TY  - JOUR
AU  - Rottoli, Paola
AU  - Cameli, Paolo
AU  - Bennett, David
AU  - Fossi, Antonella
AU  - Bargagli, Elena
AU  - Lorenzetti, Victoria
PY  - 2018
DA  - 2018/12/18
TI  - Alpha-1 Antitrypsin Deficiency and Lung Transplantation: A Clinical Case
JO  - OBM Transplantation
SP  - 033
VL  - 02
IS  - 04
AB  - Background: Alpha-1-antitrypsin (AAT) deficiency is the most common hereditary disorder in adults and is associated with an increased risk of developing lung emphysema.
Methods:Here, we report the case of a 54-year-old man, who underwent bilateral lung transplantation due to AAT deficiency emphysema in the general hospital of Siena, Italy.
Results:The patient was diagnosed with partial AAT deficiency (189 mg/dL) associated with the PIMZ genotype; although he quit smoking, his lung disease progressed to chronic respiratory failure. The patient received a lung transplant and was alive 10 months later.
Conclusions:We consider this to be an interesting clinical case regarding the opportunity to implement augmentation therapy, not only to preserve native lungs, but also to prevent chronic lung allograft dysfunction.
SN  - 2577-5820
UR  - https://doi.org/10.21926/obm.transplant.1804033
DO  - 10.21926/obm.transplant.1804033
ID  - Rottoli2018
ER  -