The laboratory profile was predominantly that of anicteric cholestasis but with normal liver synthetic function and platelet count: alanine aminotransferase 40 ± 29 IU/L (normal range [NR] ≥ 40), aspartate aminotransferase 40 ± 23 IU/L (NR ≤ 35), gamma-glutamyltranspeptidase 142 ± 191 IU/L (NR ≤ 40), ALP 188 ± 126 IU/L (NR ≤ 110), bilirubin
12.7 ± 7.6 μmol/L (NR ≤ 22), INR 0.97 ± 0.12 (NR 0.80-1.20), Platelet 259 ± 96 × 109/L (NR 150-400), and immunoglobulin M 3.4 ± 2 g/L (NR 0.4-2.3). Hemoglobin, thyroid-stimulating hormone, or creatinine levels did not identify a metabolic basis for fatigue in any patient reviewed. The average disease duration was 7.2 ± 5.4 years (range, 0-21). At diagnosis, 301 patients (92%) were AMA-positive. At the time of the questionnaire, only 258 patients (79%) remained AMA-positive, as evaluated by AMA-M2 enzyme-linked immunosorbent
assay, in keeping with the reported fall of CDK inhibition AMA titers on treatment.27 Baseline histological data were available for 291 patients (89%), with most of the patients (78%) diagnosed at an early stage of fibrosis. None of the patients with PBC included had decompensated liver disease, but 31 patients had a history of esophageal varices. The overwhelming majority of patients (n = 315, 96%) were treated with UDCA. Treatment response data28-30 were available across a total of 261 patients. Of the 327 patients, 323 patients (98.8%) successfully completed Adenylyl cyclase the PBC-40 in full. Scores for all domains selleck products were as follows: Symptoms domain 17.3 ± 4.7 (possible range, 7-3); Itch domain 4.1 ± 3.4 (possible range, 3-15); Fatigue domain 27.4 ± 11.2 (possible range, 11-55); Cognition domain 12.0 ± 5.8 (possible range, 6-30); Social and Emotional (S&E) domains 27.6 ± 11.6 (possible range, 13-65). The distributions of scores of the PBC-40 domains are as shown in Fig. 1. The most frequently seen status was none (54%) for Itch, mild for Symptoms (64%), Cognition (44%), Social and Emotional (72%), and Fatigue (50%). The scores observed in the current study are similar to, albeit numerically lower than, those previously reported from Newcastle,
UK (Table 2). There was as expected inter-domain correlation across the PBC-40 domains (Table 3), with the Fatigue domain scores showing close correlations with Symptoms, Cognition, and Social and Emotional domains score but less correlation with Itch scores. Although each domain of PBC-40 is not equally weighted, the total score similarly correlates strongly with each domain. However, the validity of a total PBC-40 score requires further evaluation. One hundred ninety-six patients completed the questionnaire for the second time, approximately 1 year later. Within the two PBC-40 domains Fatigue and Itch, there were no significant changes in the score reported, P > 0.05; in other words, the score was reproducible over time.