1. Rubin LJ: Introduction. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126: 7S-10S.
2. Hatano S, Strasser T: Primary pulmonary hypertension.
Report of commiteeWorld health organization, Geneva, 1975 3. Yamaki S,Wagenvoort CA: Comparison of primary
plexogenic arteriopathy in adults and children. A Morphometric study in 40 patients. Br Heart J 1985;
54:428-34.
4. Wagenvoort CA, Wagenvoort N: Pathology of pulmonary hypertension. John Wiley & Sons, New York, 1977; pp69-75(a), 185-95(b), 143-76(c).
5. Heath D, Edwards J: The pathology of hypertensive pulmonary vascular disease. A description of six grades of structural changes in the pulmonary arteries with special references to congenital cardiac septal defects. Circulation 1958; 18: 533-48.
6. Rossi G, Bitterman P, Rennard S. et al.: Evidence for chronic inflammation as a component of the interstitial lung disease associated with progressive systemic sclerosis. Ann Rev Respi Dis 1985; 131: 612-7.
7. 沢井高志,京極方久:膠原病における肺の血管病変.─
肺高血圧症との関係において─ 呼と循 34: 59-67.
8. Yamaki S, Tezuka F: Quantitative analysis of pulmonary vascular disease in complete transposition of the great arteries. Circulation 1976; 54: 805-9.
9. Yamaki S, Horiuchi T, Sekino Y: Quantitative analysis of pulmonary vascular disease in simple cardiac anomalies with the Down syndrome. Am J Cardiol 1983; 51: 1502-6.
10. Jones PL, Cowan KN, Rabinovitch: Tenascin - C, proliferation and subendothelial fibronectin in progressive pulmonary vascular disease. Am J Pathol 1997; 150: 1349-60.
11. Yamaki S, Mohri H, Haneda K. et al: Indication for surgery based on lung biopsy in cases of ventricular septal defect and/
or patent ductus arteriosus with severe pulmonary hypertension. Chest 1989; 96: 31-9.
12. Wagenvoort CA, Mooi WJ: Biopsy pathology of the pulmonary vasculature. Chapman and Hall Medical. London, 1989; p81-97.
13. 八巻重雄:臨床家のための肺血管病変肺生検診断.メデ イカルレビュー社 2000; pp48(a), 183-7(b), 84-5(c).
14. Yamaki S, Horiuchi T, Ishizawa E, et al: Indication for total correction of complete transposition of the great arteries with pulmonary hypertension. J Thorac Cardiovasc Surg 1980; 79:
890-5.
15. Yamaki S, Yasui H, Kado H, et al: Pulmonary vascular disease and operative indications in complete atrioventricular canal defect in early infancy. J Thorac Cardiovasc Surg 1993;
106: 398-405.
16. Yamaki S, Horiuchi T, Miura M, et al: Secundum atrial septal defect with severe pulmonary hypertension. Open lung
biopsy diagnosis of operative indication. Chest 1987; 91:
33-8.
17. Wagenvoort CA, Wagenvoort N, Draulans-Noe Y:
Reversibility of plexogenic pulmonary arteriopathy following banding of the pulmonary artery. J Thorac Cardiovasc Surg 1984; 87: 876-86.
18. Hasleton PS, Heath D, Brewer DB: Hypertensive pulmonary vasclar disease in states of chronic hypoxia. J Path Bact 1968; 95: 431-9.
19. Badesch DB, Abman SH, Ahearn GS, et al: Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126: 35S-62S.
20. Badesch DB, Abman SH, Simonneau G, et al: Medical Therapy for Pulmonary Arterial Hypertension: Updated ACCP Evidence-Based Clinical Practice Guidelines. Chest 2007; 131: 1917-28.
21. No authors: The International Primary Pulmonary Hypertension Study (IPPHS). Chest 1994; 105: 37S-41S.
22. Deng Z, Morse JH, Slager SL, et al: Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000; 67: 737-44.
23. Thomson JR, Machado RD, Pauciulo MW, et al: Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family. J Med Genet 2000; 37: 741-5.
24. Harrison RE, Flanagan JA, Sankelo M, et al: Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia. J Med Genet 2003; 40: 865-71.
25. Harrison RH, Berger R, Haworth SG, et al: Transforming Growth factor -β and pulmonary arterial hypertension in childhood. Circulation 2005; 111: 435-41.
26. Rich S, Dantzker DR, Ayres SM, et al: Primary pulmonary hypertension. A national prospective study: Annals of Internal Medicine 1987; 107: 216-23.
27. D’Alonzo GE, Barst RJ, Ayres SM, et al: Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115:
343-9.
28. D’Alonzo GE,Barst RJ, Ayres SM, et al: Survival in patients with primary pulmonary hypewrtension. Ann Intern Med 1991: 115: 343-9.
29. Miyamoto S, Nagaya N, Satoh T, et al: Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med 2000; 161: 487-92.
30. Nagaya N, Nishikimi T, Uematsu M, et al: Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation 2000; 102:
865-70.
31. Nagaya N, Uematsu M, Satoh T, et al: Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999;
160: 487-92.
32. Galie N, Seeger W, Naeije R, et al: Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43:
81S-88S.
33. Rich S, Kaufmann E, Levy PS: The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327: 76-81.
34. Fuster V, Steele PM, Edwards WD, et al: Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580-7.
35. Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005; 111: 3105-11.
36. Sitbon O, Humbert M, Jagot JL, et al: Inhaled nitric oxide as a screening agent for safely identifying responders to oral calcium-channel blockers in primary pulmonary hypertension.
Eur Respir J 1998; 12: 265-70.
37. Groves MB, Badesch DB, Turkevich D, et al: Correlation of acute prostacyclin responce in primaty (unexplained) pulmonary hypertension with efficacy of treatment with calcium channel blockers and survinal. In Weir K, ed. Ion flux in pulmonary vasvular control. New York, NY: Plenum Press, 1993; 317-30.
38. Schrader BJ, Inbar S, Kaufmann L, et al: Comparison of the effects of adenosine and nifedipine in pulmonary hypertension. J Am Coll Cardiol 1992; 19: 1060-4.
39. Moncada S, Gryglewski R, Bunting S, et al: An enzyme isolated from arteries transforms prostaglandin endoperoxides to an unstable substance that inhibits platelet aggregation.
Nature 1976; 263: 663-5.
40. Christman BW, McPherson CD, Newman JH, et al: An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension.N Engl J Med. 1992; 327:70-5.
41. Tuder RM, Cool CD, Geraci MW, et al: Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 1999; 159: 1925-32.
42. Watkins WD, Peterson MB, Crone RK, et al: Prostacyclin and prostaglandin E1 for severe idiopathic pulmonary artery hypertension. Lancet 1980; 1: 1083.
43. Higenbottam T, Wheeldon D, Wells F, et al: Long-term treatment of primary pulmonary hypertension with continuous intravenous epoprostenol (prostacyclin). Lancet 1984; 1:
1046-7.
44. Rubin LJ, Mendoza J, Hood M, et al: Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial.
Ann Intern Med 1990; 112: 485-91.
45. Barst RJ, Rubin LJ, McGoon MD, et al: Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin.Ann Intern Med 1994; 121: 409-15.
46. Barst RJ, Rubin LJ, Long WA, et al: A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension.
The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 334: 296-302.
47. McLaughlin VV, Genthner DE, Panella MM, et al:
Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 1998; 338: 273-7.
48. Badesch DB, Tapson VF, McGoon MD, et al: Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000; 132: 425-34.
49. McLaughlin VV, Shillington A, Rich S: Survival in primary pulmonary hypertension: the impact of epoprostenol therapy.
Circulation 2002; 106: 1477-82.
50. Yung D, Widlitz AC, Rosenzweig EB, et al: Outcome in Children with idiopathic pulmonary hypertension. Circulation 2004; 110: 660-5.
51. Sitbon O, Humbert M, Nunes H, et al: Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002; 40: 780-8.
52. Saji T, Ozawa Y, Ishikita T, et al: Short-term hemodynamic effect of a new oral PGI2 analogue, beraprost, in primary and secondary pulmonary hypertension. Am J Cardiol 1996; 78:
244-7.
53. Nagaya N, Uematsu M, Okano Y, et al: Effect of orally active prostacyclin analogue on survival of outpatients with primary pulmonary hypertension.J Am Coll Cardiol 1999; 34:
1188-92.
54. Galie N, Humbert M, Vachiery JL, et al: Arterial Pulmonary Hypertension and Beraprost European (ALPHABET) Study Group: Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol 2002; 39: 1496-502.
55. Barst RJ, McGoon M, McLaughlin V, et al: Beraprost therapy for pulmonary arterial hypertension. Beraprost Study Group: J Am Coll Cardiol 2003; 41: 2119-25.
56. Channick RN, Simonneau G, Sitbon O, et al: Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001; 358: 1119-23.
57. Rubin LJ, Badesch DB, Barst RJ, et al: Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346:
896-903.
58. McLaughlin VV, Sitbon O, Badesch DB, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J. 2005; 25: 244-9.
59. Sitbon O, McLaughlin VV, Badesch DB, et al. Survival in
patients with class III idiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol. Thorax. 2005; 60: 1025-30.
60. Barst RJ, Ivy D, Dingemanse J, et al: Pharmakokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther 2003;
73: 372-82.
61. 佐地 勉:肺高血圧の治療,現況と展望―Sildenafil(PDE 5阻害薬)とET-1阻害薬によるED(Endothelial Dysfunction)
への治療効果.Annual Review循環器2004 中外医学社 2004 p216-221
62. Michelakis E, Tymchak W, Lien D, et al: Oral sildenafil is an effective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension: comparison with inhaled nitric oxide. Circulation 2002; 105: 2398-403.
63. Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med.
2005; 353: 2148-57
64. Humpl T, Reyes JT, Holtby H, et al: Beneficial effect of oral Sildenafil therapy on childhood pulmonary arterial hypertension. Circulation 2005; 111: 3274-80.
65. 佐地 勉:クエン酸Sildenafilの薬理学的特徴と主要な エビデンス 日本小児循環器学会雑誌 2005; 21: 510-21.
66. Humbert M, Barst RJ, Robbins IM, et al: Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24: 353-9.
67. Doyle RL, McCrory D, Channick RN, Simonneau G, Conte J; American College of Chest Physicians. Surgical treatments/
interventions for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126:
63S-71S.
68. Glanville AR, Burke CM, Theodore J, et al: Primary pulmonary hypertension. Length of survival in patients referred for heart-lung transplantation.
69. 伊達洋至,青江基,清水信義,他:米国で肺移植を受け た日 本 人5症 例の検 討.日 本 移 植 学 会 雑 誌 1998; 33:
189-92.
70. Pasque MK, Trulock EP, Cooper JD, et al: Single lung transplantation for pulmonary hypertension. Single institution experience in 34 patients. Circulation 1995; 92: 2252-8.
71. Starnes VA, Barr ML, Schenkel FA, et al: Experience with living-donor lobar transplantation for indications other than cystic fibrosis. J Thorac Cardiovasc Surg 1997; 114: 917-21.
72. Bando K, Armitage JM, Paradis IL, et al: Indications for and results of single, bilateral, and heart-lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg 1994;
108: 1056-65.
73. Maurer JR, Frost AE, Estenne M, et al: International guidelines for the selection of lung transplant candidates. The International Society for Heart and Lung Transplantation, the American Thoracic Society, the American Society of Transplant Physicians, the European Respiratory Society.
Transplantation 1998; 66: 951-6.
74. Raine J, Hislop AA, Redington AN: Fatal persistent pulmonary hypertension presenting late in the neonatal period. Arch Dis Child 1991; 66: 398-402.
75. Weiss BM, Zemp L, Seifert B, et al: Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol 1998; 31:1650-7.
76. Elkayam U, Dave R, Bokhari SWH: Primary Pulmonary Hypertension and Pregnancy. In Elkayam U, Gleicher N, eds.
Cardiac Problem in Pregnancy. New York, NY: Wiley-Liss, 1998; 183-90.
77. Groves B, Brundage B, Elliott C, et al: Pulmonary hypertension associated with hepatic cirrhosis. In Fishman AP, ed. The pulmonary circulation: normal and abnormal.
Philadelphia, PA: Univercity of Pennsylvania, 1990; 359-369.
78. 鳥飼勝隆,深谷修作,国枝武義,他:混合性結合組織病 における肺高血圧症の合併率調査 厚生省特定疾患皮膚・
結合織疾患調査研究班混合性結合組織病分科会平成10年 度研究報告書,1999;24-27.
79. 吉田俊治,深谷修作:膠原病性肺高血圧症の頻度と病態 の解析 厚生労働科学研究費補助金免疫アレルギー疾患予 防・治療研究事業全身性自己免疫疾患における難治性病態 の診断と治療法に関する研究平成15年度総括・分担研究 報告書,2004;40-43.
80. 西間木友衛,青塚新一,国枝武義,他:混合性結合組織 病の肺 高 血 圧 症 診 断の手 引き リ ウ マ チ,1991;31:
159-166.
81. 鳥飼勝隆,深谷修作,松本美富士,他:肺高血圧症を合 併した混合性結合組織病患者の予後調査 厚生省特定疾患 皮膚・結合組織疾患調査研究班混合性結合組織病分科会平 成9年度研究報告書,1998;22-25.
82. Rich S, editor. Primary Pulmonary Hypertension:
Executive Summary from the World Symposium - Primary Pulmonary Hypertension 1998. Available from World Health Organization via the Internet (http://www.who.int/ncd/cvd/
pph.html)
83. 吉田俊治:肺高血圧症,全身性自己免疫疾患における難 治性病態の診療ガイドライン 編集,厚生労働科学研究費 免疫アレルギー疾患予防・治療研究事業全身性自己免疫疾 患における難治性病態の診断と治療法に関する研究(主任 研究者:三森経世),厚生労働省厚生労働科学研究費免疫 アレルギー疾患予防・治療研究事業全身性自己免疫疾患に おける難治性病態の診断と治療法に関する研究班,京都,
2005;38-43.
84. 鳥飼勝隆,片山雅夫,大島久二,他:膠原病合併肺高血 圧症の治療に関する研究 厚生省特定疾患調査研究重点研 究事業特定疾患に伴う肺高血圧症の発症機序の解明と内科 的治療指針確立を目指す診療科横断的研究平成11年度研 究報告書,200;169-172.
85. 吉田俊治,近藤啓文,高崎芳成,他:肺高血圧症を合併 した膠原病患者の治療状況に関する検討 厚生省特定疾患 対策研究事業混合性結合組織病に関する研究班混合性結合 組織病の病態,治療と抗U1RNP抗体に関する研究平成11 年度研究報告書,2000;15-17.
86. 吉田俊治,深谷修作:膠原病性肺高血圧症の自然経過に
関する研究 平成16年度厚生労働科学研究費補助金免疫 アレルギー疾患予防・治療研究事業研究報告書第1分冊,
2005;304-307.
87. 門間和夫,他:成人先天性心疾患診療ガイドライン.
Jap Circ J, 2000; 64: 1167-1204, suppl IV.
88. Canadian Consensus conference on adult congenital heart disease, Can J Cardiol, 1996; 14: 395-452.
89. 八巻重雄:臨床家のための肺血管病変肺生検診断,メデ イカルレビュー,東京,2001.
90. 丹羽公一郎:成人期Eisenmenger症候群の管理治療につ いての新知見,日小循誌,2000; 16: 830-41.
91. Heath D, Edwards JE: The Pathology of hypertensive pulmonary vascular disease Circulation 1958; 18: 533-47.
92. Rosenzweig EB: Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects.
Circulation 1999; 99: 1858-65.
93. Brickner ME, Hillis LD, Lange RA: Congenital Heart Disease in adults. New Engl J Med, 2000; 342: 334-42.
94. 門間和夫:Eisenmenger症候群,臨床発達心臓病学,改 訂2版,高尾篤良,門間和夫,中澤 誠,中西敏雄,編集,
中外医学社,東京,2001; p826-30.
95. Trulock EP: Lung transplantation. Am J Respir Crit Care Med, 1997; 155: 789-818.
96. F r a n c o K : L u n g t r a n s p l a n t a t i o n f o r p u l m o n a r y hypertension. In 1Pediatric Cardiopulmonary transplantaion.
1997, Futura, New York, p303-18.
97. Perloff RS, Koos: Pregnancy and congenital heart disease, In Perloff JK, Child JS, editors, Congenital Heart Disease in Adults, 1998, 2nd ed. Philadelphia; Saunders; p144-64.
98. Vongpatanasin W, Brickner E, Hillis D, et al: The Eisenmenger syndrome in Adults. Ann Intern Med. 1998;
128: 745-755.
99. 慢性心不全治療ガイドライン.Jpn Circ J 2000; 64,Suppl IV, 1023-79.
100. Pamboukian SV, Carere RG, Webb JG, et al: The use of milrinone in pre-transplant assessment of patients with congestive heart failure and pulmonary hypertension. J Heart Lung Transplant 1999; 18: 367-71
101. Califf RM, Adams KF, McKenna, et al: A randomized controlled trial of epoprostenol therapy for severe congestive heart failure: the Flolan International Randomized Survival Trial (FIRST). Am Heart J 1997; 134: 44-54.
102. Matsumoto A, Momomura S, Sugiura S, et al: Effect of inhaled nitric oxide on gas exchange in patients with congestive heart failure. A randomized, controlled trial. Ann Intern Med 1999 Jan 5; 130(1): 40-4.
103. Loh E, Stamler JS, Hare JM, et al: Cardiovascular effects of inhaled nitric oxide in patients with left ventricular dysfunction. circulation 1994; 90: 2780-5.
104. Alfonso F, Macaya C, Hernandez R, et al: Percutaneous mitral valvuloplasty with severe pulmonary artery hypertension. Am J Cardiol 1993; 72: 325-30.
105. Fawzy ME, Mimish L, Sivanandam V, et al: Immediate and long-term effect of mitral balloon valvotomy on severe
pulmonary hypertension in patients with mitral stenosis. Am Heart J 1996; 131: 89-93.
106. Zenner JC, Hancock EW, Shumway NE, et al: Regression of extreme pulmonary hypertension after mitral valve surgery.
Am. J Cardiol 1972; 30: 820-6.
107. Ward C, Hancock BW: Extreme Pulmonary hypertension caused by mitral valve disease. Natural history and results of surgery. Br Heart 1975; 37: 74-8.
108. Vincens JJ, Temizer D, Post JR, et al: Long-term outcome of cardiac surgery in patients with mitral stenosis and severe pulmonary hypertension. Circulation 1995; 92: II 137-42.
109. Yokokura Y, Imamura H, Sakamoto T, et al.: 22 cases of primary cardiac tumors. Jpn Annals Thorac Sur 1981; 1:
108-18.
110. Nakao T, Mayumi H, Morita S, et al: Left atrial myxoma associated with severe congestive heart failure, pulmonary hypertension, and multiple organ insufficiency. Jpn Heart J 1995: 36: 829-34.
111. Abramson SV, Burke JF, Kelly JJ Jr, et al: Pulmonary hypertension predicts mortality and morbidity in patients with dilated cardiomyopathy. Ann Intern Med 1992; 116: 888-95.
112. Levine TB, Levine AB, Goldberg AD, et al: Impact of medical therapy on pulmonary hypertension in patients with congestive heart failure awaiting cardiac transplantation. Am J Cardiol. 1996; 78: 440-3.
113. Kirklin JK, Naftel DC, Kirklin JW, et al: Pulmonary vascular resistance and the risk of heart transplantation. J Heart Transplant 1988; 7(:331-672)
114. Costard-Jackle A, Fowler MB: Influence of preoperative pulmonary artery pressure on mortality after heart transplantation: testing of potential reversibility of pulmonary hypertension with nitroprusside is useful in defining a high risk group. J Am Coll Cardiol 1992; 19: 48-54.
115. Butler J, Chomsky DB, Wilson JR: Pulmonary hypertension and exercise intolerance in patients with heart failure. J Am Coll Cardiol 1999; 34: 1802-6.
116. Peacock AJ: pulmonary circulation-a handbook for clinicians. London: Chapman & Hall, 1996. pp159.
117. COPD(慢性閉塞性肺疾患)診断と治療のためのガイド ライン(第2版) 日本呼吸器学会COPDガイドライン第2 版 作 成 委 員 会 編.社 団 法 人 日 本 呼 吸 器 学 会,2004.
pp1-50.
118. 平賀俊尚,岸不尽弥,川上義和,他:慢性呼吸不全患者
における低酸素血症の肺循環に及ぼす影響─動脈血酸素分 圧・基礎疾患別検討─.厚生省特定疾患呼吸不全調査研究 班(編):昭和62年度研究報告書.pp135-40.
119. World Health Organization: Chronic cor pulmonale-a report of the expert committee. Circulation 1963; 27: 594-615.
120. Burrows B, Kettel LJ, Niden AH, et al: Patterns of cardiovascular dysfunction in chronic obstructive lung disease. N Eng J Med 1972; 286: 912-8.
121.F l e t c h e r E C a n d L e v e n D C : C a r d i o p u l m o n a r y hemodynamics during sleep in subjects with chronic obstructive pulmonary disease: the effect of short-and