{"title":"Readability, Understandability, and Actionability of Online Cardiovascular Risk Assessment Tools and Patient Educational Material: A Systematic Review.","authors":"Ashraf Roshan, Jason Choo, Cynthia Lim","doi":"10.1159/000528118","DOIUrl":"https://doi.org/10.1159/000528118","url":null,"abstract":"<p><strong>Introduction: </strong>Individuals with kidney diseases have increased risk of cardiovascular disease and death. Online cardiovascular risk assessment tools can educate patients on risks and modifiable factors. Since patients have variable health literacy, we evaluated the readability, understandability, and actionability of publicly available online cardiovascular risk assessment tools.</p><p><strong>Methods: </strong>We systematically searched, reviewed, characterized, and assessed English-language cardiovascular risk assessment tools online for readability (Flesch-Kincaid Grade Level [FKGL] score), understandability, and actionability (Patient Education Materials Assessment Tool for printable materials [PEMAT-P]).</p><p><strong>Results: </strong>After screening 969 websites, 69 websites employing 76 risk tools were included. The most frequently used tools were the Framingham Risk Score (<i>n</i> = 13) and the Atherosclerotic Cardiovascular Disease score (<i>n</i> = 12). Most tools were intended for the general population and estimated the 10-year incident cardiovascular risk. Patient education was provided in the form of targets for blood pressure (<i>n</i> = 17), lipids (<i>n</i> = 15), or glucose (<i>n</i> = 5); and advice regarding diet (<i>n</i> = 18), exercise (<i>n</i> = 19), and smoking cessation (<i>n</i> = 20). The median FKGL, PEMAT understandability, and actionability scores were 6.2 (4.7, 8.5), 84.6% (76.9%, 89.2%), and 60% (40%, 60%), respectively.</p><p><strong>Conclusion: </strong>The online cardiovascular risk tools were generally easy to read and understand, but only a third provided education on risk modification. Judicious selection of an online cardiovascular risk assessment tool may help patients in self-management.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"56-68"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/30/71/gdz-0003-0056.PMC10126735.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9364657","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}
Lanny T DiFranza, Eleas Chafouleas, Swapna Katipally, M Barry Stokes, Satoru Kudose, Miroslav Sekulic
{"title":"Crescentic Fibrillary Glomerulonephritis in the Setting of Immune Checkpoint Inhibitor Therapy: A Report of Two Cases.","authors":"Lanny T DiFranza, Eleas Chafouleas, Swapna Katipally, M Barry Stokes, Satoru Kudose, Miroslav Sekulic","doi":"10.1159/000528881","DOIUrl":"https://doi.org/10.1159/000528881","url":null,"abstract":"<p><strong>Introduction: </strong>Immune checkpoint inhibitor (ICPI) therapy is used to treat various malignancies; however, it can be associated with off-target effects including kidney injury. Acute tubulointerstitial nephritis is the most commonly described renal pathology associated with ICPIs, although less frequently, glomerulopathies may be identified when a kidney biopsy is performed in the work-up of acute kidney injury (AKI).</p><p><strong>Case presentation: </strong>Two patients with small cell carcinoma of the lung were treated with etoposide, carboplatin, and the ICPI atezolizumab. During 2 and 1.5 months of atezolizumab therapy, respectively, patients developed AKI, hematuria, and proteinuria, and kidney biopsies were performed. Both biopsies showed fibrillary glomerulonephritis with focal crescentic features. One patient died 5 days after the kidney biopsy, while the second showed improvement of renal function after discontinuation of atezolizumab and initiation of corticosteroid therapy.</p><p><strong>Discussion: </strong>We describe two cases of fibrillary glomerulonephritis with crescents after administration of atezolizumab. Development of impaired kidney function following initiation of ICPI therapy in both cases raises the possibility that ICPI therapy may potentiate the development of endocapillary proliferation and crescents (i.e., an \"active\" glomerulitis) <i>via</i> immune modulation. Thus, exacerbation of underlying glomerulonephritis should be kept in the differential diagnosis of patients who develop AKI, proteinuria, and hematuria following ICPI therapy.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"69-74"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/eb/b8/gdz-0003-0069.PMC10126733.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9364658","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":"Prostate Cancer Associated with Minimal Change Disease: A Case Report.","authors":"Yuta Nakano, Mariko Yoshida, Naohiro Muraki, Kouhei Sugita, Saori Ishihara, Jiro Kumagai, Hajime Fujisawa","doi":"10.1159/000525040","DOIUrl":"https://doi.org/10.1159/000525040","url":null,"abstract":"<p><strong>Introduction: </strong>Minimal change disease (MCD), a common cause of primary nephrotic syndrome that accounts for 10%-15% of all primary nephrotic syndrome cases in adults, is frequently associated with malignant lymphoma. However, studies on MCD associated with prostate cancer are scarce.</p><p><strong>Case presentation: </strong>A 73-year-old male with prostate cancer was referred to our department with hypoalbuminemia and severe proteinuria while waiting for prostatectomy. We diagnosed the patient with nephrotic syndrome and performed a renal biopsy. Renal pathological findings were consistent with those of MCD. The clinical course suggested an association between prostate cancer and MCD as our patient achieved complete remission of MCD after receiving androgen deprivation and radiation therapy for prostate cancer without the use of glucocorticoids or other immunosuppressants.</p><p><strong>Discussion: </strong>Although MCD can be associated with solid tumors, MCD associated with prostate cancer is very rare. The current case is the first to directly raise the possibility that secondary MCD may develop due to prostate cancer in some patients.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 3","pages":"145-150"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a8/1c/gdz-0002-0145.PMC9710312.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10681234","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}
Ryan Malcolm Hum, Sarah Elyoussfi, Benjamin J Parker, Graeme Reid, Durga A K Kanigicherla
{"title":"A Case of IgG4-Related Disease and Membranous Nephropathy Associated with Thrombospondin Type-1 Domain-Containing 7A.","authors":"Ryan Malcolm Hum, Sarah Elyoussfi, Benjamin J Parker, Graeme Reid, Durga A K Kanigicherla","doi":"10.1159/000524014","DOIUrl":"https://doi.org/10.1159/000524014","url":null,"abstract":"<p><strong>Background: </strong>IgG4-related disease (IgG4-RD) is a systemic multi-organ inflammatory disorder which affects the kidney 20% of the time. Patients with intrinsic IgG4-related kidney disease (IgG4-RKD) often have tubulointerstitial nephritis (TIN) whereas glomerular lesions like membranous nephropathy (MN) are less common. Antibodies to thrombospondin type-1 domain-containing 7A (THSD7A) have been described in primary MN, but never in association with IgG4-RKD.</p><p><strong>Case report: </strong>We report the first case of IgG4-MN associated with THSD7A antibodies in serum and positivity on glomerular staining, in a 57-year-old Caucasian male with IgG4-RD affecting the pancreas, liver, lacrimal glands, extraocular muscles, and kidneys. This patient presented initially with glomerular disease including significant proteinuria consistent with MN. Glomerular staining for THSD7A antigen and serum THSD7A antibody titres was positive. Treatment with corticosteroids and cyclophosphamide successfully induced remission with resolution of proteinuria, and improvement in renal function. However, despite maintenance azathioprine, the patient relapsed 39 months later. On relapse, there was minimal proteinuria but a significant rise in creatinine. Subsequent renal biopsy showed less glomerular disease and instead a TIN pattern. Subsequent treatment with Rituximab and corticosteroids successfully induced remission.</p><p><strong>Conclusion: </strong>The role of THSD7A autoantibodies in MN is emerging, and as both IgG4-MN and presence of THSD7A antibody are rare occurrences in themselves, we speculate that there may be an undiscovered association between THSD7A and IgG4-MN. Routine testing for THSD7A in IgG4-MN may help to identify the link.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 3","pages":"139-144"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ca/3f/gdz-0002-0139.PMC9710322.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10672234","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":"C3 Glomerulopathy: A Review with Emphasis on Ultrastructural Features.","authors":"Jean Hou, Kevin Yi Mi Ren, Mark Haas","doi":"10.1159/000524552","DOIUrl":"https://doi.org/10.1159/000524552","url":null,"abstract":"<p><p>C3 glomerulopathy (C3G) is a rare disease resulting from dysregulation of the alternative complement pathway, resulting in the deposition of complement component 3 (C3) in the kidney. It encompasses two major subgroups: dense deposit disease and C3 glomerulonephritis (C3GN). Although the alternative complement pathway is typically a very tightly controlled system, dysregulation can be a result of genetic mutations in the fluid phase or membrane-bound inhibitors or accelerators. In addition, de novo/acquired autoantibodies against any of the regulatory proteins can alter complement activation either by negating an inhibitor or activating an accelerator. Triggering events can be complex; however, the final pathway is characterized by the uncontrolled deposition of C3 in glomeruli and the formation of the membrane attack complex. Light microscopic findings can be quite heterogeneous with a membranoproliferative pattern most commonly encountered. Diagnostic confirmation of C3G is based on a characteristic pattern of glomerular immunofluorescence staining, with C3-dominant deposits that are at least 2 orders of intensity greater than staining for any immunoglobulin (Ig) or C1q. Electron microscopy is necessary for diagnosing DDD in particular, but can also help to distinguish C3GN from other glomerular disease mimickers.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 3","pages":"107-120"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6f/c7/gdz-0002-0107.PMC9710331.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10681241","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}
Timothy D Cummins, David W Powell, Daniel W Wilkey, Makayla Brady, Fredrick W Benz, Michelle T Barati, Dawn J Caster, Jon B Klein, Michael L Merchant
{"title":"Quantitative Mass Spectrometry Normalization in Urine Biomarker Analysis in Nephrotic Syndrome.","authors":"Timothy D Cummins, David W Powell, Daniel W Wilkey, Makayla Brady, Fredrick W Benz, Michelle T Barati, Dawn J Caster, Jon B Klein, Michael L Merchant","doi":"10.1159/000522217","DOIUrl":"https://doi.org/10.1159/000522217","url":null,"abstract":"<p><p>Chronic kidney disease (CKD) affects 30 million adults, costs ~$79 billion dollars (2016) in Medicare expenditures, and is the ninth leading cause of death in the United States. The disease is silent or undiagnosed in almost half of people with severely reduced kidney function. Urine provides an ideal biofluid that is accessible to high-sensitivity mass spectrometry-based proteomic interrogation and is an indicator of renal homeostasis. While the accurate and precise diagnosis and better disease management of CKD can be aided using urine biomarkers, their discovery in excessive protein or nephrotic urine samples can present challenges. In this work we present a mass spectrometry-based method utilizing multiplex tandem mass tag (TMT) quantification and improved protein quantification using reporter ion normalization to urinary creatinine to analyze urinary proteins from patients with a form of nephrotic syndrome (FSGS). A comparative analysis was performed for urine from patients in remission versus active disease flare. Two-dimensional LC-MS/MS TMT quantitative analysis identified over 1058 urine proteins, 580 proteins with 2 peptides or greater and quantifiable. Normalization of TMT abundance values to creatinine per ml of urine concentrated reduced variability in 2D-TMT-LC-MS/MS experiments. Univariate and multivariate analyses showed that 27 proteins were significantly increased in proteinuric disease flare. Hierarchical heatmap clustering showed that SERPINA1 and ORM1 were >1.5 fold increased in active disease versus remission urine samples. ELISA validation of SERPINA1 and ORM1 abundance agreed with our quantitative TMT proteomics analysis. These findings provide support for the utility of this method for identification of novel diagnostic markers of CKD and identify SERPINA1 and ORM1 as promising candidate diagnostic markers for FSGS.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 3","pages":"121-131"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6d/7e/gdz-0002-0121.PMC9529004.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10656292","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":"Repository Corticotropin in Treating de novo C3 Glomerulonephritis after Transplantation.","authors":"Muhammad Saad Naseer, Ayush Singh, Neeraj Singh","doi":"10.1159/000520387","DOIUrl":"https://doi.org/10.1159/000520387","url":null,"abstract":"<p><strong>Introduction: </strong>De novo C3 glomerulonephritis (C3GN) after transplant is uncommon. Although eculizumab has been used successfully in several cases, the response is heterogeneous, and treatment strategies remain undefined. The use of repository corticotropin in C3GN has not been described in the literature.</p><p><strong>Case report: </strong>A 48-year-old African American male with kidney transplantation secondary to presumed diabetic nephropathy presented 6 years after transplant with lower extremity edema and nephrotic range proteinuria. His urine protein to creatinine ratio (UPCR) was 8.2 g/g. Renal allograft biopsy confirmed the diagnosis of C3GN. He was treated with eculizumab (Solaris®) 900 mg IV once weekly for 4 weeks and repository corticotropin (H.P. Acthar® gel) 80 units SQ twice weekly for 6 months with a near-complete resolution of proteinuria within 3 months of the treatment. The patient presented again 6 months after completing the therapy with a recurrence of proteinuria, which peaked at 11.6 g/g of UPCR. Repeat kidney allograft biopsy was consistent with C3GN. He was started on repository corticotropin 80 units SQ twice weekly, which resulted in a reduction of proteinuria to >50% within 2 months of therapy. When eculizumab 900 mg IV weekly for 4 weeks was added with repository corticotropin, the proteinuria resolved within 10 weeks of treatment. The patient was maintained on monotherapy of repository corticotropin and has been in complete remission of proteinuria for more than a year until his last follow-up.</p><p><strong>Conclusion: </strong>This is the first case report describing the role of repository corticotropin as an effective therapy in reducing proteinuria and maintaining patients with C3GN in proteinuria remission.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 2","pages":"100-105"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/81/cc/gdz-0002-0100.PMC9670034.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10674928","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}
Gabriel B W Lerner, Gary G Singer, Christopher P Larsen, Tiffany N Caza
{"title":"Immunoglobulin-Negative Fibrillary Glomerulonephritis Masked in Diabetic Nephropathy: A Case Report and Discussion of a Diagnostic Pitfall.","authors":"Gabriel B W Lerner, Gary G Singer, Christopher P Larsen, Tiffany N Caza","doi":"10.1159/000520071","DOIUrl":"https://doi.org/10.1159/000520071","url":null,"abstract":"<p><strong>Introduction: </strong>Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with poor prognosis, characterized by deposition of randomly arranged fibrillar material measuring 10-30 nm in diameter. This diagnosis is confirmed with DNAJB9 immunohistochemistry as well as ultrastructural examination. Ultrastructurally, the fibrillary material seen in this entity may be confused with diabetic fibrillosis occurring in diabetic nephropathy.</p><p><strong>Case presentation: </strong>We present a case of a 63-year-old African American male with remote hepatitis C virus (HCV) infection and type II diabetes mellitus who presented with chronic kidney disease and nephrotic range proteinuria. A kidney biopsy revealed PAS-positive mesangial matrix expansion consistent with diabetic nephropathy and focal randomly oriented fibril deposition on ultrastructural examination. Immunofluorescence for immunoglobulin G and light chains was negative by both routine and paraffin immunofluorescence. Immunohistochemistry for DNAJB9 was diffusely positive, confirming co-existing FGN.</p><p><strong>Discussion/conclusion: </strong>Patients with diabetic nephropathy and FGN have similar clinicopathologic presentations with a slowly progressive onset of kidney failure and proteinuria. In diabetic patients with fibrillary deposits under ultrastructural examination, concurrence of these disease entities must be considered. In this patient with remote HCV infection that was successfully treated years before, it is possible that in the absence of an FGN trigger, there was a loss of antigenicity with a loss of immunoglobulin staining. Therefore, we recommend DNAJB9 immunostaining for patients with remote HCV infection to avoid this diagnostic pitfall. Further studies are needed to determine the potential role of HCV infection in the initiation and etiopathogenesis of FGN.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 2","pages":"95-99"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/89/c1/gdz-0002-0095.PMC9670039.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10681229","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}