bluuebunny/crossref_metadata_2025_split huggingface dataset:
CDDUX: A Disease‐specific Health‐related Quality‐of‐life Questionnaire for Children With Celiac Disease
Authors: van Doorn Roesja K , Winkler Lex MF , Zwinderman Koos H , Mearin M Luisa , Koopman Hendrik M 
Published: March 2009

ABSTRACTObjective:The development of a disease‐specific, health‐related, quality‐of‐life questionnaire for children ages 8 to 18 with celiac disease (CD), together with a parent‐as‐proxy version.Materials and Methods:We used a focus‐group method (bottom‐up approach) to investigate the impact of CD on children's everyday lives and selected 24 items to create a preliminary disease‐specific questionnaire. This questionnaire, together with the complementary generic quality‐of‐life questionnaire DUX‐25, was sent to 756 children with CD in the Netherlands and was returned by 530 of them. With the help of statistical analyses (Cronbach α, factor analysis, Pearson correlation, Student t test, paired samples t test, and item response theory), we tested the psychometric performance of the 24 items.Results:We reduced the questionnaire to 12 items: the Celiac Disease DUX (CDDUX). The CDDUX has 3 subscales: “Communication” (3), “Diet” (6), and “Having CD” (3). This questionnaire proved to be reliable, valid, and feasible and able to discriminate between perception of severity in cases of CD as assessed by parents.Conclusions:Children with a better perception of their own health status have a higher score on the CDDUX questionnaire. The whole group seems to have a lower quality of life than the healthy reference group on all domains of the DUX‐25. The new disease‐specific questionnaire CDDUX provides information about how children with CD think and feel about their illness. The questionnaire may enable researchers and clinicians to determine the consequences of this illness and the effects of clinical interventions on several aspects of daily living.
Chubby Face and the Biochemical Parameters for the Early Diagnosis of Neonatal Intrahepatic Cholestasis Caused by Citrin Deficiency
Authors: Chen Hung‐Wen , Chen Huey‐Ling , Ni Yen‐Hsuan , Lee Ni‐Chung , Chien Yin‐Hsiu , Hwu Wuh‐Liang , Huang Yuan‐Te , Chiu Pao‐Chin , Chang Mei‐Hwei 
Published: March 2009

ABSTRACTObjectives:To identify facial and biochemical characteristics as early clinical features of neonatal intrahepatic cholestasis due to citrin deficiency (NICCD).Patients and Methods:Ten patients with diagnoses of NICCD by SLC25A13 mutation analysis in Taiwan were recruited. A “Chubby Index” was developed for objective measurement of their facial characteristics. Liver function profiles were analyzed and compared with data on neonatal hepatitis and biliary atresia.Results:Chubby face was observed in early infancy in all 5 patients whose serial photographs were taken. A significant difference in the Chubby Index was seen between NICCD infants and healthy infants (1.331 ± 0.07 vs 1.068 ± 0.059; P < 0.05). NICCD is characterized by an aspartate aminotransferase–to–alanine aminotransferase ratio of 2 or greater, a direct bilirubin–to–total bilirubin ratio under 0.67, and a standard deviation score for α‐fetoprotein of 4 or greater, with respect to neonatal hepatitis and biliary atresia. Although chubby face, abnormal liver function profiles, and multiple amino acidemia gradually disappeared after age 1 year, an increase in hepatic echogenicity was observed in most patients in long‐term follow‐up.Conclusions:Our Chubby Index is an informative measurement of the facial characteristics of infants with NICCD. The chubby face features, along with an aspartate aminotransferase–to–alanine aminotransferase ratio of 2 or greater, a direct bilirubin–to–total bilirubin ratio under 0.67, and a standard deviation score for α‐fetoprotein of 4 or greater, may serve as useful clinical indicators for diagnosing NICCD early in infancy.
Safety and Efficacy of Adalimumab in Pediatric Patients With Crohn Disease
Authors: Wyneski Matthew J , Green Alex , Kay Marsha , Wyllie Robert , Mahajan Lori 
Published: March 2009

ABSTRACTObjectives:Adalimumab has recently become available for adult patients with Crohn disease (CD) as a viable alternative tumor necrosis factor‐α inhibitor to infliximab. To our knowledge, there have been no studies reviewing the use of adalimumab in pediatric patients with CD. Our aim was to examine the safety and efficacy of adalimumab therapy in pediatric patients with CD.Patients and Methods:We performed a retrospective chart review of 15 pediatric patients with CD who received adalimumab at a single institution between January 2003 and March 2007. All of the patients had a history of an attenuated response or anaphylaxis to infliximab. Each patient's chart was reviewed for age at diagnosis, sex, extent of disease, age at start of adalimumab therapy, course of therapy, side effects noted during therapy, concurrent medications, and response to adalimumab. Clinical response to adalimumab was classified as complete, partial, or no response based on the patients' ability to be weaned from steroids, increased or decreased need for steroids, or need for surgery during the course of treatment. This study was approved by the Cleveland Clinic Institutional Review Board.Results:Fifteen pediatric patients with CD received adalimumab for a 33‐month period. Of those, 14 patients had adequate follow‐up, and 1 patient was lost to follow‐up. The mean age at initiation of therapy was 16.6 years (median 17.9 years, range 10.3–21.8 years, SD 3.1 years). The majority of patients received an 80‐mg loading dose administered subcutaneously and 40‐mg doses subsequently every 2 weeks. The median duration of therapy was 6.5 months (range 1–31 months). A total of 272 injections were given. Of the 14 patients with sufficient data for follow‐up, 7 (50%) had a complete response, 2 (14%) had a partial response, and 5 (36%) had no response to adalimumab. Complete response was achieved after a median of 5 injections (range 3–11). Of the 14 patients with adequate follow‐up, 5 had fistulizing disease; 3 of these maintained fistula closure, 1 had temporary closure, and 1 required surgery to assist with closure. Twenty‐six adverse events occurred during therapy. Eight (57%) patients had at least 1 adverse effect. The most common events were abdominal pain and nausea. No serious adverse events were reported, no serious infections occurred, and no adverse events required discontinuation of adalimumab.Conclusions:Adalimumab was well tolerated in pediatric patients with CD. Complete or partial response was observed in 64% of patients. No serious adverse events occurred during therapy. Additional studies are needed to evaluate the efficacy and to determine optimal dosing of adalimumab in the pediatric population with CD.
Comparison of Epidermal Growth Factor and Heparin‐binding Epidermal Growth Factor–like Growth Factor for Prevention of Experimental Necrotizing Enterocolitis
Authors: Dvorak Bohuslav , Khailova Ludmila , Clark Jessica A , Hosseini Dania Molla , Arganbright Kelly M , Reynolds Charity A , Halpern Melissa D 
Published: March 2009

ABSTRACTBackground:Necrotizing enterocolitis (NEC) is a devastating gastrointestinal disease of prematurely born infants. Epidermal growth factor (EGF) and heparin‐binding EGF‐like growth factor (HB‐EGF) have protective effects against intestinal injury. The aim of this study was to compare the effect of oral administration of HB‐EGF, EGF, or both on the incidence of NEC in a neonatal rat model.Materials and Methods:Premature rats were fed by hand and exposed to asphyxia and cold stress to develop NEC. Four diets were used: formula (NEC), formula supplemented with 500 ng/mL HB‐EGF (HB), 500 ng/mL EGF (EGF), or a combination of both (E+HB). Ileal injury, endogenous HB‐EGF production, expression of EGF receptors, goblet cell density, and expression of apoptotic proteins were evaluated.Results:Oral administration of either EGF or HB‐EGF significantly reduced the incidence of NEC; however, EGF provided better protection in physiologically relevant doses. Simultaneous administration of both growth factors did not result in any synergistic protective effect against NEC. There were no significant differences between treatment groups in ileal gene expression of EGF receptors or HB‐EGF. However, the balance of apoptotic proteins in the ileum was shifted in favor of cell survival in EGF‐treated rats. This mechanism may be responsible for the higher efficiency of EGF protection against NEC.Conclusions:These data suggest that a physiological dosage of EGF or a pharmacological dosage of HB‐EGF could be used for prevention of NEC.
Overview of Pediatric Short Bowel Syndrome
Authors: Duro Debora , Kamin Daniel , Duggan Christopher 
Published: March 2009

ABSTRACTShort bowel syndrome (SBS) is a malabsorptive state occuring as a result of surgical resection or congenital disease of a significant portion of the small intestine (1). The amount of resection or remaining bowel generally dictates the degree of malabsorption and consequentely the need for specialized enteral nutrition or parenteral nutrition (PN). Intestinal failure in the context of SBS is defined as a dependence on PN to maintain minimal energy and fluid requirement for growth in children. Common causes of SBS in infants and children include necrotizing enterocolitis, midgut volvulus, intestinal atresia, and gastroschisis. Early identification of patients at risk for long‐term PN dependency is the first step toward avoiding severe complications. Close monitoring of nutritional status, steady and early introduction of enteral nutrition, and aggressive prevention, diagnosis, and treatment of infections such as central venous catheter sepsis and bacterial overgrowth can significantly improve the prognosis. Intestinal transplantation is an emerging treatment that may be considered when intestinal failure is irreversible and children are experiencing serious complications related to TPN administration.
Omega‐3 LC‐PUFA Supply and Neurological Outcomes in Children With Phenylketonuria (PKU)
Authors: Koletzko Berthold , Beblo Skadi , Demmelmair Hans , Hanebutt Fabienne L 
Published: March 2009

ABSTRACTChildren with phenylketonuria (PKU) follow a diet with very low intakes of natural protein, which is devoid of food sources of the omega‐3 docosahexaenoic acid (DHA). A resulting DHA depletion has been demonstrated in PKU children and may account for detectable subtle neurological deficits that are not explained by variation in plasma phenylalanine concentrations. We supplemented 36 children with PKU ages 1 to 11 years for 3 months with encapsulated fish oil providing a daily dose of 15 mg DHA/kg body weight. DHA supplementation resulted in significantly faster visual evoked potential latencies, indicating more rapid central nervous system information processing. In addition, DHA significantly improved outcomes in a test of motor function and coordination. No changes over time were seen in age‐matched healthy controls. Because the PKU children had a good supply of the omega‐3 precursor alpha‐linolenic acid, these observations lead us to conclude that endogenous conversion of alpha‐linolenic acid is not sufficient to provide adequate amounts of DHA that support optimal function, and hence DHA appears to be a conditional essential substrate for children with PKU. Because early treated PKU children are healthy, with normal fatty acid turnover, these data may indicate a need to supply some DHA to children in general. Further studies are ongoing aiming at establishing quantitative DHA requirements in children.
Early Flavor Learning and Its Impact on Later Feeding Behavior
Authors: Beauchamp Gary K , Mennella Julie A 
Published: March 2009

ABSTRACTIn this review, we first outline the role and mechanisms of the chemical senses (taste, smell, and chemical irritation) in the perception of the flavor of a food or beverage. We then describe research findings, much of them from our laboratories, on the ontogeny of flavor perception and the interacting roles of innate responses and learning in the establishment of flavor preference of infants and children. Broadly, taste (sweet, sour, salty, bitter, umami, or savory) preferences have a strong innate component. Sweet, umami, and salty substances are innately preferred, whereas bitter and many sour substances are innately rejected. Nevertheless, these innate tendencies can be modified by pre‐ and postnatal experiences. Volatile components of flavor, detected by the olfactory system, are strongly influenced by early exposure and learning beginning in utero and continuing during early milk (breast milk or formula) feedings. These experiences set the stage for later food choices and are important in establishing life‐long food habits. As many of the diseases plaguing developed and developing societies involve excess consumption of some foods, an understanding of factors that determine choice and ingestion, particularly an understanding of the early factors, is important in designing strategies to enhance the health of the infant, child, and adult.
Nitric Oxide Is Involved in Cadmium-Induced Programmed Cell Death in Arabidopsis Suspension Cultures
Authors: De Michele Roberto , Vurro Emanuela , Rigo Chiara , Costa Alex , Elviri Lisa , Di Valentin Marilena , Careri Maria , Zottini Michela , Sanità di Toppi Luigi , Lo Schiavo Fiorella 
Published: March 2009

AbstractExposure to cadmium (Cd2+) can result in cell death, but the molecular mechanisms of Cd2+ cytotoxicity in plants are not fully understood. Here, we show that Arabidopsis (Arabidopsis thaliana) cell suspension cultures underwent a process of programmed cell death when exposed to 100 and 150 μ m CdCl2 and that this process resembled an accelerated senescence, as suggested by the expression of the marker senescence-associated gene12 (SAG12). CdCl2 treatment was accompanied by a rapid increase in nitric oxide (NO) and phytochelatin synthesis, which continued to be high as long as cells remained viable. Hydrogen peroxide production was a later event and preceded the rise of cell death by about 24 h. Inhibition of NO synthesis by N G-monomethyl-arginine monoacetate resulted in partial prevention of hydrogen peroxide increase, SAG12 expression, and mortality, indicating that NO is actually required for Cd2+-induced cell death. NO also modulated the extent of phytochelatin content, and possibly their function, by S-nitrosylation. These results shed light on the signaling events controlling Cd2+ cytotoxicity in plants.
The Phloem-Delivered RNA Pool Contains Small Noncoding RNAs and Interferes with Translation
Authors: Zhang Shoudong , Sun Li , Kragler Friedrich 
Published: March 2009

AbstractIn plants, the vascular tissue contains the enucleated sieve tubes facilitating long-distance transport of nutrients, hormones, and proteins. In addition, several mRNAs and small interfering RNAs/microRNAs were shown to be delivered via sieve tubes whose content is embodied by the phloem sap (PS). A number of these phloem transcripts are transported from source to sink tissues and function at targeted tissues. To gain additional insights into phloem-delivered RNAs and their potential role in signaling, we isolated and characterized PS RNA molecules distinct from microRNAs/small interfering RNAs with a size ranging from 30 to 90 bases. We detected a high number of full-length and phloem-specific fragments of noncoding RNAs such as tRNAs, ribosomal RNAs, and spliceosomal RNAs in the PS of pumpkin (Cucurbita maxima). In vitro assays show that small quantities of PS RNA molecules efficiently inhibit translation in an unspecific manner. Proof of concept that PS-specific tRNA fragments may interfere with ribosomal activity was obtained with artificially produced tRNA fragments. The results are discussed in terms of a functional role for long distance delivered noncoding PS RNAs.
Case of lupus vulgaris diagnosed 50 years after onset
Authors: UTTAWICHAI Pattanawadee , IGARASHI Tsukasa , KAWANA Seiji 
Published: March 2009

ABSTRACTCutaneous tuberculosis is an infrequent form of extrapulmonary tuberculosis, but is a symptom that can lead to diagnosis of tuberculosis. We describe a case of lupus vulgaris in a 79‐year‐old woman who had a 50‐year history of a slowly growing plaque on her right cheek. She visited many hospitals without resolution and the plaque gradually enlarged. Recently, she was misdiagnosed with eczema and prescribed topical steroids that had no effect, and she subsequently visited our outpatient clinic. A diagnosis of lupus vulgaris was made based on histopathology, culture and polymerase chain reaction, and isoniazid, rifampicin and ethambutol were administered as antituberculosis treatment. Although the incidence of cutaneous tuberculosis has decreased significantly in developed countries, knowledge and awareness of the disease are still of importance for proper diagnosis and treatment.
Genetic mapping of quantitative trait loci for resistance to Haemonchus contortus in sheep
Authors: Marshall K. , Maddox J. F. , Lee S. H. , Zhang Y. , Kahn L. , Graser H.‐U. , Gondro C. , Walkden‐Brown S. W. , Van Der Werf J. H. J. 
Published: March 2009

SummaryThis paper presents results from a mapping experiment to detect quantitative trait loci (QTL) for resistance to Haemonchus contortus infestation in merino sheep. The primary trait analysed was faecal worm egg count in response to artificial challenge at 6 months of age. In the first stage of the experiment, whole genome linkage analysis was used for broad‐scale mapping. The animal resource used was a designed flock comprising 571 individuals from four half‐sib families. The average marker spacing was about 20 cM. For the primary trait, 11 QTL (as chromosomal/family combinations) were significant at the 5% chromosome‐wide level, with allelic substitution effects of between 0.19 and 0.38 phenotypic standard deviation units. In general, these QTL did not have a significant effect on faecal worm egg count recorded at 13 months of age. In the second stage of the experiment, three promising regions (located on chromosomes 1, 3 and 4) were fine‐mapped. This involved typing more closely spaced markers on individuals from the designed flock as well as an additional 495 individuals selected from a related population with a deeper pedigree. Analysis was performed using a linkage disequilibrium–linkage approach, under additive, dominant and multiple QTL models. Of these, the multiple QTL model resulted in the most refined QTL positions, with resolutions of <10 cM achieved for two regions. Because of the moderate size of effect of the QTL, and the apparent age and/or immune status specificity of the QTL, it is suggested that a panel of QTL will be required for significant genetic gains to be achieved within industry via marker‐assisted selection.
Historical biogeography of the endemic Campanulaceae of Crete
Authors: Cellinese Nicoletta , Smith Stephen A. , Edwards Erika J. , Kim Sang‐Tae , Haberle Rosemarie C. , Avramakis Manolis , Donoghue Michael J. 
Published: March 2009

AbstractAim  The clade Campanulaceae in the Cretan area is rich in endemics, with c. 50% of its species having restricted distributions. These species are analysed in the context of a larger phylogeny of the Campanulaceae. Divergence times are calculated and hypotheses of vicariance and dispersal are tested with the aim of understanding whether Cretan lineages represent remnants of an older continental flora.Location  The Cretan area: Crete and the Karpathos Islands (Greece).Methods  We obtained chloroplast DNA sequence data from rbcL, atpB and matK genes for 102 ingroup taxa, of which 18 are from the Cretan area, 11 are endemics, and two have disjunct, bi‐regional distributions. We analysed the data using beast, a Bayesian approach that simultaneously infers the phylogeny and divergence times. We calibrated the tree by placing a seed fossil in the phylogeny, and used published age estimates as a prior for the root.Results  The phylogenetic reconstruction shows that all Campanula species fall within a well‐supported campanuloid clade; however, Campanula is highly polyphyletic. The Cretan endemics do not form a monophyletic group, and species are scattered throughout the campanuloid clade. Therefore, the Cretan taxa did not evolve following a single vicariance or dispersal event. Most Cretan lineages represent remnants of an older continental flora, with the exception of one clade that radiated in situ after island isolation, and one lineage that appears to have arrived by dispersal.Main conclusions  Most Cretan species were present in the islands at the time of their isolation, and very little long‐distance dispersal to Crete and diversification within Crete has occurred since then. Endemism is probably driven by loss of species on the mainland after island isolation. Species on the islands may have been more widespread in the past, but they are now restricted to often inaccessible areas, probably as a result of human pressure.
Nurses’ use of computerised clinical decision support systems: a case site analysis
Authors: Dowding Dawn , Mitchell Natasha , Randell Rebecca , Foster Rebecca , Lattimer Valerie , Thompson Carl 
Published: March 2009

Aims and objectives.  To explore how nurses use computerised clinical decision support systems in clinical practice and the factors that influence use.Background.  There is limited evidence for the benefits of computerised clinical decision support systems in nursing, with the majority of existing research focusing on nurses’ use of decision support for telephone triage. Research has suggested that several factors including nurses’ experience, features of the technology system and organisational factors may influence how decision support is used in practice.Design.  A multiple case site study.Methods.  Four case sites were purposively selected to provide variation in staff experience, technology used and decisions supported by the technology. Data were collected in each case site using non‐participant observation of nurse/patient consultations (n = 115) and interviews with nurses (n = 55). Data were analysed using thematic content analysis.Results.  Computerised decision support systems were used in a variety of ways by nurses, including recording information, monitoring patients’ progress and confirming decisions that had already been made. Nurses’ experience with the decision and the technology affected how they used a decision support system and whether or not they over‐rode recommendations made by the system. The ability of nurses to adapt the technology also affected its use.Conclusions.  How nurses use computerised decision support appears to be the result of an interaction between a nurses’ experience and their ability to adapt the technology to ‘fit’ with local clinical practice.Relevance to clinical practice.  One of the stated aims of introducing computerised decision support systems to assist nursing practice is to reduce variation and/or the number of errors associated with clinical practice. The study found unanticipated uses in such systems such as the routine over‐riding of recommendations which could lead to an increase rather than a decrease in variation or errors.