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Kidney glomerular filtration rate plasticity after transplantation
Aleksandar Denic, Andrew D Rule, François Gaillard
doi : 10.1093/ckj/sfab267
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 841–844
Since the first living donor kidney transplantation about six decades ago, significant progress has been made in terms of extending allograft survival. However, to date, only a small number of studies have compared the functional changes of the donated kidney to that of the remaining kidney. Although relatively small, the study by Gonzalez Rinne et al. demonstrated the adaptive capacity of the transplanted kidney in 30 donor–recipient pairs. The glomerular filtration rate (GFR) in both donors and recipients was obtained 12 months after transplantation and the authors identified three scenarios: (i) where donors had a higher GFR than recipients; (ii) where donors had a lower GFR than recipients; and (iii) where donors had a similar GFR to recipients. The mechanisms mediating GFR adaptability after kidney transplantation seem to be associated with body surface area (including sex differences in body surface area). Microstructural analysis of human and animal models of renal physiology provides some clues to the physiological adaptation of the transplanted organ. The nephron number from endowment and age-related loss and the adaptive ability for compensatory glomerular hyperfiltration likely play a major role.
Blood pressure targets in CKD 2021: the never-ending guidelines debacle
Sol Carriazo, Pantelis Sarafidis, Charles J Ferro, Alberto Ortiz
doi : 10.1093/ckj/sfac014
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 845–851
In 2021, two updated clinical guidelines were published, providing guidance on blood pressure (BP) targets for people with chronic kidney disease (CKD). Kidney Disease: Improving Global Outcomes (KDIGO) updated its 2012 Clinical Practice Guideline for the Management of BP in CKD. Different systolic blood pressure (SBP) and diastolic blood pressure (DBP) targets for CKD (<130/80 and <140/90 mmHg, respectively, for people with a urinary albumin: creatinine ratio >30 mg/g or without pathological albuminuria) were replaced by a single number: an SBP target of <120 mmHg is suggested, when tolerated. This represents a major decrease in the SBP target and the abandonment of DBP targets. The European Society of Cardiology (ESC) also published a 2021 Clinical Guideline on Cardiovascular Disease Prevention in Clinical Practice that updates a prior 2016 guideline on prevention and the 2018 ESC/European Society of Hypertension Clinical Practice Guidelines for the Management of Arterial Hypertension. The 2021 ESC guideline was endorsed by 12 European scientific societies. The recommended office BP targets for people with CKD are <140–130 mmHg SBP (lower SBP is acceptable if tolerated) and <80 mmHg DBP. The question is: What should the practicing physician do now: treat hypertension in people with CKD to an SBP target of <120 mmHg or to a target of <140–130 mmHg? Major guideline bodies are aware of the activities of other major players. There is an urgent need for guideline bodies to establish communication channels, search consensus on major issues that impact the health of hundreds of millions of people worldwide and end individualism in guidelines generation.
Should we continue to use renin–angiotensin–aldosterone system blockers in patients with COVID-19?
Sidar Copur, Asiye Kanbay, Mehmet Kanbay
doi : 10.1093/ckj/sfac001
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 852–854
Patients with chronic kidney disease, chronic heart failure and hypertension have an increased risk of coronavirus disease 2019 (COVID-19)-related death. Renin–angiotensin–aldosterone system (RAS) blockers are commonly prescribed to decrease morbidity and mortality in these conditions. Following the pre-clinical demonstration of COVID-19 viral entry into cells via angiotensin-converting enzyme-2, the use of RAS blockers was questioned in infected individuals. Theodorakopoulou et al. extensively review the pathophysiology behind that hypothesis and observational or clinical trials on RAS blockers and COVID-19. Despite being a scientific hot spot of an ongoing debate, discontinuation of RAS blockers is not associated with improved clinical outcomes in COVID-19 and may have potential harmful effects, including exacerbation of the underlying disease.
Cutaneous manifestations of acute kidney injury
Gavin A Esson, Amaani B Hussain, Simon J Meggitt, Nick J Reynolds, John A Sayer
doi : 10.1093/ckj/sfab255
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 855–864
Acute kidney injury (AKI) is a common medical problem with a multitude of aetiologies. Prompt diagnosis and management is key in the prevention of complications. Cutaneous signs can often give diagnostic clues of underlying systemic diseases causing AKI. This review summarizes cutaneous findings of diseases causing AKI in adults. Knowledge of such cutaneous signs could lead to earlier diagnosis of underlying kidney disease and facilitate management strategies in a timely manner. Acute interstitial nephritis, polyarteritis nodosa, Kawasaki's disease, granulomatosis with polyangiitis (previously Wegener's granulomatosis), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (previously Churg–Strauss syndrome), Henoch–Schönlein purpura, cryoglobulinaemia, Sjögren's syndrome, systemic sclerosis, nephrogenic systemic fibrosis, dermatomyositis, systemic lupus erythematosus, amyloidosis and cholesterol embolization syndrome were highlighted as diseases causing AKI with cutaneous manifestations.
The unmet need of evidence-based therapy for patients with advanced chronic kidney disease and heart failure: Position paper from the Cardiorenal Working Groups of the Spanish Society of Nephrology and the Spanish Society of Cardiology
Alberto Ortiz, Juan F Navarro-González, Julio Núñez, Rafael de la Espriella, Marta Cobo, Rafael SantamarÃa, Patricia de Sequera, Javier DÃez
doi : 10.1093/ckj/sfab290
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 865–872
Despite the high prevalence of chronic kidney disease (CKD) and its high cardiovascular risk, patients with CKD, especially those with advanced CKD (stages 4–5 and patients on kidney replacement therapy), are excluded from most cardiovascular clinical trials. It is particularly relevant in patients with advanced CKD and heart failure (HF) who have been underrepresented in many pivotal randomized trials that have modified the management of HF. For this reason, there is little or no direct evidence for HF therapies in patients with advanced CKD and treatment is extrapolated from patients without CKD or patients with earlier CKD stages. The major consequence of the lack of direct evidence is the under-prescription of HF drugs to this patient population. As patients with advanced CKD and HF represent probably the highest cardiovascular risk population, the exclusion of these patients from HF trials is a serious deontological fault that must be solved. There is an urgent need to generate evidence on how to treat HF in patients with advanced CKD. This article briefly reviews the management challenges posed by HF in patients with CKD and proposes a road map to address them.
Treatment of acute kidney injury in cancer patients
Pauline Braet, Giulia Vanessa Re Sartò, Marta Pirovano, Ben Sprangers, Laura Cosmai
doi : 10.1093/ckj/sfab292
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 873–884
Acute kidney injury (AKI), either of pre-renal, renal or post-renal origin, is an important complication in cancer patients, resulting in worse prognosis, withdrawal from effective oncological treatments, longer hospitalizations and increased costs. The aim of this article is to provide a literature review of general and cause-specific treatment strategies for AKI, providing a helpful guide for clinical practice. We propose to classify AKI as patient-related, cancer-related and treatment-related in order to optimize therapeutic interventions. In the setting of patient-related causes, proper assessment of hydration status and avoidance of concomitant nephrotoxic medications is key. Cancer-related causes mainly encompass urinary compression/obstruction, direct tumoural kidney involvement and cancer-induced hypercalcaemia. Rapid recognition and specific treatment can potentially restore renal function. Finally, a pre-treatment comprehensive evaluation of risks and benefits of each treatment should always be performed to identify patients at high risk of treatment-related renal damage and allow the implementation of preventive measures without losing the potentialities of the oncological treatment. Considering the complexity of this field, a multidisciplinary approach is necessary with the goal of reducing the incidence of AKI in cancer patients and improving patient outcomes. The overriding research goal in this area is to gather higher quality data from international collaborative studies.
Early glomerular filtration rate changes in living kidney donors and recipients: an example of renal plasticity
Ana González Rinne, Cristian Acosta Sorensen, Sergio Luis Lima, Marta Gómez Gil, Natalia NegrÃn Mena, Laura DÃaz MartÃn, Ana RamÃrez, Adelaida Morales, Nicanor Vega, Eduardo Gallego, Edduin MartÃn Izquierdo, Elisa Cabello, Ana Elena RodrÃguez RodrÃguez, Jesús Pimentel González, Beatriz Escamilla, Coriolano Cruz, Lourdes Pérez Tamajón, Armando Torres RamÃrez, Flavio Gaspari, Alberto Ortiz, Esteban Porrini
doi : 10.1093/ckj/sfab220
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 885–894
In living kidney transplantation there are two different individuals, a healthy donor and a renal transplant recipient. This is an excellent human model to study factors that influence kidney function in the context of reduced renal mass and the adaptation of two comparable kidneys to different metabolic demands.
Randomized clinical study to evaluate the effect of personalized therapy on patients with immunoglobulin A nephropathy
Francesco P Schena, Giovanni Tripepi, Michele Rossini, Daniela I Abbrescia, Carlo Manno
doi : 10.1093/ckj/sfab263
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 895–902
Randomized controlled trials (RCTs) have been conducted, stratifying idiopathic immunoglobulin A nephropathy (IgAN) patients based on the laboratory findings [serum creatinine, estimated glomerular filtration rate (eGFR) and daily proteinuria]. In contrast, data from kidney biopsy have been used only for clinical diagnosis. Therefore, IgAN patients with active or chronic renal lesions have been receiving the same therapy in experimental and control arms of randomized clinical trials (RCTs).
Remote digital urinalysis with smartphone technology as part of remote management of glomerular disease during the SARS-CoV-2 virus pandemic: single-centre experience in 25 patients
Madelena Stauss, Ajay Dhaygude, Arvind Ponnusamy, Martin Myers, Alexander Woywodt
doi : 10.1093/ckj/sfab286
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 903–911
The COVID-19 pandemic has necessitated the provision of healthcare through remote and increasingly digitalized means. The management of glomerular pathology, for which urinalysis is crucial, has been notably affected. Here we describe our single-centre experience of using remote digital urinalysis in the management of patients with glomerular disease during the COVID-19 pandemic.
Comparative analysis of tools to predict rapid progression in autosomal dominant polycystic kidney disease
Javier Naranjo, Mónica Furlano, Ferran Torres, Jonathan Hernandez, Marc Pybus, Laia Ejarque, Christian Cordoba, Lluis Guirado, Elisabet Ars, Roser Torra
doi : 10.1093/ckj/sfab293
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 912–921
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disease and shows a wide phenotype. Only patients with rapid progression (RP) are included in clinical trials or are approved to receive disease-modifying drugs. This study aims at comparing different available predictive tools in ADPKD with the Mayo classification (MC) identification of rapid progressors based on high total kidney volume (TKV) according to age.
C5a receptor inhibitor avacopan in immunoglobulin A nephropathy—an open-label pilot study
Annette Bruchfeld, Hasan Magin, Patrick Nachman, Samir Parikh, Richard Lafayette, Antonia Potarca, Shichang Miao, Pirow Bekker
doi : 10.1093/ckj/sfab294
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 922–928
Improvement of proteinuria as a marker for disease activity is associated with a better renal outcome in immunoglobulin A nephropathy (IgAN). Complement is an effector pathway in IgA-mediated kidney injury. Avacopan, a selective C5a receptor inhibitor, has previously shown efficacy in anti-neutrophil cytoplasmic antibody–associated vasculitis. The aim of this study was to evaluate the safety and efficacy of avacopan in patients with IgAN with persistent proteinuria despite a maximally tolerated dose of renin–angiotensin–aldosterone system blockade. The efficacy evaluation was based on the change in proteinuria.
Time-updated anion gap and cardiovascular events in advanced chronic kidney disease: a cohort study
Yuta Asahina, Yusuke Sakaguchi, Sachio Kajimoto, Koki Hattori, Yohei Doi, Tatsufumi Oka, Jun-Ya Kaimori, Yoshitaka Isaka
doi : 10.1093/ckj/sfab277
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 929–936
Studies examining associations between metabolic acidosis and cardiovascular events in chronic kidney disease (CKD) have shown conflicting results and have not differentiated between normal anion gap (hyperchloremic) acidosis and high anion gap acidosis. We aimed to examine the impact of normal and high anion gap acidosis, separately, on the risk of cardiovascular events among patients with CKD.
Incorrect application of the KDIGO acute kidney injury staging criteria
Izak A R Yasrebi-de Kom, Dave A Dongelmans, Ameen Abu-Hanna, Martijn C Schut, Nicolette F de Keizer, John A Kellum, Kitty J Jager, Joanna E Klopotowska
doi : 10.1093/ckj/sfab256
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 937–941
Recent research demonstrated substantial heterogeneity in the Kidney Disease: Improving Global Outcomes (KDIGO) acute kidney injury (AKI) diagnosis and staging criteria implementations in clinical research. Here we report an additional issue in the implementation of the criteria: the incorrect description and application of a stage 3 serum creatinine (SCr) criterion. Instead of an increase in SCr to or beyond 4.0Â mg/dL, studies apparently interpreted this criterion as an increase in SCr by 4.0Â mg/dL.
The effect of race coefficients on preemptive listing for kidney transplantation
Mersema Abate, Nicholas Jandovitz, Jamie S Hirsch, Nadine Breslin, Lawrence Lau, Ahmed E Fahmy, Kenar D Jhaveri, Safiya Richardson, Yaser Alsalmay, Anthony Baez,Akash Mishra, Siavash Bolourani, Santiago J Miyara, Aaron Winnick, Gayatri Nair, Madhu C Bhaskaran, Elliot Grodstein, Adam M Kressel, Lewis W Teperman, Ernesto P Molmenti, Vinay Nair
doi : 10.1093/ckj/sfab287
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 942–950
Race coefficients of estimated glomerular filtration rate (eGFR) formulas may be partially responsible for racial inequality in preemptive listing for kidney transplantation.
Ambulatory blood pressure trajectories and blood pressure variability in kidney transplant recipients: a comparative study against haemodialysis patients
Maria Korogiannou, Pantelis Sarafidis, Maria Eleni Alexandrou, Marieta P Theodorakopoulou, Eva Pella, Efstathios Xagas, Antonis Argyris, Athanase Protogerou, Aikaterini Papagianni, Ioannis N Boletis, Smaragdi Marinaki
doi : 10.1093/ckj/sfab275
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 951–960
Hypertension is the most prevalent cardiovascular risk factor in kidney transplant recipients (KTRs). Preliminary data suggest similar ambulatory blood pressure (BP) levels in KTRs and haemodialysis (HD) patients. This is the first study comparing the full ambulatory BP profile and short-term BP variability (BPV) in KTRs versus HD patients.
Clinical spectrum of gross haematuria following SARS-CoV-2 vaccination with mRNA vaccines
Alexander Ritter, Birgit Helmchen, Ariana Gaspert, Joerg Bleisch, Barbara Fritschi, Florian Buchkremer, Stephanie Damm, Nicolas Schmid, Thomas Schachtner, Harald Seeger
doi : 10.1093/ckj/sfab284
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 961–973
Novel messenger RNA (mRNA)-based vaccines play an important role in current vaccination campaigns against SARS-CoV-2. They are highly efficacious and generally well tolerated. Vaccination in patients with immune-mediated kidney diseases is recommended. A number of cases with de novo or relapsing glomerulonephritis shortly after vaccine application have been reported, some of which presented with gross haematuria.
Accelerated versus watchful waiting strategy of kidney replacement therapy for acute kidney injury: a systematic review and meta-analysis of randomized clinical trials
Jui-Yi Chen, Ying-Ying Chen, Heng-Chih Pan, Chih-Chieh Hsieh, Tsuen-Wei Hsu, Yun-Ting Huang, Tao-Min Huang, Chih-Chung Shiao, Chun-Te Huang, Kianoush Kashani, Vin-Cent Wu
doi : 10.1093/ckj/sfac011
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 974–984
Critically ill patients with severe acute kidney injury (AKI) requiring kidney replacement therapy (KRT) have a grim prognosis. Recently, multiple studies focused on the impact of KRT initiation time [i.e., accelerated versus watchful waiting KRT initiation (WWS-KRT)] on patient outcomes. We aim to review the results of all related clinical trials.
COVID-19-associated pulmonary aspergillosis in hemodialysis patients
Mai Yamaoka, Masataka Banshodani, Shiro Muraoka, Kenta Tanaka, Ayaka Kimura, Hiroki Tani, Shinji Hashimoto, Nobuaki Shiraki, Sadanori Shintaku, Misaki Moriishi, Shinichiro Tsuchiya, Takao Masaki, Hideki Kawanishi
doi : 10.1093/ckj/sfac027
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 985–991
Coronavirus disease 2019 (COVID-19)-associated pulmonary aspergillosis (CAPA) is a fatal complication in the general population. However, there are few reports on CAPA in patients undergoing hemodialysis (HD).
SARS-CoV-2 antibody dynamics among kidney transplant recipients 3 months after BNT162b2 vaccination: a prospective cohort study
Dana Yelin, Benaya Rozen-Zvi, Dafna Yahav, Naomi Ben-Dor, Tali Steinmetz, Timna Agur, Boris Zingerman, Shira Schneider, Shelly Lichtenberg, Haim Ben-Zvi, Tiki Mashraki, Ruth Rahamimov
doi : 10.1093/ckj/sfac031
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 992–998
Data regarding immunogenicity of mRNA severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines among kidney transplant recipients in the months following vaccination are lacking. We aimed to investigate humoral immune response at 3–4 months post-vaccination among a cohort of kidney transplant recipients, compared with a control group of dialysis patients. Anti-spike antibodies were tested at 1 and 3–4 months after vaccination. Of 259 kidney transplant recipients tested at a median time of 110 days from second vaccine dose, 99 (38%) were seropositive, compared with 83% (101/122) of control patients. Younger age, better renal function and lower immunosuppression levels were associated with seropositivity. A total of 14% (13/94) of participants seropositive at 1 month became seronegative at follow-up and 11% (18/165) became seropositive. The latter were mainly individuals with higher antibody levels at 1 month. Antibody levels at 3–4 months were significantly reduced in both study groups, although the decline was more pronounced in the control group. Kidney transplant recipients present poor antibody response to mRNA SARS-CoV-2 vaccination, with only 38% seropositive at 3–4 months. Nevertheless, the decay in antibody response over time is modest, and some patients may present delayed response, reaching adequate antibody levels at 3–4 months. Low seropositivity rates in this group call for investigating other immunization strategies.
Middle-term outcomes in renal transplant recipients with COVID-19: a national, multicenter, controlled study
Ozgur Akin Oto, Savas Ozturk, Mustafa Arici, Arzu Velioğlu, Belda Dursun, Nurana Guller, İdris Şahin, Zeynep Ebru Eser, Saime Paydaş, Sinan Trabulus, Sümeyra Koyuncu, Murathan Uyar, Zeynep Ural, Rezzan Eren Sadioğlu, Hamad Dheir, Neriman Sıla Koç, Hakan Özer, Beyza Algül Durak, Cuma Bülent Gül, Umut Kasapoğlu, Ebru Gök Oğuz, Mehmet Tanrısev, Gülşah Şaşak Kuzgun, Safak Mirioglu, Erkan Dervişoğlu, Ertuğrul Erken, Numan Görgülü, Sultan Özkurt, Zeki Aydın, İlhan Kurultak, Melike Betül Öğütmen, Serkan Bakırdöğen, Burcu Kaya, Serhat Karadağ, Memnune Sena Ulu, Özkan Güngör, Elif Arı Bakır, Ali Rıza Odabaş, Nurhan Seyahi, Alaattin Yıldız, Kenan Ateş
doi : 10.1093/ckj/sfac045
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 999–1006
In this study, we evaluated 3-month clinical outcomes of kidney transplant recipients (KTR) recovering from COVID-19 and compared them with a control group.
Central volume shift in acute heart failure revealed by blood volume monitoring during haemodialysis
Hirofumi Sumi, Akari Ishii, Yuki Yamada, Yugo Shibagaki, Naoto Tominaga
doi : 10.1093/ckj/sfab280
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 1007–1009
Central volume shift is one of the major pathophysiological mechanisms of acute pulmonary oedema in acute heart failure (AHF). Pathological vasoconstriction results in central volume shift; however, its onset and course have been rarely detected or recorded in clinical practice. We report an exceptional case of AHF developing during haemodialysis, with marked blood pressure (BP) elevation and paradoxical repeated reduction in blood volume (BV) detected by real-time BV monitoring, accompanied by worsening dyspnoea. This inverse correlation of BV and BP during haemodialysis indicates that the theoretical central volume shift was captured in real-world AHF.
COVID-19 vaccination precipitating de novo ANCA-associated vasculitis: clinical implications
Aaron Shengting Mai, Eng-King Tan
doi : 10.1093/ckj/sfac026
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 1010–1011
Sustained humoral response 6 months after the anti-SARS-CoV-2 mRNA-BNT162b2 vaccine in haemodialysis patients: should booster vaccine doses be given to all patients at the same time?
Vincenzo La Milia, Silvia Tonolo, Francesco Luzzaro, Claudio Bonato, Monica Limardo, Selena Longhi, Chiara Ravasi, Sara Viganò, Andrea Cavalli
doi : 10.1093/ckj/sfac047
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 1012–1014
COVID-19 vaccination in anti-neutrophil cytoplasmic antibody-associated vasculitis: lessons from influenza vaccination?
Jackie Sim, Tung Lin Lee, Cynthia Ciwei Lim
doi : 10.1093/ckj/sfac053
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 1015–1016
Gender distribution in presidents and board members of European nephrology societies
Svenja Ravioli, Gregor Lindner, Michael Haidinger
doi : 10.1093/ckj/sfab259
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 1017–1018
Coturnism as a cause of deadly rhabdomyolysis in Biblical times
Elias E Mazokopakis, Christos G Karagiannis
doi : 10.1093/ckj/sfab268
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 1019–1020
Early and late ANCA vasculitis relapses after kidney transplantation may have different presentations
Christophe Masset, Christine Kandel-Aznar, Jacques Dantal, Magali Giral, Maryvonne Hourmant, Gilles Blancho, Claire Garandeau
doi : 10.1093/ckj/sfac016
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 1021–1023
Hypoxia-inducible factor prolyl hydroxylase inhibitors in kidney transplant recipients
Masatomo Ogata, Takamasa Miyauchi, Yuko Sakurai, Marie Murata, Kazunobu Shinoda, Yugo Shibagaki, Masahiko Yazawa
doi : 10.1093/ckj/sfac015
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Pages 1024–1026
Correction to: Accelerated versus watchful waiting strategy of kidney replacement therapy for acute kidney injury: a systematic review and meta-analysis of randomized clinical trials
Jui-Yi Chen, Ying-Ying Chen, Heng-Chih Pan, Chih-Chieh Hsieh, Tsuen-Wei Hsu, Yun-Ting Huang, Tao-Min Huang, Chih-Chung Shiao, Chun-Te Huang, Kianoush Kashani, Vin-Cent Wu
doi : 10.1093/ckj/sfac059
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Page 1027
Randomized clinical study to evaluate the effect of personalized therapy on patients with immunoglobulin A nephropathy
Francesco P Schena, Giovanni Tripepi, Michele Rossini, Daniela I Abbrescia, Carlo Manno
doi : 10.1093/ckj/sfac063
Clinical Kidney Journal, Volume 15, Issue 5, May 2022, Page 1028
Primary hyperoxaluria type 1: time for prime time?
Justine Bacchetta, Kyle D Wood
doi : 10.1093/ckj/sfab233
Clinical Kidney Journal, Volume 15, Issue Supplement_1, May 2022, Pages i1–i3
Primary hyperoxaluria type 1: pathophysiology and genetics
Sonia Fargue, Cécile Acquaviva Bourdain
doi : 10.1093/ckj/sfab217
Clinical Kidney Journal, Volume 15, Issue Supplement_1, May 2022, Pages i4–i8
Treatment of primary hyperoxaluria type 1
Asheeta Gupta, Michael J G Somers, Michelle A Baum
doi : 10.1093/ckj/sfab232
Clinical Kidney Journal, Volume 15, Issue Supplement_1, May 2022, Pages i9–i13
Primary hyperoxaluria type 1: urologic and therapeutic management
Harjivan Kohli, Michael P Kurtz
doi : 10.1093/ckj/sfab187
Clinical Kidney Journal, Volume 15, Issue Supplement_1, May 2022, Pages i14–i16
While the surgical approaches available in primary hyperoxaluria (PH) are common to all patients requiring intervention for urolithiasis, the indications for treatment and their corresponding toxicities are unique. Being a rare disease, we are guided by case series. This review summarizes the available literature highlighting the important disease-specific considerations. Shockwave lithotripsy (SWL) is of particular interest. It is generally the first-line treatment for stones in children, but here the stones produced will be relatively resistant to fragmentation. In addition, there are concerning reports in children of sudden unilateral decline in function in the treated kidney as measured by nuclear renography. Percutaneous nephrostolithotomy might intuitively seem favorable given the shortest drain duration and the ability to treat larger stones efficiently but, similar to SWL, rapid chronic kidney disease (CKD) progression has been seen postoperatively. Ureteroscopy is therefore generally the safest option, but considerations regarding stent encrustation, the growth of residual fragments and the large volume of stone often faced may limit this approach. The surgeon must balance the above with consideration of the patient's CKD status when considering a plan of monitoring and treating stones in PH.
Primary hyperoxaluria type 1: novel therapies at a glance
Justine Bacchetta, John C Lieske
doi : 10.1093/ckj/sfab245
Clinical Kidney Journal, Volume 15, Issue Supplement_1, May 2022, Pages i17–i22
Primary hyperoxaluria type 1 (PH1) is a rare and severe autosomal recessive disease of oxalate metabolism, resulting from a mutation in the AGXT gene that encodes the hepatic peroxisomal enzyme alanine–glyoxylate aminotransferase (AGT). Until recently, treatment of PH1 was supportive, consisting of intensive hyperhydration, use of crystallization inhibitors (citrate and neutral phosphorus), in a subset of responsive PH1 patients’ pharmacologic doses of vitamin B6 (pyridoxine), and kidney and liver transplantation when patients progressed to kidney failure. Treatment approaches have been similar for PH2 caused by mutations in hepatic glyoxylate reductase/hydroxypyruvate reductase (GR/HPR), although pyridoxine does not have any benefit in this group. PH3 is caused by mutations of mitochondrial 4-hydroxy-2-oxoglutarate aldolase (HOGA1) and was the most recently described. Kidney failure appears less common in PH3, although kidney stones occur as frequently as in PH1 and PH2. Oxalate metabolism in the liver is complex. Novel therapies based on RNA interference (RNAi) have recently emerged to modulate these pathways, designed to deplete substrate for enzymes upstream and decrease/avoid oxalate production. Two hepatic enzymes have been targeted to date in PH: glycolate oxidase (GO) with lumasiran and lactate dehydrogenase A (LDH-A) with nedosiran. Lumasiran was approved for the treatment of PH1 in 2020 by both the European Medicines Agency and the Food and Drug Administration, whilst clinical trials with nedosiran are ongoing. Results with the two RNAi therapies demonstrate a significant reduction of urinary oxalate excretion in PH1 patients, but long-term data on efficacy (preservation of kidney function, decreased stone events) and safety remain to be established. Nevertheless, the hepatically targeted RNAi approach represents a potential ‘game changer’ in the field of PH1, bringing hope to families and patients that they may be able to avoid liver and/or kidney transplantation in the future and suffer fewer stone events, perhaps with less strict therapeutic regimens. Pharmacological compounds directly inhibiting GO or LDH are also under development and could be of special interest in developing countries where RNAi therapies may not be readily available in the near future. Approaches to manipulate the intestinal microbiome with a goal to increase oxalate degradation or to stimulate secretion of oxalate into the intestine from plasma are also under development. Overall, we appear to be entering a new phase of PH treatment, with an array of promising approaches emerging that will need optimization and evaluation to establish long-term efficacy and safety.
Primary hyperoxaluria: the pediatric nephrologist's point of view
Efrat Ben-Shalom, Sander F Garrelfs, Jaap W Groothoff
doi : 10.1093/ckj/sfab231
Clinical Kidney Journal, Volume 15, Issue Supplement_1, May 2022, Pages i23–i28
The clinical presentation of primary hyperoxaluria in children ranges from mildly symptomatic nephrocalcinosis to very early onset end-stage kidney failure with systemic oxalosis, a devastating complication. We review the various manifestations of pediatric hyperoxaluria, treatment options for children with preserved kidney function and appropriate dialysis regimens. Liver or combined liver/kidney transplantation is currently the only definitive treatment for primary hyperoxaluria type 1, but novel RNA interference treatments offer hope for the future. Finally, we address the medical and ethical dilemmas facing pediatricians treating children with hyperoxaluria.
Primary hyperoxaluria: the adult nephrologist's point of view
Shabbir H Moochhala, Elaine M Worcester
doi : 10.1093/ckj/sfac068
Clinical Kidney Journal, Volume 15, Issue Supplement_1, May 2022, Pages i29–i32
In adults, primary hyperoxaluria (PH) does not always present as obviously as in children, leading to delayed or even missed diagnosis. When diagnosed in adulthood, PH usually progresses at a slower rate and the focus is on the prevention of recurrent kidney stones as much as it is on the preservation of renal function. The most tragic presentation is when the diagnosis is made after primary non-function of a renal graft for treating previously unknown renal disease. Recurrent stones, nephrocalcinosis and features of systemic oxalosis can all be presenting features. For these reasons, consideration should be given to screening for this rare condition, using biochemical and/or genetic means, but being careful to exclude common differential diagnoses. Such efforts should be synchronized with diagnostic methods for other rare kidney diseases.
Primary hyperoxaluria type 1 in developing countries: novel challenges in a new therapeutic era
Neveen A Soliman, Sameh Mabrouk
doi : 10.1093/ckj/sfab203
Clinical Kidney Journal, Volume 15, Issue Supplement_1, May 2022, Pages i33–i36
Primary hyperoxaluria type 1 (PH1) is an autosomal recessive inborn error of metabolism characterized by marked hepatic overproduction of oxalate due to deficiency of hepatic peroxisomal alanine-glyoxylate aminotransferase caused by AGXT gene mutation. One major hallmark of PH1 in developed as well as developing countries (DC) is the diagnostic delay. Notably in DC, where the disease is most prevalent and probably underdiagnosed, there are many challenges in PH1 diagnosis and management, with economic constrains and ethical concerns. This has led to the existing gap in the management of PH1 between developed and DC, which is expected to further deepen with the advent of novel therapeutic agents unless appropriate actions are taken. Until recently, treatment possibilities were limited to supportive measures. Thanks to a better understanding of the molecular basis of the disease a number of new therapies are developed, or being developed, leading to profound changes in management strategies. In this review we discuss the current situation of PH1 in DC as well as the accessibility challenges and the advantages of using promising novel therapeutics to bridge the currently existing gap. We also provide an overview of an integrated approach to ensure equitable access of sustainable therapeutics to PH1 patients in DC. This is expected to reduce global PH1 healthcare disparities, improve its standard of care and reduce disability linked to extrarenal complications of PH1 by implementing personalized medicine.
