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作者：Laurie Barclay, MD  
出處：WebMD醫學新聞

　　April 13, 2010 — 根據線上發表於3月18日美國呼吸暨重症照護醫學期刊(American Journal of Respiratory and Critical Care Medicine)的一篇聲明，適當處置囊狀纖維化(cystic fibrosis，CF)之肺部併發症，可以改善此病症患者的存活期間與生活品質。囊狀纖維化基金會組成的委員會發展這些指引，提出對咳血和氣胸病患的治療建議。
　　
　　南卡羅來納醫學大學的Patrick A. Flume醫師以及肺部治療委員會臨床實務指引的同僚寫道，CF是一個複雜的遺傳疾病、影響許多器官，其中肺部疾病佔約85%的死亡率。肺部疾病的自然史包括早期與持續感染，嚴重的發炎反應，呼吸道阻塞惡化，最後導致呼吸衰竭。當呼吸道疾病惡化時，呼吸道併發症如咳血和氣胸的可能性也增加。
　　
　　CF案例中，呼吸道表面液體脫水以及黏液纖毛清除能力不佳，造成難以清除肺部病源，導致慢性肺部感染與發炎，需要更密集的治療，以防止肺部斷斷續續惡化，或因感染與阻塞而急性惡化，CF病患通常有咳血和氣胸之併發症。
　　
　　因為現有的資料不足以發展具有證據基礎的建議，該委員會使用德菲法(Delphi technique)來形成專家小組的共識，並用以發展咳血和氣胸病患的明確治療建議。
　　
　　該小組發展的特定建議如下：
　　* 輕微咳血(≧ 5 mL)的病患應聯繫他們的健康照護者
　　* 少量咳血(< 5 mL)的病患在初次發生或持續發生時應聯繫他們的健康照護者
　　* 少量咳血病患可能不需要住院
　　* 大量咳血(> 240 mL)病患一定要住院
　　* 輕微咳血(≧ 5 mL)病患建議使用抗生素治療
　　* 輕微咳血(≧ 5 mL)病患應停止使用非類固醇抗發炎藥物
　　* 支氣管動脈栓塞術建議用於臨床不穩定之大量咳血病患
　　* 對於大量咳血病患，在支氣管動脈栓塞術之前不可以進行支氣管切片。
　　* 少量咳血病患不應停止雙陽壓呼吸道正壓呼吸(BiPAP)
　　* 大量咳血病患應停止BiPAP
　　* 大量咳血病患，肺切除是最後的治療選項。
　　* 少量咳血病患不應停止呼吸道呼吸道清除治療
　　* 大量咳血病患應停止所有的呼吸道清除治療
　　* 少量咳血病患不應停止霧化器治療
　　* 大量咳血病患應停止使用霧化高張生理食鹽水
　　* 大範圍氣胸病患(> 3公分)一定要住院
　　* 臨床穩定的小範圍氣胸病患視同門診病患密切觀察
　　* 大範圍氣胸病患應接受胸管放置
　　* 臨床不穩定的小範圍氣胸病患應放置胸管
　　* 對於初次氣胸病患，不建議用肋膜黏連術預防復發。
　　* 復發大範圍氣胸的病患應進行肋膜黏連術，以預防復發。
　　* 對於進行肋膜黏連術的氣胸病患，手術肋膜黏連術優於肋膜黏連術。
　　* 只要持續有氣胸，即應停止BiPAP。
　　* 氣胸緩解之後2週，氣胸病患不應搭乘飛機、舉重物(> 5磅)或進行肺功能量計。
　　* 氣胸病患不應進行吐氣正壓或肺內叩擊呼吸器治療
　　* 氣胸病患不應停用霧化器治療
　　
　　指引作者們結論表示，CF病患咳血和氣胸的這些處置建議，適用於大多數病患，不過可以適當調整，以符合病患、家屬、健康照護者的特定需要，希望這個版本的指引可以促進適當運用這些治療方式，以改善並延長所有囊狀纖維化病患的生命。
　　
　　Am J Respir Crit Care Med. 線上發表於2010年3月8日。


Guidelines Address Pulmonary Complications of Cystic Fibrosis

By Laurie Barclay, MD
Medscape Medical News

April 13, 2010 — Optimal management of the pulmonary complications of cystic fibrosis (CF) can improve survival duration and quality of life in patients with this disease, according to a statement published online March 18 in the American Journal of Respiratory and Critical Care Medicine. These guidelines were developed by a committee formed by the Cystic Fibrosis Foundation to make recommendations for treatment of patients with hemoptysis and pneumothorax.

"...CF is a complex genetic disease affecting many organs, although 85% of the mortality results from lung disease," write Patrick A. Flume, MD, from the Medical University of South Carolina in Charleston, and colleagues from the Clinical Practice Guidelines for Pulmonary Therapies Committee. "The natural history of the lung disease consists of early and persistent infection, an exaggerated inflammatory response, and progressive airways obstruction, ultimately resulting in respiratory failure. As airways disease worsens, there is an increased likelihood of respiratory complications, such as hemoptysis and pneumothorax."

In CF, dehydration of airway surface liquid and impaired mucociliary clearance cause difficulty clearing pathogens from the lung, resulting in chronic pulmonary infections and inflammation. More intensive treatment is needed for intermittent pulmonary exacerbations, or acute worsening of infection and obstruction. Patients with CF often have complications of hemoptysis and pneumothorax.

Because existing data are insufficient to develop evidence-based recommendations, the committee used the Delphi technique to formalize the expert panel's consensus process and to develop explicit care recommendations for the treatment of hemoptysis and pneumothorax.

Specific recommendations developed by the panel are as follows:

Patients with at least mild hemoptysis (? 5 mL) should contact their healthcare provider.
Patients with scant hemoptysis (< 5 mL) that is persistent or is the first-ever episode should contact their healthcare provider.
Hospital admission may not be required for patients with scant hemoptysis.
Hospital admission is always required for patients with massive hemoptysis (> 240 mL).
Antibiotic treatment is recommended for patients with at least mild (? 5 mL) hemoptysis.
Patients with at least mild (? 5 mL) hemoptysis should discontinue use of nonsteroidal anti-inflammatory drugs.
Bronchial artery embolization is recommended for clinically unstable patients with massive hemoptysis.
Bronchoscopy should not be performed before bronchial artery embolization in patients with massive hemoptysis.
Bilevel positive airway pressure (BiPAP) should not be withheld from patients with scant hemoptysis.
For patients with massive hemoptysis, BiPAP should be withheld.
For patients with massive hemoptysis, lung resection should be performed only as a final therapeutic option.
Airway clearance therapies should not be discontinued in patients with scant hemoptysis.
Patients with massive hemoptysis should discontinue all airway clearance therapies.
Patients with scant hemoptysis should not discontinue aerosol therapies.
Patients with massive hemoptysis should discontinue use of aerosolized hypertonic saline.
Patients with a large pneumothorax (> 3 cm) should always be hospitalized.
Clinically stable patients with a small pneumothorax may be closely observed as outpatients.
Patients with a large pneumothorax should undergo chest tube placement.
Clinically unstable patients with a small pneumothorax should have a chest tube placed.
For patients with a first pneumothorax, pleurodesis to prevent recurrence is not recommended.
Patients with a recurrent large pneumothorax should undergo pleurodesis to prevent recurrence.
Surgical vs chemical pleurodesis is the preferred method for patients with a pneumothorax who is undergoing pleurodesis.
As long as pneumothorax is present, BiPAP should be withheld.
For 2 weeks after the pneumothorax has resolved, patients with pneumothorax should not fly on an airplane, lift weights (> 5 pounds), or perform spirometry.
Patients with pneumothorax should not undergo positive expiratory pressure or intrapulmonary percussive ventilation therapies.
Patients with pneumothorax should not discontinue aerosol therapies.
"These recommendations for the management of the CF patient with hemoptysis and pneumothorax are designed for general use in most individuals but should be adapted to meet specific needs as determined by the individuals, their families, and their health care providers," the guidelines authors conclude. "It is hoped that the guidelines provided in this manuscript will facilitate the appropriate application of these treatments to improve and extend the lives of all individuals with cystic fibrosis."

Am J Respir Crit Care Med. Published online March 18, 2010.