Abstract:
BACKGROUND:Feeding impairment frequently complicates the course of children with neurologic disorders and places them at risk for malnutrition and growth failure. Although feeding abnormalities have been reported in female patients with Rett syndrome, the mechanisms that account for these findings have not been elucidated fully. This study was designed to characterize the clinical features of oropharyngeal and gastroesophageal dysfunction and their impact on the dietary intake and nutritional status of female subjects with Rett syndrome. METHODS:The clinical features of oropharyngeal and gastroesophageal dysfunction in 13 female patients with Rett syndrome, (age range, 3.7 to 25.7 years) were characterized by an oral feeding assessment, swallowing function study, and upper gastrointestinal series. Growth, nutritional status, and body composition were determined by stadiometry and anthropometry. Dietary intakes were determined from 3-day food records. RESULTS:Oropharyngeal dysfunction and gastroesophageal dysmotility were present in 100% and 69%, respectively, of the study patients with Rett syndrome. The scope and severity of these abnormalities were apparent only by videofluoroscopy. Abnormalities of oropharyngeal function included poor tongue mobility, reduced oropharyngeal clearance, and laryngeal penetration of liquids and solid food during swallowing. Esophageal dysmotility included absent primary or secondary waves, delayed emptying, atony, the presence of tertiary waves, spasm, and gastroesophageal reflux. Gastric dysmotility included diminished peristalsis or atony. Lower dietary energy intakes were associated with persistence of residue in the valleculae and pyriform sinuses and less body fat. CONCLUSION:The prevalence of oropharyngeal dysfunction and gastroesophageal dysmotility warrants early diagnostic evaluation and intervention strategies to improve the nutritional status of girls and women with RS.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Motil KJ,Schultz RJ,Browning K,Trautwein L,Glaze DGdoi
10.1097/00005176-199907000-00010subject
Has Abstractpub_date
1999-07-01 00:00:00pages
31-7issue
1eissn
0277-2116issn
1536-4801journal_volume
29pub_type
杂志文章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: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...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000538
更新日期:2014-12-01 00:00:00
abstract::The contrasting effects of natural versus cow's milk formula (CMF) on upper gastrointestinal development in the newborn were studied. The passive intestinal permeability of neonatal human infants and guinea pigs was measured using the nonmetabolisable markers lactulose and mannitol. Mucosal morphology was examined in ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198705000-00008
更新日期:1987-05-01 00:00:00
abstract:OBJECTIVES:Parents of children with autism spectrum disorders (ASDs) often report gastrointestinal (GI) dysfunction in their children. The objectives of the present study were to determine whether infants at high risk for developing ASD (ie, siblings of children diagnosed as having ASD) show greater prevalence of GI pr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000907
更新日期:2016-02-01 00:00:00
abstract::We evaluated plasma PIVKA-II (protein induced by vitamin K absence or antagonist-II, acarboxy prothrombin) levels in three infants with hepatoblastoma as a tumor marker. PIVKA-II levels were highly elevated in all three patients. Vitamin K administration, performed in two patients, resulted in only moderate reduction ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198701000-00008
更新日期:1987-01-01 00:00:00
abstract::Serum immunoglobulin (Ig) A and IgG gliadin antibodies were determined with a simple, rapid, and inexpensive method--diffusion-in-gel enzyme-linked immunosorbent assay (DIG-ELISA)--and the results were related to small intestinal mucosal morphology in 234 children suspected of having malabsorption. Fifty-six of 58 chi...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198512000-00011
更新日期:1985-12-01 00:00:00
abstract::Few advances would contribute more to the growth and well-being of infants and children in the developing world than the making available of acceptable staple foods that are easy to grow and process, nutritionally complete enough to serve as the major weaning food, and safe without refrigeration. High-fat quality-prot...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199308000-00003
更新日期:1993-08-01 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::We reviewed data from 184 percutaneous liver biopsies performed as an outpatient procedure in 104 patients (5 weeks to 22 years of age) over a 4-year period. Hepatic allograft rejection was the indication for 130 of the biopsy procedures, while 54 were performed for a variety of indications in patients who had not und...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199311000-00006
更新日期:1993-11-01 00:00:00
abstract::Colonoscopy is a routinely performed procedure in children and adolescents. Proper visualization of the intestinal mucosa, completion of the procedure, including examination of the terminal ileum, detection of pathological lesions, and therapeutic maneuvers, all are highly dependent on the quality of the bowel prepara...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3181eb6a1c
更新日期:2010-09-01 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:BACKGROUND:Chronic enterocolitis is rare in infancy and accounts for less than 0.5% of all newly diagnosed inflammatory bowel disease (IBD) in the UK. Presentation at this young age is usually indicative of underlying immunodeficiency/immunodysregulation. A group of such infants suffer intractable ulcerating enterocoli...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000159622.88342.bc
更新日期:2005-05-01 00:00:00
abstract:BACKGROUND:Results of in vitro studies have documented colonic absorption of lactose in the newborn. A stable isotope model was developed for assessing the entry rate of intact lactose into the portal circulation in newborn piglets. METHODS:In experiment 1, unlabeled and [D-1-(13C)]-lactose were infused into two separ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199707000-00011
更新日期:1997-07-01 00:00:00
abstract:OBJECTIVES:: Several serologic assays are commercially available to aid in the diagnosis of gluten-sensitive enteropathy (GSE). Our objective in this study was to assess the performance of a novel combined antigen-screening assay for GSE. PATIENTS AND METHODS:: Deidentified sera from 111 pediatric patients suspected o...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181c992be
更新日期:2010-07-01 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:BACKGROUND:We analyzed the role that nutrition and the insulin-like growth factors IGF-I and IGFBP-3 play on neonatal growth. METHODS:Full-term and preterm infants with 1 and 3 weeks of postnatal life (n = 54 and n = 33, respectively) were studied. Anthropmetric variables, daily intake of energy and nutrients, and ser...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199703000-00016
更新日期:1997-03-01 00:00:00
abstract:OBJECTIVES:Autoimmune pancreatitis (AIP) represents a complex immune-mediated pancreas disorder. Pediatric AIP (P-AIP) is rare. We have recently summarized the characteristic features of P-AIP. We now aim to develop recommendation statements to standardize the diagnostic and therapeutic approach to P-AIP and facilitate...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 共识发展会议,杂志文章,实务指引
doi:10.1097/MPG.0000000000002028
更新日期:2018-08-01 00:00:00
abstract:OBJECTIVES:Functional dyspepsia (FD) is a functional abdominal pain disorder. There is paucity of data on the economic impact of FD in children. Primary aim of our study was to estimate annual evaluation cost ("diagnosis and visit" cost) and secondary aim was to identify potential prognostic factors of FD in children. ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002599
更新日期:2020-04-01 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:OBJECTIVES:Congenital portosystemic shunts (CPSSs) are rare but increasingly recognized as a cause of important multisystem morbidity. We present new cases and a systematic literature review and propose an algorithm for the identification and care of affected patients. METHODS:We reviewed the charts of consecutive pat...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e31828b3750
更新日期:2013-06-01 00:00:00
abstract::Cyclic vomiting syndrome (CVS) is a disorder noted for its unique intensity of vomiting, repeated emergency department visits and hospitalizations, and reduced quality of life. It is often misdiagnosed due to the unappreciated pattern of recurrence and lack of confirmatory testing. Because no accepted approach to mana...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 共识发展会议,杂志文章
doi:10.1097/MPG.0b013e318173ed39
更新日期:2008-09-01 00:00:00
abstract::Eicosanoids, derivatives of arachidonic acid, play a role in several inflammatory diseases of the bowel. To determine whether prostaglandin E2 (PGE2), leukotriene B4 (LTB4), and leukotriene C4D4E4 (LTC4D4E4), have a role in hypoxic insult to the intestine, we examined the levels of these mediators in a hypoxic neonata...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199208000-00005
更新日期:1992-08-01 00:00:00
abstract:OBJECTIVE:Previous studies have suggested that hepatitis B virus (HBV) vaccines may be less immunogenic in individuals with celiac disease (CD). A pre-S vaccine (Sci-B-Vac) has demonstrated superior immunogenicity compared with standard HBV vaccines in several diseases. We compared the short-term immunogenicity of a pr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/MPG.0000000000000856
更新日期:2015-10-01 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:Many patients with chronic hepatitis C do not respond to antiviral treatment. In adult patients the re-treatment of these patients has been extensively investigated. Because the response to re-treatment in children is not well defined we evaluated the efficacy and safety of interferon (IFN)-alpha plus ribavi...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章
doi:10.1097/MPG.0b013e3181d9c7f6
更新日期:2010-08-01 00:00:00
abstract:OBJECTIVES:The most common cause of encopresis in children is functional fecal retention (FFR). An international working team suggested that FFR be defined by the following criteria: a history of >12 weeks of passage of <2 large-diameter bowel movements (BMs) per week, retentive posturing, and accompanying symptoms, su...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200401000-00018
更新日期:2004-01-01 00:00:00
abstract:PURPOSE OF REVIEW:Clinical evidence of the use of probiotics in pediatric NAFLD, framed within gut microbiota response, is herein discussed. RECENT FINDINGS:The assistance of a new microbiological approach in probiotics' design is playing a central role in NAFLD clinical management. Experimental studies demonstrated t...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0000000000001220
更新日期:2016-07-01 00:00:00
abstract:BACKGROUND:In patients with biliary atresia (BA), liver fibrosis and cirrhosis commonly occur even after Kasai hepatoportoenterostomy. Although liver biopsy is the gold standard to evaluate liver fibrosis, it is invasive and may result in life-threatening complications. The aspartate aminotransferase-to-platelet ratio ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181da1d98
更新日期:2010-08-01 00:00:00
abstract:OBJECTIVE:The purpose of this study was to evaluate the effects of oropharyngeal colostrum administration in the incidence of late-onset clinical and proven sepsis and in concentrations of immunoglobulin A (IgA) in very-low-birth-weight (VLBW) infants. METHODS:We conducted a double-blinded, randomized, placebo-control...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/MPG.0000000000002356
更新日期:2019-07-01 00:00:00
abstract::We assessed the effect of pH on volatilization of short-chain fatty acids during lyophilization. Acetic, propionic, valeric, and butyric acids were added to a fecal homogenate in amounts sufficient to raise the energy density by 18-27%. Fecal homogenate samples were either acidified (pH 2.8-3.2), alkalinized (pH 7.9-8...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1987-11-01 00:00:00