Abstract:
:We describe a premature infant with cholestatic liver disease and protease inhibitor MS phenotype. This infant demonstrated an abnormally low serum alpha 1-antitrypsin concentration. Liver histologic studies revealed diastase-resistant, periodic acid-Schiff-positive globules inside hepatocytes. Immunoperoxidase staining for alpha 1-antitrypsin was positive. Electron microscopy showed amorphous material in the dilated lumina of the endoplasmic reticulum. These findings are characteristic of alpha 1-antitrypsin deficiency. We suggest that this usually nonpathologic phenotype resulted in cholestatic liver disease because of the cumulative effect of several cholestatic conditions.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Gourley MF,Gourley GR,Gilbert EF,Odell GBdoi
10.1097/00005176-198901000-00021subject
Has Abstractpub_date
1989-01-01 00:00:00pages
116-21issue
1eissn
0277-2116issn
1536-4801journal_volume
8pub_type
杂志文章,评审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:OBJECTIVES:To clarify the diagnostic usefulness of culturing Helicobacter pylori from gastric juice for the diagnosis of H. pylori infection in patients with neurologic impairment. METHODS:Seventy-five patients (44 males, 31 females) with severe neurologic impairment admitted to Tokyo Children's Rehabilitation Hospita...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200410000-00016
更新日期:2004-10-01 00:00:00
abstract:BACKGROUND AND AIM:Pediatric gastroenterology is the only pediatric subspecialty with nutrition as part of its official curriculum and objective; however, pediatric gastroenterology fellows believe that their baseline knowledge in nutrition is suboptimal. The purpose of the present study was to assess the perceived eff...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182638ce8
更新日期:2013-02-01 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: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:OBJECTIVES:The diagnosis of celiac disease (CD) is based on the histological identification of gluten-sensitive enteropathy and detection of anti-tissue transglutaminase antibodies (tTGA) and/or endomysial antibodies. Serial measurements of tTGA are now recommended as a follow-up strategy to monitor compliance with a g...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318232c459
更新日期:2012-03-01 00:00:00
abstract:OBJECTIVES:The aim of the study was to summarize short-term effectiveness, safety, and cost of using infliximab biosimilar (IFX-B) drugs, (Inflectra [Hospira] and Remsima [NAAP]) compared to originator infliximab (IFX-O) (Remicade [MSD]) in biologic naive pediatric inflammatory bowel disease in the United Kingdom. MET...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002011
更新日期:2018-10-01 00:00:00
abstract:BACKGROUND AND AIMS:The aim of the present study was to evaluate proinflammatory and anti-inflammatory cytokine levels in children with irritable bowel syndrome (IBS). PATIENTS AND METHODS:Thirty-five children with IBS (17 diarrhea-predominant, 7 constipation-predominant, and 11 mixed type) and 25 healthy children as ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181fd9816
更新日期:2011-04-01 00:00:00
abstract:BACKGROUND:Ursodeoxycholic acid (UDCA) has been shown to improve pruritus, alanine aminotransferase (ALT), and cholesterol levels in children with intrahepatic cholestatic liver disease. However, the effect of UDCA on quantitative tests of hepatic function in children is uncertain. METHODS:A 2.5-year, open label, cros...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章
doi:10.1097/00005176-199801000-00009
更新日期:1998-01-01 00:00:00
abstract:OBJECTIVES:This study was performed to compare the effects of oral midazolam and oral diazepam, administered with intravenous (IV) meperidine, on pre-procedural, procedural, and post-procedural sedation and recovery in children undergoing diagnostic upper endoscopy. The costs of pre-procedure sedation were compared for...
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:10.1097/00005176-200207000-00012
更新日期:2002-07-01 00:00:00
abstract:OBJECTIVES:Obesity has been associated with increased bone mass, but the mechanisms involved are still poorly understood. We aimed to explore the relation between bone mineral density and factors known to influence bone formation in obese and lean adolescents. METHODS:We recruited 24 obese and 25 lean adolescents in a...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000297
更新日期:2014-06-01 00:00:00
abstract::A systematic review of literature was performed on the practice of mucous fistula refeeding in neonates with short bowel syndrome. No randomised controlled studies were identified. Case series reports of 30 infants showed improved weight gain (from -2.36 +/- 1.24 to 21.06 +/- 3.02 g/d) and reduced need for total paren...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/01.mpg.0000228111.21735.02
更新日期:2006-08-01 00:00:00
abstract:BACKGROUND AND AIMS:Functional dyspepsia in childhood is commonly triggered by food allergen in sensitised individuals. We investigated the topography of eosinophils and mast cells in gastric antral lamina propria, the interaction of mast cell products with mucosal nerve fibres, and changes in gastric antral muscle slo...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318186008e
更新日期:2008-10-01 00:00:00
abstract:OBJECTIVES:Pediatric inflammatory bowel disease (IBD) often presents insidiously and standard blood tests are normal in 20% of patients. We hypothesize that fecal occult blood testing (FOBT) and the perianal examination in addition to blood tests provide important information during the screening process for IBD. The a...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000754
更新日期:2015-07-01 00:00:00
abstract::Although the pathophysiology of persistent diarrhea in children remains unclear, it has been suggested that it may be related to the composition of the food ingested. Since lactase deficiency and cow's milk protein intolerance are often identified in children with persistent diarrhea, replacement of milk with yogurt s...
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:10.1097/00005176-199011000-00011
更新日期:1990-11-01 00:00:00
abstract:BACKGROUND:There are few studies of Helicobacter pylori reinfection in childhood. In the current study the reinfection rate of H. pylori and ulcer recurrence were investigated during a follow-up period of 12 months or more in children who had undergone eradication therapy. METHODS:Twenty-seven patients aged 5 to 16 ye...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199811000-00009
更新日期:1998-11-01 00:00:00
abstract:OBJECTIVES:Inadequate response of the innate immune system to bacterial antigens present in the intestinal flora may play a role in the development of necrotizing enterocolitis (NEC). Pattern recognition receptors such as CD14, toll-like receptor (TLR) 4, and caspase-recruitment domain (CARD) 15 bind bacterial lipopoly...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000192246.47959.b2
更新日期:2006-01-01 00:00:00
abstract::We compared the in vivo transport of zinc in segments of jejunum, ileum, and colon of 2-week-old (suckling) and 6-week-old (adolescent) rats. In normal rats, net absorption and lumen to mucosa flux of zinc, normalized for differences in size of the intestine (mumol/h/g dry weight) were significantly greater in all per...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198201010-00020
更新日期:1982-01-01 00:00:00
abstract:OBJECTIVES:The aim of the present study was to describe the prevalence of childhood constipation, stool characteristics, and their relationship with toilet training and age of introduction of cow's milk. METHODS:A total of 4231 children born in 2004, from a birth cohort study, were assessed at 12, 24, and 48 months of...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318251482b
更新日期:2012-10-01 00:00:00
abstract:OBJECTIVE:Studies in adults characterized the role of the pregnane X receptor (PXR) in the pathophysiology of inflammatory bowel disease (IBD) with conflicting results; pediatric studies are still lacking. PATIENTS AND METHODS:Genotyping for the -25385C/T polymorphism of the PXR gene in 187 white children with IBD and...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31818de1a8
更新日期:2009-07-01 00:00:00
abstract:BACKGROUND:Patients with autoimmune diseases, such as type 1 diabetes mellitus, atopic dermatitis, autoimmune thyroid diseases, and multiple sclerosis have a different seasonality of month of birth (MOB) from the general population. This study was undertaken to determine the seasonality of MOB in children with celiac d...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181709530
更新日期:2009-02-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:Eosinophilic esophagitis (EE) is characterized by marked esophageal mucosal eosinophilia on histological examination. Although the clinical and histological features of EE are increasingly recognized, the overlap of clinical symptoms and histological findings with gastroesophageal reflux diseas...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181ef37a1
更新日期:2011-02-01 00:00:00
abstract:OBJECTIVES:Studies have shown that nitric oxide (NO) may play a major role in sustaining mucosal integrity; however, NO has been also implicated in the pathogenesis of ischemia/reperfusion (I/R)-related tissue injury. We investigated the effects of L-arginine and NG-nitro L-arginine methyl ester (L-NAME) on the acetylc...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000304450.54057.96
更新日期:2008-01-01 00:00:00
abstract:OBJECTIVES:Lutein and zeaxanthin are dietary carotenoids that may influence visual and cognitive development. The objective of this study was to provide the first data on distribution of carotenoids in the infant brain and compare concentrations in preterm and term infants. METHODS:Voluntarily donated brain tissues fr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000389
更新日期:2014-11-01 00:00:00
abstract:BACKGROUND:Immunologic development of soy-fed infants has not been extensively studied. Early studies of soy flour-based formulas showed decreased immunoglobulin production when soy protein intake was limited. However, there were no significant differences in rotavirus vaccine responses between breast-fed and soy prote...
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:10.1097/00005176-200202000-00007
更新日期:2002-02-01 00:00:00
abstract:OBJECTIVE:Antiendomysial antibody (EMA) production has been induced in vitro by the small bowel mucosa of celiac disease (CD) patients in clinical remission cultured in the presence of gliadin peptides. The aim of the present study was to use this in vitro system to determine whether it could be used to predict the cli...
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:10.1097/00005176-200502000-00015
更新日期:2005-02-01 00:00:00
abstract:OBJECTIVE:The aim of this study was to evaluate the accuracy of reduced-dose ¹³C-urea breath test (¹³C-UBT) and early sampling of exhaled breath for the detection of Helicobacter pylori infection in children and adolescents. METHODS:Patients up to 20 years old that underwent upper gastrointestinal endoscopy with gastr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,多中心研究
doi:10.1097/MPG.0b013e3182a02608
更新日期:2013-11-01 00:00:00
abstract::Calcium (Ca) and phosphorus (P) homeostasis were determined in 18 infants (birth weight, 2,810 +/- 135 g; gestational age, 37.4 +/- 0.5 weeks; mean +/- SEM) who received high or low Ca and P content (Ca, P) parenteral nutrition (PN) with a fixed, low dose of vitamin D (25 IU/dl). Nine infants were randomized into low ...
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:
更新日期:1987-01-01 00:00:00
abstract::BE is a disorder that occurs in children likely as a consequence of prolonged GE reflux of gastroduodenal contents. It usually presents with complications of GE reflux, but it also may be relatively silent in childhood and then present with adenocarcinoma in childhood or present in adulthood. Although seldom recognize...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:
更新日期:1993-05-01 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