• Franck Rahaghi, Laurel Omert, Virginia Clark, and Robert A Sandhaus.
• Pulmonary and Critical Care Division, Cleveland Clinic Florida, Weston, Florida, United States. Electronic address: rahaghf@ccf.org.
• J Crit Care. 2019 Dec 1; 54: 212-219.

AbstractAlpha-1 Antitrypsin Deficiency (AATD) is a progressive pulmonary disease under-recognized or misdiagnosed by clinicians. Patients with AATD can develop a variety of organ-specific complications and as a result, often require hospitalization and treatment within critical care and ICU settings. Due to the complexity of AATD there are minimal guidelines in place to address the specific and highly variable needs of these patients in the critical care setting. This review presents clinical considerations with respect to the management of patients with AATD and provides treatment recommendations for these patients in the critical care setting. In addition, we have outlined certain aspects of the care of this patient population that may be of interest to critical care practitioners. With greater disease awareness and earlier diagnosis the onset of symptoms can be delayed, which will ultimately reduce the frequency of deleterious health consequences.Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.

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