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 some GSDs has allowed better control of hypoglycemia and metabolic complications. However, growth failure and liver, renal, and other complications are frequent problems in the long-term outcome. Hypoglycemia is the main biochemical consequence of GSD type I and some of the other GSDs. The basis of dietary therapy is nutritional manipulation to prevent hypoglycemia and improve metabolic dysfunction, with the use of continuous nocturnal intragastric feeding or cornstarch therapy at night and foods rich in starches with low concentrations of galactose and fructose during the day and to prevent hypoglycemia during the night.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Heller S,Worona L,Consuelo Adoi
10.1097/MPG.0b013e3181818ea5subject
Has Abstractpub_date
2008-08-01 00:00:00pages
S15-21eissn
0277-2116issn
1536-4801pii
00005176-200808001-00006journal_volume
47 Suppl 1pub_type
杂志文章,评审abstract:OBJECTIVES:Adult-based guidelines support the use of the pull (extraction) endoscopic technique in managing esophageal food bolus impactions (FBIs) but allow the consideration of the push (advancement) technique with caution based on available published literature. The North American Society for Pediatric Gastroenterol...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001639
更新日期:2018-01-01 00:00:00
abstract::To determine the frequency of gastric acid hypersecretion in infants with chronic malabsorption due to short bowel syndrome, acid secretory function was determined in 23 infants with malabsorption 2-22 months following small bowel resection and in a control group of 14 chronically ill, age- and weight-matched infants ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1986-03-01 00:00:00
abstract:BACKGROUND:The role of milk polyamines in the development of the gastrointestinal tract of human infants is presently unknown. Polyamine concentrations are higher in human milk than in infant formulas. The aim of the present study was to gather data on luminal polyamines by measuring gastric fluid and fecal polyamine c...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199704000-00006
更新日期:1997-04-01 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::In progressive familial intrahepatic cholestasis type 2 (PFIC-2), severe steatorrhea is often documented. However, pancreatic exocrine secretion has not yet been studied. In 14 children with PFIC-2, pancreatic function was assessed using standard fecal tests. Normal fecal lipase concentrations excluded isolated lipase...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000218154.26792.6a
更新日期:2006-04-01 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:The aims of this study were to compare laboratory indices of spontaneous bacterial peritonitis (SBP) and noninfected ascites in children with chronic liver disease and to determine the infectious agents involved in SBP. METHODS:The medical records of 90 children with chronic liver disease and ascites studie...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000177
更新日期:2014-01-01 00:00:00
abstract:OBJECTIVE:The aim of the study was to evaluate the tolerability, safety, and efficacy of polyethylene glycol (PEG) 3350 without electrolytes as a 1-day bowel preparation for colonoscopy in children. METHODS:A prospective study of 45 children undergoing colonoscopy prescribed PEG 3350 without electrolytes mixed with a ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182758c69
更新日期:2013-02-01 00:00:00
abstract::Sixteen children (3.5-14.7 years) with portal hypertension and variceal hemorrhage were treated by direct endoscopic variceal sclerotherapy. Follow-up clinical and endoscopic evaluations have been carried out over a 1-6-year period (mean, 2.3 years). Prior to sclerotherapy, three patients had undergone unsuccessful su...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198609000-00005
更新日期:1986-09-01 00:00:00
abstract::Acarboxy prothrombin was detected in the plasma of 48 of 128 umbilical cord plasma samples and in all of 65 patients receiving coumadin. None was found in 20 normal adults. Although the prevalence was the same in blacks, whites, and neonates of mixed origin, levels were significantly higher in blacks. There was a nega...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198406000-00025
更新日期:1984-06-01 00:00:00
abstract:OBJECTIVE:The present understanding of inflammatory bowel disease pathogenesis mainly relies on studies of adult patients. Therefore, we studied the balance between T-effector and regulatory cells in pediatric inflammatory bowel disease. METHODS:Quantitative polymerase chain reaction and immunohistochemistry served to...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182979252
更新日期:2013-09-01 00:00:00
abstract::A patient with chronic active hepatitis and medically unmanageable ascites developed staphylococcal bacteremia after insertion of a peritoneovenous LeVeen shunt. The patient developed glomerulonephritis, manifested by moderate proteinuria, microscopic hematuria with red cell casts, azotemia, and evidence of complement...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198510000-00029
更新日期:1985-10-01 00:00:00
abstract:OBJECTIVES:The aim of this review was to examine the characteristics of the faecal calprotectin assay in neonates and the evidence for its use as a noninvasive marker of intestinal illnesses during the neonatal period. METHODS:Bibliographic searches were performed in the MEDLINE electronic database up to February 2010...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3181e2ad72
更新日期:2010-11-01 00:00:00
abstract::Cholestatic liver disorders account for a large proportion of chronic liver ailments in adults, children and infants, and are among the leading indications for liver transplantation in all age groups. Recent studies have begun to characterize the cellular and molecular mechanisms of hepatocyte injury caused by the ret...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/01.mpg.0000226384.71859.16
更新日期:2006-07-01 00:00:00
abstract:OBJECTIVES:Intestinal failure-associated liver disease (IFALD) contributes to significant morbidity in pediatric patients with intestinal failure (IF); however, the use of parenteral nutrition (PN) with a fish oil-based intravenous (IV) emulsion (FO) has been associated with biochemical reversal of cholestasis and impr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000609
更新日期:2015-03-01 00:00:00
abstract:OBJECTIVES:A large retrospective multicentre study was conducted in Spain to evaluate the efficiency of the new European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) criteria for the diagnosis of coeliac disease (CD). METHODS:The study protocol was approved by the ethics committee of Hos...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,多中心研究
doi:10.1097/MPG.0000000000000870
更新日期:2016-02-01 00:00:00
abstract::This randomized, double-blind trial determined whether adding 90 mmol/L of alanine with a reduction in glucose to 90 mmol/L (alanine ORS) improves the efficacy of the standard oral rehydration solution (WHO-ORS). One hundred twenty-nine males aged 3-48 months with weight for length greater than or equal to 70% of NCHS...
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:1991-05-01 00:00:00
abstract::Eighteen children and adolescents, of a total of 90 treated in our unit between 1982 and 1992, underwent colectomy for ulceractive colitis. Six (6.7%) of the 90 patients had short stature, i.e., a height below the 3rd centile, and 8 of the 18 who had surgery had a subnormal height velocity, i.e., below the 25th centil...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199507000-00014
更新日期:1995-07-01 00:00:00
abstract:OBJECTIVES:The aim of the present study was to determine the incidence of peptic ulcer bleeding (PUB) in pediatric patients. METHODS:A hospital inpatient database, Premier Perspective, and an insurance claims database, MarketScan, were analyzed to estimate upper and lower limits for the annual incidence of PUB in the ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31824fb7f9
更新日期:2012-06-01 00:00:00
abstract::Eighteen infants with severe hypernatremic dehydration secondary to acute gastroenteritis were rehydrated during the 1st day with an oral glucose electrolyte solution containing 60 mmol sodium/L at a mean rate of 120 ml/kg/24 h. These 18 children were safely treated with oral therapy alone. No convulsions were observe...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1986-03-01 00:00:00
abstract:OBJECTIVES:Gastroesophageal reflux and aspiration can occur in premature infants who are supported with mechanical ventilation. The relation between physical positioning and gastric aspiration in ventilated infants has not been studied. Pepsin measured in tracheal aspirate (TA) emerged as a specific marker for aspirati...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/MPG.0000000000000601
更新日期:2015-03-01 00:00:00
abstract:OBJECTIVES:Patients with inflammatory bowel disease (IBD) are at risk for vitamin and mineral deficiencies because of long-term inflammation in the gut mucosa and decreased oral intake. The aim of the study is to investigate the prevalence of vitamin and zinc deficiencies in patients with newly diagnosed IBD compared w...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31826a105d
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:Lower gastrointestinal bleeding (LGB), particularly in newborns, is of serious concern and requires urgent investigation and hospital care. Whereas allergic proctocolitis caused by food protein is a significant cause of LGB in infants with eosinophilia, there are several cases of diseases with symptoms simil...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000252194.06955.18
更新日期:2007-04-01 00:00:00
abstract:OBJECTIVE:To conduct a comprehensive study of postendoscopic adverse events (AEs) in children, categorizing them by the level of intervention required. BACKGROUND:Previous studies of endoscopic AE in children have focused on intraprocedural and short-term outcomes, such as bleeding, perforation, and infection, and may...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001038
更新日期:2016-06-01 00:00:00
abstract::The plasma endotoxin levels of 16 severely malnourished Thai children were determined by the Limulus amebocyte lysate assay. Endotoxemia was present in seven children (44%) on admission to the hospital and was associated with the coexistence of vitamin A deficiency. Protein-energy malnutrition is associated with the p...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198803000-00012
更新日期:1988-03-01 00:00:00
abstract:OBJECTIVES:Differentiating ulcerative colitis (UC) and Crohn disease (CD) can be clinically challenging, especially in children. Granulomatous inflammation has traditionally been attributed to CD. Crypt-associated giant cells and granulomas, however, have been observed in colonic biopsies of patients with UC. This phen...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001771
更新日期:2018-04-01 00:00:00
abstract::Dual pH-multichannel intraluminal impedance (pH-MII) is a sensitive tool for evaluating overall gastroesophageal reflux disease, and particularly for permitting detection of nonacid reflux events. pH-MII technology is especially useful in the postprandial period or at other times when gastric contents are nonacidic.pH...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3181ffde67
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:OBJECTIVES:Zinc (Zn) homeostasis in adults is achieved principally through a balance between intestinal absorption and excretion involving adaptive mechanisms programmed by levels of dietary Zn. Zn absorption in infants is not as tightly regulated as that in adults, which may induce potential toxicity in infants due to...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181d98e85
更新日期:2010-06-01 00:00:00
abstract::Inflammatory bowel disease (IBD) affects many children and adolescents in terms of their confidence, acceptance, and ability to build friendships. New Zealand held its first summer camp for children with IBD in January 2015. We obtained feedback from the campers (ages 10-18 years) in terms of their confidence, accepta...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001526
更新日期:2017-09-01 00:00:00