Biomedical subjects

George R MacFaul

Publications and source records attributed to George R MacFaul.

3 recordsLinked to original sources

PubMed2006

Sclerosing cholangitis.

PURPOSE OF REVIEW: Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree complicated by cirrhosis and cholangiocarcinoma. It is immune mediated although the precise etiology remains unknown. RECENT FINDINGS: Research into etiopathogenesis, epidemiology, the relationship with inflammatory bowel disease, diagnosis of cholangiocarcinoma, medical therapy, and the outcome of liver transplantation are discussed. SUMMARY: It is likely that a number of patients previously diagnosed with primary sclerosing cholangitis have autoimmune pancreatitis in association with primary sclerosing cholangitis, a syndrome with distinct clinicopathological features including steroid responsiveness. Primary sclerosing cholangitis-inflammatory bowel disease probably represents a distinct inflammatory bowel disease phenotype, which has implications for colonoscopic surveillance of these patients. CA19-9 plays no surveillance role for the early detection of cholangiocarcinoma. The best-studied drug in primary sclerosing cholangitis is ursodeoxycholic acid, which, despite a range of potentially valuable actions on the cholestatic liver, has not yet been proved to make a substantial impression on the course of the disease. Orthotopic liver transplantation remains the only established long-term treatment for primary sclerosing cholangitis.

Bile Duct Neoplasms↗

PubMed2005

Sclerosing cholangitis.

PURPOSE OF REVIEW: Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree. It is immune mediated, although the precise cause remains unknown. Recent reports have shown a higher prevalence and burden of disease than was previously suspected. RECENT FINDINGS: The research into the etiopathogenesis, epidemiology, diagnosis of cholangiocarcinoma, medical and surgical therapy, and timing and outcome of liver transplantation is discussed. SUMMARY: Genetic heterogeneity among patients with primary sclerosing cholangitis is supported, and further gene polymorphisms associated with protection against primary sclerosing cholangitis have been elucidated. Bile duct injury seems to be a multistep process. Magnetic resonance cholangiopancreatography is a cost-effective and accurate way of diagnosing primary sclerosing cholangitis in comparison with endoscopic retrograde cholangiopancreatography. Ursodeoxycholic acid may have a role as a colorectal and hepatobiliary cancer chemopreventive agent. Liver transplantation remains the only treatment in end-stage disease. The 5-year and 10-year patient and graft survival rates are comparable with those in patients without primary sclerosing cholangitis, but there is a higher rate of retransplantation for primary sclerosing cholangitis in most centers. Hepatobiliary malignancy is found in a minority of patients at transplantation, although 5-year survival rates for these patients are still promising.

Cholangitis, Sclerosing↗

PubMed2004

Sclerosing cholangitis.

PURPOSE OF REVIEW: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by strictures of the biliary tree. It is closely associated with inflammatory bowel disease, particularly ulcerative colitis, although the precise etiology remains unknown. The prevalence of PSC, with its attendant medical burdens, appears to be higher than previously estimated; consequently, increasing numbers of patients are being diagnosed and treated in secondary care. RECENT FINDINGS: Research into etiopathogenesis, epidemiology, small-duct PSC, and associations with cholangiocarcinoma and colorectal carcinoma are discussed, along with medical therapy and transplantation. SUMMARY: Small-duct PSC is a distinct clinical entity associated with a benign course and minimal risk of cholangiocarcinoma compared with large-duct PSC. The incidence of gall bladder cancer is increased in patients with PSC, and yearly ultrasonographic screening, with cholecystectomy for gall bladder polyps, is recommended. Recent pilot studies have suggested that high-dose ursodeoxycholic acid (20 mg/kg body weight) may slow liver fibrosis and cholangiographic appearances. Importantly, ursodeoxycholic acid has also been shown to reduce the incidence of colonic dysplasia and colorectal cancer in patients with PSC and ulcerative colitis. Endoscopic therapy, usually balloon dilatation, is effective for benign dominant strictures that are causing jaundice. Liver transplant remains the only treatment in end-stage disease; however, posttransplant recurrence of PSC occurs in as many as one third of patients. Colorectal cancer develops posttransplant in 5 to 10% of those with inflammatory bowel disease. Annual screening colonoscopy is recommended for all patients with PSC with coexisting inflammatory bowel disease.

Journal Article↗