alpha 1-Antitrypsin Deficiency (alpha 1 Antitrypsin Deficiency)

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205 relevant articles (5 outcomes, 17 trials/studies) found for this Disease

Description: Deficiency of the protease inhibitor ALPHA 1-ANTITRYPSIN, leading primarily to degradation of elastin of the alveolar walls, as well as other structural proteins of a variety of tissues. (From Scriver, Beaudet, Sly, & Valle, The Metabolic and Molecular Bases of Inherited Disease, 7th ed, p4125)

Also Known As:

alpha 1 Antitrypsin Deficiency; Deficiencies, alpha 1-Antitrypsin; Deficiency, alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiencies

Relationship Network

Disease Context: Research Results

Skin and Connective Tissue Diseases

Connective Tissue Diseases: 1632

alpha 1-Antitrypsin Deficiency: 205

Related Diseases

1.	Liver Diseases (Liver Disease)
2.	Hepatolenticular Degeneration (Wilson's Disease)
3.	Hepatitis B
4.	Emphysema
5.	Fibrosis (Cirrhosis)

Experts

1.	Stoller, James K: 2 articles (01/2008 - 12/2002)
2.	Brantly, Mark L: 2 articles (01/2008 - 10/2005)
3.	Rennard, Stephen I: 1 article (01/2008)
4.	Demeo, Dawn L: 1 article (01/2008)
5.	Silverman, Edwin K: 1 article (01/2008)
6.	Strange, Charlie: 1 article (01/2008)
7.	Barker, Alan F: 1 article (01/2008)
8.	Turino, Gerard: 1 article (01/2008)
9.	Sandhaus, Robert A: 1 article (01/2008)
10.	Campbell, Edward J: 1 article (01/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to alpha 1-Antitrypsin Deficiency:

1.	alpha 1-Antitrypsin (alpha 1 Antitrypsin)IBA 04/23/1987 - "We evaluated the feasibility, safety, and biochemical efficacy of intermittent infusions of alpha 1-antitrypsin in the treatment of patients with alpha 1-antitrypsin deficiency" 01/01/1983 - "Increased intestinal clearance of alpha 1-antitrypsin in patients with alpha 1-antitrypsin deficiency." 03/14/1983 - "The study group included 59 PI type Z patients with alpha 1-antitrypsin deficiency, five with the rare alpha 1-antitrypsin null genotype (PI Q0 or --), and seven with alpha 1-antitrypsin deficiency of the rare PI types MmaltonZ or MduarteZ" 12/01/1996 - "This report describes the clinical characteristics of a group of 59 individuals with the PISZ phenotype and alpha 1-antitrypsin (alpha 1-AT) deficiency, identified during recruitment of a registry for subjects with severe alpha 1-antitrypsin deficiency" 01/01/1996 - "Gene therapy in alpha 1 antitrypsin deficiency rests on the transfer and the expression of a copy of the normal human alpha 1 antitrypsin gene by the cells of an organism of a subject suffering from the disease" Order ALL the reference details at left...*
2.	Leukocyte Elastase (Neutrophil Elastase)IBA 02/01/1980 - "A matched-pair study of the leukocyte elastase-like activity in normal persons and in emphysematous patients with and without alpha 1-antitrypsin deficiency." 01/01/2000 - "alpha 1-Antitrypsin deficiency is a model disorder resulting in an insufficient control of leukocyte elastase with a subsequent alveolar tissue damage" 02/01/1980 - "In persons without alpha 1-antitrypsin deficiency, however, the leukocyte elastase-like activity may be a contributing factor in the development of this disease" 02/01/1980 - "In 16 emphysematous patients without alpha 1-antitrypsin deficiency (PiMM) however, the leukocyte elastase-like activity tended to be higher than that of normal control subjects" 01/01/1990 - "The importance of the destructive element in emphysema and the gradual focusing on neutrophil elastase as a key enzyme in the pathogenesis of emphysema in alpha 1-antitrypsin deficiency is emphasized" Order ALL the reference details at left...
3.	alpha-Macroglobulins (alpha 2 Macroglobulin)IBA 03/14/1983 - "alpha 2-Macroglobulin in patients with obstructive lung disease, with and without alpha 1-antitrypsin deficiency." 02/18/1979 - "Two pictures of alpha 1-antitrypsin deficiency, one associated with alpha 2-macroglobulin deficiency and one isolated case of the latter deficiency have been observed in three patients suffering from cirrhosis of the liver and/or hepatoma" 10/01/1981 - "These studies demonstrated that: (a) alpha 1-antitrypsin is the major antielastase of the normal human lower respiratory tract; (b) alpha 2-macroglobulin, a large serum antielastase, and the bronchial mucous inhibitor, an antielastase of the central airways, do not contribute to the antielastase protection of the human alveolar structures; (c) individuals with PiZ alpha 1-antitrypsin deficiency have little or no alpha 1-antitrypsin in their lower respiratory tract and have no alternative antiprotease protection against neutrophil elastase; and (d) the lack of antiprotease protection of the lower respiratory tract of PiZ individuals is a chronic process, suggesting their vulnerability to neutrophil elastase is always present." Order ALL the reference details at left...
4.	DNA (Deoxyribonucleic Acid)IBA 09/01/1988 - "Rapid DNA analysis of alpha 1-antitrypsin deficiency: application of an improved method for amplifying mutated gene sequences." 10/01/1991 - "A method for detection of the M and Z alleles of the alpha 1-antitrypsin deficiency (AAT) gene has been developed using denaturing gradient gel electrophoresis (DGGE) of DNA samples amplified in vitro by the polymerase chain reaction" 08/01/1990 - "Analysis of alpha 1-antitrypsin deficiency using DNA methods]" 09/12/1988 - "Diagnosis of alpha 1-antitrypsin deficiency by enzymatic amplification of human genomic DNA and direct sequencing of polymerase chain reaction products." 07/01/1994 - "Furthermore, the common alpha 1-antitrypsin deficiency allele, PIZ, has a unique DNA haplotype at the CBG, PIL, and PI loci, which extends over 60 kb in 97% of cases and in 44% of cases includes the PCI and AACT loci" Order ALL the reference details at left...*
5.	CopperIBA 07/01/1992 - "Iron, copper and alpha 1-antitrypsin deficiency in the liver of cirrhotic and schistosomotic patients." 01/01/1996 - "Immunostaining for hepatitis B antigens, for alpha-1-antitrypsin and copper staining are helpful in detecting hepatitis B and congenital liver diseases (Wilson's disease, alpha-1-antitrypsin deficiency) as possible causes of chronic progressive inflammatory liver disease" Order ALL the reference details at left...
6.	Hepatitis B AntigensIBA 01/01/1996 - "Immunostaining for hepatitis B antigens, for alpha-1-antitrypsin and copper staining are helpful in detecting hepatitis B and congenital liver diseases (Wilson's disease, alpha-1-antitrypsin deficiency) as possible causes of chronic progressive inflammatory liver disease" Order ALL the reference details at left...
7.	Protease Inhibitors (Protease Inhibitor)IBA 09/01/1997 - "Further investigation demonstrated a homozygous Z phenotype, the classic protease inhibitor variant described in alpha 1-antitrypsin deficiency" 01/01/1989 - "The protease inhibitor system in children (in diseases of the lung, liver and hereditary alpha 1-antitrypsin deficiency)]" 10/01/1981 - "To directly assess the antiprotease side of this equation, the lower respiratory tract of non-smoking individuals with normal serum antiproteases and individuals with PiZ homozygous alpha 1-antitrypsin deficiency underwent bronchoalveolar lavage to evaluate the antiprotease screen of their lower respiratory tract" 10/01/1979 - "Using a case-control design, our cases included 37 heterozygotes for alpha 1-antitrypsin deficiency, protease inhibitor phenotypes MZ and MS, selected because they were parents of children with homozygous alpha 1-antitrypsin deficiency identified in a statewide newborn screening program between 1971 and 1974" 09/01/1995 - "In recent studies, a protease-to-protease inhibitor imbalance in patients with alpha 1-antitrypsin deficiency was thought to be a mechanism contributing to the development of chronic pancreatitis" Order ALL the reference details at left...
8.	AntigensIBA 08/01/1993 - "The relationship between alpha 1-antitrypsin deficiency (alpha 1-ATD) and the HLA antigen system was studied in 32 liver transplant recipients" 08/01/1993 - "Lack of association between HLA antigen DR3 and alpha 1-antitrypsin deficiency in liver transplant recipients." 04/01/1990 - "There is some evidence of a protective effect of breast-feeding, and a recent study has found the HLA class II DR3 antigen to be more common than expected in children with alpha 1-antitrypsin deficiency and liver disease" 01/01/1998 - "Reports on hereditary alpha 1-antitrypsin deficiency, the link between systemic vasculitis and human leukocyte antigen molecules, and an animal model of spontaneous granulomatous arteritis in mice with a hereditary deficit in Fas-mediated apoptosis, are some of the new data that strongly favor genetic predisposition" 02/01/1997 - "ANCA-positive vasculitis and alpha 1-antitrypsin deficiency: could free ANCA antigens released by neutrophils mediate vasculitic lesions?" Order ALL the reference details at left...
9.	Collagen Type III (Type III Collagen)IBA 11/01/1988 - "Procollagen type III peptide in asymptomatic children with alpha 1-antitrypsin deficiency." 10/01/1991 - "In 31 children with congenital alpha 1-antitrypsin deficiency the concentration of procollagen type III peptide was determined in a trial establishing liver fibrosis degree and monitoring of fibrosis progression" 12/15/1986 - "Serum type III procollagen peptide as a non-invasive marker of liver damage during infancy and childhood in extrahepatic biliary atresia, idiopathic hepatitis of infancy and alpha 1 antitrypsin deficiency." Order ALL the reference details at left...
10.	SmokeIBA 09/22/1982 - "Congenital alpha 1-antitrypsin deficiency as an aggravating factor in alveolar injury from cigarette smoke]" 02/01/1995 - "Patients with alpha 1-antitrypsin deficiency (alpha 1ATD) develop emphysema at a young age, especially if they smoke, and the purpose of this study was to calculate survival with respect to the initial FEV1 and smoking history" 01/01/1986 - "Hereditary factors, including alpha 1-antitrypsin deficiency, and environmental exposures, such as to cigarette smoke, coal dust and bacteria, are associated with a high risk for the development of emphysema through their interactions with proteolytic and oxidative processes within the lung" Order ALL the reference details at left...

Therapies and Procedures

1.	Liver Transplantation 08/01/1999 - "Three patients had meconium ileus, including one infant with concomitant alpha(1) antitrypsin deficiency, who required early liver transplantation" 03/01/1995 - "Alpha 1-antitrypsin deficiency is the most common genetic cause of liver disease in infants and children and is the most common genetic disease for which liver transplantation is indicated" 06/01/1994 - "This case report describes a child who became infected by HCV after liver transplantation for end-stage liver disease caused by alpha 1-antitrypsin deficiency" 01/01/1994 - "Advanced liver cirrhosis secondary to heterozygote alpha 1-antitrypsin deficiency in a young woman: treatment by liver transplantation]" 08/01/1991 - "A 5.7 year old boy with alpha 1-antitrypsin deficiency developed intractable ascites after liver transplantation" Order ALL the reference details at left...
2.	Lung Transplantation 01/01/1992 - "Case study: single lung transplantation for alpha 1-antitrypsin deficiency." 11/01/1997 - "We had the unique opportunity to investigate a 51-yr-old never-smoker after single lung transplantation (TX) for alpha 1-antitrypsin deficiency emphysema who received a donor's lung from a heavy cigarette smoker" 12/01/1996 - "Lung transplantation is an option for appropriately selected patients with end-stage emphysema caused by alpha 1-antitrypsin deficiency" 12/01/1996 - "Lung transplantation for alpha 1-antitrypsin deficiency emphysema." 11/01/1994 - "In conclusion, the two year mortality of emphysema patients due to alpha 1-antitrypsin deficiency increases exponentially with decreasing FEV1 and the results imply that only a few patients who underwent lung transplantation would have had a better two year prognosis without this procedure." Order ALL the reference details at left...
3.	Transplants (Transplant) 07/01/2000 - "Patient 1 is a 14-year-old girl who developed macromastia 6 months after a liver transplant for alpha 1-antitrypsin deficiency" 01/01/1991 - "It is concluded that the medium-term results of heart and lung transplantation for treatment of emphysema are good and that patients with alpha 1-antitrypsin deficiency may undergo transplant procedures without substitution therapy." 11/01/1990 - "Five children with alpha 1 antitrypsin deficiency and terminal liver disease received liver grafts; all five became hypertensive and four developed hypertensive encephalopathy" 10/01/1988 - "Children suffering from alpha 1-antitrypsin deficiency hepatopathies, autoimmune hepatitis and from extrahepatic biliary atresia are cared for with the aim of rendering possible a liver transplant in case of liver insufficiency" 01/01/1993 - "Between the 7th of June 1985 and december 1991, 71 liver transplants (LTx) were performed in 60 patients (pts) with ages ranging from 8 months to 14 years and weight between 4 and 38 kg. Sixty-two percent were cholestatic diseases (32 biliary atresia, 3 sclerosing cholangitis and 2 biliary paucity), 25% metabolic hepatic-based diseases (6 glycogen storage disease, 4 Byler disease, 3 tyrosinaemia and 2 alpha-1-antitrypsin deficiency) and 13% miscellaneous diagnosis (2 post-hepatitic cirrhosis, 2 autoimmune hepatitis, 2 fulminant hepatitis and one case of cholesteryl ester storage disease and one case of Budd-Chiari disease)" Order ALL the reference details at left...
4.	Transplantation (Transplant Recipients) 08/01/1993 - "Indications for transplantation were: biliary atresia (9), Byler's disease (1), tyrosinemia (3), and alpha 1-antitrypsin deficiency (1)" 01/01/1992 - "Univariate (unadjusted) analyses detected significant differences in survival for age (the youngest recipients had the worst survival), UNOS description (poorer functional status just prior to transplantation led to poorer survival), and primary liver disease (survival was worst for recipients transplanted due to fulminant liver failure, and best for patients with alpha-1 antitrypsin deficiency).(ABSTRACT TRUNCATED AT 400 WORDS)" 08/01/1993 - "The relationship between alpha 1-antitrypsin deficiency (alpha 1-ATD) and the HLA antigen system was studied in 32 liver transplant recipients" 08/01/1993 - "Lack of association between HLA antigen DR3 and alpha 1-antitrypsin deficiency in liver transplant recipients." 03/31/1994 - "The diagnoses were: emphysema (n = 5), idiopathic pulmonary fibrosis (n = 5), emphysema in alpha 1-antitrypsin deficiency (n = 1), mucoviscidosis (n = 1), drug-induced fibrosis (n = 1), chronic lung rejection after heart-lung transplantation (n = 1), secondary pulmonary hypertension with persistent ductus arteriosus (n = 1), sarcoidosis (n = 1) and chronic transplant failure after unilateral transplantation (n = 1)" Order ALL the reference details at left...
5.	Heart-Lung Transplantation 05/29/1989 - "The case reports of the first two Danes (both with emphysema secondary to alpha-1-antitrypsin deficiency) who underwent successful heart-lung transplantations in England are presented" 03/31/1994 - "The diagnoses were: emphysema (n = 5), idiopathic pulmonary fibrosis (n = 5), emphysema in alpha 1-antitrypsin deficiency (n = 1), mucoviscidosis (n = 1), drug-induced fibrosis (n = 1), chronic lung rejection after heart-lung transplantation (n = 1), secondary pulmonary hypertension with persistent ductus arteriosus (n = 1), sarcoidosis (n = 1) and chronic transplant failure after unilateral transplantation (n = 1)" Order ALL the reference details at left...

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