RRAGD-Associated Autosomal Dominant Kidney Hypomagnesemia with Cardiomyopathy: A Review on the Clinical Manifestations and Therapeutic Options.

Background: A hereditary condition primarily affecting the kidneys and heart has newly been identified: the RRAGD-associated autosomal dominant kidney hypomagnesemia with cardiomyopathy (ADKH-RRAGD). This disorder is characterized by renal loss of magnesium and potassium, coupled with varying degrees of cardiac dysfunction. These range from arrhythmias to severe dilated cardiomyopathy, which may require heart transplantation.

Cutaneous targets for topical pain medications in patients with neuropathic pain: individual differential expression of biomarkers supports the need for personalized medicine.

Introduction: Numerous potential cutaneous targets exist for treating chronic pain with topically applied active pharmaceutical ingredients. This preliminary human skin tissue investigation was undertaken to characterize several key biomarkers in keratinocytes and provide proof-of-principle data to support clinical development of topical compounded formulations for peripheral neuropathic pain syndromes, such as postherpetic neuralgia (PHN).

Objectives: The study intended to identify objective biomarkers in PHN skin on a patient-by-patient personalized medicine platform.

Anti-Phospholipase A2 Receptor 1 and Anti-Cysteine Rich Antibodies, Domain Recognition and Rituximab Efficacy in Membranous Nephropathy: A Prospective Cohort Study.

Rationale & Objective: Proteinuria and anti-phospholipase A receptor 1 (anti-PLAR1) antibody titers are associated with primary membranous nephropathy (MN) outcomes. We evaluated the association of antibodies against the cysteine-rich (CysR) and C-type lectin 1, 7, and 8 (CTLD1, CTLD7, and CTLD8) domains of PLAR1 with MN outcomes.

Study Design: Prospective cohort study.

Immunophenotypic Alterations in Adult Patients with Steroid-Dependent and Frequently Relapsing Nephrotic Syndrome.

Immune dysregulation plays a key role in the pathogenesis of steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS). However, in contrast with evidence from the pediatric series, no major B- or T-cell alterations have been described for adults. In these patients, treatment with rituximab allows safe discontinuation of steroids, but long-term efficacy is variable, and some patients experience NS relapses after B cell reconstitution.

Sexual dimorphic response to rituximab treatment: A longitudinal observational study in a large cohort of patients with primary membranous nephropathy and persistent nephrotic syndrome.

Rituximab is one of the first-line therapies for patients with membranous nephropathy (MN) at high risk of progression towards kidney failure. We investigated whether the response to Rituximab was affected by sex and anti-PLAR antibody levels in 204 consecutive patients (148 males and 56 females) with biopsy-proven MN who were referred to the Nephrology Unit of the Azienda Socio Sanitaria Territoriale Papa Giovanni XXIII from March 2001 to October 2016 and managed conservatively for at least 6 months. The primary outcome was a combined endpoint of complete (proteinuria <0.

Validation of Tabletop Microscopes for Microsurgery Simulation and Training.

Purpose: Oral and maxillofacial surgery residency programs are increasingly adopting microsurgery as a core element of training; however, many barriers exist that limit trainees' proficiency. The purpose of this study was to perform a validation of 2 tabletop microscope simulations for their use as a training tool, which could serve as an affordable, alternative method to traditional microsurgery training methods.

Methods: A prospective, single-institution, multidepartmental validation study was performed.

Fistulating diverticular disease masquerading as a peri-anal abscess: a laparoscopic approach to management.

We present a rare case of complicated sigmoid diverticulitis presenting as a peri-anal abscess from an extra-sphincteric fistulous tract. This presentation of a colocutaneous peri-anal abscess is extremely rare, with only a handful of cases described in the literature. Most are managed with an open sigmoid colectomy, however, this case was successfully managed laparoscopically.

Reduced graphene oxide on silicon-based structure as novel broadband photodetector.

Heterojunction photodetector based on reduced graphene oxide (rGO) has been realized using a spin coating technique. The electrical and optical characterization of bare GO and thermally reduced GO thin films deposited on glass substrate has been carried out. Ultraviolet-visible-infrared transmittance measurements of the GO and rGO thin films revealed broad absorption range, while the absorbance analysis evaluates rGO band gap of about 2.

Functional Magnetic Resonance Imaging Versus Kidney Biopsy to Assess Response to Therapy in Nephrotic Syndrome: A Case Report.

In recent years, kidney functional magnetic resonance imaging (MRI) has seen great advances, with several cross-sectional studies demonstrating correlations between MRI biomarkers and glomerular filtration rate. However, the potential of MRI to monitor response to therapy in kidney disease remains undescribed. In this case report, a man in his 40s with drug-resistant membranous nephropathy was addressed to ofatumumab therapy.

Results of the PROPINE randomized controlled study suggest tapering of prednisone treatment for relapses of steroid sensitive nephrotic syndrome is not necessary in children.

Corticosteroid-related toxicity in children with steroid-sensitive nephrotic syndrome is primarily related to the cumulative dose of prednisone. To optimize treatment of relapses, we conducted the PROPINE study, a multicentric, open-label, randomized, superiority trial. Seventy-eight relapsing children aged 3-17 years who had not received steroid-sparing medications during the previous 12 months were randomized to receive, from day five after remission, either 18 doses of 40 mg/m of prednisone on alternate days (short arm), or the same cumulative dose tapered over double the time (long arm).

IgM on the surface of T cells: a novel biomarker of pediatric-onset systemic lupus erythematosus.

Background: Children with systemic lupus erythematosus (SLE) frequently have kidney involvement. Lupus nephritis sometimes presents alone, without systemic SLE features, representing the so-called full-house nephropathy (FHN). Distinguishing patients with SLE or FHN has therapeutic and prognostic implications.

Australasian ACPGBI risk prediction model for 30-day mortality after colorectal cancer surgery.

Background: Postoperative mortality after colorectal cancer surgery varies across hospitals and countries. The aim of this study was to test the Association of Coloproctologists of Great Britain and Ireland (ACPGBI) models as predictors of 30-day mortality in an Australian cohort.

Methods: Data from patients who underwent surgery in six hospitals between 1996 and 2015 (CRC data set) were reviewed to test ACPGBI models, and patients from 79 hospitals in the Bi-National Colorectal Cancer Audit between 2007 and 2016 (BCCA data set) were analysed to validate model performance.

Ofatumumab for multirelapsing membranous nephropathy complicated by rituximab-induced serum-sickness.

Rituximab (375 mg/m) achieved remission of the first episode and six relapses of nephrotic syndrome (NS) in a young male patient with podocyte phospholipase A receptor (PLAR)-related membranous nephropathy (MN) refractory to steroids and cyclosporine. Between-treatments interval averaged 17.4±4.

Detection of Magnetomechanical Effect in Structural Steel Using GMR 2nd Order Gradiometer Based Sensors.

The magneto-mechanical behaviour of structural steel specimens stressed up to the plastic deformation stage was investigated using a 2nd order gradiometer based on Giant Magneto Resistive (GMR) sensors. The correlation between the gradient of the magnetization and the dislocation density before the crack initiation inside the test material was reported. The capability of the GMR scanning sensor to detect the residual magnetization due to the tensile stress with a non-invasive technique was demonstrated.

Fine-Tuning and Optimization of Superconducting Quantum Magnetic Sensors by Thermal Annealing.

In the present article, we present the experimental results concerning the fine-tuning and optimization of superconducting quantum interference device (SQUID) parameters by thermal annealing. This treatment allows for the modification of the parameters in order to meet a specific application or to adjust the device parameters to prevent the increase of magnetic field noise and work instability conditions due to a different critical current with respect to the design value. In particular, we report the sensor critical current, the voltage-flux (V-Φ) characteristics and the spectral density of the magnetic field of SQUID magnetometers for different annealing temperatures.

Effects of Sevelamer Carbonate in Patients With CKD and Proteinuria: The ANSWER Randomized Trial.

Rationale & Objective: Hyperphosphatemia is associated with increased risk for chronic kidney disease (CKD) progression and reduced antiproteinuric effects of renin-angiotensin system (RAS) blockers. We investigated whether the phosphate binder sevelamer carbonate may enhance the antiproteinuric effect of RAS inhibitors in patients with CKD.

Study Design: Phase 2, randomized, controlled, open-label, crossover trial.

Effects of valsartan, benazepril and their combination in overt nephropathy of type 2 diabetes: A prospective, randomized, controlled trial.

Aims: To evaluate whether angiotensin-converting enzyme (ACE) inhibitor and angiotensin II receptor blocker (ARB) combination therapy is more nephroprotective than ACE inhibitor or ARB monotherapy in people with type 2 diabetes and overt nephropathy.

Materials And Methods: In this prospective, randomized, open, blind-endpoint phase III trial sponsored by the Italian Drug Agency, 103 consenting patients with type 2 diabetes, aged >40 years, with serum creatinine levels 159 to 309 μmol/L, spot morning urinary albumin-creatinine ratio > 1000 mg/g (or > 500 mg/g in those on ACE inhibitor or ARB therapy at inclusion) were stratified by centre and randomized to 4.5-year treatment with valsartan 320 mg/d (n = 36), benazepril 20 mg/d (n = 34) or halved doses of both medications (n = 33).

Surgical outcome after standard abdominoperineal resection: A 15-year cohort study from a single cancer centre.

Background: Abdominoperineal resection (APR) is associated with a poorer oncological outcome than anterior resection. This may be due to higher rates of intra-operative perforation and circumferential resection margin involvement. The aim of this study was to audit our short and long-term results of abdominoperineal resection performed using conventional techniques and to compare this with other published series.

Gastrothorax: A case of mistaken identity.

Introduction: Acute wrap failure post fundoplication is a rare but recognized complication and can be due to patient factors, disease factors and surgical factors. Herniation of the stomach into the thorax can mimic a pneumothorax clinically and radiologically and thus lead to bad outcomes for patients.

Presentation Of Case: We report the case of a 20-year-old male who presented to the emergency department with progressively worsening upper abdominal pain, nausea and vomiting followed by acute onset dyspnoea, six days post a laparoscopic repair of a small hiatus hernia and a Nissen fundoplication.

The Role of Angiotensin II in Parietal Epithelial Cell Proliferation and Crescent Formation in Glomerular Diseases.

Crescentic glomerulonephritis (GN) is a devastating disease with rapidly progressive deterioration in kidney function, which, histologically, manifests as crescent formation in most glomeruli. We previously found that crescents derive from the aberrant proliferation and migration of parietal epithelial cells (PECs)/progenitor cells, and that the angiotensin (ang) II/ang II type-1 (AT) receptor pathway may participate, together with the stromal cell-derived factor-1 (SDF-1)/C-X-C chemokine receptor 4 axis, in the development of those lesions. Herein, we elucidated sequential events and cellular and molecular interactions occurring during crescentic lesion onset and evolution.

Safety of Rituximab Compared with Steroids and Cyclophosphamide for Idiopathic Membranous Nephropathy.

Guidelines recommend steroid plus cyclical cyclophosphamide (St-Cp) therapy for patients with idiopathic membranous nephropathy at high risk of progression to ESRD. Rituximab (Rtx) may be a safer alternative. In this retrospective, observational cohort study, we compared time to any adverse event (primary outcome); serious or nonserious events; partial and complete remission of the nephrotic syndrome; and a composite of doubling of serum creatinine, ESRD, or death between 100 Rtx-treated patients and 103 patients who received daily St-Cp We monitored patients with standardized protocols and adjusted for baseline characteristics by Cox regression.

Minimal Change Disease.

Minimal change disease (MCD) is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion. In adults, it accounts for approximately 15% of patients with idiopathic NS, reaching a much higher percentage at younger ages, up to 70%-90% in children >1 year of age. In the pediatric setting, a renal biopsy is usually not performed if presentation is typical and the patient responds to therapy with oral prednisone at conventional doses.