bluuebunny/crossref_metadata_2025_split huggingface dataset:
Gastroesophageal Reflux in Familial Dysautonomia: Correlation with Crisis Frequency and Sensory Dysfunction
Authors: Sundaram Vinay , Axelrod Felicia B 
Published: March 2005

ABSTRACTObjectives:Familial dysautonomia (FD), a genetic disorder resulting in sensory and autonomic nervous system dysfunction, is associated with a high frequency of gastroesophageal reflux (GER). In this study the authors attempted to determine if GER prevalence correlated with dysautonomic crisis frequency and small fiber sensory dysfunction.Methods:Of an initial population of 252 FD patients who had survived past their seventh year and were alive during the year of study from June 1, 2001 to June 1, 2002, 174 (69.0%) had sufficient data in their patient files to be included in this retrospective analysis. Subjects were stratified into three groups: those with no GER, those medically treated for GER and those surgically treated for GER with fundoplication. Data were obtained regarding frequency and severity of dysautonomic crises and sensory function including pain sensation, cold and warm thresholds and vibration perception.Results:There were no significant differences among the three groups regarding age or severity of sensory dysfunction. However, dysautonomic crises were significantly more frequent in FD patients who were presumed to have more intractable GER, as they had required fundoplication (P < 0.05).Conclusions:The data confirm the high incidence of GER in the FD population and demonstrate that GER is independent of severity of sensory dysfunction. In addition, the authors demonstrate that fundoplication does not alleviate the central autonomic cause of retching, the dysautonomic crisis.
Effects of Dexamethasone on Antral Mucosal Protein and Gastric Development in Postnatal Rats
Authors: Petri Michael K , Lee Ping C 
Published: March 2005

ABSTRACTBackground:The rat stomach undergoes major developmental changes postnatally. An antral mucosal protein (AMP‐18) with in vitro mitogenic activity has recently been found in the mammalian stomach.Methods:To evaluate the role of AMP‐18 in gastric development in postnatal rats, the AMP‐18 content of glandular stomach extracts from postnatal rats at different ages were measured by Western blots using anti‐AMP‐18 specific serum. Dexamethasone administration was used to evaluate the corticosteroid regulation of AMP‐18 expression.Results:AMP‐18 was detected only in glandular stomach homogenate and gastric mucus. AMP‐18 and pepsin levels were high at birth. Both decreased postnatally to low levels at 7 days of age, then increased at 10‐15 days of age and reached peak levels around weaning (18‐21 days of age). The glandular stomach showed stepwise growth relative to body weight, with a significant increase at 18‐20 days of age. The forestomach showed a decrease in growth relative to body weight during the same period. Dexamethasone given to pups before 7 days of age induced the accumulation of AMP‐18 and pepsin. Induction of AMP‐18 by dexamethasone was evident by 7 hours with a delayed increase in glandular stomach mass 30 hours after dexamethasone injection. Induction of AMP‐18 by dexamethasone was attenuated by RU‐486 but not by spironolactone.Conclusion:Increases in AMP‐18, pepsin and glandular stomach mass during normal postnatal development suggest that AMP‐18 might be involved in gastric maturation, at least in the glandular portion. Dexamethasone induction of pepsin and AMP‐18 and the subsequent increase in glandular stomach mass also suggest a possible role for AMP‐18 in glandular stomach maturation. Dexamethasone apparently acts through the type II corticosteroid receptor to induce AMP‐18.
Diet‐Induced Changes in Membrane Gangliosides in Rat Intestinal Mucosa, Plasma and Brain
Authors: Park Eek Joong , Suh Miyoung , Ramanujam Kal , Steiner Kurt , Begg David , Clandinin M Thomas 
Published: March 2005

ABSTRACTObjectives:The objective of this study was to determine if dietary gangliosides induce changes in the ganglioside content of intestinal mucosa, plasma and brain and to identify where GM3 and GD3 are localized in the enterocyte membrane.Methods:Male 18‐day‐old Sprague‐Dawley rats were fed a semipurified diet containing 20% (w/w) fat. The control diet contained triglyceride, reflecting the fat formulation of an existing infant formula. Two experimental diets were formulated by adding sphingomyelin (1% w/w of total fat) or a ganglioside‐enriched lipid (0.1% w/w of total fat) to the control diet fat. The ganglioside fraction of ganglioside‐enriched lipid diet contained more than 80% GD3. After 2 weeks of feeding, the total and individual ganglioside and cholesterol content was measured in small intestinal mucosa, plasma and brain.Results:The ganglioside‐enriched lipid diet significantly increased total gangliosides in the intestinal mucosa, plasma and brain compared with the control diet. The ganglioside‐enriched lipid diet significantly increased the level of GD3 (7.5% w/w) in the intestine compared with control (3.2% w/w) while decreasing the level of GM3, the major ganglioside in the intestine. The ratio of cholesterol to ganglioside in the intestinal mucosa, plasma and brain decreased significantly in rats fed the ganglioside‐enriched lipid diet compared with controls. Confocal microscopy showed that GM3 is exclusively localized in the apical membrane of the enterocyte whereas GD3 is primarily localized in the basolateral membrane.Conclusions:The authors conclude that dietary ganglioside is absorbed in the small intestine and transported to different membrane sites, altering ganglioside levels in the intestinal mucosa, plasma and brain and thus possibly having the potential to change developing enterocyte function (and possibly that of other cell lines).
Hepatocyte Glycogen Accumulation in Patients Undergoing Dietary Management of Urea Cycle Defects Mimics Storage Disease
Authors: Miles L , Heubi J E , Bove Kevin E 
Published: March 2005

ABSTRACTObjectives:Anecdotal reports have described excess hepatocyte glycogen in patients with urea cycle enzyme defects. Retrospectively, the authors evaluated the prevalence and possible cause of liver glycogen accumulation in such patients.Methods:The authors searched the files of the Division of Pathology at Cincinnati Children's Hospital from 1975 and 2004 for cases of urea cycle enzyme defects and identified 11 patients who had had liver biopsy performed and/or liver transplantation. All patients were on diets containing essential amino acids as the protein source before liver biopsy and/or transplantation.Results:All but one patient had focal or diffuse glycogen accumulation in hepatocytes in at least one specimen by light microscopic examination. Two young infants also had cholestasis. Electron microscopy performed on six patients showed diffuse or focal glycogen excess in the cytoplasm of individual hepatocytes. Biochemical studies of three patients revealed two with hepatic glycogen content in the upper normal range and one that was abnormally high. Glycolytic enzyme activities were normal in two patients, and one patient had low phosphorylase activity.Conclusions:Hepatocyte glycogen accumulation in urea cycle enzyme defects resembles that seen in glycogen storage disease but can be distinguished in most cases by non‐uniformity of distribution and/or the absence of sinusoidal compression by expanded hepatocytes. We speculate that therapeutic modification of dietary protein content by restriction to essential amino acids, including leucine, may promote glycogen accumulation by increasing insulin secretion.
The Bioavailability of Oral Methotrexate in Children with Inflammatory Bowel Disease
Authors: Stephens Michael C , Baldassano Robert N , York Amy , Widemann Brigitte , Pitney Aaron C , Jayaprakash Nalini , Adamson Peter C 
Published: March 2005

ABSTRACTObjectives:Methotrexate is used to treat patients with inflammatory bowel disease. Although no available pharmacologic data support the assumption that the bioavailability of methotrexate is diminished in patients with inflammatory bowel disease, most such patients receive methotrexate parenterally.Methods:The oral bioavailability of methotrexate was determined in 11 pediatric patients being treated with methotrexate for inflammatory bowel disease. Serial plasma methotrexate concentrations were determined after equal subcutaneous and oral doses of methotrexate.Results:The mean bioavailability of methotrexate in patients with inflammatory bowel disease was 84% ± 38%. Interpatient variability in drug exposure was similar after oral and subcutaneous administration.Conclusions:The bioavailability of methotrexate in patients with inflammatory bowel disease is no different from that observed in other disease states. Subcutaneous administration of methotrexate does not appear to decrease the interpatient variability in drug exposure. There is no sound pharmacologic basis for favoring administration of methotrexate via the subcutaneous route for patients with inflammatory bowel disease.
Randomized Trial Comparing Two Methods of Increasing Dietary Calcium Intake in Children with Inflammatory Bowel Disease
Authors: Stark Lori J , Hommel Kevin A , Mackner Laura M , Janicke David M , Davis Ann M , Pfefferkorn Marian , Crandall Wallace , Heubi James 
Published: March 2005

ABSTRACTObjective:To examine the efficacy of behavioral intervention compared with enhanced standard of care nutrition intervention on increasing dietary calcium intake of children with inflammatory bowel disease.Method:Children aged 5 to 12 years old (N = 32) were randomly assigned to the behavioral intervention or enhanced standard of care conditions. Three‐day food diaries collected on all participants at baseline and post‐treatment were analyzed for dietary calcium intake.Results:Children in the behavioral intervention group achieved a significantly greater mean increase in dietary calcium intake (M= 984 mg) than children in the enhanced standard of care group (M = 274 mg) (P < 0.05). In the behavioral intervention group 81% of children achieved the daily calcium intake goal of 1500 mg/day compared with only 19% of children in the enhanced standard of care group, (P < 0.05).Conclusions:Behavioral modification appears to be significantly more effective than nutrition education alone in modifying calcium intake in children with inflammatory bowel disease.
Gastrointestinal Symptoms Associated with Orthostatic Intolerance
Authors: Sullivan Sean D , Hanauer Joseph , Rowe Peter C , Barron Diana F , Darbari Anil , Oliva‐Hemker Maria 
Published: March 2005

ABSTRACTBackground:The term orthostatic intolerance is used to describe symptoms of hemodynamic instability such as lightheadedness, fatigue, impaired cognition and syncope that develop on assuming an upright posture. Common forms of orthostatic intolerance in childhood include postural tachycardia syndrome and neurally mediated hypotension.Objective:A descriptive report of the clinical characteristics of patients presenting with gastrointestinal symptoms who are ultimately found to have orthostatic intolerance.Methods:A medical record review of all patients referred to the pediatric gastroenterology service at the Johns Hopkins Children's Center who had an abnormal tilt table test between June 1996 and December 2000.Results:Of 24 eligible subjects aged 9‐17 years (mean, 14.3 years), four had postural tachycardia syndrome, eight had both postural tachycardia and neurally mediated hypotension, and 12 had neurally mediated hypotension alone. The most common presenting gastrointestinal symptoms were abdominal pain, nausea and vomiting. Median number of gastrointestinal symptoms per patient was 3 (range, 1‐7), and 87% of the patients experienced gastrointestinal symptoms for more than 1 year and 48% experienced gastrointestinal symptoms for more than 3 years. Follow‐up information was available on 18 patients. Seventy‐eight percent of patients (14 of 18) had complete resolution of symptoms with treatment of orthostatic intolerance.Conclusion:Pediatric patients with chronic upper gastrointestinal symptoms may have underlying orthostatic intolerance. In patients with upper gastrointestinal symptoms and orthostatic intolerance, treatment of orthostatic intolerance may result in resolution of gastrointestinal symptoms.
Fecal Elastase: Pancreatic Status Verification and Influence on Nutritional Status in Children with Cystic Fibrosis
Authors: Cohen Jacqueline R , Schall Joan I , Ittenbach Richard F , Zemel Babette S , Stallings Virginia A 
Published: March 2005

ABSTRACTObjectives:To evaluate fecal elastase 1 (FE) levels in young children with cystic fibrosis and pancreatic insufficiency and to explore the relationship between FE and growth, nutrition, pulmonary status and fat absorption over a 24‐month period.Methods:FE, indicating pancreatic lipase activity, was assessed in children (6.0 to 8.9 years of age) with cystic fibrosis and pancreatic insufficiency. FE >15 μg/g stool was defined as residual pancreatic activity, suggesting some pancreatic function, and FE ≤15 μg/g stool was defined as no pancreatic activity, suggesting pancreatic insufficiency. Seven‐day weighed food records were collected and coefficient of fat absorption was calculated using dietary records and 72‐hour stool collection. Height adjusted for mid‐parental height, weight, body mass index, upper arm muscle area and upper arm fat area were measured and Z scores were calculated. Forced expiratory volume in 1 second was the measure of pulmonary function.Results:Ten children (12%) had residual pancreatic activity (range, 17 to 667 μg/g stool) and 75 had no pancreatic activity. Children with no pancreatic activity had significantly lower coefficients of fat absorption than did those with residual pancreatic activity (81% ± 14% versus 94% ± 3%; P = 0.009) and significantly lower adjusted height Z score (−0.8 ± 1.0 versus −0.1 ± 1.3; P = 0.03). Children with residual pancreatic activity had significantly greater improvement in weight for age Z scores, upper arm muscle area Z scores, and upper arm fat area Z scores than children with no pancreatic activity (P ≤ 0.01). Children with no pancreatic activity were significantly (P = 0.007) more likely to have the ΔF508/ΔF508 genotype than were those with residual pancreatic activity (61% versus 11%).Conclusions:These data suggest that a substantial number (12% in this sample) of children with cystic fibrosis have a misclassified pancreatic status. Children with residual FE had greater fat absorption and improved growth and nutritional status over 24 months. FE assessment should be used to verify pancreatic status in patients with cystic fibrosis.
Pediatric Gastroenterology Workforce Survey, 2003‐2004
Authors: Crandall Wallace V , Barnard John A , Cohen Mitchell B , Colletti Richard B , Ferry George D , Harnsberger Janet K , Hart Michael H , Hayes John R , Rosenthal Philip , Sherman Philip M , Smith Lesley J ,   A Report of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition
Published: March 2005

ABSTRACTBackground:The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) performed a workforce survey to determine the number, distribution, and work‐related activities of pediatric gastroenterologists in the United States of America (USA) and Canada, and compared these findings with the first workforce survey completed in 1996.Methods:The survey queried pediatric gastroenterologists in the USA and Canada between November, 2003 and June 2004. To permit the optimal comparison to the 1996 survey data, the original survey was used as a template for development of the current instrument and, when possible, the questions were left unchanged. Additional questions were added to address important contemporary issues not present in the initial survey. Limited income information was also collected. The survey was posted on the NASPGHAN website, and the NASPGHAN membership was notified of the survey by electronic mail via its electronic mail distribution list. This was followed by a three‐part postal mail survey to all non‐respondents. After the Internet and postal mail requests, all non‐respondent physicians were telephoned a minimum of three times. If unsuccessful in contacting the physicians directly, office personnel were queried to facilitate survey completion regarding the provision of pediatric gastroenterology, nutrition or hepatology services in either clinical care or research.Results:The response rate based on the potential contact list for Part I of the survey was 69%. The final phone call or electronic mail contact of an office staff member with questions regarding gender and delivery of pediatric gastroenterology services yielded a total contact rate of 88%. There were 699 pediatric gastroenterologists identified in North America, as compared with 672 in 1996. If known non‐respondents are included, there could be as many as 794 pediatric gastroenterologists. Time spent in clinical activities increased from 60% to 66% in the USA and from 43% to 53% in Canada. The use of nurse practitioners and physician assistants has increased considerably over the past 7 years. Fifty‐three percent of respondents feel there are too few pediatric gastroenterologists. Fifty percent of section and practice heads report that they are currently recruiting partners. Limited income information is presented.Conclusions:There is currently a self‐perceived shortage of pediatric gastroenterologists as compared with 7 years ago, despite a constant proportion of pediatric gastroenterologists per million children. In the USA, nurse practitioners and physician assistants are being increasingly used to fill this need, and physicians in both Canada and the USA have increased the time they spend in clinical care.
Transmission dynamics of parasitic sea lice from farm to wild salmon
Authors: Krkošek Martin , Lewis Mark A , Volpe John P 
Published: April 2005

Marine salmon farming has been correlated with parasitic sea lice infestations and concurrent declines of wild salmonids. Here, we report a quantitative analysis of how a single salmon farm altered the natural transmission dynamics of sea lice to juvenile Pacific salmon. We studied infections of sea lice (
Lepeophtheirus salmonis
and
Caligus clemensi
) on juvenile pink salmon (
Oncorhynchus gorbuscha
) and chum salmon (
Oncorhynchus keta
) as they passed an isolated salmon farm during their seaward migration down two long and narrow corridors. Our calculations suggest the infection pressure imposed by the farm was four orders of magnitude greater than ambient levels, resulting in a maximum infection pressure near the farm that was 73 times greater than ambient levels and exceeded ambient levels for 30 km along the two wild salmon migration corridors. The farm-produced cohort of lice parasitizing the wild juvenile hosts reached reproductive maturity and produced a second generation of lice that re-infected the juvenile salmon. This raises the infection pressure from the farm by an additional order of magnitude, with a composite infection pressure that exceeds ambient levels for 75 km of the two migration routes. Amplified sea lice infestations due to salmon farms are a potential limiting factor to wild salmonid conservation.
A gender‐based categorization for conflict resolution
Authors: Brahnam Sheryl D. , Margavio Thomas M. , Hignite Michael A. , Barrier Tonya B. , Chin Jerry M. 
Published: March 2005

PurposeAs the workforce becomes increasingly diversified, it becomes increasingly important for managers to understand the conflict resolution attitudes brought to information systems (IS) by both men and women. This research was designed to investigate assumptions that may exist regarding the relationship between gender and conflict resolution. Specifically, the intent of this study was to compare the conflict resolution strategies of males and females majoring in IS in order to determine if gender‐based differences exist.Design/methodology/approachThe Thomas‐Kilmann Conflict Mode Instrument was utilized to assess the conflict resolution styles of 163 traditional‐age (18‐22) students enrolled in undergraduate IS courses at a large Midwestern university. Both ANOVA andt‐test analyses were utilized to investigate the relationship between gender and conflict resolution style.FindingsResults of this study indicate that, when compared with their male counterparts, women are more likely to utilize a collaborative conflict resolution style and men are more likely to avoid conflict. As collaboration is generally considered more productive and avoidance more disruptive in the conflict resolution process, the study suggests that women may possess more effective conflict resolution attributes than their male counterparts.Originality/valueThe results of this paper lend support to the theory that an individual's gender may be related to the development of conflict resolution styles. These findings also support the premise that female students in IS are highly adapted with regard to their ability to work collaboratively (and thereby successfully) in situations where conflict is likely to occur.
The evolving role of strategic management development
Authors: Brown Paul 
Published: March 2005

PurposeTo validate conceptual frameworks for strategic management development. Also, to test the hypothesis that the objectives and design of a strategic management development programme need to match the organisation's level of commitment to strategic management and the degree of maturity of its strategic management processes and competencies, in order that the programme can be effective in enhancing the strategic capability of the organisation.Design/methodology/approachEarlier work by the author (based on literature review and one case study) had generated two conceptual models which could help in the understanding of strategic management development. One provides a life‐cycle typology matching an organisation's level of commitment to strategic management with the design of an effective strategic management development programme. The second provides a causal network showing how strategic management capability may be developed. In this paper case study research is reported from six organisations to provide data, which are mainly qualitatative, to test the hypothesis and conceptual models.FindingsBoth the life‐cycle typology and the conceptual models are supported by the further case study work. It was also found that the dominant strategy‐making mode in the organisation can influence the potential for strategic management development. Where the command mode of strategy making is dominant the strategic aspects of a management development programme are inhibited because this is not consistent with the command culture.Research limitations/implicationsThe generalisability of the findings is constrained by the small sample size of six organisations. However, given the paucity of theory in the field of strategic management development, the findings contribute to the conceptual understanding of this subject.Originality/valueThe models proposed give insights into the complexities of strategic management development and can be used to inform analysis and planning of more effective strategic management development interventions.