Alpha-1 antitrypsin deficiency–associated panniculitis
نویسندگان
چکیده
منابع مشابه
Review of Alpha-1-Antitrypsin Deficiency Associated Panniculitis
Alpha-1-Antitrypsin (AAT) deficiency associated panniculitis is commonly included in the dermatologist's differential diagnosis but the disease is rarely ever diagnosed or discussed. The pathophysiology, clinical presentation, histopathology, diagnosis, and treatment of AAT deficiency panniculitis will be reviewed.
متن کاملAlpha 1 antitrypsin deficiency.
Alpha-1 antitrypsin deficiency is an inherited disorder that may cause severe lung and liver disease.
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Introduction: α1-antitrypsin deficiency (α1-ATD) is one of the most common genetic disorders in white race, a usual cause of liver disease in children, and hepatopulmonary involvement in children and adult. The aim of this case description is presenting a child with early lung disease without liver parenchymal disorder. Case presentation: We describe a 13 year old boy because of exertional dysp...
متن کاملAlpha - 1 - antitrypsin - Pittsburgh A
Alpha-l-antitrypsin-Pittsburgh is a human variant that resulted from a point mutation in the plasma protease inhibitor, a,-antitrypsin (358 Met Arg). This defect in the a,-antitrypsin molecule causes it to have greatly diminished anti-elastase activity but markedly increased antithrombin activity. In this report, we demonstrate that this variant protein also has greatly increased inhibitory act...
متن کاملALPHA-1-ANTITRYPSIN DEFICIENCY Determinants of airflow obstruction in severe alpha-1-antitrypsin deficiency
Dawn L DeMeo, Robert A Sandhaus, Alan F Barker, Mark L Brantly, Edward Eden, N Gerard McElvaney, Stephen Rennard, Esteban Burchard, James M Stocks, James K Stoller, Charlie Strange, Gerard M Turino, Edward J Campbell, Edwin K Silverman . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ....
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ژورنال
عنوان ژورنال: Journal of the American Academy of Dermatology
سال: 2021
ISSN: 0190-9622
DOI: 10.1016/j.jaad.2021.01.074