International Journal of Nephrology and Renovascular Disease最新文献

{"title":"Lumasiran in the Management of Patients with Primary Hyperoxaluria Type 1: From Bench to Bedside.","authors":"Viola D'Ambrosio, Pietro Manuel Ferraro","doi":"10.2147/IJNRD.S293682","DOIUrl":"10.2147/IJNRD.S293682","url":null,"abstract":"<p><p>Primary hyperoxaluria (PH) is a rare genetic disease caused by excessive hepatic production and elevated urinary excretion of oxalate that leads to recurrent nephrolithiasis, nephrocalcinosis and, eventually, kidney failure. As glomerular filtration rate declines, oxalate accumulates leading to systemic oxalosis, a debilitating condition with high morbidity and mortality. Although PH is usually diagnosed during infancy, it can present at any age with different phenotypes, ranging from mild symptoms to extremely debilitating manifestations. PH is an autosomal recessive disorder and, to date, three types have been identified: PH1, PH2 and PH3. PH1 is the most common and most aggressive type, accounting for almost 80% of primary hyperoxaluria diagnoses. Until 2020, general treatment for PH1 consisted mainly in high fluid intake, urine alkalization, surgical management of recurrent nephrolithiasis and eventually, if and when kidney failure occurred, intensive dialysis regimens and transplantation strategies (simultaneous or sequential liver-kidney transplant or isolated liver/kidney transplant in carefully selected patients). Specific treatment did and still consists in administration of pyridoxine hydrochloride, although it is only effective in a subset of PH1 patients. Lumasiran, a novel biological drug based on mRNA interference that has been recently approved in the US and European Union, showed promising results and is set to be a turning point in the management of PH1. This literature review aims to summarize the available evidence on PH1 treatment with lumasiran, in order to provide both pediatric and adult nephrologists and clinicians with the knowledge for the identification and management of PH1 patients suitable for treatment.</p>","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2022-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ce/b9/ijnrd-15-197.PMC9211742.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40391158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Light Chain Cast Nephropathy in Multiple Myeloma: Prevalence, Impact and Management Challenges","authors":"P. Menè, A. Stoppacciaro, S. Lai, F. Festuccia","doi":"10.2147/IJNRD.S280179","DOIUrl":"https://doi.org/10.2147/IJNRD.S280179","url":null,"abstract":"Abstract “Cast nephropathy” (CN) is a pathological feature of myeloma kidney, also seen to a lesser extent in the context of severe nephrotic syndrome from non-haematological diseases. The name relates to obstruction of distal tubules by “casts” of luminal proteins concentrated by intensive water reabsorption resulting from dehydration or high-dose diuretics. Filtered proteins form complexes with endogenous tubular Tamm-Horsfall glycoprotein. The resulting gel further slows or stops luminal flow upon complete obstruction of distal convoluted tubules and collecting ducts. Thus, a tubular obstructive form of acute kidney injury (AKI) is a common consequence of CN. The pathogenesis of CN will be reviewed in light of recent advances in the understanding of monoclonal disorders of B lymphocytes, leading to the release of immunoglobulin components (free light chains, FLC) into the bloodstream and their filtration across the glomerular basement membrane. Treatment aiming at reduction of the circulating burden of FLC may help recovery of renal function in a fraction of these patients, besides filling the void between the onset of AKI, histopathological diagnosis, and full response to pharmacologic treatment.","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46610530","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}

{"title":"Clinical Factors Associated with Serum Magnesium Concentration in Patients Undergoing Peritoneal Dialysis: A Single-Center Observational Study","authors":"S. Kaneko, S. Ookawara, Y. Morishita","doi":"10.2147/IJNRD.S357130","DOIUrl":"https://doi.org/10.2147/IJNRD.S357130","url":null,"abstract":"Purpose Magnesium (Mg) is an essential element that is associated with various physiological functions, such as maintenance of blood pressure, muscle contraction, and nerve function. In patients undergoing hemodialysis, hypomagnesemia is associated with cardiovascular and all-cause mortality. However, in patients undergoing peritoneal dialysis (PD), clinical factors associated with Mg have not been fully investigated. Patients and Methods Clinical factors including anthropometric data, comorbidities, laboratory data, medications, and dialysis methods were collected from the medical records of patients undergoing PD. The associations of these factors with the serum Mg concentration were investigated by univariate and multivariate analyses. Results Sixty patients undergoing PD were investigated. The univariate analysis showed that the serum Mg concentration was significantly associated with treatment by hybrid PD (daily PD + once-weekly hemodialysis) (β = 0.264, P = 0.04), administration of phosphate binders (β = 0.294, P = 0.02), the serum C-reactive protein concentration (β = −0.318, P = 0.01), the serum potassium (K) concentration (β = 0.451, P < 0.01), and the serum intact parathormone concentration (β = −0.333, P = 0.01). The multivariate analysis using these factors showed an independent association between the serum Mg and K concentrations (β = 0.333, P = 0.01). Conclusion The serum Mg concentration was independently associated with the serum K concentration in patients undergoing PD.","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41829673","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}

{"title":"The Influence of an Unexpected Switch of Hemodialysis Facilities on the Quality of Life (QOL) in Hemodialysis Patients","authors":"Y. Shimizu, Keiichi Wakabayashi, Junichiro Nakata, H. Io, C. Hamada, Y. Tomino, Yusuke Suzuki","doi":"10.2147/IJNRD.S358915","DOIUrl":"https://doi.org/10.2147/IJNRD.S358915","url":null,"abstract":"Background We experienced a sudden breakdown of hemodialysis system during a clinical study of dermal itch and serum BNP concentrations. Patients and Methods Forty-eight hemodialysis patients were enrolled in the itch-related study. It was intended to improve itch by lowering BNP with supportive communication between the patients and the dialysis staff. We planned to collect data, including visual analogue scale (VAS), dermatology life quality index (DLQI), blood samples and QOL score (KDQOL-SF), four times over a four week interval. The first data was obtained just prior to switching facilities due to a breakdown. The patients underwent hemodialysis in other facilities for two weeks and underwent determination of their clinical data, including QOL scores, three times. Results While mean blood pressure showed no significant differences, serum albumin, iron and phosphate levels were worsened significantly between pre- and post-relocation. Serum BNP and DLQI showed no significant changes. VAS was significantly worsened. The mean values of the cognitive function in the KDQOL-SF and sleep and the role-physical, role-emotional, social function, dialysis staff’s encouragement in SF-36 analysis were identified as statistically significant items by MANOVA. Both SF-12 physical and mental composites were also statistically significant. Although SF-12 physical composites were significant among the patients under 66 yrs of age (median), eight factors were significant among those over 66 yrs. Independent analyses revealed every item that was detected worsened significantly after the switch of facilities. Conclusion Unexpected switching of hemodialysis facilities severely impacts the QOL for a long duration as well as the patients’ symptom and laboratory data.","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47410249","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}

{"title":"Incremental Hemodialysis: What We Know so Far","authors":"V. Soi, M. Faber, Ritika Paul","doi":"10.2147/IJNRD.S286947","DOIUrl":"https://doi.org/10.2147/IJNRD.S286947","url":null,"abstract":"Abstract Traditionally, patients that develop progressive chronic kidney disease in need of kidney replacement therapy are prescribed thrice weekly in-center hemodialysis sessions at the beginning of therapy. This empiric prescription is based on historic trials that were comprised of mostly prevalent patients. Incremental hemodialysis is the process of performing <3 sessions of dialysis per week or limiting dialysis dose by duration at the initial onset of treatment to provide a more gradual transition, mimicking the progressive nature of kidney disease. Adding clearance contributions from residual kidney function is the standard of care with peritoneal dialysis but has not routinely been employed with hemodialysis. Accounting for residual kidney function accompanied by improvement in adjuvant pharmacotherapy, such as newer potassium binding agents and dietary modification, can augment dialytic clearances and allow for an incremental approach. Utilizing incremental dialysis has been associated with both preserving residual kidney function as well as improving patient quality of life. Barriers to this approach include concerns regarding patient acceptance of dialysis prescription changes, adherence to therapy, and provider factors that would require a restructuring of the current thrice weekly hemodialysis rubric. Candidacy for incremental therapy has shown the best outcomes when urea clearances exceed 3 mL/min and urine volumes are >500 mL/day, although these measures have been deemed conservative. A significant amount of retrospective and registry data has been supportive of initiating incremental hemodialysis and several pilot studies have shown the feasibility of implementing such an approach. Larger, randomized control trials are needed to fully evaluate safety and efficacy to allow for more widespread acceptance of this patient-centered approach to chronic kidney disease.","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41335501","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}

{"title":"Serum Phosphorus and Pill Burden Among Hemodialysis Patients Prescribed Sucroferric Oxyhydroxide: One-Year Follow-Up on a Contemporary Cohort","authors":"J. Kendrick, Mei Zhou, Linda H. Ficociello, Vidhya Parameswaran, C. Mullon, M. Anger, D. Coyne","doi":"10.2147/IJNRD.S353213","DOIUrl":"https://doi.org/10.2147/IJNRD.S353213","url":null,"abstract":"Purpose In prior analyses of real-world cohorts of hemodialysis patients switched from one phosphate binder (PB) to sucroferric oxyhydroxide (SO), SO therapy has been associated with improvements in serum phosphorus (sP) and reductions in daily PB pill burden. To characterize how SO initiation patterns have changed over time, we examined the long-term effectiveness of SO in a contemporary (2018–2019) cohort. Patients and Methods Adult Fresenius Kidney Care hemodialysis patients first prescribed SO monotherapy as part of routine care between May 2018 and May 2019 (N = 1792) were followed for 1 year. All patients received a non-SO PB during a 91-day baseline period before SO prescription. Mean PB pills/day and laboratory parameters were compared before and during SO treatment. Results were divided into consecutive 91-day intervals (Q1–Q4) and analyzed using linear mixed-effects regression and Cochran’s Q test. These results were contrasted with findings from a historical (2014–2015) cohort (N = 530). Results The proportion of patients achieving sP ≤5.5 mg/dl increased after switching to SO (from 27.0% at baseline to 37.8%, 45.1%, 44.7%, and 44.0% at Q1, Q2, Q3, and Q4, respectively; P < 0.0001 for all). The mean daily PB pill burden decreased from a baseline of 7.7 to 4.4, 4.6, 4.8, and 4.9, respectively, across quarters (P < 0.0001 for all). Patients in the contemporary cohort had improved sP control (27.0% achieving sP ≤5.5 mg/dl vs 17.7%) and lower daily PB pill burden (mean 7.7 vs 8.5 pills/day) at baseline than those in the historical cohort. Overall use of active vitamin D was similar between cohorts, although higher use of oral active vitamin D (63.9% vs 15.7%) and lower use of IV active vitamin D lower (23.4% vs 74.2%) was observed in the contemporary cohort. Conclusion Despite evolving treatment patterns, switching to SO resulted in improved sP control with fewer pills per day in this contemporary hemodialysis cohort.","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41324026","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}

{"title":"Epidemiology, Impact, and Management Strategies of Anti-Glomerular Basement Membrane Disease","authors":"Muhammad Asim, M. Akhtar","doi":"10.2147/IJNRD.S326427","DOIUrl":"https://doi.org/10.2147/IJNRD.S326427","url":null,"abstract":"Abstract Anti-glomerular basement membrane (anti-GBM) disease is a rare but serious autoimmune disease, which is characterized by the development of pathogenic antibodies to type IV collagen antigens in the glomerular and alveolar basement membranes. This results in rapidly progressive glomerulonephritis (GN), alveolar hemorrhage, or both. A variety of environmental factors can trigger the disease in genetically predisposed patients. Temporal associations with influenza, SARS-CoV-2 infection, and COVID-19 vaccination have been described although there is insufficient evidence to suggest causality. Anti-GBM disease accounts for approximately 20% of the cases of rapidly progressive GN cases secondary to crescentic GN, but is an uncommon cause of end-stage kidney disease. Early diagnosis by detection of circulating antibodies, increased awareness of atypical as well as complex clinical variants of the disease, and combined therapy with immunosuppression and plasma exchange has improved the prognosis of patients with this potentially fatal disease. Progress has been hampered by the rarity of anti-GBM disease, but new agents and therapeutic regimens are emerging.","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45401220","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}

{"title":"Fat Free Weight Prediction in Morbidly Obese Females [Corrigendum]","authors":"","doi":"10.2147/IJNRD.S366708","DOIUrl":"https://doi.org/10.2147/IJNRD.S366708","url":null,"abstract":"[This corrects the article DOI: 10.2147/IJNRD.S24173.].","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44804738","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}

{"title":"Subjective Assessment of Sleep Quality and Excessive Daytime Sleepiness in Conventional Hemodialysis Population: A Single-Center Experience.","authors":"Shanmuganathan Velu,&nbsp;Arul Rajagopalan,&nbsp;Jegan Arunachalam,&nbsp;Arun Prasath,&nbsp;Rakesh Durai","doi":"10.2147/IJNRD.S351515","DOIUrl":"https://doi.org/10.2147/IJNRD.S351515","url":null,"abstract":"<p><strong>Introduction: </strong>Sleep disturbances are common in patients with end-stage kidney disease on hemodialysis (hemodialysis population: HDP). Higher rates of primary sleep disorders, demographic characteristics, metabolic abnormalities, and the efficacy of treatment place HDP at higher risk. The pattern observed is delayed onset of sleep, frequent awakening episodes, insomnia, sleep apnoea, excessive daytime sleepiness, restless leg syndrome, abnormal limb movements, pain in limbs, confusion, and nightmares. Two commonly used subjective assessment scores are the Pittsburgh Sleep Quality Index (PSQI) to assess sleep quality and the Epworth Sleepiness Scale (ESS) to assess excessive daytime sleepiness.</p><p><strong>Objective: </strong>Subjective assessment of sleep using PSQI and ESS scores in HDP and correlation with clinical and demographic characteristics.</p><p><strong>Patients and methods: </strong>A cross-sectional descriptive study of 148 patients with ESKD undergoing in-center hemodialysis. From June 2021 to October 2021 in Madurai medical college, Madurai, India. Subjective assessment with PSQI and ESS scores was done to identify sleep quality and daytime sleepiness, respectively.</p><p><strong>Results: </strong>The median PSQI score was 6 (IQ:4-10), and as much as 68.24% scored >5 on the PSQI (poor sleepers). The median ESS score of the study participants was 4 (Iq range 3-7), and 19.59% had a total ESS score of more than 10 (excessive daytime sleepiness). The mean age of the participants was 44±14.5. Age more than 60, lower body mass index, unemployment, higher dialysis vintage of more than 2 years, lower hemoglobin, high calcium-phosphorus product are statistically significant for both PSQI and ESS scores.</p><p><strong>Conclusion: </strong>The prevalence of poor sleep quality and excessive daytime sleepiness is high in HDP. Subjective assessment scores (PSQI and ESS) on the bedside are valuable tools in identifying sleep quality and EDS where objective assessment methods are not feasible and will help in the short time identification and management of sleep disturbances.</p>","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/fe/c3/ijnrd-15-103.PMC8932937.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40305835","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Glomerulus-on-a-Chip: Current Insights and Future Potential Towards Recapitulating Selectively Permeable Filtration Systems.","authors":"Kotaro Doi, Hiroshi Kimura, Yukiko T Matsunaga, Teruo Fujii, Masaomi Nangaku","doi":"10.2147/IJNRD.S344725","DOIUrl":"10.2147/IJNRD.S344725","url":null,"abstract":"<p><p>Glomerulopathy, characterized by a dysfunctional glomerular capillary wall, results in proteinuria, leading to end-stage renal failure and poor clinical outcomes, including renal death and increased overall mortality. Conventional glomerulopathy research, including drug discovery, has mostly relied on animal experiments because in-vitro glomerulus models, capable of evaluating functional selective permeability, was unavailable in conventional in-vitro cell culture systems. However, animal experiments have limitations, including time- and cost-consuming, multi-organ effects, unstable reproducibility, inter-species reliability, and the social situation in the EU and US, where animal experiments have been discouraged. Glomerulus-on-a-chip, a new in-vitro organ model, has recently been developed in the field of organ-on-a-chip research based on microfluidic device technology. In the glomerulus-on-a-chip, the podocytes and endothelial cells are co-cultured in a microfluidic device with physical stimuli that mimic the physiological environment to enhance cell function to construct a functional filtration barrier, which can be assessed by permeability assays using fluorescently labeled molecules including inulin and albumin. A combination of this glomerulus-on-a chip technology with the culture technology to induce podocytes and endothelial cells from the human pluripotent stem cells could provide an alternative organ model and solve the issue of animal experiments. Additionally, previous experiments have verified the difference in the leakage of albumin using differentiated podocytes derived from patients with Alport syndrome, such that it could be applied to intractable hereditary glomerulopathy models. In this review, we provide an overview of the features of the existing glomerulus-on-a-chip systems, focusing on how they can address selective permeability verification tests, and the challenges they involved. We finally discuss the future approaches that should be developed for solving those challenges and allow further improvement of glomerulus-on-a-chip technologies.</p>","PeriodicalId":14181,"journal":{"name":"International Journal of Nephrology and Renovascular Disease","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2022-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8922329/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46334448","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}