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Clinical Images

A Patient with Alpha-1 Antitrypsin Deficiency Presenting with Dyspnea


Authors: Todd Thomas, DO, Carmen Vesbianu, MD

A 37-year-old man presented to the emergency department with shortness of breath and subjective fevers for 3 days.  He also complained of pain located in the middle of his chest, rated 5/10, worsened by cough and associated with wheezing. The patient’s past medical history was significant for alpha-1 antitrypsin deficiency and chronic obstructive pulmonary disease. Patient was a current smoker with a 20-pack-year history but denied alcohol or drug use. His vital signs were BP of 138/93, HR of 79, respiratory rate of 27, and oxygen saturation of 97% on room air. Home medications included budesonide-formoterol fumurate, tiotropium, and albuterol inhaler. A recent pulmonary function test showed severe pulmonary disease with FEV1 of 21% predicted.

 

Physical exam was significant for symmetric chest excursion, absence of subcutaneous emphysema, decreased breath sounds to auscultation on the right side, and extensive expiratory wheezing bilaterally. Blood work was unremarkable. A chest X-ray and a CT-chest were ordered (Figures 1 and 2).

 

MCQ 1: This patients X- ray is consistent with:

  1. Tension Pneumothorax
  2. Giant bullae
  3. Cavitary masses
  4. Bronchiectasis

MCQ 2: What is the proper management for this patient?

  1. Smoking cessation
  2. Optimizing medical therapy and supportive care
  3. Chest tube placement
  4. Bullectomy

Click here for MCQ Answers and Discussion

Author Bios:

 

Dr. Todd Thomas is a PGY-2 Internal Medicine Resident at the University Of Oklahoma School Of Community Medicine. He is from Fort Smith, Arkansas, but completed his bachelors, MBA, and medical degree all in Tulsa, OK. He is married to his beautiful wife, Scarlett, and has a spunky one-year-old daughter. Dr. Thomas plans to pursue a Cardiovascular Disease fellowship followed by Interventional Cardiology.

 

Dr. Carmen Vesbianu is an Assistant Professor at the University Oklahoma School of Community Medicine in Tulsa.