Heterozygous MZ alpha 1-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis

The New England Journal of Medicine
J R HodgesR Wright

Abstract

To determine the prevalence of alpha 1-antitrypsin deficiency in patients with cirrhosis or chronic active hepatitis, we performed a five-year prospective study of liver-biopsy specimens from 1055 adults. Thirty-four patients whose specimens contained hepatocyte inclusions characteristic of the deficiency were phenotyped, and 25 had phenotype MZ (2.4 per cent). The distribution of patients with this phenotype among the 185 patients with cirrhosis diagnosed histologically was three of 84 patients with alcoholic cirrhosis (3.5 per cent), seven of 34 with non-B chronic active hepatitis (20.5 per cent), six of 28 with cryptogenic cirrhosis (21 per cent), and one of 39 with other kinds of cirrhosis (2.6 per cent). The increased prevalence of MZ in patients with cryptogenic cirrhosis and with chronic active hepatitis is highly significant (P < 0.001). Because serum levels of alpha 1-antitrypsin may be unreliable for identification of the subgroup of patients with chronic active hepatitis or cryptogenic cirrhosis, analysis of serum for the MZ phenotype and meticulous examination of biopsy specimens may be necessary.

References

Sep 1, 1975·American Journal of Clinical Pathology·J LiebermanJ McMahon
Oct 1, 1975·Acta Medica Scandinavica·S ErikssonI Hägerstrand
Feb 1, 1977·Journal of Clinical Pathology·W K Blenkinsopp, G P Haffenden
Dec 1, 1978·The American Journal of Medicine·C H ChanE A Jones
Jan 1, 1978·Annals of Internal Medicine·P E PalmerH J Wolfe
Nov 9, 1978·The New England Journal of Medicine·J O Morse
May 1, 1979·The Histochemical Journal·B L MephamB S Mitchell
Dec 21, 1972·The New England Journal of Medicine·N O Berg, S Eriksson
Jun 1, 1968·Gut·G Walker, D Doniach
Jun 1, 1974·Acta Medica Scandinavica·S Eriksson, I Hägerstrand
Dec 1, 1974·Annals of Internal Medicine·W RawlingsS R Hamilton
Feb 1, 1973·Annals of Internal Medicine·J L CampraT B Reynolds
Sep 1, 1965·Immunochemistry·G ManciniJ F Heremans
Aug 19, 1972·British Medical Journal·C A PorterR Williams
Nov 23, 1972·The New England Journal of Medicine·R C TalamoH Kazemi

❮ Previous
Next ❯

Citations

Mar 1, 1992·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·M RabinovitzD H Van Thiel
Oct 2, 1998·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·I W GraziadeiM K Porayko
Dec 1, 1987·Digestive Diseases and Sciences·J RakelaJ Ludwig
Mar 3, 2006·Current Gastroenterology Reports·Jeffrey H Teckman, Douglas Lindblad
Nov 30, 2000·Current Treatment Options in Gastroenterology·D H Perlmutter
Nov 15, 1985·Clinica Chimica Acta; International Journal of Clinical Chemistry·B E Lindmark, S G Eriksson
Jan 28, 1984·Lancet·R SchnabelF Janssen
Dec 29, 2000·Journal of Hepatology·H P FischerH Zhou
Apr 12, 2005·Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association·Christopher L BowlusCharlie Strange
Jul 16, 1981·The New England Journal of Medicine·J Lieberman
Sep 1, 1984·Proceedings of the National Academy of Sciences of the United States of America·A WallmarkS Eriksson
Jun 18, 1998·The American Journal of Surgical Pathology·H Zhou, H P Fischer
Dec 1, 1983·British Journal of Haematology·R F StevensR T Wensley
Feb 1, 1994·Acta Paediatrica. Supplement·J R Poley
Oct 3, 2003·American Journal of Respiratory and Critical Care Medicine·UNKNOWN American Thoracic Society, UNKNOWN European Respiratory Society
Dec 1, 1986·The Journal of Clinical Investigation·R W Carrell
Apr 1, 1989·The Journal of Clinical Investigation·J A CarlsonS L Woo
Apr 9, 1999·The Journal of Clinical Investigation·R MahadevaD A Lomas
Nov 20, 2012·Hepatitis Monthly·Aleksandra TopicDragica Radojkovic
Sep 26, 1981·British Medical Journal
Mar 1, 1990·Baillière's Clinical Gastroenterology·A M Brind, M F Bassendine
Jun 8, 2011·Digestive Diseases and Sciences·Albert J Czaja
Jul 1, 1990·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·S A GellerJ A Sorge
Jun 1, 1985·Clinical Immunology and Immunopathology·S N BreitR Penny
Apr 25, 2008·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Keli Hansen, Simon Horslen
Feb 13, 2014·European Journal of Gastroenterology & Hepatology·Tessa M CacciottoloWilliam J H Griffiths
Dec 17, 2008·Journal of Perinatology : Official Journal of the California Perinatal Association·D J Askew, G A Silverman
Jan 13, 2000·American Journal of Clinical Pathology·K G Ishak
Sep 1, 1984·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·R Hultcrantz, S Mengarelli
Sep 11, 1998·The American Journal of Gastroenterology·B F BannerH L Bonkovsky
Jan 6, 2007·Journal of Pediatric Gastroenterology and Nutrition·Kathleen M CampbellJorge A Bezerra
Sep 18, 2008·The American Journal of Gastroenterology·Kyrsten D Fairbanks, Anthony S Tavill
Jan 16, 2007·The American Journal of Gastroenterology·David A ElphickDermot Gleeson

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.