11 results on '"Ure, Benno M."'
Search Results
2. Quality of Life in Patients With Progressive Familial Intrahepatic Cholestasis: No Difference Between Post-liver Transplantation and Post-partial External Biliary Diversion
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Wassman, Sonja, Pfister, Eva-Doreen, Kuebler, Joachim F., Ure, Benno M., Goldschmidt, Imeke, Dingemann, Jens, Baumann, Ulrich, and Schukfeh, Nagoud
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- 2018
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3. The Esophageal-Atresia-Quality-of-life Questionnaires
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Dellenmark-Blom, Michaela, Dingemann, Jens, Witt, Stefanie, Quitmann, Julia H., Jönsson, Linus, Gatzinsky, Vladimir, Chaplin, John E., Bullinger, Monika, Flieder, Sofie, Ure, Benno M., Dingemann, Carmen, and Abrahamsson, Kate
- Abstract
Esophageal atresia (EA) is a rare malformation characterized of discontinuity of the esophagus, concurrent with or without a tracheoesophageal fistula (TEF). We report the feasibility validity and reliability of a condition-specific quality-of-life (QOL) tool for EA/TEF children, the age-adapted EA-QOL-questionnaires, when used in Sweden and Germany. A total of 124 families of children with EA/TEF participated in the study; 53 parents completed the EA-QOL-questionnaire for children aged 2 to 7 years; 62 children/71 parents the EA-QOL-questionnaire for children 8 to 17 years. Feasibility was determined from the percentage of missing item responses. Based on clinical data and previously validated generic QOL-instruments (PedsQL 4.0, DISABKIDS-12), the final EA-QOL scores were evaluated against hypotheses of validity (known-groups/concurrent/convergent) and reliability (internal consistency/retest reliability of scores for 3 weeks). Significant level was P< 0.05. In the questionnaire for EA/TEF children aged 2 to 7 years, 16/18 items were completed with missing values <6% (range 0%–7.5%), and in the questionnaire for 8 to 17-year-olds, 24/24 child-reported items (range 0%–4.8%) and 21/24 parent-reported items (range 0%–7.0%). In both age-specific EA-QOL-questionnaires, desirable standards for known-groups and concurrent validity were fulfilled; digestive symptoms and feeding difficulties negatively impacted EA-QOL-Total-scores (P< 0.001), and as hypothesized, in 2 to 7-year-olds, respiratory symptoms decreased EA-QOL-Total-scores (P= 0.002). Correlations between the EA-QOL and generic QOL questionnaires supported convergent validity. Internal consistency reliability was satisfactory. The level of agreements of EA-QOL-scores between the field- and retest study were good to excellent. The overall psychometric performance of the EA-QOL-questionnaires for EA/TEF children is satisfactory and can enhance outcome evaluations in future research and clinical practice.
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- 2018
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4. Copy number variations in 375 patients with oesophageal atresia and/or tracheoesophageal fistula
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Brosens, Erwin, Marsch, Florian, de Jong, Elisabeth M, Zaveri, Hitisha P, Hilger, Alina C, Choinitzki, Vera Gisela, Hölscher, Alice, Hoffmann, Per, Herms, Stefan, Boemers, Thomas M, Ure, Benno M, Lacher, Martin, Ludwig, Michael, Eussen, Bert H, van der Helm, Robert M, Douben, Hannie, Van Opstal, Diane, Wijnen, Rene M H, Beverloo, H Berna, van Bever, Yolande, Brooks, Alice S, IJsselstijn, Hanneke, Scott, Daryl A, Schumacher, Johannes, Tibboel, Dick, Reutter, Heiko, and de Klein, Annelies
- Abstract
Oesophageal atresia (OA) with or without tracheoesophageal fistula (TOF) are rare anatomical congenital malformations whose cause is unknown in over 90% of patients. A genetic background is suggested, and among the reported genetic defects are copy number variations (CNVs). We hypothesized that CNVs contribute to OA/TOF development. Quantifying their prevalence could aid in genetic diagnosis and clinical care strategies. Therefore, we profiled 375 patients in a combined Dutch, American and German cohort via genomic microarray and compared the CNV profiles with their unaffected parents and published control cohorts. We identified 167 rare CNVs containing genes (frequency<0.0005 in our in-house cohort). Eight rare CNVs – in six patients – were de novo, including one CNV previously associated with oesophageal disease. (hg19 chr7:g.(143820444_143839360)_(159119486_159138663)del) 1.55% of isolated OA/TOF patients and 1.62% of patients with additional congenital anomalies had de novo CNVs. Furthermore, three (15q13.3, 16p13.3 and 22q11.2) susceptibility loci were identified based on their overlap with known OA/TOF-associated CNV syndromes and overlap with loci in published CNV association case–control studies in developmental delay. Our study suggests that CNVs contribute to OA/TOF development. In addition to the identified likely deleterious de novo CNVs, we detected 167 rare CNVs. Although not directly disease-causing, these CNVs might be of interest, as they can act as a modifier in a multiple hit model, or as the second hit in a recessive condition.
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- 2016
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5. The viral dsRNA analogue poly (I:C) induces necrotizing enterocolitis in neonatal mice
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Ginzel, Marco, Yu, Yi, Klemann, Christian, Feng, Xiaoyan, von Wasielewski, Reinhard, Park, Joon-Keun, Hornef, Mathias W., Torow, Natalia, Vieten, Gertrud, Ure, Benno M., Kuebler, Joachim F., and Lacher, Martin
- Abstract
Background:Necrotizing enterocolitis (NEC) is a life-threatening gastrointestinal disease in premature infants with high mortality and morbidity with uncertain pathogenesis. Recent research focused on the role of intraluminal bacteria and lipopolysaccharide (LPS). However, an additional role of viral agents in the pathogenesis of NEC has recently been postulated. We assessed the role of polyinosinic:polycytidylic acid (pIC) mimicking viral dsRNA in contributing to the development of NEC in neonatal mice.Methods:Four-d-old C57BL/6J pups were stressed by asphyxia and hypothermia twice daily. Animals were either fed by formula only (FO), formula containing LPS or pIC. After 72?h, mice were euthanized, intestines harvested, and the severity of NEC was assessed.Results:Breastfed mice showed no evidence of NEC. Very mild NEC-like lesions were observed in mice fed by FO. Supplementation of LPS or pIC to the formula led to increased intestinal tissue damage and inflammation compared with FO in a similar manner.Conclusion:Our study demonstrates the ability of viral factors to induce NEC in neonatal mice even in the absence of LPS. Furthermore, we present a new mouse model of pIC-induced NEC which may be used to obtain further mechanistic insights in the pathogenesis of this disease.
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- 2016
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6. Laparoscopic Anterior Hemifundoplication Improves Key Symptoms Without Impact on GE in Children With and Children Without Neurodevelopmental Delays
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Engelmann, Carsten, Gritsa, Stella, Gratz, Klaus F, and Ure, Benno M
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We investigated the impact of laparoscopic anterior hemifundoplication on gastric emptying (GE) and specific symptoms in children with and children without neurodevelopmental delays gastroesophageal reflux. Scintigraphic and ultrasonographic GE measurements were correlated.
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- 2010
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7. Leptin accelerates enterocyte turnover during methotrexate-induced intestinal mucositis in a rat model
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Sukhotnik, Igor, Mogilner, Jorge G., Shteinberg, Dan, Karry, Rahel, Lurie, Michael, Ure, Benno M., Shaoul, Ron, and Coran, Arnold G.
- Abstract
Gastrointestinal mucositis occurs as a consequence of cytotoxic treatment. In the present study, we tested whether leptin can protect gut epithelial cells from methotrexate (MTX)-induced intestinal damage. Non-pretreated and pretreated with MTX Caco-2 cells were incubated with increasing concentrations of leptin for 24 hours. Cell proliferation and apoptosis were assessed using FACS analysis. Adult rats were divided into three experimental groups: Control rats; MTX-rats were treated with a single dose of MTX, and MTX- LEP rats were also treated with leptin for 3 days. Intestinal mucosal damage (Park's score), mucosal structural changes (bowel and mucosal weight, mucosal DNA and protein content, villus height and crypt depth), enterocyte proliferation, and enterocyte apoptosis were measured at sacrifice. RT-PCR was used to determine the level of bax and bcl-2 mRNA expression. In the vitro experiment, treatment with leptin of Caco-2 cells pre-treated with MTX resulted in a significant stimulation of cell proliferation and inhibition of cell apoptosis in a dose-dependent manner. In the vivo experiment, MTX-LEP rats demonstrated a greater jejunal and ileal bowel and mucosal weight, mucosal DNA and protein, villus height and crypt depth, as well as a greater enterocyte proliferation index compared to MTX-animals. MTX-LEP rats also showed a trend toward an increase in enterocyte apoptosis that was accompanied by an increase in bax mRNA and decrease in bcl-2 mRNA expression. In conclusion, leptin enhances proliferation and decreases apoptosis in Caco-2 cells pretreated with MTX. In a rat model of MTX-induced mucositis, treatment with leptin improves intestinal recovery and enhances enterocyte turnover.
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- 2009
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8. Oral Insulin Enhances Cell Proliferation and Decreases Enterocyte Apoptosis During Methotrexate-induced Mucositis in the Rat
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Sukhotnik, Igor, Shehadeh, Naim, Coran, Arnold G, Mogilner, Jorge G, Karry, Rahel, Shamian, Benhoor, Ure, Benno M, and Shamir, Raanan
- Abstract
Oral insulin (INS) has been shown to protect intestinal epithelial cells from injury caused by ischemia-reperfusion and endotoxemia. In the present study, we tested whether oral insulin can protect gut epithelial cells from methotrexate (MTX)-induced intestinal damage. Adult male Sprague-Dawley rats were divided into 3 experimental groups. Control rats were treated with normal saline given intraperitoneally (CONTR), MTX rats were treated with a single dose (20 µg/kg) of MTX given intraperitoneally, and MTX-INS rats were treated with oral insulin given in drinking water (1 U/mL) 72 hours after IP injection of a single dose of MTX (similar to MTX rats). Three days after either MTX or saline injection, rats were killed. Intestinal mucosal damage (Park injury score), mucosal structural changes, enterocyte proliferation, and enterocyte apoptosis were measured. Reverse transcription polymerase chain reaction was used to determine the level of bax and bcl-2 mRNA expression. MTX-INS rats demonstrated a greater jejunal and ileal mucosal weight, ileal mucosal DNA, greater jejunal villus height, greater jejunal and ileal crypt depth, greater enterocyte proliferation index in ileum, and lower enterocyte apoptosis in ileum than did MTX-nontreated animals. Treatment with insulin did not change the injury score grade in comparison with MTX animals. A significant decrease in cell apoptosis was observed in MTX-INS rats (vs MTX) and also a decrease in a bax mRNA expression and decrease in a bax/bcl-2 ratio. In a rat model of MTX-induced mucositis, oral insulin supplementation does not prevent mucosal injury but improves intestinal recovery and enhances enterocyte survival.
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- 2008
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9. Thorakoskopie im Kindesalter
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Metzelder, Martin L., Glüer, Sylvia, and Ure, Benno M.
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- 2007
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10. Type-I But Not Type-II Interferon Receptor Knockout Mice Are Susceptible to Biliary Atresia
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KUEBLER, JOACHIM F., CZECH-SCHMIDT, GERARD, LEONHARDT, JOHANNES, URE, BENNO M., and PETERSEN, CLAUS
- Abstract
The etiology of biliary atresia (BA) is not yet understood, but recent studies have shown inflammation with an up-regulated interferon (IFN) activity in the intra- and extrahepatic bile ducts of patients with BA. These findings support an inflammatory/infectious cause of BA as mimicked in our infective murine model. To study the role of the IFN receptors in our model, we used mice with inactivated INF-alpha/beta receptor A129, with inactivated IFN-gamma receptor G129, or inactivation of both interferon receptors AG129 as well as the wild type controls W129. Mice were infected with rotavirus within 48h of birth and 7 d postpartum. The incidence of BA in each group was determined during a 3 wk period. In the second week the virus load was measured. BA incidence was 76% in A129 and 67% in AG129 animals, whereas in the G129 group only 33% of the pups developed BA. The wild type presented with a BA-incidence of 15%, while 7 d old mice failed to develop BA. There was no significant difference in the virus load of the livers between the groups independent of clinical symptoms. In conclusion, inactivation of type I INF-receptor significantly increases the incidence of BA following postpartal rotavirus infection. This effect is independent of the presence of type II-INF-receptors. Thus, in our model a type I IFN-linked deregulation of the innate immune system appears to be crucial for the induction of biliary atresia.
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- 2006
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11. A prospective study of minimally invasive techniques in pediatric surgical oncology: preliminary report
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Warmann, Steven, Fuchs, Jörg, Jesch, Nathalie K., Schrappe, Martin, and Ure, Benno M.
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Thoracoscopic and laparoscopic techniques play a major role in pediatric surgery. However, minimally invasive surgery (MIS) has not yet established itself in pediatric surgical oncology. The authors present a prospective study investigating the role of MIS in children with cancer. All children with abdominal or thoracic tumors requiring surgery were registered between September, 2000 and February, 2002. Decisions regarding procedures and approachesconventional or minimally invasivewere made by the interdisciplinary team. Data on diagnoses, surgical procedures, complications, and conversion rates were registered prospectively. Seventy-four patients received 78 operations, 21 (26.9%) of the 78 operations were minimally invasive. Seven of 16 tumor biopsies (43.8%) and 9 of 57 tumor resections (15.8%) were performed using MIS, which was also exclusively used for diagnostic interventions. Conversions to standard techniques only occurred in 5 of 9 tumor resections. No major complications were encountered in the MIS group. MIS was practical in every fourth patient in our experience so far. It proved to be an excellent approach in diagnostic interventions and tumor biopsies, whereas efficacy is limited in tumor resections. Further factors (tumor recurrence, trocar site recurrence, tumor growth, and dissemination after CO
2 insufflation) have to be evaluated. Our data encourage the continuation of the study. Med Pediatr Oncol 2003;40:155157. © 2003 Wiley-Liss, Inc.- Published
- 2003
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