ERSD

Objective: This study explores the effects of Angiotensin-Converting Enzyme Inhibitors (ACEI) or Angiotensin Receptor Blockers (ARB) on the incidence of end-stage renal disease (ESRD) in diabetic nephropathy (DN) patients. Methods: Literatures were searched in PubMed, Embase, Medline, CENTRAL, and CNKI databases. These literatures included a randomized controlled trial to evaluate the efficacy of ACEI and ARB among patients with DN. The endpoint event included the occurrence of ERSD...

Dysfunction of cellular homeostasis can lead to misfolding of proteins thus acquiring conformations prone to polymerization into pathological aggregates. This process is associated with several disorders, including neurodegenerative diseases, such as Parkinson's disease (PD), and endoplasmic reticulum storage disorders (ERSDs), like alpha-1-antitrypsin deficiency (AATD) and hereditary hypofibrinogenemia with hepatic storage (HHHS). Given the shared pathophysiological mechanisms involved in such conditions, it is necessary to deepen our understanding of the basic principles of misfolding and aggregation akin to these diseases which, although heterogeneous in symptomatology, present similarities that could lead to potential mutual treatments...

Previous systematic reviews elucidate the efficacy of Tai Chi on the rehabilitation and treatment for various chronic diseases. Yet, no consensus has been reached on its efficacy and safety from those with chronic kidney disease (CKD). Therefore, we conducted a systematic review to critically summarize what is already known about the prevailing benefits of Tai Chi for CKD patients. There was no evidence that Tai Chi had adverse effects on CKD patients. Long-term Tai Chi exercises could improve quality of life, cardiorespiratory fitness, and physical motor function for the end-stage renal disease (ERSD) patients undergoing dialysis...

In this article, we review the biological and clinical implication of the Recruitment-Secretory Block ("R-SB") phenomenon. The phenomenon refers to the reaction of the liver with regard to protein secretion in conditions of clinical stimulation. Our basic knowledge of the process is due to the experimental work in animal models. Under basal conditions, the protein synthesis is mainly carried out by periportal (zone 1) hepatocytes that are considered the "professional" synthesizing protein cells. Under stimulation, midlobular and centrolobular (zones 2 and 3) hepatocytes, are progressively recruited according to lobular gradients and contribute to the increase of synthesis and secretion...

Alpha-1-antitrypsin (AAT) and fibrinogen are secretory acute phase reactant proteins. Circulating AAT and fibrinogen are synthesized exclusively in the liver. Mutations in the encoding genes result in conformational abnormalities of the two molecules that aggregate within the rough endoplasmic reticulum (RER) instead of being regularly exported. That results in AAT-deficiency (AATD) and in hereditary hypofibrinogenemia with hepatic storage (HHHS). The association of plasma deficiency and liver storage identifies a new group of pathologies: endoplasmic reticulum storage disease (ERSD)...

The revolutionary evolution in science and technology over the last few decades has made it possible to face more adequately three main challenges of modern medicine: changes in old diseases, the appearance of new diseases, and diseases that are unknown (mostly genetic), despite research efforts. In this paper we review the road travelled by pathologists in search of a method based upon the use of routine instruments and techniques which once were available for research only. The application to tissue studies of techniques from immunology, molecular biology, and genetics has allowed dynamic interpretations of biological phenomena with special regard to gene regulation and expression...

OBJECTIVES: End-stage renal disease (ESRD) is one of the most critical risk factors of intracerebral hemorrhage (ICH). We aimed to investigate the effects of maintenance hemodialysis on hematoma volume, edema volume, and prognosis in patients with comorbid ESRD and ICH. MATERIALS AND METHODS: Patients with comorbid ESRD and ICH were divided into two groups based on whether receiving maintenance hemodialysis. Hematoma and perihemorrhagic edema (PHE) volumes and relative edema ratio after admission were assessed on head computed tomography scans...

Pathological calcification is a major cause of cardiovascular morbidities primarily in population with chronic kidney disease (CKD), end stage renal diseases (ERSD) and metabolic disorders. Investigators have accepted the fact that vascular calcification is not a passive process but a highly complex, cell mediated, active process in patients with cardiovascular disease (CVD) resulting from, metabolic insults of bone fragility, diabetes, hypertension, dyslipidemia and atherosclerosis. Over the years, studies have revealed various mechanisms of vascular calcification like induction of bone formation, apoptosis, alteration in Ca-P balance and loss of inhibition...

Individuals harboring the loss-of-function (LOF) proprotein convertase subtilisin/kexin type 9 Gln152His variation (PCSK9Q152H) have low circulating low-density lipoprotein cholesterol levels and are therefore protected against cardiovascular disease (CVD). This uncleavable form of proPCSK9, however, is retained in the endoplasmic reticulum (ER) of liver hepatocytes, where it would be expected to contribute to ER storage disease (ERSD), a heritable condition known to cause systemic ER stress and liver injury...

BACKGROUND: T-cell immunity is affected in end stage renal disease (ESRD). However, whether this happens at pre- or post-dialysis stage and what is the impact of different renal replacement methods, remains unclear. We investigated the alterations of T-cell subtypes in patients at pre-dialysis ESRD and their further changes during dialysis. METHODS: CD4+, CD8+, CD4 + CD28null and CD8 + CD28null T-cells were analysed in 40 ESRD patients at two different time points, (a) the day started on dialysis (ESRD-T0) and (b) 6 months later (ESRD-T6), while being on haemodialysis (HD) or continuous ambulatory peritoneal dialysis (CAPD)...

BACKGROUND: WT1 mutations cause a wide spectrum of renal and extrarenal manifestations concerning urogenital development and the development of tumors. METHODS: We retrospectively collected the information on the genotype and phenotype of WT1 nephropathy from the multicenter registry since 2014 to 2019. All patients were stratified by renal function decline status or by sequence timing. Rapid progressive group was defined as rapidly developing into ERSD within 12 months since disease onset...

BACKGROUND: A number of inborn errors of metabolism caused by abnormal protein trafficking that lead to endoplasmic reticulum storage diseases (ERSD) have been defined in the last two decades. One such disorder involves biallelic mutations in the gene encoding endoplasmic reticulum resident co-chaperone DNAJC3 (P58IPK ) that leads to diabetes in the second decade of life, in addition to multiple endocrine dysfunction and nervous system involvement. OBJECTIVE: The aim of this study was to define the natural history of this new form of diabetes, especially the course of abnormalities related to glucose metabolism...

[i]Clostridioides difficile[/i] infection (CDI) is a leading cause of a healthcare-associated diarrhea worldwide. Recently, an increased number of new cases and growing mortality due to CDI have been observed. Patients suffering from end-stage renal disease (ESRD) are most exposed to CDI. It has been proven that CDI in patients receiving renal replacement therapy (RRT) significantly increases mortality, prolongs hospitalization and increases the cost of treatment. Important risk factors of CDI in ERSD patients include hospitalization or stay in an intensive care unit in the last 90 days, HIV infection, bacteremia, prolonged antibiotic therapy and hypoalbuminemia...

Patients with end-stage renal disease (ESRD) are notably accompanied by cognitive disorder and anxiety or depressive symptom. We aimed to explore the linkages of the amygdala-based MR parameters, cognitive and mood performance, systematic inflammation and gut microbiota in ESRD. This prospective study enrolled 28 ESRD patients (13 males and 15 females, mean age of 43.9 ± 13.8 years) and 19 age- and sex-matched healthy control (HC) (12 males and 7 females, mean age of 44.1 ± 10.0 years). All subjects underwent cognitive assessment, inflammatory factor and stool microbiota analysis, and brain MRI analysis [amygdala-based functional connectivity and voxel-based morphometry (VBM)]...

<b/> Background : To analyze long-term outcomes and possible influencing factors in patients with endstage renal disease (ESRD) and critical limb ischemia (CLI) after major amputation compared to patients with normal renal function and non-dialysis-dependent chronic kidney disease. Patients and methods : Abstraction of single-center medical records of patients undergoing above knee (AKA) and below knee (BKA) amputation over a 10 years period (n = 436; 2009-2018). Excluded were amputations due to trauma or tumor...

Aim The advanced glycation end products (AGEs) are among the mechanisms responsible for the pathogenesis and development of chronic kidney disease. The aim of this study was to estimate the dietary AGE intake and to assess its correlation with hematological and biochemical markers of patients with end-stage renal disease (ESRD) undergoing hemodialysis. Materials and methods For this study, a structured questionnaire of the exogenous AGEs was developed, whose reliability and validity were evaluated in the pilot phase of the study including 50 participants...

It is unclear how the brain network changed after kidney transplantation (KT). We explored the patterns of large-scale complex network after KT in end-stage renal disease (ESRD) patients with resting-state functional MRI (rs-fMRI) and diffusion tensor imaging (DTI). Twenty-one ESRD patients (14 men; mean age, 31.5 ± 9.9 years) scheduled for KT and 17 age- and gender-matched healthy controls (HC) (8 men; mean age, 28.9 ± 7.2 years) were enrolled in this study. Each participant underwent rs-fMRI and DTI scans in three time points (pre-KT, 1 and 6 months after KT [for ESRD])...

Background: This study aimed to investigate whether hemodialysis (HD) affects tissue factor (TF), tissue factor pathway inhibitor (TFPI), and polymorphonuclear elastase (PMNE) in endstage renal disease (ESRD) patients when eliminating the effects of heparin. Also, to explore the interaction of TF, TFPI, and PMNE throughout a single HD session. Methods: We enrolled 57 ESRD patients who had undergone hemodialysis for >3 months as an experimental group. Plasma levels of TF, TFPI and PMNE were measured by ELISA in 24 ERSD patients on intermittent HD using low-molecular-weight heparin (LMWH) as anticoagulation (LMWH group) and 33 ESRD patients using citrate as anticoagulation (citrate group) at the start and at 1, 2 and 5 h of the HD session...

OBJECTIVE: The present study investigated the prevalence of anemia, depression, and duration of hemodialysis among ERSD patients and its relation with quality of sleep. METHOD: A total of 70 ESRD patients undergoing hemodialysis in referral Hospital in Bogor, Indonesia participated in the study. PSQI questionnaire and BDI II questionnaire were used to measure the quality of sleep and status of depression, respectively. The medical record was used to record the value of hemoglobin, and the demographic questionnaire was used to determine the duration of hemodialysis...

AIMS/INTRODUCTION: Patients with type 2 diabetes mellitus have an increased hip fracture risk. We investigated the relationship between hip fracture and all-cause death in patients with type 2 diabetes in comparison with cardiovascular disease (CVD) or end-stage renal disease (ERSD). MATERIALS AND METHODS: In total, 4,923 Japanese participants with type 2 diabetes (mean age 65 years, 2,790 men, 2,133 women) were followed for a median of 5.3 years (follow-up rate 99...