Primary Biliary Cirrhosis Scenario of Libyan Patients in Tripoli University Hospital
DOI:
https://doi.org/10.54361/ljmr.17-04Abstract
Background and aims. Primary biliary cirrhosis (PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies (AMA), and characteristic liver biopsy. A variant, called PBC-autoimmune hepatitis (AIH) overlap, is characterized by the above findings of PBC together with findings of elevated ALT, elevated Ig G, and ASMA. PBC is hypothesized to be related to environmental exposure in genetically vulnerable individuals. It typically occurs in middle-aged females. The aim of the current study is to describe the clinical and biochemical profile of adult Libyan Primary Biliary Cirrhotic patients. Method. This was a case series study conducted by reviewing the medical records of the 50 patients diagnosed as PBC and followed in gastro-enterology clinic in Tripoli university hospital during the study period. A relevant data obtained from the records in a preformed case sheet. Result. The total number of the diagnosed Primary Biliary Cirrhotic patients were 50 patients. All of them were females. Patient age during the study were from 35 to 65 years, about 90% of patients were symptomatic like gastrointestinal symptoms (jaundice in 100%, abdominal distention in 24%, and encephalopathy in 5 patients 10%), while, non- gastrointestinal symptoms like fatigue and weakness in 35 patients70%, skin disorders(pruritis) were diagnosed in 18 patients 36%), dyslipidemia in 15 patients 30%, and osteopenia in 7 patients 14%. Serological tests (AMA) was positive in ail patients and (ASMA) in 20% of patients, biochemical profile showed that Alkaline phosphatase was raised in all patients, while, 10 patients had raised AST and ALT 20%. Conclusion: middle-aged female patient with jaundice, fatigue and elevated alkaline phosphatase should be routinely undergoing serological testing (AMA) to check for primary biliary cirrhosis which should subsequently be verified by liver biopsy.
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References
Kaplan MM Primary biliary cirrhosis,Engl J Med 1996;335;1570-1580[PMD;8900092].
Hirchfield GM, Gershwin ME,,The immunobiology and pathophysiology of primary biliary cirrhosis. Annu Rev Pathol 2013; 8: 303-330 [PMID:23347352. DOI: 10.1146/annurev-pathol-020712-164014].
Addison T, Gull W. On a certain affection of the skin vitiligoidea-a plana, β tuberosa, Guy , s Hosp Rep 1851;7:265-276.
Ahrens EH, Payne MA, Kunkel HG, Eisenmenger WJ. Blondheim SH. Primary biliary cirrhosis. Medicine (Baltimore) 1950; 29: 299-364 [PMID: 14796348].
Walker JG, Doniach D, Roitt IM, Sherlock S. Serological tests in diagnosis of primary biliary cirrhosis. Lancet 1965; 1: 827-831 [PMID: 14796348].
Griffiths L, Dyson JK, Jones DE. The new epidemiology of primary biliary cirrhosis. Semin Liver Dis 2014; 34: 318-328 [PMID; 25057954 DOI:10.1055/s-0034-1383730].
Watson RG, Angus PW, Dewar M, Goss B, Sewell RB, Smallwood RA. Low prevalence of primary biliary cirrhosis in Victoria, Australia. Melboume Liver Group. Gut 1995; 36: 927-930 [PMID: 7615286].
Juran BD, Lazaridis KN. Enviromental factors in primary biliary cirrhosis. Semin Liver Dis 2014; 34: 265-272 [PMID: 25057950 DOI: 10.1055/s-0034-1383726].
Corpechot C, Chretien Y, Chazouilleres O, Poupon R. Demographic, lifestyle, medical and familial factors associated with imary biliary cirrhosis. J Hepatol 2010; 53: 162-169 [PMID: 20471130 DOI:10.1016/j-jhep. 2010.02.019].
Bowlus CL, Gershwin ME. The diagnosis of primary biliary cirrhosis. Autoimmune Rev 2014; 13: 441-444 [PMID:24424173 DOI: 10.1016/j,autrev.2014.01.041].
Floreani A, Franceschet I, Cazzagon N. Primary biliary cirrhosis: overlaps with other autoimmune disorders. Semin Liver Dis 2014; 34: 352-360 [PMID:25057958 DOI:10.1055/s-0034-1383734].
Trivedi PJ, Hirschfield GM. Review article: overlap syndromes and autoimmune liver diseases. Aliment Pharmacol Ther 2012; 36: 517-533[PMID: 22817525 DOI: 10.1111/j.1365-2036.2012.05223. x].
Chazouilleres O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, Poupon R. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy, Hepatology 1998; 28: 296-301 [PMID:9695990]
Rudic JS. Poropat G, Krstic MN, Bjelakovic G, Gluud C. Ursodeoxycholic acid for primary cirrhosis. Cochrane Database Syst Rev 2012; 12: CD000551 [PMID:23235576 DOI: 10.1002/14651858. CD000551.PUB3].
Mazzaferro V, Bhoori S, Sposito C, Bongini M, Langer M, Miceli R, Mariani L. Milan criteria in liver transplantation for hepatocellular carcinoma an evidence-based analysis of 15 years of experience. Liver Transpl 2011; 17 suppl 2: S44-S57 [PMID: 21695773 DOI: 10.1002/lt.22365].:
Raczynska J, Habior A, Paczek L, Foroncewicz B, Pawelas A, Mucha K. Primary biliary cirrhosis in the era of Liver transplantation. Ann Transplant 2014; 19: 488-493 [PMID: 25262831 DOI: 10.12659/AOT.890753].
Invernizzi P, Selmi C, Ranfler C, Podda M, Wesierska-Gadek J. Antinuclear antibodies in primary biliary cirrhosis. Semin Dis 2005; 25:289-310 [PMID: 16143945].
Griffiths L, Dyson jk, Jones DE. The new epidemiology of primary biliary cirrhosis. Semin Liver Dis 2014; 34: 318-328 [PMID: 25057954 DOI: 10.1055/s-0034-1383730].
Cauch-Dudetek K, Abbey S, Stewart DE, Healthcote EJ. Fatigue in primary biliary cirrhosis. Gut 1998; 43: 705-710 [PMID:9824355].
Leuschner U. Primary biliary cirrhosis— presentation and diagnosis. Clin Liver Dis 2003; 7: 741-758 [PMID: 14594129].
Mattalia A,Quaranta S, Leung PS, et al. Characterization of anti-mitochondrial antibodies in health adults. Hepatology 1998; 27: 656-.
Corechot C, Chretien Y, Chazouilleres O, Poupon R. Demographic, Lifestyle, medical and familial factors associated with primary biliary cirrhosis. J Hepatol 2010; 53: 162-169.
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