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PubMed1992

[An autopsy case of Marfan syndrome with bronchiectasis and multiple bullae].

We report an autopsy case (27-year-old male) with Marfan syndrome, who died of chronic respiratory failure due to bronchiectasis and multiple bullae in both lungs. He had suffered from expectoration of massive amounts of sputum since the age of 15 years. At this time, chest roentgenogram had revealed bronchiectatic changes in the bilateral lower lung fields. Seven years later at the age of 22 years, the formation multiple bullae in both lungs were added to the bronchiectatic changes on chest roentgenogram. Administration of erythromycin (400 mg/day) was started in February, 1987, and the massive sputum volume markedly decreased according to appearance of bullous formation. He was admitted to our department because of deterioration with chronic respiratory failure and right heart failure at 26 years in December, 1989. Although various therapy was performed, he died of chronic respiratory failure in February, 1990. Autopsy findings were as follows: (1) cyclindrical bronchiectatic changes in bilateral lower lobes and (2) extensive multiple bullae in the subpleural areas with bronchiectatic changes in the middle and bilateral lower lobes, with no bronchiectatic changes in the bilateral upper lobes. Several pulmonary disorders accompanying Marfan syndrome have been reported, especially in children. However, the present case demonstrated that fetal pulmonary involvement by Marfan syndrome may not present until adulthood, and affect both airways and lung parenchyma.

Adult↗
PubMedDate not supplied

Tracheal bronchus associated with bronchiectasis. Case report.

The authors describe a case of ectopic tracheal bronchus (TB) in a 42 years old female. This TB presented bronchiectasis determining various episodes of inflammation in the pulmonary district depending on it. The result of those episodes was atelectasis and fibrotic alterations of the superior right lobe. The mean diagnostic procedure was bronchography. Surgical treatment was performed (superior right lobectomy) with a good result controlled in a 5 year follow-up.

Adult↗
PubMed1992

[Bronchiectasis with bronchopleural fistula].

We report on a female patient who is now 56 years of age suffering from bronchiectasis disease. Due to the bronchopleural fistula in the middle lobe we decided to perform an operation despite left-sided bronchiectases and in spite of the obstructive airways disease.

Bronchial Fistula↗
PubMed1952

Antibiotic therapy of abscess of the lung and bronchiectasis.

Since the fusospirochetal group of bacteria are the commonest etiologic agents in abscess of the lung, aqueous crystalline penicillin is the agent of first choice in the majority of cases. Streptomycin is indicated for a small group of cases in which Klebsiella is the etiologic agent. Aureomycin, chloramphenicol or terramycin may produce an excellent therapeutic response either initially or after therapeutic failure with penicillin. Administration of antibiotics by inhalation should be carried out in conjunction with systemic forms of treatment. In the treatment of bronchiectasis, the antibiotics are most useful in the control of acute exacerbations of pulmonary infection which punctuate the course of this disease.

Abscess↗
PubMed2004

[Diagnosis and treatment of bronchiectasis].

Patients with bronchiectasis usually have chronic pulmonary infection with secondary obstructive airway disease. This disease has to be distinguished from chronic obstructive pulmonary disease (COPD). The diagnosis is established with the help of high resolution CT scan of the thorax. Further diagnostic evaluation is necessary to identify aetiologic factors. The treatment is complicated and involves many aspects: treatment of underlying conditions, recognition of exacerbations, boosting of tracheobronchial clearance, treatment of haemoptysis, surgical resection of affected lung segments and antibiotic therapy. Antibiotic therapy can focus on treatment of exacerbations but chronic suppressive antibiotic therapy is also used. Favourable results have been reported for maintenance treatment with macrolids as well as anti-inflammatory therapy.

Anti-Bacterial Agents↗
PubMed2005

[Case reports of the treatment with tobramycin solution for inhalation in severe bronchiectasis patients continuous infection of Pseudomonas aeruginosa].

Aerosolized tobramycin has been frequently used in cystic fibrosis patients in order to directly deliver the antibiotic to the endobronchial site of infection. Recently, we experienced three cases of severe chronic bronchial infection of Pseudomonas aeruginosa treated by aerosolized tobramycin inhalation (TOBI). For two cases, we succeeded in improving the patient's medical condition, quality of life, and reduced the amount of sputum. We consider that TOBI should be useful for chronic bronchial infection by Pseudomonas aeruginosa in the case of a patient who does not respond to usual antibiotics via other routes. But further investigation is necessary to reveal clinical efficacy and safety in non-CF patients with bronchiectasis.

Administration, Inhalation↗
PubMedDate not supplied

Acromesomelic dysplasia with bronchiectasis.

We report a rare case of acromesomelic dysplasia with bilateral bronchiectasis and obstructive sleep apnoea. Diagnosis of acromesomelic dysplasia was based on radiographs of whole skeleton.

Adult↗
PubMed2005

[Bronchiectasis--an orphan disease? Diagnosis and treatment in the 21st century].

Bronchiectasis has been termed an "orphan disease" since its decline in the developed world but it is still common in developing countries and in lower socioeconomic classes. Recurrent respiratory infections, under treatment with antibiotics and poor immunization rates are the main causes for the relatively high prevalence in these populations. Today the disease is not often adequately diagnosed nor treated. Airways dilatation, poor clearance of secretions and recurrent infections are the basis of the disease that is characterized by chronic productive purulent cough and recurrent exacerbations. The disease can be localized or diffuse, primary or secondary to other systemic disease. High resolution CT is the examination of choice for diagnosis. Lack of sufficient randomized control trials makes it difficult to establish guidelines. Nevertheless, partial information favors the use of prolonged antibiotic treatment, mucolytic agents and inhaled corticosteroids. Treatment should be tailored to the individual patient guided by the clinical picture while bronchodilators, bronchopulmonary hygiene and physical therapy are optional. Surgical resection is recommended for symptomatic localized bronchiectatic disease while lung transplantation should be reserved, as a last resort, for end-stage bilateral disease.

Bronchiectasis↗
PubMedDate not supplied

[Bronchiectasis worsening by p-ANCA (bactericidal/permeability-increasing protein) positive vasculitis. A case report and review of the literature].

A 67-year-old woman was hospitalized with recurrent fever, arthralgia and erythema of the arms and legs. She had suffered from bronchiectasis for the previous 4 years, and Pseudomonas was persistently detected in her sputum. During the course of her illness, she developed distal sensitive and motor polyneuropathy. Serum test was positive for myeloperoxidase and bactericidal/permeability-increasing protein antineutrophil cytoplasmic antibodies. Nerve biopsy showed vasculitis infiltration of the vasa nervorum. She started immunoglobulin therapy, and after methylprednisolone and pulse cyclophosphamide therapy once monthly for 6 months she showed a good response and a reduction in symptoms.

Aged↗
PubMed1990

[The dynamics of the indices of reactivity of the body in patients with chronic bronchitis and bronchiectasis in the course of treatment].

Complex determination of lymphocyte functional activity and the condition of the organism's immunological values in patients with chronic bronchitis and bronchiectasis helps in obtaining additional objective information which makes it possible to appraise the severity of the course of the disease, correct the treatment and determine its efficacy in different stages of the disease, come to a decision in regard to operative intervention, and form a judgement on the fullness of recovery. These tests acquire additional diagnostic value in patients with mild clinical manifestations of the disease as well as in the absence of its laboratory signs. A direct dependence between the severity of the disease and the above named values was revealed.

Bronchiectasis↗
PubMed1990

Nephronophthisis with bronchiectasis.

A boy with nephronophthisis and bronchiectasis, a previously unrecognised association is described. Electron-microscopic examination of a renal biopsy from an asymptomatic sibling revealed marked thickening and lamination of the tubular basement membranes.

Biopsy↗
PubMed1990

[Antibody-coated bacteria and quantitative culture in sputum samples from patients with bronchiectasis].

The results of the quantitative culture of sputum samples from patients with bronchiectasis were compared with those obtained in the same samples with the detection of antibody-coated bacteria (ACB), in the acute phase of the disease (group I), after antibiotic therapy (group II), and in the phase of clinical stability (group III). In quantitative cultures at least one potentially pathogen species was isolated, at a concentration of greater than or equal to 10(6) colony forming units/ml in the 7 sputum samples from group I, in 4 of the 5 samples from group II, and in 10 of the 11 samples from group III. The immunofluorescence technique detected ACB in all samples from group I and group II, and in 10 of the 11 from group III. The sample from group III in which ACB were not detected was the same in which potentially pathogen organisms were not detected by culture.

Acute Disease↗
PubMed1990

[Comparison of immunological aspects in diffuse panbronchiolitis and cystic bronchiectasis].

We examined immunological parameters in 35 patients with diffuse panbronchiolitis (DPB) and 20 patients with cystic bronchiectasis (CBE) to analyze the relation between local immune responses and subsequent clinical courses. Patients with DPB showed more impaired cell-mediated immunity than those with CBE. On analysis of peripheral blood lymphocytes from DPB and CBE, the significant increase in CD4 positive T-cells and Fc alpha R positive T-cells and decrease in Leu-11 positive cells were recognized. The observation of humoral immunity revealed a tendency for local immunity to be accelerated such as increases of IgA and IgG2 in both diseases. The titer of serum cold agglutinin and rheumatoid factor were elevated in DPB. However, these fluctuations of immunological factors were normalized according to improvement of inflammatory processes by chemotherapy. The analysis of the distribution of lymphocyte subsets in lung tissues taken from DPB showed that CD4 positive T-cells always overwhelmed CD8-positive T-cells and that secretary IgA-positive B-cells were abundant in bronchioles. On the other hand, bronchiolitis was recognized in about 80% of cases with CBE, although to a less striking degree than DPB. Lymphocyte subsets were similarly distributed in the respiratory tract. DPB and CBE could be considered as the same disease entity in the spectrum of chronic respiratory tract infection based on mucociliary disorder. Sinusitis and immunological abnormalities are often complicated by both diseases. However, precise analysis demonstrated that there are some obvious differences of systemic and local immune responses between DPB and CBE, probably due to the conditions of each diseases such as primary focus, severity and causative microorganism.

Adult↗
PubMed1990

Mucociliary clearance and transport in bronchiectasis: global and regional assessment.

Global and regional mucociliary clearance and transport in the lungs was studied in 20 patients with bronchiectasis by radioaerosol inhalation lung cine-scintigraphy and the quantitative analysis following inhalation of ultrasonically-generated 99mTc-tagged human serum albumin aerosol (mass median diameter; 1.93 microns with geometric s.d. of 1.52). In bronchiectatic lung regions, deposition of inhaled aerosol was diminished or inhomogeneous. Transport of inhaled radioactivity from the bronchiectatic regions was deranged in 95% of the patients (19/20). The following abnormal mucous transport patterns were regionally observed; stasis in 12 of the 20 patients (12/20), regurgitation or reversed transport in 14/20, straying in 8/20, spiral or zigzag transport in 1/20, and/or various combinations of these four abnormal transport patterns. When coughs occurred, regurgitation and stray became more marked in the bronchiectatic regions. These regional abnormalities in mucociliary transport seem to be responsible for the development of infections and hemoptysis in the bronchiectatic regions.

Administration, Inhalation↗
PubMed1987

Bronchiectasis--new therapeutic approaches based on pathogenesis.

This article has highlighted several new concepts in the pathogenesis of bronchiectasis and proposed a hypothesis that could explain the deterioration seen in some of the patients. Modification of our current antibiotic policy has been shown to be beneficial in both the short and long term. The results of recent studies would suggest that patients with purulent sputum benefit from antibiotic therapy whether this represents an acute exacerbation or the usual clinical state. Exacerbations respond well to broad-spectrum antibiotics effective against H. influenzae and S. pneumoniae in conventional doses. However failure to respond, particularly when the usual secretions are purulent, often indicates that dosage is inadequate and a trial of a higher dosage of an agent such as amoxicillin may well prove effective. Treatment of patients who are also penicillin sensitive needs to be clarified, although no orally effective alternative has yet been identified. Relapse is rapid in patients whose usual secretions are purulent, and long-term therapy improves well-being and several biochemical indices of important pathogenic mechanisms. However, as we understand the reasons for bacterial colonization and persistence better, it should prove possible to modify other mechanisms therapeutically, thus negating the need for long-term antibiotic therapy and the associated potential risks of resistance.

Acute-Phase Proteins↗