• Tuberculosis and Respiratory Diseases
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• v.47 no.2
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• pp.150-160
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• 1999

Background: The CREST syndrome is an indolent form of progressive systemic sclerosis. Although its clinical progress is indolent, pulmonary hypertension(PH) associated with CREST syndrome have grave prognosis with over 40 percent mortality rate at 2 year follow-up. But the pathogenesis of pulmonary hypertension in this disease is not known, and classified as either primary or secondary PH. Clonality of endothelial cell proliferation in plexiform lesion is a molecular marker which allows distinction between primary and secondary PH. We performed this study to know whether the PH associated with CREST syndrome is a variant of primary PH or is a secondary PH. Methods: We assessed the X-chromosome inactivation based on the methylation pattern of the human androgen-receptor gene by PCR(HUMARA). Endothelial cells in plexiform lesions from female patients(n=3) with PH associated with CREST syndrome were microdissected from paraffin blocks. Vascular smooth muscle cells and lung parenchyma were also microdissected for clonality studies. Results: The proliferating endothelial cells in 14 plexiform lesions were all polyclonal. Similarly proliferated smooth muscle cells from 5 vessels with medial hypertrophy were also polyclonal. Conclusion: These results suggest that the pulmonary hypertension associated with CREST syndrome has different pathogenesis from primary PH and to be classified as secondary PH.

• Clinical and Experimental Pediatrics
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• v.52 no.10
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• pp.1175-1180
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• 2009

We report a case of a full-term neonate with persistent pulmonary hypertension who developed asphyxia after birth and was treated with iloprost. The neonate had persistent hypoxia and did not respond to supportive treatment. Because inhaled nitric oxide (iNO) was not available in our hospital, inhaled iloprost was administered via an endotracheal tube. This resulted in an immediate elevation of oxygen saturation. Echocardiography revealed the conversion of the right-to-left ductal shunt to the left-to-right one and a decrease of the right ventricular pressure. The use of inhaled iloprost did not cause any significant side effects. Here, we describe our experience where iloprost was used in a neonate with persistent pulmonary hypertension because the standard therapy with inhaled nitric oxide was not available.

• Journal of Acupuncture Research
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• v.28 no.2
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• pp.117-123
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• 2011

목적 : 엘라스타제는 폐의 탄성 섬유를 파괴하여 폐기종과 폐고혈압을 유발하는 역할을 한다. 이에 본 연구는 문합약침액(文蛤藥鍼液)의 엘라스타제 억제효능과 항산화효능을 측정하여 폐기종과 폐고혈압 치료의 가능성을 확인하고자 실시하였다. 방법 : 문합약침액(文蛤藥鍼液)의 엘라스타제에 대한 억제효능과 di(phenyl)-(2,4,6-trinitrophenyl) iminoazanium (DPPH) 및 nitric oxide(NO) 자유기 소거능을 측정하였다. 결과 : 문합약침액(文蛤藥鍼液)은 엘라스타제 활성을 통계적으로 유의하게 억제하였고, DPPH 및 NO 자유기 소거능이 있음을 통계적으로 유의하게 확인하였다. 결론 : 본 연구를 통해 문합약침액(文蛤藥鍼液)의 엘라스타제 억제효능과 항산화 효능이 확인되었다. 본 연구 결과를 근거로 시험관 내 실험 이외의 추가적인 연구를 통해 폐기종과 폐고혈압 치료제로서 문합약침액(文蛤藥鍼液)의 활용을 고려해 볼 수 있을 것으로 기대한다.

• Korean Journal of Acupuncture
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• v.28 no.1
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• pp.15-22
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• 2011

엘라스타제는 폐의 탄성 섬유를 파괴하여 폐기종과 폐고혈압을 유발하는 역할을 한다. 이에 본 연구는 택사(澤瀉) 약침액(藥鍼液)의 엘라스타제 억제효능과 항산화효능을 측정하여 폐기종과 폐고혈압 치료의 가능성을 확인하고자 실시 하였다. 방법 : 택사(澤瀉) 약침액(藥鍼液)의 엘라스타제에 대한 억제효능과 di(phenyl)-(2,4,6-trinitrophenyl) iminoazanium (DPPH) 및 nitric oxide (NO) 자유기 소거능을 측정하였다. 결과 : 택사(澤瀉) 약침액(藥鍼液)은 엘라스타제 활성을 통계적으로 유의하게 억제하였고, DPPH 및 NO 자유기 소거능이 있음이 통계적으로 유의하게 확인되었다. 결론 : 본 연구를 통해 택사(澤瀉) 약침액(藥鍼液)의 엘라스타제 억제효능과 항산화 효능이 확인되었다. 본 연구 결과를 근거로 시험관내 실험이외의 추가적인 연구를 통해 폐기종과 폐고혈압 치료제로서 택사(澤瀉) 약침액(藥鍼液)의 활용을 고려해 볼 수 있을 것으로 기대한다.

• Neonatal Medicine
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• v.18 no.1
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• pp.96-103
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• 2011

Purpose: Although infants with bronchopulmonary dysplasia (BPD) are at risk of developing secondary pulmonary hypertension (PH), which is associated with significant morbidity and mortality, little has been reported about the incidence, clinical course and prognosis of PH secondary to BPD in premature infants. This study was done to investigate the incidence, risk factors, clinical course, and the ultimate prognosis of PH developed secondary to BPD in very low birth weight infants (<1,500 g). Methods: Medical records of very low birth weight infant (VLBWI) admitted to Samsung Medical Center NICU from January 2000 to July 2007 were reviewed retrospectively. BPD was defined by Jobe's classification. The diagnosis of pulmonary hypertension was established as velocity of tricuspid valve regurgitation (TR) ${\geq}$3 m/s and a flattening of the intraventricular septum by conducting Doppler echocardiography. Results: The incidence of pulmonary hypertension was 6% in VLBWI with BPD and it developed in moderate to severe BPD. The diagnosis of pulmonary hypertension was made on postnatal 133 days (range 40-224 days) and the risk factors related to developing pulmonary hypertension were severe BPD, small for gestational age and outborn infants. The mortality rate was 57% and especially higher in severe BPD (70%). The time to recovery spent 3 months (range 1-10 months) in survived patients. Conclusion: Based on the results of this research, pulmonary hypertension secondary to BPD in VLBWI related to severity of BPD and had a poor prognosis. We expect that regular long-term echocardiography may be helpful in treating reversible in VLBWI with moderate to severe BPD.

• Tuberculosis and Respiratory Diseases
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• v.49 no.1
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• pp.5-17
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• 2000

• Tuberculosis and Respiratory Diseases
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• v.48 no.3
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• pp.291-297
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• 2000

• Tuberculosis and Respiratory Diseases
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• v.48 no.6
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• pp.964-972
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• 2000

We report three patients with pulmonary hypertension in Takayasu's arteritis, who showed long-term favorable response, clinically and hemodynamically, to the nitric oxide donor, molsidomine. In these patients, the inhaled nitric oxide was effective in reducing pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) as was shown in the acute vasodilator response test using the invasive hemodynamic monitoring. Molsidomine (single oral dose of 4 mg) was also effective in reducing PAP and PVR in the acute test, but nifedipine was not. With 4 mg of molsidomine three times daily, their dyspnea, exercise capacity and hemodynamic parameters were improved. These favorable responses have lasted during the 1st and 3rd month follow-up in all patients.

• Tuberculosis and Respiratory Diseases
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• v.57 no.1
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• pp.66-71
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• 2004

Primary pulmonary hypertension (PPH) is a rare, progressive and incurable disease, which is characterized by an increase in the pulmonary artery pressure without a demonstrable cause. The most common presenting symptom is dyspnea on exertion, with other symptoms comprising of chest pain, syncope and hemoptysis. The diagnosis is one of exclusion of any of the known causes of pulmonary hypertension. When associated with pregnancy, the maternal mortality ranges from 30 to 50%. Because pregnancy and labor are very serious problems for patients with PPH, the available evidence suggests that pregnancy when afflicted with PPH should be avoided. In account the case of a 33-year old patient, reporting with massive hemoptysis, and diagnosed with PPH during her twenty seventh week of gestation, is presented. She was treated with conservative management, including oxygen and a vasodilator, and underwent a pregnancy termination. However, due to aggravation of right heart failure, she presented with severe systemic hypotension and hypoxemia, and eventually died. This case is reported, with brief review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.48 no.1
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• pp.67-71
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• 2000

Since its first description in 1951 by Mantz and Craig, pulmonary hypertension associated with portal hypertension has been observed more frequently. In a recent prospective study Hadengue et al. reported 2 % incidence of pulmonary hypertension in patients with portal hypertension. Thus this simultaneous occurrence can no longer be considered to be coincidental. The etiology remains unclear. It is most likely that vasoactive substances, normally metabolized by the liver, may have gained access to pulmonary circulation through portosystemic collaterals in portal hypertension. In genetically susceptible individuals, these substances could lead to pulmonary hypertension by inducing vasoconstriction or direct toxic damage to the wall of the small pulmonary arteries. A recent case of pulmonary hypertension in a 49-year-old woman with portal hypertension due to liver cirrhosis is reported as well as a review of the literature.