Abstract:
BACKGROUND:Cystinosis is a metabolic disease characterized by accumulation of cystine in different organs and tissues, leading to potentially life-threatening organ dysfunction. Infantile cystinosis typically leads to end-stage renal disease, necessitating renal replacement therapy. Liver disease in cystinosis is rare and is mostly reported as nodular regenerative hyperplasia leading to portal hypertension. METHODS:Two patients with infantile cystinosis developed cholestatic liver disease (increasing alkaline phosphatases, gamma-glutamyltransferase and mild increase in transaminases). Severe accumulation of cystine was demonstrated on liver biopsy, predominantly localized in Kupffer cells, together with morphological signs of sclerosing cholangitis on liver biopsy. One patient showed changes compatible with sclerosing cholangitis on magnetic resonance imaging. Therapy with ursodeoxycholic acid led to biochemical improvement in one and stabilization in the other patient. CONCLUSION:Long-term infantile nephropathic cystinosis can be associated with a form of sclerosing cholangitis, which can respond to therapy with ursodeoxycholic acid.
journal_name
J Gastroenterol Hepatoljournal_title
Journal of gastroenterology and hepatologyauthors
Cornelis T,Claes K,Gillard P,Nijs E,Roskams T,Lombaerts R,Nevens F,Cassiman Ddoi
10.1111/j.1440-1746.2008.05312.xsubject
Has Abstractpub_date
2008-08-01 00:00:00pages
e428-31issue
8 Pt 2eissn
0815-9319issn
1440-1746pii
JGH5312journal_volume
23pub_type
杂志文章abstract:BACKGROUND:The purpose of the present study was to investigate microvessel density (MVD), and the expression of CD44 adhesion molecule, p53 protein and c-erbB2 in gallbladder carcinoma, and their relation to histological grade and tumor invasiveness. METHODS:Immunohistochemical staining with antibodies against factor ...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.2004.03357.x
更新日期:2004-07-01 00:00:00
abstract:AIM:To determine whether liver biopsy might be useful in the diagnosis of visceral leishmaniasis when bone marrow examination and serologic tests are inconclusive. METHODS:Over a 10-year period, liver biopsy was performed in five children with suspected visceral leishmaniasis when indirect hemagglutination tests and b...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.2006.04172.x
更新日期:2006-01-01 00:00:00
abstract:BACKGROUND AND AIM:Same-day bidirectional endoscopy (BDE) is a commonly performed procedure, but the optimal sequence for the procedure with moderate conscious sedation is not well established. This study investigated the optimal sequence for same-day BDE under moderate conscious sedation and carbon dioxide insufflatio...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章,随机对照试验
doi:10.1111/jgh.13971
更新日期:2018-03-01 00:00:00
abstract::The relationship between the intragastric pH measured by 24 h pH monitoring system and the serum pepsinogen I/II ratio was studied in 68 cases. When pepsinogen I/II ratio was compared with pH 3.0 holding time (the percentage time during which the gastric pH is above 3.0), there was a negative correlation between these...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.1995.tb01070.x
更新日期:1995-03-01 00:00:00
abstract:BACKGROUND:CYP2C19 polymorphism plays an important role in the metabolism of proton pump inhibitors. The multidrug resistance (MDR)1 genotype is associated with the successful eradication of Helicobacter pylori. The aim of the present study was to investigate the effects of CYP2C19 and MDR1 genotypes on the eradication...
journal_title:Journal of gastroenterology and hepatology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1440-1746.2008.05605.x
更新日期:2009-02-01 00:00:00
abstract::The main goals of chronic hepatitis B treatment should be the long-term suppression of viral replication to minimize disease progression and the risk for the development of hepatocellular carcinoma. Treatment end-points, depending on surrogate markers alone, in particular hepatitis B e-antigen seroconversion, may not ...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.2008.05400.x
更新日期:2008-08-01 00:00:00
abstract:BACKGROUND AND AIM:Secreted protein acidic and rich in cysteine (SPARC) is a matricellular glycoprotein that plays a significant role in tumor development. SPARC has been indicated that promotes tumorigenesis, metastasis, and poor prognosis in prostate cancer and lung cancer. Therefore, we sought to investigate the mol...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.14692
更新日期:2019-10-01 00:00:00
abstract:BACKGROUND AND AIMS:The binucleation of hepatocytes, which was known as an important feature of liver growth and physiology, has been reported to be increased during the chronic oxidative injury stage and has been regarded as an age-related change of hepatic structures. Therefore, we investigated the binuclearity patte...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.2010.06239.x
更新日期:2010-11-01 00:00:00
abstract::Spontaneous regression of hepatocellular carcinoma (HCC) is a rare phenomenon. This case of a 65-year-old Korean man with HCC and metastatic frontal bone mass that regressed after radiotherapy for frontal bone mass without any other therapeutic modalities is described. The clinical diagnosis of HCC was made because of...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.2005.03243.x
更新日期:2005-03-01 00:00:00
abstract::Although the advancement of molecular oncology in gastric cancer lags behind that of colorectal cancer, the rapid developments witnessed in recent years have improved our understanding of the carcinogenesis, aetiology, progression and metastasis of gastric cancer. The different molecular genetic alterations in intesti...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章,评审
doi:10.1046/j.1440-1746.1999.02000.x
更新日期:1999-12-01 00:00:00
abstract:BACKGROUND AND AIM:The high-mobility group box 1 (HMGB1) protein plays an important role in a lot of biological behaviors, including DNA damage repair, gene transcription, cell replication, and cell death, and its expression is higher in many solid tumors tissues than in their adjacent normal tissues, and it is always ...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.14371
更新日期:2019-04-01 00:00:00
abstract::One of the characteristic features of the majority of pancreatic ductal adenocarcinomas is an abundant desmoplastic/stromal reaction. Until recently, this stroma had received little attention from researchers studying the pathogenesis of pancreatic cancer, with most of the research focus resting on the biology of tumo...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章,评审
doi:10.1111/j.1440-1746.2011.07000.x
更新日期:2012-03-01 00:00:00
abstract:BACKGROUND AND AIM:The presence of spur-cell anemia (SCA) is due to lipid disturbances of the erythrocyte membrane and may develop in patients with advanced liver cirrhosis. The accurate predicting value of SC for survival has not been clarified. The aim of this study was to evaluate SCA as a prognostic indicator in pa...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.12473
更新日期:2014-04-01 00:00:00
abstract:BACKGROUND AND AIM:The aim of this study is to evaluate the epidemiology and impact of bacterial infections at admission in patients with acute decompensation (AD) of cirrhosis. METHODS:A cohort with AD of cirrhosis (European Association for the Study of the Liver criteria) admitted at a tertiary center was evaluated ...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.14788
更新日期:2020-03-01 00:00:00
abstract:BACKGROUND AND AIM:Although several molecular biomarkers for esophageal adenocarcinoma (EAC) have been shown to be useful disease indicators, none has been established as a reliable indicator for risk of EAC or have progressed to routine use. The aim was to identify biomarkers of high risk for EAC in patients with Barr...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.13055
更新日期:2016-01-01 00:00:00
abstract::Primary squamous cell carcinoma of the liver is very rare; only 18 cases have been recorded. In the present report we document an additional case who had a unique history of stage III non-keratinizing nasopharyngeal carcinoma treated with a full course of radiotherapy in July 1993. Two small hypoechoic cyst-like lesio...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.1997.tb00413.x
更新日期:1997-03-01 00:00:00
abstract::Mucus and bicarbonate secretions have been widely implicated as an important pre-epithelial protective barrier against autodigestion of the gastric mucosa by acid and pepsin. Evidence from several independent studies shows there is a continuous layer of resilient viscoelastic mucus gel adherent to the surface of the g...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.1994.tb01303.x
更新日期:1994-01-01 00:00:00
abstract:BACKGROUND AND AIM:This study aimed to evaluate the outcomes and toxicities of repeated stereotactic ablative radiotherapy (SABR) in hepatocellular carcinoma (HCC). METHODS:Fourteen HCC patients with local recurrence (18 lesions) after liver SABR received repeated radiotherapy with SABR using CyberKnife. No patients e...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.12659
更新日期:2014-11-01 00:00:00
abstract::The identification of activated hepatic stellate cells and related cell types as key fibrogenic effectors during liver injury has led to intense evaluation of transcriptional events underlying their behavior. While initial studies focused on characterizing interactions between transcription factors and regulatory regi...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章,评审
doi:10.1111/j.1440-1746.2006.04585.x
更新日期:2006-10-01 00:00:00
abstract:BACKGROUND:The aim of this study was to determine whether the amount of Helicobacter pylori and the extent of gastric metaplasia in the duodenal mucosa play critical roles in the pathogenesis of duodenal ulcer. METHODS:Duodenal and gastric biopsy specimens were obtained from H. pylori-positive patients with duodenal u...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1046/j.1440-1746.1999.01824.x
更新日期:1999-02-01 00:00:00
abstract::It was recently reported that mutations in the precore and core promoter region of hepatitis B virus (HBV) are associated with fulminant hepatitis. The aim of this study was to investigate the association of mutations in the precore and core promoter region of HBV with fulminant and severe acute hepatitis. We studied ...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.1998.tb00588.x
更新日期:1998-11-01 00:00:00
abstract:BACKGROUND:Although there is strong implication that retinoids regulate Ito cell proliferation and collagen synthesis, results from in vivo studies on the relationship between vitamin A and liver fibrosis are conflicting. The present study focuses on the role of vitamin A in carbon tetrachloride (CCl4)-induced fibrosis...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1046/j.1440-1746.2002.02804.x
更新日期:2002-07-01 00:00:00
abstract:BACKGROUND AND AIMS:Intestinal endotoxin (lipopolysaccharide) is thought to contribute to liver injury in both alcoholic and nonalcoholic steatohepatitis (NASH). Tumor necrosis factor alpha (TNFalpha) is an important mediator of this process and is considered central to the inflammatory response in NASH. This study aim...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.2005.04220.x
更新日期:2006-01-01 00:00:00
abstract::We evaluated the clinical usefulness of a protein induced by vitamin K absence, antagonist-prothrombin (PIVKA-II), in detecting hepatocellular carcinoma (HCC) specifically in patients with liver cirrhosis, and the possible correlation between levels of PIVKA-II and pathological features of HCC. Plasma levels of PIVKA-...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.1992.tb00925.x
更新日期:1992-01-01 00:00:00
abstract:BACKGROUND AND AIM:This study investigated whether diabetes mellitus (DM) increased the risk of pancreatic cancer and whether anti-diabetic drugs reduced the risk in Taiwan. METHODS:We designed a population-based cohort study using the Taiwan National Health Insurance Database, which consisted of 49,803 patients aged ...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.2011.06938.x
更新日期:2012-04-01 00:00:00
abstract:BACKGROUND AND AIMS:Probucol is a lipid-lowering agent with anti-oxidant effects. Oxidative stress and inflammation are important in the pathophysiology of insulin resistance. We aimed to evaluate the effects of probucol on liver histological changes, serum and hepatic levels of adipokines in rats with high fat-induced...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/j.1440-1746.2008.05719.x
更新日期:2009-06-01 00:00:00
abstract:BACKGROUND AND AIM:Albumin-bilirubin (ALBI) grade was developed as a new method to assess hepatic function. Sorafenib has been confirmed to be effective in improving survival in patients with advanced hepatocellular carcinoma (HCC). In this study, we investigated the impact of ALBI grade versus Child-Pugh classificatio...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.14564
更新日期:2019-06-01 00:00:00
abstract:BACKGROUND AND AIMS:The aim of the present study was to clarify relationship between macroscopic and microscopic features of herpes simplex esophagitis (HSE), and localization of herpes simplex virus type 1 (HSV-1)-infected cells in esophageal lesions from autopsy cases of histopathologically proven HSE. METHODS:The s...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1046/j.1440-1746.2003.03166.x
更新日期:2003-12-01 00:00:00
abstract:BACKGROUND AND AIM:Patients with advanced pancreatic cancer have severe pain, anxiety, and depression, and these symptoms deteriorate quality of life (QOL). Previous study reported that early psychiatric intervention for advanced cancer patients may improve QOL. We evaluated the trajectory of health-related QOL (HRQOL)...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.13172
更新日期:2016-03-01 00:00:00
abstract:BACKGROUND AND AIM:White opaque substance (WOS) is a novel endoscopic finding in gastric neoplasms, indicating the intracellular accumulation of lipid droplets (LDs). However, gastric lipid metabolism has not been extensively investigated, even in normal mucosa. We investigated the expression profiles of lipid-metaboli...
journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.13216
更新日期:2016-04-01 00:00:00