Carbamylation Is Instrumental in End-Stage Kidney Disease Coagulopathies: The Impact on von Willebrand Factor and Platelet Functionality.

Background:  Chronic kidney disease (CKD) is a progressive, irreversible, and incurable condition characterized by high morbidity and mortality, affecting approximately one-tenth of the global population. Rise of urea-derived cyanate levels in CKD patients, severalfold higher in comparison to those found in healthy individuals, leads to an increased rate of carbamylation of lysine residues of proteins and peptides. This posttranslational modification plays an important role in the progression of kidney failure but also in the onset of CKD-related complications, including previously reported coagulopathies.

Use of corticosteroids in Norwegian patients with immunoglobulin a nephropathy progressing to end-stage kidney disease: a retrospective cohort study.

Background: Despite several clinical trials, the use of corticosteroid therapy for treating immunoglobulin A nephropathy (IgAN) remains controversial. We aimed to describe the use of corticosteroid therapy combined with supportive therapy in Norwegian patients with IgAN who had progressed to end-stage kidney disease.

Methods: We conducted a retrospective cohort study using data from the Norwegian Renal Registry.

Long-term follow-up of IgA nephropathy: clinicopathological features and predictors of outcomes.

Background: The establishment of the Oxford classification and newly developed prediction models have improved the prognostic information for immunoglobulin A nephropathy (IgAN). Considering new treatment options, optimizing prognostic information and improving existing prediction models are favorable.

Methods: We used random forest survival analysis to select possible predictors of end-stage kidney disease among 37 candidate variables in a cohort of 232 patients with biopsy-proven IgAN retrieved from the Norwegian Kidney Biopsy Registry.

Antiglomerular Basement Membrane Disease Possibly Triggered by Undiagnosed Renal Cell Carcinoma: A Case Report.

Antiglomerular basement membrane (anti-GBM) disease is a rare, small-vessel vasculitis that affects the capillary beds of the kidneys and lungs. Although exceedingly rare, several case reports have described anti-GBM disease with a concurrent cancer diagnosis, suggesting a possible correlation between these 2 conditions. Herein, we describe the first known case to our knowledge of a woman in her early 60s with simultaneous anti-GBM disease and clear cell renal cell carcinoma, in which the tumor was thought to have been the substrate for anti-GBM disease.

[IgA nephropathy].

Immunoglobulin A (IgA) nephropathy is the most common form of primary glomerulonephritis globally and a frequent cause of severe kidney failure in young adults. Kidney biopsy is used to diagnose the condition. Despite it being identified more than fifty years ago, understanding of the disease's pathophysiology is limited, and there is currently no targeted treatment apart from supportive therapy.

Validation of two IgA nephropathy risk-prediction tools using a cohort with a long follow-up.

Background: Recently, two immunoglobulin A (IgA) nephropathy-prediction tools were developed that combine clinical and histopathologic parameters. The International IgAN Prediction Tool predicts the risk for 50% declines in the estimated glomerular filtration rate or end-stage kidney disease up to 80 months after diagnosis. The IgA Nephropathy Clinical Decision Support System uses artificial neural networks to estimate the risk for end-stage kidney disease.

Utilizing the MEST score for prognostic staging in IgA nephropathy.

Background: The Oxford classification/MEST score is an established histopathologic scoring system for patients with IgA nephropathy (IgAN). The objective of this study was to derive a prognostic model for IgAN based on the MEST score and histopathologic features.

Methods: A total of 306 patients with biopsy-proven primary IgAN were included.

[A young woman with dyspnoea and proteinuria].

Background: Systemic lupus erythematosus and anti-phospholipid syndrome may cause severe haematological complications despite few other symptoms of disease.

Case Presentation: A previously healthy woman in her late twenties was admitted to hospital with chest pain and dyspnoea. CT of the thorax revealed bilateral pulmonary embolism and urine sampling showed haematuria and proteinuria.

Kidney Failure and Abdominal Discomfort as Initial Signs of Extramedullary Acute Myelogenous Leukemia.

Although rare, acute myelogenous leukemia (AML) can include extramedullary manifestations, sometimes presenting as a solid tumor called a myeloid sarcoma. Myeloid sarcoma can be the cause of the initial presenting complaint before AML diagnosis, or may be detected as a sign of disease-relapse after treatment. Here, we report a case in which the initial presentation included abdominal discomfort and signs of kidney failure.

Severe nephritis as initial sign of Waldenström's macroglobulinemia.

Waldenström's macroglobulinemia (WM), characterized with monoclonal immunoglobulins of type M and lymphoplasmacytic lymphoma, is a rare clonal Bcell disorder. WM usually present as an indolent lymphoma, and renal involvement is, in contrast to multiple myeloma, very rarely seen. We present a patient presenting with severe nephritis and nephrotic range proteinuria of more than 9 g/day as initial manifestations of WM.

Long-term outcome in 145 patients with assumed benign immunoglobulin A nephropathy.

Background: Patients with immunoglobulin A nephropathy (IgAN) who present with mild to moderate proteinuria and normal renal function are assumed to have excellent short-term renal prognosis, but the long-term prognosis is uncertain.

Methods: Patients were selected from the Norwegian Kidney Biopsy Registry based on the following criteria: diagnostic renal biopsy performed in the period 1988-99, with estimated glomerular filtration rate (eGFR) ≥60 mL/min/1.73 m2 and proteinuria <1 g/24 h at the time of biopsy.

Addition of eGFR and Age Improves the Prognostic Absolute Renal Risk-Model in 1,134 Norwegian Patients with IgA Nephropathy.

Background: Predicting outcome in individual patients with IgA nephropathy (IgAN) is difficult but important. For this purpose, the absolute renal risk (ARR) model has been developed in a French cohort to calculate the risk of end-stage renal disease (ESRD) and death. ARR (0-3) is scored in individual IgAN patients based on the presence of proteinuria ≥1 g/24 h, hypertension, and severe histopathological lesions (1 point per risk factor).

Improved prognosis in Norwegian patients with glomerulonephritis associated with anti-neutrophil cytoplasmic antibodies.

Background: Glomerulonephritis associated with anti-neutrophil cytoplasmic antibodies (ANCA) is associated with increased mortality and a high risk of end-stage renal disease (ESRD). Here, we investigated whether the prognosis has improved over the last 25 years.

Methods: Patients were identified in the Norwegian Kidney Biopsy Registry.

Mortality in patients with IgA nephropathy.

Background: Immunoglobulin A nephropathy (IgAN) is the most common glomerulonephritis globally. Few studies have investigated mortality in patients with IgAN compared with the age- and sex-adjusted general population.

Study Design: Cohort study with record linkage between the Norwegian Kidney Biopsy Registry, Norwegian Cause of Death Registry, and Norwegian Renal Registry.