IgA vasculitis nephritis-outcomes in adult-onset disease.

Objectives: IgA vasculitis (IgAV) in adults has been relatively under-investigated. Since outcomes are worse in other forms of vasculitis with increasing age, we investigated the outcomes of IgAV comparing younger adults (18-34), middle aged adults (35-64) and elderly patients (≥64 years) focusing on kidney outcomes.

Methods: We identified patients with renal biopsy confirmed IgAV nephritis and collected data regarding clinical features and progression to end stage kidney disease (ESKD).

Rituximab versus the modified Ponticelli regimen in the treatment of primary membranous nephropathy: a Health Economic Model.

Background: Membranous nephropathy is among the most common causes of nephrotic syndrome worldwide, with a high healthcare burden. Treatment using the modified Ponticelli regimen (mPR) has remained the standard of care for decades, but newer therapies such as rituximab offer promising results with reduced side effects. The cost of this treatment, however, is perceived as a barrier to widespread use, especially in resource limited healthcare systems.

Randomized Trial of C5a Receptor Inhibitor Avacopan in ANCA-Associated Vasculitis.

Alternative C activation is involved in the pathogenesis of ANCA-associated vasculitis. However, glucocorticoids used as treatment contribute to the morbidity and mortality of vasculitis. We determined whether avacopan (CCX168), an orally administered, selective C5a receptor inhibitor, could replace oral glucocorticoids without compromising efficacy.

Long-term outcomes of persistent disease and relapse in primary membranous nephropathy.

Background: Primary membranous nephropathy is associated with variable clinical course ranging from spontaneous remission to slow progression to end stage renal failure. Achieving remission confers better renal survival in primary membranous nephropathy (PMN). Longer term outcomes such as patient survival and relapse of active disease remain poorly understood.

Urinary protein selectivity in nephrotic syndrome and pregnancy: resurrection of a biomarker when renal biopsy is contraindicated.

Significant proteinuria in pregnancy can indicate the presence of serious conditions requiring investigation and treatment. The nephrotic syndrome in pregnancy presents a multitude of difficulties and is a relative contraindication of renal biopsy, particularly in the third trimester. We present a case of nephrotic syndrome of unknown cause presenting at 33 weeks of pregnancy.

Successful outcome of pregnancy in patients with anti-neutrophil cytoplasm antibody-associated small vessel vasculitis.

Pregnancy in patients with anti-neutrophil cytoplasm antibody-associated vasculitis is reportedly associated with a high risk of fetal and maternal complications. Here we describe the outcome of pregnancies in patients with granulomatosis with polyangiitis and microscopic polyangiitis at five centers in the United Kingdom using a retrospective case review of all women who became pregnant following diagnosis. We report 15 pregnancies in 13 women resulting in 15 live births including one twin pregnancy and 13 singleton pregnancies.

Rituximab in combination with corticosteroids for the treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis: a NICE single technology appraisal.

As part of its single technology appraisal (STA) process, the National Institute for Health and Care Excellence (NICE) invited the manufacturer of rituximab (Roche Products) to submit evidence of the clinical and cost effectiveness of rituximab in combination with corticosteroids for treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The School of Health and Related Research Technology Appraisal Group at the University of Sheffield was commissioned to act as the independent Evidence Review Group (ERG). The ERG produced a critical review of the evidence for the clinical and cost effectiveness of the technology, based upon the manufacturer's submission to NICE.

The effects of selected Australian snake venoms on tumour-associated microvascular endothelial cells (TAMECs) in vitro.

The effects of various viperid and elapid venoms on the cellular biology of tumour-associated microvascular endothelial cells (TAMECs) were determined in the current study using cells isolated from a rat mammary adenocarcinoma. Previous studies to determine the effects of snake venoms on endothelial cells in vitro have in the main been performed on either human umbilical vein endothelial cells (HUVECs), bovine aortic endothelial cells (BAECs) or endothelial cell lines. These cell populations are accessible and easy to maintain in culture, however, it is well established that endothelial cells display vast heterogeneity depending upon the local microenvironment of the tissue from which they are isolated.

Anti-PLA2R antibodies measured by ELISA predict long-term outcome in a prevalent population of patients with idiopathic membranous nephropathy.

Antibodies to the phospholipase A2 receptor 1 (PLA2R1) have been reported in 70% of cases of idiopathic membranous nephropathy (IMN). The genetic susceptibility of IMN has been accounted for by HLA DQA1 and PLA2R1 genes. Here we retrospectively quantified PLA2R antibodies by ELISA, and genotyped DQ alleles and PLA2R1 single-nucleotide polymorphisms for association with clinical criteria for disease activity at the time of first sample and with outcome over a median total follow-up of 90 months.

Outpatient management of Gitelman's syndrome in pregnancy.

Gitelman's syndrome is a congenital renal tubular defect which affects the apical membrane of the distal convoluted tubule of the renal system. The syndrome is characterised by hypokalaemia, hypomagnesaemia, metabolic alkalosis and hypocalcuria. There are only a few cases describing the impact of Gitelman's syndrome on pregnancy and the foetus.

Maternal deaths in women with lupus nephritis: a review of published evidence.

Background And Objectives: Pregnancies in women with systemic lupus erythematosus (SLE) and lupus nephritis are considered high-risk due to high rates of maternal and fetal complications. However, there has not been a formal analysis addressing the issue of maternal deaths in these women. The aim of this study was to perform a literature review of the maternal deaths in women with SLE and lupus nephritis to: (1) identify the main causes of death and (2) discuss possible reasons for these causes, and strategies that may improve patient care and outcomes.

The challenges of dialysis in systemic sclerosis: between the devil and the deep blue sea?

We present the case of a patient with systemic sclerosis (SSc) and end stage renal disease (ESRD) who experienced complications of both peritoneal and haemodialysis. We review previously reported outcomes of patients with systemic sclerosis on dialysis and discuss potential shared mechanisms in both the disease pathogenesis and dialysis-related complications, particularly with regards to encapsulating peritoneal sclerosis (EPS).

Factors associated with major infections in patients with granulomatosis with polyangiitis and systemic lupus erythematosus treated for deep organ involvement.

This study is an audit and a comparison of major infective complications in patients with granulomatosis with polyangiitis (GPA) and systemic lupus erythematosis (SLE). Data were collected on consecutive patients attending a single treatment approach, multidisciplinary vasculitis centre who met diagnostic criteria for GPA and SLE from 01/01/2006 to 30/06/2006. Immunosuppressive treatment is used in this clinic with guidelines targeting avoidance of neutropenia.

Idiopathic or iatrogenic membranous glomerulonephritis? A case of spironolactone-induced membranous glomerulonephritis.

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in adults in the UK. In most cases, the aetiology remains unknown, although recent data suggested a clear mechanism of pathogenesis. In approximately a quarter of cases, however, a presumed cause is found, such as systemic lupus nephritis, malignancy, hepatitis B and various drugs.

Proteolytic activity of Elapid and Viperid Snake venoms and its implication to digestion.

Testing whether venoms may aid in digestion of the prey, eleven snake venoms were compared for the presence of proteases and endopeptidases that function in alkaline pH conditions. In vitro experiments examined the relative protease and endopeptidase activity of the venoms, which involved combining bovine muscle and snake venom in a buffered solution, encased within dialysis tubing. This mixture was then incubated at room temperature (∼20°C) for 24hr, with constant shaking.

Predictive value of myocardial and coronary imaging in the long-term outcome of potential renal transplant recipients.

Background: Coronary artery disease is a major cause of morbidity and mortality in renal transplant recipients, but there is no agreed screening protocol. The value of myocardial perfusion imaging (MPI) and coronary angiography (CA) in predicting future cardiovascular events and mortality in unselected dialysis patients was studied.

Methods: Forty seven patients (mean age 51±14 years, 37 males), underwent both CA and MPI as part of pre-renal transplant assessment between 1995 and 1999.

Proteinuria as a predictor of renal functional outcome after revascularization in atherosclerotic renovascular disease (ARVD).

Background: Renal revascularization is performed in 16% of newly diagnosed patients with atherosclerotic renovascular disease (ARVD). Although there may be some improvement in hypertension control as a result of intervention, renal functional outcomes are known to vary. Pre-existing renal parenchymal injury, as manifested by proteinuria, is associated with poor functional outcome in conservatively managed ARVD patients, but this association has not been investigated in patients undergoing revascularization.

Digestive properties of the venom of the Australian Coastal Taipan, Oxyuranus scutellatus (Peters, 1867).

The digestive properties of Australian elapid snake venoms have not been studied to any great extent. To address this, the in vitro digestive properties of Oxyuranus scutellatus (Australian Coastal Taipan) venom were investigated in a simulation of the in vivo conditions using the parameters reported for the stomach of snakes and representative prey for this species. The amount of soluble protein released was measured over time using a bicinchoninic acid (BCA) assay.

Resistance to macrocyclic lactone anthelmintics by Haemonchus contortus and Ostertagia circumcincta in sheep in New Zealand.

Aim: To establish the efficacy of oral formulations of ivermectin and moxidectin against naturally acquired abomasal nematode infections on a North Island sheep farm.

Methods: Two controlled slaughter trials were undertaken. In the first, 30 sheep on pasture were randomly allocated on the basis of faecal egg count to 1 of 3 groups, comprising an untreated control group and 2 treatment groups.

End-stage renal disease in Indo-Asians in the North-West of England.

Background: The incidence of end-stage renal disease (ESRD) in England is increasing. There is a higher incidence of ESRD in British Indo-Asians than in the White population.

Aim: To determine to what degree the increasing demand for renal replacement therapy in the UK is due to Indo-Asian patients.

The effect of different methods of storage on the results of serum total CO2 assays.

Metabolic acidosis frequently complicates end-stage renal failure. In haemodialysis patients its severity is usually monitored by measurement of the total CO(2) (TCO(2)) level. Samples from 'satellite dialysis' patients are often stored prior to analysis.