Low-Dose Intravenous Methylprednisolone in Remission Induction Therapy for ANCA-Associated Vasculitis.

Key Points: The contribution of IV methylprednisolone to glucocorticoid toxicity is often overlooked with limited evidence supporting its use. Markedly reduced cumulative glucocorticoid dosing for remission induction therapy in AAV is safe and effective. Reduced IV methylprednisolone and radical steroid avoidance strategies have not been shown to have any significant adverse effect on outcomes.

Randomized, Controlled Trial of Tacrolimus and Prednisolone Monotherapy for Adults with Minimal Change Disease: A Multicenter, Randomized, Controlled Trial.

Background And Objectives: Minimal change disease is an important cause of nephrotic syndrome in adults. Corticosteroids are first-line therapy for minimal change disease, but a prolonged course of treatment is often required and relapse rates are high. Patients with minimal change disease are therefore often exposed to high cumulative corticosteroid doses and are at risk of associated adverse effects.

Meningococcal B Vaccine Failure With a Penicillin-Resistant Strain in a Young Adult on Long-Term Eculizumab.

We describe a case of invasive meningococcal disease due to a vaccine-preventable and penicillin-resistant strain in a fully immunized young adult on long-term complement inhibitor therapy and daily penicillin chemoprophylaxis. Eculizumab is a humanized monoclonal antibody that binds human complement C5 protein and inhibits the terminal complement pathway. It is currently recommended for the treatment of complement-mediated thrombotic microangiopathies.

Rate of Decline of Kidney Function, Modality Choice, and Survival in Elderly Patients with Advanced Kidney Disease.

Aim: In elderly, dependent patients with advanced chronic kidney disease, dialysis may confer only a small survival advantage over conservative kidney management (CKM). We investigated the role of rate of decline of kidney function on treatment choices and survival.

Methods: We identified a retrospective (1995-2010) cohort of patients aged over 75 years, with progressive kidney impairment and an estimated glomerular filtration rate (eGFR) between 10 and 15 ml/min/1.

Factors determining penetrance in familial atypical haemolytic uraemic syndrome.

Background: Inherited abnormalities of complement are found in ∼60% of patients with atypical haemolytic uraemic syndrome (aHUS). Such abnormalities are not fully penetrant. In this study, we have estimated the penetrance of the disease in three families with a CFH mutation (c.

Survival of elderly patients with stage 5 CKD: comparison of conservative management and renal replacement therapy.

Background: Elderly patients with end-stage renal disease and severe extra-renal comorbidity have a poor prognosis on renal replacement therapy (RRT) and may opt to be managed conservatively (CM). Information on the survival of patients on this mode of therapy is limited.

Methods: We studied survival in a large cohort of CM patients in comparison to patients who received RRT.

Successful stabilisation of nephropathy in a patient with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-band, skin changes) syndrome on treatment with mycophenolate and steroids: a case report.

Introduction: Renal involvement in POEMS (polyneuropathy, organomegaly, endocrinopathy, M-band, skin changes) syndrome is considered to be an under-diagnosed phenomenon with no clear treatment path. The limited literature suggests steroids to be the drug of choice, although improvements are limited and usually reverse on withdrawal of the drug.

Case Presentation: A 52-year-old Caucasian woman presenting with features consistent with POEMS syndrome developed progressive renal impairment with proteinuria.

Superior vena cava obstruction presenting with epistaxis, haemoptysis and gastro-intestinal haemorrhage in two men receiving haemodialysis with central venous catheters: two case reports.

Introduction: Superior vena cava (SVC) obstruction secondary to central venous catheterization is an increasingly recognized complication.

Case Presentation: We present two cases of superior vena cava obstruction secondary to indwelling central venous catheters used for haemodialysis access. One of the patients developed the unusual complications of torrential epistaxis and haemoptysis, which has been reported only once so far in the literature.

Infective spondylodiscitis in patients on high-flux hemodialysis and on-line hemodiafiltration.

Infective spondylodiscitis (ISD) is a rare but potentially devastating condition in hemodialysis (HD) patients. Reports are limited especially in patients receiving high-flux HD and hemodiafiltration (HDF). In a retrospective analysis, 13 patients on our maintenance high-flux HD/HDF program were identified as having has infective spondylodiscitis over a 10-year period (1997-2006), an incidence of approximately 1 episode every 215 patient-years.

Familial membranous nephropathy: an X-linked genetic susceptibility?

Background: Membranous nephropathy (MN) is the most common histological diagnosis in adults with nephrotic syndrome and a leading cause of end-stage kidney failure from glomerulonephritis. Little is known about the underlying aetiology, although anti-glomerular antibodies have been implicated. No specific underlying genetic defect has yet been identified.

Factors affecting long-term survival of tunnelled haemodialysis catheters--a prospective audit of 812 tunnelled catheters.

Background: In 2001, in the US, 23% of haemodialysis patients were dialysing through tunnelled venous catheters (TVCs), and in the UK (2006) there were 28% of prevalent patients using catheters. It is unlikely that numbers will significantly decrease. We present the results of a prospective audit of the survival of 812 TVCs placed in 492 patients at our institution over a 6-year period (comprising 212 048 patient catheter days or 7068 patient catheter months of follow-up).

Atypical haemolytic uraemic syndrome associated with a hybrid complement gene.

Background: Sequence analysis of the regulators of complement activation (RCA) cluster of genes at chromosome position 1q32 shows evidence of several large genomic duplications. These duplications have resulted in a high degree of sequence identity between the gene for factor H (CFH) and the genes for the five factor H-related proteins (CFHL1-5; aliases CFHR1-5). CFH mutations have been described in association with atypical haemolytic uraemic syndrome (aHUS).

Choosing not to dialyse: evaluation of planned non-dialytic management in a cohort of patients with end-stage renal failure.

Objectives: To study factors influencing the recommendation for palliative (non-dialytic) treatment in patients approaching end-stage renal failure and to study the subsequent outcome in patients choosing not to dialyse.

Design: Cohort study of patients approaching end-stage renal failure who underwent multidisciplinary assessment and counselling about treatment options. Recruitment was over 54 months, and follow-up ranged from 3 to 57 months.

Spinal cord infarction following central-line insertion.

Hemothorax is a recognized complication of central line insertion into the jugular or subclavian vein. We describe a case of hemothorax consequent upon acute dialysis catheter insertion, which resulted in spinal cord infarction and quadriplegia. We postulate that the extensive mediastinal shift induced after insertion of the catheter resulted in stretching of the veins draining the cord with a resultant drop in perfusion pressure and infarction.

Acute renal failure and metabolic disturbances in the short bowel syndrome.

Background: Short bowel syndrome (SBS) describes a malabsorptive state caused by extensive loss of small intestinal length.

Aim: To improve understanding of the metabolic complications of SBS.

Design: Observational study of five patients with SBS who presented with acute renal failure.

Primary, nonsyndromic vesicoureteric reflux and its nephropathy is genetically heterogeneous, with a locus on chromosome 1.

Primary vesicoureteric reflux (VUR) affects 1%-2% of whites, and reflux nephropathy (RN) causes up to 15% of end-stage renal failure in children and adults. There is a 30-50-fold increased incidence of VUR in first-degree relatives of probands, compared with the general population. We report the results of the first genomewide search of VUR and RN; we studied seven European families whose members exhibit apparently dominant inheritance.

Familial relapsing haemolytic uraemic syndrome and complement factor H deficiency.

Background: In a recent study of three families we have found that inherited haemolytic uraemic syndrome (HUS) maps to a region of chromosome 1q containing the gene for complement factor H. In one of these families and also in a case of sporadic D-HUS, we have identified mutations in the factor H gene. A further family with inherited HUS has therefore been investigated.