Mizoribine halts kidney fibrosis in childhood IgA nephropathy: association with modulation of M2-type macrophages.

Background: The immunosuppressant mizoribine (Miz) can reduce progression of childhood IgA nephropathy (IgAN). This study examined whether Miz affects CD163 M2-type macrophages which are associated with kidney fibrosis in childhood IgAN.

Methods: A retrospective cohort of 90 children with IgAN were divided into groups treated with prednisolone (PSL) alone (P group; n = 42) or PSL plus Miz (PM group; n = 48) for a 2-year period.

A novel COL4A5 splicing variant causing X-linked Alport syndrome: A case report.

Alport syndrome is a hereditary disorder characterized by renal impairment, hearing loss, and ocular symptoms and is caused by COL4A3, COL4A4, and COL4A5 mutations. Here, we report the case of 3-year-old boy with isolated hematuria detected in routine preventative urinary screening conducted in 3-year-old children. He carried a novel variant, NM_033380.

Transient Type 3 Renal Tubular Acidosis during Cyclic Vomiting Syndrome.

Type 3 renal tubular acidosis is a pathological condition characterized by the simultaneous occurrence of distal renal tubular acidosis, which causes urinary acidification disorders, and proximal renal tubular acidosis, which causes impaired reabsorption of bicarbonate ions. Type 3 renal tubular acidosis is considered rare. A 5-year-old boy was admitted to our hospital because of frequent vomiting, poor vitality, and fever.

Mycophenolate Mofetil after Rituximab for Childhood-Onset Complicated Frequently-Relapsing or Steroid-Dependent Nephrotic Syndrome.

Background: Rituximab is the standard therapy for childhood-onset complicated frequently relapsing or steroid-dependent nephrotic syndrome (FRNS/SDNS). However, most patients redevelop FRNS/SDNS after peripheral B cell recovery.

Methods: We conducted a multicenter, randomized, double-blind, placebo-controlled trial to examine whether mycophenolate mofetil (MMF) administration after rituximab can prevent treatment failure (FRNS, SDNS, steroid resistance, or use of immunosuppressive agents or rituximab).

Steroid treatment promotes an M2 anti-inflammatory macrophage phenotype in childhood lupus nephritis.

Background: M1-type proinflammatory macrophages (MΦ) promote glomerular injury in lupus nephritis (LN). However, whether this phenotype is altered by steroid therapy is unclear. Therefore, we investigated the effect of steroid treatment on MΦ phenotype in LN.

Management of a Preterm Infant with Renal Tubular Dysgenesis: A Case Report and Review of the Literature.

Renal tubular dysgenesis (RTD) is the absence or poor development of the renal proximal tubules caused by gene mutations in the renin-angiotensin system. Although RTD has been considered fatal, improving neonatal intensive care management has enhanced survival outcomes. However, little has been reported on the survival of extremely preterm infants.

Bortezomib Eliminates Plasma Cells From a Renal Graft in Plasma Cell-Rich Acute Rejection.

Plasma cell-rich acute rejection (PCAR) and antibody-mediated rejection (ABMR), for which a standard treatment has not yet been established, are associated with poor graft survival after kidney transplantation. Here, we report a case series of 3 Japanese patients diagnosed with PCAR accompanied by ABMR. Steroid pulse therapy and rabbit antithymocyte globulin, plasma exchange, intravenous immunoglobulin, and rituximab therapies were sequentially performed in the first case.

Assessment of factors associated with mizoribine responsiveness in children with steroid-dependent nephrotic syndrome.

Background: Several immunosuppressants have been used to treat children with steroid-dependent nephrotic syndrome (SDNS). Mizoribine (MZR) is an immunosuppressant used to maintain remission in children with SDNS, although its effectiveness for treating SDNS remains controversial. Therefore, in this study, we assessed the clinical factors associated with children having SDNS who were successfully treated with MZR.

[LONG-TERM OUTCOME OF PEDIATRIC KIDNEY TRANSPLANTATION: A SINGLE-CENTER EXPERIENCES].

(Background) Long-term care is necessary for normal growth and development of pediatric recipients of kidney transplants. We report on our experience with pediatric kidney transplantation (KTx) during the past 19 years. (Methods) We retrospectively analyzed the data from 26 recipients who received KTx between 1996 and 2014 at Niigata University Hospital (one patient underwent two consecutive KTx during the designated period).

Glomerular Density and Volume in Renal Biopsy Specimens of Children with Proteinuria Relative to Preterm Birth and Gestational Age.

Background And Objectives: A low total nephron number, which is associated with low birth weight (LBW), may indicate increased susceptibility to early-onset renal diseases in children. However, few studies have assessed renal biopsy findings in LBW children. We examined the relationship between LBW and glomerular density (GD) and/or glomerular volume (GV) in renal biopsy samples as a surrogate for total nephron number.

Rituximab protects podocytes and exerts anti-proteinuric effects in rat adriamycin-induced nephropathy independent of B-lymphocytes.

Aim: This study was aimed at examining the effects of treatment with rituximab, a chimeric monoclonal antibody against the protein CD20, in a B-lymphocyte independent adriamycin-induced rat model of nephrotic syndrome. Rituximab is an emerging rescue therapy used in patients with complicated nephrotic syndrome and, therefore, we sought to elucidate the apparent B-lymphocyte independent mechanism underlying its anti-proteinuric effect.

Methods: Adriamycin-induced nephropathy was established in Wistar rats by intravenously injecting 10 mg/kg of adriamycin, which were then treated with rituximab or purified human IgG weekly and euthanized on day 28.

Local leukocyte proliferation as a target for cyclophosphamide in the treatment of Henoch-Schönlein purpura nephritis grade VI.

Henoch-Schönlein purpura nephritis (HSPN) is one of the most common types of chronic glomerulonephritis in children; however, there have been few reports on the pathogenesis and management of grade VI HSPN. We present the case of a 6-year-old boy with grade VI HSPN accompanied by severe nephrotic syndrome and hypocomplementaemia. Immunohistological studies revealed profound glomerular accumulation of CD45- and CD68-positive inflammatory cells.

Alternatively activated macrophages in the pathogenesis of chronic kidney allograft injury.

Background: Prevention of chronic kidney allograft injury (CAI) is a major goal in improving kidney allograft survival; however, the mechanisms of CAI are not clearly understood. The current study investigated whether alternatively activated M2-type macrophages are involved in the development of CAI.

Methods: A retrospective study examined kidney allograft protocol biopsies (at 1 h and at years 1, 5, and 10--a total of 41 biopsies) obtained from 13 children undergoing transplantation between 1991 and 2008 who were diagnosed with CAI: interstitial fibrosis and tubular atrophy (IF/TA) not otherwise specified (IF/TA-NOS).

Beta-2 microglobulin-based equation for estimating glomerular filtration rates in Japanese children and adolescents.

Background: Although the creatinine (Cr)-based equation is widely used for estimating glomerular filtration rate (GFR), this equation is not ideally suited for children with low body weight or aged <2 years. Therefore, we established a new equation using serum beta-2 microglobulin (β2MG) levels for Japanese children with chronic kidney disease (CKD).

Methods: Inulin clearance and standardized serum β2MG and Cr levels were measured in 137 CKD patients aged 1 month-18 years.

Low birthweight and premature birth are risk factors for podocytopenia and focal segmental glomerulosclerosis.

Background: Recent reports suggest that low birthweight (LBW) is a risk factor for kidney diseases, including focal segmental glomerulosclerosis (FSGS), although the underlying pathological mechanism remains unknown. Podocyte loss triggers glomerulosclerosis; however, whether FSGS in LBW children is associated with podocytopenia is unclear.

Methods: We reviewed the birthweights and gestational age of all patients who underwent renal biopsies from 1995 to 2011 at our Institute.

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.

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.

Is the new Schwartz equation derived from serum creatinine and body length suitable for evaluation of renal function in Japanese children?

Unlabelled: The present study was performed to determine whether the new Schwartz "bedside" equation can be used to estimate the glomerular filtration rate (GFR) in Japanese children as there are differences in renal function and muscle mass between Japanese and American individuals. It is also important to determine whether one common equation can be used in children from 1 to 16 years old, including the period of adolescence. Blood samples were collected from a total of 1,074 healthy children (466 males and 608 females) between 1 and 16 years old.

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.

Identification of alternatively activated macrophages in new-onset paediatric and adult immunoglobulin A nephropathy: potential role in mesangial matrix expansion.

Aims: New onset of the clinical symptoms of immunoglobulin A (IgA) nephropathy (IgAN) manifests with proliferative glomerular lesions in children, whereas adults exhibit mesangial matrix expansion and interstitial fibrosis. Alternatively, activated (M2) macrophages have been implicated in promoting tissue fibrosis in some settings. Therefore, the aim of this study was to investigate whether M2 macrophages are present in new-onset IgAN and if they are related to pathological differences between paediatric and adult disease.