Factors Affecting the Discrepancy Between Coagulation Times on Extracorporeal Circulation Using Unfractionated Heparin in Children and Young Adults.

In unfractionated heparin (UFH) monitoring during extracorporeal circulation, the traditional measures of activated clotting time (ACT) or activated partial thromboplastin time (APTT) may diverge, confounding anticoagulant adjustments. We aimed to explore the factors explaining this discrepancy in children and young adults. This retrospective observational study, conducted at an urban regional tertiary hospital, included consecutive pediatric patients who received UFH during extracorporeal circulation (continuous kidney replacement therapy or extracorporeal membrane oxygenation) between April 2017 and March 2021. After patients whose ACT and APTT were not measured simultaneously or who were also taking other anticoagulants were excluded, we analyzed 94 samples from 23 patients. To explain the discrepancy between ACT and APTT, regression equations were created using a generalized linear model (family = gamma, link = logarithmic) with ACT as the response variable. Other explanatory variables included age, platelet count, and antithrombin. Compared to APTT alone as an explanatory variable, the Akaike information criterion and pseudo-coefficient of determination improved from 855 to 625 and from 0.01 to 0.42, respectively, when these explanatory variables were used. In conclusion, we identified several factors that may explain some of the discrepancy between ACT and APTT in the routinely measured tests. Evaluation of these factors may aid in appropriate adjustments in anticoagulation therapy.

A case of anti-NPX-2 antibody-positive dermatomyositis resulting in massive haemothorax with acquired factor XIII deficiency.

Autoimmune diseases, including dermatomyositis, can be complicated by an acquired autoimmune coagulation factor XIII deficiency, which sometimes results in fatal bleeding. Here, we report the case of a young woman with anti-NPX-2 antibody-positive dermatomyositis who developed massive haemothorax with acquired factor XIII deficiency during treatment, including plasma exchange therapy. Emergency transcatheter arterial embolisation was performed and coagulation factor XIII concentrates (Fibrogammin P® 240 U/day for 5 days) were supplemented. Subsequently, the patient was discharged and managed with oral prednisolone and tacrolimus. Coagulation system test results were followed up regularly and remained within normal limits and the patient progressed without recurrence of bleeding symptoms. Coagulation factor XIII deficiency cannot be assessed without measuring coagulation factor XIII activity because common coagulation-fibrinolytic system test results are not abnormal. The measurement of factor XIII activity should be performed when autoimmune diseases are complicated by unexplained bleeding.

Effect of post-ICU follow-up by a rapid response team after congenital heart surgery.

Patients with congenital heart disease who have a variety of cardiac/extracardiac problems are at high risk for deterioration. This study aimed to determine the effectiveness of post-intensive care unit (ICU) follow-up by a rapid response team (RRT) after congenital heart surgery. This before-and-after study was conducted at an urban regional tertiary hospital. We enrolled 572 consecutive patients who underwent congenital heart surgery and were transferred alive from the paediatric ICU (PICU) between April 2015 and March 2020. Post-ICU follow-up for 48 h was started in April 2018. The primary and secondary endpoints were unplanned ICU readmission and clinical outcomes at ICU readmission, respectively. Overall, 346 and 226 patients were analysed pre- and post-intervention, respectively. Patient demographics were similar between groups, but in the post-intervention group, patients tended to have had more complicated surgery. Unplanned ICU readmission rates within 30 days were similar between groups. Regarding the demographics and outcomes at ICU readmission, patients in the post-intervention group had lower predicted mortality rates (1.7% vs 5.3%, P = 0.001), required less ventilator days (median, 0.5 days [interquartile range (IQR) 0-1] vs median, 3 days [IQR 0.5-4], P = 0.02), and had a shorter ICU stay (median, 3 days [IQR 2-4] vs median, 6 days [IQR 3-9], P = 0.03), but there was no significant between-group difference in ICU mortality. Post-ICU follow-up by a RRT after congenital heart surgery did not decrease unplanned ICU readmission but improved several outcomes at ICU readmission.