Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation

Search Page

Filters

My NCBI Filters

Results by year

Table representation of search results timeline featuring number of search results per year.

Year Number of Results
1973 1
1974 1
1975 3
1976 2
1977 2
1978 5
1979 3
1980 2
1981 5
1982 6
1983 9
1984 9
1985 8
1986 8
1987 8
1988 11
1989 13
1990 13
1991 17
1992 13
1993 12
1994 14
1995 12
1996 14
1997 18
1998 15
1999 8
2000 30
2001 16
2002 17
2003 19
2004 17
2005 16
2006 18
2007 18
2008 27
2009 32
2010 27
2011 18
2012 31
2013 29
2014 25
2015 35
2016 27
2017 46
2018 34
2019 25
2020 25
2021 22
2022 22
2023 17
2024 3

Text availability

Article attribute

Article type

Publication date

Search Results

784 results

Results by year

Filters applied: . Clear all
Page 1
Alpha(1)-Antitrypsin Deficiency.
Strnad P, McElvaney NG, Lomas DA. Strnad P, et al. N Engl J Med. 2020 Apr 9;382(15):1443-1455. doi: 10.1056/NEJMra1910234. N Engl J Med. 2020. PMID: 32268028 Review. No abstract available.
Alpha-1 antitrypsin deficiency: outstanding questions and future directions.
Torres-Durán M, Lopez-Campos JL, Barrecheguren M, Miravitlles M, Martinez-Delgado B, Castillo S, Escribano A, Baloira A, Navarro-Garcia MM, Pellicer D, Bañuls L, Magallón M, Casas F, Dasí F. Torres-Durán M, et al. Orphanet J Rare Dis. 2018 Jul 11;13(1):114. doi: 10.1186/s13023-018-0856-9. Orphanet J Rare Dis. 2018. PMID: 29996870 Free PMC article. Review.
BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is a rare hereditary condition that leads to decreased circulating alpha-1 antitrypsin (AAT) levels, significantly increasing the risk of serious lung and/or liver disease in children and adults, in whi …
BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is a rare hereditary condition that leads to decreased circula …
Emphysema: looking beyond alpha-1 antitrypsin deficiency.
Janssen R, Piscaer I, Franssen FME, Wouters EFM. Janssen R, et al. Expert Rev Respir Med. 2019 Apr;13(4):381-397. doi: 10.1080/17476348.2019.1580575. Epub 2019 Feb 22. Expert Rev Respir Med. 2019. PMID: 30761929 Review.
Emphysema is a COPD-phenotype characterized by destruction of lung parenchyma. Alpha-1 antitrypsin deficiency (AATD) is a genetic cause of emphysema, whereas smoking is the most important risk factor of non-AATD emphysema. A general underappreciation o …
Emphysema is a COPD-phenotype characterized by destruction of lung parenchyma. Alpha-1 antitrypsin deficiency (A …
α1-Antitrypsin deficiency.
Greene CM, Marciniak SJ, Teckman J, Ferrarotti I, Brantly ML, Lomas DA, Stoller JK, McElvaney NG. Greene CM, et al. Nat Rev Dis Primers. 2016 Jul 28;2:16051. doi: 10.1038/nrdp.2016.51. Nat Rev Dis Primers. 2016. PMID: 27465791 Review.
Alpha-1 antitrypsin deficiency: current therapy and emerging targets.
McElvaney OF, Fraughen DD, McElvaney OJ, Carroll TP, McElvaney NG. McElvaney OF, et al. Expert Rev Respir Med. 2023 Mar;17(3):191-202. doi: 10.1080/17476348.2023.2174973. Epub 2023 Mar 15. Expert Rev Respir Med. 2023. PMID: 36896570 Review.
EXPERT OPINION: With a relatively small population able to participate in clinical studies, increased awareness and diagnosis of AATD is urgently needed. Better, more sensitive clinical parameters will assist in the generation of acceptable and robust evidence of th …
EXPERT OPINION: With a relatively small population able to participate in clinical studies, increased awareness and diagnosis of AATD …
Fazirsiran for Liver Disease Associated with Alpha(1)-Antitrypsin Deficiency.
Strnad P, Mandorfer M, Choudhury G, Griffiths W, Trautwein C, Loomba R, Schluep T, Chang T, Yi M, Given BD, Hamilton JC, San Martin J, Teckman JH. Strnad P, et al. N Engl J Med. 2022 Aug 11;387(6):514-524. doi: 10.1056/NEJMoa2205416. Epub 2022 Jun 25. N Engl J Med. 2022. PMID: 35748699 Clinical Trial.
Fibrosis regression was observed in 7 of 15 patients and fibrosis progression in 2 of 15 patients after 24 or 48 weeks. There were no adverse events leading to trial or drug discontinuation. Four serious adverse events (viral myocarditis, diverticulitis, dyspnea, and vesti …
Fibrosis regression was observed in 7 of 15 patients and fibrosis progression in 2 of 15 patients after 24 or 48 weeks. There were no advers …
Alpha-1 antitrypsin deficiency-associated panniculitis.
Franciosi AN, Ralph J, O'Farrell NJ, Buckley C, Gulmann C, O'Kane M, Carroll TP, McElvaney NG. Franciosi AN, et al. J Am Acad Dermatol. 2022 Oct;87(4):825-832. doi: 10.1016/j.jaad.2021.01.074. Epub 2021 Jan 29. J Am Acad Dermatol. 2022. PMID: 33516773 Free article. Review.
BACKGROUND: Panniculitis represents a rare and potentially lethal manifestation of alpha-1 antitrypsin deficiency (AATD). Evidence regarding management is limited to case reports and small case series. ...SEARCH METHODOLOGY: Articles and abstracts publ …
BACKGROUND: Panniculitis represents a rare and potentially lethal manifestation of alpha-1 antitrypsin deficiency …
Augmentation Therapy for Severe Alpha-1 Antitrypsin Deficiency Improves Survival and Is Decoupled from Spirometric Decline-A Multinational Registry Analysis.
Fraughen DD, Ghosh AJ, Hobbs BD, Funk GC, Meischl T, Clarenbach CF, Sievi NA, Schmid-Scherzer K, McElvaney OJ, Murphy MP, Roche AD, Clarke L, Strand M, Vafai-Tabrizi F, Kelly G, Gunaratnam C, Carroll TP, McElvaney NG. Fraughen DD, et al. Am J Respir Crit Care Med. 2023 Nov 1;208(9):964-974. doi: 10.1164/rccm.202305-0863OC. Am J Respir Crit Care Med. 2023. PMID: 37624745 Free PMC article.
Rationale: Intravenous plasma-purified alpha-1 antitrypsin (IV-AAT) has been used as therapy for alpha-1 antitrypsin deficiency (AATD) since 1987. Previous trials (RAPID and RAPID-OLE) demonstrated efficacy in preserving computed tomography of lung den …
Rationale: Intravenous plasma-purified alpha-1 antitrypsin (IV-AAT) has been used as therapy for alpha-1 antitrypsin …
α1-Antitrypsin Deficiency.
Hatipoğlu U, Stoller JK. Hatipoğlu U, et al. Clin Chest Med. 2016 Sep;37(3):487-504. doi: 10.1016/j.ccm.2016.04.011. Epub 2016 Jun 25. Clin Chest Med. 2016. PMID: 27514595 Review.
Alpha1-antitrypsin review.
Stockley RA. Stockley RA. Clin Chest Med. 2014 Mar;35(1):39-50. doi: 10.1016/j.ccm.2013.10.001. Epub 2013 Dec 12. Clin Chest Med. 2014. PMID: 24507836 Review.
The genetic defects leading to deficiency have been elucidated and the pathophysiologic processes, clinical variation in phenotype, and the role of genetic modifiers have been recognized. ...
The genetic defects leading to deficiency have been elucidated and the pathophysiologic processes, clinical variation in phenotype, a …
784 results