Morbidity in children with frequently relapsing nephrosis: 10-year follow-up of a randomized controlled trial.

Background: To investigate the long-term outcome in children with frequently relapsing nephrotic syndrome (FRNS) we conducted a follow-up of a previous randomized controlled trial (RCT) 10 years after the initiation of the treatment protocol.

Methods: We previously conducted an RCT on the efficacy of cyclosporine for treating children with FRNS. After 2 years of treatment, a recommended a management protocol of steroids, and immunosuppressants was provided.

Cyclosporine C2 monitoring for the treatment of frequently relapsing nephrotic syndrome in children: a multicenter randomized phase II trial.

Background And Objectives: An open-label, multicenter, randomized phase II trial was conducted from July 1, 2005 to March 29, 2011 to compare two protocols for treating children with frequently relapsing nephrotic syndrome using microemulsified cyclosporine.

Design, Setting, Participants, & Measurements: Ninety-three children with frequently relapsing nephrotic syndrome were randomly assigned to group A (n=46) or group B (n=47). In both groups, the 2-hour postdose cyclosporine level was monitored.

High plasma linezolid concentration and impaired renal function affect development of linezolid-induced thrombocytopenia.

Objectives: Thrombocytopenia is sometimes observed during linezolid therapy. Here, we aimed to investigate the factors affecting linezolid-induced thrombocytopenia.

Methods: A prospective observational study was performed between October 2009 and February 2011; 30 patients were included.

Reference ranges for serum cystatin C measurements in Japanese children by using 4 automated assays.

Objective: The data available on reference ranges for cystatin C in children are limited, and there are discrepancies among the available data. The aim of this study was to describe the reference ranges for cystatin C in Japanese children by using 4 automated assays.

Methods: Serum cystatin C levels were measured in 1128 Japanese children aged 3 month to 16 years without kidney disease.

Two-year follow-up of a prospective clinical trial of cyclosporine for frequently relapsing nephrotic syndrome in children.

Background And Objectives: Although the safety and efficacy of cyclosporine in children with frequently relapsing nephrotic syndrome (FRNS) have been confirmed, no prospective follow-up data on relapse after cyclosporine have appeared. This study is a prospective follow-up trial after 2-year treatment with cyclosporine to investigate cyclosporine dependency after its discontinuation.

Design, Setting, Participants, & Measurements: Participants who had undergone 2-year protocol treatment with microemulsified cyclosporine for FRNS between January 2000 and December 2005 were followed for an additional 2 years.

Establishment of a normal reference value for serum β2 microglobulin in Japanese children: reevaluation of its clinical usefulness.

Objective: Serum β2 microglobulin (β2MG) is considered to be a marker of renal function, which is independently associated with age. However, only a few studies have reported the reference values for β2MG in children thus far, particularly in young children. In this study, we evaluated the distribution of serum β2MG values in healthy Japanese children and assessed its clinical usefulness.

Unadjuvanted pandemic H1N1 influenza vaccine in HIV-1-infected adults.

We evaluated the immune response to a 2009 influenza A (H1N1) unadjuvanted vaccine in HIV-infected patients and assessed the boosting effect of a second dose. HIV-infected adults were enrolled and scheduled to receive the H1N1 unadjuvanted vaccine containing 15μg of A/California/7/2009 haemagglutinin. Anti-H1N1 antibody titers were measured at enrollment and 4-8 weeks after each vaccination by using haemagglutination inhibition (HI) and virus neutralization (NT) assays.

Age, gender, and body length effects on reference serum creatinine levels determined by an enzymatic method in Japanese children: a multicenter study.

Background: Enzymatic methods have recently been used to measure creatinine (Cr) instead of the Jaffe method. Therefore, it is necessary to determine the reference serum Cr value for these enzymatic methods to evaluate renal function in Japanese children.

Methods: To determine reference values of serum Cr in Japanese children, 1151 children (517 male, 634 female) aged between 1 month and 18 years had their serum Cr values measured by an enzymatic method.

Henoch-Schönlein purpura nephritis with nephrotic state in children: predictors of poor outcomes.

Nephritis develops in 18-81% of Henoch-Schönlein purpura patients, and the long-term outcomes of this nephritis show great variation. A nephrotic state at disease onset has been proposed as a predictor of poor renal outcomes. We studied 42 children with Henoch-Schönlein purpura nephritis (HSPN) who presented with a nephrotic state during the early phase of the disease.

Treatment with microemulsified cyclosporine in children with frequently relapsing nephrotic syndrome.

Background: We previously established a treatment protocol for conventional cyclosporine (Sandimmune, Novartis, Basel, Switzerland) in children with frequently relapsing nephrotic syndrome; ∼50% of patients remained relapse free for 2 years, without serious adverse events. Recently, microemulsified cyclosporine (Neoral, Novartis), which has a more stable absorption profile than conventional cyclosporine, has been developed. We tested the hypothesis that microemulsified cyclosporine is at least as effective as conventional cyclosporine.

Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome.

We conducted a prospective, multicenter trial to evaluate the efficacy and safety of a 12-month course of cyclosporine in children with steroid-resistant nephrotic syndrome (SRNS). Thirty-five patients were enrolled, of whom 28 had minimal change or diffuse mesangial proliferation (MC/DMP), and seven had focal segmental glomerulosclerosis (FSGS). All patients received cyclosporine and prednisolone; patients with FSGS additionally received methylprednisolone pulse therapy (MPT).

Improved renal survival in Japanese children with IgA nephropathy.

Since the beginning of the 1990s, Japanese medical practitioners have extensively prescribed angiotensin-converting enzyme (ACE) inhibitors for children with mild IgA nephropathy (IgA-N) and steriods for those with severe IgA-N. We have performed a retrospective cohort study to clarify whether the long-term outcome has improved in Japanese children with IgA-N. Renal survival was defined as the time from onset to end-stage renal disease (ESRD).

Segmental membranous glomerulonephritis in children: comparison with global membranous glomerulonephritis.

Generally, idiopathic membranous glomerulonephritis (MGN) is a global glomerular disease that affects the whole of the glomerulus. However, idiopathic segmental MGN (SMGN) that shows IgG deposits in a portion of the glomerulus is encountered often. For clarification of whether SMGN is the same entity as idiopathic global MGN (GMGN), the two diseases were compared.

Analysis of NPHS1, NPHS2, ACTN4, and WT1 in Japanese patients with congenital nephrotic syndrome.

Background: Congenital nephrotic syndrome (CNS) causes significant renal failure, and is classified into two types: (1) Finnish type; and (2) other, including diffuse mesangial sclerosis. Mutations of NPHS1 and NPHS2, which encode the slit diaphragm components nephrin and podocin, cause CNS and autosomal-recessive familial steroid-resistant nephrotic syndrome, respectively. Most patients with Finnish-type CNS in Europe and the United States have NPHS1 mutations.

Evaluation of the inferior vena cava in potential pediatric renal transplant recipients.

Vascular anomalies due to occlusion or thrombosis of the inferior vena cava (IVC) may be hazardous to renal transplantation, and preoperative vascular evaluation is important for safe and successful surgery. The purpose of this study was to assess the utility and accuracy of two-dimensional time-of-flight (2D-TOF) magnetic resonance venography (MRV) as an alternative to conventional angiography for evaluating the IVC and iliac vein in potential pediatric renal transplant recipients. Four children with chronic renal failure were evaluated with 2D-TOF MRV by superior presaturation pulse and target maximum intensity projection.

Typical MPGN with few urinary abnormalities.

We describe the concurrent appearance of membranoproliferative glomerulonephritis (MPGN) in renal biopsy samples and normal urinary sediment without hematuria, proteinuria, or renal dysfunction in 1 child and asymptomatic microscopic hematuria without significant proteinuria or renal dysfunction in 2 children who were subsequently followed up for many years. The only other abnormality detected was hypocomplementemia. This is the first report of biopsy-proven typical MPGN in patients with few urinary abnormalities.

A-20C angiotensinogen gene polymorphism and proteinuria in childhood IgA nephropathy.

We have previously reported that the TT genotype of the angiotensinogen gene and the ID/DD genotype of the angiotensin-converting enzyme gene are associated with increased severity of proteinuria in IgA nephropathy in Japanese children. Recently it was reported that polymorphism at -20 from adenine to cytosine in the angiotensinogen gene, increasing the level of this transcript, was associated with the progression of renal dysfunction in adult IgA nephropathy. We therefore investigated whether this polymorphism is involved in IgA nephropathy in Japanese children.

[Peritoneal dialysis catheter-related complications].

Unlabelled: The introduction of a double-cuff swan neck type catheter has reduced the frequency of peritonitis. The frequency of complications associated with insertion of this catheter has remained unknown. We evaluated these complications in patients aged < 20 years at the start of the chronic peritoneal dialysis using double-cuff swan neck catheters.