国际呼吸杂志
國際呼吸雜誌
국제호흡잡지
INTERNATIONAL JOURNAL OF RESPIRATION
2014年
12期
905-909
,共5页
肺纤维化%肺气肿%肺纤维化合并肺气肿综合征%吸烟%肺功能
肺纖維化%肺氣腫%肺纖維化閤併肺氣腫綜閤徵%吸煙%肺功能
폐섬유화%폐기종%폐섬유화합병폐기종종합정%흡연%폐공능
Pulmonary fibrosis%Emphysema%Combined pulmonary fibrosis and emphysema%Smoking%Pulmonary function
目的 分析、总结肺纤维化合并肺气肿(CPFE)综合征患者的一般资料、临床表现及体征、肺功能、肺动脉压力、肺癌标志物、CT表现及预后等特点.方法 对我院2010-2012年诊断的7例CPFE综合征的患者一般资料、临床表现及体征、肺功能、肺动脉压力、肺癌标志物及CT表现结合临床文献进行分析、归纳、总结.结果 6例为男性,1例为女性,年龄62～84岁,中位年龄77.4岁;6例有吸烟史,其中有1例从事酒渣运输工作40余年.全部病例均有活动后呼吸困难,双下肺均可闻及吸气相velcro哕音.肺功能结果[中位数(范围)]:FEV1％pred平均为73％(39％～98％),FVC％ pred平均为73％(54％～101％),FEV1/FVC平均为81％(61％～96％),肺活量％pred平均为79％(57％～86％),肺总量％ pred平均为85％(53％～110％),残气量/肺总量平均为41％(21％～58％),DLCO％ pred平均为46％(20％～58％),DLCO/肺泡气量平均为53％ (19％～98％).有4例有肺动脉高压,平均为45 mmHg(32～69 mmHg).肺癌标志物中有2例出现神经元特异性烯醇化酶升高.胸部CT可见双上肺肺气肿及双下肺磨玻璃样、网格状、蜂窝样及牵拉性支气管扩张的肺纤维化表现.结论 CPFE综合征患者好发于男性有吸烟史的患者,且死亡率与之相关.临床特征包括呼吸困难、低氧血症、肺动脉高压.肺功能表现为肺容积相对正常而弥散能力显著下降.高分辨率CT的特点为同时存在上肺野肺气肿和下肺野纤维化.其常见并发症为肺动脉高压、ALI、肺癌.目前该病的发病机制、治疗及预后等尚未明确,待进一步探讨.
目的 分析、總結肺纖維化閤併肺氣腫(CPFE)綜閤徵患者的一般資料、臨床錶現及體徵、肺功能、肺動脈壓力、肺癌標誌物、CT錶現及預後等特點.方法 對我院2010-2012年診斷的7例CPFE綜閤徵的患者一般資料、臨床錶現及體徵、肺功能、肺動脈壓力、肺癌標誌物及CT錶現結閤臨床文獻進行分析、歸納、總結.結果 6例為男性,1例為女性,年齡62～84歲,中位年齡77.4歲;6例有吸煙史,其中有1例從事酒渣運輸工作40餘年.全部病例均有活動後呼吸睏難,雙下肺均可聞及吸氣相velcro噦音.肺功能結果[中位數(範圍)]:FEV1％pred平均為73％(39％～98％),FVC％ pred平均為73％(54％～101％),FEV1/FVC平均為81％(61％～96％),肺活量％pred平均為79％(57％～86％),肺總量％ pred平均為85％(53％～110％),殘氣量/肺總量平均為41％(21％～58％),DLCO％ pred平均為46％(20％～58％),DLCO/肺泡氣量平均為53％ (19％～98％).有4例有肺動脈高壓,平均為45 mmHg(32～69 mmHg).肺癌標誌物中有2例齣現神經元特異性烯醇化酶升高.胸部CT可見雙上肺肺氣腫及雙下肺磨玻璃樣、網格狀、蜂窩樣及牽拉性支氣管擴張的肺纖維化錶現.結論 CPFE綜閤徵患者好髮于男性有吸煙史的患者,且死亡率與之相關.臨床特徵包括呼吸睏難、低氧血癥、肺動脈高壓.肺功能錶現為肺容積相對正常而瀰散能力顯著下降.高分辨率CT的特點為同時存在上肺野肺氣腫和下肺野纖維化.其常見併髮癥為肺動脈高壓、ALI、肺癌.目前該病的髮病機製、治療及預後等尚未明確,待進一步探討.
목적 분석、총결폐섬유화합병폐기종(CPFE)종합정환자적일반자료、림상표현급체정、폐공능、폐동맥압력、폐암표지물、CT표현급예후등특점.방법 대아원2010-2012년진단적7례CPFE종합정적환자일반자료、림상표현급체정、폐공능、폐동맥압력、폐암표지물급CT표현결합림상문헌진행분석、귀납、총결.결과 6례위남성,1례위녀성,년령62～84세,중위년령77.4세;6례유흡연사,기중유1례종사주사운수공작40여년.전부병례균유활동후호흡곤난,쌍하폐균가문급흡기상velcro홰음.폐공능결과[중위수(범위)]:FEV1％pred평균위73％(39％～98％),FVC％ pred평균위73％(54％～101％),FEV1/FVC평균위81％(61％～96％),폐활량％pred평균위79％(57％～86％),폐총량％ pred평균위85％(53％～110％),잔기량/폐총량평균위41％(21％～58％),DLCO％ pred평균위46％(20％～58％),DLCO/폐포기량평균위53％ (19％～98％).유4례유폐동맥고압,평균위45 mmHg(32～69 mmHg).폐암표지물중유2례출현신경원특이성희순화매승고.흉부CT가견쌍상폐폐기종급쌍하폐마파리양、망격상、봉와양급견랍성지기관확장적폐섬유화표현.결론 CPFE종합정환자호발우남성유흡연사적환자,차사망솔여지상관.림상특정포괄호흡곤난、저양혈증、폐동맥고압.폐공능표현위폐용적상대정상이미산능력현저하강.고분변솔CT적특점위동시존재상폐야폐기종화하폐야섬유화.기상견병발증위폐동맥고압、ALI、폐암.목전해병적발병궤제、치료급예후등상미명학,대진일보탐토.
Objective To investigate the general,clinical,pulmonary function,pulmonary arterial pressure,the biomarker of lung cancer,radiological features,and prognosis of patients with combined pulmonary fibrosis and emphysema (CPFE) syndrome.Methods The general,clinical,pulmonary function,pulmonary arterial pressure,the biomarker of lung cancer,radiological features,and prognosis of seven patients diagnosed with CPFE syndrome were retrospectively analyzed.Results The subject consisted of seven patients (six males and one female),aged from 62 to 84 years old,with the average of 77.4 years old.Six of them were smokers,the other one had been working as transplanting wine slag for more than 40 years.All patients had a dyspnea on exertion and basal crackles could be heard at the lower fields of their both sides of lung.The pulmonary function demonstrated that FEV1％ pred was 73 ％ (39 ％-98％),FVC％pred was 73％(54％-101％),FEV1/FVC％ was 81％(61％-96％),VC％pred was 79％ (57％-86％),TLC％pred was 85％ (53％-110％),RV/TLC％ was 41％ (21％-58％).DLCO％ pred was 46％(20％-58％),DLCO/VA was 53％ (19％-98％).Four cases had pulmonary hypertension with an average of 45 mmHg.The level of neuron specic enolase increased in two cases.High resolution computerized tomography showed emphysema predominantly at the upper lobes and reticular opacities,honeycombing and traction bronchiectasis in the lower lobes.Conclusions The CPFE syndrome typically occurs in male smokers,and the mortality is associated with this.Clinical characteristics include the presence of dyspnea,pulmonary hypertension,and hypoxemia.Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange.Radiographic characteristics include the presence of upper-lobe emphysema and lower-lobe pulmonary fibrosis.The complication of this syndrome frequently is pulmonary hypertension,acute lung injury,and lung cancer.Further studies are needed to explore the pathogenesis,treatment,and prognosis of CPFE.
