Long-term survival in patients with hereditary hemochromatosis.

Abstract:

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.

journal_name

Gastroenterology

journal_title

Gastroenterology

authors

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

doi

10.1053/gast.1996.v110.pm8613000

subject

Has Abstract

pub_date

1996-04-01 00:00:00

pages

1107-19

issue

4

eissn

0016-5085

issn

1528-0012

pii

S0016508596001667

journal_volume

110

pub_type

杂志文章
  • Primary sarcoma of the liver. Report of a case with excellent response to hepatic artery ligation and infusion chemotherapy.

    abstract::A case of primary undifferentiated sarcoma of the liver in a 66-year-old woman is reported. The similarity of the presenting symptoms to those of a hepatic abscess is emphasized. The patient's response to palliative treatment by means of hepatic artery ligation and chemotherapy was excellent. We believe that this is t...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:

    authors: Tanner AR,Bolton PM,Powell LW

    更新日期:1978-01-01 00:00:00

  • Hepatic artery embolization for metastatic endocrine-secreting tumors of the pancreas. Report of two cases.

    abstract::Two patients with hormone-secreting metastatic tumors of the liver (carcinoid, islet cell) were treated with transcatheter arterial embolization. In both cases, occlusion of blood supply to the tumors was accomplished, resulting in cessation of the systemic symptoms. Arterial embolization provides another useful palli...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:

    authors: Clouse ME,Lee RG,Duszlak EJ,Lokich JJ,Alday MT

    更新日期:1983-11-01 00:00:00

  • The effect of continuous enteral nutrition on gastric acidity in humans.

    abstract::The effect of continuous intraduodenal enteral nutrition on gastric pH was compared with the effects of fasting and of parenteral and standard nutrition control regimens containing equal amounts of carbohydrate, protein, and lipid. Eleven healthy volunteers underwent four 24-hour intragastric pH-metry studies; serum g...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/0016-5085(92)91708-c

    authors: Armstrong D,Castiglione F,Emde C,Cilluffo T,Duroux P,Koerfer J,Temler E,Lamers CB,Jansens JB,Blum AL

    更新日期:1992-05-01 00:00:00

  • Stimulation of gastric inhibitory polypeptide in normal and duodenal ulcer patients.

    abstract::To investigate the role of gastric inhibitory polypeptide (GIP), a potent inhibitor of gastric acid secretion, in the hypersecretion associated with duodenal ulcer, we compared the serum GIP concentrations in 11 healthy subjects and 16 duodenal ulcer patients after the stimulation of GIP release by a mixed meal. Fasti...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:

    authors: Cataland S,O'Dorisio TM,Brooks R,Mekhjian HS

    更新日期:1977-07-01 00:00:00

  • Loss of Raf kinase inhibitor protein promotes cell proliferation and migration of human hepatoma cells.

    abstract:BACKGROUND & AIMS:The Raf kinase inhibitor protein (RKIP) has been identified as a suppressor of the mitogen-activated protein kinase (MAPK) pathway. Loss of RKIP function promotes tumor metastasis in prostate cancer and melanoma. The insulin-like growth factor I (IGF-I)-mediated MAPK cascade is often activated in hepa...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1053/j.gastro.2006.07.012

    authors: Lee HC,Tian B,Sedivy JM,Wands JR,Kim M

    更新日期:2006-10-01 00:00:00

  • Etiological spectrum of post-transfusion hepatitis.

    abstract::Frequent occurrence of post-transfusion hepatitis continues despite screening for Australia antigen in blood before transfusion and elimination of commercial donor sources. The majority of these cases appears unrelated to hepatitis B virus. Preoperative, acute, and convalescent serra were screened for evidence of hepa...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:

    authors: Knodell RG,Conrad ME,Dienstag JL,Bell CJ

    更新日期:1975-12-01 00:00:00

  • The genetically programmed down-regulation of lactase in children.

    abstract:BACKGROUND & AIMS:Intestinal lactase activity is high in all healthy human babies, but in adults a genetic polymorphism, which acts in cis to the lactase gene, determines high or low messenger RNA (mRNA) expression and activity (lactase persistence and nonpersistence, respectively). Our aim was to investigate the onset...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/s0016-5085(98)70429-9

    authors: Wang Y,Harvey CB,Hollox EJ,Phillips AD,Poulter M,Clay P,Walker-Smith JA,Swallow DM

    更新日期:1998-06-01 00:00:00

  • Human intestinal motor activity and transport: effects of a synthetic opiate.

    abstract::Effects of opiates on intestinal motor activity and transport of water and electrolytes have been studied separately in previous investigations. The aim of these experiments was to evaluate simultaneously the effects of a synthetic opiate, loperamide, on motor activity and transport in the human intestine. Jejunal, il...

    journal_title:Gastroenterology

    pub_type: 临床试验,杂志文章

    doi:10.1016/0016-5085(86)90079-x

    authors: Kachel G,Ruppin H,Hagel J,Barina W,Meinhardt M,Domschke W

    更新日期:1986-01-01 00:00:00

  • Gastrointestinal food hypersensitivity: basic mechanisms of pathophysiology.

    abstract::Gastrointestinal symptoms occur in a large number of patients with food allergies. Immediate hypersensitivity mechanisms may give rise to the nausea, vomiting, abdominal pain, and diarrhea experienced by these patients. However, there are limited human data about the pathophysiological basis for these symptoms. Most o...

    journal_title:Gastroenterology

    pub_type: 杂志文章,评审

    doi:10.1016/0016-5085(92)90047-3

    authors: Crowe SE,Perdue MH

    更新日期:1992-09-01 00:00:00

  • Neurohumoral pathways mediating stress-induced changes in rat gastrointestinal transit.

    abstract::Pharmacologic and surgical procedures were used to define the neurohumoral pathways that mediate stress-induced changes in gastrointestinal transit in the rat. Physical restraint (a validated stressor) resulted in significant inhibition of gastric emptying and small bowel transit but in stimulation of large bowel tran...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/0016-5085(89)91439-x

    authors: Lenz HJ

    更新日期:1989-07-01 00:00:00

  • An update on the epidemiology, molecular characterization, diagnosis, and screening strategies for early-onset colorectal cancer.

    abstract::Rising trends in the incidence and mortality of early-onset CRC in those who are ages less than 50 years have been well-established. These trends have spurred intense investigation focused on elucidating the epidemiology and characteristics of early-onset CRC, as well on identifying strategies for early detection and ...

    journal_title:Gastroenterology

    pub_type: 杂志文章,评审

    doi:10.1053/j.gastro.2020.12.068

    authors: Burnett-Hartman AN,Lee JK,Demb J,Gupta S

    更新日期:2021-01-05 00:00:00

  • Enteropathogenic Escherichia coli infection inhibits intestinal serotonin transporter function and expression.

    abstract:BACKGROUND & AIMS:Serotonin transporter (SERT) plays a critical role in regulating serotonin (5-hydroxytryptamine [5-HT]) availability in the gut. Elevated 5-HT levels are associated with diarrheal conditions such as irritable bowel syndrome and enteric infections. Whether alteration in SERT activity contributes to the...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1053/j.gastro.2009.09.002

    authors: Esmaili A,Nazir SF,Borthakur A,Yu D,Turner JR,Saksena S,Singla A,Hecht GA,Alrefai WA,Gill RK

    更新日期:2009-12-01 00:00:00

  • Infliximab maintenance treatment reduces hospitalizations, surgeries, and procedures in fistulizing Crohn's disease.

    abstract:BACKGROUND & AIMS:Infliximab is effective in closing fistulas in patients with Crohn's disease. We examined the effect of infliximab maintenance treatment on hospitalizations, surgeries, and procedures in patients with fistulizing Crohn's disease enrolled in the ACCENT II study. METHODS:After 5 mg/kg infliximab at wee...

    journal_title:Gastroenterology

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验

    doi:10.1053/j.gastro.2005.01.048

    authors: Lichtenstein GR,Yan S,Bala M,Blank M,Sands BE

    更新日期:2005-04-01 00:00:00

  • Right and left colonic transit after eating assessed by a dual isotopic technique in healthy humans.

    abstract::Propulsion of colonic contents after eating in the whole colon was studied in 15 volunteers by scintigraphy with injection of 111In-diethylenetriamine-pentaacetic acid and 99mTc-sulfur colloid into the colon through a nasogastric tube. The radionuclide was injected into the cecoascending colon (n = 7), the hepatic fle...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/0016-5085(92)91098-o

    authors: Picon L,Lémann M,Flourié B,Rambaud JC,Rain JD,Jian R

    更新日期:1992-07-01 00:00:00

  • Heterotopic liver transplantation for fulminant Wilson's disease.

    abstract::Wilson's disease may present with severe acute hepatocellular failure. The only effective treatment for fulminant Wilson's disease is liver transplantation, which may lead to reversal of the underlying disease. Some patients with cirrhosis who are too ill to undergo orthotopic liver transplantation have been treated w...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/0016-5085(90)90497-o

    authors: Stampfl DA,Muñoz SJ,Moritz MJ,Rubin R,Armenti VT,Jarrell BE,Maddrey WC

    更新日期:1990-12-01 00:00:00

  • Isolation and morphologic characterization of bile duct epithelial cells from normal rat liver.

    abstract::To study directly the functions of the cells that line the bile ducts inside the liver, we developed a new technique for isolating intrahepatic bile duct epithelial cells (IBDECs) from normal rat liver. Parenchymal and nonparenchymal cells were separated from whole liver by enzymatic digestion and mechanical disruptio...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/0016-5085(89)91695-8

    authors: Ishii M,Vroman B,LaRusso NF

    更新日期:1989-11-01 00:00:00

  • Incidence and recurrence rates of colorectal adenomas: a prospective study.

    abstract:BACKGROUND/AIMS:The frequency of colorectal adenomas, the precursor lesions for most cases of colorectal carcinoma, has been generally measured as prevalence rates of adenomas at autopsy or colonoscopy. The aim of this study was to estimate the incidence rate of adenomas and compare it with the adenoma recurrence rate....

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/0016-5085(95)90066-7

    authors: Neugut AI,Jacobson JS,Ahsan H,Santos J,Garbowski GC,Forde KA,Treat MR,Waye J

    更新日期:1995-02-01 00:00:00

  • Steady-state extrarenal sorbitol clearance as a measure of hepatic plasma flow.

    abstract::Hepatic plasma flow was assessed with sorbitol (hepatic extraction = 0.96) at steady state. After infusion of 50 mg/min for 3 h, total and renal sorbitol clearances were calculated, and the extrarenal clearance was obtained by taking the difference between the two. In normal volunteers, the mean (+/- SD) extrarenal so...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/s0016-5085(88)80024-6

    authors: Zeeh J,Lange H,Bosch J,Pohl S,Loesgen H,Eggers R,Navasa M,Chesta J,Bircher J

    更新日期:1988-09-01 00:00:00

  • Normalization of plasma vitamin B12 concentration by intranasal hydroxocobalamin in vitamin B12-deficient patients.

    abstract:BACKGROUND & AIMS:Patients with previous stomach and terminal ileum resections are often treated with intramuscular vitamin B12 injections. Disadvantages are, on a worldwide scale, the frequent need for medical personnel to administer injections and the sometimes painful way of application. This study was designed to i...

    journal_title:Gastroenterology

    pub_type: 临床试验,杂志文章

    doi:10.1053/gast.1997.v113.pm9247460

    authors: Slot WB,Merkus FW,Van Deventer SJ,Tytgat GN

    更新日期:1997-08-01 00:00:00

  • Precore mutations and core clustering mutations in chronic hepatitis B virus infection.

    abstract:BACKGROUND:Mutant hepatitis B virus is often associated with severe liver damage. The purpose of this study is to elucidate the relationship between mutations in hepatitis B precore/core gene and the severity of liver damage. METHODS:The hepatitis B precore/core gene from 20 patients with chronic hepatitis B virus inf...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/0016-5085(93)90861-6

    authors: Chuang WL,Omata M,Ehata T,Yokosuka O,Ito Y,Imazeki F,Lu SN,Chang WY,Ohto M

    更新日期:1993-01-01 00:00:00

  • Occurrence of specific secretory immunoglobulin A in bile after inoculation of Giardia lamblia trophozoites into rat duodenum.

    abstract::We studied the appearance of specific secretory immunoglobulin A (IgA) antibody in bile after inoculation of live Giardia lamblia trophozoites into rat intestine. Serial bile specimens collected before and after inoculation of trophozoites were assayed for IgA antibodies by indirect immunofluorescence. Secretory IgA a...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:

    authors: Loftness TJ,Erlandsen SL,Wilson ID,Meyer EA

    更新日期:1984-11-01 00:00:00

  • Granulocyte colony-stimulating factor mobilizes CD34(+) cells and improves survival of patients with acute-on-chronic liver failure.

    abstract:BACKGROUND & AIMS:Acute-on-chronic liver failure (ACLF) develops in patients with chronic liver disease and has high mortality. Mobilization of bone marrow-derived stem cells with granulocyte colony-stimulating factor (G-CSF) could promote hepatic regeneration. METHODS:Consecutive patients with ACLF were randomly assi...

    journal_title:Gastroenterology

    pub_type: 杂志文章,随机对照试验

    doi:10.1053/j.gastro.2011.11.027

    authors: Garg V,Garg H,Khan A,Trehanpati N,Kumar A,Sharma BC,Sakhuja P,Sarin SK

    更新日期:2012-03-01 00:00:00

  • Seasonal variation in flares of inflammatory bowel disease.

    abstract:BACKGROUND & AIMS:Previous research has yielded conflicting data as to whether the natural history of inflammatory bowel disease follows a seasonal pattern. The purpose of this study was to determine whether relapse of inflammatory bowel disease follows a seasonal pattern either across a cohort of patients or within in...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1053/j.gastro.2003.12.003

    authors: Lewis JD,Aberra FN,Lichtenstein GR,Bilker WB,Brensinger C,Strom BL

    更新日期:2004-03-01 00:00:00

  • Chromosomal alterations in ulcerative colitis-related neoplastic progression.

    abstract:BACKGROUND & AIMS:It is unclear whether genomic derangement precedes the histological development of dysplasia in ulcerative colitis (UC)-related neoplastic progression. The primary aim of this study was to determine if chromosomal alterations occur early in the progression pathway of UC-related neoplasia. METHODS:Flu...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/s0016-5085(97)70173-2

    authors: Willenbucher RF,Zelman SJ,Ferrell LD,Moore DH 2nd,Waldman FM

    更新日期:1997-09-01 00:00:00

  • CD4+ T-cell population mediates development of inflammatory bowel disease in T-cell receptor alpha chain-deficient mice.

    abstract:BACKGROUND & AIMS:Increase of T cells expressing CD4 and T-cell receptor (TCR) alpha- beta+ (beta[dim]) was observed in the mucosal and peripheral lymphoid tissues of TCR alpha-/- mice with inflammatory bowel disease (IBD). The aim of this study was to characterize the CD4+ TCR alpha-beta+ T cells. METHODS:Cytokine pr...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1053/gast.1997.v112.pm9178680

    authors: Takahashi I,Kiyono H,Hamada S

    更新日期:1997-06-01 00:00:00

  • Entecavir therapy for up to 96 weeks in patients with HBeAg-positive chronic hepatitis B.

    abstract:BACKGROUND & AIMS:Entecavir demonstrated superior benefit to lamivudine at 48 weeks in nucleoside-naive patients with hepatitis B e antigen (HBeAg)-positive chronic hepatitis B (CHB). We evaluated continued entecavir and lamivudine treatment through 96 weeks. METHODS:709 HBeAg-positive CHB patients were randomized to ...

    journal_title:Gastroenterology

    pub_type: 杂志文章,随机对照试验

    doi:10.1053/j.gastro.2007.08.025

    authors: Gish RG,Lok AS,Chang TT,de Man RA,Gadano A,Sollano J,Han KH,Chao YC,Lee SD,Harris M,Yang J,Colonno R,Brett-Smith H

    更新日期:2007-11-01 00:00:00

  • Des-acyl ghrelin acts by CRF type 2 receptors to disrupt fasted stomach motility in conscious rats.

    abstract:BACKGROUND & AIMS:Although it has been shown that des-acyl ghrelin decreases food intake and gastric emptying, no previous studies have examined the effects of des-acyl ghrelin on physiologic fed and fasted motor activity in the gastrointestinal tract. METHODS:We examined the effects of intraperitoneal (IP) administra...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1053/j.gastro.2005.04.015

    authors: Chen CY,Inui A,Asakawa A,Fujino K,Kato I,Chen CC,Ueno N,Fujimiya M

    更新日期:2005-07-01 00:00:00

  • Role of inducible nitric oxide synthase in postoperative intestinal smooth muscle dysfunction in rodents.

    abstract:BACKGROUND & AIMS:We have shown that intestinal manipulation leads to a significant inhibition of circular muscle contraction. We hypothesized that the inflammatory mediator inducible nitric oxide (NO) plays a role in surgically induced ileus. METHODS:Rats and inducible NO synthase (iNOS) knockout and wild-type mice u...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1016/s0016-5085(00)70214-9

    authors: Kalff JC,Schraut WH,Billiar TR,Simmons RL,Bauer AJ

    更新日期:2000-02-01 00:00:00

  • Effects of caloric and noncaloric materials in fasting hyperbilirubinemia.

    abstract::Fasting hyperbilirubinemia (FHB) has previously been shown to be rapidly reversed by the ingestion of a mixed diet. This study examines the effect of carbohydrate, fat, amino acids, and noncaloric materials on FHB. After an initial fast of 15 to 39 hr, caloric and noncaloric materials were administered by mouth or vei...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:

    authors: Barrett PV

    更新日期:1975-02-01 00:00:00

  • Sex and acquired cofactors determine phenotypes of ferroportin disease.

    abstract:BACKGROUND & AIMS:Ferroportin disease is characterized by iron overload. It has an autosomal-dominant pattern of inheritance and has been associated with mutations in the SLC40A1 gene, which encodes the cellular iron exporter ferroportin. Since the first description in 2001, about 30 mutations have been reported; the h...

    journal_title:Gastroenterology

    pub_type: 杂志文章

    doi:10.1053/j.gastro.2010.12.049

    authors: Le Lan C,Mosser A,Ropert M,Detivaud L,Loustaud-Ratti V,Vital-Durand D,Roget L,Bardou-Jacquet E,Turlin B,David V,Loréal O,Deugnier Y,Brissot P,Jouanolle AM

    更新日期:2011-04-01 00:00:00