肝肺綜合征Ⅱ 型肺血管異常合并血胸病例報道并文獻復習_《中國呼吸與危重監護雜志》

作者：

金建敏 ¹ , 孫永昌 ¹ , 陳東寧 ¹ , 劉曉芳 ¹ , 梁熙虹 ²

1 首都醫科大學附屬北京同仁醫院呼吸內科（北京 100730）;;
2 首都醫科大學附屬北京同仁醫院放射科（北京 100730）通訊作者: 孫永昌, E-mail: sunych@ trhos. com;

關鍵詞：

肝肺綜合征肺動靜脈畸形血胸肺動脈造影

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目的了解肝肺綜合征( HPS) Ⅱ型肺血管異常( 肺動靜脈畸形) 合并血胸的臨床特征和處理原則。方法對1 例HPS Ⅱ型肺血管異常合并反復血胸患者的臨床資料進行分析, 并結合國內外相關文獻進行復習。結果患者男性,72 歲, 以反復加重的喘憋和包裹性胸腔積液就診。先后4次胸穿, 總共抽取血樣胸液2510 mL, 胸液常規紅細胞計數在100 ×109 /L 以上, 細胞學檢查和腫瘤標記物均陰性。后經CTPA 和肺動脈造影證實為HPS Ⅱ型肺血管異常合并血胸。患者立臥位PaO2 分別為58. 3 mmHg 和66. 3 mmHg。對血管畸形行栓塞治療后, 低氧血癥改善, 血胸得到控制。總結文獻, HPS Ⅱ型肺血管異常合并血胸既往未見報道。HPS 最突出的臨床表現是呼吸困難和發紺。直立位低氧血癥或臥位呼吸是HPS 較為特異的表現。P( A-a) O2 是篩查HPS 低氧血癥的最敏感指標。HPS胸部CT 特點為被強化的團片狀密度增高影或多發小結節影, 以雙下肺野多見。肺動脈造影可對肺
血管異常予以分型。HPS肺血管病變Ⅱ型患者對吸氧反應不佳, 血管畸形栓塞治療有助于緩解癥狀,改善低氧血癥。結論 HPS Ⅱ型肺血管異常破裂是血胸的少見病因。肺血管畸形栓塞治療可有效改善低氧血癥, 控制血胸。對于具有肝臟病史者, 出現肺血管擴張和低氧血癥應警惕HPS 的存在。

引用本文： 金建敏,孫永昌,陳東寧,劉曉芳,梁熙虹. 肝肺綜合征Ⅱ 型肺血管異常合并血胸病例報道并文獻復習. 中國呼吸與危重監護雜志, 2010, 9(1): 61-65. doi: 復制

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8.	Ference BA,Shannon TM,White RI Jr,et al.Life-threatening pulmonary hemorrhage with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia.Chest,1994,106:1387-1390.
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10.	Rodríguez-Roisin R,Krowka MJ.Hepatopulmonary syndrome--a liver-induced lung vascular disorder.N Engl J Med,2008,358:2378-2387.
11.	Leung AN.Case 63:hepatopulmonary syndrome.Radiology,2003,229:64-67.
12.	McAdams HP,Erasmus J,Crockett R,et al.The hepatopulmonary syndrome: radiologic findings in 10 patients.AJR Am J Roentgenol,1996,166:1379-1385.
13.	Stratakos G,Malagari K,Broutzos E,et al.Dyspnoea and cyanosis in a cirrhotic patient.Eur Respir J,2002,19:780-783.
14.	Manson D,Traubici J,Mei-Zahav M,et al.Pulmonary nodular opacities in children with hereditary hemorrhagic telangiectasia.Pediatr Radiol,2007,37:264-268.
15.	Hirai N,Toyota N,Kakizawa H,et al.Endovascular therapy of pulmonary arteriovenous malformations: Utility of 16-detector-row computed tomography pulmonary angiography as a pre-embolization survey.J Comput Assist Tomogr,2006,30:238-243.

1. Alizadeh AH,Fatemi SR,Mirzaee V,et al.Clinical features of hepatopulmonary syndrome in cirrhotic patients.World J Gastroenterol,2006,12:1954-1956.
2. Yilmaz S,Dursum M,Canoruç F,et al.A severe (type II) hepatopulmonary syndrome in a patient with idiopathic portal hypertension and treatment with paroxetine.Neth J Med,2005,63:448-452..
3. 薛雁山,王峻,陳麥林，等.肝肺綜合征的CT表現及臨床意義.中國醫學影像學雜志，2004，12:246-248.
4. 平紅霞,姚保珍,王瑞英.肝肺綜合征一例誤診分析.臨床誤診誤治,2003,16:419．.
5. Groves AM,See TC,Appleton DS,Win T,et al.Transpleural ultrasound diagnosis of a pulmonary arteriovenous malformation.Br J Radiol,2004,77:620-622.
6. Thomas R,Christopher DJ,Chacko J,et al.Pulmonary arteriovenous malformation in a patient with tuberculosis--an association? Eur J Cardiothorac Surg,2006,30:405-407.
7. Ho V.Current concepts in the management of hepatopulmonary syndrome.Vasc Health Risk Manag,2008,4:1035-1041.
8. Ference BA,Shannon TM,White RI Jr,et al.Life-threatening pulmonary hemorrhage with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia.Chest,1994,106:1387-1390.
9. Khandaker MH,Knoll BM,Arora AS.63-year-old man with cryptogenic cirrhosis and dyspnea.Mayo Clin Proc,2008,83:580-583.
10. Rodríguez-Roisin R,Krowka MJ.Hepatopulmonary syndrome--a liver-induced lung vascular disorder.N Engl J Med,2008,358:2378-2387.
11. Leung AN.Case 63:hepatopulmonary syndrome.Radiology,2003,229:64-67.
12. McAdams HP,Erasmus J,Crockett R,et al.The hepatopulmonary syndrome: radiologic findings in 10 patients.AJR Am J Roentgenol,1996,166:1379-1385.
13. Stratakos G,Malagari K,Broutzos E,et al.Dyspnoea and cyanosis in a cirrhotic patient.Eur Respir J,2002,19:780-783.
14. Manson D,Traubici J,Mei-Zahav M,et al.Pulmonary nodular opacities in children with hereditary hemorrhagic telangiectasia.Pediatr Radiol,2007,37:264-268.
15. Hirai N,Toyota N,Kakizawa H,et al.Endovascular therapy of pulmonary arteriovenous malformations: Utility of 16-detector-row computed tomography pulmonary angiography as a pre-embolization survey.J Comput Assist Tomogr,2006,30:238-243.

《中國呼吸與危重監護雜志》

肝肺綜合征Ⅱ 型肺血管異常合并血胸病例報道并文獻復習

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《中國呼吸與危重監護雜志》

肝肺綜合征Ⅱ 型肺血管異常合并血胸病例報道并文獻復習

摘要 全文 圖表 視頻 參考文獻 施引文獻 補充材料

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