This educational activity was made possible in part by educational grants from the following:

Baxter Healthcare Corporation

CSL Behring LLC

Grifols

Faculty Disclosures:

The following faculty and planners declare that they, or their spouses/partners, do not have any relevant financial relationship in any amount with a commercial interest to disclose:

Brian W. Carlin, M.D.

Charles U. Gbadouwey, M.D.

Chanda Nicole Holsey, Dr.PH, M.P.H.

Colin C. Syphax

Alpha-one-antitrypsin deficiency predisposes to lung disease (eg: emphysema and bronchiectasis), liver disease (eg: chronic hepatitis, cirrhosis, and hepatoma), skin disease (eg: panniculitis), and vasculitis (eg: C-ANCA vasculitis such as Wegnener granulomatosis). This review will focus largely on  Alpha-one-Antitrypsin  as a predisposing factor for early onset lung disease, generally occurring in the fourth to fifth decades of life. Individuals with AAT deficiency present with the usual signs and symptoms of COPD, including dyspnea, productive cough, and wheezing  in the presence of bronchitis.  Imaging tend to show panacinar emphysema with  involvement of the lower lobes of the lung, as opposed to destruction of the upper lobes of the lungs in typical emphysema. Spirometry reveals an obstructive pattern with an FEV1/FVC ratio less than 70. There may be partial reversibility with bronchodilators as indicated by a 12% and 200ml rise in the FEV1 or FVC postbronchodilator. It is therefore important for primary care providers to screen for this condition with the use of spirometry.

Pulmonary hypertension is pathophysiological and hemodynamic state associated with several clinical conditions that can lead to progressive right ventricular failure and death.  It is often misdiagnosed and when diagnosed it is often delayed.  While it is complex and difficult to treat, new knowledge about the pathophysiology has led to developments in diagnostic and treatment options that can be used to reduce symptoms and prolong life.

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