Pub Date : 2023-07-27DOI: 10.33590/emjnephrol/10308654
J. George
IgA nephropathy (IgAN) is a rare, life-limiting disease for which there is significant unmet need. Until recently, drug development for IgAN had been impeded by the requirement for large-scale, long-term clinical trials to measure clinical outcomes. However, the recent establishment of ‘reduction in proteinuria’ as a surrogate endpoint to predict clinical outcomes in IgAN, as a basis for accelerated drug approval, has transformed the field. At the 60th European Renal Association (ERA) Congress in June 2023, two oral poster presentations focused on the use of early reduction in proteinuria as an endpoint for clinical studies in IgAN. Alex Mercer, Consultant in Clinical Drug Development at JAMCO Pharma Consulting in Stockholm, Sweden, presented data estimating the long-term clinical outcome of reductions in proteinuria (clinically meaningful extensions in time to kidney failure or death), which could help predict the future protective effect of any new intervention on kidney function. Following this, Jonathan Barratt, Mayer Professor of Renal Medicine at the University of Leicester, and Honorary Consultant Nephrologist at Leicester General Hospital, UK, described findings from the prespecified interim analysis of the Phase III PROTECT study of sparsentan (a novel dual endothelin angiotensin receptor antagonist) in IgAN, which included reduction in proteinuria as a primary endpoint. In patients with IgAN at high risk of disease progression, sparsentan produced a rapid and significant reduction in proteinuria of a level that, according to the study data presented by Mercer, would correspond to a substantially lowered risk of kidney failure or death. Long-term data to confirm this predicted clinical outcome on disease progression are anticipated.
{"title":"Proteinuria as a Surrogate Endpoint for Disease Progression in IgA Nephropathy: Predicting Long-Term Treatment Effects of Sparsentan","authors":"J. George","doi":"10.33590/emjnephrol/10308654","DOIUrl":"https://doi.org/10.33590/emjnephrol/10308654","url":null,"abstract":"IgA nephropathy (IgAN) is a rare, life-limiting disease for which there is significant unmet need. Until recently, drug development for IgAN had been impeded by the requirement for large-scale, long-term clinical trials to measure clinical outcomes. However, the recent establishment of ‘reduction in proteinuria’ as a surrogate endpoint to predict clinical outcomes in IgAN, as a basis for accelerated drug approval, has transformed the field. At the 60th European Renal Association (ERA) Congress in June 2023, two oral poster presentations focused on the use of early reduction in proteinuria as an endpoint for clinical studies in IgAN. Alex Mercer, Consultant in Clinical Drug Development at JAMCO Pharma Consulting in Stockholm, Sweden, presented data estimating the long-term clinical outcome of reductions in proteinuria (clinically meaningful extensions in time to kidney failure or death), which could help predict the future protective effect of any new intervention on kidney function. Following this, Jonathan Barratt, Mayer Professor of Renal Medicine at the University of Leicester, and Honorary Consultant Nephrologist at Leicester General Hospital, UK, described findings from the prespecified interim analysis of the Phase III PROTECT study of sparsentan (a novel dual endothelin angiotensin receptor antagonist) in IgAN, which included reduction in proteinuria as a primary endpoint. In patients with IgAN at high risk of disease progression, sparsentan produced a rapid and significant reduction in proteinuria of a level that, according to the study data presented by Mercer, would correspond to a substantially lowered risk of kidney failure or death. Long-term data to confirm this predicted clinical outcome on disease progression are anticipated.","PeriodicalId":348431,"journal":{"name":"EMJ Nephrology","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122330973","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 : 2023-07-27DOI: 10.33590/emjnephrol/10303666
M. Salvadori
Several diseases are related to complement involvement. In particular, its role is essential for the pathogenesis of several renal disease. On the other hand, the complement role may also be protective, and this possibility should be well known when managing complement inhibitors. Complement inhibitors are relatively newly discovered therapies that are essential in some diseases, and in others improve the efficacy of the already known therapeutic measures that represent the standard of care. In the case of glomerular diseases, complement plays a role in almost all diseases. In some diseases, complement abnormalities represent the prevailing factor in the pathogenesis. In such diseases, complement inhibition represents an essential therapy. In other diseases, complement plays an important role, but other factors are involved in the pathogenesis. Clearly, in these diseases, complement inhibition represents a therapy that could be added to the standard of care therapy, according to the physician’s judgement. Examples of these diseases are lupus nephritis, thrombotic microangiopathy when associated to some cases of lupus nephritis, and IgA nephropathy. The latter is one of the first primary glomerulonephritides in which a relevant role of complement is documented. These three diseases are the object of this brief review. Particular concern should be given to ongoing clinical trials. Indeed, many of these anti-complement therapies are still in different phases of clinical trials. Finally, particular concern must be ascribed to the problem of associating these emerging therapies to already existing and proven treatments.
{"title":"Recent Advances in Targeting Complement in Glomerular Disease","authors":"M. Salvadori","doi":"10.33590/emjnephrol/10303666","DOIUrl":"https://doi.org/10.33590/emjnephrol/10303666","url":null,"abstract":"Several diseases are related to complement involvement. In particular, its role is essential for the pathogenesis of several renal disease. On the other hand, the complement role may also be protective, and this possibility should be well known when managing complement inhibitors.\u0000\u0000Complement inhibitors are relatively newly discovered therapies that are essential in some diseases, and in others improve the efficacy of the already known therapeutic measures that represent the standard of care.\u0000\u0000In the case of glomerular diseases, complement plays a role in almost all diseases. In some diseases, complement abnormalities represent the prevailing factor in the pathogenesis. In such diseases, complement inhibition represents an essential therapy. In other diseases, complement plays an important role, but other factors are involved in the pathogenesis. Clearly, in these diseases, complement inhibition represents a therapy that could be added to the standard of care therapy, according to the physician’s judgement. Examples of these diseases are lupus nephritis, thrombotic microangiopathy when associated to some cases of lupus nephritis, and IgA nephropathy. The latter is one of the first primary glomerulonephritides in which a relevant role of complement is documented. These three diseases are the object of this brief review. Particular concern should be given to ongoing clinical trials. Indeed, many of these anti-complement therapies are still in different phases of clinical trials. Finally, particular concern must be ascribed to the problem of associating these emerging therapies to already existing and proven treatments.","PeriodicalId":348431,"journal":{"name":"EMJ Nephrology","volume":"57 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130340842","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 : 2023-07-27DOI: 10.33590/emjnephrol/10302800
E. Koutsouki
ACUTE kidney injury (AKI) was discussed in a session chaired by Jolanta Malyszko, Medical University of Warsaw, Poland, and Danilo Fliser, Saarland University Medical Centre, Homburg, Germany, at the 60th European Renal Association (ERA) Congress, which took place in Milan, Italy, between 15th–18th June 2023. The moderators started by announcing that ERA has created a new working group on AKI.
{"title":"Contemporary Acute Kidney Injury Management","authors":"E. Koutsouki","doi":"10.33590/emjnephrol/10302800","DOIUrl":"https://doi.org/10.33590/emjnephrol/10302800","url":null,"abstract":"ACUTE kidney injury (AKI) was discussed in a session chaired by Jolanta Malyszko, Medical University of Warsaw, Poland, and Danilo Fliser, Saarland University Medical Centre, Homburg, Germany, at the 60th European Renal Association (ERA) Congress, which took place in Milan, Italy, between 15th–18th June 2023. The moderators started by announcing that ERA has created a new working group on AKI.","PeriodicalId":348431,"journal":{"name":"EMJ Nephrology","volume":"11 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132429723","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 : 2023-07-23DOI: 10.33590/emjhepatol/10307663
Keiya Aono, Takahiro Suzuki, Masaoki Hattori, M. Yoshihara
Splenosis should be suspected when a patient has a history of trauma or abdominal surgery. Intrahepatic splenosis is a rare disease that is often difficult to distinguish from liver malignancy, especially hepatocellular carcinoma. The cause of intrahepatic splenosis may be though the auto-transplantation of splenic tissue on the surface of the liver. The authors report a case of intrahepatic splenosis that presented as a liver tumour in an 81-year-old female treated for autoimmune hepatitis, who had no history of splenectomy or abdominal trauma. Laparoscopic hepatectomy was performed and the specimen demonstrated characteristic histopathological findings of the spleen. Only one case of a patient who had no history of splenectomy or abdominal trauma has been reported in the literature. It may be hypothesised that erythropoiesis induced by local hypoxia in the chronic hepatitis may cause the growth of splenic erythrocytic progenitor cells, which have migrated via portal vein to the liver.
{"title":"Intrahepatic Splenosis in a Patient with Autoimmune Hepatitis with No History of Splenectomy or Abdominal Trauma","authors":"Keiya Aono, Takahiro Suzuki, Masaoki Hattori, M. Yoshihara","doi":"10.33590/emjhepatol/10307663","DOIUrl":"https://doi.org/10.33590/emjhepatol/10307663","url":null,"abstract":"Splenosis should be suspected when a patient has a history of trauma or abdominal surgery. Intrahepatic splenosis is a rare disease that is often difficult to distinguish from liver malignancy, especially hepatocellular carcinoma. The cause of intrahepatic splenosis may be though the auto-transplantation of splenic tissue on the surface of the liver. The authors report a case of intrahepatic splenosis that presented as a liver tumour in an 81-year-old female treated for autoimmune hepatitis, who had no history of splenectomy or abdominal trauma. Laparoscopic hepatectomy was performed and the specimen demonstrated characteristic histopathological findings of the spleen. Only one case of a patient who had no history of splenectomy or abdominal trauma has been reported in the literature. It may be hypothesised that erythropoiesis induced by local hypoxia in the chronic hepatitis may cause the growth of splenic erythrocytic progenitor cells, which have migrated via portal vein to the liver.","PeriodicalId":348431,"journal":{"name":"EMJ Nephrology","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129481974","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 : 2023-07-19DOI: 10.33590/emjnephrol/10301955
Uzma Zahid, M. Mahmood
Nephrotic syndrome is a frequently encountered disease in children. It is mostly responsive to high-dose steroids, with some requiring steroid-sparing immunosuppressive regimens, or further, a renal biopsy if resistant to steroid therapy. However, nephrotic syndrome in children post-allogeneic bone marrow transplant is rarely encountered. The authors report here a child who developed nephrotic syndrome post-allogeneic bone marrow transplant for β-thalassaemia major, with the suspicion of graft-versus-host disease that was difficult-to-treat, who had frequent relapses with multiple hospital admissions, and prolonged treatment course. For the last 5 years, the disease has been in remission, on a low dose of prednisolone and mycophenolate mofetil-based maintenance immunosuppressive treatment.
{"title":"Steroid-Dependent Nephrotic Syndrome in a Child After an Allogeneic Bone Marrow Transplant: A Case Report","authors":"Uzma Zahid, M. Mahmood","doi":"10.33590/emjnephrol/10301955","DOIUrl":"https://doi.org/10.33590/emjnephrol/10301955","url":null,"abstract":"Nephrotic syndrome is a frequently encountered disease in children. It is mostly responsive to high-dose steroids, with some requiring steroid-sparing immunosuppressive regimens, or further, a renal biopsy if resistant to steroid therapy. However, nephrotic syndrome in children post-allogeneic bone marrow transplant is rarely encountered. The authors report here a child who developed nephrotic syndrome post-allogeneic bone marrow transplant for β-thalassaemia major, with the suspicion of graft-versus-host disease that was difficult-to-treat, who had frequent relapses with multiple hospital admissions, and prolonged treatment course. For the last 5 years, the disease has been in remission, on a low dose of prednisolone and mycophenolate mofetil-based maintenance immunosuppressive treatment.","PeriodicalId":348431,"journal":{"name":"EMJ Nephrology","volume":"636 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117095047","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 : 2023-06-14DOI: 10.33590/emjnephrol/10305420
J. George
Anaemia is a common and serious complication of chronic kidney disease (CKD) that can greatly impact the daily lives of patients. However, poor awareness around anaemia of CKD (aCKD), from both physicians and patients, may impede its identification and treatment. During interviews conducted by EMJ in April 2023, leading nephrologist Christoph Wanner, University Hospital of Würzburg, Germany, and two patients/patient advocates, Daniel Gallego and Jemma Reast, gave their informed opinions on this topic. From their different viewpoints, they described how greater understanding of symptoms and treatment options could empower patients to make better choices for their own care. At the same time, they considered how greater physician awareness of aCKD, and the human impact beyond haemoglobin levels could influence diagnosis and treatment priorities. Aligning these two perspectives, they also discussed the powerful benefits of improved communication and shared decision-making between patient and physician, and its potential for relieving the burden of aCKD.
{"title":"Anaemia of Chronic Kidney Disease: Aligning Patient and Physician Awareness – Interviews with Three Key Opinion Leaders and Patient Advocates","authors":"J. George","doi":"10.33590/emjnephrol/10305420","DOIUrl":"https://doi.org/10.33590/emjnephrol/10305420","url":null,"abstract":"Anaemia is a common and serious complication of chronic kidney disease (CKD) that can greatly impact the daily lives of patients. However, poor awareness around anaemia of CKD (aCKD), from both physicians and patients, may impede its identification and treatment. During interviews conducted by EMJ in April 2023, leading nephrologist Christoph Wanner, University Hospital of Würzburg, Germany, and two patients/patient advocates, Daniel Gallego and Jemma Reast, gave their informed opinions on this topic. From their different viewpoints, they described how greater understanding of symptoms and treatment options could empower patients to make better choices for their own care. At the same time, they considered how greater physician awareness of aCKD, and the human impact beyond haemoglobin levels could influence diagnosis and treatment priorities. Aligning these two perspectives, they also discussed the powerful benefits of improved communication and shared decision-making between patient and physician, and its potential for relieving the burden of aCKD.","PeriodicalId":348431,"journal":{"name":"EMJ Nephrology","volume":"62 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116696289","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 : 2023-05-18DOI: 10.33590/emjnephrol/10308560
Flavis
{"title":"The Importance of Medical Nutrition Therapy in Chronic Kidney Disease Management","authors":"Flavis","doi":"10.33590/emjnephrol/10308560","DOIUrl":"https://doi.org/10.33590/emjnephrol/10308560","url":null,"abstract":"","PeriodicalId":348431,"journal":{"name":"EMJ Nephrology","volume":"59 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132456362","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 : 2023-05-17DOI: 10.33590/emjnephrol/10308389
Matthew Sparks
{"title":"Interview: Matthew A. Sparks","authors":"Matthew Sparks","doi":"10.33590/emjnephrol/10308389","DOIUrl":"https://doi.org/10.33590/emjnephrol/10308389","url":null,"abstract":"","PeriodicalId":348431,"journal":{"name":"EMJ Nephrology","volume":"77 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-05-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134381224","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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