Objective: To investigate the effect on early mobilization in patients undergoing extra-corporeal membrane oxygenation (ECMO) and acute blood purification therapy in the intensive care unit (ICU). Methods: We conducted this multicenter retrospective cohort study by collecting data from six ICUs in Japan. Consecutive patients who were admitted to the ICU, aged ≥18 years, and received mechanical ventilation for >48 hours were eligible. The analyzed were divided into two groups: ECMO/blood purification or control group. Clinical outcomes; time to first mobilization, number of total ICU rehabilitations, mean and highest ICU mobility scale (IMS); and daily barrier changes were also investigated. Results: A total of 204 patients were included in the analysis, 43 in the ECMO/blood purification group and 161 in the control group. In comparison of clinical outcome, the ECMO/blood purification group had a significantly longer time to first mobilization: ECMO/blood purification group 6 vs. control group 4 (p=0.003), higher number of total ICU rehabilitations: 6 vs. 5 (p=0.042), lower mean: 0 vs. 1 (p=0.043) and highest IMS: 2 vs. 3 (p=0.039) during ICU stay. Circulatory factor were most frequently described as barriers to early mobilization on days 1 (51%), 2 (47%), and 3 (26%). On days 4 to 7, the most frequently described barrier was consciousness factors (21%, 16%, 19%, and 21%, respectively) Conclusion The results of this study comparing the ECMO/blood purification group and the untreated group in the ICU showed that the ECMO/blood purification group had significantly longer days to mobilization and significantly lower mean and highest IMS.

Autoimmune enteropathy (AIE) is a rare disease, characterized by intractable diarrhea, villous atrophy of the small intestine, and the presence of circulating anti-enterocyte autoantibodies. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, and mutations in FOXP3, which is a master gene of regulatory T cells (Tregs), are major causes of AIE. Recent studies have demonstrated that mutations in other Treg-associated genes, such as CD25 and CTLA4, show an IPEX-like phenotype. We present the case of a 13-year-old girl with CTLA4 haploinsufficiency, suffering from recurrent immune thrombocytopenic purpura and intractable diarrhea. We detected an autoantibody to the AIE-related 75 kDa antigen (AIE-75), a hallmark of the IPEX syndrome, in her serum. She responded well to a medium dose of prednisolone and a controlled dose of 6-mercaptopurine (6-MP), even after the cessation of prednisolone administration. Serum levels of the soluble interleukin-2 receptor and immunoglobulin G (IgG) were useful in monitoring disease activity during 6-MP therapy. In conclusion, autoimmune-mediated mechanisms, similar to the IPEX syndrome, may be involved in the development of enteropathy in CTLA4 haploinsufficiency. Treatment with 6-MP and monitoring of disease activity using serum levels of soluble interleukin-2 receptor and IgG is suggested for such cases.

Objective: This study aimed to reveal the prevalence and characteristics of anguish among delirious patients with advanced cancer receiving specialized palliative care services. Methods: We conducted a subanalysis of a multicenter, prospective, observational study at 14 inpatient palliative care units and 10 general wards that offered psycho-oncology consultation service in Japan. We consecutively enrolled the patients with advanced cancer who were diagnosed with delirium and prescribed antipsychotics. Palliative care specialists decided whether patients suffered from anguish or not. We assessed patients’ background and severity of delirium with the Delirium Rating Scale-Revised (DRS-R-98). Results: Of 818 enrolled patients, 99 (12.1%) suffered from anguish. We observed a significant difference in the mean age (68.9±12.6 vs. 72.1±11.2, p=0.009), prevalence of dementia (2% vs. 10.4%, p=0.005) between patients with anguish and those without anguish. Patients with anguish had lower DRS-R-98 total scores before medication than those without anguish (15.3±8.1 vs. 17.3±7.8, p=0.018), but higher severity score in lability of affect (1.2±0.8 vs 1.0±0.9, p=0.023). Conclusions: The results of this study suggested that patients with anguish tend to be younger, mostly do not have dementia, and have lower delirium severity score but higher score in lability of affect. Nevertheless, further research, investigating appropriate evaluations and medical interventions for patients with anguish is warranted.