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Archive: Disease Week

Disease Week: Alport Syndrome

Monday In 1927, Dr. Cecil Alport published a series on "hereditary familial congenital haemorrhagic nephritis" where he described its association with deafness and the gender differences in disease severity. https://www.ncbi.nlm.nih.gov/pubmed/20773074          Interestingly, Dr Alport initially believed the etiology of the disorder was an individual susceptibility to a toxin of an unknown organism, probably belonging to the streptococcal group. https://www.ncbi.nlm.nih.gov/pubmed/20773074   The pathogenesis of the disorder known as Alport syndrome remained unknown until early 1970's, when advances in electron microscopy allowed the identification of characteristic abnormalities in GBMs. https://www.ncbi.nlm.nih.gov/pubmed/4343992   Dr Curtis Atkin, who suffered from Alport syndrome...

Disease Week: Zebrafish Model Organism

Monday Introduction into zebrafish as a model organism Did you know? We have a state-of-the-art zebrafish facility here at Arkana Laboratories where we perform basic research with the goal of finding novel diagnostic and therapies for kidney diseases. https://www.arkanalabs.com/research/zebrafish/   Zebrafish (Danio rerio) is a small tropical fish native to South Asia that can be purchased from your local pet shop. Indeed, the first laboratory strains were derived from several pet shops in Oregon.   Traditionally, it has been a popular vertebrate model to study developmental biology due to its rapid development outside of the uterus and transparent embryos. https://dev.biologists.org/content/123/1/461...

Disease Week: Infection-Associated Glomerulonephritis and Endocarditis-Associated Glomerulonephritis

Monday Renal disease related to infective endocarditis was first reported over 100 years ago. However, the initial literature describing nephritis associated with infective endocarditis relied primarily on autopsy-based studies from the pre- and early post-antibiotic era.  Reviews from recent decades note the evolution in renal complications of infectious diseases. Demographics have changed from younger to older patients. The frequency of comorbidities including diabetes has increased.  Recent decades have seen a change in the infectious agents that cause renal disease, from primarily Streptococcal to a broader array of organisms compared to the past, with predominance of Staphylococci. The historical division into...

Disease Week: Onco-Nephrology

Monday Onco-nephrology is a rapidly developing area of nephrology designed to help address and prevent kidney problems and the many complex issues that arise in cancer patients. AKI is a common complication in cancer patients. The highest 1-year risk of AKI was seen in patients with kidney cancer (44%), liver cancer (33%), and multiple myeloma (31.8%) in one large population-based cohort study. Patients with distant metastases were at highest risk. https://www.ncbi.nlm.nih.gov/pubmed/21767759 What is the most common glomerular pathology reported in patients with solid tumors? Minimal change disease Membranoproliferative glomerulonephritis Membranous nephropathy Thrombotic microangiopathy   Answer: Membranous nephropathy.  The incidence of cancer was significantly higher in patients with...

Disease Week: Monoclonal Gammopathy of Renal Significance

Disease Week: Monoclonal Gammopathy of Renal Significance   Summary: Renal disease caused by parenchymal deposition of circulating monoclonal immunoglobulins (mIg) is a well established phenomenon. Light chain cast nephropathy, light chain deposition disease and AL amyloidosis are probably the best known entities; nevertheless, the list also includes a number of less common and recently described entities, some of which are still poorly understood.  These disorders may be seen in the setting of either a B-cell or plasma cell proliferative disorder with production of a mIg. When these proliferative disorders represent an overtly malignant disease (e.g. multiple myeloma, Waldenström macroglobulinemia, high-grade...

Disease Week: Lupus Nephritis

Monday:  Introduction to Systemic Lupus Erythematosus and Clinical Diagnostic Criteria Answer:  C.  50%  50% of adults with SLE will develop lupus nephritis.   In children with SLE, 80% have nephritis. References: Madhok R.  Systemic lupus erythematosus: lupus nephritis.  BMJ Clinical Evidence 2015 Dec 18; pp 1123. https://www.ncbi.nlm.nih.gov/pubmed/26683208 Liu CC, Kao AH, Manzi S, Ahearn JM.  Biomarkers in systemic lupus erythematosus: challenges and prospects for the future.  Therapeutic Advances in Musculoskeletal Disease 2013; 5 (4): 210-233. https://www.ncbi.nlm.nih.gov/pubmed/23904865 In a majority of cases, renal involvement in SLE often occurs in the first year following diagnosis, but can occur at any time in the disease course. ...

NIH Lupus Nephritis Activity and Chronicity Indices

NIH Lupus Nephritis Activity and Chronicity Indices: A way to monitor response to treatment and disease progression. A semi-quantitative grading system of pathologic features on kidney biopsies allows for monitoring response to treatment and showing disease progression. Lupus nephritis disease activity can be assessed on a renal biopsy using the modified NIH activity and chronicity indices. Indicators of disease activity include endocapillary hypercellularity, neutrophils or karyorrhexis within glomerular capillary loops, fibrinoid necrosis, hyaline deposits, cellular or fibrocellular crescents, and interstitial inflammation. Crescents and fibrinoid necrosis are weighted twice as they have a worse impact on prognosis. The scoring is based...

International Society of Nephrology / Renal Pathology Society Classification of Lupus Nephritis

How do we classify lupus nephritis on a renal biopsy?  SLE patients with an active urinary sediment or proteinuria may undergo a kidney biopsy to evaluate for nephritis.  The International Society of Nephrology (ISN) and Renal Pathology Society (RPS) created a lupus nephritis classification system based on morphologic findings on kidney biopsies.  A simplified view of the classification algorithm is based on the sites of immune deposits within glomeruli.  A flow-chart demonstrating that algorithm is shown below.   Mesangial immune deposits can be present in all classes. A summary in tabular format is shown below.   While a majority of SLE patients...

Introduction to Systemic Lupus Erythematosus and Clinical Diagnostic Criteria

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which affects over 5 million people worldwide, and manifests as tissue damage within multiple organ systems.  It has a complex etiology, involving a combination of multiple genetic and environmental factors.  Women are disproportionately affected, with a 9:1 female-to-male ratio. The diagnostic criteria are determined by the American College of Rheumatology (ACR) and the European League against Rheumatism (EULAR).  In the 1997 revision of the 1982 ACR diagnostic criteria for diagnosis of SLE, 4 of 11 disease manifestations are required (Hochberg, 1997).  These are shown below: Notice that “renal disorder” is one...

Disease Week: C3 Glomerulonephritis

Monday Rediscovery of Complement Mesangial C3 deposition only within the glomerulus has been reported since 1980. Only recognized recently as a distinct entity when CFHR5 nephropathy was reported. CFHR5 mutation is speculated to represent a founder mutation which can be traced back to the Troodos mountains of Cyprus. https://www.ncbi.nlm.nih.gov/pubmed/21566112   1 in 6500 people in Cyrus carry a mutation in CFHR5 (duplication of exons 2 and 3) and inheritance is autosomal dominant. C3 and C4 complement levels are normal. 80% of males with CFHR5 nephropathy progress to ESRD while only 20% of females progress to ESRD. https://www.ncbi.nlm.nih.gov/pubmed/22065842 CFHR5 nephropathy is...