支气管扩张症辨证模式初探

目的:探讨支气管扩张症的中医辨证分型规律及证候特点.方法:通过对563例支气管扩张症的临床流行病学调查,采集以症状、体征、舌、脉及相关理化检测为变量的基本信息,以频数分析、聚类分析、方差分析等方法,提炼支气管扩张症的证候分布规律及证候特点.结果:临床上支气管扩张症多见4种证候类型,分别为痰热壅肺证(45.65%)、肝火犯肺证(24.51%)、肺脾气虚证(22.38%)、气阴两虚证(7.46%).结论:较大样本的临床流行病学调查为研究支气管扩张症辨证分型规律提供了科学依据,并可以通过主症判别分析法建立证候识别模式,为临床实践提供依据.     

Diffuse panbronchiolitis: Case report and review of the literature

Diffuse panbronchiolitis is an uncommon disease of unknown aetiology mainly occurring in Asian races. Treatment with erythromycin acting through unclarif ied mechanisms is promising and may improve the poor prognosis currently associated with the disease. A case is presented illustrating how the imaging features of the disease, particularly on high-resolution computed tomography, may be used to support a diagnosis suggested on clinical grounds.     

支气管扩张症中神经内分泌免疫网络及肥大细胞的变化

目的：探讨支气管扩张症中神经内分泌免疫网络的异常和肥大细胞（MC）在该病发病中的作用及关系。方法：应用组织化学、免疫组化、组织化学与免疫组化结合的方法和形态计量学方法进行观测。结果：支气管扩张症中，支气管上皮蛙皮素（Bombesin）阳性细胞、固有膜S-100蛋白和神经特异性烯醇化酶（NSE）阳性神经纤维、IgE阳性细胞、MC和IgE阳性MC均显著增多，且在支气管相关淋巴组织（BALT）增生的区域上述肺内分泌细胞、神经纤维和IgE阳性细胞增多尤为显著，S-100蛋白和NSE阳性神经纤维分布于弥散淋巴组织和BALT中，MC与S-100蛋白阳性神经纤维紧密接触．MC表面有IgE阳性环状带，MC和IgE阳性细胞出现在支气管上皮间和肺泡壁。结论：支气管扩张症的发病与局部神经内分泌免疫网络异常有关；MC可能作为感受器、分泌细胞或靶细胞参与神经内分泌免疫网络，在支气管扩张症的发病中起重要作用。     

强力稀化粘素治疗稳定期支气管扩张症的疗效观察

目的 观察强力稀化粘素对稳定期支气管扩张症的疗效.方法 对35例稳定期支气管扩张患者,强力稀化粘素300 mg每日3次口服治疗,疗程6个月.观察并记录支扩急性加重的频率、临床表现并与治疗前6个月进行比较.结果 35例患者完成了6个月的治疗和随访.6个月来患者的平均恶化次数从治疗前4.2次减少到1.3次；痰量从治疗前的平均0.066 L/d减少至0.034 L/d.结论 长期强力稀化粘素治疗能减少支扩恶化次数,改善临床症状.     

Total thoracoscopic combined lingulectomy and pericardial cystectomy

Thoracoscopic anatomic segmentectomy is usually more complex than lobectomy. This video shows a 58-year-old female who suffered from limited bronchiectasis of the lingular segment of the left upper lung and a pericardial cyst on the same side. Both of these benign thoracic diseases can cause pleural adhesions. Repeated chronic inflammation contributes to hypervascularity and lymph node enlargement, making surgery more difficult. We used single-direction thoracoscopic segmentectomy via a three-port approach and successfully removed the lingula and pericardial cyst.     

Recurrent polymyositis triggered by bronchiectasis‐associated infections

Polymyositis is a cell‐mediated autoimmune disease and various systemic infections can trigger it. However, there have been only rare reports of recurrent polymyositis triggered by systemic infections. We report the case of a 69‐year‐old woman with recurrent polymyositis triggered by bronchiectasis‐associated infections. This case indicates that recurrent polymyositis triggered by systemic infections suggests a non‐specific autoimmune antigenic response of polymyositis.     

Bronchiectasis and deteriorating lung function in agammaglobulinaemia despite immunoglobulin replacement therapy

Immunoglobulin replacement therapy enhances survival and reduces infection risk in patients with agammaglobulinaemia. We hypothesized that despite regular immunoglobulin therapy, some patients will experience ongoing respiratory infections and develop progressive bronchiectasis with deteriorating lung function. One hundred and thirty‐nine (70%) of 199 patients aged 1–80 years from nine cities in the United Kingdom with agammaglobulinaemia currently listed on the UK Primary Immune Deficiency (UKPID) registry were recruited into this retrospective case study and their clinical and laboratory features analysed; 94% were male, 78% of whom had Bruton tyrosine kinase (BTK) gene mutations. All patients were on immunoglobulin replacement therapy and 52% had commenced therapy by the time they were 2 years old. Sixty per cent were also taking prophylactic oral antibiotics; 56% of patients had radiological evidence of bronchiectasis, which developed between the ages of 7 and 45 years. Multivariate analysis showed that three factors were associated significantly with bronchiectasis: reaching 18 years old [relative risk (RR) = 14·2, 95% confidence interval (CI) = 2·7–74·6], history of pneumonia (RR = 3·9, 95% CI = 1·1–13·8) and intravenous immunoglobulin (IVIG) rather than subcutaneous immunoglobulin (SCIG) = (RR = 3·5, 95% CI = 1·2–10·1), while starting immunoglobulin replacement after reaching 2 years of age, gender and recent serum IgG concentration were not associated significantly. Independent of age, patients with bronchiectasis had significantly poorer lung function [predicted forced expiratory volume in 1 s 74% (50–91)] than those without this complication [92% (84–101)] (P < 0·001). We conclude that despite immunoglobulin replacement therapy, many patients with agammaglobulinaemia can develop chronic lung disease and progressive impairment of lung function.     

Bronchiectasis in indigenous and non‐indigenous residents of Australia and New Zealand

Background and objective

Bronchiectasis not associated with cystic fibrosis is an increasingly recognized chronic lung disease. In Oceania, indigenous populations experience a disproportionately high burden of disease. We aimed to describe the natural history of bronchiectasis and identify risk factors associated with premature mortality within a cohort of Aboriginal Australians, New Zealand Māori and Pacific Islanders, and non‐indigenous Australians and New Zealanders.

Methods

This was a retrospective cohort study of bronchiectasis patients aged >15 years at three hospitals: Alice Springs Hospital and Monash Medical Centre in Australia, and Middlemore Hospital in New Zealand. Data included demographics, ethnicity, sputum microbiology, radiology, spirometry, hospitalization and survival over 5 years of follow‐up.

Results

Aboriginal Australians were significantly younger and died at a significantly younger age than other groups. Age‐ and sex‐adjusted all‐cause mortality was higher for Aboriginal Australians (hazard ratio (HR): 3.9), and respiratory‐related mortality was higher for both Aboriginal Australians (HR: 4.3) and Māori and Pacific Islander people (HR: 1.7). Hospitalization was common: Aboriginal Australians had 2.9 admissions/person‐year and 16.9 days in hospital/person‐year. Despite Aboriginal Australians having poorer prognosis, calculation of the FACED score suggested milder disease in this group. Sputum microbiology varied with Aspergillus fumigatus more often isolated from non‐indigenous patients. Airflow obstruction was common (66.9%) but not invariable.

Conclusions

Bronchiectasis is not one disease. It has a significant impact on healthcare utilization and survival. Differences between populations are likely to relate to differing aetiologies and understanding the drivers of bronchiectasis in disadvantaged populations will be key.

     

经纤维支气管镜局部冲洗治疗支气管扩张症53例报告

目的探讨经纤维支气管镜（以下简称纤支镜）局部灌洗注药治疗支气管扩张症的疗效。方法53例支气管扩张病人除常规治疗外，还采用纤维支气管镜反复气道灌洗和注药，在纤维支气管镜治疗前后常规监测患者临床情况、通气换气功能及血气指标。结果灌洗注药后患者体温迅速下降，外周血白细胞、痰液量明显减少，X线胸片明显改善。灌洗后血气指标（PaO2，SaO2及PaO2／FiO2）改善；肺通气功能检测（VC、FEVI、FEVI％、VS0、V25及MVV）明显改善（P〈0．05）。结论经纤维支气管镜肺泡灌洗和注药能有效清除痰痂和气道分泌物，解除支气管的阻塞，改善患者通气换气功能，是一种安全、简便实用的治疗支气管扩张的有效手段。