PMID- 31614323 OWN - NLM STAT- MEDLINE DCOM- 20200807 LR - 20200807 IS - 1557-8615 (Electronic) IS - 0883-9441 (Linking) VI - 54 DP - 2019 Dec TI - Managing the Alpha-1 patient in the ICU: Adapting broad critical care strategies in AATD. PG - 212-219 LID - S0883-9441(19)30076-0 [pii] LID - 10.1016/j.jcrc.2019.08.027 [doi] AB - Alpha-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. CI - Copyright (c) 2019 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Rahaghi, Franck AU - Rahaghi F AD - Pulmonary and Critical Care Division, Cleveland Clinic Florida, Weston, Florida, United States. Electronic address: rahaghf@ccf.org. FAU - Omert, Laurel AU - Omert L AD - CSL Behring, King of Prussia, PA, United States. Electronic address: Laurel.Omert@cslbehring.com. FAU - Clark, Virginia AU - Clark V AD - Division of Gastroenterology, Hepatology, and Nutrition, University of Florida, Gainesville, Florida, United States. Electronic address: Virginia.Clark@medicine.ufl.edu. FAU - Sandhaus, Robert A AU - Sandhaus RA AD - Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, Denver, CO, United States. Electronic address: RSandhaus@alphanet.org. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20190829 PL - United States TA - J Crit Care JT - Journal of critical care JID - 8610642 SB - IM MH - Clinical Protocols MH - Critical Care MH - Humans MH - Intensive Care Units MH - Pulmonary Disease, Chronic Obstructive/complications/*therapy MH - alpha 1-Antitrypsin Deficiency/complications/*therapy OTO - NOTNLM OT - Alpha-1 Antitrypsin Deficiency OT - Cardiovascular disease OT - Critical care OT - Intensive care unit OT - Liver disease OT - Pulmonary disease, chronic obstructive EDAT- 2019/10/16 06:00 MHDA- 2020/08/08 06:00 CRDT- 2019/10/16 06:00 PHST- 2019/01/15 00:00 [received] PHST- 2019/07/17 00:00 [revised] PHST- 2019/08/27 00:00 [accepted] PHST- 2019/10/16 06:00 [pubmed] PHST- 2020/08/08 06:00 [medline] PHST- 2019/10/16 06:00 [entrez] AID - S0883-9441(19)30076-0 [pii] AID - 10.1016/j.jcrc.2019.08.027 [doi] PST - ppublish SO - J Crit Care. 2019 Dec;54:212-219. doi: 10.1016/j.jcrc.2019.08.027. Epub 2019 Aug 29.