Alpha-1 antitrypsin deficiency

Patients with lung disease require pulmonologist evaluation for any exacerbations of COPD. Initial evaluation should include full pulmonary function testing (volumes, flows, diffusion).[38]Sandhaus RA, Turino G, Brantly ML, et al. The diagnosis and management of alpha-1 antitrypsin deficiency in the adult. Chronic Obstr Pulm Dis (Miami). 2016 Jun 6;3(3):668-82. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5556762 http://www.ncbi.nlm.nih.gov/pubmed/28848891?tool=bestpractice.com A baseline CT of the chest is recommended. When well controlled, patients require annual follow-up with spirometry testing in order to assess pulmonary function changes. Annual CT is not suggested.

Individuals with liver disease require regular evaluations with liver function tests, whether symptomatic or asymptomatic.[38]Sandhaus RA, Turino G, Brantly ML, et al. The diagnosis and management of alpha-1 antitrypsin deficiency in the adult. Chronic Obstr Pulm Dis (Miami). 2016 Jun 6;3(3):668-82. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5556762 http://www.ncbi.nlm.nih.gov/pubmed/28848891?tool=bestpractice.com Annual liver ultrasound is recommended.