Efficacy and safety of ketamine for pediatric and adolescent super-refractory status epilepticus and the effect of cerebral inflammatory conditions.

OBJECTIVE: To investigate the short-term benefits and adverse effects of ketamine in the treatment of pediatric and adolescent super-refractory status epilepticus (SRSE), with a focus on the inflammatory etiology. METHODS: This retrospective observational cohort study included a consecutive series of 18 pediatric to adolescent patients with SRSE admitted between 2008 and 2023 and treated with ketamine. Seizure frequency per hour before and after ketamine administration and response rate were calculated. Neurological decline, catecholamine administration, and adverse effects were also assessed. The patients were divided into inflammatory and non-inflammatory etiology groups. RESULTS: The median age at SRSE onset was 1 year 5 months (range: 11 days-24 years), and 78% of the patients were male individuals. The median duration of treatment was 7.5 days (interquartile range: 2.8-15.5 days). Fifteen (83%) patients achieved >50% seizure reduction. The median seizure frequency before and after ketamine treatment was 5.9 and 0.9, respectively, showing a significant reduction in seizure frequency (p < 0.0001). Ten patients had inflammatory etiologies including bacterial meningitis (n = 2), viral encephalitis (n = 3), and febrile infection related epilepsy syndrome (n = 5). The inflammatory etiology group required a longer treatment duration (p = 0.0453) and showed lower seizure reduction (p = 0.0264), lower response rate (p = 0.0044), and higher neurological decline (p = 0.0003) than the non-inflammatory etiology group. Three (17%) patients experienced transient adverse events requiring intervention within 24 h of initiating ketamine administration. CONCLUSIONS: Ketamine administration was associated with fewer serious adverse events and a reduced seizure frequency. Additionally, inflammatory conditions may weaken the efficacy of ketamine in patients with SRSE.

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.

Association Between Post-procedure Intracardiac Pressures and the Use of Temporary Epicardial Pacing Wires After Pediatric Cardiac Surgery.

The indications for using temporary epicardial pacing wires after pediatric cardiac surgery remain unclear. Post-procedure intracardiac pressure is valuable for detecting circulatory disturbances and residual lesions. This study aimed to examine the association between post-procedure intracardiac pressures and the use of temporary epicardial pacing wires. We performed a retrospective, case-control study at the pediatric intensive care unit of an urban regional tertiary hospital that included patients who had undergone congenital heart surgery between January 2015 and December 2016. We measured post-procedure intracardiac pressures, and data regarding baseline characteristics, procedures performed, and intraoperative variables were collected as covariates. Of the 186 included patients, 34 (18.3%) were treated using temporary epicardial pacing wires. The optimal cutoff values used to predict the use of pacing wires for central venous pressure, left atrial pressure, pulmonary arterial pressure/systemic blood pressure ratio, and right ventricular pressure/left ventricular pressure ratio were 11 mmHg (55.6% sensitivity, 86.2% specificity), 13 mmHg (50% sensitivity, 84.6% specificity), 0.39 (69.6% sensitivity, 78.7% specificity), and 0.51 (74.1% sensitivity, 64.2% specificity), respectively. Multivariable logistic regression analyses showed that the use of temporary epicardial pacing wires was significantly associated with left atrial pressure ≥ 11 mmHg (odds ratio 4.4; 95% confidence interval 1.01-18.9), and a pulmonary arterial pressure/systemic blood pressure ratio ≥ 0.39 (odds ratio 6.3; 95% confidence interval 1.3-31.4). High post-procedure intracardiac pressures were associated with the use of temporary epicardial pacing wires. These data can aid in the decision-making for the proper use of temporary epicardial pacing wires.

Cardiac arrest caused by rapidly increasing ascites in a patient with TAFRO syndrome: a case report.

Case: Thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly (TAFRO) syndrome is a newly defined systemic inflammatory disorder with gradual progression of symptoms. A 59-year-old man with fever and ascites of unknown cause developed sudden-onset shock and respiratory failure in the general ward. Cardiac arrest immediately followed. Although he was resuscitated, frequent administration of adrenaline was required to maintain his blood pressure. His circulation was most effectively stabilized by drainage of fluid from his distended abdomen. The volume of discharged ascites reached 4,000 mL at that time, and several liters continued to be discharged for >1 month. The diagnosis of TAFRO syndrome was based on the clinical features and laboratory and histological findings. Outcome: The ascites volume and concentrations of inflammatory parameters decreased with treatment using several immunosuppressive agents. Conclusion: The newly defined TAFRO syndrome may be life-threatening. Patients should be monitored for progression to shock and cardiac arrest, especially those with rapidly increasing ascites.

Dexmedetomidine sedation for pediatric post-Fontan procedure patients.

OBJECTIVE: The hemodynamic, respiratory, and sedative effects of dexmedetomidine (DEX) for pediatric patients post-Fontan surgery. DESIGN: Retrospective. SETTING: Single institutional intensive care unit. PARTICIPANTS: Fourteen patients undergoing Fontan-type surgery. RESULT: A retrospective review was conducted on 14 pediatric patients who had undergone a Fontan procedure for congenital heart disease. A vital component of postoperative management of these patients is to prevent an increase in pulmonary vascular resistance (PVR) that may lead to a serious reduction in cardiac output. DEX an alpha-2 adrenergic receptor agonist might offer an advantage over current sedation methods in preventing a rise in PVR. Nine patients received sedation with DEX and five patients in a control group were administered standard regimens of sedation and analgesia. The DEX group exhibited no evidence of an increased partial pressure of arterial carbon dioxide postoperatively as opposed to the control group. This lack of respiratory depression made the DEX group less likely to increase their PVR. However, the DEX group did experience a significant incidence of bradycardia that required the use of a cardiac pacemaker. CONCLUSIONS: The results of this retrospective review of the role of DEX in the management of the post-Fontan surgical pediatric patient indicate some potential advantages.