatient dialysis center to assess the significance of the components of the proposed control and interventions program.","PeriodicalId":19089,"journal":{"name":"Nephrology (Saint-Petersburg)","volume":"64 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88954339","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}
引用次数: 1
The effectiveness of various approaches to the use of renal replacement therapy in the treatment of toxic rhabdomyolysis complicated by acute kidney injury 不同方法使用肾脏替代疗法治疗中毒性横纹肌溶解合并急性肾损伤的有效性
Pub Date : 2022-11-24 DOI: 10.36485/1561-6274-2022-26-4-40-49
S. V. Masolitin, D. Protsenko, I. N. Tyurin, O. Mamontova, M. Magomedov, T. G. Kim, M. V. Zakharov, A. V. Marukhov, N. V. Chubchenko
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引用次数: 0
Glomerular fi ltration rate and erythrocyte sedimentation rate in various bronchial asthma variants 不同支气管哮喘变异的肾小球滤过率和红细胞沉降率
Pub Date : 2022-11-24 DOI: 10.36485/1561-6274-2022-26-4-97-104
V. N. Mineev, T. Vasilieva, I. Nesterovich, T. M. Lalaeva
BACKGROUND. Previously, we postulated the common pathogenetic mechanisms in bronchial asthma (BA) and chronic kidney disease (CKD). Given that both the glomerular filtration rate and the erythrocyte sedimentation rate directly depend on the rheological properties of the blood, it was of interest to compare these two important characteristics in different types of bronchial asthma. At the same time, we considered the erythrocyte sedimentation rate (ESR) not only as a factor in systemic inflammation, but also as a model of erythrocyte aggregation and hemorheology. THE AIM: to compare the level of glomerular filtration rate and erythrocyte sedimentation rate in different types of BA. PATIENTS AND METHODS. 215 BA patients with various BA variants were examined. The glomerular filtration rate (eGFR) was calculated using CKD-EPI. Erythrocyte sedimentation rate (ESR) was determined by the Panchenkov method. The integral eGFR/ESR index was used as the ratio of eGFR and ESR values in each individual patient. RESULTS. The glomerular filtration rate is significantly reduced, and the ESR values are significantly higher in non-allergic and hormone-dependent BA compared with the allergic variant of the disease. In the same groups of patients, a significant decrease in the eGFR/ESR index was revealed. Factor analysis revealed that Factor 1, which characterizes the non-allergic variant of BA, had the component of the eGFR/ESR index with a very high negative factor load along with a high negative factor load of the FEV1 component. Factor 2 reflects the features of endothelial dysfunction in the allergic variant of BA, the allergic variant of BA, and the component of the eGFR/ESR index has practically no factor load in this factor. Factor 3, reflecting the manifestations of an atopic state, with a positive factor load, includes a component of the eGFR/ESR index. CONCLUSION. The data obtained suggest that the development of CKD in bronchial asthma depends primarily on the variant of the disease. The decrease in the eGFR/ ESR index in non-allergic and hormone-dependent variants of BA compared with the allergic variant of the disease indicates the involvement of blood microrheological properties to the development of CKD in these two variants of the disease. On the contrary, in the allergic variant of BA, the development of CKD under these conditions can be restrained.
背景。以前,我们假设支气管哮喘(BA)和慢性肾脏疾病(CKD)的共同发病机制。鉴于肾小球滤过率和红细胞沉降率直接取决于血液的流变学特性,比较这两个重要特征在不同类型支气管哮喘中的作用是有意义的。同时,我们认为红细胞沉降率(ESR)不仅是全身性炎症的一个因素,也是红细胞聚集和血液流变学的一个模型。目的:比较不同类型BA患者肾小球滤过率和红细胞沉降率的变化。患者和方法。对215例不同BA变异的BA患者进行了检查。采用CKD-EPI计算肾小球滤过率(eGFR)。采用Panchenkov法测定红细胞沉降率(ESR)。采用积分eGFR/ESR指数作为每位患者eGFR和ESR值的比值。结果。与变应性BA相比,非变应性和激素依赖性BA的肾小球滤过率显著降低,ESR值显著升高。在同一组患者中,eGFR/ESR指数显著下降。因子分析显示,BA非过敏性变异的特征因子1在eGFR/ESR指数中负因子负荷非常高,FEV1负因子负荷也很高。因子2反映了BA变应性变异体中内皮功能障碍的特征,BA变应性变异体的eGFR/ESR指数成分在该因子中几乎没有因子负荷。因子3反映特应性状态的表现,因子负荷为正,包括eGFR/ESR指数的一个组成部分。结论。所获得的数据表明,支气管哮喘CKD的发展主要取决于疾病的变异。与疾病的变应性变异相比,非变应性和激素依赖性BA变异的eGFR/ ESR指数下降,表明血液微流变学特性参与了这两种疾病变异的CKD发展。相反,在BA的变应性变异中,在这些条件下CKD的发展可以受到抑制。
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引用次数: 0
IgA-nephropathy in children with alport syndrome alport综合征患儿iga肾病
Pub Date : 2022-11-24 DOI: 10.36485/1561-6274-2022-26-4-74-79
M. Aksenova, E. Stolyarevich, P. Povilaitite
BACKGROUND. The widespread use of genetic methods in clinical practice has shown that pathogenic variants in COL4A3, COL4A4, COL4A5 genes associated with Alport syndrome (AS) are detected in 10 % of sporadic and in 20 % of familial cases of IgA nephropathy (IgAN), which suggested a relationship between the two diseases. THE AIM was to determine the frequency and characteristics of the course of IgAN in children with AS. PATIENTS AND METHODS. A single-centre retrospective pilot study included 102 patients with AS. The inclusion criteria were: age 2-18 years, genetic and/or morphological confirmation of AS, availability of morphological data of pts. The comparison group included children and adolescents 2-18 years with morphologically confirmed primary IgAN; the exclusion criterion was the presence of AS-specific glomerular basement membrane changes. IgAN was classified according to the MESTC scale. Demographic (gender, age), clinical (arterial hypertension, AH) and laboratory data (proteinuria (Pr, mg/m2/day), (Schwartz eGFR, ml/min/1.73m2) at the time of the biopsy and at the last examination of patients were assessed. Arterial pressure ≥95‰ for sex, age, height was defined as AH. Pr >100 mg/m2/day, Pr≥500 mg/m2/day and Pr>1000 mg/m2/day were defined as proteinuria, high-level proteinuria and nephrotic level proteinuria, respectively. The statistic parametric and nonparametric methods were used ("Statistica 10", StatSoft Russia). RESULTS. IgAN was detected in 3 of 102 children with AS (q=0.03): 2 girls had heterozygous variants in COL4A3 and COL4A4, a boy had X-linked AS. Two patients had nephrotic proteinuria, 1 had SRNS at onset of IgAN. The comparison group included 25 children with IgAN (17M). Baseline patients age (9±4.2 vs 13±2.7 years), frequency of AH (q1=0.66 vs q2=0.28), eGFR decrease (q1=0.33 vs q2=0.44), eGFR level (91±24 vs 90.8±24 ml/ min/1.73 m2), morphological characteristics of IgAN did not differ significantly by groups; patients with AS were more likely to have nephrotic proteinuria (q1=1 vs q2=0.32, p=0.023). At follow-up (3.8±1.4 years), the groups were comparable in age (12.3±5.2 vs 15±1.8 years), AH frequency (q1=0.66 vs q2=0.5), eGFR level (87±16 vs 91±13 ml/min/1.73m2); children with AS had higher grade Pr (800[0;1150] vs 30[10;100] mg/m2/day, p=0.048) and more often had high-level Pr (q1=0.66 vs q2=0.06, p=0.006) at follow-up observation. The AS was associated with the development of nephrotic-level Pr at onset (r=0.41, p=0.008) and with high-level Pr (r=0.38, p=0.012) during follow-up. CONCLUSION. IgAN was detected in 3 % of children with AS. The presence of COL4A3, COL4A4, COL4A5 genes variants is associated with more pronounced proteinuria at the onset of IgAN and its preservation in the follow-up, and may be a risk factor for more severe course glomerulonephritis. The main limitations of the study: small sample size and duration of follow-up.
背景。遗传方法在临床实践中的广泛应用表明,与Alport综合征(AS)相关的COL4A3, COL4A4, COL4A5基因的致病变异在10%的散发和20%的IgA肾病(IgAN)家族病例中检测到,这表明这两种疾病之间存在关联。目的是确定AS患儿IgAN病程的频率和特征。患者和方法。一项纳入102例AS患者的单中心回顾性先导研究。纳入标准为:年龄2-18岁,AS的遗传和/或形态学确认,患者形态学资料的可用性。对照组包括2-18岁形态学证实的原发性IgAN的儿童和青少年;排除标准是as特异性肾小球基底膜改变。根据MESTC分级对IgAN进行分类。评估患者在活检时和最后一次检查时的人口统计学(性别、年龄)、临床(动脉高血压、AH)和实验室数据(蛋白尿(Pr, mg/m2/day)、(Schwartz eGFR, ml/min/1.73m2)。动脉压≥95‰,性别、年龄、身高均为AH。Pr>100 mg/m2/day、Pr≥500 mg/m2/day和Pr>1000 mg/m2/day分别定义为蛋白尿、高水平蛋白尿和肾病水平蛋白尿。采用统计参数和非参数方法(“Statistica 10”,StatSoft Russia)。结果。102例AS患儿中有3例检测到IgAN (q=0.03),其中2例女孩有COL4A3和COL4A4杂合变异体,1例男孩有x连锁AS。2例患者有肾病性蛋白尿,1例患者在IgAN发作时出现SRNS。对照组为25例IgAN患儿(17M)。基线患者年龄(9±4.2 vs 13±2.7岁)、AH发病频率(q1=0.66 vs q2=0.28)、eGFR下降(q1=0.33 vs q2=0.44)、eGFR水平(91±24 vs 90.8±24 ml/ min/1.73 m2)、IgAN形态学特征各组间无显著差异;AS患者更容易发生肾病性蛋白尿(q1=1 vs q2=0.32, p=0.023)。在随访(3.8±1.4年)时,两组在年龄(12.3±5.2 vs 15±1.8岁)、AH频率(q1=0.66 vs q2=0.5)、eGFR水平(87±16 vs 91±13 ml/min/1.73m2)方面具有可比性;在随访观察中,AS患儿的Pr水平较高(800[0,1150]vs 30[10,100] mg/m2/day, p=0.048),且Pr水平较高(q1=0.66 vs q2=0.06, p=0.006)。AS与发病时肾水平Pr的发展(r=0.41, p=0.008)和随访期间高水平Pr的发展(r=0.38, p=0.012)相关。结论。3%的AS患儿检测到IgAN。COL4A3, COL4A4, COL4A5基因变异的存在与IgAN发病时更明显的蛋白尿及其在随访中的保存相关,并且可能是更严重的肾小球肾炎病程的危险因素。本研究的主要局限性:样本量小,随访时间长。
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引用次数: 0
Are Probiotics Needed in Nephrology? 肾病学需要益生菌吗?
Pub Date : 2022-11-23 DOI: 10.36485/1561-6274-2022-26-4-18-30
A. B. Kuznetzova, E. Prazdnova, V. Chistyakov, O. Kutsevalova, M. Batiushin
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引用次数: 0
期刊
Nephrology (Saint-Petersburg)
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