These results show a significantly greater incidence of hernia, which could be an additional manifestation of the underlying defect in extracellular matrix production identified in patients with ADPKD.

No abstract

A variable number tandem repeat (VNTR) polymorphism exists within intron 2 of the human interleukin-1 receptor antagonist gene, consisting of perfect repeats of an 86-bp sequence. Five allelic variants have been identified wherein the number of repeats varies from two to six. This is the first report of a rare, single-copy allele designated IL1RN*0.

Granulomatous inflammation appears to be part of a spectrum of sigmoid diverticulitis. In this setting, caution should be exercised to avoid an inappropriate diagnosis of Crohn's disease.

Aim: To report the prevalence and outcome of cholangiocarcinoma arising in primary sclerosing cholangitis for a British tertiary referral centre. Methods: All patients diagnosed with primary sclerosing cholangitis and concurrent cholangiocarcinoma were identified from a prospectively maintained departmental database, and the mode of presentation, management and outcome were determined. Results: Of 370 patients with primary sclerosing cholangitis, 48 patients (13%) were diagnosed with a cholangiocarcinoma within a median time of 0.51 months (range: 0–73.12) from presentation to the unit. Mode of presentation included: inoperable tumours (n = 14); incidental findings in transplant hepatectomy specimens (n = 13); primary sclerosing cholangitis follow-up (n = 9); transplant work-up (n = 5); transplant waiting list (n = 5); suspected tumour confirmed at transplant (n = 1), and incidental finding at cholecystectomy (n = 1). The diagnosis was confirmed by: radiology-guided biopsy (n = 27); MRI (n = 3); CT (n = 2); laparoscopy or laparotomy (n = 2), and frozen section at transplant (n = 1). Management consisted of: transplantation (n = 14, including 1 abandoned); hepatic resection (n = 8), and palliation through stenting (n = 26). The overall median survival of the cohort was 4.9 months (range: 0.09–104.5). Median survival ranged from 2.6 months (range: 0.09–35.3) for palliation to 7.6 months (range: 0.6–99.6) for transplantation and 52.8 months (range: 3.7–104.5) for resection. There was no difference in survival between the transplant and resection groups (p = 0.14). Conclusions: Cholangiocarcinoma is a common finding in primary sclerosing cholangitis and regular screening of this cohort of patients at referring centres is advocated to detect early tumours, as surgical treatment at an early stage offers significantly better outcomes for this cohort of patients.