The Urofacial Syndrome or Ochoa is a very rare clinical condition, and is unknown by a large part of the medical community; it is characterized by an inverted facial expression, resulting from abnormal contraction of facial and ocular muscles, especially when smiling, in addition to the presence of urinary abnormalities. Patients with this syndrome are at a higher risk of developing urinary incontinence, changes in the bladder, vesicoureteral reflux, hydroureteronephrosis, and predisposition to severe urinary infections, in addition to chronic kidney disease. This article presents a case of a 22-year-old female, resident of Piauí/Brazil, who presented at the age of 5, the first symptoms of the disease mainly related to the urinary tract (such as urinary frequency), in addition to the sign of inverted face, in which the patient presents the inverted smile characteristic of the disease when commanded to smile, associated with nocturnal lagophthalmos. The patient evolved at 12 years of age, with end-stage chronic kidney disease and a need for renal replacement therapy. This is one of the rare cases of the disease, in which the patient presents the complete characteristics of the inverted smile pathology and complications in the urinary tract. The inverted facial expression is an easily recognized sign, and it is a very characteristic finding of the disease, not finding explanations of morphological alterations or lesions. therefore, it is evident that early diagnosis with the institution of appropriate treatment, avoids possible damage to the urinary tract from childhood, allowing better management and quality of life in these patients.
{"title":"Ochoa syndrome: An overlooked diagnosis – A case report","authors":"Barros Jacobino Mário Nicolau, Aquino Jacobino Patrícia Barros, Lopes Matheus Saraiva, Avelino Fontenele Júnior Francisco Arisneto","doi":"10.17352/acn.000065","DOIUrl":"https://doi.org/10.17352/acn.000065","url":null,"abstract":"The Urofacial Syndrome or Ochoa is a very rare clinical condition, and is unknown by a large part of the medical community; it is characterized by an inverted facial expression, resulting from abnormal contraction of facial and ocular muscles, especially when smiling, in addition to the presence of urinary abnormalities. Patients with this syndrome are at a higher risk of developing urinary incontinence, changes in the bladder, vesicoureteral reflux, hydroureteronephrosis, and predisposition to severe urinary infections, in addition to chronic kidney disease. This article presents a case of a 22-year-old female, resident of Piauí/Brazil, who presented at the age of 5, the first symptoms of the disease mainly related to the urinary tract (such as urinary frequency), in addition to the sign of inverted face, in which the patient presents the inverted smile characteristic of the disease when commanded to smile, associated with nocturnal lagophthalmos. The patient evolved at 12 years of age, with end-stage chronic kidney disease and a need for renal replacement therapy. This is one of the rare cases of the disease, in which the patient presents the complete characteristics of the inverted smile pathology and complications in the urinary tract. The inverted facial expression is an easily recognized sign, and it is a very characteristic finding of the disease, not finding explanations of morphological alterations or lesions. therefore, it is evident that early diagnosis with the institution of appropriate treatment, avoids possible damage to the urinary tract from childhood, allowing better management and quality of life in these patients.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"17 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121512401","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}
Cystinosis is a rare autosomal recessive lysosomal storage disorder affecting 1 in 100,000 – 200,000 live births. It is caused by a mutation in the Cystinosin (CTNS) gene, a cystine-proton cotransporter, the absence of which results in intra-lysosomal accumulation of cystine. Kidneys are affected first, presenting as Fanconi syndrome in infancy, followed by widespread involvement of the eyes, endocrine and neuromuscular system later in life. Cystinosis since being first described in 1903 to the discovery of CTNS gene defect a century later in 1998, has proven to be a complex disease. Clinical features are a manifestation of intra-lysosomal accumulation and interruption of cellular metabolic pathways in the affected organs. In this review, we explore the various pathophysiologic mechanisms underlying the manifestations of this complex disease.
{"title":"Cystinosis - Pathophysiology","authors":"Rairikar Mugdha, Hohenfellner Katharina, Elenberg Ewa","doi":"10.17352/acn.000064","DOIUrl":"https://doi.org/10.17352/acn.000064","url":null,"abstract":"Cystinosis is a rare autosomal recessive lysosomal storage disorder affecting 1 in 100,000 – 200,000 live births. It is caused by a mutation in the Cystinosin (CTNS) gene, a cystine-proton cotransporter, the absence of which results in intra-lysosomal accumulation of cystine. Kidneys are affected first, presenting as Fanconi syndrome in infancy, followed by widespread involvement of the eyes, endocrine and neuromuscular system later in life. Cystinosis since being first described in 1903 to the discovery of CTNS gene defect a century later in 1998, has proven to be a complex disease. Clinical features are a manifestation of intra-lysosomal accumulation and interruption of cellular metabolic pathways in the affected organs. In this review, we explore the various pathophysiologic mechanisms underlying the manifestations of this complex disease.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"176 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132930514","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}
Rates of kidney transplantation in patients over 70 years of age have steadily increased over the last 20 years, however age-appropriate immunosuppression regimens in the elderly remain unclear. Investigators utilized the SRTR database to evaluate elderly kidney transplant recipients’ outcomes against a younger population. Post-transplant outcomes measured at an approximately 1-year time interval included graft survival, patient survival, rejection, malignancy, and serum creatinine. Elderly patient survival was improved for those patients that were on dialysis for less than 1 year (95.4% vs. 91.4% p < .01). Patients able to be maintained on CNI immunosuppression regimens also had improved graft survival compared to those managed with other immunosuppression (95.5% vs. 91.1%, p < .01). Patients maintained on mTOR inhibitors had the lowest patient survival (85.5% vs. 92.6%, p < .01). The choice of induction therapy did not affect long term patient or graft survival. These results translated to investigators’ own centers in patients over 60. Results for the SRTR database showed that minimizing time on dialysis prior to transplant improved graft and patient survival, while the type of induction agent had minimal effect on all outcomes at the time of follow-up. The results also support the use of CNI’s and belatacept for maintenance immunosuppression but did not encourage the use of mTOR inhibitors.
在过去的20年里，70岁以上患者的肾移植率稳步上升，然而，适合年龄的老年人免疫抑制方案仍不清楚。研究人员利用SRTR数据库来评估老年肾移植受者与年轻人群的结果。在大约1年的时间间隔内测量移植后的结果包括移植物存活、患者存活、排斥反应、恶性肿瘤和血清肌酐。透析时间少于1年的老年患者生存率提高(95.4%比91.4% p < 0.01)。与其他免疫抑制方案相比，能够维持CNI免疫抑制方案的患者移植物存活率也有所提高(95.5%对91.1%，p < 0.01)。维持mTOR抑制剂治疗的患者生存率最低(85.5%比92.6%，p < 0.01)。诱导治疗的选择不影响患者或移植物的长期生存。这些结果转化为研究人员自己的60岁以上患者中心。SRTR数据库的结果显示，移植前透析时间的最小化改善了移植和患者的生存，而诱导剂的类型对随访时的所有结果影响最小。结果也支持使用CNI和belataccept维持免疫抑制，但不鼓励使用mTOR抑制剂。
{"title":"Analysis of induction and maintenance immunosuppression choices in the US during the first year post kidney transplant for patients over 70","authors":"White Amy H, Hunton John, Karim Saleema, Wells Allison, Jensen Hanna, Derringer Darby, Karr Misha, Kumaran Sathyanand, Burdine Lyle","doi":"10.17352/acn.000063","DOIUrl":"https://doi.org/10.17352/acn.000063","url":null,"abstract":"Rates of kidney transplantation in patients over 70 years of age have steadily increased over the last 20 years, however age-appropriate immunosuppression regimens in the elderly remain unclear. Investigators utilized the SRTR database to evaluate elderly kidney transplant recipients’ outcomes against a younger population. Post-transplant outcomes measured at an approximately 1-year time interval included graft survival, patient survival, rejection, malignancy, and serum creatinine. Elderly patient survival was improved for those patients that were on dialysis for less than 1 year (95.4% vs. 91.4% p < .01). Patients able to be maintained on CNI immunosuppression regimens also had improved graft survival compared to those managed with other immunosuppression (95.5% vs. 91.1%, p < .01). Patients maintained on mTOR inhibitors had the lowest patient survival (85.5% vs. 92.6%, p < .01). The choice of induction therapy did not affect long term patient or graft survival. These results translated to investigators’ own centers in patients over 60. Results for the SRTR database showed that minimizing time on dialysis prior to transplant improved graft and patient survival, while the type of induction agent had minimal effect on all outcomes at the time of follow-up. The results also support the use of CNI’s and belatacept for maintenance immunosuppression but did not encourage the use of mTOR inhibitors.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"16 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-02-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116956938","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}
Ablaikhanova Nt, Tusupbekova Ga, Beissova A, Ussipbek B, Mukhitdinov Am, Yessenbekova A, Balmaganbet Za, Ursheeva B, Atanbayeva Gk, Ye Zb
The current article examines the effectiveness of nanoenterosorbent to correct violations of the functional state of the kidneys in the experimental renal failure model. The obtained data open up opportunities for further research aimed at studying the possibility of using nanoenterosorbent in practical medicine as a new nanoenterosorbent and a means of drug delivery. The study was conducted in the following groups of animals: group 1 - control group; group 2 - an experimental model of acute renal failure; group 3 - nanoenterosorbent was administered intragastrically at a dose of 650 mg/kg per day to animals with acute renal failure. During the experiment for 3, 14 and 21 days, an analysis of biochemical parameters was obtained from each group. During the investigation, intragastric administration of the nanoenterosorbent did not reduce the dynamics of experimental uremia but reduced the concentration of level of molecular of average mass within 3 days, it also did not improve the functional state of the kidneys according to the readings of urea and creatinine for 14 days after the formation of renal failure, however, it statistically reduced endogenous intoxication according to EI data. Daily intragastric administration of nanoenterosorbent at a dose of 650 mg/kg after the formation of renal failure reduced uremia (urea, creatinine levels) and endogenous intoxication (level of molecular of average mass) after 21 days. Based on the studies, it was found that animals that received nanoenterosorbent at a daily dose of 650 mg/kg, show an optimal improvement in some biochemical parameters.
{"title":"Studying the efficiency of carbon nano enterosorbents in the model of experimental renal failure","authors":"Ablaikhanova Nt, Tusupbekova Ga, Beissova A, Ussipbek B, Mukhitdinov Am, Yessenbekova A, Balmaganbet Za, Ursheeva B, Atanbayeva Gk, Ye Zb","doi":"10.17352/acn.000062","DOIUrl":"https://doi.org/10.17352/acn.000062","url":null,"abstract":"The current article examines the effectiveness of nanoenterosorbent to correct violations of the functional state of the kidneys in the experimental renal failure model. The obtained data open up opportunities for further research aimed at studying the possibility of using nanoenterosorbent in practical medicine as a new nanoenterosorbent and a means of drug delivery. The study was conducted in the following groups of animals: group 1 - control group; group 2 - an experimental model of acute renal failure; group 3 - nanoenterosorbent was administered intragastrically at a dose of 650 mg/kg per day to animals with acute renal failure. During the experiment for 3, 14 and 21 days, an analysis of biochemical parameters was obtained from each group. During the investigation, intragastric administration of the nanoenterosorbent did not reduce the dynamics of experimental uremia but reduced the concentration of level of molecular of average mass within 3 days, it also did not improve the functional state of the kidneys according to the readings of urea and creatinine for 14 days after the formation of renal failure, however, it statistically reduced endogenous intoxication according to EI data. Daily intragastric administration of nanoenterosorbent at a dose of 650 mg/kg after the formation of renal failure reduced uremia (urea, creatinine levels) and endogenous intoxication (level of molecular of average mass) after 21 days. Based on the studies, it was found that animals that received nanoenterosorbent at a daily dose of 650 mg/kg, show an optimal improvement in some biochemical parameters.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"25 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126320470","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}
Objective: To investigate the occurrence and influencing factors of fatigue and sleep disturbance in Maintenance Hemodialysis (MHD) patients. Methods: A total of 170 patients with end-stage renal disease who underwent MHD treatment in the hemodialysis room of Shaanxi Provincial Hospital of Traditional Chinese Medicine from October 2021 to March 2022 were selected as the research subjects. The basic information and laboratory indicators of the patients were collected by cross-sectional survey. The survey methods were evaluated by the revised Piper Fatigue Scale and the Pittsburgh Sleep Quality Rating Scale and the incidence and influencing factors of fatigue and sleep disturbance in MHD patients were analyzed. Results: Fatigue occurred in 135 cases, the incidence rate was 79.41%; sleep disturbance occurred in 124 cases and the incidence rate was 72.94%. After one-way analysis of variance, factors such as exercise, Albumin (ALB, serum Creatinine(CRE), Phosphorus(P) and Hemoglobin(HGB) in MHD patients can affect fatigue; while age, gender, exercise, primary disease, dialysis frequency, Phosphorus(P), Hemoglobin(HGB) and high-sensitivity C-Reactive Protein (hs-CRP) factors can affect sleep. A multiple linear regression model was constructed for the factors affecting fatigue (F = 81.110, p < 0.001), and it showed that 70.3% of fatigue (adjusted R2 = 0.703) was related to albumin (ALB), serum creatinine(CRE), and hemoglobin(HGB) (all p < 0.05); A multiple linear regression model was constructed based on the factors of 58% (F = 26.933, p < 0.001), which showed that 58% of sleep disorders (adjusted R2 = 0.580) were significantly related to age, gender, exercise or not, phosphorus(P), high-sensitivity C-reactive protein(hs-CRP) (all p < 0.05) related. Pearson correlation analysis was used to analyze sleep disturbance and fatigue in MHD patients and the results showed that there was a positive correlation between the two (r = 0.478, p < 0.001). Conclusion: The proportion of fatigue and sleep disturbance in MHD patients is relatively high, mainly mild to moderate and the two influence each other. Exercise intervention and nutritional support can effectively improve the occurrence of fatigue and sleep disturbance in MHD patients.
目的:探讨维持性血液透析(MHD)患者疲劳、睡眠障碍的发生及影响因素。方法:选取2021年10月至2022年3月在陕西省中医院血液透析室接受MHD治疗的终末期肾病患者170例作为研究对象。采用横断面调查法收集患者基本信息和实验室指标。采用修订后的Piper疲劳量表和匹兹堡睡眠质量评定量表对调查方法进行评价，分析MHD患者疲劳和睡眠障碍的发生率及影响因素。结果:疲劳发生135例，发生率为79.41%;发生睡眠障碍124例，发生率为72.94%。经单因素方差分析，MHD患者的运动、白蛋白(ALB)、血清肌酐(CRE)、磷(P)、血红蛋白(HGB)等因素可影响疲劳;而年龄、性别、运动、原发疾病、透析频率、磷(P)、血红蛋白(HGB)、高敏c反应蛋白(hs-CRP)等因素均可影响睡眠。对影响疲劳的因素建立多元线性回归模型(F = 81.110, p < 0.001)，结果显示70.3%的疲劳与白蛋白(ALB)、血清肌酐(CRE)、血红蛋白(HGB)相关(调整后R2 = 0.703)(均p < 0.05);以58%的因子(F = 26.933, p < 0.001)构建多元线性回归模型，结果显示58%的睡眠障碍(校正R2 = 0.580)与年龄、性别、是否运动、磷(p)、高敏c反应蛋白(hs-CRP)相关(均p < 0.05)。对MHD患者的睡眠障碍与疲劳进行Pearson相关分析，结果显示两者呈正相关(r = 0.478, p < 0.001)。结论:MHD患者出现疲劳和睡眠障碍的比例较高，且以轻中度为主，两者相互影响。运动干预和营养支持能有效改善MHD患者疲劳和睡眠障碍的发生。
{"title":"The occurrence and influencing factors of fatigue and sleep disturbance in maintenance hemodialysis patients","authors":"Kaixuan Dong, Xi Chen, Rong Zhou, Xiaoyong Yu","doi":"10.17352/acn.000061","DOIUrl":"https://doi.org/10.17352/acn.000061","url":null,"abstract":"Objective: To investigate the occurrence and influencing factors of fatigue and sleep disturbance in Maintenance Hemodialysis (MHD) patients. Methods: A total of 170 patients with end-stage renal disease who underwent MHD treatment in the hemodialysis room of Shaanxi Provincial Hospital of Traditional Chinese Medicine from October 2021 to March 2022 were selected as the research subjects. The basic information and laboratory indicators of the patients were collected by cross-sectional survey. The survey methods were evaluated by the revised Piper Fatigue Scale and the Pittsburgh Sleep Quality Rating Scale and the incidence and influencing factors of fatigue and sleep disturbance in MHD patients were analyzed. Results: Fatigue occurred in 135 cases, the incidence rate was 79.41%; sleep disturbance occurred in 124 cases and the incidence rate was 72.94%. After one-way analysis of variance, factors such as exercise, Albumin (ALB, serum Creatinine(CRE), Phosphorus(P) and Hemoglobin(HGB) in MHD patients can affect fatigue; while age, gender, exercise, primary disease, dialysis frequency, Phosphorus(P), Hemoglobin(HGB) and high-sensitivity C-Reactive Protein (hs-CRP) factors can affect sleep. A multiple linear regression model was constructed for the factors affecting fatigue (F = 81.110, p < 0.001), and it showed that 70.3% of fatigue (adjusted R2 = 0.703) was related to albumin (ALB), serum creatinine(CRE), and hemoglobin(HGB) (all p < 0.05); A multiple linear regression model was constructed based on the factors of 58% (F = 26.933, p < 0.001), which showed that 58% of sleep disorders (adjusted R2 = 0.580) were significantly related to age, gender, exercise or not, phosphorus(P), high-sensitivity C-reactive protein(hs-CRP) (all p < 0.05) related. Pearson correlation analysis was used to analyze sleep disturbance and fatigue in MHD patients and the results showed that there was a positive correlation between the two (r = 0.478, p < 0.001). Conclusion: The proportion of fatigue and sleep disturbance in MHD patients is relatively high, mainly mild to moderate and the two influence each other. Exercise intervention and nutritional support can effectively improve the occurrence of fatigue and sleep disturbance in MHD patients.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"89 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131530647","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}
Ozer Hakan, Ozturk Yasin, Baloglu Ismail, Turkmen Kultigin
Hyponatremia is the most common electrolyte disorder in clinical practice: Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) is the most common cause of hyponatremia, especially in euvolemic patients. Drugs are among the most common causes of SIADH. While our patient was using hydroxyurea due to polycythemia vera, he was diagnosed with SIADH due to hydroxyurea treatment after investigation due to resistant hyponatremia. The improvement of hyponatremia after hydroxyurea is discontinued and the development of hyponatremia after the drug is re-started supports the development of drug-induced hyponatremia. Careful follow-up is required in terms of hyponatremia in patients using hydroxyurea.
{"title":"Syndrome of Inappropriate Antidiuretic Hormone secretion due to hydroxyurea","authors":"Ozer Hakan, Ozturk Yasin, Baloglu Ismail, Turkmen Kultigin","doi":"10.17352/acn.000060","DOIUrl":"https://doi.org/10.17352/acn.000060","url":null,"abstract":"Hyponatremia is the most common electrolyte disorder in clinical practice: Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) is the most common cause of hyponatremia, especially in euvolemic patients. Drugs are among the most common causes of SIADH. While our patient was using hydroxyurea due to polycythemia vera, he was diagnosed with SIADH due to hydroxyurea treatment after investigation due to resistant hyponatremia. The improvement of hyponatremia after hydroxyurea is discontinued and the development of hyponatremia after the drug is re-started supports the development of drug-induced hyponatremia. Careful follow-up is required in terms of hyponatremia in patients using hydroxyurea.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"118 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134325255","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}
Cellular interorganelle crosstalk in medical sciences is a new discussion of mechanisms and pathways of physiological functions and pathogenesis of diseases. The organelles (“mitochondria”, nucleus, lysosome, endoplasmic reticulum, Golgi apparatus) are members of such functional units that are needed to perform specific tasks. Mitochondria are essential metabolic organelles in cells, but they also contribute to iron and calcium homeostasis, as well as in the regulation of apoptosis, and they are increasingly recognized as key signaling platforms. In the kidney, crosstalk between mitochondria with endoplasmic reticulum is at mitochondria-associated endoplasmic reticulum membrane in regulating calcium homeostasis. Another crosstalk between these organelles is about autophagic mechanisms. Autophagy is triggered in the kidney in response to acute kidney injury and supports against kidney injury. High glucose-induced reactive oxygen species can be produced by both enzymatic and nonenzymatic pathways. The nucleotide-binding domain and leucine-rich repeat (NLR) family or nucleotide-binding and oligomerization domain (NOD)-like receptors family pyrin domain containing 3 (NLRP3) inflammasome plays a role in inducing infectious defenses via inflammatory cytokines. The NLRP3 inflammasome is activated by the mitochondria-associated endoplasmic reticulum membrane. It has a role in nephrocalcinosis-related chronic kidney disease. This review article is a summary of interorganelle crosstalk in health and disease states, especially in kidney and nephrology-related conditions.
{"title":"Cellular interorganelle crosstalk in health and disease states: A glimpse on nephrology-related conditions","authors":"Amiri Fateme Shamekhi","doi":"10.17352/acn.000059","DOIUrl":"https://doi.org/10.17352/acn.000059","url":null,"abstract":"Cellular interorganelle crosstalk in medical sciences is a new discussion of mechanisms and pathways of physiological functions and pathogenesis of diseases. The organelles (“mitochondria”, nucleus, lysosome, endoplasmic reticulum, Golgi apparatus) are members of such functional units that are needed to perform specific tasks. Mitochondria are essential metabolic organelles in cells, but they also contribute to iron and calcium homeostasis, as well as in the regulation of apoptosis, and they are increasingly recognized as key signaling platforms. In the kidney, crosstalk between mitochondria with endoplasmic reticulum is at mitochondria-associated endoplasmic reticulum membrane in regulating calcium homeostasis. Another crosstalk between these organelles is about autophagic mechanisms. Autophagy is triggered in the kidney in response to acute kidney injury and supports against kidney injury. High glucose-induced reactive oxygen species can be produced by both enzymatic and nonenzymatic pathways. The nucleotide-binding domain and leucine-rich repeat (NLR) family or nucleotide-binding and oligomerization domain (NOD)-like receptors family pyrin domain containing 3 (NLRP3) inflammasome plays a role in inducing infectious defenses via inflammatory cytokines. The NLRP3 inflammasome is activated by the mitochondria-associated endoplasmic reticulum membrane. It has a role in nephrocalcinosis-related chronic kidney disease. This review article is a summary of interorganelle crosstalk in health and disease states, especially in kidney and nephrology-related conditions.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"45 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126324038","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}
R. Scarpioni, S. De Amicis, Bodini FC Bodini, V. Albertazzi, E. Michieletti
Renal Infarction (RI), a rare cause of renal damage characterized by the abrupt interruption of flow in the renal artery, is often recognized late or may even remain undiagnosed since symptoms are non-specific and may be confused with other pathologies, such as pyelonephritis or nephrolithiasis. In situ thrombosis and thromboembolism are the main causes, but often the real cause is, gf unrecognized. The disease is often underdiagnosed and the diagnosis of certainty can be established with ultrasonography Doppler of renal arteries or with second-level diagnostic tools (contrast-enhanced computer tomography, magnetic resonance with gadolinium, and renal scintigraphy) or third level tests (renal arteriography). The therapeutic approach depends on the cause of RI, from the time from onset of ischemia, from the presence of kidney function impairment, and may include systemic anticoagulant therapy, renal angioplasty with or without stenting, loco-regional endovascular fibrinolytic therapy or surgery, as the last chance. In literature, there are neither guidelines nor evidence about any treatment superiority. Here we describe a paradigmatic case in a 51-years-old man hospitalized because of sudden flank pain: the clinical picture, the high serum level. Moreover, we report our 7-years’ experience with 24 cases of RI, mean age 70 /±15 years, 14/24 men, 16/24 presented with hematuria, frequently associated with the history of CKD (16/24). Fifteen of them (62%) were classified as idiopathic and 9 of them were successfully treated with endovascular fibrinolytic treatment. A review of the literature is also provided.
{"title":"Acute kidney infarction: Not so rare renal disease. A single-center experience with endovascular fibrinolytic therapy","authors":"R. Scarpioni, S. De Amicis, Bodini FC Bodini, V. Albertazzi, E. Michieletti","doi":"10.17352/acn.000058","DOIUrl":"https://doi.org/10.17352/acn.000058","url":null,"abstract":"Renal Infarction (RI), a rare cause of renal damage characterized by the abrupt interruption of flow in the renal artery, is often recognized late or may even remain undiagnosed since symptoms are non-specific and may be confused with other pathologies, such as pyelonephritis or nephrolithiasis. In situ thrombosis and thromboembolism are the main causes, but often the real cause is, gf unrecognized. The disease is often underdiagnosed and the diagnosis of certainty can be established with ultrasonography Doppler of renal arteries or with second-level diagnostic tools (contrast-enhanced computer tomography, magnetic resonance with gadolinium, and renal scintigraphy) or third level tests (renal arteriography). The therapeutic approach depends on the cause of RI, from the time from onset of ischemia, from the presence of kidney function impairment, and may include systemic anticoagulant therapy, renal angioplasty with or without stenting, loco-regional endovascular fibrinolytic therapy or surgery, as the last chance. In literature, there are neither guidelines nor evidence about any treatment superiority. Here we describe a paradigmatic case in a 51-years-old man hospitalized because of sudden flank pain: the clinical picture, the high serum level. Moreover, we report our 7-years’ experience with 24 cases of RI, mean age 70 /±15 years, 14/24 men, 16/24 presented with hematuria, frequently associated with the history of CKD (16/24). Fifteen of them (62%) were classified as idiopathic and 9 of them were successfully treated with endovascular fibrinolytic treatment. A review of the literature is also provided.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"7 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124608891","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}
Abbasi Muhammad Tanzeel, Arif Mariam, Saleem Nayyar
Diabetes Mellitus (DM) is a potent risk factor for post-transplant cardiovascular complications and infections. Management of diabetes and its complications in renal transplant recipients is a challenging task. This is a frequently encountered predicament in transplant setups. An erratic glycemic control during dialysis is a predictor of poor graft and patient outcomes after kidney transplantation. Literature review reveals majority studies explaining post-transplant diabetes and its role in graft and patient survival. However, a wide range of opinion exists about the impact of pre-transplant DM on transplant outcomes. Measurement of HbA1c levels is a significant tool for assessment of glycemic control. A target HbA1c level of <7% is recommended for diabetic patients irrespective of presence or absence of Chronic Kidney Disease (CKD). However, diabetic patients with CKD are at risk of hypoglycemia owing to decreased insulin metabolism so it is safe to keep HbA1c levels between 7-8% in this population. Immunosuppressive medications have a strong contributory role in deterioration of glycemic control. So, it is imperative to achieve strict pre-transplant diabetes control in order to avoid post-transplant complications. Post-transplant diabetes mellitus (PTDM) has been a subject of a large number of trials and is not only considered a serious metabolic complication but also a predisposing factor of diabetic nephropathy in transplanted kidney.
{"title":"Impact of Diabetes Mellitus on Renal transplant outcome","authors":"Abbasi Muhammad Tanzeel, Arif Mariam, Saleem Nayyar","doi":"10.17352/acn.000057","DOIUrl":"https://doi.org/10.17352/acn.000057","url":null,"abstract":"Diabetes Mellitus (DM) is a potent risk factor for post-transplant cardiovascular complications and infections. Management of diabetes and its complications in renal transplant recipients is a challenging task. This is a frequently encountered predicament in transplant setups. An erratic glycemic control during dialysis is a predictor of poor graft and patient outcomes after kidney transplantation. Literature review reveals majority studies explaining post-transplant diabetes and its role in graft and patient survival. However, a wide range of opinion exists about the impact of pre-transplant DM on transplant outcomes. Measurement of HbA1c levels is a significant tool for assessment of glycemic control. A target HbA1c level of <7% is recommended for diabetic patients irrespective of presence or absence of Chronic Kidney Disease (CKD). However, diabetic patients with CKD are at risk of hypoglycemia owing to decreased insulin metabolism so it is safe to keep HbA1c levels between 7-8% in this population. Immunosuppressive medications have a strong contributory role in deterioration of glycemic control. So, it is imperative to achieve strict pre-transplant diabetes control in order to avoid post-transplant complications. Post-transplant diabetes mellitus (PTDM) has been a subject of a large number of trials and is not only considered a serious metabolic complication but also a predisposing factor of diabetic nephropathy in transplanted kidney.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"36 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127757974","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}
S. Favre, M. Sauvezie, S. Vigouroux, R. Tabrizi, M. Dilhuydy, G. Labouré, M. Robles, N. Milpied, K. Bouabdallah
Background: Following shortage of Carmustine, BeEAM regimen (Bendamustine, Etoposide, Cytarabine and Melphalan) was used before autologous transplant in relapsed/refractory lymphoma patients. We evaluated safety and efficacy of BeEAM compared to BEAM. Patients and methods: Ninety consecutive patients receiving BeEAM (30pts) (Bendamustine 100, 120 or 200 mg/m²/d) or BEAM (60 pts) were retrospectively analyzed. Results: In the BEAM group, 68% had NHL and 32% HL compared to 87% and 13% in the BeEAM group (p = 0,014). Pts were in CR or PR at time of transplant. There was no difference regarding hematologic recovery and transfusion requirements. Highest dose of Bendamustine were associated with grade ≥ 2 kidney toxicity. We observed a significant higher incidence of symptomatic HHV-6 infection (53.3% versus 8.3%), digestive toxicity (36.6% versus 15%) and prolonged hospitalization (25 versus 21 days) with BeEAM. After a median follow up of 61 and 49 months for BEAM and BeEAM, 5y-OS and PFS (76% versus 67% and 56% versus 70%) and TRM (0% versus 3%) were not different. Conclusions: BeEAM with the highest doses of Bendamustine was associated with increased risk of HHV-6 infection, longer duration of hospitalization, higher rate of digestive toxicity and increased acute kidney failure while survival was comparable.
{"title":"High incidence rate of human herpesvirus 6 infection after Bendamustine, Cytarabine, Etoposide and Melphalan conditioning regimen: A monocentric and retrospective study","authors":"S. Favre, M. Sauvezie, S. Vigouroux, R. Tabrizi, M. Dilhuydy, G. Labouré, M. Robles, N. Milpied, K. Bouabdallah","doi":"10.17352/acn.000054","DOIUrl":"https://doi.org/10.17352/acn.000054","url":null,"abstract":"Background: Following shortage of Carmustine, BeEAM regimen (Bendamustine, Etoposide, Cytarabine and Melphalan) was used before autologous transplant in relapsed/refractory lymphoma patients. We evaluated safety and efficacy of BeEAM compared to BEAM. \u0000Patients and methods: Ninety consecutive patients receiving BeEAM (30pts) (Bendamustine 100, 120 or 200 mg/m²/d) or BEAM (60 pts) were retrospectively analyzed. \u0000Results: In the BEAM group, 68% had NHL and 32% HL compared to 87% and 13% in the BeEAM group (p = 0,014). Pts were in CR or PR at time of transplant. There was no difference regarding hematologic recovery and transfusion requirements. Highest dose of Bendamustine were associated with grade ≥ 2 kidney toxicity. We observed a significant higher incidence of symptomatic HHV-6 infection (53.3% versus 8.3%), digestive toxicity (36.6% versus 15%) and prolonged hospitalization (25 versus 21 days) with BeEAM. After a median follow up of 61 and 49 months for BEAM and BeEAM, 5y-OS and PFS (76% versus 67% and 56% versus 70%) and TRM (0% versus 3%) were not different. \u0000Conclusions: BeEAM with the highest doses of Bendamustine was associated with increased risk of HHV-6 infection, longer duration of hospitalization, higher rate of digestive toxicity and increased acute kidney failure while survival was comparable.","PeriodicalId":127781,"journal":{"name":"Archives of Clinical Nephrology","volume":"246 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115205943","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}
京公网安备 11010802042870号
京ICP备2023020795号-1
扫码关注我们
求助内容：
应助结果提醒方式：