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Alpha‐1‐Antitrypsin Deficiency
Clinical Liver Disease, 2022Content available: Author Audio Recording.
Anandini Suri +2 more
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Alpha-1 Antitrypsin Deficiency
Journal of Insurance Medicine, 2015Alpha-1 antitrypsin deficiency is an inherited disorder that may cause severe lung and liver disease.
SK Kabra, Rakesh Lodha, Kana Jat
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Alpha-1 Antitrypsin Deficiency
2023Alpha-1 antitrypsin (AAT) deficiency is a common monogenic disorder in which there is a strong founder effect of a single missense mutation in SERPINA1, the gene encoding this major circulating serum anti-protease that is normally expressed primarily in hepatocytes.
Alisha M, Gruntman +2 more
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Alpha-1-antitrypsin augmentation therapy for alpha-1-antitrypsin deficiency
The American Journal of Medicine, 1988Alpha-1-antitrypsin (A1AT) deficiency is a genetic disorder characterized by low serum levels of A1AT and a high risk for the development of emphysema. A1AT is the principal inhibitor of neutrophil elastase, such that a deficiency of A1AT results in insufficient anti-elastase protection in the lower respiratory tract, thus allowing neutrophil elastase ...
R C, Hubbard, R G, Crystal
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1976
Proteinasen sind proteinspaltende Enzyme, die normalerweise nicht als solche im Plasma vorkommen, sondern als Vorstufen, die ihre Aktivitat erst durch Interaktion mit einem oder mehreren Aktivatoren erhalten. Dabei handelt es sich entweder um „selbstandige“ Proenzyme oder um solche, die zu Kaskadensystemen gehoren (ein aus einer Vorstufe entstehendes ...
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Proteinasen sind proteinspaltende Enzyme, die normalerweise nicht als solche im Plasma vorkommen, sondern als Vorstufen, die ihre Aktivitat erst durch Interaktion mit einem oder mehreren Aktivatoren erhalten. Dabei handelt es sich entweder um „selbstandige“ Proenzyme oder um solche, die zu Kaskadensystemen gehoren (ein aus einer Vorstufe entstehendes ...
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[Alpha 1-antitrypsin deficiency].
Revue des maladies respiratoires, 2016Pulmonary emphysema and liver disease are the clinical expressions of alpha 1-antitrypsin deficiency, an autosomal recessive genetic disease.Alpha 1-antitrypsin deficiency is usually associated with the homozygous Z variant of the SERPINA1 gene.
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Alpha-1-antitrypsin in malakoplakia
Virchows Archiv A Pathological Anatomy and Histology, 1982Macrophages in malakoplakia contain large amounts of immunoreactive alpha-1-antitrypsin (AAT). The amount of AAT remains unchanged during the morphogenetic stages of the pathological process (early, granulomatous, fibrosing phases), and does not correlate with the number or the presence of Michaelis-Gutmann (M.G.) bodies.
F, Callea, B, Van Damme, V J, Desmet
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Alpha-1-Antitrypsin Deficiency Panniculitis
Dermatologic Clinics, 2008Alpha-1-antitrypsin deficiency is a congenital error of metabolism linked to pulmonary (emphysema) and liver (cirrhosis) disease. Since 1972, panniculitis has been associated with this deficiency, initially related to Weber-Christian syndrome and finally as a differentiated entity.
Ricardo, Valverde +4 more
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