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Clinical Presentation with High Penetrance in a Korean Family with Pulmonary Arterial Hypertension Associated with a BMPR2 Intron 3 Splice Site Pathogenic Variant

Kim MJ, Lee S, Jekarl DW, Chae H, Kim M, Jung HO, Jeon DS

Pathogenic variants of bone morphogenic protein receptor type 2 gene (BMPR2) are related to the majority of cases of heritable pulmonary arterial hypertension (PAH). Over 400 pathogenic variants have been...
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15 years journey of idiopathic pulmonary arterial hypertension with BMPR2 mutation

Ahn KJ, Jang AY, Park SJ, Chung WJ

Pulmonary arterial hypertension (PAH) is known as one of diseases with the worst prognosis. Recently, targeted PAH drugs have been developed and approved for use; therefore, the treatment strategy and...
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Survival benefits of warfarin in Korean patients with idiopathic pulmonary arterial hypertension

Kang BJ, Oh YM, Lee SD, Lee JS

BACKGROUND/AIMS: Idiopathic pulmonary arterial hypertension (IPAH) is an incurable disease with high mortality. Although most studies recommend anticoagulation treatment for IPAH, the benefits are uncertain, particularly in Korea, where it...
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Functional Class and Targeted Therapy Are Related to the Survival in Patients with Pulmonary Arterial Hypertension

Park YM, Chung WJ, Choi DY, Baek HJ, Jung SH, Choi IS, Shin EK

PURPOSE: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. MATERIALS AND METHODS: Patients who were...
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Optimal Dose and Timing of Umbilical Stem Cells Treatment in Pulmonary Arterial Hypertensive Rats

Lee H, Kim KC, Choi SJ, Hong YM

PURPOSE: Pulmonary arterial hypertension (PAH) is a fatal disease which is characterized by an increase in pulmonary arterial pressure leading to increases in right ventricular afterload. Human umbilical cord blood...
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VEGF Promoter Polymorphism Confers an Increased Risk of Pulmonary Arterial Hypertension in a Chinese Population

Zhuo Y, Zeng Q, Zhang P, Li G, Xie Q, Cheng Y

PURPOSE: Evidence on the contribution of genes to the hereditary predisposition to pulmonary arterial hypertension (PAH) is limited. MATERIALS AND METHODS: In this study, we hypothesized that single nucleotide variants in...
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Baseline Characteristics of the Korean Registry of Pulmonary Arterial Hypertension

Chung WJ, Park YB, Jeon CH, Jung JW, Ko KP, Choi SJ, Seo HS, Lee JS, Jung HO, KORPAH Investigators

Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of...
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