Results 11 to 20 of about 24,356 (213)

Utility of routine screening for alpha-1 antitrypsin deficiency in patients with bronchiectasis [PDF]

, 2020
Alpha-1 antitrypsin deficiency (AATD) is a cause of bronchiectasis. Guidelines for bronchiectasis from the British Thoracic Society do not recommend to routinely test patients for AATD. In contrast, guidelines for AATD recommend routine screening.
Carreto, Luis, Chalmers, James D., Donovan, Jackie, Fardon, Tom, Finch, Simon, Furrie, Elisabeth, Loebinger, Michael, Morrison, Meghan, Tan, Gan Liang, Wilson, Robert   +9 more
core   +3 more sources

Clarifying the relationship between pulmonary langerhans cell histiocytosis and Alpha 1 antitrypsin deficiency

Orphanet Journal of Rare Diseases, 2021
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking related, progressive diffuse cystic lung disease that occurs primarily in smokers. The aim of this study was to determine if there was an increase in alpha-1 antitrypsin deficient alleles ...
Cormac McCarthy, Emmanuelle Bugnet, Amira Benattia, Michael P. Keane, Benoit Vedie, Gwenaël Lorillon, Abdellatif Tazi   +6 more
doaj   +1 more source

Liver Transplantation for Advanced Liver Disease with Alpha-1antitrypsin Deficiency [PDF]

, 1980
ALPHA-1-antitrypsin deficiency associated with chronic obstructive airway disease was recognized in 1963 by Laurell and Ericksson.1 In 1969, Sharp2 described the first cases of alpha-1-antitrypsin-deficiency disease in children with cirrhosis. Since then,
Allan G. Redeker, Anonymous, Brand, Cox, Eisen, Fagerhol, Gastel, Gerhard P. J. Schröter, Hirschberger, Jacobsson, Janoff, John M. Hood, Kueppers, Kuhlenschmidt, Laurell, Lawrence J. Koep, Odièvre, Putnam, Richard Weil, Robert L. Peters, Roy, Rynbrandt, Sharp, Sharp, Sharp, Thomas E. Starzl, Tálamo   +26 more
core   +1 more source

Case-finding for alpha1-antitrypsin deficiency in Kazakh patients with COPD

Multidisciplinary Respiratory Medicine, 2017
Background Alpha-1-antitrypsin deficiency (AATD) is an under-diagnosed condition in patients with chronic obstructive pulmonary disease (COPD). The aim of this study was to screen for AATD in Kazakh patients with COPD using dried blood spot specimens ...
Ardak Zhumagaliyeva, Stefania Ottaviani, Timm Greulich, Marina Gorrini, Claus Vogelmeier, Ludmila Karazhanova, Gulmira Nurgazina, Annalisa DeSilvestri, Victor Kotke, Valentina Barzon, Michele Zorzetto, Angelo Corsico, Ilaria Ferrarotti   +12 more
doaj   +1 more source

Alpha-1 Antitrypsin Deficiency: Home Therapy

Frontiers in Pharmacology, 2021
While available in only a few countries, home therapy is a possible strategy for the treatment of alpha-1 antitrypsin deficiency. We want to describe our experience in the management of human alpha-1 antitrypsin using home care intravenous augmentation ...
Anna Annunziata, Maurizia Lanza, Antonietta Coppola, Paolo Andreozzi, Sara Spinelli, Giuseppe Fiorentino   +5 more
doaj   +1 more source

Alpha-1 antitrypsin deficiency

Medicina Clínica (English Edition), 1977
Alpha-1 antitrypsin deficiency (AATD) is a rare hereditary condition caused by decreased plasma and tissue levels of alpha-1 antitrypsin (AAT) that can lead to serious lung and liver disease in children and adults. AATD patients face challenges such as under diagnosis, clinical variability, and limited treatment options for liver disease.
  +9 more sources

REVERSAL OF HEPATIC ALPHA-1-ANTITRYPSIN DEPOSITION AFTER PORTACAVAL SHUNT [PDF]

, 1983
End-to-side portacaval shunts were carried out in three children with the liver disease of alpha-1-antitrypsin deficiency and complications of portal hypertension. Their clinical courses have been stable for 31/2 to almost 7 years.
Aagenaes, Bell, Bismuth, Fisher, Hercz, Hood, Jamieson, Kuhlenschmidt, Lawford, Putnam, Sharp, Sotos, Starzl, Starzl, Starzl, Starzl, Starzl, Starzl   +17 more
core   +1 more source

Idiopathic Hemochromotosis and Alpha‐1‐Antitrypsin Deficiency: Coexistence in a Family with Progressive Liver Disease in the Proband [PDF]

, 1983
A patient with coexistent hemochromatosis and alpha‐1‐antitrypsin deficiency which led to cirrhosis and death despite adequate therapy for hemochromatosis is reported. Evaluation of the family revealed first degree relatives with iron overload and others
Addison, Allen, Amos, Anonymous, Bomford, Brozovich, Caraway, Chapuis-Cellier, Gadek, Gerblich, Hodges, Hood, Larson, Lloyd, Powell, Reintoft, Saugier, Simon, Simon, Simon, Sternberger, Sveger   +21 more
core   +1 more source

Update on and future perspectives for the diagnosis of alpha-1 antitrypsin deficiency in Brazil

Jornal Brasileiro de Pneumologia, 2021
Alpha-1 antitrypsin deficiency (AATD) is a rare genetic disorder caused by a mutation in the SERPINA1 gene, which encodes the protease inhibitor alpha-1 antitrypsin (AAT). Severe AATD predisposes individuals to COPD and liver disease.
José R Jardim, Francisco Casas-Maldonado, Frederico Leon Arrabal Fernandes, Maria Vera Cruz de O Castellano, María Torres-Durán, Marc Miravitlles   +5 more
doaj   +2 more sources

Leukocyte adhesion and recruitment, and alpha-1-antitrypsin deficiency: a report from ATS 2001, May 18-23, San Francisco [PDF]

, 2001
The program at this year's American Thoracic Society international conference included over 300 scientific and clinical symposia. In this report I have reviewed the data presented on two important areas of lung inflammation, namely leukocyte recruitment ...
Woolhouse Ian
core   +2 more sources