Abstract:
OBJECTIVES: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 (Cftr) 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.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Ohlsson L,Hjelte L,Hühn M,Scholte BJ,Wilke M,Flodström-Tullberg M,Nilsson Adoi
10.1097/MPG.0b013e3181826dafsubject
Has Abstractpub_date
2008-11-01 00:00:00pages
547-54issue
5eissn
0277-2116issn
1536-4801pii
00005176-200811000-00004journal_volume
47pub_type
杂志文章abstract::A 12-year-old boy presented with a 2-month history of abdominal pain and distention. A diagnosis of Wilson's disease was established, and D-penicillamine therapy was initiated. An associated pancreatitis was diagnosed on presentation, based on elevated serum amylase and an enlarged pancreas ultrasonically. Subsequentl...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198811000-00024
更新日期:1988-11-01 00:00:00
abstract:BACKGROUND:There is a strong genetic influence on the susceptibility to celiac disease but it is also evident that environmental factors play a role in the development of the disease. Diverging studies about adenovirus infection as a possible triggering factor in the pathogenesis have been reported. Our study was under...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 评论,杂志文章
doi:10.1097/00005176-200211000-00011
更新日期:2002-11-01 00:00:00
abstract:AIM AND OBJECTIVE:: Chronic models of inflammatory bowel disease are lacking in preadult rodents. The primary goal of our study was to develop a chronic model of hapten-induced intestinal inflammation and fibrosis in young rats. Second, we aimed to determine the profiles of key Th-1, Th-2, and Th-17 proinflammatory and...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181cb8f4a
更新日期:2010-03-01 00:00:00
abstract:BACKGROUND:Although a dextran sodium sulfate (DSS)-induced colitis is commonly used as an ulcerative colitis (UC) model in adult rodents, there are no studies using this model in young animals. We examined differences in the severity of DSS-induced colitis as a function of the concentration of DSS administered and soug...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200310000-00015
更新日期:2003-10-01 00:00:00
abstract:BACKGROUND:Health-related quality of life (HRQOL) describes the physical, social, and emotional attitudes and behavior of people in relation to health status. By the 1980s, HRQOL was noted to be impaired in chronic diseases but inflammatory bowel disease, had not been extensively described. METHODS:This review summari...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/00005176-199904001-00003
更新日期:1999-04-01 00:00:00
abstract:OBJECTIVES:In developed countries, small bowel histology in coeliac disease is a spectrum, ranging from normal with increased intraepithelial lymphocytes to the classic flat mucosa. In developing countries, mild to moderate enteropathies in children with chronic diarrhea and growth failure are assumed to be caused by t...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000172261.24115.29
更新日期:2005-08-01 00:00:00
abstract:OBJECTIVE:Capsule endoscopy (CE) is a noninvasive diagnostic tool for the digestive tract. We aim to investigate the feasibility and safety of newly developed magnetically controlled capsule endoscopy (MCE) in children. METHODS:A total of 129 children who underwent MCE in Shanghai Children's Hospital were retrospectiv...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002292
更新日期:2019-07-01 00:00:00
abstract::A retrospective approach has been adopted to investigate the frequency and duration of breast feeding and the time of gluten introduction in the diet in 216 celiac children and their healthy siblings of three different centers--Naples, Milan, and Turin. In this matched case-control study, the selected controls were he...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198302030-00006
更新日期:1983-01-01 00:00:00
abstract:OBJECTIVES:Discordance exists between outcomes of endoscopy, multichannel intraluminal impedance monitoring (pH-MII), MII baselines, and gastroesophageal reflux symptoms. The aim of the present study was to determine the association between endoscopy, pH-MII and MII baselines, in children with gastroesophageal reflux s...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31827167e2
更新日期:2013-02-01 00:00:00
abstract::Safflower oil emulsion (Liposyn 10%) was infused intravenously to supplement energy intake in five low-birth-weight infants. Respiratory gas exchange was measured before and after the addition of fat emulsion in doses of 1-2 g of fat/kg/day to an intravenous feeding regimen of dextrose and amino acids. The oxygen cons...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198302030-00020
更新日期:1983-01-01 00:00:00
abstract::Serum carboxyterminal propeptide of type I procollagen (PICP) and cross-linked carboxyterminal telopeptide of type I collagen (ICTP), new markers of bone collagen type I biosynthesis and degradation, have not been studied in small for gestational age (SGA) infants. In an earlier study, we found a lower bone mineral co...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199610000-00004
更新日期:1996-10-01 00:00:00
abstract:OBJECTIVES:In traditional access endoscopy (TAE), patients are booked for endoscopy following a gastroenterology clinic assessment. In contrast, open access endoscopy (OAE) patients are seen for the first time on the day of the procedure, providing same day procedural consent. Controversy exists over the use of OAE in ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000677
更新日期:2015-05-01 00:00:00
abstract::In this prospective study, neonates with clinical congenital syphilis were investigated to determine if penicillin therapy caused a deterioration in liver function. The relationship between circulating immune complexes and liver involvement was monitored, and the efficacy of steroid therapy as an adjunct in the treatm...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1097/00005176-199104000-00003
更新日期:1991-04-01 00:00:00
abstract:OBJECTIVES:The aim of the study was to develop a questionnaire evaluating the frequency of symptoms over time of concomitant childhood bladder and bowel dysfunctions (CBBDs) in 5- to 12-year-old children and to assess its feasibility and aspects of validity and reliability. METHODS:The Childhood Bladder and Bowel Dysf...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001461
更新日期:2017-06-01 00:00:00
abstract::We reviewed data from 184 percutaneous liver biopsies performed as an outpatient procedure in 104 patients (5 weeks to 22 years of age) over a 4-year period. Hepatic allograft rejection was the indication for 130 of the biopsy procedures, while 54 were performed for a variety of indications in patients who had not und...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199311000-00006
更新日期:1993-11-01 00:00:00
abstract::It appears that neonates, especially those with very low birthweights, may be at especially high risk of developing cholestasis associated with total parenteral nutrition (TPN). Within 2 weeks of starting intravenous alimentation, it would appear highly desirable to be able to start at least small enteral feedings to ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/00005176-198601000-00004
更新日期:1986-01-01 00:00:00
abstract::We studied jejunal biopsy specimens of 13 IgA-deficient persons (IgAdp) and 12 controls. Four Ig-Adp had celiac disease, in the others the jejunal mucosa appeared normal. Monoclonal antibodies and the peroxidase technique were used to identify T lymphocytes, T-lymphocyte subsets, HLA-DR antigens, and IgE-containing ce...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198807000-00010
更新日期:1988-07-01 00:00:00
abstract::To characterize the mechanisms leading to dietary evoked increases of lactase and sucrase activities by carbohydrates, we performed a quantitative comparison of the effects of lactose and sucrose on the corresponding disaccharidases in the jejunum of 2-month-old rats. For 7-10 days the rats were fed a low-starch (5 ca...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198512000-00024
更新日期:1985-12-01 00:00:00
abstract:BACKGROUND:Catheter-related bloodstream infection remains the most common serious complication associated with long-term parenteral nutrition. Taurolock (Bio-Implant HealthCare, Winsen, Germany), which contains taurolidine, is being marketed as a central venous catheter lock solution for decreasing the risk of catheter...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,meta分析,评审
doi:10.1097/MPG.0b013e318162c428
更新日期:2008-08-01 00:00:00
abstract:INTRODUCTION:With the current coronavirus disease 2019 (COVID-19) pandemic, concerns have been raised about the risk to children with inflammatory bowel diseases (IBD). We aimed to collate global experience and provide provisional guidance for managing paediatric IBD (PIBD) in the era of COVID-19. METHODS:An electroni...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002729
更新日期:2020-06-01 00:00:00
abstract:BACKGROUND:The aim of the current study was to evaluate the prevalence of lactose malabsorption (LM) in Galicia (NW Spain) in order to design nutritional intervention and/or public education strategies for high risk groups. METHODS:We conducted a study of LM by breath-hydrogen carbohydrate absorption test (BH2 test) i...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199709000-00009
更新日期:1997-09-01 00:00:00
abstract:BACKGROUND:Granulomas are pathognomonic findings of Crohn disease (CD); however, their occurrence and clinical significance are not well characterized. Our aim was to determine the frequency and distribution of granulomas in untreated and treated patients with CD and their relation to age and disease severity. PATIENT...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31812e95e1
更新日期:2008-04-01 00:00:00
abstract:BACKGROUND:Many patients with chronic hepatitis C do not respond to antiviral treatment. In adult patients the re-treatment of these patients has been extensively investigated. Because the response to re-treatment in children is not well defined we evaluated the efficacy and safety of interferon (IFN)-alpha plus ribavi...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章
doi:10.1097/MPG.0b013e3181d9c7f6
更新日期:2010-08-01 00:00:00
abstract::We have previously demonstrated that a spiral myotomy and delayed definitive procedure is a viable alternative for esophageal reconstruction in long-gap esophageal atresia. In this study we sought to determine whether this procedure leads to esophageal motility disturbances and to compare the manometric findings with ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199004000-00019
更新日期:1990-04-01 00:00:00
abstract::The levels of secretory IgA and of IgM and IgG in colostrums and milks obtained at various times postpartum were measured by radial immunodiffusion. Immunoglobulin A was the dominant immunoglobulin in all samples, and concentrations in colostrum ranged from 1.5 to 83.7 g/L (mean 32 g/L). Immunoglobulin M and IgG had m...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198201030-00018
更新日期:1982-01-01 00:00:00
abstract::We performed a Japanese nationwide survey of pediatric-onset chronic nonspecific multiple ulcers of the small intestine between January 2000 and July 2013 in 176 institutions of pediatric surgery or pediatric gastroenterology and clarified the clinical features associated with genetic abnormalities in the Solute Carri...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001321
更新日期:2017-04-01 00:00:00
abstract:BACKGROUND:Posttransplantation lymphoproliferative disorder (PTLD) may manifest a variety of nonspecific symptoms and must be suspected in the patient who undergoes solid organ transplantation. Common sites of occurrence include the gastrointestinal tract, the central nervous system, and lymphoid tissue of the oral pha...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200010000-00026
更新日期:2000-10-01 00:00:00
abstract:BACKGROUND:Familial defective apolipoprotein B-100, a dominantly inherited form of hypercholesterolemia caused by a single Arg3500Gln mutation, is silent in childhood but may confer a high risk of cardiovascular disease in adulthood. The objective was to determine the prevalence of familial defective apolipoprotein B-1...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,多中心研究
doi:10.1097/00005176-200108000-00005
更新日期:2001-08-01 00:00:00
abstract:OBJECTIVES:The epidemiology and clinical significance of disaccharidase deficiencies have not been thoroughly characterized. Recent work suggests at least genetic sucrase-isomaltase deficiency is more prevalent than previously believed. Because lactase deficiency (LD) is well described, the present study focuses on the...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001961
更新日期:2018-06-01 00:00:00
abstract::Lactoferrin is an iron-binding protein present in high concentrations in human milk. The efficacy of supplementing iron bound to lactoferrin to iron-deficient and iron-sufficient young mice was evaluated in comparison with supplementation of iron as iron chloride. Mice fed a nonsupplemented milk diet (approximately 1 ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198311000-00021
更新日期:1983-11-01 00:00:00