American Journal of Kidney Diseases最新文献

{"title":"Patient, Parental, and Health Professional Perspectives on Growth in Children With CKD.","authors":"Justin G Wu, Chandana Guha, Anastasia Hughes, Luca G Torrisi, Jonathan C Craig, Aditi Sinha, Allison Dart, Allison A Eddy, Detlef Bockenhauer, Hui-Kim Yap, Jaap Groothoff, Stephen I Alexander, Susan L Furth, Susan Samuel, Simon A Carter, Amanda Walker, Joshua Kausman, Allison Jaure","doi":"10.1053/j.ajkd.2024.06.016","DOIUrl":"https://doi.org/10.1053/j.ajkd.2024.06.016","url":null,"abstract":"<p><strong>Rationale & objective: </strong>Growth failure is a common problem among children with chronic kidney disease (CKD). Reduced height is associated with psychosocial burden, social stigma, and impaired quality of life. This study aimed to describe the aspects of growth impairment that are most impactful from the perspectives of children with CKD, their parents, and health professionals.</p><p><strong>Study design: </strong>Qualitative study.</p><p><strong>Settings & participants: </strong>120 children with CKD (aged 8-21 years), 250 parents, and 445 health professionals from 53 countries participated in 16 focus groups, two consensus workshops, and a Delphi survey.</p><p><strong>Analytical approach: </strong>A thematic analysis of all qualitative data concerning growth from the Standardized Outcomes in Nephrology - Children and Adolescents (SONG-Kids) initiative.</p><p><strong>Results: </strong>We identified five themes: diminishing psychological wellbeing (compared to and judged by peers, tired of explaining to others, damaging self-esteem), constrained life participation and enjoyment (deprived of normal school experiences, excluded from sports or competing at a disadvantage, impaired quality of life in adulthood); grappling with impacts of symptoms and treatment (difficulty understanding short stature and accessing help, lack of appetite, uncertainty regarding bone pains, medication side effects, burden of growth hormone treatment); facilitating timely interventions and optimizing outcomes (early indicator of disease, assessing management, maximizing transplant outcomes, minimizing morbidity); and keeping growth and health priorities in perspective (quality of life and survival of utmost priority, achieved adequate height).</p><p><strong>Limitations: </strong>Only English-speaking participants were included.</p><p><strong>Conclusions: </strong>Impaired growth may diminish psychological wellbeing, self-esteem, and participation in daily activities for children with CKD. Balancing different treatments that can affect growth complicates decision-making. These findings may inform the psychosocial support needed by children with CKD and their caregivers to address concerns about growth.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141911353","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Symptom Burden and Its Impact on Quality of Life in Patients With Moderate to Severe CKD: The International Chronic Kidney Disease Outcomes and Practice Patterns Study (CKDopps).","authors":"Elodie Speyer, Charlotte Tu, Jarcy Zee, Ricardo Sesso, Antonio A Lopes, Emilie Moutard, Abdou Y Omorou, Bénédicte Stengel, Fredric O Finkelstein, Roberto Pecoits-Filho, Natalia Alencar de Pinho, Ronald L Pisoni","doi":"10.1053/j.ajkd.2024.06.011","DOIUrl":"https://doi.org/10.1053/j.ajkd.2024.06.011","url":null,"abstract":"<p><strong>Rationale & objective: </strong>Recent evidence suggests substantial burden of symptoms experienced by people with non-dialysis chronic kidney disease (ND-CKD), but informative large-scale studies are scarce. We aimed to assess the prevalence of symptoms, and the association of overall symptom burden with quality of life in patients with moderate to severe CKD.</p><p><strong>Study design: </strong>Cross-sectional study.</p><p><strong>Setting & participants: </strong>4430 patients with ND-CKD stages 3-5 enrolled into the CKDopps Study in Brazil, France, and the US between 2013 and 2021.</p><p><strong>Exposures: </strong>13 individual patient-reported symptoms from the KDQOL-SF questionnaire and an overall symptom burden score (low, intermediate, and high).</p><p><strong>Outcomes: </strong>Physical and mental component summary scores (PCS, MCS) of the KDQOL-SF.</p><p><strong>Analytical approach: </strong>Adjusted prevalence ratios and generalized estimating equations.</p><p><strong>Results: </strong>Patients (mean age: 68 years; 40% women; mean baseline eGFR: 30 mL/min/1.73m²) were very much to extremely bothered by numerous symptoms [\"soreness in muscles\" (23%), \"washed out or drained\" (21%), \"cramps, shortness of breath, dry skin, diminished sex life, or numbness in hands or feet\" (14-17%)]. The adjusted prevalences of \"cramps\", \"washed out or drained\", \"lack of appetite\", \"nausea/upset stomach\", and \"sex life\" were greater with more severe CKD, and, except for \"sex life\", in women. A high overall symptom burden was more common in women, in France, and in patients with severe albuminuria and various comorbidities, but not with lower eGFR. PCS and MCS scores were 13.4 and 7.7 points lower, respectively, for high vs. low overall symptom burden.</p><p><strong>Limitations: </strong>Generalizability limited to patients under nephrology care, residual confounding and inaccurate Brazilian translation of some symptoms.</p><p><strong>Conclusions: </strong>The high symptom burden observed in this large cohort of ND-CKD patients across three diverse countries and its strong association with poorer HRQOL should inform clinical management of and clinical research in CKD.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141905565","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Representation of Low- and Middle-Income Countries in CKD Drug Trials: A Systematic Review.","authors":"Gabriel Cojuc-Konigsberg, Alberto Guijosa, Alberto Moscona-Nissan, Alberto Nordmann-Gomes, Vianca Anabel Canaviri-Flores, Alan Braverman-Poyastro, Regina de la Fuente-Ramírez, Denisse Tinajero-Sánchez, Alejandra de Las Fuentes Cepeda, Andrés Noyola-Pérez, Rafael Lozano, Ricardo Correa-Rotter, Juan C Ramírez-Sandoval","doi":"10.1053/j.ajkd.2024.06.012","DOIUrl":"https://doi.org/10.1053/j.ajkd.2024.06.012","url":null,"abstract":"<p><strong>Rationale & objective: </strong>Almost 80% of individuals with chronic kidney disease (CKD) reside in low- and middle-income countries (LMICs) and are potentially under-represented in randomized controlled clinical trials (RCTs). We assessed the global distribution of RCTs comparing pharmacological treatments for CKD over the past two decades, as well as the magnitude and evolution of participation by LMICs.</p><p><strong>Study design: </strong>Systematic review.</p><p><strong>Setting & study populations: </strong>RCTs evaluating pharmacological interventions in adults with CKD.</p><p><strong>Selection criteria for studies: </strong>RCTs published between 2003-2023 and indexed in MEDLINE.</p><p><strong>Data extraction: </strong>Each trial was reviewed and extracted independently by two investigators. Disagreements were settled by consensus or a third reviewer.</p><p><strong>Analytical approach: </strong>RCT participation of World Bank-defined income groups and geographic regions were described and the representation indices (RI) according to RCT participants and estimated CKD prevalences were calculated. RCTs were also categorized as global, regional, or national in scope.</p><p><strong>Results: </strong>Among 7,760 identified studies, we included 1,366 RCTs conducted in 84 countries with 301,158 participants. National, regional, and global RCTs represented 85.4%, 3.5%, and 11.1% of studies, respectively. LMICs were included in 34.7% of RCTs. No RCTs included participants from low-income countries, and lower-middle-income countries participated in 13.2%. Of participants from RCTs with available information, 25.4% (n=64,843/255,237) were from LMICs. According to the RI, six LMICs were over-represented (>1.25), seven adequately represented (0.75-1.25), and 26 under-represented (<0.75). Most (80.2%) global CKD RCTs included LMICs; however, LMIC participants constituted only 32.9% of the global trial population. We observed a positive trend in LMIC inclusion over time, rising from 22.9% (n=71/310) in 2003-2007 to 45.5% (n=140/308) in 2018-2023.</p><p><strong>Limitations: </strong>The use of an income-group dichotomy, exclusion of non-randomized studies of intervention, and studies identified in one database.</p><p><strong>Conclusions: </strong>Despite an increase in participation over the past two decades, individuals with CKD from LMICs remain significantly under-represented in RCTs. These findings suggest that increased efforts are warranted to increase LMIC representation in pharmacological CKD RCTs.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141905564","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Continuous Kidney Replacement Therapy in Pediatric Intensive Care Unit: Little People, Big Gaps.","authors":"Jia Xin Huang, Jessica Ashley J Williams, Raymond K Hsu","doi":"10.1053/j.ajkd.2024.06.010","DOIUrl":"https://doi.org/10.1053/j.ajkd.2024.06.010","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141873898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Patterns in Emergency Clinician Management of Acute Kidney Injury.","authors":"Jonathon Mitchell, Michael R Ehmann, Scott Levin, Xihan Zhao, Steven Menez, Chirag R Parikh, Eili Y Klein, Jeremiah S Hinson","doi":"10.1053/j.ajkd.2024.05.017","DOIUrl":"https://doi.org/10.1053/j.ajkd.2024.05.017","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141878213","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Homozygosity for a Rare FASTKD2 Variant Resulting in an Adult Onset Autosomal Recessive Mitochondrial Podocytopathy.","authors":"Francisco Pereira Gonçalves, Isabel Tavares, Roberto Silva, Ana Teresa Nunes, Luciano Pereira, Andreia Campos, Joel Pinto, Ana Lopes, Marta Simões, Manuela Grazina, Agnes B Fogo, João Paulo Oliveira","doi":"10.1053/j.ajkd.2024.05.018","DOIUrl":"10.1053/j.ajkd.2024.05.018","url":null,"abstract":"<p><p>Mitochondrial cytopathies can have kidney involvement in up to half of cases. Their diagnosis is challenging due to phenotypic variability, lack of noninvasive tests to assess mitochondrial dysfunction, and genetic heterogeneity. We report on a young adult male with hypertrophic cardiomyopathy (HCM) and chronic kidney disease (CKD) with subnephrotic proteinuria who presented to the emergency department with kidney failure and hypervolemia requiring dialysis. A kidney biopsy showed focal segmental and global glomerulosclerosis, extensive foot process effacement, and abnormal mitochondria in podocytes and tubular epithelial cells; the genetic workup identified a rare FASTKD2 exon 2 variant, c.29G>C p.(Ser10Thr), in homozygosity; and functional mitochondrial assays in cultured skin fibroblasts showed reduction in FASTKD2 protein expression and moderate combined impairment in mitochondrial respiratory chain (MRC) assembly and function. This is the first report of a FASTKD2-associated cardiorenal mitochondrial cytopathy, characterized by young adult-onset proteinuric CKD and dilated HCM, in the absence of the severe neurologic manifestations described in patients with biallelic FASTKD2 variants. We hypothesize that the increased production of reactive oxygen species associated with moderate MRC impairment could result in a smoldering podocytopathy with progressive proteinuric CKD, without overt tubulopathy or encephalomyopathy-which might be, instead, pathogenically related to adenosine triphosphate deficiency.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141878212","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Causal Relationship Between Kidney Function and Cancer Risk: A Mendelian Randomization Study.","authors":"Ellen Dobrijevic, Anita van Zwieten, Andrew J Grant, Clement T Loy, Jonathan C Craig, Armando Teixeira-Pinto, Germaine Wong","doi":"10.1053/j.ajkd.2024.05.016","DOIUrl":"10.1053/j.ajkd.2024.05.016","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Rationale & objective: &lt;/strong&gt;Patients treated with kidney replacement therapy experience a 1.5- to 2-fold increased risk of cancer and cancer mortality compared with the general population. Whether this excess risk extends to people with earlier stage chronic kidney disease and whether reduced kidney function is causally related to cancer is unclear.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Study design: &lt;/strong&gt;Two-sample Mendelian randomization (MR).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Setting & participants: &lt;/strong&gt;Genome-wide association study (GWAS) summary statistics for estimated glomerular filtration rate (eGFR) (n=567,460) and urinary albumin-creatine ratio (UACR) (n=127,865) from the CKDGen consortium and cancer outcomes from the UK Biobank (n = 407,329).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Exposure: &lt;/strong&gt;eGFR and UACR.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Outcome: &lt;/strong&gt;Overall cancer incidence, cancer-related mortality and site-specific colorectal, lung, and urinary tract cancer incidence.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Analytical approach: &lt;/strong&gt;Univariable and multivariable MR conducted for all outcomes.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;The mean eGFR and median UACR were 91.4mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; and 9.32mg/g, respectively, in the CKDGen, and 90.4mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; and 9.29mg/g, respectively, in the UK Biobank. There were 98,093 cases of cancer, 15,850 cases of cancer-related death, 6,664 colorectal, 3584 lung, and 3,271 urinary tract cancer cases, respectively. The genetic instruments for eGFR and UACR comprised 34 and 38 variants, respectively. Genetically predicted kidney function (eGFR and UACR) was not associated with overall cancer risk or cancer death. The association between genetically predicted eGFR and UACR and overall cancer incidence had an odds ratio of 0.88 ([95% CI, 0.40-1.97], P=0.8) and 0.90 ([95% CI, 0.78-1.04], P=0.2) respectively, using the inverse-variance weighted method. An adjusted generalized additive model for eGFR and cancer demonstrated evidence of nonlinearity. However, there was no evidence of a causal association between eGFR and cancer in a stratified MR.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Limitations: &lt;/strong&gt;To avoid overlapping samples a smaller GWAS for UACR was used, which reduced the strength of the instrument and may introduce population stratification.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;Our study did not show a causal association between kidney function, overall cancer incidence, and cancer-related death.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Plain-language summary: &lt;/strong&gt;Does reduced kidney function cause cancer? Patients with chronic kidney disease have been shown to have an increased risk of cancer and cancer-related death. However, it is not clear whether kidney disease is causally related to cancer or the association is due to other factors such as immune suppression and inflammation or a result of distortion of the analyses from unidentified variables (confounding). We used large, published genetic studies as well a database including 407,329 people in the United Kingdom in a series of Mendelian rand","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141858749","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cumulative Blood Pressure Load and Incident CKD.","authors":"Hye-Sun Park, Sang Ho Park, Yeseul Seong, Hyo Jeong Kim, Hoon Young Choi, Hyeong Cheon Park, Jong Hyun Jhee","doi":"10.1053/j.ajkd.2024.05.015","DOIUrl":"10.1053/j.ajkd.2024.05.015","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Rationale & objective: &lt;/strong&gt;The association of long-term cumulative blood pressure (BP) loads with the risk of incident chronic kidney disease (CKD) remains a matter of debate. This study investigated this association among healthy Korean adults with normal kidney function.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Study design: &lt;/strong&gt;Prospective cohort study.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Setting & participants: &lt;/strong&gt;We analyzed 5,221 participants without CKD in the Korean Genome and Epidemiology Study. Cumulative systolic and diastolic BP (SBP and DBP) loads were calculated as the ratios of the areas under the curve (AUC) for SBP≥120mm Hg or≥80mm Hg for DBP divided by the AUC for all SBP or DBP measurements during the exposure period. These AUCs were categorized into 4 groups: group 0 (reference), cumulative BP load of 0 and groups 1-3, tertiles of cumulative BP loads.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Outcome: &lt;/strong&gt;Primary end point was incident CKD defined as a composite of an estimated glomerular filtration rate (eGFR) below 60mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; or proteinuria greater than 1+on dipstick examination for at least 2 consecutive measurements≥90 days apart.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Analytical approach: &lt;/strong&gt;Multivariable Cox proportional hazards regression to estimate the independent association of cumulative BP loads with incident CKD.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Higher cumulative SBP and DBP loads were associated with an increased risk of incident CKD (HR, 1.23 [95% CI, 1.12-1.35] for SBP; and HR, 1.14 [95% CI, 1.04-1.26] for DBP loads for each 1.0-unit greater load). Compared with SBP group 0, groups 2 and 3 were associated with 1.94- and 1.89-fold greater risk of incident CKD. Compared with DBP group 0, groups 2 and 3 were associated with 1.42- and 1.54-fold greater risks. These associations of high cumulative BP loads with an increased risk of incident CKD remained consistent even in the subgroups not taking antihypertensive agents or without prior hypertension diagnosis.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Limitations: &lt;/strong&gt;The assessment of CKD outcomes relied on eGFR and spot urine tests.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;These findings highlight the association between high cumulative SBP and DBP loads and the occurrence of CKD, even in individuals with normal BP levels.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Plain-language summary: &lt;/strong&gt;Although hypertension is a major risk factor for chronic kidney disease (CKD), most studies have focused on single-point blood pressure (BP) measurements. To explore the association between long-term cumulative BP load and the development of CKD, 5,221 Korean adults with normal kidney function were included in this study. Cumulative systolic BP and diastolic BP load both exhibited a significant association with an increased risk of incident CKD. Notably, the association of cumulative BP loads with elevated risk of incident CKD was evident also in individuals who were not taking antihypertensive agents or who had no previous history of hypertension. These findings underscore ","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141858750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Burden of CKD-Associated Pruritus and Adverse Clinical Outcomes in Patients Receiving Dialysis: The Stockholm CREAtinine Measurements (SCREAM) Project.","authors":"Anne-Laure Faucon, Catherine M Clase, Helena Rydell, Milica Uhde, Peter Barany, Marie Evans, Juan-Jesús Carrero","doi":"10.1053/j.ajkd.2024.05.013","DOIUrl":"https://doi.org/10.1053/j.ajkd.2024.05.013","url":null,"abstract":"<p><strong>Rationale & objective: </strong>Pruritus is a common but not well-characterized complaint of patients receiving maintenance dialysis. This study sought to quantify the burden of pruritus and its associated adverse health outcomes in this population.</p><p><strong>Study design: </strong>Observational study.</p><p><strong>Setting & participants: </strong>All patients receiving maintenance dialysis in Stockholm, Sweden, during 2005-2021.</p><p><strong>Exposure: </strong>Clinically recognized pruritus, defined using ICD-10 codes or the prescription for anti-pruritus treatments (including UV-therapy).</p><p><strong>Outcomes: </strong>All-cause mortality, severe infection-related hospitalizations (composite of endocarditis, peritoneal dialysis-related peritonitis, hemodialysis/peritoneal dialysis-related catheter infection, sepsis due to Staphylococcus Spp., or skin infection) and incident diagnoses of anxiety/depression and sleep disorders.</p><p><strong>Analytical approach: </strong>Multivariable logistic regression and cause-specific hazards models to analyze factors associated with prevalent and new-onset pruritus, respectively. Multivariable cause-specific hazards models with time-varying exposure to explore the association of prevalent and new-onset pruritus with adverse health outcomes.</p><p><strong>Results: </strong>Among 3281 dialysis patients (median age 64 years, 66% men, 69% on hemodialysis,77% incident dialysis patients), 456 (14%) had pruritus at enrollment. During a median follow-up of 3.3 [IQR: 1.3-9.2] years, 539 (19%) additional patients developed pruritus. Older age, female sex, a lower serum albumin level, and higher C-reactive protein, serum calcium and phosphorus levels were independently associated with pruritus. Compared to patients without pruritus, patients with pruritus were at a higher risk of suffering sleep disorders (adjusted HR: 1.96 [95%CI 1.60-2.39]), developing anxiety/depression (aHR: 1.56 [1.23-1.98]), and being hospitalized for severe infections (aHR: 1.36 [1.18-1.57]), the latter attributed to higher risk of sepsis and peritoneal dialysis-related peritonitis. There was no detectable association between developing pruritus and all-cause mortality.</p><p><strong>Limitations: </strong>Potential misclassification bias if pruritus is not clinically recognized; lack of information on pruritus intensity/severity; use of diagnostic codes for exposure and outcome diagnoses.</p><p><strong>Conclusion: </strong>At least one-third of patients experience pruritus during their first years on dialysis, and pruritus was consistently associated with adverse health outcomes.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141787040","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lupus Nephritis Patterns and Response to Type I Interferon in Patients With DNASE1L3 Mutations: Report of Three Cases.","authors":"Stefano Volpi, Maria L Angelotti, Giulia Palazzini, Giulia Antonelli, Fiammetta Ravaglia, Federica Garibotto, Anna Agrusti, Alice Grossi, Alberto Magnasco, Giovanni M Rossi, Carmela Errichiello, Francesco Peyronel, Elisa Buti, Lorenzo Lodi, Gian M Ghiggeri, Paola Romagnani, Augusto Vaglio","doi":"10.1053/j.ajkd.2024.05.014","DOIUrl":"10.1053/j.ajkd.2024.05.014","url":null,"abstract":"<p><p>DNASE1L3 is an extracellular nuclease that digests chromatin released from apoptotic cells. DNASE1L3 mutations impair the enzyme function, enhance autoantibody production and type I interferon (IFN-I) responses, and cause different autosomal recessive phenotypes ranging from hypocomplementemic urticarial vasculitis syndrome to full-blown systemic lupus erythematosus (SLE). Kidney involvement in patients with DNASE1L3 mutations is poorly characterised. Herein, we describe the clinical course of three children with monogenic SLE due to DNASE1L3 mutations who developed refractory glomerulonephritis leading to kidney failure. They had different renal histopathological patterns (i.e., membranous, endo- and extra-capillary glomerulonephritis and thrombotic microangiopathy), all belonging to the lupus nephritis (LN) spectrum. One patient had a mixed phenotype, showing an overlap between SLE and ANCA-associated vasculitis. Using immunofluorescence, we detected glomerular expression of the IFN I-induced human myxovirus resistance protein 1 (MXA), which was particularly evident in glomerular endothelial cells. 2/3 patients had increased expression of interferon-stimulated genes in the peripheral blood and all three patients had reduced serum DNAse activity. Our findings suggest that DNASE1L3-related glomerulonephritis can be included in the spectrum of IFN I-mediated kidney disorders, and provide the rationale for IFN I-directed therapies in order to improve the poor outcome of this rare condition.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141764749","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}