To evaluate analytic and clinical performance of plasma thrombin–antithrombin complex (TAT) and d-dimer assay in assessing the severity and outcome of acute ischemic stroke. The prospective study was conducted and extended from January 2018 to December 2018. A total of 236 patients admitted within 24 h after neurologic symptoms onset were recruited. The median TAT and d-dimer levels were significantly higher in the acute ischemic stroke patients than in the controls. The average TAT levels in patients with mild, moderately severe and severe stroke were 1.75 [interquartile ranges (IQR), 1.1–2.6], 3.3 (IQR, 1.8–4.5) and 13.5 (IQR, 7.2–15.3) ng/ml. The d-dimer levels of respective patient groups were 0.39 (IQR, 0.22–0.73), 0.58 (IQR, 0.39–1.25) and 3.59 (IQR, 1.73–4.74) mg/l. With the optimal cut-off TAT level (1.75 ng/ml) determined from receiver operating characteristic analysis, the Area under the curve (AUC), the sensitivity and specificity of TAT for stroke diagnosis were 0.763, 58.1 and 87.8%. The cut-off d-dimer level was 0.38 mg/l and the AUC, the sensitivity and specificity were 0.772, 60.2 and 88.9%. The Area under the receiver operating characteristic curves (AUROCs) and sensitivity in the moderate to severe stroke increased to 0.903 and 86.9% for TAT, and 0.880 and 80.3% for d-dimer, respectively. Age and high TAT level were significant independent risk factors for stroke severity. Age, high initial National Institutes of Health Stroke Scale score and high TAT level were significant independent poor prognostic factors on multivariate analysis. TAT and d-dimer were superior in separating the moderate-to-severe stroke than mild stroke. A high TAT plasma level is an independent predictor for stroke severity and poor prognosis during 1-month follow-up.
Gilbert syndrome (GS) is a hereditary unconjugated hyperbilirubinemia that results from mutations in the bilirubin uridine diphosphate-glucuronosyltransferase (UGT1A1) gene. To the best of our knowledge, there are currently no reports that focus on patients with systemic lupus erythematosus (SLE) coexisting with GS. The present study aimed to evaluate the clinical characteristics and genotype of UGT1A1 in a Chinese patient with SLE and GS. Complete medical records and laboratory data were reviewed for a patient with SLE referred to Ruijin Hospital (Shanghai, China) for treatment between March 2016 and January 2020. Genetic analysis of the UGT1A1 gene was performed by PCR amplification and Sanger sequencing. The serum total bilirubin and unconjugated bilirubin concentrations on admission were 96.2 and 86.8 µmol/l, respectively. The homozygous mutation c.1456T>G (p.Y486D) in exon 5 was detected in this patient. The patient had a good response to phenobarbital orally at a dose of 30 mg/day and a decrease in serum bilirubin was observed. Elevated unconjugated hyperbilirubinemia in SLE needs to be differentiated from other diseases, such as GS, which can be diagnosed by UGT1A1 genetic sequencing.
Objectives: Acquired hemophilia A (AHA) is a rare disease resulting from autoantibodies against coagulation factor VIII that leads to spontaneous bleeding. This study reports the clinical characteristics and treatment outcomes of a relatively sizable cohort of patients with AHA. Methods: We retrospectively analyzed the characteristics and outcomes of 42 patients with AHA diagnosed in our center from January 2014 through December 2018. Results: The FVIII activity (FVIII: C) was significantly suppressed (median 1.5%; interquartile range [IQR]: 0.9-3.5) by FVIII inhibitor (median 8 BU/mL; IQR: 4.0-16.0). Bypassing agents, PCC or FVIIa, were used in 14 patients for bleeding control without any adverse reaction; and most patients (90.5%, 38/42) were placed on immunosuppressive regimen, corticosteroid alone or in combination with cyclophosphamide. Patients treated with corticosteroids alone had a lower median inhibitor titer (8 BU/mL) than those treated with combination corticosteroids of cyclophosphamide (16 BU/mL) (p < 0.001). 97.4% (37/38) patients achieved complete remission (CR) after immunosuppression therapy, and the median time to CR in patients treated with corticosteroids alone was shorter than those with combination corticosteroids of cyclophosphamide (median 40 days; IQR: 31-65 vs. 51 days; IQR: 38-83, p = 0.301). 10 (26.3%) patients relapsed thereafter and were placed on combined corticosteroid and cyclophosphamide treatment, which yielded second remission in 8 patients (80%). Two patients died, one from uncontrolled post-surgical retroperitoneal hemorrhage and one from sepsis complicating corticosteroid therapy. Conclusion: The corticosteroid achieves a satisfactory outcome, particularly with low inhibitors titers; and combination of cyclophosphamide will facilitate remission in sever patients with high titers of inhibitors.
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