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Funaki T.,National Center for Child Health and Development | Inoue E.,Center for Clinical Research and Development | Miyairi I.,National Center for Child Health and Development
BMC Infectious Diseases | Year: 2016

Background: Invasive diseases due to Moraxella catarrhalis are rare in children, even in immunocompromised hosts. Therefore, data regarding clinical characteristics and risk factors of such patients are limited. The aim of this study is to compare the clinical characteristics of patients with bacteremia due to Moraxella catarrhalis against those with bacteremia due to Streptococcus pneumoniae and Haemophilus influenzae. Methods: We performed a retrospective case-control study to compare patients younger than 18years of age with positive blood cultures for the three pathogens between June 2008 and May2014 at our institution. Data regarding patients' demographics and clinical course were collected from their medical records. Three group comparisons, with M. catarrhalis as reference, were made by the Fisher's exact test and Wilcoxon rank sum test for discrete and continuous variables, respectively. Results: There were eight cases of M. catarrhalis, 110 cases of S. pneumoniae (105 patients) and 22 cases of H. influenzae. The M. catarrhalis group consisted of six females (75%) with a mean age of 16months. The majority of patients (7/8, 88%) had underlying diseases; however, only one was immunocompromised. Characteristically, six patients (75%) had medical devices including trans-nasal devices (5/8, 63%). Univariate analysis revealed that underlying conditions (P=0.005), trans-nasal devices (P<0.001), and lower body weight (P=0.016) and low white blood cell count (P=0.011) at the onset of illness were associated with the M. catarrhalis group compared to the S. pneumoniae group. Meanwhile, the higher rates of the patients with underlying conditions and trans-nasal devices were associated with the M. catarrhalis group compared to the H. influenzae group (P=0.039, P<0.001, respectively). Conclusions: The presence of underlying conditions, particularly in those with trans-nasal devices, were characteristic features of patients with bacteremia due to M. catarrhalis. © 2016 Funaki et al. Source


Iijima K.,Kobe University | Sako M.,Center for Clinical Research and Development | Nozu K.,Kobe University
Clinical and Experimental Nephrology | Year: 2016

Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. At least 20 % of children with this syndrome show frequent relapses and/or steroid dependence during or after immunosuppressive therapies, a condition defined as complicated frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS). Approximately 1–3 % of children with idiopathic nephrotic syndrome are resistant to steroids and all immunosuppressive agents, a condition defined as refractory steroid-resistant nephrotic syndrome (SRNS); these SRNS children have a high risk of end-stage renal failure. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be effective for patients with complicated FRNS/SDNS and refractory SRNS. This review describes the recent results of rituximab treatment applied to pediatric nephrotic syndrome, as well as those of our recent study, a multicenter, double-blind, randomized, placebo-controlled trial of rituximab for childhood-onset complicated FRNS/SDNS (RCRNS01). The overall efficacy and safety of rituximab for this disease are discussed. © 2016 The Author(s) Source


Miyoshi Y.,Osaka University | Yorifuji T.,Childhood Cancer Survivor Committee | Horikawa R.,Childhood Cancer Survivor Committee | Horikawa R.,National Center for Child Health and Development | And 21 more authors.
Clinical Pediatric Endocrinology | Year: 2016

An increasing number of pediatric cancer patients survive, and treatment-related infertility represents one of the most important issues for these patients. While official guidelines in Japan recommend long-term follow-up of childhood cancer survivors (CCSs), their gonadal function and fertility have not been clarified. To address this issue, we organized a working panel to compile evidence from long-term survivors who received treatments for cancer during childhood or adolescence. In collaboration with members of the CCS Committee of the Japanese Society for Pediatric Endocrinology (JSPE), we conducted a questionnaire survey regarding reproductive function in pediatric cancer patients. A cross-sectional survey was sent to 178 JSPE-certified councilors who were asked to self-evaluate the medical examinations they had performed. A total of 151 responses were obtained, revealing that 143 endocrinologists were involved in the care of CCSs. A quarter of the respondents reported having experienced issues during gonadal or reproductive examinations. Several survivors did not remember or fully understand the explanation regarding gonadal damage, and faced physical and psychological distress when discussing the risk of becoming infertile. Pediatric endocrinologists had anxieties regarding their patients’ infertility and the risk of miscarriage, premature birth, and delivery problems. Only a limited number of endocrinologists had experience with managing childbirth and fertility preservation. Many councilors mentioned the necessity for inter-disciplinary communication among healthcare providers. Both endocrinologists and oncologists should set and follow a uniform clinical guideline that includes management of fertility of CCSs. © 2016 by The Japanese Society for Pediatric Endocrinology. Source


Komatsu H.,University of Miyazaki | Fujimoto S.,University of Miyazaki | Yoshikawa N.,Center for Clinical Research and Development | Kitamura H.,Clinical Research Center | And 2 more authors.
Clinical and Experimental Nephrology | Year: 2015

Background: The clinical presentation of Henoch–Schönlein purpura nephritis (HSPN) has not been thoroughly investigated among patients of different ages. We therefore compared the features of HSPN and IgA nephropathy (IgAN) based on data from the Japan Renal Biopsy Registry (J-RBR). Methods: This cross-sectional study analyzed data from patients who were registered in the J-RBR between 2007 and 2012. Clinico-pathological findings at diagnosis were compared among children (aged ≤18 years), adult (aged 19–64 years) and elderly (aged ≥65 years) patients with HSPN (n = 513) and IgAN (n = 5679). Results: The age at diagnosis considerably differed between HSPN and IgAN; HSPN peaked at 1–19 and at 60–69 years, whereas IgAN peaked at 30–39 years. The clinical features were significantly more severe for HSPN than IgAN, especially proteinuria (children, 1.28 vs. 0.57; adult, 1.95 vs. 1.05; elderly patients, 2.71 vs. 1.64 g/day), and low albumin levels (children, 3.72 vs. 4.13; adults, 3.62 vs. 3.99; elderly patients, 3.07 vs. 3.57 g/dL). The rate (%) of histologically classified endocapillary proliferative or crescentic glomerulonephritis was higher in patients with HSPN than with IgAN. Multiple regression analysis revealed that low albumin level and high BP were independent factors associated with decreased estimated glomerular filtration rates in adult and elderly patients with HSPN. Conclusions: Age at HSPN diagnosis was bimodally distributed, and the clinical features of HSPN were more severe than those of IgAN across all age groups. © 2015 The Author(s) Source


Katagiri S.,National Center for Child Health and Development | Katagiri S.,Jikei University School of Medicine | Yokoi T.,National Center for Child Health and Development | Mikami M.,Center for Clinical Research and Development | And 2 more authors.
Graefe's Archive for Clinical and Experimental Ophthalmology | Year: 2016

Purpose: To investigate the relationship between vision and foveal maturity, especially in foveal hypoplasia exhibiting severe structural immaturity. Methods: This retrospective observational case series included 42 eyes of 23 patients (mean age, 7.0 ± 5.0 years; 9 patients with foveal hypoplasia as an isolated entity and 14 patients with aniridia). A complete ophthalmic examination included measurement of best-corrected visual acuity (BCVA) and spectral-domain optical coherence tomography (SD-OCT). The sensory retina, ganglion cell complex (GCC), and outer retinal layers, including Henle’s fiber layer (HFL), were measured and analyzed. Results: Using SD-OCT images, eyes were classified as having a differentiated (6 eyes), diffuse (19 eyes), or no HFL (17 eyes), based on the appearance of the HFL around the foveal region. The logMAR BCVA was significantly worse (p < 0.0001) in eyes with diffuse HFL and those with no HFL than in those with differentiated HFL. Outer retinal thickness (outer plexiform layer + HFL + outer nuclear layer) was less (p = 0.0051) in eyes with no HFL than in those with differentiated HFL. The logMAR BCVA, GCC thickness, and outer retinal thickness in eyes with foveal hypoplasia with aniridia were significantly worse (p = 0.0083), thicker (p = 0.0039), and thinner (p = 0.0001), respectively, than in eyes with foveal hypoplasia as an isolated entity. Conclusions: In eyes with foveal hypoplasia with severe structural immaturity, diffuse HFL or no HFL was associated with worse vision. There was greater foveal immaturity in eyes with foveal hypoplasia with aniridia compared with foveal hypoplasia as an isolated entity. © 2016 Springer-Verlag Berlin Heidelberg Source

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