Real-life experience with inotersen at CEPARM, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro.

Background:  Hereditary transthyretin amyloidosis (ATTRv) is an inherited, progressive, and fatal disease still largely underdiagnosed. Mutations in the () gene cause the TTR protein to destabilize, misfold, aggregate, and deposit in body tissues, which makes ATTRv a disease with heterogeneous clinical phenotype.

Objective:  To describe the long-term efficacy and safety of inotersen therapy in patients with ATTRv peripheral neuropathy (ATTRv-PN).

Patellar and quadriceps tendon rupture are associated with hip fracture in hemodialysis patients with severe hyperparathyroidism.

Spontaneous rupture of the patellar (PTR) and quadriceps (QTR) tendon is infrequent. Systemic diseases such as diabetes mellitus, CKD, and secondary hyperparathyroidism (SHPT) are risk factors. The present cohort study aimed to evaluate risk factors associated with tendon rupture in hemodialysis (HD) patients with SHPT, as well as outcomes including surgical complications, re-ruptures, and fracture.

Chronic hypoparathyroidism is associated with increased cortical bone density evaluated using high-resolution peripheral quantitative computed tomography.

Purpose: This cross-sectional study aimed to assess bone mineral density (BMD), bone microarchitecture and fracture prevalence in women with chronic postsurgical hypoparathyroidism (hypoPT).

Methods: Twenty-seven women with postsurgical hypoPT and 44 age-matched healthy women were included. Dual-energy X-ray absorptiometry was used to evaluate areal BMD and vertebral fracture assessment.

Rapamycin treatment induces tubular proteinuria: role of megalin-mediated protein reabsorption.

Rapamycin is an immunosuppressor that acts by inhibiting the serine/threonine kinase mechanistic target of rapamycin complex 1. Therapeutic use of rapamycin is limited by its adverse effects. Proteinuria is an important marker of kidney damage and a risk factor for kidney diseases progression and has been reported in patients and animal models treated with rapamycin.

Inhibition of SGLT2 co-transporter by dapagliflozin ameliorates tubular proteinuria and tubule-interstitial injury at the early stage of diabetic kidney disease.

Diabetic kidney disease (DKD) is characterized by progressive impairment of kidney function. It has been postulated that tubule-interstitial injury, associated with tubular albuminuria, precedes glomerular damage in the early stage of DKD. Here, we wanted to determine if the development of tubule-interstitial injury at the early stage of DKD implies modulation of megalin-mediated protein reabsorption in proximal tubule epithelial cells (PTECs) by SGLT2-dependent high glucose influx.

Subclinical Acute Kidney Injury in COVID-19: Possible Mechanisms and Future Perspectives.

Since the outbreak of COVID-19 disease, a bidirectional interaction between kidney disease and the progression of COVID-19 has been demonstrated. Kidney disease is an independent risk factor for mortality of patients with COVID-19 as well as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection leading to the development of acute kidney injury (AKI) and chronic kidney disease (CKD) in patients with COVID-19. However, the detection of kidney damage in patients with COVID-19 may not occur until an advanced stage based on the current clinical blood and urinary examinations.

SARS-CoV-2 spike protein inhibits megalin-mediated albumin endocytosis in proximal tubule epithelial cells.

Patients with COVID-19 have high prevalence of albuminuria which is used as a marker of progression of renal disease and is associated with severe COVID-19. We hypothesized that SARS-CoV-2 spike protein (S protein) could modulate albumin handling in proximal tubule epithelial cells (PTECs) and, consequently contribute to the albuminuria observed in patients with COVID-19. In this context, the possible effect of S protein on albumin endocytosis in PTECs was investigated.

Assessment of trabecular and cortical parameters using high-resolution peripheral quantitative computed tomography, histomorphometry and microCT of iliac crest bone core in hemodialysis patients.

Patients with end-stage renal disease develop changes in bone quality and quantity, which can be assessed using different methods. This study aimed to compare and to correlate bone parameters obtained using high-resolution peripheral quantitative computed tomography (HR-pQCT) with those obtained by bone biopsy using histomorphometry and microcomputed tomography (microCT) analysis of the iliac crest core, and to evaluate if HR-pQCT is helpful in aiding with categorization of those with high turnover. Twenty hemodialysis patients, 13 females (7 postmenopausal), underwent bone biopsy from 2018 to 2020.

Contribution to the Knowledge of Rocky Plant Communities of the Southwest Iberian Peninsula.

The rocky habitats of southern Portugal are ecosystems with extreme xericity conditions, associated with special abiotic strains. In these unstable ecological conditions, a considerable diversity of plant communities occurs. The objective of this study, carried out in the Algarve and Monchique, and the Mariánica Range biogeographical sectors, is to compare chasmo-chomophytic communities of the southwestern Iberian Peninsula, using a phytosociological approach (Braun-Blanquet methodology) and numerical analysis (hierarchical cluster analysis).

High ankle-brachial index predicts cardiovascular events and mortality in hemodialysis patients with severe secondary hyperparathyroidism.

Introduction: Vascular calcification related to severe secondary hyperparathyroidism (SHPT) is an important cause of cardiovascular and bone complications, leading to high morbidity and mortality in patients with chronic kidney disease (CKD) undergoing hemodialysis (HD). The present study aimed to analyze whether ankle-brachial index (ABI), a non-invasive diagnostic tool, is able to predict cardiovascular outcomes in this population.

Methods: We selected 88 adult patients on HD for at least 6 months, with serum iPTH>1,000pg/mL.

Aminotransferase-to-platelet ratio index and Fibrosis-4 index score predict hepatic fibrosis evaluated by transient hepatic elastography in hepatitis C virus-infected hemodialysis patients.

Objective: This study aimed to evaluate the performance of aminotransferase-to-platelet ratio index (APRI) and Fibrosis-4 index (FIB-4) in chronic kidney disease stage 5D HCV-infected patients compared to transient hepatic elastography (TE) as the gold standard.

Methods: Hemodialysis HCV-infected patients submitted to TE (FibroScan, Echosens, Paris, France) had APRI and FIB-4 calculated. Based on the best area under receiver operating characteristic curve (AUROC) for significant fibrosis and cirrhosis, APRI and FIB-4 cutoffs were determined and their performances were compared.

HR-pQCT detects alterations in bone microstructure in men with CKD stages 3 and 4, which are influenced by hormonal changes and body composition .

Introduction: Factors associated with osteodystrophy in predialysis patients are poorly understood. In the present study, we attempted to evaluate the impact of body composition and hormonal regulatory factors on the bone microstructure in a group of men with chronic kidney disease (CKD) stages 3 and 4.

Materials And Methods: 46 men, aged 50 - 75 years, with previously unrecognized CKD were evaluated by high-resolution peripheral quantitative computed tomography (HR-pQCT), and dual-energy X-ray absorptiometry (DXA).

Beneficial effects of intradialytic cardiopulmonary rehabilitation.

Introduction: Patients on hemodialysis (HD) present high mortality from cardiovascular complications and high morbidity, including decreasing functional capacity and quality of life.

Objective: To analyze clinical and laboratory responses of patients in HD to intradialytic cardiopulmonary rehabilitation on an outpatient basis.

Methods: We evaluated 14 patients in a prospective study for 8 months using cardiopulmonary rehabilitation protocol (CRehab) consisted of intradialytic aerobic exercise with a cycle ergometer.

Tubular dysfunction in renal transplant patients using sirolimus or tacrolimus.

Background: Tubular dysfunction is prevalent among kidney transplant patients using calcineurin inhibitors, but our knowledge of the tubular effects of mTOR inhibitors is more limited.

Methods: 60 kidney transplant outpatients using either the calcineurin inhibitor tacrolimus or the mTOR inhibitor sirolimus were investigated for renal tubular dysfunction. Proximal tubule function was assessed by quantification of albumin and β2-microglobulin, tubular reabsorption of phosphate and fractional excretion of bicarbonate.

Osteoporosis management in patient with renal function impairment.

Aging is associated with decreases in bone quality and in glomerular filtration. Consequently, osteoporosis and chronic kidney disease (CKD) are common comorbid conditions in the elderly, and often coexist. Biochemical abnormalities in the homeostasis of calcium and phosphorus begin early in CKD, leading to an increase in fracture risk and cardiovascular complications since early stages of the disease.

Bone Density Is Directly Associated With Glomerular Filtration and Metabolic Acidosis but Do Not Predict Fragility Fractures in Men With Moderate Chronic Kidney Disease.

Hyperparathyroidism, vitamin D deficiency, increased fibroblast growth factor-23 (FGF-23), and metabolic acidosis promote bone fragility in chronic kidney disease (CKD). Although useful in predicting fracture risk in the general population, the role of dual-energy X-ray absorptiometry (DXA) in CKD remains uncertain. This cross-sectional study included 51 men aged 50-75 yr with moderate CKD.

Renal tubular dysfunction in patients with primary Sjögren syndrome.

Primary Sjögren's syndrome (pSS) is an important cause of renal tubular dysfunction in adults, mainly due to acquired type 1 distal renal tubular acidosis (RTA 1) and concentration defects (CD). This cross-sectional study evaluated renal tubular function of patients with pSS, by detecting proximal tubular injury (through measurements of urinary β2 microglobulin and albumin), RTA 1 (through an acidification protocol using furosemide and fludrocortisone), and CD (through water deprivation test, WDT). A total of 25 patients with pSS were evaluated and despite a preserved renal function (eGFR 92.

Is it possible to use modification of diet in renal disease (MDRD) equation in a Brazilian population?

Background: Accurate assessment of kidney function level is the key to the identification and management of chronic kidney disease (CKD). Glomerular filtration rate (GFR) is the best measure of overall kidney function in health and disease. There is no consensus about the method to be used routinely to measure and/or estimate GFR.

Bone disease in patients with chronic kidney disease under conservative management.

Context And Objective: Few studies have focused on bone disease in patients with chronic kidney disease under conservative treatment. The objective was to evaluate bone disease in patients with chronic kidney disease.

Design And Setting: Case series, at the Nephrology Division, Hospital Universitário Pedro Ernesto.