Pulmonary arterial hypertension populations of special interest: portopulmonary hypertension and pulmonary arterial hypertension associated with congenital heart disease.

Guidelines exist for management of pulmonary arterial hypertension (PAH), but information is limited for certain patient subgroups, including adults with portopulmonary hypertension (PoPH) or with PAH associated with congenital heart disease (PAH-CHD). This article discusses screening, clinical management, and prognosis in PoPH and PAH-CHD and, as such, considers the most recent clinical data and expert advice. A multidisciplinary consultation and follow-up by specialists are crucial for management of both PoPH and PAH-CHD, but each condition presents with unique challenges.

Correction to: A practical algorithmic approach to mature aggressive B cell lymphoma diagnosis in the double/triple hit era: selecting cases, matching clinical benefit.

The first and family names of the authors were interchanged and are now presented correctly. The original article has been corrected.

Pathobiological and Radiological Approach For Hepatocellular Carcinoma Subclassification.

Many advances have been made in the imaging diagnosis and in the histopathological evaluation of HCC. However, the classic imaging and histopathological features of HCC are still inadequate to define patient's prognosis. We aimed to find the link between new proposed morphovascular patterns of hepatocellular carcinoma (HCC) and magnetic resonance imaging (MRI) features to identify pre-operatory markers of biologically aggressive HCC.

A practical algorithmic approach to mature aggressive B cell lymphoma diagnosis in the double/triple hit era: selecting cases, matching clinical benefit : A position paper from the Italian Group of Haematopathology (G.I.E.).

An accurate diagnosis of clinically distinct subgroups of aggressive mature B cell lymphomas is crucial for the choice of proper treatment. Presently, precise recognition of these disorders relies on the combination of morphological, immunophenotypical, and cytogenetic/molecular features. The diagnostic workup in such situations implies the application of costly and time-consuming analyses, which are not always required, since an intensified treatment option is reasonably reserved to fit patients.

Immune responses towards bioengineered tissues and strategies to control them.

Purpose Of Review: Research into development of artificial tissues and bioengineered organs to replace physiological functions of injured counterparts has highlighted a previously underestimated challenge for its clinical translatability: the immune response against biomaterials. Herein, we will provide an update and review current knowledge regarding this important barrier to regenerative medicine.

Recent Findings: Although a clear understanding of the immune reactivity against biomaterials remains elusive, accumulating evidence indicates that innate immune cells, primarily neutrophils and macrophages, play a key role in the initial phases of the immune response.

Factors affecting the number of lymph nodes retrieved after colo-rectal cancer surgery: A prospective single-centre study.

Background: The number of harvested lymph nodes (LNs) in colorectal cancer surgery relates to oncologic radicality and accuracy of staging. In addition, it affects the choice of adjuvant therapy, as well as prognosis. The American Joint Committee on Cancer defines at least 12 LNs harvested as adequate in colorectal cancer resections.

The Evolving Role of Prostate-Specific Membrane Antigen-Based Diagnostics and Therapeutics in Prostate Cancer.

Prostate-specific membrane antigen (PSMA)-based imaging seeks to fill some critical gaps in prostate cancer staging and response assessment, and may select patients for treatment with radiolabeled PSMA conjugates. In biochemical recurrence, at prostate-specific antigen (PSA) levels as low as 0.2 ng/dL, Ga-PSMA imaging has demonstrated a 42% detection rate of occult metastatic disease, and detection has been greater than 95% when PSA levels are higher than 2 ng/dL.

Erythropoietin inhibits SGK1-dependent TH17 induction and TH17-dependent kidney disease.

IL-17-producing CD4+ cells (TH17) are pathogenically linked to autoimmunity including to autoimmune kidney disease. Erythropoietin's (EPO) newly recognized immunoregulatory functions and its predominant intra-renal source suggested that EPO physiologically regulates TH17 differentiation, thereby serving as a barrier to the development of autoimmune kidney disease. Using in vitro studies of human and murine cells and in vivo models, we show that EPO ligation of its receptor (EPO-R) on CD4+ T cells directly inhibits TH17 generation and promotes trans-differentiation of TH17 into IL-17-FOXP3+CD4+ T cells.

Erythropoietin, a multifaceted protein with innate and adaptive immune modulatory activity.

Erythropoietin (EPO) is a glycoprotein produced mainly by the adult kidney in response to hypoxia and is the crucial regulator of red blood cell production. EPO receptors (EPORs), however, are not confined to erythroid cells, but are expressed by many organs including the heart, brain, retina, pancreas, and kidney, where they mediate EPO-induced, erythropoiesis-independent, tissue-protective effects. Some of these tissues also produce and locally release small amounts of EPO in response to organ injury as a mechanism of self-repair.

HCV-Associated Nephropathies in the Era of Direct Acting Antiviral Agents.

Hepatitis C virus (HCV) infection is a systemic disorder that frequently associates with extrahepatic manifestations, including nephropathies. Cryoglobulinemia is a typical extrahepatic manifestation of HCV infection that often involves kidneys with a histological pattern of membranoproliferative glomerulonephritis. Other, less common renal diseases related to HCV infection include membranous nephropathy, focal segmental glomerulosclerosis, IgA nephropathy, fibrillary and immunotactoid glomerulopathy.

Early use of PEPA dialyzer for light chains removal and for the recovery from myeloma cast nephropathy: A case report.

Chemotherapy and extracorporeal treatment reduce serum free light chains (FLCs) allowing the recovery of acute kidney injury (AKI) caused by myeloma cast nephropathy (MCN). We report the first case of recovery from AKI in a patient with MCN who underwent the removal of FLCs using the PEPA filter, with an undisclosed cut-off, combined with chemotherapy for multiple myeloma (MM).

Is gastroparesis a gastric disease?

Background: Gastroparesis is a digestive syndrome characterized by delayed gastric emptying (GE) and by symptoms that are suggestive of gastroduodenal motor disorders. There are three grades of gastroparesis of increasing severity: (a) mild gastroparesis; (b) compensated gastroparesis; and (c) gastric failure. GE abnormalities are partially related to symptom type and severity, and other mechanisms may be involved.

Impact of 3 Major Maintenance Immunosuppressive Protocols on Long-term Clinical Outcomes: Result of a Large Multicenter Italian Cohort Study Including 5635 Renal Transplant Recipients.

Background: Although optimization of immunosuppressive schemes in renal transplantation have minimized acute posttransplant complications, long-term outcomes are still not optimal and most of the chronic graft damage is drug-related. Therefore, to define the best long-term maintenance immunosuppressive regimen is of major importance in renal transplantation. To assess this objective, we undertook a large, multicenter cohort study in Italy.

"Wandering spleen" as a rare cause of recurrent abdominal pain: a systematic review.

Introduction: Wandering spleen (WS) is a rare and generally acquired condition, resulting from abnormal ligamentous laxity failing to fixate the spleen in its normal location in the left upper quadrant, thus leading to its migration to the pelvis due to gravity. Such migration leads to an elongated vascular pedicle, which is prone to torsion causing splenic infarction; thus, a prompt surgical intervention is recommended. Since this adverse event affects childbearing women or children, it is crucial to choose the most appropriate surgical strategy, such as splenectomy or splenopexy, both effective and widely diffused options.

Unilateral pulmonary vein atresia without anomalous connection in adult patient with recurrent severe hemoptysis.

Isolated unilateral pulmonary vein atresia (UPVA) is a rare congenital malformation and the management remains controversial. In adults, pneumonectomy is the treatment of choice when significant hemoptysis becomes life-threatening. We report a case of a 28-year-old male with isolated unilateral right atresia of the pulmonary vein who had life-threatening hemoptysis treated with bronchial arteries embolization followed by successful right pneumonectomy.

Uniportal thoracoscopic resection of intralobar and extralobar pulmonary sequestration.

Pulmonary sequestration (PS) is a rare congenital malformation of the respiratory tract. Two main variants are described, the intralobar and the extralobar PS. Clinical manifestations vary from accidental findings to life threatening complications.

Uniportal video-assisted removal of a right paratracheal pericardial cyst: an unusual location.

Cystic lesions of the pericardium are a rare entity. Generally, they are congenital and located in the cardiophrenic angle. The right paratracheal location is unusual and differential diagnosis in particular with bronchogenic cyst is difficult even when using magnetic resonance imaging (MRI).

Rapid Biolayer Interferometry Measurements of Urinary CXCL9 to Detect Cellular Infiltrates Noninvasively After Kidney Transplantation.

Introduction: Measuring the chemokine CXCL9 in urine by enzyme-linked immunosorbent assay (ELISA) can diagnose acute cellular rejection (ACR) noninvasively after kidney transplantation, but the required 12- to 24-hour turnaround time is not ideal for rapid, clinical decision-making.

Methods: We developed a biolayer interferometry (BLI)-based assay to rapidly measure urinary CXCL9 in <1 hour. We validated this new assay versus standard ELISA in 86 urine samples from kidney transplantation recipients with various diagnoses.

Sodium Prescription in the Prevention of Intradialytic Hypotension: New Insights into an Old Concept.

Background: Sodium prescription in patients with intradialytic hypotension remains a challenge for the attending nephrologist, as it increases dialysate conductivity in hypotension-prone patients, thereby adding to dietary sodium levels.

Methods: New sodium prescription strategies are now available, including the use of a mathematical model to compute the sodium mass to be removed during dialysis as a physiological controller.

Results: This review describes the sodium load of patients with end-stage renal disease on chronic hemodialysis (HD) and discusses 2 strategies to remove excess sodium in patients prone to intradialytic hypotension, namely, Profiled HD and the hemodiafiltration Aequilibrium System.