A case of cholestatic autoimmune hepatitis and acute liver failure: an unusual hepatic manifestation of mixed connective tissue disease and Sjögren's syndrome.

Abstract:

:Although hepatomegaly is reported to occur occasionally in patients with mixed connective tissue disease (MCTD) or Sjögren's syndrome (SS), autoimmune liver diseases such as primary biliary cirrhosis, sclerosing cholangitis, and autoimmune hepatitis in association with MCTD or SS have rarely been described. We report a case of severe cholestatic autoimmune hepatitis presenting with acute liver failure in a 40-yr-old female patient suffering from MCTD and SS. The diagnosis of MCTD and SS was made at the age of 38. The patient presented severe jaundice and elevation of conjugated bilirubin. The patient denied alcohol and drug use and had no evidence of viral hepatitis. On the 8th day of her hospitalization, the patient developed grade III hepatic encephalopathy. She was diagnosed as autoimmune hepatitis presenting with acute liver failure based on clinical features, positive FANA and anti-smooth muscle antibodies, negative anti-mitochondrial antibodies, high titers of serum globulin, liver biopsy findings, and a good response to corticosteroid therapy, The patient was managed with prednisolone and the clinical symptoms, liver function test results, and liver biopsy findings showed much improvement after steroid therapy.

journal_name

J Korean Med Sci

authors

Min JK,Han NI,Kim JA,Lee YS,Cho CS,Kim HY

doi

10.3346/jkms.2001.16.4.512

keywords:

subject

Has Abstract

pub_date

2001-08-01 00:00:00

pages

512-5

issue

4

eissn

1011-8934

issn

1598-6357

pii

200108512

journal_volume

16

pub_type

杂志文章
  • Endothelial nitric oxide gene T-786C polymorphism and subarachnoid hemorrhage in Korean population.

    abstract::We aimed to elucidate whether the eNOS T-786C mutant allele is implicated in subarachnoid hemorrhage (SAH) susceptibility or vasospasm after SAH, and whether the mutant allele is differentially expressed in those with small and large ruptured aneurysms in Korean population. 136 consecutive patients diagnosed with aneu...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2006.21.5.922

    authors: Song MK,Kim MK,Kim TS,Joo SP,Park MS,Kim BC,Cho KH

    更新日期:2006-10-01 00:00:00

  • Association of angiotensin II type 2 receptor gene A1818T polymorphism with progression of immunoglobulin A nephropathy in Korean patients.

    abstract::We determined the relationship between the progression of immunoglobulin A nephropathy (IgAN) and the A1818T polymorphism in intron 2 of Angiotensin II type 2 receptor (AT2R) gene, which might play protective roles in the pathogenesis of IgAN. Patients with biopsy-proven IgAN were recruited from the registry of the Pr...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2009.24.S1.S38

    authors: Yoon HJ,Chin HJ,Na KY,Chae DW,Kim S,Jeon US,Chung WK,Lee HH,Yang J,Kim S,Kwon YJ,Kim HC,Park SB,Kim HY,Lee TW,Progressive REnal disease and Medical Informatics and gEnomics Research (PREMIER) members.

    更新日期:2009-01-01 00:00:00

  • Adjustment of urinary mercury in health risk assessment of mercury.

    abstract::The determination of adjustment method of urinary mercury in spot urine is one of the important issues in assessing the health risks of mercury workers. But there have been debates about whether creatinine or other forms of correction for urinary concentration are better in reducing the variation of urinary mercury. W...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.1996.11.4.319

    authors: Lee E,Park HK,Kim HJ

    更新日期:1996-08-01 00:00:00

  • Estimation of cancer deaths in Korea for the upcoming years.

    abstract::Since the cancer has been the leading cause of deaths in Korea, estimation of the cancer deaths for the upcoming years in the population using the vital statistics is considered to be necessary. The aim of this study was to estimate the number and trends of cancer deaths in Korea. The expected numbers of cancer deaths...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2002.17.5.611

    authors: Bae JM,Jung KW,Won YJ

    更新日期:2002-10-01 00:00:00

  • Primary well differentiated lymphocytic lymphoma of the lung--a clinical and immunohistochemical study of four cases.

    abstract::Four cases of well differentiated lymphocytic lymphoma with or without plasmacytoid differentiation of the lung are described. Two cases were single and the others were multiple. Histologic pictures of the lesion showed mass with perivascular, interstitial and alveolar extension in three cases and only interstitial an...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.1987.2.2.103

    authors: Kim I

    更新日期:1987-06-01 00:00:00

  • Molecular analysis of prion protein gene (PRNP) in Korean patients with Creutzfeldt-Jakob disease.

    abstract::Creutzfeldt-Jakob disease (CJD), a relatively uncommon human dementia, is caused by an unconventional slow infectious agent. Several cases of CJD, clinically or histopathologically diagnosed, have been reported in Korea. In order to confirm the diagnosis of CJD and also differential diagnosis of sporadic and familial ...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.1998.13.3.234

    authors: Jeong BH,Ju WK,Huh K,Lee EA,Choi IS,Im JH,Choi EK,Kim YS

    更新日期:1998-06-01 00:00:00

  • Surgical treatment of primary malignant melanoma of the esophagus: a case report.

    abstract::Primary malignant melanoma of the esophagus (PMME) is an extremely rare tumor with only scattered cases reported. Although surgical resection has been considered as the best possible option, the prognosis has been nonetheless poor. We report a case of PMME which was treated by surgical resection and additionally follo...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2007.22.1.149

    authors: Lee SA,Hwang JJ,Choi YH

    更新日期:2007-02-01 00:00:00

  • Review of the Registration in the Clinical Research Information Service.

    abstract::Clinical research registration is required in many countries to improve transparency of clinical research and to ensure subject safety. Developed in February 2010, the Clinical Research Information Service (CRIS) is an online registration system for clinical studies in Korea and one of the primary registries of the Wo...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章,评审

    doi:10.3346/jkms.2016.31.1.1

    authors: Choi EK,Kim MJ,Lim NK,Park HY

    更新日期:2016-01-01 00:00:00

  • The Association between Parameters of Socioeconomic Status and Hypertension in Korea: the Korean Genome and Epidemiology Study.

    abstract::We investigated the association between socioeconomic status and hypertension in Korea, a country that has experienced a dynamic socioeconomic transition. We analyzed participants of a prospective cohort study-the Korean Genome and Epidemiology Study-enrolled between 2001 and 2003. We recruited 7,089 subjects who unde...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2016.31.12.1922

    authors: Park CS,Ha KH,Kim HC,Park S,Ihm SH,Lee HY

    更新日期:2016-12-01 00:00:00

  • Muscular sarcoidosis detected by F-18 FDG PET/CT in a hypercalcemic patient.

    abstract::Sarcoidosis is a systemic granulomatous disease of unknown etiology that involves many organs, occasionally mimicking malignancy. We herein report a 50-yr-old woman of muscular sarcoidosis of chronic myopathic type, manifested by hypercalcemia and muscle wasting. Besides insignificant hilar lymphadenopathy, her sarcoi...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2013.28.9.1399

    authors: Han EJ,Jang YS,Lee IS,Lee JM,Kang S,Kim HS

    更新日期:2013-09-01 00:00:00

  • Cytomegalovirus ventriculoencephalitis after unrelated double cord blood stem cell transplantation with an alemtuzumab-containing preparative regimen for Philadelphia-positive acute lymphoblastic leukemia.

    abstract::Despite the prophylaxis and preemptive strategies using potent antiviral agents, cytomegalovirus (CMV) remains a major infectious cause of morbidity and mortality in allogeneic stem cell transplantation (SCT) recipients. Delayed immune reconstitution after SCT, such as cord blood and T-cell depleted SCT with the use o...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2010.25.4.630

    authors: Lee S,Kim SH,Choi SM,Lee DG,Kim SY,Lee JW,Min WS,Shin WS,Kim CC

    更新日期:2010-04-01 00:00:00

  • Differential expression of matrix metalloproteinases and tissue inhibitors of metalloproteinases in thioacetamide-induced chronic liver injury.

    abstract::Hepatic fibrogenesis, a complex process that involves a marked accumulation of extracellular matrix components, activation of cells capable of producing matrix materials, cytokine release, and tissue remodeling, is regulated by matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs). The M...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2010.25.4.570

    authors: Park SY,Shin HW,Lee KB,Lee MJ,Jang JJ

    更新日期:2010-04-01 00:00:00

  • Influenza Vaccination Coverage and Its Associated Factors among North Korean Defectors Living in the Republic of Korea.

    abstract::This study aimed to examine influenza vaccination coverage of North Korean defectors (NKD) in the Republic of Korea (Korea) and explore the factors affected the vaccination coverage. Total 378 NKD were analyzed. Four Korean control subjects were randomly matched by age and gender from the Korea National Health and Nut...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2015.30.9.1226

    authors: Song IG,Lee H,Yi J,Kim MS,Park SM

    更新日期:2015-09-01 00:00:00

  • The Cut-off Values of Surrogate Measures for Insulin Sensitivity in a Healthy Population in Korea according to the Korean National Health and Nutrition Examination Survey (KNHANES) 2007-2010.

    abstract:Background:This study aimed to identify the gender-specific characteristics of the surrogate measures of insulin resistance and to establish valid cut-off values for metabolic abnormalities in a representative sample in Korea. Methods:Data were collected from the datasets of the Korean National Health and Nutrition Ex...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2018.33.e197

    authors: Moon S,Park JH,Jang EJ,Park YK,Yu JM,Park JS,Ahn Y,Choi SH,Yoo HJ

    更新日期:2018-06-05 00:00:00

  • Expression of NAD(P)H oxidase subunits and their contribution to cardiovascular damage in aldosterone/salt-induced hypertensive rat.

    abstract::NAD(P)H oxidase plays an important role in hypertension and its complication in aldosterone-salt rat. We questioned whether NAD(P)H oxidase subunit expression and activity are modulated by aldosterone and whether this is associated with target-organ damage. Rats were infused with aldosterone (0.75 microg/hr/day) for 6...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2008.23.6.1039

    authors: Park YM,Lim BH,Touyz RM,Park JB

    更新日期:2008-12-01 00:00:00

  • Diverse molecular targets for therapeutic strategies in Alzheimer's disease.

    abstract::Alzheimer's disease (AD) is the most common form of dementia caused by neurodegenerative process and is tightly related to amyloid β (Aβ) and neurofibrillary tangles. The lack of early diagnostic biomarker and therapeutic remedy hinders the prevention of increasing population of AD patients every year. In spite of acc...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章,评审

    doi:10.3346/jkms.2014.29.7.893

    authors: Han SH,Mook-Jung I

    更新日期:2014-07-01 00:00:00

  • Pretransplant Hepatic Malignancy Increases Risk of De Novo Malignancy after Liver Transplantation.

    abstract:BACKGROUND:Hepatocellular carcinoma (HCC) recurrence and development of de novo malignancy (DNM) after liver transplantation (LT) are the major causes of late recipient death. METHODS:We analyzed the incidence of extrahepatic DNM following living donor LT according to the status of pretransplant hepatic malignancy. We...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2020.35.e69

    authors: Park GC,Hwang S,Ahn CS,Kim KH,Moon DB,Ha TY,Song GW,Jung DH,Yoon YI,Cho HD,Kwon JH,Chung YK,Kang SH,Choi JU,Jung IJ,Lee SG

    更新日期:2020-03-23 00:00:00

  • A case of isolated light chain deposition disease in the duodenum.

    abstract::Light chain deposition disease (LCDD) is a rare disorder associated with a clonal proliferation of plasma cells, which synthesize abnormal monoclonal immunoglobulin light chains. LCDD is characterized by systemic deposition of light chains in various organs, with the kidneys being most commonly affected. There have be...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2012.27.2.207

    authors: Kim HJ,Park E,Lee TJ,Do JH,Cha YJ,Lee SJ

    更新日期:2012-02-01 00:00:00

  • A case of myxedema coma presenting as a brain stem infarct in a 74-year-old Korean woman.

    abstract::Myxedema coma is the extreme form of untreated hypothyroidism. In reality, few patients present comatose with severe myxedema. We describe a patient with myxedema coma which was initially misdiagnosed as a brain stem infarct. She presented to the hospital with alteration of the mental status, generalized edema, hypoth...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2010.25.9.1394

    authors: Ahn JY,Kwon HS,Ahn HC,Sohn YD

    更新日期:2010-09-01 00:00:00

  • Changes of Epidemiological Characteristics of Japanese Encephalitis Viral Infection and Birds as a Potential Viral Transmitter in Korea.

    abstract::Japanese encephalitis (JE) cases have been increasingly reported recently especially in Seoul and its vicinity. Pigs are known as amplifying host of JE virus (JEV), but do not play an important role in these recent events because pig-breeding is not common in Seoul. The distribution and the density of migratory birds ...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2018.33.e70

    authors: Bae W,Kim JH,Kim J,Lee J,Hwang ES

    更新日期:2018-02-26 00:00:00

  • Ultrasonographic Characteristics of the Follicular Variant Papillary Thyroid Cancer According to the Tumor Size.

    abstract::Follicular variant papillary thyroid cancer (FVPTC) is the second most common subtype after conventional PTC. We compared ultrasonographic (US) features of FVPTC to those of conventional PTC according to tumor size. We reviewed US findings, pathologic reports, and medical charts of 249 PTC patients with surgically pro...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2016.31.3.397

    authors: Jeon EJ,Jeong YJ,Park SH,Cho CH,Shon HS,Jung ED

    更新日期:2016-03-01 00:00:00

  • Association between central obesity and circadian parameters of blood pressure from the korean ambulatory blood pressure monitoring registry: Kor-ABP registry.

    abstract::Central obesity has been reported as a risk for atherosclerosis and metabolic syndrome. The influence of central obesity on diurnal blood pressure (BP) has not been established. In this study, we investigated the influence of central obesity on the circadian parameters of BP by 24 hr ambulatory BP monitoring. Total 1,...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2013.28.10.1461

    authors: Kang IS,Pyun WB,Shin J,Kim JH,Kim SG,Shin GJ

    更新日期:2013-10-01 00:00:00

  • Silencing of ABCG2 by MicroRNA-3163 Inhibits Multidrug Resistance in Retinoblastoma Cancer Stem Cells.

    abstract::To investigate the function and regulation mechanism of ATP-binding cassette, subfamily G, member 2 (ABCG2) in retinoblastoma cancer stem cells (RCSCs), a long-term culture of RCSCs from WERI-Rb1 cell line was successfully established based on the high expression level of ABCG2 on the surface of RCSCs. To further expl...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2016.31.6.836

    authors: Jia M,Wei Z,Liu P,Zhao X

    更新日期:2016-06-01 00:00:00

  • Effect of Phacoemulsification on Intraocular Pressure in Healthy Subjects and Glaucoma Patients.

    abstract:Background:We investigated how cataract surgery might influence long-term intraocular pressure (IOP) change in both healthy subjects and glaucoma patients. Methods:A retrospective analysis of patients who had had clear corneal phacoemulsification with a minimum of 12 months of follow up was performed. Glaucoma patient...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2019.34.e47

    authors: Baek SU,Kwon S,Park IW,Suh W

    更新日期:2019-01-30 00:00:00

  • Clinical characteristics and risk factors for nosocomial candidemia in medical intensive care units: experience in a single hospital in Korea for 6.6 years.

    abstract::The aim of this study was to determine candidemia incidence among patients in a medical intensive-care unit (MICU) and the associated mortality rate and to identify risk factors associated with candidemia. We retrospectively performed a 1:3 matched case-control study of MICU patients with candidemia. Controls were mat...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2010.25.5.671

    authors: Han SS,Yim JJ,Yoo CG,Kim YW,Han SK,Shim YS,Lee SM

    更新日期:2010-05-01 00:00:00

  • Development of a rat model of graded contusive spinal cord injury using a pneumatic impact device.

    abstract::An animal model of spinal cord trauma is essential for understanding the injury mechanisms, cord regeneration, and to aid the development of new therapeutic modalities. This study focused on the development of a graded experimental contusion model for spinal cord injury (SCI) using a pneumatic impact device made in Ko...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2004.19.4.574

    authors: Yeo SJ,Hwang SN,Park SW,Kim YB,Min BK,Kwon JT,Suk JS

    更新日期:2004-08-01 00:00:00

  • Telomere length changes in colorectal cancers and polyps.

    abstract::Telomere shortening and telomerase activation occur frequently in cases of colorectal carcinoma. In this study, we correlated the clinicopathological parameters with the telomere length in colorectal carcinomas, colonic polyps, and normal colonic tissues. We also investigated whether the telomere length changes reflec...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2002.17.3.360

    authors: Kim HR,Kim YJ,Kim HJ,Kim SK,Lee JH

    更新日期:2002-06-01 00:00:00

  • X-linked hyper-IgM syndrome associated with Cryptosporidium parvum and Cryptococcus neoformans infections: the first case with molecular diagnosis in Korea.

    abstract::X-linked hyper-IgM syndrome (XHIM) is a rare primary immunodeficiency disorder, caused by mutations of the gene encoding CD40 ligand (CD40L; CD154). We report the clinical manifestations and mutational analysis of the CD40L gene observed in a male patient from a XHIM family. Having hypogammaglobulinemia and elevated I...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2002.17.1.116

    authors: Jo EK,Kim HS,Lee MY,Iseki M,Lee JH,Song CH,Park JK,Hwang TJ,Kook H

    更新日期:2002-02-01 00:00:00

  • A case of santorinicele without pancreas divisum: diagnosis with multi-detector row computed tomography.

    abstract::A santorinicele is defined as a focal cystic dilatation of the terminal portion of the dorsal pancreatic duct at the minor papilla. Most cases reported previously were associated with pancreas divisum and a santorinicele without pancreas divisum is known to be rare. We recently experienced a typical case of a santorin...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2006.21.2.358

    authors: Nam KD,Joo KR,Jang JY,Kim NH,Lee SK,Dong SH,Kim HJ,Kim BH,Chang YW,Lee JI,Chang R

    更新日期:2006-04-01 00:00:00

  • Donor specific response of estrogen and progesterone on cultured human melanocytes.

    abstract::The mechanisms of estrogen and progesterone in human cutaneous pigmentation are largely unknown. The molecular identification of estrogen receptor (ER) and progesterone receptor (PR) in the human melanocytes is of great importance to understand the mechanisms. We performed immunocytochemistry analysis and demonstrated...

    journal_title:Journal of Korean medical science

    pub_type: 杂志文章

    doi:10.3346/jkms.2002.17.1.58

    authors: Im S,Lee ES,Kim W,On W,Kim J,Lee M,Kang WH

    更新日期:2002-02-01 00:00:00