Abstract:
BACKGROUND:Liver transplantation is established treatment for children with end-stage liver disease and has a 5-year survival rate of 80% to 85%, even in infants under 12 months. Long-term outcome in nutritional rehabilitation and normal development is unknown. This study aimed to prospectively evaluate growth and psychoneurologic performance of children who undergo liver transplantation in infancy. METHODS:Twenty-five infants (18 girls, 7 boys) who underwent liver transplantation at less than 12 months of age (median age, 9 months) were evaluated for 4 years. Growth measurements were expressed as standard deviation scores (SDSs; mean +/- SEM), and psychoneurologic performance was assessed with the unrevised Griffiths Mental Ability Scales (normal range, 80-120). RESULTS:Four children died during the study (4-year survival, 84%). The children were malnourished before transplantation (SDSs: weight, -1.9 +/- 0.2; midarm muscle area, -0.93 +/- 0.3; midarm fat area, -1.52 +/- 0.3; and height, -0.95 +/- 0.3). Nutritional rehabilitation for all parameters occurred within 12 to 24 months after transplantation, which was most significant for weight (-1.1 +/- 0.2, P = 0.001), midarm muscle area (0.74 +/- 0.3, P = 0.001), and midarm fat area (-0.44 +/- 0.3, P = 0.01). There was some improvement in height (-0.72 +/- 0.3, P = 0.14), which was not significant, although infants who were severely stunted before transplantation (mean height standard deviation score [SDS] -2.46) showed significant catch-up at 1 year after transplantation (mean height SDS -1.2, P = 0.003). Psychoneurologic scores were within normal limits before transplantation and were maintained for the 4-year follow-up period, although individual scores varied during this period. Improved nutritional status was associated with increased muscle bulk and subsequent improvement in motor scores from 90.6 at initial assessment to 97.3 at 4 years (P = 0.28). There was a temporary reduction in social skills and eye-hand coordination in the first year, which may have been an effect of the hospital environment or cyclosporine immunosuppression. Language abilities also regressed during the first year, possibly related to the effect of nasogastric tube feeding in delaying normal speech development. CONCLUSIONS:Liver transplantation in infancy has not only a successful outcome but is also associated with long-term catch-up growth and nutrition and maintenance of normal development.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
van Mourik ID,Beath SV,Brook GA,Cash AJ,Mayer AD,Buckels JA,Kelly DAdoi
10.1097/00005176-200003000-00011subject
Has Abstractpub_date
2000-03-01 00:00:00pages
269-75issue
3eissn
0277-2116issn
1536-4801journal_volume
30pub_type
杂志文章abstract:BACKGROUND:Shwachman's syndrome is the second most common cause of inherited/congenital pancreatic insufficiency after cystic fibrosis. The main associated features are usually cyclic neutropenia, metaphyseal dysostosis, and growth retardation. Other organs or functions may be involved in this syndrome, showing a wide ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199909000-00006
更新日期:1999-09-01 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:OBJECTIVE:The hypothesis that prenatal stress lowers the levels of protective microflora and increases the risk for postpartum Gram-negative pathogens was tested in infant monkeys. METHODS:Female monkeys were left undisturbed or were stressed during pregnancy using an acoustical startle paradigm for 6 weeks either ear...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200404000-00009
更新日期:2004-04-01 00:00:00
abstract::Preterm infant formulas (PIFs) for very-low-birth-weight (VLBW) infants (birth weight, < 1,500 g) are augmented to provide daily riboflavin and pyridoxine at levels up to five-fold greater than in term infant formula and 18-fold greater than in human milk. We evaluated plasma riboflavin and pyridoxine concentrations i...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章
doi:10.1097/00005176-199608000-00008
更新日期:1996-08-01 00:00:00
abstract::The synthetic opiate loperamide (LPMD) is an antidiarrheal compound which affects both intestinal motility and the transport of water and electrolytes. We have investigated its effects on ion transport in the stripped ileal mucosa of rabbits, mounted in Ussing chambers in vitro. Addition of LPMD to serosal bathing med...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198409000-00020
更新日期:1984-09-01 00:00:00
abstract:OBJECTIVES:The aim of the present study was to determine whether serial gastric volumes and intragastric curding are repeatable within individual preterm infants when given sequential feeds of the same volume and composition. Infant and feeding characteristics that may influence differences in measured gastric volumes,...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000397
更新日期:2014-08-01 00:00:00
abstract::To characterize the mechanisms leading to dietary evoked increases of lactase and sucrase activities by carbohydrates, we performed a quantitative comparison of the effects of lactose and sucrose on the corresponding disaccharidases in the jejunum of 2-month-old rats. For 7-10 days the rats were fed a low-starch (5 ca...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198512000-00024
更新日期:1985-12-01 00:00:00
abstract:OBJECTIVE:The aim of this study was to study the effect of continued Lactobacillus rhamnosus GG strain (LGG) feeding on rotavirus gastroenteritis in the gnotobiotic (Gn) pig model of virulent human rotavirus (HRV) infection. METHODS:Gn pigs were assigned to treatment groups: mock control, LGG only, HRV only, or LGG pl...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182a356e1
更新日期:2013-12-01 00:00:00
abstract::This clinical report aims to review key self-management and adherence issues in pediatric inflammatory bowel disease (IBD) and to provide recommendations for health care providers regarding evidence-based assessment and treatment approaches to promote optimal self-management. Self-management difficulties in the form o...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182999b21
更新日期:2013-08-01 00:00:00
abstract::The levels of secretory IgA and of IgM and IgG in colostrums and milks obtained at various times postpartum were measured by radial immunodiffusion. Immunoglobulin A was the dominant immunoglobulin in all samples, and concentrations in colostrum ranged from 1.5 to 83.7 g/L (mean 32 g/L). Immunoglobulin M and IgG had m...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198201030-00018
更新日期:1982-01-01 00:00:00
abstract:OBJECTIVES:Glucagon-like peptide 2 (GLP-2) is a 33 amino acid peptide hormone released from enteroendocrine L-cells following nutrient ingestion. It has been shown to exert trophic effects on the gut. We set out to measure GLP-2 concentrations in blood in children with diarrhoea and malnutrition. METHODS:GLP-2 levels ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002633
更新日期:2020-04-01 00:00:00
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::The aim of this study was to investigate the feasibility of using applied potential tomography (APT), a noninvasive and nonradioactive method, to measure the rate of gastric emptying in preterm babies and infants < 3 months old. APT, a form of electrical impedance tomography, creates tomographic images of tissue resis...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199501000-00012
更新日期:1995-01-01 00:00:00
abstract:OBJECTIVES:The response to hepatitis A vaccine has not been studied in children with celiac disease (CD). The aim of the present study was to evaluate the immunogenicity of an inactivated hepatitis A virus (HAV) vaccine and the effect of the human leukocyte antigen (HLA) type on immunogenicity in children with CD. PAT...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318223b3ed
更新日期:2011-11-01 00:00:00
abstract:OBJECTIVE:The outcomes of fundoplication for gastroesophageal reflux disease are suboptimal in many children, and alternatives are clearly needed. Dextranomer hyaluronic acid (DxHA) copolymer, an agent with proven efficacy in vesicoureteral reflux, was studied with respect to its effects on the gastroesophageal junctio...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000259
更新日期:2014-05-01 00:00:00
abstract::The apolipoprotein (apo) A-I:B ratio and the apo B concentration were determined by radial immunodiffusion in dried blood spot samples from 1,767 10- and 11-year-old children. Children with either apo A-I:B ratios below the first percentile or apo B levels above the 99th were recalled and plasma lipid and apolipoprote...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1990-02-01 00:00:00
abstract::Somatic gene therapy represents a new approach for treating a variety of genetic and acquired diseases and has many potential applications in gastroenterology. Somatic gene therapy is based on the ability to transfer recombinant genes efficiently into somatic tissues, such as the liver or intestine, and achieve expres...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/00005176-199204000-00017
更新日期:1992-04-01 00:00:00
abstract::Because of the variability of human milk carbohydrate composition, we determined the discriminant carbohydrate components of the milk of 18 mothers according to their ABH and Lewis secretor types during the first week of lactation. Comparative chromatograms revealed that the presence of neuraminyloligosaccharides is l...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199010000-00014
更新日期:1990-10-01 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::Stools of 65 patients with exacerbation of symptoms of inflammatory bowel disease were examined for the presence of enteric pathogens and Clostridium difficile. Ten (16%) had C. difficile toxin. Symptoms in all patients cleared after therapy, with improvement correlating with elimination of toxin from the stool. ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199107000-00007
更新日期:1991-07-01 00:00:00
abstract:OBJECTIVES:The aim of this systematic review and meta-analysis was to assess immune response to vaccination in children and young people with inflammatory bowel disease (IBD). In patients with IBDs, both the disease itself and its treatment can affect the vaccine response. METHODS:Medical databases were searched for r...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002810
更新日期:2020-10-01 00:00:00
abstract:OBJECTIVES:Little is known about the effect of diet on the oral microbiota of infants, although diet is known to affect the gut microbiota. The aims of the present study were to compare the oral microbiota in breast-fed and formula-fed infants, and investigate growth inhibition of streptococci by infant-isolated lactob...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31826f2bc6
更新日期:2013-02-01 00:00:00
abstract:BACKGROUND:Health-related quality of life (HRQOL) describes the physical, social, and emotional attitudes and behavior of people in relation to health status. By the 1980s, HRQOL was noted to be impaired in chronic diseases but inflammatory bowel disease, had not been extensively described. METHODS:This review summari...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/00005176-199904001-00003
更新日期:1999-04-01 00:00:00
abstract::To determine whether zinc has a specific role on weight gain and intestinal disaccharidase activity, 42 male Sprague-Dawley rats were assigned to one of seven groups (n = 6 each). These were a baseline control group (0) that was killed to analyze initial intestinal disaccharidase (sucrase and maltase) activity, a seco...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199001000-00019
更新日期:1990-01-01 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::A prolonged but transient neonatal jaundice due to unconjugated hyperbilirubinemia was observed in two unrelated babies who were siblings of patients with Crigler--Najjar disease (Arias type I). The bilirubin-UDP-glucuronyl transferase activity measured in the liver of both patients, at the age of 6 and 7 months, resp...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1982-01-01 00:00:00
abstract:OBJECTIVE:P-glycoprotein (P-gp), the functional product of the multidrug resistance gene (MDR), is a transmembrane protein that extrudes substrates from the intracellular environment. P-gp is expressed on the apical surface of epithelial cells and on cells from the hematopoietic lineage. Human MDR polymorphisms have be...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31822860f1
更新日期:2011-12-01 00:00:00
abstract::Since the first description of celiac disease (CeD) by Samuel Gee in 1888 and the later "miraculous discovery" that bread was responsible for this condition following World War II in Europe, there has been an exponential growth of knowledge regarding CeD. Just when we thought that we knew everything there was to know ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001312
更新日期:2017-01-01 00:00:00
abstract::Recent reports show that the incidence of and deaths caused by necrotizing enterocolitis (NEC) in preterm very-low-birth-weight (PVLBW) infants are on the rise. Unfortunately, NEC often rapidly progresses from early signs of intestinal inflammation to extensive necrosis within a matter of hours, making treatment and s...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3182a7dc74
更新日期:2014-01-01 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