Recent advance in very early-onset inflammatory bowel disease

Recent studies on pediatric inflammatory bowel disease (IBD) have revealed that early-onset IBD has distinct phenotypic differences compared with adult-onset IBD. In particular, very early-onset IBD (VEO-IBD) differs in many aspects, including the disease type, location of the lesions, disease behavior, and genetically attributable risks. Neonatal or infantile-onset IBD develops in less than 1% of pediatric patients. Children with infantile-onset IBD have high rates of affected first-degree relatives and severe disease course. The suspicion of a monogenic cause of VEO-IBD was first confirmed by the discovery of mutations in the genes encoding the interleukin 10 (IL-10) receptors that cause impaired IL-10 signaling. Patients with such mutations typically presented with perianal fistulae, shows a poor response to medical management, and require early surgical interventions in the first year of life. To date, 60 monogenic defects have been identified in children with IBD-like phenotypes. The majority of monogenic defects presents before 6 years of age, and many present before 1 year of age. Next generation sequencing could become an important diagnostic tool in children with suspected genetic defects especially in children with VEO-IBD with severe disease phenotypes. VEO-IBD is a phenotypically and genetically distinct disease entity from adult-onset or older pediatric IBD

Clinical Characteristics and Genotypes of Rotaviruses in a Neonatal Intensive Care Unit

BackgroundThere are few reports on the symptoms of rotavirus infections in neonates. This study aims to describe clinical signs of rotavirus infections among neonates, with a particular focus on preterm infants, and to show the distribution of genotypes in a neonatal intensive care unit (NICU).MethodsA prospective observational study was conducted at a regional NICU for 1 year. Stool specimens from every infant in the NICU were collected on admission, at weekly intervals, and from infants showing symptoms. Rotavirus antigens were detected by enzyme-linked immunosorbent assay (ELISA), and genotypes were confirmed by Reverse transcription-Polymerase chain reaction (RT-PCR). The infants were divided into three groups: symptomatic preterm infants with and without rotavirus-positive stools [Preterm(rota+) and Preterm(rota–), respectively] and symptomatic full- or near-term infants with rotavirus-positive stools [FT/NT(rota+)]. Demographic and outcome data were compared among these groups.ResultsA total of 702 infants were evaluated for rotaviruses and 131 infants were included in this study. The prevalence of rotavirus infections was 25.2%. Preterm(rota+) differed from Preterm(rota–) and FT/NT(rota+) with respect to frequent feeding difficulty (p = 0.047 and 0.034, respectively) and higher percentage of neutropenia (p = 0.008 and 0.011, respectively). G4P[6] was the exclusive strain in both the Preterm(rota+) (97.7%) and FT/NT(rota+) (90.2%), and it was the same for nosocomial, institutional infections, and infections acquired at home.ConclusionSystemic illness signs such as feeding difficulty and neutropenia are specific for preterm infants with rotavirus infections. G4P[6] was exclusive, regardless of preterm birth or locations of infections. This study might be helpful in developing policies for management and prevention of rotavirus infections in NICUs

Eosinophilic gastroenteritis in an 18-year-old male with prolonged nephrotic syndrome

Eosinophilic gastroenteritis is a rare disease characterized by prominent eosinophilic tissue infiltration of the gastrointestinal tract. Here, we report a case of eosinophilic gastroenteritis in an 18-year-old patient with prolonged nephrotic syndrome who presented with abdominal pain and peripheral hypereosinophilia. During the previous 2 years, he had visited local Emergency Department several times because of epigastric pain and nausea. He had been treated with steroid-dependent nephrotic syndrome since 3 years of age. Tests ruled out allergic and parasitic disease etiologies. Gastroduodenoscopy with biopsy revealed marked eosinophilic infiltration in the duodenum. Renal biopsy findings indicated minimal change disease spectrum without eosinophilic infiltration. The oral deflazacort dosage was increased, and the patient was discharged after abdominal pain resolved. To our knowledge, this is the first report of eosinophilic gastroenteritis in a patient with minimal change disease

Helicity-driven chiral self-sorting supramolecular polymerization with Ag+: right- and left-helical aggregates

The study of chiral self-sorting is extremely important for understanding biological systems and for developing applications for the biomedical field. In this study, we attempted unprecedented chiral self-sorting supramolecular polymerization accompanying helical inversion with Ag+ in one enantiomeric component. Bola-type terpyridine-based ligands (R-L-1 and S-L-1) comprising R- or S-alanine analogs were synthesized. First, R-L-1 dissolved in DMSO/H2O (1 : 1, v/v) forms right-handed helical fibers (aggregate I) via supramolecular polymerization. However, after the addition of AgNO3 (0.2-1.1 equiv.) to the R-L-1 ligand, in particular, it was found that aggregate II with left-handed helicity is generated from the [R-L-1(AgNO3)(2)] complex through the [R-(LAg)-Ag-1](+) complex via the dissociation of aggregate I by a multistep with an off pathway, thus demonstrating interesting self-sorting properties driven by helicity and shape discrimination. In addition, the [R-L-1(AgNO3)(2)] complex, which acted as a building block to generate aggregate III with a spherical structure, existed as a metastable product during the formation of aggregate II in the presence of 1.2-1.5 equiv. of AgNO3. Furthermore, the AFM and CD results of two samples prepared using aggregates I and III with different volume ratios were similar to those obtained upon the addition of AgNO3 to free R-L-1. These findings suggest that homochiral self-sorting in a mixture system occurred by the generation of aggregate II composed of the [R-(LAg)-Ag-1](+) complex via the rearrangement of both, aggregates I and III. This is a unique example of helicity- and shape-driven chiral self-sorting supramolecular polymerization induced by Ag+ starting from one enantiomeric component. This research will improve understanding of homochirality in complex biological models and contribute to the development of new chiral materials and catalysts for asymmetric synthesis

Suppression of STAT3 and HIF-1 Alpha Mediates Anti-Angiogenic Activity of Betulinic Acid in Hypoxic PC-3 Prostate Cancer Cells

Background: Signal transducer and activator of transcription 3 (STAT3) is a transcription factor that regulates various cellular processes such as cell survival, angiogenesis and proliferation. In the present study, we examined that betulinic acid (BA), a triterpene from the bark of white birch, had the inhibitory effects on hypoxia-mediated activation of STAT3 in androgen independent human prostate cancer PC-3 cells. Methodology/Principal Findings: BA inhibited the protein expression and the transcriptional activities of hypoxia-inducible factor-1a (HIF-1a) under hypoxic condition. Consistently, BA blocked hypoxia-induced phosphorylation, DNA binding activity and nuclear accumulation of STAT3. In addition, BA significantly reduced cellular and secreted levels of vascular endothelial growth factor (VEGF), a critical angiogenic factor and a target gene of STAT3 induced under hypoxia. Furthermore, BA prevented in vitro capillary tube formation in human umbilical vein endothelial cells (HUVECs) maintained in conditioned medium of hypoxic PC-3 cells, implying anti-angiogenic activity of BA under hypoxic condition. Of note, chromatin immunoprecipitation (ChiP) assay revealed that BA inhibited binding of HIF-1a and STAT3 to VEGF promoter. Furthermore, silencing STAT3 using siRNA transfection effectively enhanced the reduced VEGF production induced by BA treatment under hypoxia. Conclusions/Significance: Taken together, our results suggest that BA has anti-angiogenic activity by disturbing th

Novel CFTR Mutations in a Korean Infant with Cystic Fibrosis and Pancreatic Insufficiency

Cystic fibrosis (CF) is an autosomal recessive disease that is very rare in Asians: only a few cases have been reported in Korea. We treated a female infant with CF who had steatorrhea and failure to thrive. Her sweat chloride concentration was 102.0 mM/L. Genetic analysis identified two novel mutations including a splice site mutation (c.1766+2T>C) and a frameshift mutation (c.3908dupA; Asn1303LysfsX6). Pancreatic enzyme replacement and fat-soluble vitamin supplementation enabled the patient to get a catch-up growth. This is the first report of a Korean patient with CF demonstrating pancreatic insufficiency. CF should therefore be considered in the differential diagnosis of infants with steatorrhea and failure to thrive

Sammelrezension

1.) Hanft, Anke / Simmel, Annika (Hrsg.): Vermarktung von Hochschulweiterbildung. Waxmann Verlag: Münster, 2007. 192 S. ISBN 978-3-8309-1785-4. 2.) Bremer, Helmut: Soziale Milieus, Habitus und Lernen: Zur sozialen Selektivität des Bildungswesens am Beispiel der Weiterbildung. Juventa Verlag: Weinheim, 2007. 308 S. ISBN 978-37799-1585-0. 3.) Dust, Martin: 'Unser Ja zum neuen Deutschland': Katholische Erwachsenenbildung von der Weimarer Republik zur Nazi-Diktatur. Studien zur Bildungsreform, Bd. 49. Peter Lang: Frankfurt, 2007. 631 S. ISBN 978-3-631-55693-1. 4.) Gieseke, Wiltrud: Lebenslanges Lernen und Emotionen: Wirkungen von Emotionen auf Bildungsprozesse aus beziehungstheoretischer Perspektive. W. Bertelsmann Verlag: Bielefeld, 2007. 280 S. ISBN 978-3-7639-3331-0. 5.) Heuer, Ulrike / Siebers, Ruth: Weiterbildung am Beginn des 21. Jahrhunderts: Festschrift für Wiltrud Gieseke. Erwachsenenpädagogisches Institut Berlin e.V. Waxmann Verlag: Münster, 2007. 496 S. ISBN 978-3-8309-1811-0. 6.) Janetzko, Dietmar: Eigenlogik: Zur Rolle subjektiver Theorien bei der Bildungsmotivation. Waxmann Verlag: Münster, 2007. 188 S. ISBN 978-3-8309-1693-2. 7.) Kaiser, Arnim / Kaiser, Ruth / Hohmann, Reinhard (Hrsg.): Lernertypen - Lernumgebung - Lernerfolg: Erwachsene im Lernfeld. W. Bertelsmann Verlag: Bielefeld, 2007. 284 S. ISBN 978-3-7639-3560-4. 8.) Koerrenz, Ralf / Meilhammer, Elisabeth / Schneider, Käthe (Hrsg.): Wegweisende Werke zur Erwachsenenbildung. Verlag IKS Garamond: Jena, 2007. 613 S. ISBN 978-3-938203-51-4. 9.) Schiersmann, Christiane: Berufliche Weiterbildung: Lehrbuch. VS Verlag für Sozialwissenschaften: Wiesbaden, 2007. 272 S. ISBN 3-8100-3891-1. 10.) West, Linden / Alheit, Peter / Andersen, Anders Siig / Merill, Barbara (Hrsg.): Using Biographical and Life History Approaches in the Study of Adult and Lifelong Learning. European Perspectives European Studies in Lifelong Learning and Adult Learning Research, Vol. 2. Peter Lang Verlag: Frankfurt a. M., 2007. 310 S. ISBN 978-3-631-56286-4

Clinical practice guideline for the diagnosis and treatment of pediatric obesity: recommendations from the Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology Hepatology and Nutrition

The Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition newly developed the first Korean Guideline on the Diagnosis and Treatment of Obesity in Children and Adolescents to deliver an evidence-based systematic approach to childhood obesity in South Korea. The following areas were systematically reviewed, especially on the basis of all available references published in South Korea and worldwide, and new guidelines were established in each area with the strength of recommendations based on the levels of evidence: (1) definition and diagnosis of overweight and obesity in children and adolescents; (2) principles of treatment of pediatric obesity; (3) behavioral interventions for children and adolescents with obesity, including diet, exercise, lifestyle, and mental health; (4) pharmacotherapy; and (5) bariatric surgery