A final smaller cohort can be identified due to perinatal jaundic

A final smaller cohort can be identified due to perinatal jaundice, a recognised presenting feature of AATD [27] providing an opportunity for long term monitoring and earlier detection of deteriorating lung function. This wide range of presenting age from and features provides the managing physician with a challenge in determining the best care, monitoring and, importantly, deciding whether or when to introduce augmentation therapy. Such therapy cannot be expected to improve already damaged lungs and leads to the strategy of either preventing the development of lung pathology or stabilising that already present. Currently augmentation is aimed at the latter approach of stabilising the established lung disease and thereby preventing future progression.

It is recognised that at presentation with established disease, especially at a young age, the preceding period of the patients life must have been characterised by a decline in lung function that was in excess of the normal aging process. The first step in management however must be to stop smoking or endorse recent cessation, if that is the status. Usual management for COPD, such as bronchodilators, are prescribed or continued in order to maximise airflow physiology and/or reduce exacerbation frequency as in usual COPD. Indeed reversibility and exacerbations have been recognised as factors that influence spirometric [14,19] and gas transfer [19] decline in AATD and although few clinical trial data are available of the usual symptomatic and preventative therapies, they seem to be effective in AATD [28].

A summary of factors that have been associated with decline in FEV1 in patients with AATD is presented in Table 1. Table 1 Factors influencing the natural course of emphysema in patients with AATD Where available, augmentation is usually prescribed especially if the FEV1 is in range of 35-60% predicted (as suggested by the observational NIH study), where spirometric benefit has best been demonstrated. However, despite the preceding decline in spirometry being obviously excessive prior to diagnosis, after the cessation of smoking and introduction of usual therapy for COPD there is no certainty that disease progression will continue, particularly in patients with no other recognised risk factors for progression (e.g. professional exposure to dusts and fumes, bronchial hyperresponsiveness, frequent exacerbations, etc.) [14,19,32,36-39]. Measurement of lung physiology is complex Cilengitide and single measures are subject to patient effort (even for simple spirometry) and day to day variability.

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