Recovery from rituximab-associated persistent hypogammaglobulinaemia in children with nephrotic syndrome.

Background And Hypothesis: There are limited data on the long-term outcomes and risk factors for non-recovery after development of rituximab (RTX)-associated persistent hypogammaglobulinaemia among children with idiopathic nephrotic syndrome (NS).

Methods: A nationwide Japanese survey was conducted to determine the prognosis of patients with childhood-onset idiopathic NS who developed persistent hypogammaglobulinaemia after RTX administration. Specifically, predictors of IgG level recovery and risk factors for serious infection were examined.

Clinical spectrum of male patients with OFD1 mutations.

Oral-facial-digital syndrome type 1 (OFD1) is a ciliopathy characterized by oral, facial, and digital malformations that are often accompanied by polycystic lesion of the kidney and central nervous involvement. OFD1 shows an X-linked recessive inheritance caused by mutation in the OFD1 gene (Xp22.2).

End-stage renal disease in Japanese children: a nationwide survey during 2006-2011.

Background: End-stage renal disease (ESRD) in children is considered a rare, but serious condition. Epidemiological and demographic information on pediatric ESRD patients around the world is important to better understand this disease and to improve patient care. The Japanese Society for Pediatric Nephrology (JSPN) reported epidemiological and demographic data in 1998.

Simple surface treatment of cell-culture scaffolds with ultrafine bubble water.

We propose a novel method to treat polymeric scaffold surfaces for cell culture with water containing nanobubbles, called ultrafine bubbles (UFBs), with typical diameters less than 1 μm. A thin film of polystyrene (PS) prepared on a solid substrate was exposed to UFB water for 2 days at room temperature. The PS surface was characterized by X-ray photoelectron spectroscopy (XPS), static contact angle measurements in water, and atomic force microscopy (AFM).

Eculizumab in the treatment of atypical hemolytic uremic syndrome in an infant leads to cessation of peritoneal dialysis and improvement of severe hypertension.

Background: Severe hypertension (HTN) and acute kidney injury frequently associated with atypical hemolytic uremic syndrome (aHUS) were refractory to various therapies in the pre-eculizumab era. Here we report the case of a 4-month-old boy who developed aHUS presenting with undetectable C3 protein, no predisposing mutations in complement factors, and no antibodies against factor H.

Methods: Repeated plasma infusions and nine sessions of plasmapheresis were ineffective.

Darbepoetin alfa for the treatment of anemia in children undergoing peritoneal dialysis: a multicenter prospective study in Japan.

Background: Darbepoetin alfa (DA) is an attractive alternative to recombinant human erythropoietin (rHuEPO) in managing renal anemia. Since DA has not been approved by the appropriate Japanese drug regulatory agencies for the indication of renal anemia in children in Japan, we have conducted a multicenter prospective study to determine the efficacy and safety of DA in Japanese children undergoing peritoneal dialysis (PD).

Methods: Pediatric patients subcutaneously receiving rHuEPO were switched to DA treatment for a period of 28 weeks.

Porous silicon/Ni composites of high coercivity due to magnetic field-assisted etching.

Ferromagnetic nanostructures have been electrodeposited within the pores of porous silicon templates with average pore diameters between 25 and 60 nm. In this diameter regime, the pore formation in general is accompanied by dendritic growth resulting in rough pore walls, which involves metal deposits also offering a branched structure. These side branches influence the magnetic properties of the composite system not only due to modified and peculiar stray fields but also because of a reduced interpore spacing by the approaching of adjacent side pores.

Effect of single-dose oral mizoribine pulse therapy twice per week for frequently relapsing steroid-dependent nephrotic syndrome.

Aim: To evaluate the efficacy of single-dose oral mizoribine (MZB) pulse therapy given twice weekly for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS).

Methods: The subjects were 8 patients with FR-SDNS with a median age of 6.9 years old (range 3.

Etanercept in combination with conventional disease-modifying antirheumatic drugs (DMARDs) in the treatment of rheumatoid arthritis patients intolerant to methotrexate.

Although etanercept (ETN) is effective when used in monotherapy for the treatment of rheumatoid arthritis (RA), ETN/methotrexate (MTX) combination therapy is more efficacious. However, some patients show MTX intolerance; these patients may develop adverse events (AEs) or have risk factors for AEs. There is limited published information regarding the efficacy of combination therapy involving ETN and disease-modifying antirheumatic drugs other than MTX.

[Changes in the drug resistance of Haemophilus influenzae and Streptococcus pneumoniae isolated between 1997 and 2006].

We studied changes in the drug resistance of 606 strains of Haemophilus influenzae (H. influenzae) and 502 strains of Streptococcus pneumoniae (S. pneumoniae) isolated from our patients between 1997 and 2006.

[Nationwide survey for autoantibodies and proposal of a new method for standardization of rheumatoid factors].

There exist inter-laboratory differences in measurements of rheumatoid factor (RF) and antinuclear antibodies (ANA), leading to a misdiagnosis of rheumatoid arthritis (RA) and other collagen diseases. This study was carried out to bring the positivity of RF and ANA of different reagents into accord by standardizing their data. The titer and cutoff value was inconsistent among the 17 different kits.

A case of carbamoyl phosphate synthetase 1 deficiency presenting symptoms at one month of age.

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle which causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed onset type. Neonatal CPS1D cases often present their symptoms within the first days of life.

Neonatal pertussis presenting as acute bronchiolitis: direct detection of the Bordetella pertussis genome using loop-mediated isothermal amplification.

We report a 28-day-old female infant with pertussis presenting as severe acute bronchiolitis with cyanosis. On admission, the patient's symptoms were similar to those of acute bronchiolitis. However, occasional apneic episodes with cyanosis and peripheral lymphocytosis suggested neonatal pertussis and prompted us to examine the presence of Bordetella pertussis using loop-mediated isothermal amplification (LAMP) based on the insertion sequence IS481.

Kidney transplantation in pediatric recipients with mental retardation: clinical results of a multicenter experience in Japan.

Background: There are few published reports on kidney transplantation (KT) in physically handicapped patients with mental retardation. The aim of this study is to clearly identify the outcome of KT in these patients and clarify whether handicapped patients can be candidates for KT.

Methods: Our study identified 25 multiply handicapped transplant recipients from 8 institutions.

[A bacteriological study of multiple-drug-resistant Pseudomonas aeruginosa isolates derived from 2 patients].

We investigated the isolation circumstances of multiple-drug-resistant Pseudomonas aeruginosa (MDRP) in the UOEH hospital and the bacterial analysis of isolated MDRP. From January to October 2003, MDRP was isolated from 2 patients. During this period, the isolation frequency of MDRP was 0.

Exercise-induced acute renal failure associated with renal hypouricaemia: results of a questionnaire-based survey in Japan.

Background: A retrospective investigation was conducted to define the clinical features of exercise-induced acute renal failure (ARF) associated with renal hypouricaemia with the aim of clarifying further the clinical features of the disease entity.

Methods: A questionnaire was mailed to 43 institutions in Japan that had experienced case(s) of exercise-induced ARF associated with renal hypouricaemia. Fifty-four patients (48 males and six females) were identified from 38 institutions.

Reversible posterior leukoencephalopathy in a patient with Wegener granulomatosis.

A 14-year-old girl with rapidly progressive glomerulonephritis was transferred to our hospital because of acute renal failure. A diagnosis of Wegener granulomatosis was made according to the symptom triad of a renal biopsy demonstrating crescentic glomerulonephritis, severe sinusitis, and serological findings of raised proteinase 3 anti-neutrophil cytoplasmic antibody level. In spite of combination therapy with methylprednisolone, cyclophosphamide, and plasma exchange, her renal function gradually deteriorated.