Results 1 to 10 of about 177,414 (289)

Alpha-1 antitripsyn deficiency and augmentation therapy in pregnancy: two case reports [PDF]

Frontiers in Medicine
Alpha-1 antitrypsin deficiency (AATD) is an inherited condition characterized by reduced plasma levels of alpha-1 antitrypsin (AAT), often leading to pulmonary diseases primarily emphysema and/or chronic obstructive pulmonary disease (COPD), but also ...
Anna Annunziata, Giuseppe Fiorentino, Antonietta Coppola, Rosa Cauteruccio, Laura Ferrentino, Luigi Fiorentino, Cecilia Calabrese   +6 more
doaj   +4 more sources

Lung transplant outcomes for recipients with alpha-1 antitrypsin deficiency, by use of alpha-1 antitrypsin augmentation therapy [PDF]

JHLT Open
Background: For patients with alpha-1 antitrypsin (AAT) deficiency, AAT augmentation therapy can be an important part of care. However, for those who require a lung transplant (LT), there is currently only limited information to guide the use of AAT ...
Atharv V. Oak, MEng, Jessica M. Ruck, MD, PhD, Alfred J. Casillan, MD, PhD, Armaan F. Akbar, BS, Ramon A. Riojas, MD, PhD, Pali D. Shah, MD, Jinny S. Ha, MD, MHS, Sara Strout, PharmD, Allan B. Massie, PhD, Dorry L. Segev, MD, PhD, Christian A. Merlo, MD, MPH, Errol L. Bush, MD   +11 more
doaj   +4 more sources

Long Non-Coding RNA Expression in Alpha-1 Antitrypsin Deficient Monocytes Pre- and Post-AAT Augmentation Therapy [PDF]

Non-Coding RNA, 2023
Long non-coding RNAs (lncRNAs) regulate gene expression. Their expression in alpha-1 antitrypsin (AAT) deficiency has not been investigated. Treatment of AAT deficiency involves infusion of plasma-purified AAT and this augmentation therapy has previously
Stephen G. J. Smith, Catherine M. Greene
doaj   +4 more sources

Diagnosis and augmentation therapy for alpha-1 antitrypsin deficiency: current knowledge and future potential [PDF]

Drugs in Context, 2023
The underdiagnosis of alpha-1 antitrypsin (AAT) deficiency (AATD) has been recognized for many years, yet little progress has been made in treatment of the disease.
Paulo Henrique Feitosa
doaj   +2 more sources

Progression and Augmentation Therapy in PiSZ and PiZZ Alpha-1 Antitrypsin Deficiency: A Longitudinal Functional and Densitometric Study [PDF]

Biomolecules
Background: Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder associated with an increased risk of developing chronic obstructive pulmonary disease (COPD) with variable phenotypic expression among different genotypes. While the PiZZ genotype is
Soha Esmaili, Juan Luis Rodríguez Hermosa, Gianna Vargas Centanaro, José Luis Álvarez-Sala, Iman Esmaili, Myriam Calle Rubio   +5 more
doaj   +2 more sources

Opinions and Attitudes of Pulmonologists About Augmentation Therapy in Patients with Alpha-1 Antitrypsin Deficiency. A Survey of the EARCO Group [PDF]

International Journal of COPD, 2022
Timm Greulich, 1,* Anna Albert, 1,* Werner Cassel, 2 Tobias Boeselt, 1 Erika Peychev, 1 Andreas Klemmer, 1 Francini Ferrerira, 3 Christian Clarenbach, 4 Maria L Torres-Duran, 5, 6 Alice M Turner, 7 Marc Miravitlles 8 1University Medical Centre Giessen ...
Greulich T, Albert A, Cassel W, Boeselt T, Peychev E, Klemmer A, Ferrerira F, Clarenbach C, Torres-Duran ML, Turner AM, Miravitlles M   +10 more
doaj   +2 more sources

Augmentation therapy with human alpha-1-proteinase inhibitor reduces exacerbations in patient with bronchiectasis and alpha-1-antitrypsin deficiency [PDF]

Respiratory Medicine Case Reports, 2022
Alpha-1-antitrypsin deficiency (AATD) is a rare cause of noncystic fibrosis (CF) bronchiectasis. The benefits of augmentation therapy in patients with chronic obstructive pulmonary disease (COPD) and pulmonary emphysema are well established.
Emanuel Buck, Maria Ada Presotto, Judith Brock, Kai Schlamp, Martina Veith, Felix J.F. Herth, Franziska Christina Trudzinski   +6 more
doaj   +2 more sources

Efficacy of alpha1-antitrypsin augmentation therapy in conditions other than pulmonary emphysema [PDF]

Orphanet Journal of Rare Diseases, 2011
Up to now alpha 1-antitrypsin (AAT) augmentation therapy has been approved only for commercial use in selected adults with severe AAT deficiency-related pulmonary emphysema (i.e.
de Serres Frederick, Lara Beatriz, Blanco Ignacio   +2 more
doaj   +2 more sources

Long-term effect of α1-antitrypsin augmentation therapy on the decline of FEV1 in deficient patients: an analysis of the AIR database [PDF]

ERJ Open Research, 2021
Background Patients with ZZ (Glu342Lys) α-1-antitrypsin deficiency (ZZ-AATD) who received augmentation therapy with α-1-antitrypsin (AAT) in randomised controlled trials over 2–3 years failed to show a significant reduction of the annual decline of ...
Iris G.M. Schouten, Marise J. Kasteleyn, Roula Tsonaka, Robert Bals, Alice C. Turner, Ilaria Ferrarotti, Angelo G. Corsico, Beatriz Lara, Marc Miravitlles, Robert A. Stockley, Jan Stolk   +10 more
doaj   +2 more sources

Personalised indication of augmentation therapy for emphysema associated with severe alpha-1 antitrypsin deficiency: a case series [PDF]

Therapeutic Advances in Respiratory Disease
Severe alpha-1 antitrypsin deficiency (AATD) is associated with an increased risk of emphysema. However, the clinical manifestations are very heterogeneous, and an individual prognosis is very difficult to establish. Intravenous augmentation therapy with
Cristina Aljama, Teresa Martin, Galo Granados, Marc Miravitlles, Miriam Barrecheguren   +4 more
doaj   +2 more sources