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 based on right-sided heart catheterization displaying mean pulmonary artery pressure (mPAP) >25 mmHg, indexed pulmonary vascular resistances >3 Wood units · m, and pulmonary wedge pressure <15 mmHg. A literature review added 84 patients. RESULTS:In our unit, POPH was found in 0.5% of the children with portal hypertension, 0.9% of the children with end-stage liver disease awaiting transplantation, and 3 children with congenital portosystemic shunts (CPSSs). Analysis of 98 reported patients, including the 14 presented here, showed the cause of liver disease to be chronic liver disease or portal cavernoma in 76 instances (34 with a history of surgical portosystemic shunt) and CPSS in 22 instances. There was a precession with proven hypoxemia caused by hepatopulmonary syndrome in 6 patients. Median survival was 3 months in 56 untreated patients. An 80% 5-year probability of survival in 42 patients was treated by CPSS closure, pulmonary vasodilators, and/or liver transplantation. Mean pretransplant mPAP was 34 and 49 mmHg in transplant survivors and nonsurvivors, respectively. CONCLUSIONS:POPH is a rare but extremely severe complication of childhood liver disease. Portosystemic shunts, whether congenital or acquired, likely play an important causative role. Early diagnosis is crucial and requires systematic screening by echocardiography in children at risk.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Ecochard-Dugelay E,Lambert V,Schleich JM,Duché M,Jacquemin E,Bernard Odoi
10.1097/MPG.0000000000000821subject
Has Abstractpub_date
2015-09-01 00:00:00pages
346-54issue
3eissn
0277-2116issn
1536-4801journal_volume
61pub_type
杂志文章abstract::Duodenal biopsies from 33 celiac and 16 nonceliac patients were kept in organ culture for 24 h. Quantities of immunoglobulins were measured by rocket immunoelectrophoresis in mucus removed from the biopsy surface after culture and in culture media. Increased amounts of immunoglobulins were recorded after culture of bi...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198302010-00008
更新日期:1983-01-01 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:OBJECTIVE:Breast milk fatty acids may have immunomodulatory properties related to the development of atopic disease. The aim of this study was to assess the impact of the breast milk fatty acid composition on the development of atopic dermatitis (AD) in high-risk infants. METHODS:Mothers with atopic disease were recru...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000168992.44428.fa
更新日期:2005-09-01 00:00:00
abstract:OBJECTIVES:In the present study, we studied a cohort of patients with very early onset inflammatory bowel disease (IBD) to determine the frequency of mutations in the interleukin 10 (IL10) receptor genes as a cause of early-onset IBD. METHODS:Sanger sequencing was performed to determine the presence of IL10 and/or IL1...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000621
更新日期:2015-03-01 00:00:00
abstract:BACKGROUND AND AIMS:Pediatric ulcerative colitis (UC) shares many features with adult-onset disease but there are some unique considerations; therefore, therapeutic approaches have to be adapted to these particular needs. We aimed to formulate guidelines for managing UC in children based on a systematic review (SR) of ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,实务指引
doi:10.1097/MPG.0b013e3182662233
更新日期:2012-09-01 00:00:00
abstract:BACKGROUND:Animal and human studies have suggested that trans fatty acids might alter some physiological functions and adversely affect the growth and essential fatty acid balance of infants. In this context it is important to know the fatty acid composition, including the levels of trans isomers of oleic, linoleic and...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199710000-00007
更新日期:1997-10-01 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
abstract:OBJECTIVES:: Biliary atresia (BA) is a leading cause of end-stage paediatric liver disease. Standard BA treatment is sequential surgery with an initial Kasai procedure (KP) followed by liver transplant (LT) for patients who progress to liver failure. A key determinant for the post-KP patient survival with their native ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181d67e5e
更新日期:2010-07-01 00:00:00
abstract::Alkaline phosphatase (AP) activity is an accepted marker of epithelial cell differentiation and integrity in intestinal mucosal explant systems. In experiments involving AP it is usually expressed as activity per unit protein in tissue culture. It has been previously demonstrated that during organ culture a considerab...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198403000-00007
更新日期:1984-03-01 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: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:OBJECTIVES:Beneficial therapeutic effect of probiotics has been reported in children with the irritable bowel syndrome (IBS) but not consistently in other functional abdominal pain-related disorders. The aim of the present study was to investigate the effect of Lactobacillus reuteri DSM 17938 in the treatment of functi...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/MPG.0000000000001478
更新日期:2017-06-01 00:00:00
abstract:OBJECTIVE:To investigate and compare the effects of enteral glutamine and arginine supply on lipid peroxidation and antioxidant enzyme levels in the small intestine of healthy breast-fed rats. MATERIALS AND METHODS:The study comprised 40 newborn Sprague-Dawley rats born to 5 mother rats. Newborn rats were randomly div...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318198cd36
更新日期:2009-07-01 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:BACKGROUND:Selective cannulation of the common bile duct (CBD) during endoscopic retrograde cholangiopancreatography (ERCP) can be difficult. Several techniques have been described to assist endoscopists in obtaining access when initial cannulation fails. The objective of this report is to describe our initial experien...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318054e1f4
更新日期:2007-10-01 00:00:00
abstract:OBJECTIVES:The aim of the present review was to provide recommendations on the use of hydrolysates in infants when formula feeding is initiated. METHODS:We performed an overview of reviews followed by a systematic review of subsequently published trials. RESULTS:We found 8 systematic reviews; only 1 study of limited ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0000000000000318
更新日期:2014-05-01 00:00:00
abstract:OBJECTIVES:Small for gestational age (SGA) infants are more susceptible to infectious morbidity and growth faltering compared to their appropriate for gestational age (AGA) counterparts. Zinc supplementation of SGA infants may be beneficial but the underlying susceptibility to zinc deficiency of SGA infants has not bee...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001778
更新日期:2018-03-01 00:00:00
abstract::To evaluate the possible contribution of enteric losses of alpha 1-antitrypsin (alpha 1-AT) to the low serum levels of alpha 1-AT seen in patients with alpha 1-AT deficiency, we investigated intestinal clearance of alpha 1-AT (C-alpha 1-AT) in five of these patients (mean age 3.4 years) and compared it to that of 10 p...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198302010-00010
更新日期:1983-01-01 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::In preterm infants, protein-turnover rates obtained by [15N]glycine as a tracer are known to be overestimated. This may reflect the insufficient supply of dietary glycine. In this randomized study, the influence of a glycine-rich diet on whole body protein turnover rates in eight male preterm infants (29-32 weeks, 1,2...
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:10.1097/00005176-199610000-00014
更新日期:1996-10-01 00:00:00
abstract:BACKGROUND:Immune response to hepatitis B virus (HBV) antigens or mitogens in Asian children with chronic HBV infection who are mainly perinatally infected has not been studied in connection with the production of various cytokines, although these patients are considered to be less responsive to antiviral therapy. MET...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199911000-00013
更新日期:1999-11-01 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:BACKGROUND:In clinical and field conditions, breath gas analysis has been widely used in evaluating carbohydrate digestion. A field study was performed to determine the prevalence of lactose malabsorption in Myanmar children and to evaluate the possibility of using breath methane excretion to indicate lactose malabsorp...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199905000-00006
更新日期:1999-05-01 00:00:00
abstract:OBJECTIVES:This study aims to develop a new prognostic score based on changes in serial laboratory data from patients with pediatric acute liver failure (PALF). METHODS:We retrospectively reviewed data on 146 patients with PALF at the Seoul National University Children Hospital (SNUCH) and the Asan Medical Center (AMC...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002625
更新日期:2020-06-01 00:00:00
abstract::A 3-year-old boy with transient protein loss due to hypertrophic gastropathy is described. Serologic evidence of concomitant Mycoplasma pneumoniae infection was present. Although sometimes associated with infections, protein-losing hypertrophic gastropathy has not been reported previously in association with Mycoplasm...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199202000-00021
更新日期:1992-02-01 00:00:00
abstract::We describe a child born to unrelated parents who developed severe protracted secretory type diarrhea associated with subtotal villus atrophy and intestinal inflammation at the age of 19 months. No infectious, metabolic, or anatomical basis for this condition was identified and the child required total parenteral nutr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199002000-00021
更新日期:1990-02-01 00:00:00
abstract:BACKGROUND:There are few data concerning the risk of contamination of enteral feeding systems via gastrostomy in children, and none for conditions that pertain to home-based care. METHODS:To investigate the risk of contamination of enteral feeding systems during the home-based care of 20 children receiving gastrostomy...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200109000-00007
更新日期:2001-09-01 00:00:00
abstract::Glutamine is the major fuel for enterocytes and prevents mucosal atrophy in certain animal models. Previous studies in our laboratory have failed to show a trophic effect of glutamine on the small-bowel mucosa following massive resection when added to a chow diet. However, the complexity of the chow diet might potenti...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199511000-00005
更新日期:1995-11-01 00:00:00
abstract:BACKGROUND:Early postnatal glucocorticoid exposure accelerates the maturation of the bowel mucosa but results in bowel wall thinning in the newborn mouse ileum and increases the risk of focal ileal perforation in extremely premature infants. We have previously demonstrated a redistribution of insulin-like growth factor...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200210000-00014
更新日期:2002-10-01 00:00:00
abstract:OBJECTIVES:Environmental enteropathy (EE) is likely associated with growth retardation in children, but the association between EE and length velocity z score (LVZ) has not been investigated. The objective of the study was to assess associations between fecal markers for intestinal inflammation and LVZ and whether thes...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001990
更新日期:2018-08-01 00:00:00