Abstract:
OBJECTIVES:The aims of the study were to perform a retrospective observational review of the present management and outcome of cholestatic pruritus in children with Alagille syndrome (AGS) at King's College Hospital and to use results to inform appropriate guidelines. METHODS:A retrospective review of 62 patients diagnosed as having AGS from January 1995 to November 2010 treated at King's College Hospital was performed. The departmental database of the Paediatric Liver Centre was searched to identify all patients and the clinical records were then analysed. RESULTS:Fifty-one (82.3%) patients experienced pruritus and 50 (80.6%) received antipruritic medication. Ursodeoxycholic acid was the most prescribed drug (n = 40). Other drugs prescribed were rifampicin (n = 39), cholestyramine (n = 18), naltrexone (n = 14), alimemazine (n = 13), nonsedating antihistamine agents (n = 7), ondansetron (n = 5), and phenobarbitone (n = 1). Albumin dialysis using the molecular adsorbent recirculation system was used in 1 patient. Sixteen patients (25.8%) were listed for liver transplantation, and 11 had undergone transplantation by November 2010. Patient survival was high at 95.2%. Pruritus resolved permanently in 39.2% (n = 20) of patients. Fifty-five percent (n = 11) of such patients had undergone liver transplantation. Pruritus was controlled by medication in 41.2% (n = 21). Itching remained a significant problem, affecting quality of life in 19.6% of patients (n = 10). CONCLUSIONS:The management of cholestatic pruritus in AGS is difficult and often suboptimal. Pruritus may remain intractable even with combination medical treatment, and at this stage, surgery or liver transplantation is indicated. At our centre, pruritus was successfully treated in 80.4% of patients with medical and surgical management.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Kronsten V,Fitzpatrick E,Baker Adoi
10.1097/MPG.0b013e318297e384subject
Has Abstractpub_date
2013-08-01 00:00:00pages
149-54issue
2eissn
0277-2116issn
1536-4801journal_volume
57pub_type
杂志文章abstract::The effect of protein-calorie malnutrition during gestation on the brain amino acids of rat pups was studied following nutritional recovery during lactation. The brain amino acids of rat pups born to dam rats malnourished during gestation were studied after these rat pups received proper nutrition during lactation. Pr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198411000-00027
更新日期:1984-11-01 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: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::A 17-year-old male with Crohn's disease presented with persistent fever and right upper quadrant tenderness. Ultrasonography and abdominal computerized axial tomography (CAT scan) showed two large loculated abscess cavities in the right lobe of the liver. Surgical drainage and antibiotic therapy led to resolution of b...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198302010-00021
更新日期:1983-01-01 00:00:00
abstract::We studied the effects of breast milk feeding versus formula feeding during the first 8 weeks of life on the development of local gastrointestinal humoral immune response by measuring fecal secretory immunoglobulin A (SIgA). Forty-four infants were studied and classified into two groups: breast milk (n = 21) and stand...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1989-07-01 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::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:OBJECTIVES:This study was performed to compare the effects of oral midazolam and oral diazepam, administered with intravenous (IV) meperidine, on pre-procedural, procedural, and post-procedural sedation and recovery in children undergoing diagnostic upper endoscopy. The costs of pre-procedure sedation were compared for...
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:10.1097/00005176-200207000-00012
更新日期:2002-07-01 00:00:00
abstract::The general goals of treatment of cyclic vomiting syndrome (CVS) are: interruption of established episodes, amelioration of symptoms in patients whose episodes cannot be interrupted, aborting episodes during prodromal symptoms, prophylaxis to abolish or lessen the frequency of episodes, and recovery. Complications of ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/00005176-199501001-00015
更新日期:1995-01-01 00:00:00
abstract:OBJECTIVES:Dexlansoprazole MR 30 mg once daily (QD) is approved in adults for the treatment of symptomatic nonerosive gastroesophageal reflux disease (GERD) and maintenance of healed erosive esophagitis (EE); 60 mg is approved for healing EE. The present study assesses the pharmacokinetic (PK) profile and safety of d...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1097/MPG.0b013e31822a323a
更新日期:2012-01-01 00:00:00
abstract:OBJECTIVE:Capsule endoscopy (CE) is a noninvasive diagnostic tool for the digestive tract. We aim to investigate the feasibility and safety of newly developed magnetically controlled capsule endoscopy (MCE) in children. METHODS:A total of 129 children who underwent MCE in Shanghai Children's Hospital were retrospectiv...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002292
更新日期:2019-07-01 00:00:00
abstract:BACKGROUND:It has been shown that gum arabic, a soluble fiber, enhances water, electrolyte, and glucose absorption from oral rehydration solutions in jejunal perfusion of healthy rats and in animals with theophylline-induced secretion or chronic osmotic-secretory diarrhea. This report concerns a study of the effectiven...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199910000-00008
更新日期:1999-10-01 00:00:00
abstract::Three distinct forms of familial intrahepatic cholestasis are the result of mutations in the ATP8B1, ABCB11, and ABCB4 genes. The pathophysiologies of the latter 2 of these diseases are well characterized and are the result of abnormalities in canalicular excretion of bile acids and phospholipids, respectively. The mo...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3181596060
更新日期:2008-03-01 00:00:00
abstract:OBJECTIVES:To assess and compare the long-term therapeutic response to lamivudine compared with interferon-alpha (IFN-alpha) in children with chronic hepatitis B. METHODS:A total of 40 children (27 male; age, 1.3-18 y, mean, 7.7 y) with chronic hepatitis B who received lamivudine for at least 12 months were followed f...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000243439.47334.4e
更新日期:2007-01-01 00:00:00
abstract:OBJECTIVES:The aim of the study was to assess health-related quality of life (HRQOL) using the Coeliac Disease Dutch Questionnaire (CDDUX) in Spanish children with coeliac disease. METHODS:The CDDUX was cross-culturally adapted according to international consensus guidelines. HRQOL was assessed in coeliac members of t...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000963
更新日期:2016-04-01 00:00:00
abstract:OBJECTIVES:Questionnaires exist to assess inflammatory bowel disease (IBD)-related knowledge of adults. Owing to wording and content concerns, these were believed to be inappropriate for use in pediatric patients. The aim of this study was to develop a questionnaire to assess disease-related knowledge of pediatric pati...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000210
更新日期:2014-03-01 00:00:00
abstract:OBJECTIVES:Duodenal tube test (DTT) is used as a preoperative screening to rule out biliary atresia (BA). In previous reports, DTT was assessed by the color of the duodenal fluid, but there were no quantitative criteria. The aim of this study was to examine the efficacy of DTT based on the total bile acid (TBA) concent...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002037
更新日期:2018-09-01 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::Ulcerative colitis and Crohn disease may present before the age of 20 years in 25% to 30% of all patients with inflammatory bowel disease. Reported incidence figures vary considerably depending on the collection of data. Multicenter, multinational collaboration is needed when studying pediatric inflammatory bowel dise...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 指南,杂志文章,实务指引
doi:10.1097/01.mpg.0000163736.30261.82
更新日期:2005-07-01 00:00:00
abstract::We compared the in vivo transport of zinc in segments of jejunum, ileum, and colon of 2-week-old (suckling) and 6-week-old (adolescent) rats. In normal rats, net absorption and lumen to mucosa flux of zinc, normalized for differences in size of the intestine (mumol/h/g dry weight) were significantly greater in all per...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198201010-00020
更新日期:1982-01-01 00:00:00
abstract::To explore the relationship between vitamin A deficiency and persistent diarrhea among young children, we studied the vitamin A status of 23 children greater than 5 years of age with persistent diarrhea by performing conjunctival impression cytology (CIC) and the relative dose-response test (RDR) as a measure of liver...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199108000-00009
更新日期:1991-08-01 00:00:00
abstract::We investigated the mechanisms of gastroesophageal reflux (GER) and esophageal motility during endogenous esophageal acid exposure in 17 patients with reflux disease alone (age range 3-20 months) (group A) and in 10 patients with reflux disease complicated by esophagitis (age range 4-19 months) (group B), by simultane...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198811000-00006
更新日期:1988-11-01 00:00:00
abstract:OBJECTIVES:The geometric mean is the recommended method for calculating gastric emptying in adults to correct for variable attenuation during the study. In children, it has been reported that a single posterior acquisition is sufficient. Our objective was to evaluate the relation between the values of gastric emptying ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31824ee2fa
更新日期:2012-08-01 00:00:00
abstract::Pediatric gastrointestinal endoscopy has been established as safe and effective for diagnosis and management of many pediatric gastrointestinal diseases. Nevertheless, certain patient and procedure factors should be recognized that increase the risk of intra- and/or postprocedural adverse events (AEs). AEs associated ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,实务指引
doi:10.1097/MPG.0000000000002277
更新日期:2019-04-01 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:OBJECTIVES:Oxidative stress has been implicated in the development of nonalcoholic fatty liver disease (NAFLD) and progression to the more severe form, nonalcoholic steatohepatitis (NASH), in children. We aimed to study the clinical correlation between bilirubin, a potent endogenous antioxidant with cytoprotective prop...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318291fefe
更新日期:2013-07-01 00:00:00
abstract::Mucins are complex glycoproteins that cover the intestinal mucosal surface and are thought to provide protection against enteropathogenic infections by preventing bacterial adherence and subsequent colonization, invasion and toxin delivery. The aims of this study was to evaluate the capacity of intestinal mucin to inh...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199510000-00004
更新日期:1995-10-01 00:00:00
abstract:BACKGROUND:Results from the 13C mixed triglyceride (MTG) breath test correlate with duodenal lipase activity in adults. This noninvasive test is a potential screening and diagnostic tool for children with fat malabsorption. The aim of this study was to adapt the methodology of the MTG breath test to study test meals an...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200105000-00017
更新日期:2001-05-01 00:00:00
abstract::The system of institutional review boards (IRBs) was established about 30 years ago for the protection of human subjects and as a mechanism for assuring an independent peer review of research activities. To keep pace with an evolving environment, a new model for human research oversight is needed that focuses less on ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200311001-00002
更新日期:2003-11-01 00:00:00
abstract:OBJECTIVES:Familial intrahepatic cholestasis 1 (FIC1) deficiency is caused by a mutation in the ATP8B1 gene. Partial external biliary diversion (PEBD) is pursued to improve pruritus and arrest disease progression. Our aim is to describe clinical variability after PEBD in FIC1 disease. METHODS:We performed a single-cen...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001493
更新日期:2017-03-01 00:00:00