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PubMed1978

Selective IgA deficiency and Pi ZZ-antitrypsin deficiency. Association with recurrent sinopulmonary infections, emphysema, and bronchiectasis.

We describe a patient in whom selective IgA deficiency and homozygous alpha1-antitrypsin deficiency were discovered. Clinically, the patient suffered from chronic sinopulmonary infections, destructive emphysema, and bronchiectasis. The interrelation of IgA and alpha1-antitrypsin was studied. Twenty-three alpha1-antitrypsin-deficient sera were screened for IgA deficiency. None of these sera were deficient in IgA. Fifteen IgA-deficient sera were screened for alpha1-antitrypsin deficiency. In this group, three patients were found to have variant alpha1-antitrypsin phenotypes. Respiratory infections were a prominent complaint in all three of these patients, with bronchiectasis in two patients. We believe that the combination of IgA and alpha1-antitrypsin deficiencies should be considered in the evaluation of any patient with idiopathic bronchiectasis.

Bronchiectasis↗

PubMed1999

Bronchiectasis with myeloperoxidase antineutrophil cytoplasmic antibody and bactericidal/permeability-increasing protein antineutrophil cytoplasmic antibody.

A 56-year-old woman was hospitalized for recurrent hemoptysis. She had been suffering from bronchiectasis for 4 years. Pseudomonas aeruginosa was persistently detected in her sputum. Serum was positive for Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) and bactericidal/permeability-increasing protein antineutrophil cytoplasmic antibody (BPI-ANCA). She underwent lung resection. Histopathologically, the resected lung showed bronchiectasis with pulmonary fibrosis but did not show vasculitis. Her serum became negative for the ANCAs after the operation. To date, she has no recurrence of hemoptysis. We discuss this case of bronchiectasis with MPO-ANCA and BPI-ANCA and suggest a possible role for ANCAs in chronic airway infection.

Antibodies, Antineutrophil Cytoplasmic↗

PubMed1999

Bronchiectasis: the 'other' obstructive lung disease.

Bronchiectasis belongs to the family of chronic obstructive lung diseases, even though it is much less common than asthma, chronic bronchitis, or emphysema. Clinical features of these entities overlap significantly. The triad of chronic cough, sputum production, and hemoptysis always should bring bronchiectasis to mind as a possible cause. Chronic airway inflammation leads to bronchial dilation and destruction, resulting in recurrent sputum overproduction and pneumonitis. Once the diagnosis is confirmed, any potential predisposing conditions should be aggressively sought. The relapsing nature of bronchiectasis can be controlled with antibiotics, chest physiotherapy, inhaled bronchodilators, proper hydration, and good nutrition. In rare circumstances, surgical resection or bilateral lung transplantation may be the only option available for improving quality of life. Prognosis is generally good but varies with the underlying syndrome.

Adult↗

PubMed2006

Carcinoid tumour behind bronchiectasis.

This report describes a female patient with bronchiectasis, presented to our department with recurrent hemoptysis. Bronchoscopy revealed nothing else but blood arising from the upper lobe bronchus. High resolution computing tomography of the lung (HRCT) revealed bronchiectasis of the upper lobe. A right upper lobectomy was performed. Behind bronchiectasis multiple nodular lesions, 5-10 mm were observed. Histological and immunohistochemical examination revealed findings consistent with peripheral typical bronchial carcinoids.

Bronchi↗

PubMed1999

[Severe adult-type Williams-Campbell syndrome (Williams-Campbell-type bronchiectasis)].

A 70-year-old man was admitted to our hospital because of dyspnea. Arterial blood gas analysis indicated severe type-II respiratory failure. Williams-Campbell-type bronchiectasis was suspected because chest radiographs disclosed multiple cystic shadows in both lungs. Although inspiratory chest radiographs and computed tomographic (CT) scans showed cystic bronchiectasis, expiratory chest radiographs and CT scans demonstrated characteristic collapse of the ectatic bronchi. Continuous fluoroscopic visualization of the respiratory phase demonstrated bronchial dilatation during inhalation and collapse during exhalation. Williams-Campbell-type bronchiectasis was diagnosed on the basis of the radiological findings. Compared with previous cases of Williams-Campbell syndrome reported in Japan, this case was interesting because the patient exhibited severe respiratory failure and because the dilatating and collapsing bronchi were demonstrated by fluoroscopy.

Aged↗

PubMed1999

[Adult bronchiectasis revealing familial ciliary anomaly].

We report a case of bronchiectasis in a 26-year-old man associated with the following congenital abnormalities: deafness, purulent bronchorrhea, nasal polyps, dysmorphic physical pattern and chronic sinusitis. Situs inversus was absent. A sampling was performed on the posterior nasal mucous membrane and displayed structural ciliary abnormality: a deficiency of the intern dynein-arm. The patient's bother was affected and had similar features: congenital bronchiectasis, deafness, mental deficiency and sinusitis. Young's syndrome was relevant in this case. Hereditary ciliary dyskinesia should be considered in adults with bronchiectasis together with rhinologic and alimentary canal disorders. Nasal biopsies are safe and allow cilia examination.

Adult↗

PubMed1999

[Predisposing factors for bronchiectasis--analysis of 69 patients treated in the years 1995-1999].

The aim of this study was to analyze the predisposing factors for bronchiectasis in 69 patients hospitalized in the 3rd Dept. of the Institute of TB and Lung Diseases in Warsaw in years 1995-1999. Bronchiectasis was diagnosed on the basis of the high resolution computed tomography (HRCT) scan. Among 69 patients at the age of 15-72 years there were 45 women (65%) and 24 men (35%). Fifty patients were nonsmokers. The most frequent predisposing factors of bronchiectasis in that group of patients were as follows: pneumonia (30.1%, in it recurrent pneumonia--19.3%, a single pneumonia--10.8%), sinobronchial syndrome (19.3%), pulmonary tuberculosis (12.1%), nontuberculous mycobacterial lung infections (7.2%), recurrent pneumonia and bronchitis in childhood (7.2%) and connective-tissue diseases (3.6%). Among other predisposing factors there were allergic bronchopulmonary aspergillosis, foreign body in bronchus, hypogammaglobulinemia and colitis ulcerosa.

Adolescent↗

PubMedDate not supplied

The current physiotherapy management of patients with bronchiectasis: a UK survey.

The purpose of this survey was to ascertain which physiotherapy techniques are currently used, how interventions are assessed and to determine the views of physiotherapists in the UK on the need for further research. Senior physiotherapists in 100 randomly selected hospitals providing respiratory care in the UK were sent a previously piloted questionnaire on their bronchiectasis practice. The response rate was 82%. A variety of physiotherapy treatment techniques and outcome measures are used, and there are no national guidelines on the physiotherapy management of patients with bronchiectasis. There is a clear need for research into the effectiveness of physiotherapy techniques in bronchiectasis including a cost-benefit analysis which would enable targeting of resources and the development of evidence-based practice guidelines.

Attitude of Health Personnel↗

PubMed2002

Clinical bronchiectasis complicating pulmonary sarcoidosis: case series of seven patients.

BACKGROUND AND AIM OF WORK: It has been known for years that pathological changes of bronchiectasis may be seen on tissue examination of the lungs in pulmonary sarcoidosis. However, the clinical picture that may develop as a result of these changes has been under-appreciated. METHODS: We report seven patients with advanced stage IV pulmonary sarcoidosis in whom clinical bronchiectasis developed, seen in our university based pulmonary clinic between 1978 and 1999. RESULTS: These patients frequently exhibited symptoms and signs that are uncommon in sarcoidosis - hemoptysis, recurrent infection, lung crackles and digital clubbing. The clinical course was worse than similar stage nonbronchiectatic sarcoidosis, with more frequent infections and a higher mortality rate. CONCLUSIONS: Recognition of this subset of patients may improve management when complications arise. Therapy directed at bronchiectasis rather than aimed at suppressing putative continued sarcoidosis activity appears to be more important at this late stage of disease.

Adult↗

PubMed1955

Planned care for patients with bronchiectasis.

Bronchiectasis, for which once only the most elementary palliation could be offered, now often can be cured by operation or well controlled by conservative therapy. Since true bronchiectasis implies irreversible anatomic changes, operation offers the only hope of cure, and it should be undertaken whenever it is not contraindicated by extent of involvement, age of the patient or other factors. Surgical results are excellent and mortality is at a minimum. When operation is prohibited, good results can still be anticipated by conservative measures. Such conservative therapy should combine prolonged use of antimicrobial drugs with adequate drainage of the diseased segments and general supportive measures. Any residual infection can be controlled by moderate use of appropriate antimicrobial agents. It is emphasized, however, that the control of bronchiectasis requires very careful diagnostic studies and a detailed analysis of the patient's condition, and that the therapy itself must be carefully adjusted in terms of the individual situation.

Anti-Infective Agents↗

PubMed1992

[Two probable cases of adult Williams-Campbell syndrome--advocation of Williams-Campbell-type bronchiectasis].

Williams-Campbell syndrome is a unique type of bronchiectasis which shows ballooning during expiration and collapse during inspiration. Its etiology is thought to be a congenital decrease in bronchial cartilage. We experienced two adult cases of Williams-Campbell syndrome. Since the mucociliary transport system was normal in both cases and neither case had chronic sinusitis, these cases differed from sino-bronchial syndrome. Cases of Williams-Campbell syndrome reported in Japan show characteristic bronchography, but a decrease in bronchial cartilage may or may not be present and its congenital nature is very difficult to demonstrate. We therefore advocate referring to such cases of bronchiectasis with these bronchographic characteristics Williams-Campbell-type bronchiectasis, even if a decrease in bronchial cartilage is not observed.

Aged↗

PubMedDate not supplied

Bronchiectasis in HIV disease.

An increased frequency of bacterial pneumonia occurs in HIV-infected individuals: however the development of bronchiectasis is not well recognized. We describe seven patients with HIV infection who developed chronic symptomatic lung disease, six with troublesome recurrent infective exacerbations. Bronchiectasis was demonstrated by computed tomography in five patients, and bronchial wall thickening was shown in a further two patients. The characteristics of the patients are described, and possible aetiological factors are discussed. As measures become available which prolong the later stages of HIV disease, bronchiectasis may become an increasing problem in this patient population. Early recognition and appropriate management may significantly alter morbidity in advanced HIV disease.

AIDS-Related Opportunistic Infections↗

PubMedDate not supplied

Bronchiectasis consequent upon prolonged foreign body retention.

OBJECTIVE: To review our experience with bronchiectasis complicating prolonged endobronchial retention of foreign bodies (FB) among patients with a history of aspiration of FB seen between 1975 and 1998. DESIGN: A retrospective review of clinical data of consecutive patients with a history of aspiration of FB treated during the time of study. SETTING: The Cardiothoracic Unit of the University College Hospital, Ibadan, directly serves a population of about 40 to 60 million in the South Western Nigeria and the community clinics. SUBJECTS: All patients with a history of FB aspiration (or suggestive of FB aspiration) from whom patients with prolonged FB retention were selected. MAIN OUTCOME MEASURES: Patients with a relevant history or chronic respiratory symptoms had their clinical examination findings, plain chest radiographic (or/and bronchographic) findings, treatment modalities and outcome studied. RESULTS: Sixteen patients among 203 patients who had tracheobronchial foreign bodies removed had this complication. The mean age at presentation was 13.0 +/- 17.3 years. Twelve patients (75%) retained inorganic materials and in four patients (25%) it was organic. The period of retention ranged between 4.8 to 108 months (mean 17.0 +/- 22.6 months). Main presenting symptoms were: productive cough, 16 patients; pyrexia, seven patients; combination of chest pain, fever and copious sputum, five patients. In none of the patients was the history of aspiration of FB definitely elicited before commencement of treatment. Symptoms were worst among patients who retained inorganic materials. All of the patients had bronchoscopy but retrieval of FB by this means was successful in one patient (6.3%). Six patients (37.5%) had thoracotomy and bronchotomy; nine patients had bronchotomy and lung resection [seven (77.8%) lobectomies, one (11.1%) pneumonectomy and one (11.1%) segmentectomy] as the definitive treatment. The one patient who had brochoscopic retrieval subsequently had lobectomy to control symptoms of bronchiectasis. Five out of six (83.3%) who had bronchotomy had persistent symptoms of bronchiectasis. All the nine (56.3%) who had lung resection remained asymptomatic at follow up. There was one mortality (6.3%) in the series--a 75 year old who died from poorly controlled Diabetes mellitus. CONCLUSION: Prevention of aspiration of FB is better than cure. In every medically treated child with persistent cough with or without a history of aspirated FB, the possibility of retained FB should be borne in mind.

Adolescent↗

PubMed2005

[Pulmonary infection caused by non-tuberculous mycobacteria in two patients with bronchiectasis].

Two patients, a woman aged 67 years and a man aged 80 years, had chronic cough among other respiratory symptoms. In the woman, chest radiograph and CT-scan revealed partial atelectasis of the middle lobe and bronchiectasis. In the man, an interstitial pattern was seen on chest radiograph, and CT scan showed diffuse bronchiectasis. In both the man and the woman, non-tuberculous mycobacteria were identified (Mycobacterium avium complex and Mycobacterium abscessus, respectively). Treatment was successful in both patients. Non-tuberculous mycobacteria can cause considerable pulmonary infection in patients with bronchiectasis.

Aged↗

PubMed1992

[Alpha 1-antitrypsin deficiency with bronchiectasis in two sisters].

Two sisters with alpha 1-antitrypsin deficiency and bronchiectasis are reported. The 53-year-old older sister (propositus) had pneumonia 3 times during her forties. She developed dyspnea on exertion in February, 1988, and a few months later she was seen at our hospital. Her serum alpha 1-antitrypsin level was 11 mg/dl. Vascular markings on chest X-ray were mildly decreased. Bronchography showed generalized cystic bronchiectasis. The younger sister was seen at our hospital at the age of 50. She had been in good health until one year previously when she had developed pneumonia, and thereafter she had suffered from productive cough and dyspnea on exertion. Her alpha 1-antitrypsin level was 22 mg/dl. Chest X-ray showed ring-like shadows and tram lines. Chest CT scans of both sisters revealed cystic changes, dilatation of bronchi, and the connection of these lesions diffusely. The alpha 1-antitrypsin phenotype of these sisters was found to be PiSiiyama (homozygote). Family study revealed that alpha 1-antitrypsin levels of 7 members were intermediate and no members had symptoms. We consider that bronchiectasis may have been related to alpha 1-antitrypsin deficiency in these sisters.

Bronchiectasis↗

PubMed1991

[Functional activity of the neutrophils in the bronchoalveolar space in chronic bronchitis and bronchiectasis].

Bronchoalveolar lavage fluid neutrophils were studied by the cytologic methods in 58 patients with chronic bronchitis, 63 ones with bronchiectasis, and 8 normal controls. The study included cytospectrophotometry of myeloperoxidase and alkaline phosphatase activity and estimation of active oxygen-producing cells in the NBT test. Neutrophilic functional activity was different in the patients with chronic bronchitis and bronchiectasis. Neutrophilic myeloperoxidase and alkaline phosphatase activities were lower in the patients with chronic bronchitis than in those with bronchiectasis, whereas the counts of cells active in the NBT test were low in both patient populations.

Bronchiectasis↗

PubMed1991

Bronchiectasis.

Bronchiectasis is important because (1) it predisposes the patient to infection; (2) it may be accompanied by increased bronchial blood flow and hemoptysis; (3) when widespread, it may be associated with significant airway obstruction; and (4) when focal, it may simulate neoplasms and other diseases. The widespread use of computed tomography (CT) for evaluation of lung diseases has revealed that bronchiectasis is common--even in patients who do not have clinical or plain radiographic suspicion of bronchiectasis. The pathology, pathogenesis, and radiographic findings are presented herein.

Bronchiectasis↗

PubMed1975

[Clinical effects of lividomycin on respiratory tract infections, primarily wet cases of bronchiectasis (author's transl)].

Patients with various respiratory infections, primarily wet cases of bronchiectasis, were treated with lividomycin (LVDM) at a dose of 1 g/day for about a week. The results obtained are as follows: 1. Of the 15 patients with refractory bronchiectasis who had previously been treated with various other antibiotics, 11 patients (73.3%) responded to LVDM. Although there was no patient who responded to LVDM excellently, good cures were achieved in 5 pateints and fair cures in 6 patients. 2. In bronchiectasis patients having infections in association with pulmonary tuberculosis (mixed infections), LVDM did not show any remarkable effects, excepting that fair cures were achieved in 3 patients. 3. In the 7 patients with acute bronchopneumonia, excellent, good and fair cures were achieved in 1, 5 and 1 patient, respectively. Although LVDM was effective in all of the patients with the said infection, the number of the patients responded excellently was meager and the onset of action seemed to be somewhat slow. 4. In one patient with lobar pneumonia, LVDM was not effective. 5. The MIC values of LVDM for clinically isolated various organisms are summarized below: Pseudomonas aeruginosa 50 mcg/ml, Klebsiella pneumoniae 3.13 to 6.25 mcg/ml, Haemophilis influenzae 1.56 to 6.25 mcg/ml, Diplococcus pneumoniae 50 mcg/ml, alpha-Streptococcus 50 to 100 mcg/ml, beta-Streptococcus 100 mcg/ml, and Staphylococcus aureus 1.56 mcg/ml. 6. In spite of the fact that a majority of the patients participated in this study were the aged, LVDM's side effects were limited to an increase of BUN (one patient) and eruption (one patient). Any noticeable changes in hepatic and renal function, hearing acuity, etc. were not observed. As indicated above, LVDM was effective against, respiratory infections as well as infections of the urinary tract on which a considerable number of research has already been reported. Thus, it is considered to be worthwhile to use LVDM for the treatment of respiratory infections.

Adult↗