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The aim was to study the outcome of donor candidate investigations for living-related donor liver transplantation from adult to child. The charts of 25 donor candidates were reviewed. All 22 recipients, of whom 18 had BA, were already listed for DD organ transplantation. Eleven donor candidates were accepted. Seven of them successfully donated the left lateral liver segment. At follow-up, all donors and recipients were well from the surgery. However, one donor developed Crohn's disease. In the four remaining cases the recipient deteriorated before transplantation was possible or other surgical approaches were utilized. For three candidates the investigations were never finalized, due to either clinical deterioration of the recipient or the availability of a DD organ. Eleven donor candidates were rejected. Four of them (three being parents of BA patients) had liver abnormalities. Another three were rejected for cardiopulmonary disorders and the remaining four for other reasons. We conclude that only seven out of 25 (28%) candidates donated a liver segment. The fact that parents of BA patients have potential liver pathology may be of importance for the understanding of the etiology of the disease and have possible implications for the choice of donors.

CCR5 is a chemokine receptor expressed on T-cells and macrophages. A 32-base pair deletion in the chemokine receptor 5 gene (CCR5-Delta32) leads to a non-functional receptor. Conflicting evidence exists whether this deletion is associated with primary sclerosing cholangitis (PSC). We genotyped the CCR5-Delta32 variant in 363 PSC patients and 366 controls. No significant increase in the Delta32 allele frequency was detected in the PSC patients compared to controls (12.7% vs 10.7% OR = 1.22, 95% CI [0.88, 1.68], P = 0.23). Survival analysis did not reveal any significant effects from CCR5-Delta32 genotypes on disease progression. Thus, in this study (power > 90%, given OR = 2, alpha = 0.05), we were unable to replicate previous findings and our results do not support an involvement of CCR5-Delta32 in either PSC susceptibility or progression.

The association of primary sclerosing cholangitis (PSC) to HLA class II genes was studied by comparing patients from five different European populations. Deduced HLA-DRB1, DQA1, DQB1 haplotypes of 256 PSC patients from England, Italy, Norway, Spain and Sweden were compared to those observed in 764 ethnically-matched controls. Increased frequencies of the DRB1*03, DQA1*0501, DQB1*02 (RR=3.0, P