Primary sclerosing cholangitis in two brothers: report of a family with special emphasis on molecular HLA and MICA genotyping.

Immune mechanisms play a role in the pathogenesis of primary sclerosing cholangitis (PSC), as suggested by its association with certain HLA haplotypes. Genetic predisposition is supported by its occurrence in families, but data are scarce. Our aim is to report on two brothers with PSC, and to investigate HLA and MICA alleles in this family. The clinical, biochemical, radiological, and pathological findings in two brothers with PSC as well as in their sister and parents were reviewed. Molecular genotyping of HLA class II and MICA alleles was performed in all five family members. In two brothers, p-ANCA positive PSC was found. The youngest also had ulcerative colitis, and had evolved into cirrhosis at the age of 17 years. Their mother had positive p-ANCA and mild cholestatic changes. Their father and sister were unaffected. Both brothers were homozygous for the MICA*00801 allele, and were positive for the susceptibility HLA haplotypes DR3-DQ2 and DR6-DQ6. Their unaffected father and sister both carried the protective DR4 allele. The presence of PSC in two brothers, and the distribution of HLA haplotypes and MICA alleles, adds supportive evidence for an immunogenetic origin of PSC.

A randomized trial comparing injection therapy with hemoclip and with injection combined with hemoclip for bleeding ulcers.

BACKGROUND: A randomized comparative study was conducted of injection therapy with epinephrine-polidocanol (1%) versus hemoclip application, versus injection combined with hemoclip for bleeding peptic ulcers. METHODS: One hundred five patients were randomized and 101 could be evaluated (46 had active spurting or oozing of blood; 55 a visible vessel). Patients were randomized to 1 of the 3 treatment modalities during endoscopy performed within 12 hours of admission. Endoscopy was repeated after 1 day or at recurrence of bleeding and before discharge. In case of recurrent bleeding, patients were retreated with the same modality. RESULTS: Initial failure or the rate of early recurrence of bleeding was highest (but not statistically significant) in the hemoclip group (13/35; 37%), versus the injection (5/34; 15%) and combination (8/32; 25%) groups. Overall failure was significantly (p = 0.01) different among the 3 groups with the highest rate in the hemoclip group (12/35; 34%), versus the injection (2/34; 6%) and combination therapy (8/32; 25%) groups. The use of hemoclips alone appeared to fail because of difficulty with hemoclip placement and incomplete vessel compression. Complications included 1 perforation in the injection group and possibly 1 case of septic arthritis in the combination therapy group. CONCLUSION: In this study, endoscopic treatment of bleeding peptic ulcers with the hemoclip was inferior overall to injection therapy.