Haploidentical Hematopoietic Stem Cell Transplant Complicated by Atypical Hemolytic Uremic Syndrome and Kidney Transplant From the Same Donor With No Immunosuppression but C5 Inhibition.

Background: Atypical hemolytic uremic syndrome (aHUS) is life-threatening condition particularly when complicating allograft hematopoietic stem cell transplant (HSCT). In the past, the outcome was very poor with the majority of patients reaching end-stage renal disease or dying with little or no chances of kidney transplant (KTx) due to the high risk of relapse. The availability of C5 inhibition has opened up significant therapeutic opportunities and has improved the outcome particularly if complement dysregulation (CD) is the underlying pathogenetic mechanism.

A simple prognostic index for Shigatoxin-related hemolytic uremic syndrome at onset: data from the ItalKid-HUS network.

Shigatoxin Escherichia coli-related hemolytic uremic syndrome (eHUS) is a severe thrombotic microangiopathy (TMA) burdened by life-threatening complications and long-term sequelae. Since hemoconcentration is associated with worse outcome, we tried to develop a reliable and easy-to-calculate index for predicting complications and sequelae based on hemoglobin (Hb) at presentation. The first laboratory examinations with signs of TMA in eHUS patients were analyzed in relation to the outcomes with the receiver operating characteristic curves and their areas under the curve (AUC) for Hb and creatinine (sCr).

The peritoneal sieving of sodium: a simple and powerful test to rule out the onset of encapsulating peritoneal sclerosis in patients undergoing peritoneal dialysis.

Background And Aims: Encapsulating peritoneal sclerosis (EPS) is an uncommon but severe complication of peritoneal dialysis (PD). A reliable screening tool to identify patients at risk of developing or not EPS is currently not available. We aimed to evaluate whether the reduction in dialysate sodium concentration (sodium sieving) at 60 min (ΔD), during a peritoneal equilibration test with 3.

Low-dose rituximab is poorly effective in patients with primary membranous nephropathy.

Background: The optimal dosing and the efficacy of rituximab for primary membranous nephropathy (PMN) has not been established. This multicentric prospective study evaluates the efficacy and safety of low-dose rituximab (RTX) therapy in patients with PMN in clinical practice.

Methods: Thirty-four consecutive patients with PMN and nephrotic syndrome were included and received RTX (375 mg/m2) once (18 patients) or twice (16 patients).

Safety concerns about intravenous iron therapy in patients with chronic kidney disease.

Anaemia in chronic kidney disease (CKD) is managed primarily with erythropoiesis-stimulating agents (ESAs) and iron therapy. Following concerns around ESA therapy, intravenous (IV) iron is being administered more and more worldwide. However, it is still unclear whether this approach is safe at very high doses or in the presence of very high ferritin levels.

Current Evidence in Haemodiafiltration.

Background: Standard low-flux haemodialysis (HD) is not very efficacious, and patient morbidity and mortality rates are still very high. According to the initial study design, the MPO study reported that high-flux HD (hf-HD) showed a significant 37% relative risk reduction of mortality in patients with serum albumin ≤4 g/dl; online haemodiafiltration (HDF) is considered the most efficient technique of using high-flux membranes, as clearances of small solutes, like urea, are higher than in haemofiltration and clearances of middle solutes, like β2-microglobulin, are higher than in hf-HD.

Summary: Three randomized trials have recently been published analysing the effect of online HDF on mortality.

The impact of recurrence of primary glomerulonephritis on renal allograft outcome.

Twenty-yr patient and death-censored graft survival of 348 kidney transplant recipients with primary glomerulonephritis (GN) and of 696 matched controls were 82.2% in GN patients and 75% in controls (p = 0.037) and 49.

What happens after complete withdrawal of therapy in patients with lupus nephritis.

Objectives: Whether and when is it possible to completely stop immunosuppression in patients with lupus nephritis is still poorly defined.

Methods: An attempt to slowly and progressively eliminate steroids and immunosuppressive drugs was tried in 73 of 161 (45.3%) patients with lupus nephritis who achieved a stable clinical remission defined as normal serum creatinine, proteinuria <0.

TLR-4 and VEGF polymorphisms in chronic periaortitis.

Objective: Chronic periaortitis (CP) is a rare disease that is characterised by fibro-inflammatory tissue surrounding the abdominal aorta and has both non-aneurysmal (idiopathic retroperitoneal fibrosis [IRF]) and aneurysmal forms (inflammatory abdominal aortic aneurysm [IAAA]). We investigated whether toll-like receptor 4 (TLR-4) and vascular endothelial growth factor (VEGF) polymorphisms were associated with susceptibility to, and the clinical features of CP.

Methods: One hundred and two CP patients and 200 healthy controls were molecularly genotyped for TLR-4 gene polymorphism (+896 A/G) (rs4986790), VEGF mutations +936 C/T (rs3025039) and -634 C/G (rs2010963), and an 18 base pair (bp) insertion/deletion (I/D) polymorphism at -2549 of the VEGF promoter region.

The long-term outcome of renal transplantation of IgA nephropathy and the impact of recurrence on graft survival.

Background: Few data are available on allograft survival at 15 years, the impact and the predictors of recurrence of the original disease in renal transplanted patients with IgA nephropathy (IgAN).

Methods: In this retrospective study, we compared the long-term outcome of renal transplant in 190 patients with IgAN with that of 380 non-diabetic controls and evaluated the impact of recurrence of IgAN on the graft outcome.

Results: At 15 years, the patient survival was 88.

The value of (18)F-FDG PET/CT in the assessment of active idiopathic retroperitoneal fibrosis.

Purpose: The different stages in idiopathic retroperitoneal fibrosis (IRF) are generally assessed by assay of inflammatory markers and analysis of contrast-enhanced CT images of the retroperitoneal mass. We investigated the potential role of (18)F-FDG PET/CT in this clinical setting.

Methods: (18)F-FDG uptake was assessed visually and semiquantitatively (using maximum standardized uptake values, SUVmax) in images of the abdominal mass in 22 patients prospectively enrolled from June 2008 to December 2010 who underwent a total of 33 PET/CT studies.