Alpha 1-Antitrypsin Deficiency Presenting as Cryptogenic Cirrhosis in Adults Over 50

Thatcher, Bryan S.; Winkelman, Eugene I.; Tuthill, Ralph J.

doi:10.1097/00004836-198510000-00006

Cited by 7 publications

(2 citation statements)

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“…a-1-AT is the principal inhibitor of potentially destructive enzymes such as leukocyte elastase that can cleave connective tissue elements of the extracellular matrix of a variety of tissues (2). a-1-AT deficiency (a-1-ATd) is an autosomal recessive disorder in which low concentrations of serum a-1-AT have been clinically associated with the progressive development of emphysema, liver disease or both (3)(4)(5)(6)(7)(8)(9). At least 75 different alleles of the a-1-AT gene have been identified, any two of which define an individual's phenotype.…”

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confidence: 99%

Histopathology of α1–Antitrypsin Liver Disease in A Transgenic Mouse Model

Geller

Nichols

Dycaico³

et al. 1990

Hepatology

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Transgenic mice were constructed using human alpha 1-antitrypsin M and Z genomic clones. Livers of the M lineage mice showed slight cellular pleomorphism and immunohistochemically demonstrable finely granular alpha 1-antitrypsin material in hepatocytes. Z lineage mice with five gene copies per haploid mouse genome (Z#1) demonstrated fine granular alpha 1-antitrypsin material and a few large globules. In contrast, Z lineage mice with 12 gene copies per haploid mouse genome (Z#2) demonstrated hepatocytes filled with homogeneous, eosinophilic globules that were strongly reactive with diastase and periodic acid-Schiff and antibody to alpha 1-antitrypsin. Scattered microscopic polymorphonuclear leukocyte accumulations were seen that contained extracellular alpha 1-antitrypsin material, but there was neither histological nor serological evidence of mouse infectious hepatitis. In young animals, small clusters of hepatocytes lacking alpha 1-antitrypsin material were seen. These cells were the dominant population in older animals and formed nodular arrangements. Fibrosis was not demonstrable in neonatal and young animals or in any of the controls, but perisinusoidal fibrosis was seen in older Z#2 mice. Groups of hepatocytes without alpha 1-antitrypsin material showed dysplastic changes. We conclude that the transgenic mouse is a reliable and useful model in which to study the effects of alpha 1-antitrypsin in the liver because it demonstrates changes similar to those in the human disease.

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confidence: 99%

Histopathology of α1–Antitrypsin Liver Disease in A Transgenic Mouse Model

Geller

Nichols

Dycaico³

et al. 1990

Hepatology

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“…Institutions that apply nonstandard serological tests, such as antibodies to soluble liver antigen (anti-SLA) [3][4][5], liver cytosol type 1 (anti-LC1) [6], asialoglycoprotein receptor (anti-ASGPR) [7,8] or uridine diphosphate glucuronosyltransferases (anti-UGT) [9] or that have focused expertise in metabolic disorders (diabetes and metabolic syndrome) [10][11][12][13], hereditary conditions (Wilson disease, hemochromatosis, and alpha-1 anti-trypsin deficiency) [14][15][16][17][18][19][20][21][22][23][24][25][26], nonstandard viral infections (hepatitis E virus, hepatitis G virus, and immunodeficiency virus) [27][28][29][30][31][32][33][34][35] and celiac disease [36][37][38][39][40][41] may have different concepts about the true nature of cryptogenic chronic hepatitis. Similarly, experiences from liver transplantation centers may differ from those that assess patients with active precirrhotic disease.…”

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confidence: 99%

Cryptogenic Chronic Hepatitis and Its Changing Guise in Adults

Czaja

2011

Dig Dis Sci

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Cryptogenic chronic hepatitis is a disease that is unexplained by conventional clinical, laboratory and histological findings, and it can progress to cirrhosis, develop hepatocellular carcinoma, and require liver transplantation. The goals of this review are to describe the changing phenotype of cryptogenic chronic hepatitis in adults, develop a diagnostic algorithm appropriate to current practice, and suggest treatment options. The frequency of cryptogenic hepatitis is estimated at 5.4%. Cryptogenic cirrhosis is diagnosed in 5-30% of patients with cirrhosis, and it is present in 3-14% of adults awaiting liver transplantation. Nonalcoholic fatty liver disease has been implicated in 21-63% of patients, and autoimmune hepatitis is a likely diagnosis in 10-54% of individuals. Viral infections, hereditary liver diseases, celiac disease, and unsuspected alcohol or drug-induced liver injury are recognized infrequently in the current cryptogenic population. Manifestations of the metabolic syndrome heighten the suspicion of nonalcoholic fatty liver disease, and the absence of hepatic steatosis does not discount this possibility. The diagnostic scoring system of the International Autoimmune Hepatitis Group can support the diagnosis of autoimmune hepatitis in some patients. Certain genetic mutations may have disease-specificity, and they suggest that some patients may have an independent and uncharacterized disease. Corticosteroid therapy is effective in patients with autoimmune features, and life-style changes and specific therapies for manifestations of the metabolic syndrome are appropriate for all obese patients. The 1- and 5-year survivals after liver transplantation have ranged from 72-85% to 58-73%, respectively.

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Nutritional and Metabolic Liver Diseases

Sherlock¹,

Dooley²

2001

Diseases of the Liver and Biliary System

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Alpha 1-Antitrypsin Deficiency Presenting as Cryptogenic Cirrhosis in Adults Over 50

Cited by 7 publications

References 0 publications

Histopathology of α1–Antitrypsin Liver Disease in A Transgenic Mouse Model

Histopathology of α1–Antitrypsin Liver Disease in A Transgenic Mouse Model

Cryptogenic Chronic Hepatitis and Its Changing Guise in Adults

Nutritional and Metabolic Liver Diseases

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