Background: The PLASMIC score was recently published to aid in the early identification of thrombotic thrombocytopenic purpura (TTP) patients. This study aims to evaluate whether this score is suitable for Chinese suspected TTP patients and find the utility of patients' other characteristics in predicting severe ADAMTS13 deficiency. Methods: We retrospectively studied a Chinese cohort of 38 consecutive hospitalized patients with suspected TTP, ADAMTS13 test results, and other clinical data from September 2016 to May 2018. The predictive power of PLASMIC score in our cohort was evaluated, and patients' other characteristics, especially the high lactate dehydrogenase/the upper limit of normal (LDH/ULN), were studied to determine their distinguishing ability for TTP patients.Results: In this Chinese cohort, 17 patients were diagnosed with TTP according to ADAMTS13 activity results. When dichotomized at intermediate-high risk (scores 5-7) vs low risk (scores 0-4), the PLASMIC score predicted TTP with a sensitivity of 100%, a specificity of 9.52%, and a misdiagnosis rate of 90.48%. And the LDH/ULN alone, or plus platelet count, reticulocyte percentage and indirect bilirubin (IBIL) both had excellent predictive power (area under the curve [AUC] 0.937, 95% confidence interval [CI] 0.863-1.000, P = .000, and AUC 0.994, 95% CI 0.980-1.000, P = .000, respectively). The model including platelet count, reticulocyte percentage, IBIL, and LDH/ULN ratio had a sensitivity of 100%, a specificity of 95.2%, and a misdiagnosis rate of 4.8%.Conclusions: A modified PLASMIC score plus LDH/ULN ratio might be more suitable for identifying ADAMTS13 deficiency patients, especially for making an earlier diagnosis, guiding the immediate and reasonable plasma exchange, and also avoiding unnecessary allocation of plasma. K E Y W O R D SADAMTS13 activity, lactate dehydrogenase, PLASMIC score
Objectives To systematically review studies on the diagnostic accuracy of spleen stiffness measurement (SSM) for the detection of clinical significant portal hypertension (CSPH), severe portal hypertension (SPH), esophageal varices (EV), and high-risk esophageal varices (HREV) in patients with chronic liver diseases (CLD). Methods Through a systematic search, we identified 32 studies reporting the accuracy of SSM for the diagnosis of portal hypertension (PH) and/or EV in adults with CLD. A bivariate random-effects model was performed to estimate pooled sensitivity, specificity, likelihood ratio, positive predictive value (PPV), negative predictive value (NPV), and diagnostic odds ratios (DOR). The clinical utility of SSM was evaluated by Fagan plot. Results A total of 32 studies assessing 3952 patients were included in this meta-analysis. The pooled sensitivities of SSM were 0.85 (95% confidence interval (CI), 0.69–0.93) for CSPH; 0.84 (95% CI, 0.75–0.90) for SPH; 0.90 (95% CI, 0.83–0.94) for any EV; and 0.87 (95% CI, 0.77–0.93) for HREV. The pooled specificities of SSM were 0.86 (95% CI, 0.74–0.93) for CSPH; 0.84 (95% CI, 0.72–0.91) for SPH; 0.73 (95% CI, 0.66–0.79) for EV; and 0.66 (95% CI, 0.53–0.77) for HREV. Summary PPV and NPV of SSM for detecting HREV were 0.54 (95% CI, 0.47–0.62) and 0.88 (95% CI, 0.81–0.95), respectively. Conclusions Our meta-analysis suggests that SSM could be used as a helpful surveillance tool in management of CLD patients and was quite useful for ruling out the presence of HREV thereby avoiding unnecessary endoscopy. Key Points • SSM could be used to rule out the presence of HREV in patients with CLD thereby avoiding unnecessary endoscopy. • SSM has significant diagnostic value for CSPH and SPH with high sensitivity and specificity in patients with CLD. • SSM could be used as a helpful surveillance tool for clinicians managing CLD patients.
Background Patients with primary biliary cholangitis (PBC) often have comorbid dyslipidemia, and determining the degree of hepatic steatosis can help predict the risk of cardiovascular events in PBC patients. The aim of our study was to analyze the characteristics of lipid distribution and the degree of hepatic steatosis in PBC. Methods We retrospectively analyzed 479 cases of PBC, chronic hepatitis B (CHB), chronic hepatitis C (CHC), non-alcoholic fatty liver disease (NAFLD), and healthy subjects (Normal) diagnosed by liver biopsy or definitive clinical diagnosis. Controlled attenuation parameter (CAP) values were applied to assess the degree of steatosis of the liver, and lipid levels were also compared in the five cohorts. Results We found that among the five groups of subjects, the PBC group had the lowest CAP values (P < 0.001), and the high-density lipoprotein cholesterol (HDL-C) level in the PBC group was higher than normal, CHC and CHB group (P = 0.004, P = 0.033, P < 0.001, respectively).In the multivariate linear analysis, only BMI (β = 1.280, P = 0.028), ALP (β = − 0.064, P = 0.012), TBA (β = − 0.126, P = 0.020), TG (β = 12.520, P = 0.000), HDL-C (β = − 11.338, P = 0.001) and LDL-C (β = 7.012, P = 0.002) were independent predictors of CAP. Conclusions Among PBC, CHB, CHC, NAFLD and healthy subjects, PBC had the lowest degree of hepatic steatosis and higher HDL-C levels, all of which were found to be protective factors against atherosclerosis and cardiovascular risk and would provide a valuable reference for the risk of developing cardiovascular events in PBC patients.
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