Cystinuria is an autosomal recessive disorder associated with defective proximal tubular reabsorption of divalent amino acids. It leads to increased cystine, ornithine, lysine, and arginine excretion ...in the urine. Cystine is insoluble in physiological pH, and cystinuria leads to crystalluria and nephrolithiasis. We present a case of acquired cystinuria in a renal transplant recipient, that is, to the best of our knowledge, the first case of acquired cystinuria ever documented in the literature.
In this systematic review and meta-analysis, we explored the utilization of cardiac magnetic resonance imaging (CMR) to detect fibrotic changes secondary to uremic cardiomyopathy during the early ...stages of chronic kidney disease (CKD) and in patients with end-stage kidney disease (ESKD). Uremic myocardial fibrosis can lead to arrhythmia and heart failure, and it is important to detect these changes. CMR offers a noninvasive way to characterize the severity of cardiac remodeling. A comprehensive search of multiple electronic databases was conducted. Studies were divided according to scanner field strength (1.5 or 3 Tesla). The random effects model was used to calculate the pooled mean, 95% confidence interval (CI), standard error, and standardized mean difference (SMD). The I2 statistic was used to assess the heterogeneity between study-specific estimates. The search retrieved 779 studies. From these, 20 studies met the inclusion criteria and had 642 CKD patients (mean age of 56.8 years; 65.2% males; mean estimated glomerular filtration rate (eGFR) of 33 mL/min/1.73 m2) and 658 ESKD patients on dialysis (mean age of 55.6 years; 63.3% males; mean dialysis duration of 3.47 years). CKD patients had an increased left ventricular mass index (LVMi) compared to controls, with an SMD of 0.37 (95% CI: 0.20-0.54; I2 0%; p-value <0.05). ESKD patients also had increased LVMi compared to controls, SMD 0.88 (95% CI: 0.35-1.41; I2 79.1%; p-value 0.001). Myocardial fibrosis assessment using T1 mapping showed elevated values; the SMD of native septal T1 values between CKD and controls was 1.099 (95% CI: 0.73-1.46; I2 33.6%; p-value <0.05), and the SMD of native septal T1 values between ESKD patients and controls was 1.12 (95% CI: 0.85-1.38; I2 33.69%; p-value <0.05). In conclusion, patients with CKD and ESKD with preserved left ventricular ejection fraction (LVEF) have higher LVMi and T1 values, indicating increased mass and fibrosis. T1 mapping can be used for the early detection of cardiomyopathy and as a risk stratification tool. Large, randomized trials are needed to confirm these findings and determine the effect of long-term dialysis on cardiac fibrosis.
COVID-19-Associated C-ANCA Vasculitis Vaitla, Pradeep; Atari, Mohammad; Kapoor, Sanjana ...
Journal of the American Society of Nephrology,
11/2023, Letnik:
34, Številka:
11S
Journal Article
Unique Case of Fibrillary Glomerulonephritis Murari, Ujjwala; Shawwa, Khaled; Simhadri, Prathap ...
Journal of the American Society of Nephrology,
11/2023, Letnik:
34, Številka:
11S
Journal Article
