Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study.

BACKGROUND AND AIMS: Little is known regarding the epidemiology and outcomes of patients with primary sclerosing cholangitis (PSC) in Australia. We, therefore, evaluated the epidemiology and clinical outcomes of PSC in a large cohort of Australian patients and compared these to the general population. METHODS: We conducted a multicentre, retrospective cohort study of PSC patients at nine tertiary liver centers across three Australian states, including two liver transplant centers. RESULTS: A total of 413 PSC patients with 3,285 person-years of follow-up were included. Three hundred and seventy-one (90%) patients had large duct PSC and 294 (71%) had associated inflammatory bowel disease. A total of 168 (41%) patients developed cirrhosis (including 34 at the time of PSC diagnosis) after a median of 15.8 (95% CI 12.4, NA) years. The composite endpoint of death or liver transplantation occurred in 49 (12%) and 78 (19%) patients, respectively, with a median transplant-free survival of 13.4 (95% CI 12.2-15) years. Compared to the general population, PSC accounted for a 240-fold increased risk of development of cholangiocarcinoma (CCA) and CCA-related death. CCA risk was increased with older age of PSC diagnosis, presence of dominant stricture and colectomy. Compared to same-aged counterparts in the general population, PSC patients who were diagnosed at an older age or with longer disease duration had reduced relative survival. CONCLUSION: In this large retrospective cohort study of PSC patients in Australia, increased age and time from diagnosis was associated with increased mortality and morbidity particularly from CCA and development of cirrhosis, necessitating need for liver transplant.

The effectiveness of MMPI-A items in discriminating between normative and clinical samples.

The Minnesota Multiphasic Personality Inventory-Adolescent (MMPI-A; Butcher et al., 1992) was released in 1992 and has rapidly become the most widely used objective personality assessment instrument with adolescents. Although the MMPI-A reduced or eliminated several problems associated with the use of the original MMPI (Hathaway & McKinley, 1943) with adolescents, the MMPI-A does produce a high frequency of within normal limits basic scale profiles for individuals with substantial psychopathology including adolescents in inpatient psychiatric settings. To better understand the reasons for this phenomenon, we compared the item endorsement frequencies for the MMPI-A normative sample with results from two adolescent clinical samples, and these results were contrasted to the item endorsement frequencies for the MMPI-2 (Butcher, Dahlstrom, Graham, Tellegen, & Kaemmer, 1989) normative sample and a clinical sample of adult psychiatric inpatients. Results showed that the MMPI-A contains a substantial number of items that do not show a significant difference in item endorsement frequency between normative and clinical samples. Furthermore, MMPI-A basic and content scales generally show a much lower percentage of effective items than do the corresponding scales for the MMPI-2. We discuss the findings in relation to the frequent occurrence of low range MMPI-A profiles in clinical samples and the potential usefulness of these results in future efforts to develop viable short forms for the MMPI-A.