Results 71 to 80 of about 410,523 (291)

Long-term augmentation therapy with Alpha-1 Antitrypsin in an MZ-AAT severe persistent asthma

Monaldi Archives for Chest Disease, 2016
A young Caucasian female with severe bronchial asthma and Alpha1-antitrypsin (AAT) deficiency, MZ phenotype, experienced a quick and severe limitation of her physical capacity, which negatively affected her psychological state and social life, though she
I. Blanco, H. Canto, J. Flóres, C. Camblor, V. Cárcaba, F.J. de Serres, S. Janciauskiene, E.F. Bustillo   +7 more
doaj   +1 more source

Decrease in alpha-1 antiproteinase antitrypsin is observed in primary Sjogren’s syndrome condition

Autoimmunity, 2020
Primary Sjogren’s syndrome (pSS) is a systemic autoimmune disease that is characterized by the infiltration of immune cells. Although the loss of salivary gland function is a major manifestation observed in pSS, the factors that could promote these ...
Brij B. Singh, Joyce Ohm, Fredice O. Quenum Zanbede, Pooja Chauhan, Frans G. M. Kroese, Arjan Vissink, Julian L. Ambrus, Bibhuti B. Mishra   +7 more
doaj   +1 more source

Management of chronic airway diseases:What can we learn from real-life data? [PDF]

, 2017
Chronic obstructive pulmonary disease (COPD), alpha-1 antitrypsin deficiency (AATD) and non-cystic fibrosis bronchiectasis (hereafter referred to as bronchiectasis) are distinct but related airway diseases: COPD is characterised by persistent and usually
Greulich T, James D. Chalmers
core   +3 more sources

The Impact of Delayed Diagnosis of Alpha-1 Antitrypsin Deficiency: The Association Between Diagnostic Delay and Worsened Clinical Status

Respiratory care, 2019
BACKGROUND: Alpha-1 antitrypsin deficiency is frequently underrecognized. Individuals with symptoms often experience long diagnostic delays. Although a delayed diagnosis is logically presumed to be associated with adverse effects, confirmatory evidence ...
V. Tejwani, A. Nowacki, Erica Fye, C. Sanders, J. Stoller   +4 more
semanticscholar   +1 more source

Gross Intrahepatic Mass Formation Predicts the Primary Site of Perihilar Cholangiocarcinoma Based on Molecular Pathologic Studies

Journal of Hepato-Biliary-Pancreatic Sciences, EarlyView.
ABSTRACT Background/Purpose Intrahepatic cholangiocarcinoma (iCCA) and extrahepatic cholangiocarcinoma (eCCA) are clinically and genetically distinct. However, the classification of perihilar cholangiocarcinoma (phCCA) with an intrahepatic tumor mass remains unclear.
Yuki Masuda, Naoto Kubota, Ryo Takemura, Yasuhito Arai, Yuta Abe, Osamu Itano, Minoru Esaki, Tatsuhiro Shibata, Yuko Kitagawa, Hidenori Ojima   +9 more
wiley   +1 more source

Preliminary evidence of improved liver biomarkers in adolescents with obesity and suspected metabolic dysfunction‐associated steatotic liver disease treated with semaglutide: A case series

JPGN Reports, EarlyView.
Abstract Metabolic dysfunction‐associated steatotic liver disease (MASLD) affects 30%–40% of youth with obesity and lacks approved pharmacologic therapies. In this single‐center retrospective case series at a tertiary care safety‐net children's hospital, we evaluated five adolescents (mean age: 17.0 years; 100% Hispanic; 60% male; mean body mass index:
Rachel Schenker, Katya Fomenko, Fotini T. Mitsinikos, Alaina P. Vidmar   +3 more
wiley   +1 more source

Genetic Variants of Serum Alpha 1 Antitrypsin [PDF]

, 1995
Complete absence of data on alpha 1 antitrypsin in this country prompted us to determine serum levels using radial immunodiffusion (RID) and phenotypes by isoelectric focusing (IEF) in 100 healthy adults (52 males and 48 females).
Shahid, Anjum, Siddiqui, Anwar Ali, Waqar, Mohammed Anwar, Zuberi, Sarwar J   +3 more
core   +1 more source

The European Alpha-1 Research Collaboration (EARCO): a new ERS Clinical Research Collaboration to promote research in alpha-1 antitrypsin deficiency

European Respiratory Journal, 2019
Alpha-1 antitrypsin deficiency (AATD) is a common but under-recognised genetic condition that affects approximately 1 in 2000 to 1 in 5000 individuals and predisposes to early-onset emphysema and liver disease [1].
M. Miravitlles, J. Chorostowska-Wynimko, I. Ferrarotti, N. McElvaney, K. O'hara, J. Stolk, R. Stockley, A. Turner, Marion Wilkens, T. Greulich   +9 more
semanticscholar   +1 more source

Hepatopulmonary syndrome as presentation of pediatric metabolic dysfunction‐associated steatohepatitis

JPGN Reports, EarlyView.
Abstract Childhood obesity is rising and leading to serious co‐morbidities, among which is metabolic dysfunction‐associated steatotic liver disease (MASLD) predisposing individuals to cirrhosis. We describe a young 11‐year‐old Hispanic male who presented with hepatopulmonary syndrome secondary to cirrhotic portal hypertension from metabolic dysfunction‐
Shruti Sakhuja, Stacey S. Beer, Kalyani R. Patel, Alexiss M. Williams, Nhu Thao Galvan, John A. Goss, Krupa R. Mysore   +6 more
wiley   +1 more source

Rare variants in alpha 1 antitrypsin deficiency: a systematic literature review

Orphanet Journal of Rare Diseases
Background Alpha 1 Antitrypsin Deficiency (AATD) is a largely underrecognized genetic condition characterized by low Alpha 1 Antitrypsin (AAT) serum levels, resulting from variations in SERPINA1.
Ilaria Ferrarotti, Marion Wencker, Joanna Chorostowska-Wynimko   +2 more
doaj   +1 more source