Abstract:
:In the absence of hypertension, hypertrophic cardiomyopathy is the most common cause of left ventricular hypertrophy (LVH). However, it has been reported that up to 3% of males with unexplained LVH have Fabry disease, an X-linked disorder of glycophospholipid metabolism that is due to a deficiency in the lysosomal enzyme alpha-galactosidase A (alpha-Gal A). A 44-year-old man was admitted to our hospital with palpitations. He had a history of chronic renal failure diagnosed at age 33 followed by kidney transplantation performed at our institution 2 years later, as well as long-standing hypohidrosis. His medications included prednisolone (5 mg daily), mycophenolate mofetil (1,000 mg, bid), and cyclosporine (150 mg, bid). On hospital day two, an echocardiogram demonstrated increased left ventricular wall thickness (septal wall thickness of 28 mm, posterior wall thickness of 20 mm). Diastolic dysfunction was noted on transmitral flow patterns and tissue Doppler imaging. The patient was found to have low plasma alpha-Gal A activity. A previously reported H46R missense mutation was detected in his alpha-Gal A gene and the patient was subsequently diagnosed with Fabry disease.
journal_name
Korean Circ Jjournal_title
Korean circulation journalauthors
Nah JC,Kim WS,Cho WH,Choi SK,Yoo HW,Takenaka T,Tei Cdoi
10.4070/kcj.2009.39.8.335subject
Has Abstractpub_date
2009-08-01 00:00:00pages
335-9issue
8eissn
1738-5520issn
1738-5555journal_volume
39pub_type
杂志文章abstract:BACKGROUND AND OBJECTIVES:Impaired recovery from left ventricular (LV) dysfunction is a major prognostic factor after myocardial infarction (MI). Because P2Y₁₂ receptor blockade inhibits myocardial injury, ticagrelor with off-target properties may have myocardial protection over clopidogrel. In animal models, ticagrelo...
journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2018.0415
更新日期:2019-07-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Recent surgical results from total anomalous pulmonary venous return (TAPVR) are reported to have improved; however, mortality and morbidity are still high in the univentricles. This review was performed in order to demonstrate surgical results from TAPVR for recent years in a single institute...
journal_title:Korean circulation journal
pub_type: 杂志文章
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journal_title:Korean circulation journal
pub_type: 杂志文章
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更新日期:2011-09-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2010.40.4.191
更新日期:2010-04-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
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更新日期:2016-05-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2014.44.6.386
更新日期:2014-11-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2016.46.4.580
更新日期:2016-07-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
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更新日期:2017-03-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2020.0055
更新日期:2020-09-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2014.44.3.184
更新日期:2014-05-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2019.0074
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abstract:BACKGROUND AND OBJECTIVES:Pentraxin 3 (PTX3) was shown to be elevated in the acute phase of acute myocardial infarction (AMI) and to have prognostic significance in AMI patients. The aim of this study was to estimate whether the value of PTX3 could be used as a prognostic biomarker, with the global registry of acute co...
journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2010.40.8.370
更新日期:2010-08-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2012.42.5.295
更新日期:2012-05-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2013.43.6.408
更新日期:2013-06-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2012.42.5.366
更新日期:2012-05-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Dysregulation of histone deacetylase expression and enzymatic activity is associated with a number of diseases. It has been reported that protein levels of histone deacetylase (HDAC)1 and HDAC5 increase during human pulmonary hypertension, and that the enzymatic activity of HDAC6 is induced in...
journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2016.0266
更新日期:2017-05-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2013.43.5.336
更新日期:2013-05-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2011.41.12.763
更新日期:2011-12-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章
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更新日期:2017-01-01 00:00:00
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journal_title:Korean circulation journal
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更新日期:2017-11-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:The high-sensitivity C-reactive protein (hs-CRP), a marker of inflammation, has been known to be elevated in patients with coronary artery disease. However, there is controversy about the predictive value of hs-CRP after acute myocardial infarction (MI). Therefore, we evaluated the impact of i...
journal_title:Korean circulation journal
pub_type: 杂志文章
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更新日期:2013-10-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Endomyocardial biopsy is obligatory during the first year after heart transplant (HTx) for the surveillance of acute rejection. Previous attempts using cardiac biomarkers for the detection of rejection failed to show enough evidence to substitute endomyocardial biopsy. Therefore, this study so...
journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2016.46.6.811
更新日期:2016-11-01 00:00:00
abstract::Treatment of cardiac disease via the epicardium fell under the domain of cardiac surgery due to the need for an open thoracotomy. Since an open thoracotomy is invasive in nature and has the potential for complications, a minimally invasive and percutaneous approach would be more attractive for suitable patients. The r...
journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2010.40.10.479
更新日期:2010-10-01 00:00:00
abstract::Traumatic thoracic aortic injury is typically fatal. However, recent improvements in pre-hospital care and diagnostic modalities have resulted in an increased number of patients with traumatic aortic injury arriving alive at the hospital. Also, the morbidity and mortality associated with endovascular repair are signif...
journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2012.42.5.341
更新日期:2012-05-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章,评审
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更新日期:2017-07-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Previous studies have evaluated atrial electromechanical delays (AEMDs) with a number of different definitions to discriminate patients with paroxysmal atrial fibrillation (PAF) from controls without PAF. However, their discriminative values for PAF have not previously been directly compared. ...
journal_title:Korean circulation journal
pub_type: 杂志文章
doi:10.4070/kcj.2015.45.6.479
更新日期:2015-11-01 00:00:00
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journal_title:Korean circulation journal
pub_type: 杂志文章,评审
doi:10.4070/kcj.2018.0246
更新日期:2019-02-01 00:00:00
abstract:: ...
journal_title:Korean circulation journal
pub_type: 社论
doi:10.4070/kcj.2018.0269
更新日期:2018-12-01 00:00:00