• Tuberculosis and Respiratory Diseases
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• 제48권6호
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• pp.973-979
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• 2000

제 I 형 당원병에서 폐고혈압은 드문 합병증으로 외국 문헌에서 6예만이 보고되었으나 국내에는 아직 보고된 바가 없다. 저자들은 최근 제 Ia형 당원병으로 진단 받았던 31세 남자 환자에서 폐고혈압이 동반되었던 예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• 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.

• Journal of Ginseng Research
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• 제40권3호
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• pp.285-291
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• 2016

Background: Ginsenosides have been shown to exert beneficial pharmacological effects on the central nervous, cardiovascular, and endocrine systems. We sought to determine whether total ginsenosides (TG) inhibit monocrotaline (MCT)-induced pulmonary hypertension and to elucidate the underlying mechanism. Methods: MCT-intoxicated rats were treated with gradient doses of TG, with or without $N^G$-nitro-$\small{L}$-arginine methyl ester. The levels of molecules involving the regulation of nitric oxide and mitogen-activated protein kinase pathways were determined. Results: TG ameliorated MCT-induced pulmonary hypertension in a dose-dependent manner, as assessed by the right ventricular systolic pressure, the right ventricular hypertrophy index, and pulmonary arterial remodeling. Furthermore, TG increased the levels of pulmonary nitric oxide, endothelial nitric oxide synthase, and cyclic guanosine monophosphate. Lastly, TG increased mitogen-activated protein kinase phosphatase-1 expression and promoted the dephosphorylation of extracellular signal-regulated protein kinases 1/2, p38 mitogen-activated protein kinase, and c-Jun NH2-terminal kinase 1/2. Conclusion: TG attenuates MCT-induced pulmonary hypertension, which may involve in part the regulation of nitric oxide and mitogen-activated protein kinase pathways.

• 한국임상수의학회지
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• 제39권5호
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• pp.240-245
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• 2022

A 15-year-old, neutered male, Shih-Tzu, was presented at the Chonnam National Veterinary Medical Teaching Hospital for evaluation of acute onset of persistent coughing, exercise intolerance, and abnormal heart sound. On thoracic auscultation, a split-second heart sound and a wheezing sound were detected on both sides of the chest walls. On physical examination, the dog's body condition score (BCS) was 7/9, and had stenotic nares. Thoracic radiographs revealed right-sided enlargement of the cardiac silhouette (vertebral heart score (VHS) 11.2; reference interval = 8.9-10.1), mild main pulmonary artery (MPA) bulging, mild interstitial infiltration, and hepatomegaly. The electrocardiogram showed right axis deviation, suggesting right ventricular hypertrophy. The echocardiographic study showed moderate pulmonary hypertension and moderate tricuspid regurgitation. There were no findings of a tracheobronchial disease, pulmonary thromboembolism, congenital shunt, left heart disease, or parasitic disease. Based on clinical signs and diagnostic findings, the dog was diagnosed with pulmonary hypertension secondary to brachycephalic syndrome. To rectify respiratory exacerbating factors, the dog was recommended weight control by restricting dietary intake and managing concurrent Cushing's syndrome. Treatments included sildenafil, pimobendan, furosemide, and ramipril. After five months of taking medications and weight control, the severity of pulmonary hypertension improved from moderate to mild. The clinical signs of the patient, including coughing and exercise intolerance, improved a lot. For 5 months of follow-up, the patient has not reported further recurrence of respiratory distress.

• 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.

• Journal of Chest Surgery
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• 제7권1호
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• pp.85-92
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• 1974

During the years 1959 to 1974, 99 patients with patent ductus arteriosus were admitted to National University Hospital. These includes 5 patients with additional cardiovascular and 5 patients with severe pulmonary hypertension. All were operated upon except three refused operation. In all instances, the diagnosis was made by history and physical, roentgenological and electrographic examinations. In addition, in 53 patients, special diagnostic procedures were carried out either for diagnosis or for evaluation of pulmonary hypertension and associated cardiovascular anomalies. Right cardiac catheterization was resorted to in 51 patients. In one of these patients catheterization was incorrectly interpreted [ventricular septal defect]. Retrograde aortogram was performed in two patients. In both cases the ductus itself was visualized on the x-ray film. An additional vascular anomaly, namely the persistent left superior vena cava, was confirmed by retrograde angiogram in one of them. In 5 cases the pulmonary arterial pressure was elevated well over 80 mmHg. In these instances,the operative mortality was 80% [4 out of 5 patients]. The management of patent ductus arteriosus when associated with severe pulmonary hypertension. and/or other cardiac anomalies is controversial. Opinions differ as to how to close the ductus and to repair the cardiac anomalies as well as to whether a one-staged or two-staged procedure should be resorted to. The author is of the that each case must be evaluated individually before any specific surgical treatment is ou.tlined. The literature on the subject is reviewed in this paper.

• Tuberculosis and Respiratory Diseases
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• 제63권4호
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• pp.346-352
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• 2007

연구 배경: 폐동맥 고혈압은 만성 폐쇄성 폐질환 환자에서 중요한 심혈관계 합병증이다. 뇌나트륨이뇨펩티드는 허혈성 심질환과 심부전의 경우에서 진단적 효율성과 예후 예측인자로서의 효율성이 잘 알려져 있고 우심에 압력 과부하가 있는 상황에서도 증가된다. 뇌나트륨이뇨펩티드의 측정이 만성 폐쇄성 폐질환 환자에서 폐동맥 고혈압의 진단에 유용성이 있는지 알아보고자 폐동맥압과 뇌나트륨이뇨펩티드와의 상관관계를 분석하였다. 연구 방법: 의무 기록 분석을 통하여 뇌나트륨이뇨펩티드, 심초음파, 폐기능 검사를 모두 실시한 만성 폐쇄성 폐질환 환자들을 대상으로 연구를 수행하였다. 뇌나트륨이뇨펩티드(NT-proBNP), 폐동맥압(PAP), 1초간 노력성 폐활량($FEV_1$)과의 상관관계를 분석하였다. 결과: 총 31명의 환자가 분석에 포함되었으며 폐동맥 고혈압이 동반된 환자는 9명(26.7%)이었다. 폐동맥압과 뇌나트륨이뇨펩티드 사이에 유의한 상관관계가 관찰되었다(r=0.589, p=0.002). 결론: 뇌나트륨이뇨펩티드는 만성 폐쇄성 폐질환 환자들에서 동반된 폐동맥 고혈압의 진단에 사용될 수 있을 것으로 사료된다.

• BMB Reports
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• 제47권6호
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• pp.311-317
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• 2014

microRNAs (miRNAs) are a class of small, non-coding RNAs that play critical posttranscriptional regulatory roles typically through targeting of the 3'-untranslated region of messenger RNA (mRNA). Mature miRNAs are known to be involved in global cellular processes, such as differentiation, proliferation, apoptosis, and organogenesis, due to their capacity to target multiple mRNAs. Thus, imbalances in the expression and/or activity of miRNAs are involved in the pathogenesis of numerous diseases, including pulmonary arterial hypertension (PAH). PAH is a progressive disease characterized by vascular remodeling due to excessive proliferation of pulmonary artery endothelial cells (PAECs) and pulmonary artery smooth muscle cells (PASMCs). Recently, studies have evaluated the roles of miRNAs involved in the pathogenesis of PAH in these pulmonary vascular cells. This review provides an overview of recent discoveries on the role of miRNAs in the pathogenesis of PAH and discusses the potential for miRNAs as therapeutic targets and biomarkers of PAH.

• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.688-693
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• 2010

An increase in the number of preterm infants and a decrease in the gestational age at birth have resulted in an increase in the number of patients with significant bronchopulmonary dysplasia (BPD) and secondary pulmonary hypertension (PH). PH contributes significantly to the high morbidity and mortality in the BPD patients. Therefore, regular monitoring for PH by using echocardiography and B-type natriuretic peptide (BNP) or N-terminal-proBNP must be conducted in the BPD patients with greater than moderate degree to prevent PH and to ensure early treatment if PH is present. In the BPD patients with significant PH, multi-modality treatment, including treatment for correcting an underlying disease, oxygen supply, use of diverse selective pulmonary vasodilators (inhaled nitric oxide, inhaled prostacyclins, sildenafil, and endothelin-receptor antagonist) and other methods, is mandatory.

• Journal of Cardiovascular Imaging
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• 제26권4호
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• pp.189-200
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• 2018

Pulmonary hypertension (PH) is defined as resting mean pulmonary artery pressure ${\geq}25mmHg$ and is caused by multiple etiologies including heart, lung or other systemic diseases. Evaluation of right ventricular (RV) function in PH is very important to plan treatment and determine prognosis. However, quantification of volume and function of the RV remains difficult due to complicated RV geometry. A number of imaging tools has been utilized to diagnose PH and assess RV function. Each imaging technique including conventional echocardiography, three-dimensional echocardiography, strain echocardiography, computed tomography and cardiac magnetic resonance imaging has-advantages and limitations and can provide unique information. In this article, we provide a comprehensive review of the utility, advantages and shortcomings of the multimodality imaging used to evaluate patients with PH.