What are the dietary recommendations in the management of alpha1-antitrypsin deficiency (AATD)?

Updated: Sep 11, 2020
  • Author: Dora E Izaguirre Anariba, MD, MPH; Chief Editor: John J Oppenheimer, MD  more...
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Answer

Patients with advanced COPD are characterized by a significant reduction in fat-free muscle mass. This pulmonary cachexia is common in patients with alpha1-antitrypsin deficiency (AATD) and is associated with a decline in clinical status and a harbinger of mortality in emphysema. The syndrome is a result of multiple factors, including hypermetabolism, drug therapy, inactivity, and aging. Prolonged glucocorticoid administration accelerates the process.

Protein-calorie supplementation, as one component of a comprehensive treatment program, may reverse the loss of muscle mass, and dietary counseling may aid patients at high nutritional risk. Adding fat-based nonprotein calories may benefit patients with respiratory failure who are receiving mechanical ventilation. However, other than this special circumstance, little evidence exists to suggest that this dietary manipulation aids ambulatory patients.


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