Abstract:
OBJECTIVES:Low γ-glutamyl transpeptidase (GGT) level is an important marker for progressive familial intrahepatic cholestasis, yet the cutoff level and clinical application is not well defined. This study aimed to evaluate the role of GGT as a screening marker among diverse etiologies of infantile cholestasis. METHODS:This retrospective study analyzed 256 cholestatic infants admitted to a tertiary referral center between 2000 and 2012. After excluding 121 infants of extrahepatic cholestasis, advanced investigations for 135 infants with intrahepatic cholestasis were performed. The etiologies, outcomes, and correlations with GGT levels were analyzed. Good prognosis was defined as clinical recovery before 1 year of age; poor prognosis as persistent disease, liver transplantation, or death before 1 year. RESULTS:Among 135 patients of intrahepatic cholestasis, >12 different etiologies were found. Neonatal hepatitis (49.6%), progressive familial intrahepatic cholestasis (21.5%), and neonatal cholestasis caused by citrin deficiency (10.4%) were the leading causes. Patients with initial GGT between 75 and 300 U/L had a higher chance of good prognosis (61/74, 82.4%) than those with GGT <75 U/L or >300 U/L (25/61, 41%, P < 0.0001). In the low-GGT group (≤ 100 U/L), 52.6% (30/57) of the patients have good prognosis; and GGT level ≤ 75 U/L has a sensitivity, specificity, and positive predictive value of 100%, 43.3%, and 61.4% in predicting poor prognosis. CONCLUSIONS:Patients with GGT levels ≤ 75 or ≥ 300 U/L should receive advanced investigations such as genetic/metabolic assays early; otherwise, the amount of diagnostic workup may be limited if no signs of progressive disease.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Lu FT,Wu JF,Hsu HY,Ni YH,Chang MH,Chao CI,Chen HLdoi
10.1097/MPG.0000000000000538subject
Has Abstractpub_date
2014-12-01 00:00:00pages
695-701issue
6eissn
0277-2116issn
1536-4801journal_volume
59pub_type
杂志文章abstract:OBJECTIVE:Supplementing nutrition in children with severe epidermolysis bullosa (EB) is challenging because of skin and mucosal fragility. Percutaneous endoscopic gastrostomy is contraindicated in EB, whereas more invasive open surgical gastrostomy placement can be complicated by chronic leakage. The aim of the study w...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000256
更新日期:2014-05-01 00:00:00
abstract:BACKGROUND:Premature infants receiving alimentation with cow milk-based formulas run a considerably high risk of incipient late metabolic acidosis, an early stage developing of manifest late metabolic acidosis. Is bone metabolism involved in pathophysiologic mechanisms characterizing this early stage of retention acido...
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:10.1097/00005176-200111000-00010
更新日期:2001-11-01 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:OBJECTIVES:Differentiation of Crohn disease (CD) from ulcerative colitis (UC) is challenging when inflammation is predominantly colonic. The paediatric inflammatory bowel disease (PIBD) classes algorithm was developed to bring consistency to labelling, but used physician-assigned diagnosis as the criterion standard. We...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002544
更新日期:2020-02-01 00:00:00
abstract:OBJECTIVE:The coexistence of celiac disease (CeD) and eosinophilic esophagitis (EoE) in pediatric patients has been increasingly recognized. In the current study, we have aimed to assess the outcomes of therapeutic dietary interventions in a cohort of pediatric patients with CeD and EoE. METHODS:Pediatric patient reco...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章
doi:10.1097/MPG.0000000000002343
更新日期:2019-08-01 00:00:00
abstract::This study of 200 Uruguayans between 0 and 86 years old was designed to determine the prevalence of lactose malabsorption. Lactose intolerance is defined as a clinical syndrome of abdominal pain, diarrhea, flatulence, and bloating after the ingestion of a standard lactose tolerance test dose (2 g of lactose per kilogr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198705000-00012
更新日期:1987-05-01 00:00:00
abstract::Glycogen storage diseases (GSDs) are a group of inherited disorders characterized by enzyme defects that affect the glycogen synthesis and degradation cycle, classified according to the enzyme deficiency and the affected tissue. The understanding of GSD has increased in recent decades, and nutritional management of so...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3181818ea5
更新日期:2008-08-01 00:00:00
abstract:BACKGROUND:Indomethacin is commonly used in the Neonatal Intensive Care Unit to induce closure of the patent ductus arteriosus and to prevent intraventricular hemorrhage. It is known that indomethacin causes intestinal ulceration in adults, but its effect in infants is less clear. In a preliminary experiment, it was fo...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200208000-00010
更新日期:2002-08-01 00:00:00
abstract:OBJECTIVES:The purpose of this study was to evaluate the effect of calcium and vitamin D2 supplementation on bone mineral density (BMD) in children with inflammatory bowel disease (IBD). PATIENTS AND METHODS:This was an open-label, prospective study conducted over a 12-month period. Seventy-two patients were divided i...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3180dca0cc
更新日期:2007-11-01 00:00:00
abstract:INTRODUCTION:Including individuals with lived experience in pediatric inflammatory bowel disease (IBD) is essential to establishing a research agenda that is mutually impactful to both those treating and those experiencing the disease. METHODS:Using the James Lind Alliance approach to research priority setting, a 10-m...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002396
更新日期:2019-09-01 00:00:00
abstract:OBJECTIVES:Tryptophan not only is an amino acid essential to protein synthesis but also serves as a precursor in 2 important metabolic pathways: the serotonin and the kynurenine pathways. Tryptophan is related to sleeping patterns. The objective of the present study was to determine the tryptophan requirement of term i...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/MPG.0000000000000434
更新日期:2014-09-01 00:00:00
abstract:BACKGROUND:Hepatic lesions have been described in Alagille syndrome (ALGS) in isolated case reports, and most of these have been reported to be hepatocellular carcinoma. OBJECTIVES:The aim of the present study was to determine the frequency, imaging, and histopathologic characteristics of hepatic lesions in children w...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000945
更新日期:2016-02-01 00:00:00
abstract:OBJECTIVES:The aim of the present study was to determine the in vitro effect(s) of a bovine-based human breast milk fortifier (HMF) on human intestinal cells. HMF increases the expression of BCL2/adenovirus E1B 19 kDa protein-interacting protein (Bnip3) and cell death; the prostaglandin analogue misoprostol will rescue...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000876
更新日期:2015-11-01 00:00:00
abstract:OBJECTIVE:Portopulmonary hypertension (POPH) is a known complication of cirrhosis in adults, but there is little information on its incidence and outcome in children with liver disease. We report 14 patients with POPH and present a synthesis of the medical literature. METHODS:Diagnosis of POPH in the 14 patients was b...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000821
更新日期:2015-09-01 00:00:00
abstract:OBJECTIVES:Hepatitis-associated aplastic anemia (HAAA) is a potentially life-threatening diagnosis without clear treatment guidelines. The goal of the study was to characterize the presentation, evaluation, histopathology, and outcomes of therapy in children with HAAA to guide future research and to develop standardize...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002940
更新日期:2021-02-01 00:00:00
abstract:OBJECTIVES:Polymorphonuclear leukocytes (PMN) feature prominently in the mucosa, including in crypt abscesses, of patients with inflammatory bowel disease, yet the mediators that are responsible for this migration are unknown. We discovered that CXCR2 chemokines (reportedly elevated in the mucosa) have reduced potency ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182318d13
更新日期:2012-03-01 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::Light and electron microscopy was applied to surgical resections and endoscopic biopsies from the ileum and colon of 15 children with Crohn's disease in order to investigate the involvement of neutrophils in the disease process. Clear evidence was found of neutrophil infiltration of the lamina propria and epithelium, ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198705000-00021
更新日期:1987-05-01 00:00:00
abstract:BACKGROUND:Cholestasis is one of the major complications of parenteral nutrition. The purpose of this experimental study was to detect the effects of acetylsalicylic acid (ASA), vitamin E (Vit E), and interferon-alpha (IFN-alpha) on prevention of parenteral nutrition-associated cholestasis. METHODS:Ten experimental gr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199903000-00014
更新日期:1999-03-01 00:00:00
abstract:OBJECTIVE:To evaluate esophageal body motor contractions occurring during esophageal reflux in pediatric patients with gastro-esophageal reflux disease (GERD). METHODS:Patients referred for the evaluation of GERD who were evaluated with combined 24-hour pH probe and esophageal manometry test (MP24) were included. Pati...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200501000-00013
更新日期:2005-01-01 00:00:00
abstract:OBJECTIVES:Gastroesophageal reflux is common but remains a controversial disease to diagnose and treat and little is known about the role of reflux testing in predicting clinical outcomes, particularly in children at risk for extraesophageal reflux complications. The aim of this study was to determine if rates of hospi...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章
doi:10.1097/MPG.0000000000001092
更新日期:2016-08-01 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:BACKGROUND:Gastric lipase has an important compensatory function in neonatal fat digestion. The activity level of pepsin and its role in protein digestion is less well understood. We have, therefore, studied the ontogeny of lipase and pepsin in the ferret, a species with a neonatal fat digestion pattern similar to that...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199802000-00007
更新日期:1998-02-01 00:00:00
abstract:OBJECTIVES:Button battery ingestions are potentially life threatening for children. Catastrophic and fatal injuries can occur when the battery becomes lodged in the esophagus, where battery-induced injury can extend beyond the esophagus to the trachea or aorta. Increased production of larger, more powerful button batte...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181f98916
更新日期:2011-05-01 00:00:00
abstract::Aspirin reduced plasma concentrations of prostaglandin (PG) F alpha in 11 of 12 children with toddler diarrhoea, and usually, but not always, controlled the symptom. Loperamide consistently controlled toddler diarrhoea in 10 patients but had no effect on plasma PGF alpha. In eight patients experiencing spontaneous rem...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1985-06-01 00:00:00
abstract:OBJECTIVES:Biliary dyskinesia is a common diagnosis that frequently results in cholecystectomy. In adults, most clinicians use a cut off value for the gallbladder ejection fraction (GBEF) of <35% to define the disease. This disorder is not well characterized in children. Our aim was to determine the relation between GB...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001065
更新日期:2016-07-01 00:00:00
abstract:BACKGROUND AND AIM:Distinguishing biliary atresia (BA) from other causes of neonatal cholestasis (NC) is challenging. Continuous BreathID C-methacetin breath test (MBT) is a novel method that determines liver function. Methacetin is metabolized uniquely by the liver and CO2 is measured passively, through a nasal cannul...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章
doi:10.1097/MPG.0b013e3182638d29
更新日期:2013-01-01 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::Nineteen patients with cystic fibrosis (CF) were studied to determine whether plasma fatty acids correlated with severity of their lung disease as assessed by pulmonary function testing. Results were compared with 19 normal subjects of similar age and sex. Linoleic acid content of all lipid fractions was significantly...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198605000-00013
更新日期:1986-05-01 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