• Journal of Chest Surgery
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• 제48권1호
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• pp.52-54
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• 2015

Idiopathic pulmonary arterial hypertension eventually leads to right-sided heart failure and sudden death. Its mortality rate in children is still high, despite improvements in pharmacological therapy, and therefore novel treatments are necessary. The Potts shunt, which creates an anastomosis between the left pulmonary artery and the descending aorta, has been proposed as a theoretically promising palliative surgical technique to decompress the right ventricle. We report the case of a 12-year-old girl with suprasystemic idiopathic pulmonary hypertension and right ventricular failure who underwent a Potts shunt for palliation with good short-term results.

• Tuberculosis and Respiratory Diseases
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• 제59권2호
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• pp.170-178
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• 2005

배 경 : 특발성 폐동맥고혈압(idiopathic pulmonary arterial hypertension, IPAH)과 만성혈전색전성 폐고혈압(chronic thromboembolic pulmonary hypertension, CTEPH)은 드문 질환이지만 만성적인 폐동맥 고혈압을 유발하는 중요한 질환이다. 두 질환은 임상소견 및 검사소견이 유사한 질환으로 감별이 어렵지만 치료의 차이 때문에 감별이 꼭 필요한 질환이다. 한 대학병원에서 경험한 두 질환의 임상상의 유사점과 차이점을 분석하고자 하였다. 대상 및 방법 : 서울아산병원에서 1995년부터 2002년까지 IPAH로 진단 받은 환자 33명과 CTEPH으로 진단 받은 환자 22명에 대해서 작성된 프로토콜과 의무기록을 검토하여 증상, 신체검사, 심전도, 흉부단순촬영, 폐기능검사, 심초음파, 핵의학검사, 심도자검사 등의 임상소견을 비교, 분석하였다. 결 과 : 나이의 중앙값은 IPAH군에서 33세(6~70세)로 CTEPH군의 52세(27~78세)보다 적었다. 성별은 IPAH군은 33명중 여자가 25명(76%)으로 남자보다 많았고 CTEPH군은 22명중 남자가 12명(55%)으로 성비에 차이가 없었다. 흉부단순촬영상 계측치, 심전도에서 계측치, 폐기능검사에서 폐활량과 폐확산능 그리고 심초음파에서 삼첨판최고역류속도는 두 군간에 차이가 없었다. 폐관류스캔에서는 IPAH군의 28.1%의 환자가 정상소견을 보였고 71.9%의 환자가 폐색전증의 낮은 가능성 소견을 보였다. 이에 반해 CTEPH군에서는 22명 모든 환자에서 폐색전의 높은 가능성소견이 관찰되었다. 결 론 : CTEPH과 IPAH는 임상증상이나 일반적인 검사소견이 유사한 질환이지만, 나이와 성별분포 및 폐관류 스캔소견에 차이를 보여 이러한 차이점들이 두 질환의 감별에 도움을 줄 것으로 사료된다.

• Tuberculosis and Respiratory Diseases
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• 제61권1호
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• pp.74-79
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• 2006

저자들은 종격동에 전반적인 섬유화성 연부조직 침윤으로 인해 폐동맥 협착을 일으켜 호흡곤란과 폐동맥 고혈압이 발생한 특발성 섬유화성 종격동염을 진단하고, 경구 부신피질 호르몬 투여 후 호전을 보인 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제63권6호
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• pp.475-479
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• 2007

• The Korean Journal of Physiology and Pharmacology
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• 제21권3호
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• pp.353-360
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• 2017

Several human diseases have been associated with mitochondrial voltage-dependent anion channel-1 (VDAC1) due to its role in calcium ion transportation and apoptosis. Recent studies suggest that VDAC1 may interact with endothelium-dependent nitric oxide synthase (eNOS). Decreased VDAC1 expression may limit the physical interaction between VDAC1 and eNOS and thus impair nitric oxide production, leading to cardiovascular diseases, including pulmonary arterial hypertension (PAH). In this report, we conducted meta-analysis of genome-wide expression data to identify VDAC1 influenced genes implicated in PAH pathobiology. First, we identified the genes differentially expressed between wild-type and Vdac1 knockout mouse embryonic fibroblasts in hypoxic conditions. These genes were deemed to be influenced by VDAC1 deficiency. Gene ontology analysis indicates that the VDAC1 influenced genes are significantly associated with PAH pathobiology. Second, a molecular signature derived from the VDAC1 influenced genes was developed. We suggest that, VDAC1 has a protective role in PAH and the gene expression signature of VDAC1 influenced genes can be used to i) predict severity of pulmonary hypertension secondary to pulmonary diseases, ii) differentiate idiopathic pulmonary artery hypertension (IPAH) patients from controls, and iii) differentiate IPAH from connective tissue disease associated PAH.

• Tuberculosis and Respiratory Diseases
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• 제85권2호
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• pp.122-136
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• 2022

Although chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) have distinct clinical features, both diseases may coexist in a patient because they share similar risk factors such as smoking, male sex, and old age. Patients with both emphysema in upper lung fields and diffuse ILD are diagnosed with combined pulmonary fibrosis and emphysema (CPFE), which causes substantial clinical deterioration. Patients with CPFE have higher mortality compared with patients who have COPD alone, but results have been inconclusive compared with patients who have idiopathic pulmonary fibrosis (IPF). Poor prognostic factors for CPFE include exacerbation, lung cancer, and pulmonary hypertension. The presence of interstitial lung abnormalities, which may be an early or mild form of ILD, is notable among patients with COPD, and is associated with poor prognosis. Various theories have been proposed regarding the pathophysiology of CPFE. Biomarker analyses have implied that this pathophysiology may be more closely associated with IPF development, rather than COPD or emphysema. Patients with CPFE should be advised to quit smoking and undergo routine lung function tests, and pulmonary rehabilitation may be helpful. Various pharmacologic agents and surgical approaches may be beneficial in patients with CPFE, but further studies are needed.

• 대한한방소아과학회지
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• 제22권2호
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• pp.37-49
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• 2008

Objectives : Idiopathic pulmonary fibrosis (IPF) is chronic fibrotic interstitial pneumonia and the pathogenesis is unknown. Peucedani Radix is well-known for the treatment of respiratory diseases and pulmonary hypertension. This study was to evaluate the effectiveness of Peucedani Radix on the bleomycin-induced lung fibrosis model (BLFM) in mouse. Methods : We induced lung fibrosis by intratracheal instillation of bleomycin in C57BL/6J. We compared two groups BLFM without Peucedani Radix (group I) and BLFM with Peucedani Radix (group II). We performed bronchoalveolar lavages (BAL) and obtained lung specimens from both group I and II on the 7th (A) and 21st (B) day, and also for the normal group. We compared with group I and II to find BAL by using ANOVA test and to find pathologic symptoms by using semiquantitative histological index (SHI). Results : In BAL, total cell counts, lymphocytes, and neutrophils was increased in both group I and II comparing with normal group. However, lymphocyte level was decreased more in group IIB than group IB. It was statistically significant. In microscopic findings, scores of SHI in normal group, group IB and IIB were 0.33, 4.47, and 1.96 each. Conclusions : Peucedani Radix might have inhibitory effect on lung fibrosis by reducing inflammatory cells in bleomycin-induced lung fibrosis model in mouse.

• Tuberculosis and Respiratory Diseases
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• 제67권2호
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• pp.113-120
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• 2009

Background: Reactive oxygen species (ROS) by oxidative stress may play an important role in the pathogenesis of various chronic diseases such as diabetes mellitus, obesity, hyperlipidemia, hypertension and malignancy that are linked to metabolic syndrome. Oxidative stress has been implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF). We examined the relationship between IPF and presenting factors associated with metabolic disorders. Methods: One hundred fourteen patients who met the current consensus of IPF definition were enrolled from March 2000 to April 2006 in Gil Hospital and Samsung Medical Center in Korea. One hundred thirty-four control subjects without pulmonary diseases were selected from subjects who visited Gil hospital for routine medical examinations, including low-dose chest computed tomography from January 2002 to July 2006. Retrospectively, we analyzed the clinical characteristics, the results of blood examinations, and lung function tests from medical records of both groups. Results: IPF patients and control subjects differed in the prevalence of diabetes mellitus as assessed by univariate analysis. Multivariate analysis demonstrated that diabetes mellitus and obesity were associated with IPF. The adjusted odds ratios for diabetes mellitus were 2.733 (95% confidence interval [CI], 1.282~5.827) and 2.001 (95% [CI], 1.063~3.766) for obesity. The remaining factors tested showed no differences between the patient group and the control. Conclusion: Diabetes mellitus and obesity may be associated with IPF development.

• 수면정신생리
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• 제24권1호
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• pp.19-23
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• 2017

Obstructive sleep apnea is a common disorder in which respiratory flow decreases or disappears despite respiratory effort due to occlusion of the upper respiratory tract during sleep. Oxidative stress and systemic inflammatory reaction induced by the obstruction cause complications such as hypertension, coronary artery disease, and diabetes and increase cancer incidence. Furthermore, in patients with interstitial lung disease, obstructive sleep apnea has a very high prevalence and is thought to have a close pathophysiological and clinical correlation. In other words, obstructive sleep apnea could be the cause or a complication of interstitial lung disease ; when these two afflictions coexist, the prognosis of the patient is worse. In patients with interstitial lung disease with obstructive sleep apnea, CPAP treatment significantly improved sleep and quality of life, as well as improved morbidity and mortality in a recent study. Therefore, early diagnosis and treatment of obstructive sleep apnea in patients with interstitial lung disease are very important, and additional studies designed to include patients with idiopathic pulmonary fibrosis as well as patients with advanced interstitial lung disease should be performed.

• Neonatal Medicine
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• 제18권1호
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• pp.124-129
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• 2011

목적: 최근 PPHN의 치료를 위해 iNO와 함께 많은 약물적 치료가 사용되고 있다. Sildenafil은 PDE5의 억제제로 선택적인 폐혈관 확장제로 알려져 있으며 iNO에 비하여 접근성이 뛰어나 iNO에 대안적인 약제로 연구되어 왔다. 저자들은 본원에서 sildenafil로 PPHN을 치료한 경험을 분석하였다. 방법: 심초음파를 통해 PPHN으로 진단된 재태 기간 35주 이상, FiO2 1.0을 필요로 하는 32명의 환아를 대상으로 하였으며 의무기록을 후향적으로 조사하였다. sildenafil은 0.5 mg/kg로 시작하여 6시간마다 1 mg/kg로 투약하였으며, 투약 전과 후 6, 12, 24, 48시간의 MAP, $FiO_2$, OI, MBP를 비교하였다. 부작용을 확인하기 위하여 환아들의 위장관 증상, 뇌초음파, 안저검사와 청성뇌간유발반응 검사의 결과를 조사하였다. 결과: 대상 환아 32명 중 태변 흡인 증후군 9명, 호흡곤란증후군 8명, 폐렴 3명이었으며, 12명은 특발성이었다. 이 중 31명이 생존하였으며 28명의 환아는 본원에서 sildenafil만으로 치료되었고 3명의 환아는 전원되어 iNO 치료 후 회복하였다. Sildenafil 단독으로 치료된 28명의 환아에서 $FiO_2$와 OI는 치료 6시간째부터 MAP는 48시간째부터 유의하게 감소하였다. 모든 환아에서 특별한 부작용은 관찰되지 않았다. 결론: Sildenafil은 35주 이상의 PPHN 환아에서 비교적 효과적이고 안전하게 시도해 볼 수 있는 대안 약제로서, 특히 iNO가 가능하지 않은 병원에서 유용할 것으로 생각된다.