A RARE CASE OF GIANT EMPHYSEMATOUS BULLAE IN LEFT LUNG DUE TO ALPHA 1- ANTI-TRYPSIN DEFICIENCY

Introduction: Alpha-1-antitrypsin deficiency (AATD) is a genetic disorder that manifests as pulmonary emphysema, liver cirrhosis and, rarely, as the skin disease panniculitis, and is characterized by low serum levels of AAT, the main protease inhibitor (PI) in human serum. The prevalence in Western Europe and in the USA is estimated at approximately 1 in 2,500 and 1: 5,000 newborns. Environmental factors such as cigarette smoking, and dust exposure are additional risk factors and have been linked to an accelerated progression of this condition.Cirrhotic liver failure may occur around age 50. The diagnosis can be established by detection of low serum levels of AAT and isoelectric focusing.

Case Report:A 22 years old male presented with complaint of Breathlessness since 6 month, Pain over left chest since 4months. Patient was chronic smoker from past 7 years and used to smoke 12 cigarettes daily.Past history suggested of Pulmonary Tuberculosis 6 years back for which he took Anti-tubercular treatment category I for 6 Months. CT chest was suggestive of middle sections of the left lungs contains gigantic bullae over ½ of hemithorax volume. Pulmonary parenchyma in lower sections was preserved, but emphysematous and Fibrotic Patch and Similar Emphysematous Bullae on right side. Serum Alpha-1-antitrypsin  level was 0.2g/L.Antero-lateral skin incision was taken a large Emphysematous Bullae found and excision of that part of lung lobe was done.one month follow up xray was done in which there was expansion of left lung was seen.patient kept under regular follow up and even after two year lung showed expansion with no recurrence of bullae.

Conclusion:In conclusion, surgical resection of giant emphysematous bullae and appropriately prescribed therapy (drug treatment and hygiene regime) allowed us to achieve a positive effect and maintain the patient’s life quality for a long time.