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: α1-antitrypsin deficiency is the most common hereditary disorder in adults and is associated to an increased risk of developing lung emphysema. METHODS: With this case report, we describe the case of a 54 years old patient, who underwent bilateral lung transplantation due to alpha-1 antitrypsin deficiency emphysema. 
RESULTS: The patient was diagnosed with partial alpha-1 antitrypsin deficiency (189 mg/dl) associated to PIMZ genotype; although he quitted smoking, lung disease progressively deteriorates to chronic respiratory failure. Now the patient is alive 10 months after lung transplant. 
CONCLUSIONS: We consider it 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  -