PMC6010612_01
PMC6010612_02
Genealogic tree of the Moroccan family with AATD (Q0cairo mutation). The arrow signifies the proband. Dark circles indicate the affected individuals.
gr1.jpg
PMC6010612_02_gr1.jpg
fig1
The genealogical tree is shown in Fig. 1.
Computed tomography of the chest in the index case at admission (a) showing bilateral basal bronchiectasis and pneumothorax and, at the age of 24 (b) showing diffuse bronchiectasis.
gr2.jpg
PMC6010612_03_gr2.jpg
fig2
The patient is currently 29 years-old; the latest CT showed more diffuse bronchiectasis (Fig. 2b), FRA revealed a severe restrictive syndrome with 46% of forced vital capacity (FVC) which was no longer sensitive to beta2 agonists.
PMC6010612_03
Computed tomography of the chest in the index case at admission (a) showing bilateral basal bronchiectasis and pneumothorax and, at the age of 24 (b) showing diffuse bronchiectasis.
gr2.jpg
PMC6010612_03_gr2.jpg
fig2
The patient is currently 29 years-old; the latest CT showed more diffuse bronchiectasis (Fig. 2b), FRA revealed a severe restrictive syndrome with 46% of forced vital capacity (FVC) which was no longer sensitive to beta2 agonists.
PMC6010612_04