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[Two cases of asymptomatic primary biliary cirrhosis (PBC) accompanied with CREST syndrome]. 1994

I Takeda, and S Igarashi, and H Oyanagi, and A Irisawa, and S Mukai, and I Shoji, and H Sakuma, and H Satho, and K Sekine, and M Kuroda
Department of Internal Medicine, Ohota Nishinouchi Hospital, Fukushima.

Two cases of asymptomatic primary biliary cirrhosis (PBC) combining CREST syndrome. We have had encountered two primary biliary cirrhosis (PBC) patients overlapped with CREST syndrome. Case 1 was a 51-year-old female, who was suffering from Raynaud's phenomenon, esophageal dysmotility and sclerodactyly. Case 2 was a 67-year-old female, who was suffering from Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia. They were free from itching and icterus. The histology of their biopsied liver specimen should stage I-II of Scheuer's classification. Their immunological findings showed anti-centromere antibody, (ACA) at a high titer (1 : 1280 dilution) and anti-mitochondrial antibody (AMA) at a low titer (1 : 40 dilution) positive in both. HLA DR types included DR2 and DRW8 in case 1, and did DR1 and DRW6 in case 2. Both patients are having good prognosis.

UI MeSH Term Description Entries
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008105 Liver Cirrhosis, Biliary FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cholangitis involves the destruction of small intra-hepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes. Biliary Cirrhosis,Biliary Cirrhosis, Primary,Biliary Cirrhosis, Secondary,Cholangitis, Chronic Nonsuppurative Destructive,Liver Cirrhosis, Obstructive,Primary Biliary Cholangitis,Biliary Cirrhosis, Primary, 1,Primary Biliary Cirrhosis,Secondary Biliary Cholangitis,Secondary Biliary Cirrhosis,Biliary Cholangitides, Primary,Biliary Cholangitis, Primary,Biliary Cholangitis, Secondary,Cholangitides, Primary Biliary,Cholangitis, Primary Biliary,Cholangitis, Secondary Biliary,Cirrhosis, Biliary,Cirrhosis, Secondary Biliary,Liver Cirrhoses, Biliary,Obstructive Liver Cirrhosis,Primary Biliary Cholangitides,Secondary Biliary Cholangitides
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D005260 Female Females
D006650 Histocompatibility Testing Identification of the major histocompatibility antigens of transplant DONORS and potential recipients, usually by serological tests. Donor and recipient pairs should be of identical ABO blood group, and in addition should be matched as closely as possible for HISTOCOMPATIBILITY ANTIGENS in order to minimize the likelihood of allograft rejection. (King, Dictionary of Genetics, 4th ed) Crossmatching, Tissue,HLA Typing,Tissue Typing,Crossmatchings, Tissue,HLA Typings,Histocompatibility Testings,Testing, Histocompatibility,Testings, Histocompatibility,Tissue Crossmatching,Tissue Crossmatchings,Tissue Typings,Typing, HLA,Typing, Tissue,Typings, HLA,Typings, Tissue
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
D017675 CREST Syndrome A mild form of LIMITED SCLERODERMA, a multi-system disorder. Its features include symptoms of CALCINOSIS; RAYNAUD DISEASE; ESOPHAGEAL MOTILITY DISORDERS; sclerodactyly, and TELANGIECTASIS. When the defect in esophageal function is not prominent, it is known as CRST syndrome. CRST Syndrome,Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia Syndrome,Calcinosis-Raynaud Phenomenon-Sclerodactyly-Telangiectasia,CREST Syndromes,CRST Syndromes,Calcinosis Raynaud Phenomenon Sclerodactyly Telangiectasia,Phenomenon-Sclerodactyly-Telangiectasia, Calcinosis-Raynaud,Syndrome, CREST,Syndrome, CRST

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