Abstract：
BACKGROUND/AIMS:The primary cause of Familial Amyloidotic Polyneuropathy is a variant transthyretin gene on chromosome 18. Progressive polyneuropathy followed by fatal cardiac and renal failure commonly manifest during middle age. Within 10 years after onset of clinical symptoms, affected individuals usually die due to malnutrition or heart failure. Currently, liver transplantation is the only available therapeutic option. METHODS:We performed liver transplantation in two patients with Familial Amyloidotic Polyneuropathy carrying the transthyretin-30 mutant. Two patients aged more than 50 years received the two explanted amyloidotic livers. This procedure is called Domino liver transplantation. We report the outcome in the studied subjects and analyze the metabolic consequences of this procedure. RESULTS:We determined the serum half-life of transthyretin-30 as 2.25 days using daily monitoring of transthyretin-30 levels. An affected amyloidotic patient had an increased serum concentration of lipoprotein(a) of 78 mg/dl before transplantation. The tumor patient, who received the organ from this affected patient, developed an almost identical serum concentration of lipoprotein(a) after liver transplantation, confirming the liver as the primary site of synthesis of this lipoprotein. CONCLUSION:Once Domino liver transplantation has been performed, the impact of the liver-dependent metabolism of specific proteins of interest can be studied.
journal_name
J Hepatoljournal_title
Journal of hepatologyauthors
Schmidt HH,Nashan B,Pröpsting MJ,Nakazato M,Flemming P,Kubicka S,Böker K,Pichlmayr R,Manns MPdoi
10.1016/s0168-8278(99)80076-2subject
Has Abstractpub_date
1999-02-01 00:00:00pages
293-8issue
2eissn
0168-8278issn
1600-0641pii
S0168-8278(99)80076-2journal_volume
30pub_type
杂志文章abstract：BACKGROUND/AIMS:Herpes simplex virus (HSV) is known to induce fulminant hepatic failure. The aim of this study is to clarify the molecular basis of liver injury in HSV-induced hepatitis. METHODS:Immunocompetent mice were inoculated with HSV or ultraviolet-inactivated HSV (UV-HSV) intravenously. Extent of liver injury ...
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abstract：BACKGROUND/AIMS:In primary biliary cirrhosis (PBC), pathogenesis is influenced by genetic factors that remain poorly elucidated up to now. We investigated the impact of sequence diversity in candidate genes involved in immunity (CTLA-4 and TNFalpha), in bile formation (10 hepatobiliary transporter genes) and in the ada...
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abstract：BACKGROUND/AIMS:To study effects of pharmacologic concentrations of azathioprine and 6-mercaptopurine (6-MP) on rat hepatocytes. METHODS:Hepatocytes cultured on matrigel were incubated with azathioprine or 6-MP; effects of putative protective agents were studied. Viability (LDH leakage), reduced (GSH) and oxidized glu...
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更新日期：2004-03-01 00:00:00
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journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/0168-8278(91)90833-w
更新日期：1991-05-01 00:00:00
abstract：:All routinely available assays to detect hepatitis C virus (HCV) infection are based on the specific reaction of serum antibodies to corresponding viral antigens. The only method to detect viral nucleic acids in serum or liver is the enzymatic amplification of reversely transcribed HCV-RNA by polymerase chain reaction...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/0168-8278(92)90074-y
更新日期：1992-07-01 00:00:00
abstract：BACKGROUND/AIMS:To elucidate the mechanisms of action of interferon (IFN) against hepatitis C virus (HCV), we studied the serum HCV dynamics of free-virions (FV) and immune-complexes (IC) in patients treated with IFN. METHODS:FV and IC were separated by immunoprecipitation using anti-human immunoglobulin and quantifie...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/s0168-8278(03)00472-0
更新日期：2003-12-01 00:00:00
abstract：BACKGROUND/AIMS:The hypothesis that chronic alcohol ingestion potentiates iron-associated liver injury was investigated in the 'carbonyl iron-overload rat model'. METHODS:Newborn male and female Wistar-Furth rats (seven per group) were used to investigate iron-alcohol interaction over a 26-week period. Groups 1 and 2 ...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/0168-8278(95)80222-3
更新日期：1995-06-01 00:00:00
abstract：BACKGROUND & AIMS:The accuracy of creatinine-based estimated GFR (eGFR) in assessing the prevalence of chronic kidney disease (CKD) and associated mortality after liver transplantation (LTx) is unknown. Using measured GFR (mGFR) by iothalamate clearance, we determined the prevalence of the entire spectrum of renal dysf...
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pub_type： 杂志文章
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更新日期：2014-08-01 00:00:00
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pub_type： 杂志文章,评审
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更新日期：1985-01-01 00:00:00
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pub_type： 杂志文章,评审
doi：10.1016/j.jhep.2009.09.012
更新日期：2009-12-01 00:00:00
abstract：BACKGROUND/AIMS:Little is known about genuine roles of androgens and their receptor in hepatocellular carcinoma. METHODS:In the present study, two sublines derived from a human hepatocellular carcinoma cell line KYN-1 were used: KYN-1/SM10 with androgen receptor and KYN-1/SM2 without androgen receptor. RESULTS:The bi...
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pub_type： 杂志文章
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journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/s0168-8278(98)80225-0
更新日期：1998-05-01 00:00:00
abstract：BACKGROUND/AIMS:This study aims at describing the natural history and outcome of small duct primary sclerosing cholangitis (PSC). METHODS:Thirty-two patients with small duct PSC were studied. The average time taken for diagnosis was 69 (1-168) months. The median follow-up time was 63 (1-194) months. RESULTS:All patie...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/s0168-8278(02)00036-3
更新日期：2002-05-01 00:00:00
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pub_type： 杂志文章
doi：10.1016/s0168-8278(02)00269-6
更新日期：2002-11-01 00:00:00
abstract：BACKGROUND/AIMS:Septins are ubiquitous and multifunctional scaffold proteins involved in cytoskeletal organization, exocytosis and other cellular processes. We disclose the quiescent hepatic stellate cells (HSCs)-specific expression of a septin subunit Sept4 in the liver, and explore the significance of the septin syst...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/j.jhep.2008.05.026
更新日期：2008-11-01 00:00:00
abstract：BACKGROUND & AIMS:We used longitudinal data from the ANRS CO13 HEPAVIH cohort study of HIV-HCV co-infected individuals to investigate whether polyphenol rich food intake through coffee and/or daily chocolate consumption could play a role in reducing liver enzymes levels. METHODS:Longitudinal data collection included s...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/j.jhep.2013.08.014
更新日期：2014-01-01 00:00:00
abstract：BACKGROUND/AIMS:Hepatic encephalopathy (HE) is a serious neuropsychiatric complication of chronic liver disease. Brain monoamines have been implicated in the pathogenesis of HE. We examined the relationship between monoamine dysfunction and the degree of portal-systemic shunting (PSS) in rats with varying degrees of PS...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/j.jhep.2004.01.003
更新日期：2004-05-01 00:00:00
abstract：BACKGROUND & AIMS:The pathomechanisms underlying non-alcoholic fatty liver disease (NAFLD) and the involved molecular regulators are incompletely explored. The nuclear factor-kappa B (NF-κB)-cofactor gene B cell leukemia-3 (Bcl-3) plays a critical role in altering the transcriptional capacity of NF-κB - a key inducer o...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/j.jhep.2016.06.026
更新日期：2016-12-01 00:00:00
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journal_title：Journal of hepatology
pub_type： 杂志文章
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更新日期：2018-03-01 00:00:00
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pub_type： 杂志文章
doi：10.1016/j.jhep.2008.10.028
更新日期：2009-03-01 00:00:00
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journal_title：Journal of hepatology
pub_type： 杂志文章
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更新日期：2013-08-01 00:00:00
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journal_title：Journal of hepatology
pub_type： 杂志文章,评审
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更新日期：1996-04-01 00:00:00
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journal_title：Journal of hepatology
pub_type： 杂志文章,meta分析
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更新日期：2010-09-01 00:00:00
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journal_title：Journal of hepatology
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jhep.2010.07.011
更新日期：2011-02-01 00:00:00
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journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/j.jhep.2005.08.026
更新日期：2006-03-01 00:00:00
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journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/s0168-8278(01)00183-0
更新日期：2001-11-01 00:00:00
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journal_title：Journal of hepatology
pub_type： 杂志文章,评审
doi：10.1016/s0168-8278(99)80384-5
更新日期：1999-01-01 00:00:00
abstract：BACKGROUND & AIMS:Niemann-Pick C2 (NPC2) is a lysosomal protein involved in the egress of low-density lipoprotein-derived cholesterol from lysosomes to other intracellular compartments. NPC2 has been detected in several tissues and is also secreted from the liver into bile. We have previously shown that NPC2-deficient ...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/j.jhep.2015.10.002
更新日期：2016-02-01 00:00:00
abstract：BACKGROUND/AIMS:Liver carbohydrate metabolism and blood flow are regulated by hepatic nerves and hormones such as glucagon, insulin or catecholamines. Acute and chronic application of alcohol are known to depress the function of central and peripheral nerves. The extent of inhibition of the autonomic nervous system is ...
journal_title：Journal of hepatology
pub_type： 杂志文章
doi：10.1016/s0168-8278(97)80025-6
更新日期：1997-01-01 00:00:00
abstract：BACKGROUND & AIMS:Hepatitis B virus (HBV) and D virus (HDV) co-infections cause the most severe form of viral hepatitis. HDV induces an innate immune response, but it is unknown how the host cell senses HDV and if this defense affects HDV replication. We aim to characterize interferon (IFN) activation by HDV, identify ...
journal_title：Journal of hepatology
pub_type： 杂志文章
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更新日期：2018-07-01 00:00:00
