BackgroundPrimary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease often associated with inflammatory bowel diseases (IBD). Current epidemiological data are limited to studies of predominantly Caucasian populations. Our aim was to define the epidemiology of PSC in a large, ethnically diverse US population.MethodsThe Northern California Kaiser Permanente (KP) database includes records from over 3 million people and was searched for cases of PSC between January 2000 and October 2006. All identified charts were reviewed for diagnosis confirmation, IBD co-morbidity, and major natural history endpoints.ResultsWe identified 169 (101 males) cases fulfilling PSC diagnostic criteria with a mean age at diagnosis of 44 years (range 11-81). The age-adjusted point prevalence was 4.15 per 100,000 on December 31, 2005. The age-adjusted incidence per 100,000 person-years was not significantly greater in men 0.45 (95% CI 0.33 - 0.61) than women 0.37 (95% CI 0.26 - 0.51). IBD was present in 109/169 (64.5%) cases and was significantly more frequent in men than women with PSC (73.3% and 51.5%, respectively, p = 0.005). The cumulative average yearly mortality rate was 1.9%. Age and serum sodium, creatinine and bilirubin at diagnosis and albumin at last entry were identified as significant factors associated with death, liver transplant or cholangiocarcinoma.ConclusionsThe incidence and prevalence of PSC observed in a representative Northern California population are lower compared to previous studies in Caucasian populations and this might reflect differences in the incidence of PSC among various ethnic groups.
The high diagnostic yield of CE influences clinical management leading to improved outcomes. However, the utility of CE may be greater in patients who are referred for certain indications or have specific findings. Additional studies are needed to clarify the role of capsule endoscopy in the evaluation of various indications as well as identify factors associated with positive outcomes.
The endoscopic placement of stents to decompress biliary obstruction is accepted practice in the management of malignant biliary strictures. In our patient, a 73-year-old man with a presumed malignant common hepatic duct stricture, palliation of his obstructive jaundice has been achieved. However, his clinical course was complicated by a colonic perforation--a previously unreported complication of distal stent migration.
In today's nursing climate we are very aware of being clinically effective using research-based evidence which, when audited, gives us information that is valid and of benefit to the patient. This in turn enables us to achieve high standards of care, rather than following the Asthma Guidelines (British Thoracic Society, 1993), as Grimshaw and Russell (1994) have already shown that guidelines alone do not promote good practice.
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