How histopathological diagnosis interacts with kidney ultrasound parameters and glomerular filtration rate.

The evaluation of estimated GFR (eGFR) is a pivotal staging step in patients with chronic kidney disease (CKD), and renal ultrasound plays an important role in diagnosis, prognosis and progression of CKD. The interaction between histopathological diagnosis and ultrasound parameters in eGFR determination has not been fully investigated yet. The study examined the results of native kidney biopsies performed in 48 Italian centers between 2012 and 2020.

Cyclosporin-induced hypertension is associated with the up-regulation of Na+-K+-2Cl- cotransporter (NKCC2).

Background: The use of cyclosporin A (CsA) is hampered by the development of nephrotoxicity including hypertension, which is partially dependent on renal sodium retention. To address this issue, we have investigated in vivo sodium reabsorption in different nephron segments of CsA-treated rats through micropuncture study coupled to expression analyses of sodium transporters. To translate the findings in rats to human, kidney-transplanted patients having CsA treatment were enrolled in the study.

Next-Generation Sequencing (NGS) Analysis Illustrates the Phenotypic Variability of Collagen Type IV Nephropathies.

Mutations in cause a spectrum of glomerular disorders, including thin basement membrane nephropathy (TBMN) and Alport syndrome (AS). The wide application of next-generation sequencing (NGS) in the last few years has revealed that mutations in these genes are not limited to these clinical entities. In this study, 176 individuals with a clinical diagnosis of inherited kidney disorders underwent an NGS-based analysis to address the underlying cause; those who changed or perfected the clinical diagnosis after molecular analysis were selected.

[Methods for calculating the number of nephrons from ultrasound scans and kidney biopsies for nephrologists' use].

The interest in determining the number of nephrons in the kidney dates back to the 1960s, when an influential laboratory method for determining ex vivo the number of nephrons in the kidneys was described by Bricker. Over the years, various methods have been developed to estimate the number of nephrons in living beings as accurately as possible. These modern methods combine data such as the glomerular density, the percentage of glomeruli in sclerosis calculated from biopsy samples, and the kidney volume, which can be precisely estimated from magnetic resonance, CT scan, or specific ultrasound methods.

LIAISON Calprotectin for the prediction of relapse in quiescent ulcerative colitis: The EuReCa study.

Introduction: Fecal calprotectin (FC) is established as a diagnostic marker to differentiate between inflammatory bowel diseases and non-inflammatory conditions. Furthermore, it may be effective in monitoring response to treatment, and to predict relapse during maintenance therapy.

Design: This was a prospective longitudinal study carried out in Italy, France and Spain.

A case series of adult patients affected by EAST/SeSAME syndrome suggests more severe disease in subjects bearing truncating mutations.

EAST/SeSAME syndrome is a rare disease affecting the Central Nervous System (CNS), inner ear, and kidney. The syndrome is due to loss-of-function mutations in the gene encoding the inward-rectifying potassium channel Kir4.1.

Nephroplex: a kidney-focused NGS panel highlights the challenges of PKD1 sequencing and identifies a founder BBS4 mutation.

Background: Genetic testing of patients with inherited kidney diseases has emerged as a tool of clinical utility by improving the patients' diagnosis, prognosis, surveillance and therapy.

Methods: The present study applied a Next Generation Sequencing (NGS)-based panel, named NephroPlex, testing 115 genes causing renal diseases, to 119 individuals, including 107 probands and 12 relatives. Thirty-five (poly)cystic and 72 non (poly)cystic individuals were enrolled.

Skin Architecture, Kidney Transplantation, and Their Relationship to Basal and Squamous Cell Carcinomas.

Background/aim: Squamous cell carcinoma (SCC) is highly prevalent in kidney transplant patients (KT). It is characterized by the presence of an inflammatory infiltrate. In this study, we examined the presence of similar infiltrates in intact skin, which could be regarded as a precancerous step.

[Bardoxolone: a new potential therapeutic agent in the treatment of autosomal dominant polycystic kidney disease?].

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of chronic renal failure. The natural history of ADPKD is characterized by development of multiple bilateral renal cysts that progressively destroy the architecture of the parenchyma and lead to an enlargement in the total kidney volume (TKV) and to the decline of the renal function. Cyst growth activates the immune system response causing interstitial inflammation and fibrosis that contribute to disease progression.

The number of nephrons in different glomerular diseases.

Background: The total number of nephrons has been measured mainly from post-mortem studies and only in selected populations. Data from living subjects are scanty, and direct comparisons among different glomerular diseases are lacking. The present work exploits modern methodology to estimate the total nephron number in glomerulopathies with prevalent proteinuria/nephrotic syndrome versus glomerulopathies with nephritic syndrome (IgA nephropathy (IgAN), lupus nephritis), thus extending previous observations about the number and function of glomeruli in different physiological and pathological states.

[DFPP in myasthenia gravis: case report].

Introduction: Myasthenia Gravis (MG) is a neuromuscular disease due to a decrease in the number of acetylcholine receptors (AChR) present at the level of the neuromuscular junction. It is characterized by weakness and muscle fatigue. The pathogenesis of MG would seem to be autoimmune (autoantibodies against AChR, Musk, Titin).

Rituximab in steroid-dependent or frequently relapsing idiopathic nephrotic syndrome.

The outcome of steroid-dependent or frequently relapsing nephrotic syndrome of minimal change disease (MCD), mesangial proliferative GN (MesGN), or FSGS may be poor and with major treatment toxicity. This academic, multicenter, off-on trial (ClinicalTrials.gov #NCT00981838) primarily evaluated the effects of rituximab therapy followed by immunosuppression withdrawal on disease recurrence in 10 children and 20 adults with MCD/MesGN (n=22) or FSGS who had suffered ≥2 recurrences over the previous year and were in steroid-induced remission for ≥1 month.

A new recombinant MnSOD prevents the cyclosporine A-induced renal impairment.

Background: Cyclosporine A (CsA) is one of the most frequently used anticalcineurinic drugs for preventing graft rejection and autoimmune disease. Its use is hampered by nephrotoxic effects, namely an impairment of the glomerular filtration rate (GFR) and hypertension. Evidence suggests that reactive oxygen species (ROS) play a causal role in the nephrotoxicity.

A case of valproic acid-induced acute pancreatitis in tuberous sclerosis coexisting with end-stage renal disease.

Tuberous sclerosis complex (TCS) is a genetic disorder with a variable clinical presentation. It is commonly characterized by seizures, mental retardation and cutaneous angiofibromas. Renal manifestations frequently include angiomyolipomas and cysts which lead to chronic kidney disease.

Hyperhomocysteinemia in chronic renal failure: alternative therapeutic strategies.

Chronic renal failure and uremia represent states wherein high blood levels of homocysteine, a cardiovascular risk factor, are largely resistant to folate therapy. Indeed, normalization of homocysteine levels through vitamin administration is rarely achieved in this population, and this fact could explain, among other causes, the negative results of intervention trials designed to lower cardiovascular risk. Dialysis itself lowers homocysteine levels, albeit transitorily.

The nature of water: Greek thought from Homer to Acusilaos.

Greek philosophy finds its roots in the myth of Homer's and Hesiod's poems and especially in Orphism which introduced the concept of a soul separated from the body with an independent principle, psiche (soul), to be rewarded or punished after death. Orphism was an important step in Greek culture. It introduced the divine into man, the soul which does not die with the body and reincarnates.

[Survival is not enough: improving quality of life].

We need a new health care system that is based on patients' needs. The present cadre of health managers who acquired power by cutting expenditures must be removed from office. We need to educate a new cadre of health managers who are 1) convinced that safeguarding health does not use up the resources of the next generations; 2) capable of switching the system from curative to preventive medicine; and 3) able to reinforce clinical research.

To survive is not enough. Quality of life in CKD--the need for a new generation of health-oriented economists.

CKD is utilized as a paradigm, a chronic disease which allows decades of life conquered with great effort through a machine, a life with many losses and many dependencies. We must understand the patient's needs, which are not related to availability of drugs and machines and hospitals. We cannot provide good medical care with the limited amount of national product devoted to health care.

In vivo effect of the natural antioxidant hydroxytyrosol on cyclosporine nephrotoxicity in rats.

Background: Cyclosporine A (CsA) is the first-line immunosuppressant used in transplant patients and in auto- immune diseases. Nephrotoxicity is the major limitation of CsA use. Although the mechanisms of nephrotoxicity have not been completely defined, some evidence suggests that reactive oxygen species (ROS) play a causal role.

Plasma protein homocysteinylation in uremia.

Protein homocysteinylation is proposed as one of the mechanisms of homocysteine toxicity. It occurs through various means, such as the post-biosynthetic acylation of free amino groups (protein-N-homocysteinylation, mediated by homocysteine thiolactone) and the formation of a covalent -S-S- bond found primarily with cysteine residues (protein-S-homocysteinylation). Both protein modifications are a cause of protein functional derangements.

Acid-base transport in Henle's loop: the effects of reduced renal mass and diabetes.

The loop of Henle (LOH) is an important site of renal acidification. Using the in vivo microperfusion technique of LOH combined with quantitative polymerase chain reaction (PCR) performed on isolated thick ascending limbs (TAL), we demonstrated that the Na + -H + exchanger is the main transport mechanism involved, although a small, but significant contribution from the H+-ATPase also occurs. Among the various Na+-H+ exchanger isoforms we have evidenced that NHE3 is expressed and functionally active along the TAL.

Creatinine clearance and hemoglobin concentration before and after heart transplantation.

Clinical studies indicate that indices of glomerular filtration rate (GFR) as serum creatinine or creatinine clearance can predict the risk of death in congestive heart failure (CHF) and in heart transplantation. The study reports data on creatinine clearance before and after heart transplantation in 160 patients followed-up for 5 years at our Unit. Pre-transplant creatinine clearance averaged 83.

The kidney in heart failure.

Renal dysfunction is a constant feature of congestive heart failure and is a stronger predictor of mortality than left ventricular ejection fraction or New York Heart Association classification. In heart failure, a reduction of glomerular filtration rate and renal plasma flow occurs, although the filtration fraction increases. There are many reason for this pattern.

Level of hydration and renal function in healthy humans.

Background: High hydration is commonly used in renal studies to improve the completeness of urine collection. The renal effects of hydration are not well defined.

Methods: Renal function was studied under fasting conditions (baseline) and after a meat meal (2 g of protein/kg body weight) in 12 healthy adults on a low and high hydration regimen of 0.

Nephrotic edema.

This article starts with a concise synopsis of the history of edema. The role of underfilling, overflow, antidiuretic hormone, and acquaporins is subsequently discussed. Emphasis is given to the use of diuretics in edematous patients.