Journal of nephropathology
Volume:13 Issue: 3, May 2024

The current narrative review study aims to provide an overview of thrombotic microangiopathy (TMA) in immunoglobulin A nephropathy (IgAN), with a particular emphasis on its pathophysiology, histopathology, and treatment options. The prevalence and clinical significance of TMA in IgAN may vary across different populations. Estimates suggest that TMA events occur in 2-50% of patients with IgAN. Endothelial injury is a key factor in TMA development in IgAN, triggered by immune complex deposition, complement activation, and potentially hypertension. TMA in IgAN correlates with vascular lesions, including arterial intimal sclerosis, arteriolar lumen reduction, and smooth muscle hypertrophy. Notably, patients with TMA show more intense deposition of C4, C3d, and C5b-9 complements. Treatment involves blood pressure management, immunosuppression, and targeted therapies such as eculizumab.

Food intake during hemodialysis (HD) is a controversial issue. The potential benefits include improvement of nutritional status and patient satisfaction. However, the risks include the possibility of intradialytic hypotension (IDH) and dialysis inadequacy. There are no guidelines regarding food intake during HD.

To assess the impact of food intake during HD on IDH and dialysis adequacy.

This was a single-center quasi-experimental study. Thirty patients undergoing regular maintenance HD were recruited for the study. The patients themselves served as their controls. In three separate sessions, they were assessed for IDH and dialysis adequacy (spKt/V, URR). The first session was without a meal, the second with a small meal, and the third with a large meal. Change in measured variables (spKt/V, URR) was assessed by repeated-measures analysis of variance (ANOVA). The McNemar test was conducted to compare the incidence of IDH between three different dialysis sessions.

Nine patients (30%) had IDH when they consumed a small meal (P=0.02, McNemar test), and eight patients had IDH (26.7%) when they consumed a large meal (P=0.03, McNemar test). The mean spKt/v and URR were not significantly different in the three sessions.

There is a significantly increased risk of IDH due to food intake. IDH is associated with significant morbidity and mortality; hence, restricting food intake during HD sessions would be prudent.

Diabetes mellitus (DM) is a metabolic disorder appearing as a main public health problem nowadays.

This study aimed to evaluate the effect of calcitriol on microalbuminuria in patients with type 2 DM (T2DM).

This double-blind randomized clinical trial was performed on 38 patients with T2DM who had micro- albuminuria. These patients were randomly classified into two groups of treatment and control. The treatment group received calcitriol 0.25 μg daily since the control group received a placebo. Duration of treatment was three months. In baseline, serum creatinine (Cr), fasting blood sugar (FBS), glycated hemoglobin (HbA1c), cholesterol (Chol), triglyceride (TG), low-density lipoprotein (LDL-c), high-density lipoprotein (HDL-c), and micro-albuminuria were measured. Patients were followed up for three months. P<0.05 was set as a significant level.

In baseline, two groups did not differ significantly in terms of serum Cr, FBS, HbA1c, Chol, TG, HDL-c, LDL-c, and micro-albuminuria (P> 0.05). After the intervention, there was no significant difference between the two groups regarding the median of serum Cr, FBS, HbA1c, Chol, TG, LDL-c, HDL-c, and microalbuminuria. The median of microalbuminuria in the treatment and control groups was decreased at 46 mg/g and 11 mg/g, respectively. The difference in median of micro-albuminuria was not statically significant between the two groups; however, a significant difference was detected in the treatment group before and after the intervention (P=0.03).

Administration of calcitriol could reduce micro-albuminuria after three months. Therefore, the addition of calcitriol to angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) in patients with T2DM and micro- albuminuria may have a beneficial effect on reducing their proteinuria.

 Hypomagnesaemia secondary to the use of proton-pump inhibitor (PPI) is associated with the reduction of blood pressure.

 To determine the effect of PPI and ranitidine on the reduction of serum magnesium level and blood pressure in chronic hemodialysis patients with hypotension.

 In this double-blind randomized clinical trial, 44 hemodialysis patients who met the requirements entered the study. First, blood sample was taken from each of the patients and their serum magnesium level as well as their blood pressure was checked and recorded. Then, the patients in the intervention group received daily doses of ranitidine placebo (150 mg) and pantoprazole (40 mg) and those in the control group received daily doses of pantoprazole placebo (40 mg) and ranitidine (150 mg) for three months. After the intervention, blood samples were taken again in order to assess the patients’ serum magnesium level. The obtained data were fed into SPSS Software and analyzed.

 The mean age of the patients was 60.14±12.98 years. Moreover, 63.6% of the total patients were female. In the group of patients who had received pantoprazole, diastolic pressure reduced significantly at the end of the study as compared to the beginning of the study. Moreover, in the patients receiving pantoprazole indicated a significant reduction of magnesium at the end of the study as compared to the beginning of the study.

 In this study, a significant relationship was also observed between the use of PPI and hypomagnesemia in hemodialysis patients.
Trial registration: The trial protocol was approved in the Iranian Registry of Clinical Trials (identifier: IRCT20150808023559N19; https://en.irct.ir/trial/42478, ethical code# IR.ARUMS. REC.1398.295).

Endoscopic procedures in urology are safe although simple and impossible. We report a rare catastrophic complication of cystolitholapaxy in a patient with severe urethral stenosis. Despite laparotomy one day after cystolitholapaxy because of peritonitis and loop colostomy, the patient died. Hence, the principles of prevention and extra-care and time especially in the abnormal urethra are emphasized.

Given the pressing need for new medications with minimal adverse effects to address uncontrolled hypertension, this manuscript explores the potential of Zilebesiran as a crucial therapeutic agent. Zilebesiran is an experimental RNA interference drug that shows promise in effectively treating high blood pressure (BP) by decreasing the production of angiotensinogen, a key factor in high BP. It does this by targeting the levels of liver angiotensinogen messenger RNA (mRNA). In a study, a single injection of Zilebesiran demonstrated a noteworthy reduction in BP in individuals with mild-to-moderate hypertension, with sustained effects observed for up to 6 months. Those administered with Zilebesiran were more likely to achieve a 24-hour mean systolic BP of less than 130 mm Hg compared to the control group. The sustained reduction in BP implies that Zilebesiran holds the potential for maintaining consistent BP control, enhancing treatment adherence due to infrequent dosing, and improving outcomes for individuals with hypertension. However, it is important to note that the safety and efficacy of Zilebesiran have yet to be evaluated by regulatory bodies such as the U.S. Food and Drug Administration, the European Medicines Agency, or other health authorities. Ongoing research, exemplified by the KARDIA-2 trial, aims to further assess the efficacy and safety of Zilebesiran as a concomitant therapy for adults with hypertension not adequately controlled by standard treatments.

 Monoclonal gammopathy of renal significance (MGRS) disorders are indolent B-cell or plasma cell lymphoproliferative neoplasms which do not meet the hematological criteria for malignancy, however they cause renal dysfunction as a result of production of nephrotoxic monoclonal immunoglobulin (MIg).

 To study the clinical presentation, laboratory features, light microscopy and immunofluorescence (IF) characteristics of all cases of MGRS diagnosed at our hospital over a period of five years.

 A record of all renal biopsies performed at our hospital between 2014-2019 was accessed from the database. Out of 1356 kidney biopsies, 68 had evidence of MIg deposition on immunofluorescence. Only six cases met the criteria of MGRS. Histopathological and immunofluorescence characteristics were studied to classify the lesions as per International Kidney and Monoclonal Gammopathy (IKMG) Research Group classification.

 All six cases presented with deranged renal function. Four had sub-nephrotic and one had nephrotic range proteinuria. MIg was identified in only one case on serum protein electrophoresis and free light chain assay. Using a conjunction of histomorphology of renal lesions, special stains and immunofluorescence all six cases of MGRS were categorized as per IKMG classification into monoclonal immunoglobulin deposition disease (two cases), AL amyloidosis, light chain cast nephropathy, proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) and light chain proximal tubulopathy (LCPT).

 MGRS presents as renal failure and proteinuria. MIg may not be detected on protein electrophoresis due to low-secretion in serum. A kidney biopsy is essential to study the morphology of renal lesions and identify MIg deposition.

Primary hyperoxaluria is a rare congenital autosomal recessive disorder disrupting the glyoxylate metabolism pathway in the liver. Type1 primary hyperoxaluria is caused by a deficiency in a specific liver enzyme namely, alanine glyoxylate-aminotransferase which catalyzes the conversion of glyoxylate to glycine. By the absence of this enzyme, glyoxylate is converted to oxalate and high oxalate level causes deposition of insoluble calcium oxalate crystals in different organs specifically kidneys. The disease usually manifested by recurrent nephrolithiasis and/or nephrocalcinosis leads to renal failure. This report describes an end-stage renal disease case of a 36-year-old Iranian woman without any history of nephrolithiasis who underwent kidney transplantation. She developed an early onset transplant kidney failure. The patient underwent kidney biopsy, which revealed oxalate nephropathy, accordingly the genetic study confirmed diagnosis of primary hyperoxaluria. This rare case shows how type 1 primary hyperoxaluria can develop after kidney transplantation without having any manifestation prior to transplantation