Aetiology of chronic suppurative lung disease.

Nikolaizik, Wilfried H.; Warner, J. O.

doi:10.1136/adc.70.2.141

Cited by 113 publications

(61 citation statements)

References 6 publications

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“…It is difficult to know whether these subjects had severe or repeated episodes of respiratory sepsis leading to permanent bronchial damage that were not recalled, or whether they had an unidentified intrinsic impairment to host defence that [19] reviewed 41 cases of bronchiectasis who presented with chronic productive cough, and no cause was found in 37% of their study group. Forty-eight per cent of studied subjects from a New Zealand cohort, despite extensive investigations, had no known cause for their bronchiectasis [20].…”

Section: Discussionmentioning

confidence: 99%

Non-CF bronchiectasis: does knowing the aetiology lead to changes in management?

Sonnappa²,

Lex³

et al. 2005

European Respiratory Journal

181

167

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The aim of the current study was to review the aetiology of non-cystic fibrosis (CF) bronchiectasis from two tertiary paediatric respiratory units in order to determine how often making a specific aetiological diagnosis leads to a change in management, and to assess the contribution of computed tomography (CT) in determining the underlying diagnosis.The case records of all patients who were diagnosed as having bronchiectasis by CT, currently being seen at the Royal Brompton Hospital and Great Ormond Street Hospital for Children (London, UK), were reviewed. All patients had undergone extensive investigations, and the underlying aetiology and the area of pulmonary involvement (as seen on CT) were recorded. A total of 136 patients were identified; there were 65 young males and the group median (range) age was 12.1 yrs (3.1-18.1). Immunodeficiency, aspiration and primary ciliary dyskinesia accounted for 67% of the cases. In 77 (56%) children, the identification of a cause led to a specific change in management. There was no association between aetiology and the distribution of CT abnormalities.In conclusion, immunodeficiency and other intrinsic abnormalities account for the majority of cases of non-cystic fibrosis bronchiectasis seen in the current authors' units. Computed tomography scans do not contribute towards identifying the aetiology and, most importantly, a specific aetiological diagnosis frequently leads to a change in management.

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Section: Discussionmentioning

confidence: 99%

Non-CF bronchiectasis: does knowing the aetiology lead to changes in management?

Sonnappa²,

Lex³

et al. 2005

European Respiratory Journal

181

167

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“…The incidence of bronchiectasis in other rare PIDs such as hyper-IgE syndrome (<2.5% in children and very rare in adults) [74,84,85], phagocyte defects (<1%-10% in children and <1% in adults) [86,87], and transporter antigen peptide deficiency (rare in children and very rare in adults) [87,88] is low. The microorganisms associated with bronchiectasis in patients with PID are shown in the Table. High-resolution computed tomography (HRCT) is considered a reliable test for assessing bronchiectasis in patients with PID [89][90][91].…”

Section: Bronchiectasismentioning

confidence: 99%

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Yazdani

Abolhassani

et al. 2017

J Investig Allergol Clin Immunol

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Primary immunodeficiency disorders (PIDs) are caused by 1 or more defects of the immune system. Patients are more likely to experience recurrent and/or severe infections and tend to develop a wide range of complications. Respiratory diseases are the main and initial manifestation in most cases and the most common complication. Pulmonary complications cause significant morbidity and mortality in patients with PIDs. Early diagnosis and appropriate treatment can prevent or at least slow the development of respiratory complications. Since the spectrum of pulmonary complications in PIDs is broad, we divided pulmonary complications into upper respiratory complications (eg, sinusitis, otitis media, and laryngeal angioedema) and lower respiratory complications (eg, pneumonia, bronchitis, bronchiectasis, interstitial lung diseases, organizing pneumonia, pulmonary adenopathies and malignancies, hyperreactive airway diseases, pulmonary dysgenesis, and adverse reactions to treatment). This review covers the main respiratory manifestations in patients with PIDs. Key words: Primary immunodeficiency disorders. Pulmonary complications. Upper respiratory tract. Lower respiratory tract. ResumenLas inmunodeficiencias primarias (PIDs) son enfermedades causadas por uno o más defectos del sistema inmunológico. Estos pacientes presentan con frecuencia infecciones recidivantes y/o severas así como otro tipo de complicaciones. Las patologías respiratorias son la principal y más frecuente manifestación y complicación de las PIDs. Las complicaciones de estas patologías pulmonares constituyen una de las principales causas de morbimortalidad entre los pacientes que sufren PIDs. El diagnóstico temprano y el tratamiento adecuado pueden prevenir, o al menos retrasar, la aparición de las complicaciones respiratorias en estos pacientes. Dado que el espectro de las enfermedades pulmonares es muy amplio, hemos dividido estas complicaciones entre aquellas que afectan a las vías aéreas superiores (sinusitis, otitis media y angioedema laríngeo, etc.) y las que afectan a las vías aéreas bajas (neumonía, bronquitis, bronquiectasias, enfermedades pulmonares intersticiales, neumonía organizada, adenopatías pulmonares y neoplasias, hiperreactividad bronquial, disgenesia pulmonar y las debidas a los efectos secundarios del tratamiento instaurado). Este artículo revisa las manifestaciones respiratorias que se observan más frecuentemente en los pacientes con PIDs. Palabras clave: Inmunodeficiencias primarias. Complicaciones pulmonares. Vías respiratorias altas. Vías respiratorias bajas.

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“…Postinfectious bronchiectasis in the normal host is becoming distinctly uncommon, and, in developed countries, most patients with the disorder have an underlying systemic illness. An underlying cause of the disease was identified in 40–63% of patients in different studies [3,4,5]. Several studies reported a higher incidence of non-CF bronchiectasis in different minority groups, which was thought to be related to low socioeconomic status [6,7,8].…”

Section: Introductionmentioning

confidence: 99%

Non-Cystic-Fibrosis Bronchiectasis in Children: A Persisting Problem in Developing Countries

et al. 2005

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Background: Non-cystic-fibrosis (non-CF) bronchiectasis in childhood is still one of the most common causes of childhood morbidity in developing countries. The management of these patients remains problematic, and there are few studies of long-term outcome. Objective: The aim of this retrospective study was to define the general characteristics, underlying causative factors and long-term follow-up results of non-CF bronchiectasis patients. Methods: One hundred and eleven consecutive children, diagnosed with non-CF bronchiectasis were included in the study. General characteristics and underlying causes were recorded from the medical records. Clinical outcomes were evaluated in terms of lung function tests, annual exacerbation rates and patient/parent perception of health status. Results: Mean age of the patients was 7.4 ± 3.7 years at presentation, and patients had been followed 4.7 ± 2.7 years on average. In 62.2% of the patients, an underlying etiology was identified, whereas postinfectious bronchiectasis was the most common (29.7%). In spite of intensive medical treatment, 23.4% of the patients required surgery. The annual lower respiratory infection rate has decreased from a mean of 6.6 ± 4.0 to 2.9 ± 2.9 during follow-up (p < 0.0001). Lung function tests were also found to be improved (mean FEV₁% 63.3 ± 21.0 vs. 73.9 ± 27.9; p = 0.01; mean FVC% 68.1 ± 22.2 vs. 74.0 ± 24.8; p = 0.04). There was clinical improvement in both the surgical (73%) and medical (70.1%) groups (p > 0.05). Conclusion: In conclusion, bronchiectasis remains a disease of concern to pediatricians, particularly in developing countries. Infections are still important causes of bronchiectasis, and clinical improvement can be achieved by appropriate treatment. Although medical treatment is the mainstay of management, surgery should be considered in selected patients.

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Aetiology of chronic suppurative lung disease.

Cited by 113 publications

References 6 publications

Non-CF bronchiectasis: does knowing the aetiology lead to changes in management?

Non-CF bronchiectasis: does knowing the aetiology lead to changes in management?

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Non-Cystic-Fibrosis Bronchiectasis in Children: A Persisting Problem in Developing Countries

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