Serum ferritin as an indicator of the development of encephalopathy in enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome.

Objectives: To investigate the diagnostic value of serum ferritin levels as a marker of disease activity and the development of encephalopathy in hemolytic uremic syndrome (HUS) induced by enterohemorrhagic Escherichia coli.

Methods: Twenty patients with HUS were studied. Serum ferritin levels were compared with clinical features and serum soluble tumor necrosis factor receptor (sTNFR) I and sTNFRII levels.

The true distribution volume and bioavailability of mizoribine in children with chronic kidney disease.

Background: Mizoribine (MZR) is used kidney transplant and various kidney diseases. However, few studies reported the association between pharmacokinetics and pharmacodynamics. The Pharmacokinetics Study Group for Pediatric Kidney Disease (PSPKD) used population pharmacokinetics (PPK) analysis and Bayesian analysis to investigate the usefulness of MZR.

Female X-linked Alport syndrome with somatic mosaicism.

Background: X-linked Alport syndrome (XLAS) is a progressive, hereditary nephropathy. Although males with XLAS usually develop end-stage renal disease before 30 years of age, some men show a milder phenotype and possess somatic mosaic variants of the type IV collagen α5 gene (COL4A5), with severity depending on variant frequencies. In females, somatic mosaic variants are rarely reported in XLAS, and it is not clear what determines severity.

Angiopoietin-1 and -2 as markers for disease severity in hemolytic uremic syndrome induced by enterohemorrhagic Escherichia coli.

Background: Angiopoietin (Ang)-1 and -2 play important roles in maintaining vascular homeostasis. This study aimed to assess the roles of angiopoietin (Ang)-1 and -2 and to investigate the clinical significance of their serum levels in patients with hemolytic uremic syndrome (HUS) induced by enterohemorrhagic Escherichia coli (EHEC).

Methods: Twenty patients with HUS and 15 healthy controls were studied.

Population pharmacokinetics of mizoribine in pediatric patients with kidney disease.

Background: The present study aimed to obtain information enabling optimisation of the clinical effect of mizoribine (MZR) in pediatric patients with kidney disease.

Methods: A total of 105 pediatric patients with kidney disease treated at our institutions were enrolled. Kidney transplant patients were excluded.

Serum tau protein as a marker of disease activity in enterohemorrhagic Escherichia coli O111-induced hemolytic uremic syndrome.

Tau protein levels in cerebrospinal fluid (CSF) and serum are elevated in patients with various central nervous system diseases. We investigated whether serum tau protein levels are useful for predicting and assessing disease activity of acute encephalopathy (AE) in enterohemorrhagic Escherichia coli (EHEC) O111-induced hemolytic uremic syndrome (HUS; EHEC encephalopathy). Serum samples were obtained from 14 patients with EHEC O111/HUS, 20 patients with non-EHEC-related AE, and 20 age- and sex-matched healthy controls.

Impairment of Left Ventricular Longitudinal Contraction is Associated with Serum Brain Natriuretic Peptide Concentration in Patients with Acute-Phase Kawasaki Disease.

It is well known that brain natriuretic peptide (BNP) is elevated in the acute phase of Kawasaki disease (KD). We hypothesized that mitral annular plane systolic excursion (MAPSE) could identify LV dysfunction in the acute phase of KD. Fifty patients with KD were enrolled in this study.

Evaluation of right ventricular function using single-beat three-dimensional echocardiography in neonate.

Aim of our study was to evaluate right ventricular (RV) systolic function in neonate using newly developed single-beat three-dimensional echocardiography (sb3DE). We enrolled 15 healthy or premature neonates (0-53 days after birth). We scanned one beat full volume using Siemens ACUSON SC2000 (Siemens AG) echocardiography with 4Z1c full-volume transducer without ECG gating.

Z-values of tricuspid annular plane systolic excursion in Japanese children.

Background: The aim of this study was to establish growth-related standard values and z-values for tricuspid annular plane systolic excursion (TAPSE) for Japanese children.

Method: We examined 953 consecutive healthy children and adolescents, ranging from newborn to 22.7 years of age (mean age, 4.

Cytokine profiles of patients with enterohemorrhagic Escherichia coli O111-induced hemolytic-uremic syndrome.

Proinflammatory cytokines are related to the pathogenesis of enterohemorrhagic Escherichia coli (EHEC) infection and hemolytic-uremic syndrome (HUS). We assessed the kinetics of the release of cytokines such as neopterin, interleukin (IL)-6, IL-8 and tumour necrosis factor (TNF)-α and the soluble forms of type I and II TNF receptors during EHEC O111-induced HUS (EHEC O111/HUS). Fourteen patients with EHEC O111/HUS were enrolled in this study.

Evaluation of limited sampling designs to estimate maximal concentration and area under the curve of mizoribine in pediatric patients with renal disease.

The aim of this study was to evaluate limited sampling designs to estimate the maximal concentration (C(max)) and area under the curve (AUC) of mizoribine in pediatric patients with renal disease. We utilized 48 serum mizoribine concentration profiles obtained from the full (6-point) sampling pharmacokinetic test, and estimated 48 individual C(max) and AUC values accurately with Bayesian analysis using the full sampling data. We then developed limited sampling models (LSM) for C(max) and AUC using 1-4 serum mizoribine concentration data points.

Survey of pediatric ward hospitalization due to respiratory syncytial virus infection after the introduction of palivizumab to high-risk infants in Japan.

Background: Respiratory syncytial virus (RSV) infection is a major cause of hospitalization during the winter among infants and young children. In 2002 palivizumab was introduced to high-risk infants for RSV hospitalization in Japan. It is important to characterize the hospitalized children due to RSV infection after the introduction of palivizumab.

Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis.

Objectives: To compare the pro-inflammatory cytokine profiles and the cytokine kinetics in patients with secondary macrophage activation syndrome (MAS) due to systemic-onset juvenile idiopathic arthritis (s-JIA) and in both active and inactive disease states of s-JIA (but no MAS), with those demonstrated in EBV-induced haemophagocytic lymphohistiocytosis (HLH) and Kawasaki disease (KD), and to investigate the significance of IL-18 in the pathogenesis of s-JIA.

Methods: Five patients with MAS complicating s-JIA (MAS/s-JIA), 10 with HLH due to EBV infection (EBV-HLH), 22 with KD and 28 healthy controls were analysed. Cytokine concentrations (IL-18, IL-6, neopterin and TNF-alpha receptor Types I and II) were quantified in serum by ELISA.