Alpha 1-antitrypsin deficiency: evaluation of bronchiectasis with CT.

King, Mark A.; Stone, J A; Diaz, Philip T.; Mueller, C.; Becker, William; Gadek, James E.

doi:10.1148/radiology.199.1.8633137

Cited by 118 publications

(43 citation statements)

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“…Earlier reports of the incidence of bronchiectasis in A1AT-deficient individuals were as high as 43%, although based on Review small study populations [6,57]. In a larger study of 74 (PiZ) subjects, Parr et al reported the incidence of 'clinically relevant' bronchiectasis to be 27%, which is similar to the reported incidence in usual COPD [58].…”

Section: Bronchiectasismentioning

confidence: 58%

Recent advances in α-1-antitrypsin deficiency-related lung disease

Brebner¹,

Stockley²

2013

Expert Review of Respiratory Medicine

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Section: Bronchiectasismentioning

confidence: 58%

Recent advances in α-1-antitrypsin deficiency-related lung disease

Brebner¹,

Stockley²

2013

Expert Review of Respiratory Medicine

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“…The prevalence of bronchiectasis in patients with AAT deficiency varies greatly between studies, probably because other aetiologies were not studied in many of them [59,60]. Screening of AAT deficiency is not recommended in the study of bronchiectasis, except in cases in which emphysema on chest HRCT is identified, particularly in a panlobular or basal distribution [2].…”

Section: Alpha-1-antitrypsin (Aat) Deficiencymentioning

confidence: 99%

Diagnostic challenges of bronchiectasis

Suárez-Cuartín

Chalmers

Sibila

2016

Respiratory Medicine

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Bronchiectasis is a condition of increasing incidence and prevalence around the world.Many different diseases have been associated with bronchiectasis, and their treatment can differ widely. Recent guidelines have helped to approach aetiological diagnosis but it is still a complex process. Identifying the cause of the bronchiectasis may determine a change in the treatment of a large group of subjects. That is one of the main reasons why the aetiological diagnosis is crucial in the proper management of bronchiectasis patients.Postinfectious bronchiectasis is the most frequent entity among different studies, but a high percentage of cases still remain without a clear aetiology. Bronchiectasis related to allergic bronchopulmonary aspergillosis (ABPA), immunodeficiencies with antibody production deficiency, primary ciliary dyskinesia, cystic fibrosis and alpha-1-antitrypsin deficiency, among others, require a specific management that may improve quality of life and prognosis in a large group of individuals. Therefore, the aim of this article is to review the main bronchiectasis related diseases and to simplify the aetiological diagnosis, in order to improve the management of bronchiectasis patients, especially in those where a specific treatment is available.

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“…AATD is associated with a substantially increased risk for the development of COPD, often by the third or fourth decade, and is also associated with risks for development of liver disease (Sharp et al 1969;Lieberman, Mittman, and Gordon 1972;Sveger 1976), cutaneous panniculitis (Edmonds, Hodge, and Rietschel 1991), bronchiectasis (King et al 1996), vasculitis (Lewis et al 1985), and Wegener's granulomatosis (Barnett, Sekosan, and Khurshid 1999). AATD is characterised by misfolding of the AAT protein and belongs to a class of genetic diseases underpinned by aberrant protein folding collectively termed conformational disorders (Gooptu and Lomas 2009;Greene et al 2008).…”

Section: History and Clinical Featuresmentioning

confidence: 99%