Long-term survival in patients with hereditary hemochromatosis.


BACKGROUND & AIMS:The course of hereditary hemochromatosis may depend on the degree of iron overload and the time of therapeutic intervention. This analysis evaluates the impact of early diagnosis and iron removal on survival and complications in hereditary hemochromatosis. METHODS:A Cohort of 251 patients with hemochromatosis was followed up for 14.1 +/- 6.8 years. RESULTS:Survival was reduced in the total group of patients when compared with a matched normal population. Survival in noncirrhotic and nondiabetic patients and in patients diagnosed between 1982 and 1991 was identical with rates expected. Survival was reduced in patients with severe iron overload vs. those with less severe overload. The percentage of early diagnoses increased threefold between 1947 and 1969 to that between 1970 and 1981; there was only a further 20%-25% increase in the last decade. Deaths caused by liver cancer, cardiomyopathy, liver cirrhosis, and diabetes mellitus were increased as compared with expected rates. Liver cancers were associated with cirrhosis and amount of mobilizable iron but not with hepatitis B or C markers. CONCLUSIONS:Prognosis of hemochromatosis and most of its complications, including liver cancer, depend on the amount and duration of iron excess. Early diagnosis and therapy largely prevent the adverse consequences of iron overload.






Niederau C,Fischer R,Pürschel A,Stremmel W,Häussinger D,Strohmeyer G




Has Abstract


1996-04-01 00:00:00














  • Long Noncoding RNA GMAN, Up-regulated in Gastric Cancer Tissues, Is Associated With Metastasis in Patients and Promotes Translation of Ephrin A1 by Competitively Binding GMAN-AS.

    abstract:BACKGROUND & AIMS:We aimed to identify long noncoding RNAs (lncRNAs) that are up-regulated in gastric cancer tissues from patients and study their function in gastric tumor metastasis. METHODS:We collected gastric tumor and nontumor tissues from patients in China and analyzed levels of lncRNAs by microarray analysis, ...


    pub_type: 杂志文章


    authors: Zhuo W,Liu Y,Li S,Guo D,Sun Q,Jin J,Rao X,Li M,Sun M,Jiang M,Xu Y,Teng L,Jin Y,Si J,Liu W,Kang Y,Zhou T

    更新日期:2019-02-01 00:00:00

  • Calcitonin gene-related peptide mediates capsaicin-induced neuroendocrine responses in rat antrum.

    abstract:BACKGROUND:Calcitonin gene-related peptide (CGRP) is a 37-amino acid peptide localized to primary sensory afferent nerves in the rat stomach. The actions of CGRP in regulating antral neuroendocrine function were examined in vitro through the use of capsaicin, an agent capable of evoking neuropeptide release from periph...


    pub_type: 杂志文章


    authors: Ren J,Young RL,Lassiter DC,Harty RF

    更新日期:1993-02-01 00:00:00

  • Neurokinin 1 and 2 receptors mediate cholera toxin secretion in rat jejunum.

    abstract:BACKGROUND & AIMS:Substance P, a member of the tachykinin family, is a prosecretory neuropeptide distributed widely throughout the enteric nervous system. Implicated in inflammatory states, its role in enterotoxigenic water and electrolyte secretion is unclear. We assessed the effect of substance P antagonists and neur...


    pub_type: 杂志文章


    authors: Turvill JL,Connor P,Farthing MJ

    更新日期:2000-10-01 00:00:00

  • Linaclotide inhibits colonic nociceptors and relieves abdominal pain via guanylate cyclase-C and extracellular cyclic guanosine 3',5'-monophosphate.

    abstract:BACKGROUND & AIMS:Linaclotide is a minimally absorbed agonist of guanylate cyclase-C (GUCY2C or GC-C) that reduces symptoms associated with irritable bowel syndrome with constipation (IBS-C). Little is known about the mechanism by which linaclotide reduces abdominal pain in patients with IBS-C. METHODS:We determined t...


    pub_type: 杂志文章,随机对照试验


    authors: Castro J,Harrington AM,Hughes PA,Martin CM,Ge P,Shea CM,Jin H,Jacobson S,Hannig G,Mann E,Cohen MB,MacDougall JE,Lavins BJ,Kurtz CB,Silos-Santiago I,Johnston JM,Currie MG,Blackshaw LA,Brierley SM

    更新日期:2013-12-01 00:00:00

  • Pathophysiology of Gastroesophageal Reflux Disease.

    abstract::The pathogenesis of gastroesophageal reflux disease (GERD) is complex and involves changes in reflux exposure, epithelial resistance, and visceral sensitivity. The gastric refluxate is a noxious material that injures the esophagus and elicits symptoms. Esophageal exposure to gastric refluxate is the primary determinan...


    pub_type: 杂志文章,评审


    authors: Tack J,Pandolfino JE

    更新日期:2018-01-01 00:00:00

  • Methotrexate Is Not Superior to Placebo for Inducing Steroid-Free Remission, but Induces Steroid-Free Clinical Remission in a Larger Proportion of Patients With Ulcerative Colitis.

    abstract:BACKGROUND & AIMS:Parenteral methotrexate is an effective treatment for patients with Crohn's disease, but has never been adequately evaluated in patients with ulcerative colitis (UC). We conducted a randomized controlled trial to determine its safety and efficacy in patients with steroid-dependent UC. METHODS:We perf...


    pub_type: 杂志文章,多中心研究,随机对照试验


    authors: Carbonnel F,Colombel JF,Filippi J,Katsanos KH,Peyrin-Biroulet L,Allez M,Nachury M,Novacek G,Danese S,Abitbol V,Bossa F,Moreau J,Bommelaer G,Bourreille A,Fumery M,Roblin X,Reinisch W,Bouhnik Y,Brixi H,Seksik P,Mala

    更新日期:2016-02-01 00:00:00

  • Colonoscopic polypectomy in chronic colitis: conservative management after endoscopic resection of dysplastic polyps.

    abstract:BACKGROUND & AIMS:Adenomatous polyps are by definition dysplastic and pathologically indistinguishable from the dysplasia-associated lesion or mass (DALM) described in 1981. Yet, adenomatous polyps in noncolitic colons are usually removed definitively endoscopically, whereas DALMs are regarded as harbingers of colon ca...


    pub_type: 杂志文章


    authors: Rubin PH,Friedman S,Harpaz N,Goldstein E,Weiser J,Schiller J,Waye JD,Present DH

    更新日期:1999-12-01 00:00:00

  • Dasatinib inhibits the development of metastases in a mouse model of pancreatic ductal adenocarcinoma.

    abstract:BACKGROUND & AIMS:Pancreatic ductal adenocarcinoma (PDAC) is a highly invasive and metastatic disease for which conventional treatments are of limited efficacy. A number of agents in development are potential anti-invasive and antimetastatic agents, including the Src kinase inhibitor dasatinib. The aim of this study wa...


    pub_type: 杂志文章


    authors: Morton JP,Karim SA,Graham K,Timpson P,Jamieson N,Athineos D,Doyle B,McKay C,Heung MY,Oien KA,Frame MC,Evans TR,Sansom OJ,Brunton VG

    更新日期:2010-07-01 00:00:00

  • Effect of lactulose on ammonia production in a fecal incubation system.

    abstract::An in vitro fecal incubation system was used to demonstrate how lactulose influences ammonia metabolism in the colon. Lactulose and other fermentable substrates (glucose, mannitol, and sorbitol), pH and organic acid were varied independently so that their different effects could be determined. Fermentable substrate ca...


    pub_type: 杂志文章


    authors: Vince A,Killingley M,Wrong OM

    更新日期:1978-03-01 00:00:00

  • Fecal alpha 1-antitrypsin measurement: an indicator of Crohn's disease activity.

    abstract::Random fecal alpha 1-antitrypsin levels were determined in 34 patients, 24 with Crohn's disease, and 10 "controls" having diarrheal illnesses not associated with intestinal protein loss, in an effort to evaluate its usefulness as a measure of Crohn's disease intestinal activity. In the control group, all alpha 1-antit...


    pub_type: 杂志文章


    authors: Meyers S,Wolke A,Field SP,Feuer EJ,Johnson JW,Janowitz HD

    更新日期:1985-07-01 00:00:00

  • Role of endogenous nitric oxide in the control of canine pancreatic secretion and blood flow.

    abstract:BACKGROUND:Endogenous nitric oxide has been implicated in the control of mesenteric circulation, but its role in the control of pancreatic blood flow and exocrine pancreatic secretion has not been studied. METHODS:Secretory studies were performed on conscious dogs with chronic pancreatic fistulas, and changes in pancr...


    pub_type: 杂志文章


    authors: Konturek SJ,Bilski J,Konturek PK,Cieszkowski M,Pawlik W

    更新日期:1993-03-01 00:00:00

  • Roles of iron and HFE mutations on severity and response to therapy during retreatment of advanced chronic hepatitis C.

    abstract:BACKGROUND & AIMS:Iron overload may cause or contribute to hepatic injury and fibrosis. Mutations in the HFE gene may influence development or progression of chronic liver disease by increasing iron stores or modulating immune responses. The aim of this work was to assess the influence of HFE mutations and serum and he...


    pub_type: 临床试验,杂志文章


    authors: Bonkovsky HL,Naishadham D,Lambrecht RW,Chung RT,Hoefs JC,Nash SR,Rogers TE,Banner BF,Sterling RK,Donovan JA,Fontana RJ,Di Bisceglie AM,Ghany MG,Morishima C,HALT-C Trial Group.

    更新日期:2006-11-01 00:00:00

  • Effects of 2-camphanone on canine portal vein blood flow and rat smooth muscle.

    abstract::2-Camphanone is under clinical evaluation for alleviation of hemorrhoidal bleeding and inflammation. Reduced portal venous blood flow may distend, whereas improved portal venous blood flow may alleviate, hemorrhoidal vein distention. The effects of 2-camphanone on canine portal venous blood flow were investigated usin...


    pub_type: 杂志文章


    authors: Xie JM,Greenberg SS,Longenecker G

    更新日期:1992-02-01 00:00:00

  • Acalculous biliary pain: cholecystectomy alleviates symptoms in patients with abnormal cholescintigraphy.

    abstract::A 45-minute infusion of an octapeptide of cholecystokinin (Kinevac; Squibb Diagnostics, New Brunswick, NJ) was used to measure the gallbladder ejection fraction during cholescintigraphy in 40 normal volunteers. Cholecystokinin cholescintigraphy was shown to be a reproducible test. The maximum mean gallbladder ejection...


    pub_type: 临床试验,杂志文章,随机对照试验


    authors: Yap L,Wycherley AG,Morphett AD,Toouli J

    更新日期:1991-09-01 00:00:00

  • Interferon regulatory factor-2 regulates exocytosis mechanisms mediated by SNAREs in pancreatic acinar cells.

    abstract:BACKGROUND & AIMS:Pancreatic acinar cells are used to study regulated exocytosis. We investigated the role of interferon regulatory factor-2 (IRF2) in exocytosis in pancreatic acinar cells. METHODS:Pancreas tissues from Irf2⁺/⁺, Irf2⁺/⁻), and Irf2⁻/⁻ mice were examined by microscopy, immunohistochemical, and immunoblo...


    pub_type: 杂志文章


    authors: Mashima H,Sato T,Horie Y,Nakagawa Y,Kojima I,Ohteki T,Ohnishi H

    更新日期:2011-09-01 00:00:00

  • The bone morphogenetic protein pathway is inactivated in the majority of sporadic colorectal cancers.

    abstract:BACKGROUND & AIMS:The finding of bone morphogenetic protein (BMP) receptor 1a mutations in juvenile polyposis suggests that BMPs are important in colorectal cancer (CRC). We investigated the BMP pathway in sporadic CRC. METHODS:We investigated BMP receptor (BMPR) expression using immunoblotting and sequenced BMPR2 in ...


    pub_type: 杂志文章


    authors: Kodach LL,Wiercinska E,de Miranda NF,Bleuming SA,Musler AR,Peppelenbosch MP,Dekker E,van den Brink GR,van Noesel CJ,Morreau H,Hommes DW,Ten Dijke P,Offerhaus GJ,Hardwick JC

    更新日期:2008-05-01 00:00:00

  • Ability of human colonic epithelium to express the 4F2 antigen, the common acute lymphoblastic leukemia antigen, and the transferrin receptor. Studies in inflammatory bowel disease and after in vitro exposure to different stimuli.

    abstract::We investigated the ability of isolated human colonic epithelial cells to express the common acute lymphoblastic leukemia antigen (CALLA), the transferrin receptor, and the 4F2 antigen in response to different types of stimuli. The expression of these markers was assessed by immunofluorescence using monoclonal antibod...


    pub_type: 杂志文章


    authors: Fais S,Pallone F

    更新日期:1989-12-01 00:00:00

  • Autophagy, Inflammation, and Immune Dysfunction in the Pathogenesis of Pancreatitis.

    abstract::Pancreatitis is a common disorder with significant morbidity and mortality, yet little is known about its pathogenesis, and there is no specific or effective treatment. Its development involves dysregulated autophagy and unresolved inflammation, demonstrated by studies in genetic and experimental mouse models. Disease...


    pub_type: 杂志文章,评审


    authors: Gukovskaya AS,Gukovsky I,Algül H,Habtezion A

    更新日期:2017-11-01 00:00:00

  • Determinants of diarrhea in viral enteritis. The role of ion transport and epithelial changes in the ileum in transmissible gastroenteritis in piglets.

    abstract::To understand mechanisms of viral diarrhea further, we studied ileal ion transport in vitro in relation to mucosal changes and epithelial differentiation in transmissible gastroenteritis in piglets, an invasive viral enteritis thought to involve mainly proximal intestine. In infected pigs, at the height of diarrhea, s...


    pub_type: 杂志文章


    authors: Shepherd RW,Gall DG,Butler DG,Hamilton JR

    更新日期:1979-01-01 00:00:00

  • Pulmonary hypertension complicating hepatocellular carcinoma.

    abstract::A case of primary liver cell carcinoma is presented in which tumor emboli to the pulmonary microvasculature resulted in pulmonary hypertension, documented by clinical, radiologic, electrocardiographic, and cardiac catheter studies. Emboli arose from tumor invading the portal vein and passed via a patent splenorenal sh...


    pub_type: 杂志文章


    authors: Willett IR,Sutherland RC,O'Rourke MF,Dudley FJ

    更新日期:1984-11-01 00:00:00

  • Liver X receptors inhibit proliferation of human colorectal cancer cells and growth of intestinal tumors in mice.

    abstract:BACKGROUND & AIMS:Liver X receptors (LXRs) are transcriptional regulators of cholesterol metabolism, controlling cholesterol flow into cells, catabolism, and efflux. Cholesterol controls cell proliferation; disruptions in cholesterol metabolism have been associated with the development of colon cancer. We investigated ...


    pub_type: 杂志文章


    authors: Lo Sasso G,Bovenga F,Murzilli S,Salvatore L,Di Tullio G,Martelli N,D'Orazio A,Rainaldi S,Vacca M,Mangia A,Palasciano G,Moschetta A

    更新日期:2013-06-01 00:00:00

  • Characterization and response to interleukin 1 and tumor necrosis factor of immortalized murine biliary epithelial cells.

    abstract:BACKGROUND & AIMS:Biliary epithelial cells are the target of numerous immune-mediated liver diseases, yet their role in pathogenesis remains unclear because of difficulties in obtaining pure preparations. The aim of this study was to establish pure clones of immortalized murine intrahepatic biliary epithelial cells. M...


    pub_type: 杂志文章


    authors: Paradis K,Le ON,Russo P,St-Cyr M,Fournier H,Bu D

    更新日期:1995-10-01 00:00:00

  • Characterization of insulinlike growth factor I receptors in human colon cancer.

    abstract::A possible role of insulinlike growth factor I (IGF-I) and IGF-I receptors in the proliferation of human colon cancer has, to the best of the authors' knowledge, not been examined as yet. To determine a role of IGF-I in colon cancer, several human colon cancer cell lines and colon cancers were screened for specific bi...


    pub_type: 杂志文章


    authors: Guo YS,Narayan S,Yallampalli C,Singh P

    更新日期:1992-04-01 00:00:00

  • Classification of intractable diarrhea in infancy using clinical and immunohistological criteria.

    abstract::Several experimental studies have demonstrated that activated intestinal T cells can induce villous atrophy. This observation led us to examine the possible role of activated T cells in the pathogenesis of intestinal lesions in a group of 13 children with intractable diarrhea and villous atrophy of unknown origin. Imm...


    pub_type: 杂志文章


    authors: Cuenod B,Brousse N,Goulet O,De Potter S,Mougenot JF,Ricour C,Guy-Grand D,Cerf-Bensussan N

    更新日期:1990-10-01 00:00:00

  • Effects of corticotropin-releasing factor on plasma motilin and somatostatin levels and gastrointestinal motility in dogs.

    abstract::The effects of intracerebroventricular and intravenous administration of corticotropin-releasing factor (CRF) on gastrointestinal motility, motilin-induced gastric motor response, and plasma motilin and somatostatin levels were investigated in fasted dogs chronically prepared with strain gauge transducers on the antru...


    pub_type: 杂志文章


    authors: Bueno L,Fargeas MJ,Gue M,Peeters TL,Bormans V,Fioramonti J

    更新日期:1986-10-01 00:00:00

  • Induction of heat-shock protein 72 protects against ischemia/reperfusion in rat small intestine.

    abstract:BACKGROUND & AIMS:Induction of heat-shock protein 72 is associated with enhanced tolerance to subsequent nonthermal stresses. This study evaluated whether induction of heat-shock protein 72 protects against intestinal ischemia/reperfusion injury. METHODS:Groups of nonheated and heated rats underwent sham operation, 30...


    pub_type: 杂志文章


    authors: Stojadinovic A,Kiang J,Smallridge R,Galloway R,Shea-Donohue T

    更新日期:1995-08-01 00:00:00

  • Modification of gastric mucin oligosaccharide expression in rhesus macaques after infection with Helicobacter pylori.

    abstract:BACKGROUND & AIMS:Helicobacter pylori attaches to mucin oligosaccharides that are expressed on host gastric epithelium. We used the rhesus macaque model to characterize the effect of H. pylori infection on gastric mucin oligosaccharides during acute and chronic infection. METHODS:Specific pathogen (H. pylori)-free rhe...


    pub_type: 杂志文章


    authors: Cooke CL,An HJ,Kim J,Canfield DR,Torres J,Lebrilla CB,Solnick JV

    更新日期:2009-09-01 00:00:00

  • Gene targeting demonstrates additive detrimental effects of interleukin 1 and tumor necrosis factor during pancreatitis.

    abstract:BACKGROUND & AIMS:During severe pancreatitis, interleukin (IL)-1beta and tumor necrosis factor (TNF)-alpha are produced in large quantities. The aim of this study was to determine whether either one plays a more dominant role and if their detrimental effects are additive. METHODS:Necrotizing pancreatitis was induced i...


    pub_type: 杂志文章


    authors: Denham W,Yang J,Fink G,Denham D,Carter G,Ward K,Norman J

    更新日期:1997-11-01 00:00:00

  • Coffee and caffeine consumption reduce the risk of elevated serum alanine aminotransferase activity in the United States.

    abstract:BACKGROUND & AIMS:Based on experimental and epidemiologic studies, we investigated whether coffee and caffeine consumption reduced the risk of elevated alanine aminotransferase (ALT) activity in persons at high risk for liver injury in a national, population-based study. METHODS:Participants were 5944 adults in the Th...


    pub_type: 杂志文章


    authors: Ruhl CE,Everhart JE

    更新日期:2005-01-01 00:00:00

  • Nitric oxide production in experimental alcoholic liver disease in the rat: role in protection from injury.

    abstract:BACKGROUND & AIMS:Regulation of blood flow and oxygen supply are important pathogenetic factors in alcoholic liver disease. Because nitric oxide may have an important role, its effects on alcoholic liver injury were investigated. METHODS:Rats were fed ethanol intragastrically with either saturated fat or corn oil. Spo...


    pub_type: 杂志文章


    authors: Nanji AA,Greenberg SS,Tahan SR,Fogt F,Loscalzo J,Sadrzadeh SM,Xie J,Stamler JS

    更新日期:1995-09-01 00:00:00