Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are characterized by a cholestatic pattern of liver damage, also observed in hereditary or acquired dysfunction of the canalicular membrane transporters bile salt export pump (BSEP, ABCB11) and multidrug resistance protein type
HLA DPB polymorphism in primary sclerosing cholangitis and primary biliary cirrhosis
β Scribed by James A. Underhill; Peter T. Donaldson; Derek G. Doherty; Koji Manabe; Roger Williams
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 515 KB
- Volume
- 21
- Category
- Article
- ISSN
- 0270-9139
No coin nor oath required. For personal study only.
β¦ Synopsis
In recent years there has been an increasing interest in the link between susceptibility to autoimmune liver disease and genes of the HLA system, although the role of the DPBl locus in British patients has only been investigated in autoimmune hepatitis. The aim of the current study was to determine the distribution of DPBl alleles in a large series of British patients with the two other autoimmune liver diseases, primary biliary cirrhosis and primary sclerosing cholangitis, and compare the allele frequencies obtained with those of a geographically matched control group. Polymerase chain reaction sequence-specific oligonucleotide probing was used to as- sign 18 DPBl alleles in 82 patients with primary biliary cirrhosis (PBC), 71 patients with primary sclerosing cholangitis (PSC), and 103 controls. The frequencies of the DPBl alleles were not significantly different comparing patients and controls. However, two important observations were made. Firstly, in primary sclerosing cholangitis, the previously reported association with the haplotype Al-Bf3-DR3-DQ2 does not extend to the DPBl locus, suggesting that the genetic determinants of susceptibility for this disease lie closer to the DRB loci. Secondly, in primary biliary cirrhosis there is evidence that the reported association with DR8-DQB1*0402 includes the DPB1*0301 allele. The weak HLA association reported here is in contrast with recent data from Japan, where susceptibility is strongly linked to a particular amino acid residue encoded by the DPBl*0501 allele. These data clearly demonstrate that the alleles of the DPBl locus are not associated with susceptibility to or protection from either primary biliary cirrhosis or primary sclerosing cholangitis in British patients. (J~EPA-TOLOGY 1995; 21:959-962.
π SIMILAR VOLUMES
1. Recurrence of primary sclerosing cholangitis. a Five percent of liver transplants are performed because of end-stage liver disease secondary to primary sclerosing cholangitis. b Recurrent disease affects 10% to 27% of recipients. c Diagnostic criteria of recurrence include the following: A confir
is suggested, and transplantation should be taken into Primary sclerosing cholangitis (PSC) is a chronic inconsideration at scores above 4. (HEPATOLOGY 1996; flammatory disease associated in 10% to 36% of those 23:1105-1111.) with hepatobiliary malignancies, which are, in the majority of cases, not
The major histocompatibility complex class I1 alleles at the HLA-DPB1 locus were investigated in 32 German Caucasoid patients with primary biliary cirrhosis (PBC) and compared with those from 47 normal control patients using molecular genotyping techniques. The second exon of the HIA-DPB1 gene was a