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Alpha1-Antitrypsin (AAT) Deficiency Classic Research Papers


Introduction and Biography
Laurell CB,
Eriksson S.
The electrophoretic α¹­ globulin pattern of serum in α¹­antitrypsin deficiency. Scand J Clin Lab Invest. 1963;15:132-140.
Sharp HL,
Bridges RA,
Krivit W,
Freier EF.
Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognized inherited disorder J Lab Clin Med. 1969;73:934-939.
Janoff A, Scherer J. Mediators of inflammation in leukocyte lysosomes. IX. Elastinolytic activity in granules of human polymorphonuclear leukocytes. J Exp Med. 1968;128:1137-1155
Wewers MD,
Casolaro MA,
Sellers SE,
Swayze SC,
McPhaul KM,
Wittes JT,
Crystal RG.
Replacement therapy for alpha¹-antitrypsin deficiency associated with emphysema N Engl J Med. 1987;316:1055-1062.
Lomas DA,
Evans DLI,
Finch JT,
Carrell RW.
The mechanism of Z α¹-antitrypsin accumulation in the liver. Nature. 1992;357:605-607.
Seersholm N,
Wencker M,
Banik N,
Viskum K,
Dirksen A,
Kok-Jensen A,
Konietzko N, for the
Wissenschaftliche
Arbeitsgemeinschaft
zur Therapie von
Lungenerkrankungen
(WATL) α¹­AT study
group
Does α¹­antitrypsin augmentation therapy slow the annual decline in FEV¹ in patients with severe hereditary α¹­ antitrypsin deficiency Eur Respir J. 1997;10:2260
The Alpha
-1-Antitrypsin
Deficiency
Registry Study
Group.
Survival and FEV¹ decline in individuals with severe deficiency of α¹­ antitrypsin. Am J Respir Crit Care Med. 1998;158:49-59.
Wencker M,
Banik N,
Buhl R,
Seidel R,
Konietzko N, for the
Wissenschaftliche
Arbeitsgemeinschaft
zur Therapie von
Lungenerkrankkungen
(WATL) α¹­AT-study
group.
Long-term treatment of α¹­ antitrypsin deficiency-related pulmonary emphysema with human α¹­ antitrypsin. Eur Respir J. 1998;11:428-433
Stockley RA,
Bayley DL,
Unsal I,
Dowson LJ.
The effect of augmentation therapy on bronchial inflammation in α¹­ antitrypsin deficiency. Am J Respir Crit Care Med. 2002;165: 1494-1498.
Silverman EK,
Miletich JP,
Pierce JA,
Sherman LA,
Endicott SK,
Broze GJ, Jr.,
Campbell EJ.
Alpha-1-antitrypsin deficiency. High prevalence in the St. Louis area determined by direct population screening. Am Rev Respir Dis. 1989;140:961-966.
Stoller JK, Smith P, Yang P, Spray J. Physical and social impact of alpha¹-antitrypsin deficiency: results of a survey. Cleve Clin J Med. 1994;61:461-467
Campos MA,
Wanner A,
Zhang G,
Sandhaus RA,
Trends in the diagnosis of symptomatic patients with α¹­ antitrypsin deficiency between 1968 and 2003. Chest. 2005;128:1179-1186
American
Thoracic Society.
Guidelines for the approach to the patient with severe hereditary alpha-1-antitrypsin deficiency. Am Rev Respir Dis. 1989;140:1494-1497
American Thoracic
Society/European
Respiratory Society
Statement
Standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003;168:818-900. Executive Summary


Important Safety Information

Prolastin®, Alpha1-Proteinase Inhibitor (Human) is indicated for chronic replacement therapy of individuals having congenital deficiency of alpha-1 PI (alpha1-antitrypsin deficiency) with clinically demonstrable panacinar emphysema. Weekly Prolastin® therapy has demonstrated a low occurrence of side effects. In clinical studies with Prolastin®, reactions were observed in 1.16% of infusions, the most common events being fever (0.77%), light-headedness (0.19%), and dizziness (0.19%). As with all plasma-derived therapeutics, the potential to transmit infectious agents cannot be totally eliminated. Individuals with selective IgA deficiencies who have known antibody against IgA (anti-IgA antibody) should not receive Prolastin®, since these patients may experience severe reactions, including anaphylaxis, to IgA which may be present.

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Learn about AAT deficiency. Request your complimentary compendium of classic research papers: Alpha-1 Antitrypsin (AAT) Deficiency: A History Through the Medical Literature.
 
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