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。
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.
