Abstract:
OBJECTIVES:Congenital hepatic fibrosis (CHF) is an important cause of morbidity and mortality in patients with autosomal recessive polycystic kidney disease (ARPKD). The pathogenesis of CHF remains undefined. Several recent studies suggest that the renin-angiotensin system (RAS) is an important mediator of progressive hepatic fibrosis through activation of profibrotic mediators, such as transforming growth factor-beta (TGF-beta). RAS activation has not previously been studied in patients with CHF or in animal models. The aim of the present study was to characterize RAS expression during the course of CHF in the PCK rat. MATERIALS AND METHODS:Studies were conducted in the PCK rat, an orthologous ARPKD/CHF model, and age-matched normal control Sprague-Dawley rats. Expression of the RAS components, renin, angiotensinogen, angiotensin-converting enzyme (ACE), and angiotensin II type 1 receptor (AT1R), as well as the profibrotic mediator TGF-beta, was examined in cystic PCK and control rat livers at 2, 4, and 6 months of age by quantitative real-time polymerase chain reaction (qRT-PCR). Angiotensin II (ANG II) was examined by immunohistochemistry (IHC). Fibrosis was assessed by IHC using reticulin staining and Masson trichrome. Collagen content was determined by hydroxyproline analysis. RESULTS:Progressive fibrosis and increased hepatic collagen content occurred in PCK rats with age. In 4- and 6-month-old PCK rat livers, ACE gene expression was markedly increased, 8- and 17-fold, respectively, compared with age-matched control livers. Expression of the other RAS components, renin, angiotensinogen, and AT1R were not significantly different. IHC demonstrated prominent ANG II protein expression in periportal regions in PCK rats. In contrast, no expression was noted in control livers. TGF-beta expression was also increased in PCK rat livers with progressive disease. CONCLUSIONS:The present study demonstrates, for the first time, RAS upregulation in an orthologous rat ARPKD/CHF model. Increases in ACE and ANG II, as well as the downstream target, the profibrotic mediator TGF-beta, suggest that RAS activation may be an important mediator of CHF disease progression. The findings also suggest that treatment with RAS inhibitors, specifically ACE inhibitors or AT1R blockers, could be therapeutic in slowing disease progression in CHF.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Goto M,Hoxha N,Osman R,Wen J,Wells RG,Dell KMdoi
10.1097/MPG.0b013e3181cc80e4subject
Has Abstractpub_date
2010-06-01 00:00:00pages
639-44issue
6eissn
0277-2116issn
1536-4801journal_volume
50pub_type
杂志文章abstract::The European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Polyposis Working Group developed recommendations to assist clinicians and health care providers with appropriate management of patients with juvenile polyposis. This is the first juvenile polyposis Position Paper published by ESP...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0000000000002246
更新日期:2019-03-01 00:00:00
abstract::Ulcerative colitis and Crohn disease may present before the age of 20 years in 25% to 30% of all patients with inflammatory bowel disease. Reported incidence figures vary considerably depending on the collection of data. Multicenter, multinational collaboration is needed when studying pediatric inflammatory bowel dise...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 指南,杂志文章,实务指引
doi:10.1097/01.mpg.0000163736.30261.82
更新日期:2005-07-01 00:00:00
abstract::Gastroesophageal reflux (GER), defined as passage of gastric contents into the esophagus, and GER disease (GERD), defined as symptoms or complications of GER, are common pediatric problems encountered by both primary and specialty medical providers. Clinical manifestations of GERD in children include vomiting, poor we...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 指南,杂志文章,实务指引
doi:10.1097/00005176-200100002-00001
更新日期:2001-01-01 00:00:00
abstract:OBJECTIVES:The aim of the present review was to provide recommendations on the use of hydrolysates in infants when formula feeding is initiated. METHODS:We performed an overview of reviews followed by a systematic review of subsequently published trials. RESULTS:We found 8 systematic reviews; only 1 study of limited ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0000000000000318
更新日期:2014-05-01 00:00:00
abstract:BACKGROUND:Duodenal gastric metaplasia is rarely reported in untreated celiac disease, although it is seen in 60% to 100% of duodenal biopsy specimens in nonceliac patients with histologic duodenitis. The low incidence could represent underreporting, a decreased incidence in pediatric patients generally, or the more di...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200004000-00009
更新日期:2000-04-01 00:00:00
abstract::Acute hydrops of the gallbladder is a well-recognized complication of Kawasaki syndrome. We report a case of a child with this syndrome whose gallbladder hydrops slowly resolved after intravenous gamma-globulin therapy. However, he continued to experience postprandial right upper quadrant abdominal pain. Hepatobiliary...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199110000-00013
更新日期:1991-10-01 00:00:00
abstract:OBJECTIVE:LIPA gene mutations result in deficiency of lysosomal acid lipase and present phenotypically as Wolman disease or cholesteryl ester storage disease (CESD) depending on the level of deficiency. Patients with CESD may often be misdiagnosed because symptoms may be nonspecific. Symptoms may present in infancy if ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e31828b36ac
更新日期:2013-06-01 00:00:00
abstract::We report two infants with cystic fibrosis (CF), presenting with meconium ileus and meconium plug, who had no clinical or biochemical evidence of pancreatic insufficiency during infancy. They underwent pancreatic secretory function testing at 11 and 9 months of age, respectively. Both patients had sufficient lipase an...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198803000-00019
更新日期:1988-03-01 00:00:00
abstract:BACKGROUND:Although infection with Helicobacter pylori in children mostly induces asymptomatic chronic gastritis, the clinical outcome of H pylori infection is generally unpredictable. To identify the risk subgroup of infected children who can progress toward serious gastrointestinal disease, we assessed the prevalence...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31818f09f2
更新日期:2009-09-01 00:00:00
abstract:OBJECTIVES:The aim of the study was to evaluate erosive esophagitis healing and symptom improvement with once-daily esomeprazole in children ages 12 to 36 months with endoscopically or histologically proven gastroesophageal reflux disease (GERD). PATIENTS AND METHODS:Data from children ages 12 to 36 months were includ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1097/MPG.0b013e3181ddcf11
更新日期:2010-11-01 00:00:00
abstract::Recent reports show that the incidence of and deaths caused by necrotizing enterocolitis (NEC) in preterm very-low-birth-weight (PVLBW) infants are on the rise. Unfortunately, NEC often rapidly progresses from early signs of intestinal inflammation to extensive necrosis within a matter of hours, making treatment and s...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3182a7dc74
更新日期:2014-01-01 00:00:00
abstract:OBJECTIVES:To analyze the long-term results of Boerema anterior gastropexy using the Visick grading system in a university teaching hospital. METHODS:Retrospective review of 247 children with severe or complicated gastroesophageal reflux disease (GERD). Patients who underwent primary gastropexy between 1990 and 2001 w...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000215313.72826.bc
更新日期:2006-07-01 00:00:00
abstract:OBJECTIVES:Acute exacerbations of inflammatory bowel disease (IBD) may involve enteric pathogen. We aimed to assess the frequency and outcomes of Clostridium difficille toxin (CDT) and non-CDT enteric infections in symptomatic pediatric patients with IBD. METHODS:Patients' records were retrospectively searched for dis...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002895
更新日期:2021-01-01 00:00:00
abstract::Nonalcoholic fatty liver disease (NAFLD), defined as fat accumulation greater than 5% in hepatocytes, may progress to fibrosis or cirrhosis later in life. NAFLD prevalence in adolescents has increased significantly in direct relation with obesity prevalence. Fatty liver has become the most frequent indication for live...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002523
更新日期:2020-01-01 00:00:00
abstract:OBJECTIVES:The aim of the study was to describe the relation between sensory issues and medical complexity in a series of patients presenting to an outpatient multidisciplinary feeding team for evaluation, by a standardized measure of sensory-processing abilities. METHODS:A retrospective chart review of all of the pat...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182736e19
更新日期:2013-02-01 00:00:00
abstract:OBJECTIVE:Data suggest an increase in the incidence of pediatric inflammatory bowel disease (IBD). We examined the trend of the incidence of IBD in children. PATIENTS AND METHODS:A retrospective investigation was conducted on a cohort of children diagnosed with IBD between 1991 and 2002 who were registered in the IBD ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181b99baa
更新日期:2010-01-01 00:00:00
abstract:BACKGROUND:The efficiency with which breast- and formula-fed infants utilize dietary nutrients is likely to provide insight into their relative requirements for the growth process. METHODS:We measured longitudinal changes in growth, body composition, and dietary intakes in breast- and formula-fed infants and estimated...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199701000-00006
更新日期:1997-01-01 00:00:00
abstract:BACKGROUND:The aim of this study was to investigate whether perinatal indomethacin treatment has effects on the development of esophageal and gastric lesions in preterm infants and to evaluate other potential etiologic factors behind these lesions. METHODS:Sixty-nine infants were born at less than 33 weeks' gestation....
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200102000-00017
更新日期:2001-02-01 00:00:00
abstract:BACKGROUND:The development of de novo autoimmune hepatitis as a long-term complication after liver transplantation has been recently reported. The authors describe five liver allograft recipients who developed chronic hepatitis associated with autoimmune features. METHODS:Five of 155 liver transplant recipients at ris...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200102000-00007
更新日期:2001-02-01 00:00:00
abstract:BACKGROUND:Lactobacillus plantarum is a Gram-positive bacillus known for its effect as a probiotic agent. The goal of the study was to determine whether L. plantarum is capable of inhibiting the transepithelial neutrophil migration induced by enteropathogenic Escherichia coli (EPEC). METHODS:Cultured intestinal epithe...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200303000-00017
更新日期:2003-03-01 00:00:00
abstract::Somatic gene therapy represents a new approach for treating a variety of genetic and acquired diseases and has many potential applications in gastroenterology. Somatic gene therapy is based on the ability to transfer recombinant genes efficiently into somatic tissues, such as the liver or intestine, and achieve expres...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/00005176-199204000-00017
更新日期:1992-04-01 00:00:00
abstract:OBJECTIVES:Diagnostic and therapeutic benefits of double-balloon enteroscopy (DBE) have been documented in adults, with few data available on pediatric patients. We evaluated the diagnostic and therapeutic utility of DBE in children. METHODS:A prospective assessment of 113 DBE procedures in 58 consecutive children you...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000192
更新日期:2014-02-01 00:00:00
abstract:OBJECTIVE:The antitumor necrosis factor α (TNFα) antibodies infliximab and adalimumab are effective in inducing and maintaining remission in pediatric patients with Crohn disease (CD). The aim of the study was to evaluate the long-term efficacy and safety of biological therapy in pediatric patients with CD followed at ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000276
更新日期:2014-05-01 00:00:00
abstract:OBJECTIVES:We evaluated the response to infliximab in pediatric patients with ulcerative colitis (UC) and their long-term follow-up. We expanded our previous study of 14 patients and furthermore evaluated the success of weaning patients from infliximab. PATIENTS AND METHODS:We reviewed the charts of 27 pediatric patie...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31802e98d4
更新日期:2007-03-01 00:00:00
abstract:OBJECTIVE: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. MATERIALS AND METHODS:Adult male Spragu...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31806008f1
更新日期:2008-08-01 00:00:00
abstract:BACKGROUND:Little is known about the metabolic effects of exercise training in children with cystic fibrosis. The hypothesis for the current study was that in patients with declining clinical status, exercise increases circulating insulin-like growth factors (IGFs) and improves protein kinetics. METHODS:This was a pro...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200101000-00020
更新日期:2001-01-01 00:00:00
abstract:OBJECTIVE:Diarrhea is frequently observed among critically ill children (CIC) admitted for intensive care. There is increasing evidence that probiotics decrease the incidence of acute infectious and nosocomial and antibiotic induced diarrhea amongst children hospitalized in nonintensive care settings. Despite theoretic...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/01.mpg.0000189335.62397.cf
更新日期:2006-02-01 00:00:00
abstract:OBJECTIVE:The aim of the study was to use linear programming (LP) analyses to adapt New Complementary Feeding Guidelines (NCFg) designed for infants aged 6 to 12 months living in poor socioeconomic circumstances in Bogota to ensure dietary adequacy for young children aged 12 to 23 months. DESIGN:A secondary data analy...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/MPG.0000000000001662
更新日期:2017-12-01 00:00:00
abstract:BACKGROUND:Little is known about the enteric protein loss in patients after a modified Fontan operation before the appearance of overt symptoms or signs of protein-losing enteropathy (PLE). The authors examined the possibility of using fecal alpha1-antitrypsin concentration measurements for the early detection of posto...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200311000-00014
更新日期:2003-11-01 00:00:00
abstract:BACKGROUND:Patients with cystic fibrosis who have steatorrhea frequently are underweight and have essential fatty acid (EFA) depletion, which is associated with a poor clinical course. It has been stated that poor EFA status is difficult to correct in patients with cystic fibrosis, and an impaired EFA metabolism with r...
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:10.1097/00005176-200010000-00016
更新日期:2000-10-01 00:00:00