bluuebunny/crossref_metadata_2025_split huggingface dataset:
Early Onset of Ulcerative Colitis: Long‐term Follow‐up With Special Reference to Colorectal Cancer and Primary Sclerosing Cholangitis
Authors: Lindberg Jan , Stenling Roger , Palmqvist Richard , Rutegård Jörgen 
Published: February 2010

ABSTRACTObjectives:Ulcerative colitis (UC) is associated with an increased risk of colorectal cancer (CRC) and primary sclerosing cholangitis (PSC). The onset of UC at a young age has been considered a specific risk factor for CRC. This study reports the outcome with respect to DNA aneuploidy, dysplasia, CRC, and PSC in a cohort of patients with early‐onset UC from a defined catchment area who were followed up for 45 years.Patients and Methods:The study period was from 1961 through 2005. In all, 46 children and adolescents, ages 18 or younger from our catchment area, were affected with UC during this time. Data were collected from our colonoscopic surveillance program and other medical records in the same hospital.Results:The incidence rate of UC was 1.6 children or adolescents per 100,000 inhabitants per year. The onset of disease was at age 14 years (mean and median), and the observed duration of disease was 0 to 44 years. Five patients died, 3 of them of intercurrent disease. CRC developed in 1 patient, and no death caused by this disease was observed. PSC was diagnosed in 4 patients, in all of whom the onset of UC occurred before age 15.Conclusions:The earlier reports of increased risk of CRC in patients with early‐onset UC was not seen in this study. This could be due to a high frequency of surgery together with a well‐functioning surveillance program with excellent compliance. The recommendation of surgery in cases of high‐grade dysplasia or repeated findings of low‐grade dysplasia seems to be effective in the attempt to minimize the risk for CRC. We observed a rather high incidence of PSC, which may indicate that more attention should be paid to a search for this diagnosis in patients with early‐onset UC.
Endoscopic Ultrasonography in Pediatric Patients With Gastrointestinal Disorders
Authors: Cohen Shlomi , Kalinin Michael , Yaron Ayala , Givony Shlomo , Reif Shimon , Santo Erwin 
Published: February 2010

ABSTRACTBackground:Endoscopic ultrasound is a diagnostic tool for evaluation of gastrointestinal diseases in adults, but in children the use of endoscopic ultrasound is limited. This study retrospectively evaluates endoscopic ultrasound indications in, safety in, and impact on children.Patients and Methods:We reviewed the data of all of the pediatric patients (<18 years old) that underwent endoscopic ultrasound in our institution. The main goal was to evaluate the impact of endoscopic ultrasound in this patient population. Significant impact was defined as a new diagnosis or treatment attributed to the endoscopic ultrasound examination.Results:Thirty‐two children (21 boys, 11 girls) at mean age 12 ± 5 years; (range = 1.5–18 years) underwent endoscopic ultrasound during a period of 6 years. The pancreas and biliary tract were examined in 19 children, esophagus in 8, and the stomach and rectum in 2 children each. Only 1 child had endoscopic ultrasound for duodenal indication. The indications in the pancreas and biliary tract group were recurrent pancreatitis in 9 children, cyst or mass in 6 children, and obstructive jaundice in 4 children. Indications in the esophagus group were stenosis in 4 children, and suspected duplication and esophageal mass in 2 children each. We used conscious sedation in 18 children and unconscious sedation in 12. Two children underwent endoscopic ultrasound with no anesthesia at all. In 7 cases, endoscopic ultrasound–guided fine‐needle aspiration was performed. The procedure was successful in all of the patients with no complications. Endoscopic ultrasound changed the diagnosis or therapy in 14 cases.Conclusions:Endoscopic ultrasound is an effective tool in the evaluation of pediatric gastrointestinal patients, mostly with pancreatobiliary or esophageal disorders. Endoscopic ultrasound is a safe procedure with a significant impact in almost half of the children examined.
Prevalence of Celiac Disease in Children With Type 1 Diabetes Mellitus Increased in the Mid‐1990s: An 18‐year Longitudinal Study Based on Anti‐endomysial Antibodies
Authors: Salardi Silvana , Volta Umberto , Zucchini Stefano , Fiorini Erica , Maltoni Giulio , Vaira Bernardino , Cicognani Alessandro 
Published: February 2010

ABSTRACTBetween 1987 and 2004, 331 consecutive children, all newly diagnosed with type 1 diabetes mellitus in our pediatric clinic, underwent repeated serological screening for celiac disease (CD) by means of anti‐endomysial antibodies, measured prospectively between 1994 and 2004, and retrospectively, using frozen banked serum, between 1987 and 1993. There were 22 cases (6.6%) of biopsy‐proven CD among the 331 diabetic children. The prevalence of CD was significantly (P = 0.015) higher after 1994 (10.6%) than before 1994 (3.3%). The rapid change in the risk of CD among Italian diabetic children that occurred in the mid‐1990s could be related to changes in environmental factors, namely, eating habits and viral infections.
Impact of Neonatal Protein Metabolism and Nutrition on Screening for Phenylketonuria
Authors: Ponzone Alberto , Spada Marco , Roasio Luca , Porta Francesco , Mussa Alessandro , Ferraris Silvio 
Published: February 2010

ABSTRACTObjectives:Early blood phenylalanine (Phe) elevation after birth enables screening for and anticipation of the diagnosis of phenylketonuria. The differential impact of factors involved in this phenomenon, however, has not been elucidated. To solve this question, phenotype, genotype, dietary Phe intake, timing of blood collection, and Phe metabolism were retrospectively analyzed in 21 phenylketonuria newborns and prospectively in 1.Patients and Methods:Patients were assigned to 1 of 4 classes of phenylalanine hydroxylase (PAH) deficiency (severe, moderate, mild, and benign) on the basis of their Phe tolerance. Phe ingested, tolerated, and released from endogenous catabolism was assessed.Results:From birth to screening test, the amount of Phe tolerated ranged from 704 to 1620 mg, according to the class of PAH deficiency. The amount of Phe ingested ranged only from 204 to 405 mg, whereas the endogenous Phe breakdown ranged from 812 to 1534 mg, resulting in a rate of Phe catabolism ranging from 262 to 341 mg/day, regardless of the class of PAH deficiency.Conclusions:The high rate of protein catabolism is the main determinant of neonatal hyperphenylalaninemia. It is sufficient to turn to positive the screening test in severe and moderate PAH deficiency. In mild and benign PAH deficiency, the outcome of screening procedures can be substantially altered by the concurrence of genetic and peristatic factors. These results imply that the value of blood Phe at the screening test is not fully predictive of the phenylketonuria phenotype, and strengthen concerns regarding the reliability of early screening procedures.
Effect of Lactulose on Bacterial Translocation and Intestinal Adaptation in a Rat Model of Short Bowel Syndrome
Authors: Sukhotnik Igor , Srugo Isaac , Mogilner Jorge G , Lurie Michael , Coran Arnold G , Shaoul Ron 
Published: February 2010

ABSTRACTBackground:It is frequently assumed that dietary nondigestible carbohydrates improve host resistance to intestinal infections by stimulating proliferation of the protective gut microflora. Therefore, the aim of this study was to define the effect of lactulose, a nondigestible carbohydrate, on bacterial translocation and intestinal adaptation in a rat model of short bowel syndrome (SBS).Materials and Methods:Male Sprague‐Dawley rats were divided into 3 experimental groups. Sham rats underwent bowel transection, SBS rats underwent 75% small bowel resection, and SBS‐LAC rats underwent bowel resection and were treated with oral lactulose given in drinking water at a dose of 1.5 g · kg−1 · day−1 from the 3rd to the 15th postoperative day. Bacterial translocation (to mesenteric lymph nodes, liver, and portal and peripheral blood) and parameters of intestinal adaptation were determined on day 15.Results:Treatment with lactulose did not change bacterial translocation, but decreased ileal mucosal weight (20%; P < 0.05), ileal mucosal DNA (2‐fold decrease; P < 0.05) and protein (26%; P < 0.05), villus height in jejunum (21%; P < 0.05) and crypt depth in ileum (17%; P < 0.05), proliferation rates in jejunum (18%; P < 0.05) and ileum (15%; P < 0.05), and apoptotic index in jejunum (40%; P < 0.05) and ileum (36%; P < 0.05) compared with SBS animals. Bax expression was upregulated in SBS rats compared with control animals, but decreased following treatment with lactulose.Conclusions:In a rat model of SBS, oral lactulose does not improve bacterial translocation from the intestine, but inhibits intestinal adaptation.
What Is the Role of the Metabolic Activity of the Gut Microbiota in Inflammatory Bowel Disease? Probing for Answers With Stable Isotopes
Authors: Barclay Andrew R , Morrison Douglas J , Weaver Lawrence T 
Published: February 2010

ABSTRACTThe pathogenesis of inflammatory bowel disease remains obscure. However, there has been increasing interest in the role of the gut microbiota, focusing in particular on the “unculturable majority” of luminal and mucosal bacteria, which until recently have been difficult to study owing to the technical challenges of identification and elucidating function. Bacterial components and metabolites have been implicated in signalling to host immune systems and regulating inflammatory responses. Although the rapid expansion in techniques of molecular microbiology has increased our understanding of bacterial diversity, the tools to assess bacterial metabolic activity, and to link the 2, lag behind. Stable isotope probing is a powerful technique to link the metabolic activity and diversity of “unculturable” bacteria through isotopic labelling of biomarkers such as DNA and RNA. Progression of current stable isotope probing methodology with high‐resolution oligonucleotide 16s rRNA probe technology and high precision liquid chromatographic isotope ratio mass spectrometry may facilitate application in human microbial ecology. Progress towards stable isotope probing use in vivo, in concert with other advances in bacterial metabolome analysis, will lead to the development of a dynamic picture of the metabolic activity and diversity of intestinal bacteria in inflammatory bowel disease. Such insights will, over time, lead to fuller understanding of inflammatory bowel disease pathogenesis and the development of targeted therapies to reverse the “dysbiosis” that precedes disease relapse.
Altered Bile Acid Metabolism in Childhood Functional Constipation: Inactivation of Secretory Bile Acids by Sulfation in a Subset of Patients
Authors: Hofmann Alan F , Loening‐Baucke Vera , Lavine Joel E , Hagey Lee R , Steinbach Joseph H , Packard Christine A , Griffin Terrance L , Chatfield Dale A 
Published: February 2010

ABSTRACTObjective:An elevated concentration in the colon of the primary bile acid chenodeoxycholic acid (CDCA) or the secondary bile acid deoxycholic acid (DCA) is known to induce water secretion, causing diarrhea. We hypothesized that of the many fecal bile acids, only CDCA and DCA function as endogenous laxatives; therefore, a decrease in their proportion may be a cause of childhood functional constipation. To test this possibility, fecal bile acid composition was determined in children with functional constipation and in nonconstipated control children.Patients and Methods:Fecal samples were obtained from 207 children, 103 with functional constipation and 104 with normal bowel habits. Bile acid classes were determined by use of electrospray ionization—single ion monitoring—mass spectrometry (ESI‐SIM‐MS), and individual bile acids were measured by gas chromatography (GC)‐MS (GC‐MS). The structure of individual sulfated bile acids was obtained by use of liquid chromatography (LC)‐MS (LC‐MS).Results:By ESI‐SIM‐MS, the proportions of DCA did not differ in constipated children (n = 73) from that in control children (n = 92), but monosulfated dihydroxy bile acids were greater (P < 0.05). The difference was attributable to 6 patients in the constipated group whose major fecal bile acid by LC‐MS was the 3‐sulfate of CDCA. Sulfation of CDCA is known to abolish its secretory activity. By GC‐MS, the bile acid profile was identical in the 2 groups.Conclusions:In most children with functional constipation, the fecal bile acid profile seems to be normal. There is a small subset of children, however, whose dominant fecal bile acid is the 3‐sulfate of CDCA, indicating a novel disturbance in bile acid metabolism. Such sulfation abolishes the secretory activity of CDCA and may contribute to constipation.
Long‐chain Polyunsaturated Fat Supplementation in Children With Low Docosahexaenoic Acid Intakes Alters Immune Phenotypes Compared With Placebo
Authors: Mazurak VC , Lien V , Field CJ , Goruk SD , Pramuk K , Clandinin MT 
Published: February 2010

ABSTRACTObjectives:The objectives of this study were to assess the effects of long‐term supplementation with arachidonic acid (AA; 20:4n‐6) and docosahexaenoic acid (DHA; 22:6n‐3) on cell phenotypes and cytokine production in children.Patients and Methods:This randomized, double‐blind, placebo‐controlled trial provided children, (ages 5–7 years; n = 37) who had low intakes of DHA, with a dietary supplement containing AA (20–30 mg daily) and DHA (14–21 mg daily) or a placebo supplement for 7 months. After the supplementation period, a series of stimulants (pokeweed mitogen, phytohemagluttinin, lipopolysaccharide, β‐lactoglobulin, and ibuprofen) was used to stimulate peripheral blood mononuclear cells ex vivo. Antigen expression on T cells (CD25 and CD80), B cells, and macrophages (CD54), as well as cytokine production (interleukin [IL]‐4, IL‐10, tumor necrosis factor, IL‐2, IL‐6, and interferon‐γ), were measured using flow cytometry, monoclonal antibodies, and cytometric bead array, respectively.Results:Mononuclear cells from children provided long‐chain polyunsaturated fatty acids (LCPUFAs) had fewer CD8+ cells expressing CD25 and CD80 compared with placebo after exposure to each mitogen. The LCPUFA group also exhibited lower proportions of CD14+ cells after stimulation with β‐lactoglobulin and ibuprofen. The proportion of CD54+ cells was 2‐fold higher for the LCPUFA group compared with placebo after exposure to ibuprofen and β‐lactoglobulin (P < 0.05). Each of these immune effects related to the amount of AA and/or DHA in the plasma and erythrocyte phospholipids.Conclusions:Alterations in cell phenotypes were evident when children were supplemented with AA and DHA. The results of this study have important implications for immune development and sensitivity to antigens in children.
Carotenoids, Retinol, and Intestinal Barrier Function in Children From Northeastern Brazil
Authors: Vieira Milena M , Paik Jisun , Blaner William S , Soares Alberto M , Mota Rosa MS , Guerrant Richard L , Lima Aldo AM 
Published: February 2010

ABSTRACTObjectives:To investigate the association of carotenoids and retinol (vitamin A) with intestinal barrier function in children in an urban community in Fortaleza, northeastern Brazil.Methods:Descriptive analysis of serum carotenoids and retinol concentrations with intestinal barrier function in 102 children from an urban community, July 2000 to August 2001.Results:The weight for height z score (wasting) showed that 19.6% (20/102) had mild malnutrition (−1 to −2 z score). All of the children's serum retinol concentrations were determined and none were severely deficient (≤0.35 μmol/L), 2.9% (3/102) were moderately (0.36–0.70 μmol/L) deficient, 20.6% (21/102) were mildly (0.71–1.05 μmol/L) deficient; 76.5% (78/102) were vitamin A sufficient (>1.05 μmol/L). The lactulose:mannitol (L/M) ratio was elevated (≥0.0864) in 49% (47/97) of children when compared with healthy children with normal L/M ratio (<0.0864) in the same geographic area. Serum carotenoids, lutein, β‐cryptoxanthin and β‐carotene showed significant inverse correlations with the L/M ratio, but not lutein after adjusting for age. Acute phase proteins (C‐reactive protein and α‐acid glycoprotein) were significantly inversely correlated with retinol but not with carotenoids. Retinol and retinol‐binding protein were not significantly associated with L/M ratio.Conclusions:These data suggest a disruption of intestinal barrier function in the paracellular pathway with low serum concentrations of carotenoids. Carotenoids may provide a better marker for disrupted intestinal barrier function than retinol‐binding protein or retinol.
Germline APC Mutations Are Not Commonly Seen in Children With Sporadic Hepatoblastoma
Authors: Harvey JJ , Clark SK , Hyer W , Hadzic N , Tomlinson IPM , Hinds R 
Published: February 2010

ABSTRACTHepatoblastoma is the most common primary liver tumor in childhood and occurs more commonly in families with familial adenomatous polyposis. Germline mutations of the gene responsible for familial adenomatous polyposis—adenomatous polyposis coli (APC)—are described in patients with hepatoblastoma even without a family history. We investigated children presenting with apparently sporadic hepatoblastoma between 1991 and 2004. Blood samples were available from 29 children (18 boys) whose conditions were diagnosed at a median age of 22 months (range 6–119 months). No germline APC mutations were found, which does not support the need for routine screening in sporadic hepatoblastoma in the absence of a suggestive family history of colorectal cancer or suspicion of familial adenomatous polyposis.
Aetiology and Management of Extrahepatic Portal Vein Obstruction in Children: King's College Hospital Experience
Authors: Abd El‐hamid Nehal , Taylor Rachel M , Marinello Daniela , Mufti GJ , Patel Raj , Mieli‐Vergani Giorgina , Davenport Mark , Dhawan Anil 
Published: February 2010

ABSTRACTObjective:To study a single‐centre experience of the management of extrahepatic portal vein obstruction (EHPVO) in children during the last 3 decades.Materials and Methods:The medical records of 108 children (67 male, median age 4.75 years, range = 1 day–16.3 years) presenting with EHPVO between 1979 and 2005 were reviewed retrospectively.Results:Extended prothrombotic screening performed in 30 patients revealed low protein C activity (6 patients), low free protein S (2), and a positive lupus anticoagulant (1); factor V Leiden mutations and the JAK2V617F mutation were not identified. Associated congenital anomalies were found in 26 of the 108 children (24%). Clinical presentation included splenomegaly in 98 (91%) and ascites in 3 (3%). Elevation of liver enzymes and prolonged international normalized ratio were seen in 13 (12%) and 14 (13%) children, respectively. Haematological parameters of hypersplenism were present in 13 (12%). Bleeding occurred in 83 (77%) patients with a median age of 4.58 (0.02–16.37) years. On first endoscopy, oesophageal varices were present in 92 patients; of those subjects, 70 (76%) received sclerotherapy, 5 (5%) had band ligation, and 16 (17%) received both. Complications of endoscopy occurred in 34 (37%) patients: oesophageal ulcers in 16, oesophageal stricture in 10, both in 7, and erosive gastritis in 1. Seventeen (16%) children underwent shunt surgery for uncontrolled bleeding at a median age of 9.7 (5.2–23.7) years.Conclusions:The aetiology of EHPVO in the majority of patients remains unknown. Sclerotherapy and banding are effective treatments for bleeding varices with good long‐term outcome. Procoagulant state is an infrequent cause of EHPVO in children.
Quality of Life in Adolescents With Treated Coeliac Disease: Influence of Compliance and Age at Diagnosis
Authors: Wagner Gudrun , Berger Gabriele , Sinnreich Ursula , Grylli Vasileia , Schober Edith , Huber Wolf‐Dietrich , Karwautz Andreas 
Published: February 2010

ABSTRACTObjective:To assess the influence of gluten‐free diet (GFD) compliance on the quality of life (QOL) of adolescents with coeliac disease (CD), and the impact of patient's age at time of diagnosis.Study design:Participants included 365 subjects: 283 adolescents (10–20 years old) with biopsy‐proven CD and 82 adolescents without a chronic condition matched for age, sex, education, and social status. Their subjective QOL—comprising physical, mental, and social dimensions as defined by the World Health Organization—was measured and has been analyzed according to compliance status and age at CD diagnosis.Results:Adolescents noncompliant with GFD reported a lower general QOL, more physical problems, a higher burden of illness, more family problems, and more problems in leisure time than adolescents who are compliant with GFD. More frequent GFD transgressions were associated with poorer QOL. Higher problem anticipation and higher feelings of “ill‐being” were found in the noncompliant group. No differences between compliant patients with CD and adolescents without any chronic condition were found in all QOL aspects. Adolescents with a late CD diagnosis showed more problems at school and in social contact with peers, as well as worse physical health and higher CD‐associated burden.Conclusions:Compliance with GFD is an essential factor to obtain optimal QOL. Psychosocial and educational support should be provided for patients having difficulties strictly adhering to GFD. Early CD onset and diagnosis is associated with better physical health, lower CD‐associated burden and fewer social problems, indicating the importance of the earliest CD diagnosis possible.
Expression of Intestinal and Lung Alkaline Sphingomyelinase and Neutral Ceramidase in Cystic Fibrosis F508del Transgenic Mice
Authors: Ohlsson Lena , Hjelte Lena , Hühn Michael , Scholte Bob J , Wilke Martina , Flodström‐Tullberg Malin , Nilsson Åke 
Published: February 2010

ABSTRACTObjectives:The intestinal brush border enzymes alkaline sphingomyelinase (alk‐SMase) and neutral ceramidase (CDase) digest milk sphingomyelin in suckling neonates. In addition, alk‐SMase, CDase, and acid sphingomyelinase (acid‐SMase) have been implicated in sphingolipid signaling, which exhibits abnormalities in cystic fibrosis (CF). In this study, we tested the hypothesis that the expression of these enzymes is different in CF.Materials and Methods:We used mice with F508del (Cftrtm1Eur) mutation, a CF mouse model with well‐characterized intestinal pathology. Enzyme activities were measured using radiolabeled sphingolipid substrates incubated with tissue homogenates from different organs and intestinal contents of wild‐type mice, homozygous, and heterozygous F508del mice.Results:No difference was found in levels of CDase and alk‐SMase in the small intestinal mucosa or in their longitudinal distribution. Acid‐SMase activity was significantly lower in the mucosa of the distal half of the small intestine of F508del compared with wild‐type mice. Despite a lower body weight of F508del mice, length and weight of the small intestine and weight per centimeter of colon were larger than in wild‐type. Neutral CDase and alk‐SMase activities in lungs were lower than in the gut, whereas acid‐SMase activity was comparable in both organs. CDase activity in the spleen was significantly higher in F508del than in wild‐type mice.Conclusions:Alk‐SMase and neutral CDase are normally expressed in F508del CF mice, whereas activity of acid‐SMase in the distal small intestine is decreased. We found no differences in activity of these enzymes in lungs in this mouse model.