Abstract:
:Three distinct forms of familial intrahepatic cholestasis are the result of mutations in the ATP8B1, ABCB11, and ABCB4 genes. The pathophysiologies of the latter 2 of these diseases are well characterized and are the result of abnormalities in canalicular excretion of bile acids and phospholipids, respectively. The molecular pathophysiology of the systemic disease associated with mutations in ATP8B1 remains unclear. In all of these diseases, wide variations in clinical phenotypes have been observed. The variability can be ascribed at least in part to predicted genotype:phenotype correlations. Disease- and genotype-specific prognoses and therapeutic approaches may exist, although much more information needs to be ascertained before clinicians can confidently make decisions based on genetic information.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Alissa FT,Jaffe R,Shneider BLdoi
10.1097/MPG.0b013e3181596060subject
Has Abstractpub_date
2008-03-01 00:00:00pages
241-52issue
3eissn
0277-2116issn
1536-4801pii
00005176-200803000-00003journal_volume
46pub_type
杂志文章,评审abstract:BACKGROUND AND AIM:Baclofen, a γ-aminobutyric acid receptor agonist, has been shown to reduce the episodes of gastroesophageal reflux (GER) by reducing the incidence of transient lower esophageal sphincter relaxations. Although baclofen has been shown to reduce reflux symptoms in adults, data in pediatric patients are ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182a2747b
更新日期:2013-12-01 00:00:00
abstract:OBJECTIVES:Celiac disease (CD) represents a spectrum, which includes cases with minor histological abnormalities (potential CD). The aim of this work is to evaluate the contribution of immunohistochemical analysis of duodenal biopsies to the diagnosis of gluten-related minor enteropathy. METHODS:Duodenal biopsies from...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000675
更新日期:2015-05-01 00:00:00
abstract::We evaluated the effect of increased dietary fiber (DF) content in weaning cereals based on wheat/soy (8.0 and 1.8% DF) and wheat/milk (5.3 and 2.0% DF) in healthy, formula-fed infants 7-17 weeks old. The study had a cross-over design, each infant acting as his or her own control. Stool characteristics and anthropomet...
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:10.1097/00005176-199602000-00008
更新日期:1996-02-01 00:00:00
abstract:OBJECTIVES:The aim of this study was to evaluate the effect of autoimmune hepatitis (AIH) on the quality of life of children and adolescents and to identify which variables effect health-related quality of life (HRQoL). METHODS:The Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) was used to evaluate HRQoL. In add...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001930
更新日期:2018-06-01 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::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::To determine whether zinc has a specific role on weight gain and intestinal disaccharidase activity, 42 male Sprague-Dawley rats were assigned to one of seven groups (n = 6 each). These were a baseline control group (0) that was killed to analyze initial intestinal disaccharidase (sucrase and maltase) activity, a seco...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199001000-00019
更新日期:1990-01-01 00:00:00
abstract::Partially hydrolyzed formulas (pHFs) are increasingly used worldwide, both in the prevention of atopic disease in at-risk infants and in the therapeutic management of infants with functional gastrointestinal manifestations. Because prevention is always preferable to treatment, we reviewed the literature aiming to find...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 共识发展会议,杂志文章,评审
doi:10.1097/MPG.0000000000001014
更新日期:2016-01-01 00:00:00
abstract::Milk was obtained on postpartum days 2-3 (colostrum) and days 7, 21, 42, and 84 from mothers of 18 very premature (VPT, 26-30 weeks gestational age), 28 premature (PT, 31-36 weeks), and 6 term (T, 37-40 weeks) infants. Lipids were extracted in chloroform-methanol and analyzed by thin-layer (TLC) and gas liquid chromat...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198609000-00020
更新日期:1986-09-01 00:00:00
abstract::Anti-tumor necrosis factor-alpha (TNF-α) blockade is so far the most effective therapy for extensive pediatric Crohn disease (CD), but loss of response is frequently encountered. We describe here the use of golimumab (Simponi) in 6 pediatric CD patients with antibody formation/loss of response to infliximab and adalim...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001165
更新日期:2016-09-01 00:00:00
abstract:BACKGROUND:It is important to identify patients with monogenic IBD as management may differ from classical IBD. In this position statement we formulate recommendations for the use of genomics in evaluating potential monogenic causes of IBD across age groups. METHODS:The consensus included paediatric IBD specialists fr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000003017
更新日期:2020-12-16 00:00:00
abstract:OBJECTIVE:In 2011, the United States Surgeon General issued a call to action to "identify and address obstacles to greater availability of safe banked donor milk for fragile infants." The purpose of the present study is to analyze patterns in donor human milk (DHM) and fortifier use in level 2, 3, and 4 neonatal facili...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001790
更新日期:2018-04-01 00:00:00
abstract::R-1 anti-reticulin antibodies (ARA) were present in sera of 10 of the 12 children who subsequently fulfilled the ESPGAN diagnostic criteria for celiac disease (CD), but was not found in any children in the age-matched control groups, viz., severely malnourished (n = 20), acute gastroenteritis (n = 23), normally nouris...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1988-11-01 00:00:00
abstract::Liver transplantation is now accepted as the treatment of choice for tyrosinemia type I (hereditary tyrosinemia). In an effort to determine whether any factors in these patients would aid in predicting optimal timing of the transplant procedure, we evaluated several clinical, biochemical, and radiographic parameters i...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199107000-00002
更新日期:1991-07-01 00:00:00
abstract:OBJECTIVES:The aim of the study was to characterize the enteral feeding practices in infants after gastrointestinal surgery. METHODS:We performed a retrospective analysis of infants who underwent intestinal surgery at age <6 months who survived to be fed enterally between January 2012 and June 2017. Demographics, surg...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002128
更新日期:2019-02-01 00:00:00
abstract::Most children with chronic hepatitis C are infected vertically, have a low natural seroconversion rate, and carry a lifetime risk of cirrhosis and cancer. Affected children are usually asymptomatic, and histological findings are mild with a low risk of progression, although 5% develop significant liver disease in chil...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181f6f09c
更新日期:2011-02-01 00:00:00
abstract::Young infants with cystic fibrosis (CF) are at risk for developing symptomatic protein-calorie malnutrition (PCM) characterized by hypoalbuminemia, edema, and anemia. We reviewed the hospital charts of all infants less than 12 months of age referred to our CF clinic between 1979 and 1982 and found nine patients with P...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198605000-00010
更新日期:1986-05-01 00:00:00
abstract::Generalized edema and ascites were the main presenting features of giardiasis in a 3-year-old boy. Hypoalbuminemia, jejunal villous atrophy, Giardia lamblia in the duodenal aspirate, and abnormal gastrointestinal protein loss were present before therapy with metronidazole. Eradication of parasites resulted in complete...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198502000-00011
更新日期:1985-02-01 00:00:00
abstract::Chronic constipation is one of the most frequent complaints in childhood. Although there is evidence that gastrointestinal flora is important in gut motility, there is little evidence that gut flora is abnormal in constipation. Lactobacilli and bifidobacteria increase stool frequency and decrease consistency in normal...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3181a1603a
更新日期:2009-04-01 00:00:00
abstract:OBJECTIVES:Information on epithelial functions of the residual small or colonic bowel after resection for necrotising enterocolitis (NEC) in human infants is scarce. Our aim is to evaluate epithelial functions in the intestinal resection margins of tissue obtained at bowel resection for acute NEC and consecutive stoma ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318186d341
更新日期:2009-07-01 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:Nonalcoholic fatty liver disease (NAFLD) is a common cause of pediatric liver disease. Studies suggest decreased prevalence in blacks, females, and younger children. However, the proportion of black subjects in these studies was small, and children under 12 were not included. We propose that abnormal alanine...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000177314.65824.4d
更新日期:2005-10-01 00:00:00
abstract:OBJECTIVE:Standardized outcome measures provide a basis for comparing outcomes of different clinical trials. Consequently, they can serve as the foundation for determining which therapeutic interventions are most effective. The aim of the present study is to systematically assess how definitions and outcome measures ar...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0000000000001110
更新日期:2016-06-01 00:00:00
abstract:OBJECTIVES:The response to hepatitis A vaccine has not been studied in children with celiac disease (CD). The aim of the present study was to evaluate the immunogenicity of an inactivated hepatitis A virus (HAV) vaccine and the effect of the human leukocyte antigen (HLA) type on immunogenicity in children with CD. PAT...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318223b3ed
更新日期:2011-11-01 00:00:00
abstract::The clinical expression of allergic disease is the consequence of a series of complex gene-environment interactions that occur at the materno-fetal interface and throughout infancy, leading to persistence of the Th2 immune response. It has been proposed that atopic eczema is the cutaneous manifestation of a systemic d...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/01.mpg.0000338812.59686.6e
更新日期:2008-11-01 00:00:00
abstract:OBJECTIVES:Gender equality in the workplace has not been described in pediatric gastroenterology. METHODS:An electronic survey that explored perceptions of career parity, work-life balance, and workplace harassment was sent to all members of the North American Society for Pediatric Gastroenterology, Hepatology, and Nu...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000637
更新日期:2015-04-01 00:00:00
abstract::Thiopurines are used as a maintenance therapy in patients with ulcerative colitis (UC). For some patients the metabolism of thiopurines is unfavorable, leading to increased adverse effects, including hepatotoxicity. There are many reports in the adult literature concerning the manipulation of thiopurine metabolism wit...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000344
更新日期:2014-07-01 00:00:00
abstract:OBJECTIVES:The aim of the study was to validate a brief Bowel Habit Questionnaire (BHQ) with prospectively obtained data from a 14-day diary and to determine whether the BHQ predicts the development of medically significant constipation (MSC) during the following year. MATERIALS AND METHODS:The BHQ was distributed to ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31822504fb
更新日期:2011-11-01 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::A new statistical approach to the analysis of laboratory data has been introduced to optimize the use of absorption tests and gliadin antibody measurement for the diagnosis of childhood celiac disease. Serum antigliadin antibodies, as well as blood xylose, iron, and tryglycerides after oral load, were evaluated in 40 ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198707000-00008
更新日期:1987-07-01 00:00:00