• Tuberculosis and Respiratory Diseases
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• v.48 no.6
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• pp.973-979
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• 2000

A glycogen storage disease(GSD) type I is a metabolic disease caused by a deficiency in one of the components of the glucose-6-phosphatase(G-6-Pase) system. This disorder results in hypoglycemia, hepatomegaly, lactic acidemia, hyperlipidemia, and hyperuricemia. Comon long(-)term complications include growth retaradation, gout, hepatic adenomas, osteoporosis and renal disease. However the cardiovascular system is rarely involved, and only six cases of pulmonary hypertension associated with GSD I have been reported in the literature. We experienced a case of pulmonary hypertension with type I GSD. A 31-year-old rnan, who had discovered type I GSD and received portocaval shunt operation 22 years ago, was admitted to the hospital with the chief complaint of dyspnea. Echocardiographic examination and cardiac catheterization revealed severe pulmonary hypertension. Nitric oxide and oral prostacycline derivative(beraprost) were tried without acute favorable response. After one year with beraprost, dyspnea, exercise capacity and hemodynamic parameters were improved. We report this case with a review of the literature.

• The Korean Journal of Physiology and Pharmacology
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• v.21 no.3
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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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• v.40 no.3
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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.

• Journal of Veterinary Clinics
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• v.39 no.5
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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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• v.59 no.2
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• pp.170-178
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• 2005

Background : Idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare but significantly imperative in inducing chronic pulmonary hypertension. Clinically, it is difficult to distinguish between IPAH and CTEPH. However, the treatment of pulmonary hypertension is different depending on the disease. The present study was performed to analyze the similarities and differences in clinical features between IPAH and CTEPH. Methods : During a nine-year period, thirty-three patients with IPAH and twenty-two patients with CTEPH were enrolled. Symptoms, physical findings, chest radiograph, electrocardiograph, pulmonary function test, echocardiograph, perfusion lung scan, right heart catheterization results were analyzed between both the groups. Results : The median age of IPAH group was 33 (6~70) years that was lower than that (52(27~80) years) of CTEPH group. Amongst the IPAH patients, there was female predominance (76 %) and there was no sex difference between the patients with CTEPH. Both the groups exhibited similarity in the results of chest radiograph, electrocardiograph, pulmonary function test, and echocardiograph. In the perfusion lung scan, all IPAH patients exhibited findings with normal (28%) or low probability (72%) of pulmonary embolism and all CTEPH patients exhibited findings with high probability of pulmonary embolism. Conclusion : Although IPAH and CTEPH bear similarities in terms of symptoms, physical signs and general investigation results, there were differences in age distribution, sex predominance and results of perfusion lung scan.

• Journal of Chest Surgery
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• v.7 no.1
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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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• v.63 no.4
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• pp.346-352
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• 2007

Background: Pulmonary hypertension is one of the cardiovascular complications of in COPD. However, a diagnosis of pulmonary hypertension requires an invasive test, such as right heart catheterization. NT-proBNP is a cardiac hormone that is elevated when a cardiac volume or pressure overload is present. It was hypothesized that NT-proBNP might play a role in detecting of pulmonary hypertension in COPD patients. Method: The 31 COPD patients, who underwent all of NT-proBNP, echocardiography, and spirometry in Seoul National University Bundang Hospital during the period from November 2003 to July 2005, were retrospectively analyzed. Result: Of the 31 COPD patients, 9 patients had pulmonary hypertension. A significant positive correlation was observed between the NT-proBNP and pulmonary arterial pressure (r=0.589, p=0.002). However, there was no significant correlation observed between the $FEV_1$ and NT-proBNP and $FEV_1$ and pulmonary arterial pressure. Conclusion: NT-proBNP might indicate the presence of pulmonary hypertension in COPD patients.

• BMB Reports
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• v.47 no.6
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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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• v.53 no.6
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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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• v.26 no.4
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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.