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Surgical treatment in bronchiectasis: analysis of 166 patients.

BACKGROUND: Bronchiectasis is usually caused by pulmonary infections and bronchial obstruction. It is still a serious problem in developing countries as our country. We reviewed the morbidity and mortality rates and outcome of surgical treatment for bronchiectasis. PATIENTS AND METHODS: Between 1990 and 2000, 166 patients (92 female and 74 male patients) underwent pulmonary resection for bronchiectasis. The mean age was 34.1 years (range, 7-70 years). Mean duration of symptoms was 5.7 years. RESULTS: Symptoms were copious amount of purulent sputum in 135 patients, expectoration of foul-smelling sputum in 109, hemoptysis in 35 and cough in all patients. The indication for pulmonary resection was failure of medical therapy in 158 patients, massive hemoptysis in five and lung abscess in three. The disease was bilateral in six patients and mainly confined to the lower lobe in 127. One hundred and twenty patients had a lobectomy, 13 had a pneumonectomy, 21 had a segmentectomy and a combination of these approaches in 18. Operative morbidity and mortality were seen in 18 (10.5%) and in three (1.7%) patients, respectively. Follow-up was complete in 148 patients with a mean of 4.2 years. Overall, 111 patients were asymptomatic after surgical treatment, symptoms were improved in 31, and unchanged or worse in six. CONCLUSIONS: Surgical treatment of bronchiectasis is more effective in patient with localized disease. It is satisfactory with acceptable ratio of morbidity and mortality.

Adolescent↗

Surgical management of bronchiectasis: analysis and short-term results in 238 patients.

OBJECTIVE: Bronchiectasis is defined permanent dilatations of bronchi with destruction of the bronchial wall. It is still a major cause of morbidity and mortality in developing countries. The aim of this retrospective study is to present our surgical experiences, the early and long-term results of 238 patients with bronchiectasis during a 10-year period. METHODS: We reviewed the medical records of 238 patients who underwent surgical resection for bronchiectasis between January 1992 and December 2001, at Gülhane Military Medical Academy (GMMA) Thoracic Surgery Department. Variables of age, sex, symptoms, etiology, and type of operation, mortality, morbidity and the result of surgical therapy were analyzed. RESULTS: There were 205 (86.13%) male and 33 (13.87%) female patients with an average age of 23.7 and a range of 15-48 years. The presenting symptoms were productive cough in 133 (55.88%), fetid sputum in 116 (48.73%), recurrent infections in 84 (35.29%), and hemoptysis in 39 (12.18%) patients. The disease was bilateral in 31 patients (13.02%) and mainly confined to the lower lobes in 162 (68.06%). The surgical treatment was as follows: pneumonectomy in 13 patients (5.46%), lobectomy in 189 (79.40%), lobectomy+segmentectomy in 31 (13.02%), and wedge resection or segmentectomy in five (2.1%). Staged bilateral thoracotomy was used in 14 patients. There was no operative mortality. Complications occurred in 21 patients and the morbidity rate was 8.82%. Complete resection was achieved in 154 (64.7%) patients. Follow-up data were obtained for 229 (96.21%) of the patients. Nine patients were lost to follow-up. The mean follow-up of these patients was 9 months (range, 3 months to 4 years). The symptoms disappeared in 189 patients (79.41%) and 29 patients (12.18%) had improved, whereas 11 patients (4.62%) had no improvement. Significantly better results were obtained in patients who had undergone a complete resection. CONCLUSIONS: Surgical resection for bronchiectasis can be performed with acceptable morbidity and mortality at any age. The involved bronchiectatic sites should be resected completely for the optimum control of symptoms.

Adolescent↗

A syndrome of hypoplastic sinuses, hydrocephalus, bronchiectasis, and hypogammaglobulinemia with functional antibody deficiency in twin girls.

BACKGROUND: A variety of syndromes are known to be associated with immune deficiency, some as a major part of the syndrome and some as an occasional finding. OBJECTIVES: To report a newly defined syndrome of hydrocephalus, hypoplastic sinuses, cartilaginous webbing of the bronchi, bronchiectasis, and hypogammaglobulinemia with functional antibody deficiency in identical twin girls. METHODS: Spirometry, radiography, computed tomography (CT), bronchoscopy, and laboratory tests were performed for diagnosis. RESULTS: A girl (twin A) was evaluated at the age of 7 years for chronic cough and wheeze that worsened with exercise. Hydrocephalus had been diagnosed a year before evaluation. She was treated for cough variant asthma and was lost to follow-up until the age of 10 years. The cough had worsened progressively, and dyspnea was now apparent. Spirometry was consistent with reversible airway obstruction. A chest radiograph was suggestive of bronchiectasis. A chest CT scan showed bilateral upper lobe bronchiectasis. Hypogammaglobulinemia and functional antibody deficiency were noted. A CT scan of the sinuses revealed aplasia or severe hypoplasia of all the paranasal sinuses. Bronchoscopy revealed a grossly abnormal bronchial structure and atrophic-appearing bronchial mucosa. Twin B also had a history of hydrocephalus. Although she had no respiratory symptoms, chest CT revealed bronchiectasis, and she had obstruction on spirometry. Laboratory analysis revealed hypogammaglobulinemia and functional antibody deficiency. She became symptomatic 2 years after twin A. CONCLUSIONS: This is a newly reported syndrome of hydrocephalus, absent sinuses, abnormalities of the bronchi, and functional antibody deficiency, which initially presented as cough and wheeze.

Agammaglobulinemia↗

[Internal consistency and validity of the Spanish version of the St. George's respiratory questionnaire for use in patients with clinically stable bronchiectasis].

OBJECTIVE: To analyze the reliability and validity of the St. George's Respiratory Questionnaire (SGRQ) for use in patients with clinically stable bronchiectasis. MATERIAL AND METHODS: The SGRQ (50 items on 3 scales--symptoms, activity, and impact) was administered to 102 patients (mean [SD] age, 69.5 [8.7] years; 63% men) with clinically stable bronchiectasis. Disease severity was classified according parameters such as airflow obstruction (forced expiratory volume in 1 second), colonization by Pseudomonas aeruginosa, extent of bronchiectasis, symptoms, daily quantity of sputum, and number of exacerbations. Internal consistency (Cronbach's alpha and correlation between items and between item and scale), concurrent validity (correlation between items and clinical variables), predictive validity (correlation between items and severity), and construct validity (factorial analysis of main components) were assessed. RESULTS: The internal consistency of the SGRQ was excellent (Cronbach's alpha between 0.81 and 0.87 for the different scales, and 0.90 for the overall score). Concurrent validity was high, as correlations between items and clinical variables were significant and followed the expected distribution. The SGRQ differentiated between degrees of disease severity, regardless of the clinical variable used. The factorial analysis showed a construct of 4 factors that were only moderately similar to the original structure of the questionnaire, due mainly to inclusion of a small number of questions with conditioned response and others with low discriminatory capacity. CONCLUSIONS: The SGRQ shows excellent concurrent and predictive internal consistency and validity, though restructuring of the original construct would be advisable before use in patients with stable bronchiectasis.

Aged↗

A preliminary assessment of alpha-1 antitrypsin S and Z deficiency allele frequencies in common variable immunodeficiency patients with and without bronchiectasis.

Common variable immunodeficiency (CVID) is the name given to a clinically heterogeneous group of hypogammaglobulinaemic immunodeficiency states. Bronchiectasis is a feature of this disease and is believed to be the result of recurrent bacterial infection affecting the respiratory tract. Bronchiectasis is also a feature associated with emphysematous changes of the lung in alpha-1 antitrypsin (AAT) deficiency, a serious and relatively common disease, affecting 1 : 2000 in the United Kingdom. This has been demonstrated to result from possession of deficiency alleles, the most clinically important alleles being PI*Z and PI*S. Isolated reports of families with antibody deficiency and AAT deficiency have been published but to date no study has been performed to specifically investigate if AAT deficiency is associated with the lung damage seen in CVID patients. We have developed a PCR genotyping assay that identifies S and Z deficiency alleles and we have used this assay in a preliminary study to investigate the occurrence of these deficiency alleles of AAT in 43 CVID patients. Results of this preliminary study suggest that CVID patients did not have an altered distribution of AAT genes when compared to 70 normal controls. Subgrouping of CVID patients into those with and without bronchiectasis demonstrated a Z allele frequency of 0.077 in those patients with bronchiectasis, which is higher than found in normal controls, namely 0.029 (P < 0.15). Due to the relatively small numbers studied, these results are inconclusive in determining whether AAT deficiency may exacerbate lung damage in some CVID patient, the data does however, indicate that a larger multi-centre study involving many more CVID patients may be useful.

Adult↗

Clinical, laboratory findings and microbiologic characterization of bronchiectasis in Thai patients.

OBJECTIVE: The aim of the present study was to characterize the clinical features, underlying disease states, laboratory findings and microbiological characterization of bronchiectasis in Thai patients. METHODOLOGY: For a 2-year period all consecutive patients diagnosed with bronchiectasis at Phramongkutklao Hospital, Bangkok, Thailand, were recruited. Data including history, physical examination, underlying disease and laboratory studies were carefully reviewed and recorded. RESULTS: Fifty patients diagnosed with bronchiectasis were enrolled. Their mean age was 58 years. The most common background aetiology was tuberculosis. Six per cent of the patients were diagnosed as having diffuse panbronchiolitis. Normal chest radiographs were found in 10%. The common organisms isolated were Pseudomonas aeruginosa (20%), Haemophilus influenzae (14%), Klebsiella pneumoniae (14%) and Streptococcus pneumoniae (6%). Non-tuberculous mycobacteria which included Mycobacterium kansasii and Mycobacterium chelonae were found in 6%. CONCLUSION: We report the characteristics of bronchiectasis in Thai patients. The most common identifiable aetiology was tuberculosis.

Adult↗

Effects of childhood bronchiectasis on cardiac functions.

BACKGROUND: Bronchiectasis is still a widespread disease in developing countries. It is an important cause of mortality and morbidity. The information on cardiac involvement in bronchiectasis is limited. However, cor pulmonale is common in patients with chronic lung disease, such as cystic fibrosis. METHODS: We utilized echocardiography and exercise tests, along with clinical scoring, chest radiograph scoring, and pulmonary function tests in 21 patients to determine whether detectable changes in cardiac functions were present, and the nature of their relationship to the underlying disease severity. RESULTS: The ventricular systolic functions were preserved in all patients. Some of the patients had changes in left ventricular diastolic function indices, characterized by abnormal Ewave/Awave (E/A) ratios or isovolumetric relaxation time values. Isovolumetric relaxation time but not E/A ratios was found to have a significant negative correlation with the clinical score. In addition, exercise capacity was decreased in bronchiectatic children. Most of the patients stopped the exercise test due to exhaustion before reaching maximum heart rate. CONCLUSION: Left ventricular diastolic functions are affected in bronchiectasis, and the performance of patients is dependent on their pulmonary status. This is the first study demonstrating the cardiac effects of bronchiectasis according to our survey of the published literature.

Adolescent↗

Non cystic fibrosis bronchiectasis.

Bronchiectasis is becoming less common as the treatment for acute lower respiratory tract infections improves and immunization programmes decrease the frequency of pertussis and measles. However bronchiectasis is still a challenge to the paediatric chest physicians in many developing parts of the world and it remains a frequent problem being the final common pathway of several different lower respiratory tract insults such as cystic fibrosis, immunodeficiency, ciliary dyskinesia. Although the treatment of patients with bronchiectasis is primarily medical, surgical treatment is required in a small group of patients with recurrent episodes of pneumonia and atelectasis localized to one area, severe or recurrent hemoptysis and in those unresponsive to aggressive medical treatment with abnormal growth and development. There are unanswered questions about childhood bronchiectasis, mainly on aetiology and treatment which require more research.

Bronchiectasis↗

[Computed tomography of bronchiectasis].

Twenty-six patients with suspected bronchiectasis were examined by bronchography and CT and the CT appearances of normal and abnormal bronchi are described. Like bronchography, CT will distinguish between cylindrical and cystic bronchiectasis. Lobar and segmental localisation can be reliably achieved by both methods; there was agreement between these methods in 109 out of 114 lobes and in 391 out 468 segments. In our experience the sensitivity of CT in the diagnosis of bronchiectasis is 93% and specificity is 96%. It is possible to obtain the necessary information, i.e. the existence and localisation of bronchiectasis by means of CT.

Adult↗

Outcome in adult bronchiectasis.

The outcome in adult bronchiectasis has not been well described; in particular there has been a lack of long-term prospective studies. Therefore a follow-up study was performed to assess outcome in bronchiectasis in a cohort of adult patients. One hundred-and-one sequential adults, 33 male and 68 female; age 54 +/- 14 years (mean +/- SD) with bronchiectasis had a clinical assessment and spirometry performed. All were non-smokers and 84 were classified as having idiopathic disease. Patients were commenced on a standardized treatment regime and followed up for a minimum period of 2 years. On their last review when patients were clinically stable, a repeat clinical assessment and spirometry was performed and compared with the initial review. The primary endpoints measured were symptoms and FEV1. Subjects were followed up for 8.0 +/- 4.9 years. Clinical review showed that the patients had persistent symptoms that, in the case of dyspnea and sputum volume, were worse on follow-up. Spirometry showed a significant decline in FEV1 over the follow-up period with an average loss of 49 ml per year. This study showed in this group of predominantly female adult patients with bronchiectasis followed up for 8 years, patients had persistent symptoms and an excess loss in FEV1.

Bronchiectasis↗

Computed tomography of bronchiectasis.

Computed tomography (CT) was performed on six patients with bronchiectasis. In two cases of advanced cystic bronchiectasis, the diagnosis was apparent on plain chest roentgenograms. In four cases, bronciectasis was initially diagnosed by CT and later confirmed by bronchography. The CT signs of bronchiectasis include air--fluid levels in distended bronchi, a linear array or cluster of cysts, dilated bronchi in the periphery of the lung, and bronchial wall thickening due to peribronchial fibrosis. Distended bronchi must be distinguished from emphysematous blebs, which generally have no definable wall thickness and no accompanying vessels. It is concluded that CT should have a role in establishing the presence and anatomic extent of bronchiectasis.

Bronchiectasis↗

Bronchiectasis in persons with skin lesions resulting from arsenic in drinking water.

BACKGROUND: Arsenic is a unique human carcinogen in that it causes lung cancer by exposure through ingestion (in drinking water) as well as through inhalation. Less is known about nonmalignant pulmonary disease after exposure to arsenic in drinking water. METHODS: We recruited 108 subjects with arsenic-caused skin lesions and 150 subjects without lesions from a population survey of over 7000 people in an arsenic-exposed region in West Bengal, India. Thirty-eight study participants who reported at least 2 years of chronic cough underwent high-resolution computed tomography (CT); these scans were read by investigators in India and the United States without knowledge of the presence or absence of skin lesions. RESULTS: The mean (+/-standard deviation) bronchiectasis severity score was 3.4 (+/-3.6) in the 27 participants with skin lesions and 0.9 (+/-1.6) in the 11 participants without these lesions. In subjects who reported chronic cough, CT evidence of bronchiectasis was found in 18 (67%) participants with skin lesions and 3 (27%) subjects without skin lesions. Overall, subjects with arsenic-caused skin lesions had a 10-fold increased prevalence of bronchiectasis compared with subjects who did not have skin lesions (adjusted odds ratio=10; 95% confidence interval=2.7-37). CONCLUSIONS: These results suggest that, in addition to being a cause of lung cancer, ingestion of high concentrations of arsenic in drinking water may be a cause of bronchiectasis.

Adult↗

Ultrastructural abnormalities of bronchial cilia in children with recurrent airway infections and bronchiectasis.

Anomalies of the bronchial cilia were studied in 5 children with recurrent pulmonary infections. Case 1 had Kartagener's syndrome and an absence of the inner and outer dynein arms in most cilia, although a few shortened and even some normal arms could be seen. Cases 2 and 3 had unilateral bronchiectasis without family history of Kartagener's syndrome. Serial studies of the bronchial epithelium at times showed a bilateral lack of the inner dynein arms and a partial lack of outer arms. These abnormalities persisted in these 2 children after they had recovered from the acute pulmonary infection but disappeared after 6-8 months of antibiotic treatment. Cases 4 and 5 had recurrent pulmonary infections without bronchiectasis and many shortened outer dynein arms could be seen, but these anomalies disappeared after recovery. In all 5 children such architectural ciliary anomalies were present as megacilia, fused cilia, naked cilia, and completely disorganised axonemas. These architectural defects were particularly numerous in the children without bronchiectasis. Our observations suggest that anomalies of the bronchial ciliary microtubular system may not only be congenital but may also be acquired; this might well help to explain some cases of repeated respiratory tract infection and bronchiectasis.

Adolescent↗

Bronchiolitis obliterans, bronchiectasis, and other sequelae of adenovirus type 21 infection in young children.

A remarkably high incidence of bronchiectasis and other pulmonary sequelae was observed in young children affected during an epidemic of severe lower respiratory tract infections apparently caused by adenovirus type 21. The histopathological findings are described in four cases in which one or both lungs were obtained for examination at intervals ranging from two months to three years after the acute infections. Widespread bronchiolar obliteration (bronchiolitis obliterans) was a striking finding in all four. The severity of bronchial inflammation and of bronchiectasis was proportional to the time elapsed since the acute infections. Bronchiolar obliteration is a likely sequel of the necrotizing bronchiolitis which may occur during acute adenovirus infections. The role of bronchiolar obliteration in the pathogenesis of bronchiectasis and other chronic lung disease is discussed. Adenoviruses may be a major cause of post-infectious bronchiectasis in childhood.

Acute Disease↗

The need to redefine non-cystic fibrosis bronchiectasis in childhood.

BACKGROUND: Non-cystic fibrosis (CF) bronchiectasis has previously been reported to be rare and progressive in children living in western societies. METHOD: A clinical and radiological review was undertaken of 93 children with non-CF bronchiectasis defined by high resolution computed tomographic (HRCT) scanning presenting to a tertiary paediatric respiratory centre since 1996. RESULTS: Cases constituted 9.6% of all new referrals. Male to female ratio was 2:1. Median age at symptom onset was 1.1 years (range 0-16) and of HRCT diagnosis was 7.2 years (1.6-18.8). The most common referral diagnosis of asthma was refuted in 39 of 45 cases. Associations were previous pneumonic illness (30%), immunocompromise (21%), obliterative bronchiolitis (9%), congenital lung abnormality (5%), chronic aspiration (3%), eosinophilic oesophagitis (2%), familial syndrome (2%), primary ciliary dyskinesia (1%), and right middle lobe syndrome (1%). 8% had two associated diagnoses and 18% were idiopathic. There was agreement between the chest radiograph and HRCT scan for diagnosis and lobe affected in only five cases (5%). A repeat HRCT scan in 18 cases at a minimum interval of 18 months showed total resolution of the changes in six, improvement in one, progression in five, and was unchanged in six. CONCLUSIONS: Radiologically defined non-CF bronchiectasis in children is not uncommon. Diagnostic delay is a problem. The most common association is a previous pneumonia. Chest radiography is of little diagnostic value, but resolution is possible on HRCT scanning. Bronchiectasis is currently defined as a condition which is both permanent and progressive. This term is not necessarily appropriate for all paediatric patients for whom we suggest an alternative nomenclature.

Adolescent↗

Assessment of bronchiectasis by computed tomography.

Computed tomography and bronchography were used to assess the distribution of bronchiectasis in 15 lungs from eight patients with clinical features of the disease. Of the 36 lobes adequately displayed by bronchography, 22 were found to have bronchiectasis and 14 were found to be normal by both techniques. Cystic disease was readily identified by computed tomography but the cylindrical and varicose types of bronchiectasis could not be distinguished. Segmental localisation was less accurate, with agreement between computed tomography and bronchography in 116 out of 130 segments. It is concluded that with a modern high resolution scanner computed tomography provides a useful method of assessing lobar distribution in bronchiectasis.

Bronchiectasis↗

IgG subclasses in the serum and sputum from patients with bronchiectasis.

BACKGROUND: IgG subclass deficiency is often associated with recurrent pulmonary infections. The prevalence of deficiency in a large well characterised group of patients with bronchiectasis has not previously been established. METHODS: Serum IgG subclass concentrations in 89 patients with bronchiectasis were compared with those obtained from a group of 82 age and sex matched normal healthy controls. Sputum IgG subclass concentrations were also assessed in 44 of the patients. Albumin was measured as a marker of protein transudation from plasma to determine the degree of local IgG subclass production. RESULTS: The serum concentrations of IgG1, IgG2 and IgG3 were increased in the patients compared with the control group whereas IgG4 concentrations were not. There was an overall incidence of deficiency of 1% for subclasses 1-3 and 5% for subclass 4 in patients with bronchiectasis based on observed concentrations being below the lower limit of the control group range. The concentrations of IgG subclasses in sputum were partly dependent upon the degree of inflammation as assessed by the macroscopic appearance of purulence. A comparison of the ratio of sputum:serum subclass concentration and sputum:serum albumin, however, revealed that all of the subclasses were present at greater concentrations than could be accounted for by transudation alone. CONCLUSIONS: A new normal control range for serum IgG subclasses has been established and from this range it was found that IgG subclass deficiency in a group of unselected patients with bronchiectasis was comparatively rare. A significant degree of local IgG production was also suggested in the lungs of these patients.

Adult↗

Bullae, bronchiectasis and nutritional emphysema in severe anorexia nervosa.

STUDY OBJECTIVES: Pulmonary complications of anorexia nervosa are rarely documented. The case of a patient with anorexia nervosa and pulmonary disease is presented, a new quantitative computed tomography (CT) method for the detection of emphysema is employed, the literature is reviewed and the concept of 'nutritional' emphysema is discussed. RESULTS: The case of a 34-year-old, nonsmoking woman with long-standing severe anorexia nervosa who was evaluated for cough and progressive shortness of breath is reported. Pulmonary function testing showed a predominant restrictive pattern with a marked reduction in carbon monoxide transfer and respiratory muscle strength, and an elevated residual volume. Imaging revealed bullae and bronchiectasis, and quantitative analysis of the CT scan was consistent with mild, generalized emphysema. Bronchial washings grew Pseudomonas aeruginosa. Known causes for bronchiectasis were excluded. A literature review disclosed few reported noninfectious pulmonary complications of anorexia nervosa. CONCLUSIONS: To the authors' knowledge, this is the first report of bullae and bronchiectasis in a patient with anorexia nervosa, and the CT analysis was consistent with mild emphysema. Malnutrition has been associated with emphysematous changes in animals and may be the primary insult in the development of emphysema, bullae and bronchiectasis in the present patient.

Adult↗