Tumor necrosis factor receptor-associated factor 3 (TRAF3) is an anti-inflammatory molecule that negatively regulates the non-canonical nuclear factor-κB pathway. Although TRAF3 haploinsufficiency (TRAF3 HI) can influence innate and adaptive immune cells, its effect on inflammatory bowel disease (IBD) development remains unclear. Here, we report the first case of severe early-onset IBD with a novel TRAF3 variant leading to HI, successfully treated with ustekinumab. A 6-year-old girl with a recurrent parotitis, otitis media, tonsilitis, and atopic dermatitis developed IBD involving the stomach, small intestine, and colon. At diagnosis, the immunoglobulin (Ig)G and IgA levels were relatively high, and lymphocyte subsets showed increased counts of plasmablasts, class-switch recombination B cells, and circulating T-follicular helper cells. Treatment with azathioprine and infliximab failed to maintain remission marked by several relapses accompanied by erythema nodosum and arthritis; however, ustekinumab, an anti-interleukin (IL)-12/23p40 antibody, led to long-term clinical remission, normalizing the Ig level and reducing abnormal lymphocyte counts. Whole-exome sequencing revealed a novel heterozygous mutation in TRAF3 [p.(Pro487Leufs*8)], resulting in TRAF3 under-expression. Our case may highlight the contribution of TRAF3 HI to the development of IBD and provide insights into IBD pathophysiology, suggesting the involvement of the IL-12/23-T-follicular helper cell pathway affected by genetic mutations.

Background : The surgical repair of acute aortic dissection type A [AAD (A)] by reconstructing the left subclavian artery (LSCA) is sometimes difficult because of the deep surgical field and the occurrence of left recurrent nerve palsy or bleeding. In Japan, since 2014, a commercially available open stent graft (J-graft OPEN STENT) has been used for promoting thrombosis of the false lumen in the descending aorta. This report presents an efficacy evaluation of the surgeon-made in situ Fenestrated Open Stent (FeneOS) for LSCA reconstruction in a patient with AAD (A). Method : We performed surgery with FeneOS using the open stent graft by first deploying it from the entry of the LSCA into the descending aorta and manually making a hole on the LSCA side of the stenting portion ; then, the four-branched J graft was anastomosed between the left common carotid (lt. CCA) and SCA (ZONE 2). At our institution, 47 patients with AAD (A) underwent this surgery with FeneOS from 2014 to 2019 (FeneOS group) and 97 patients with AAD (A) underwent a normal open-stenting procedure from 2008 to 2014 (non-FeneOS group). We analyzed the postoperative results of patients in the FeneOS and non-FeneOS groups. Results : Preoperative characteristics of patients in both groups were similar. Patients in the FeneOS group had an acceptable postoperative course, with no 30-day or in-hospital deaths. The mean operation time, cardiopulmonary bypass time, selective cerebral perfusion time, and open distal anastomosis time were significantly shorter in patients in the FeneOS group (p＜0.01). None of the patients had left recurrent nerve palsy, and postoperative computed tomography or arterial echo showed that the blood flow through the LSCA was intact and revealed no endoleakage. Conclusion : FeneOS is simple, fast, and less invasive for the reconstruction of the LSCA without the risk of left recurrent nerve palsy and can be effective for treating patients with AAD (A).

This case report aimed to evaluate the efficacy of applying VIABAHN endoprosthesis at the dissection re-entry of the right renal artery after thoracic endovascular aortic repair (TEVAR) in a patient with a chronic type B dissected thoracoabdominal aneurysm. A 78-year-old man was given a diagnosis of type B aortic dissection 5 years ago and underwent a successful TEVAR operation. Two years later, he developed complications such as chronic expanding aortic dissections ; thus, he underwent a second endovascular repair. Enhanced computed tomography (CT) scanning at the five-year follow-up after initial endovascular repair showed a 58-mm diameter thoracoabdominal dissected aneurysm. It also showed an apparent entry point dissection arising from the lower thoracic aorta and a re-entry point at the base of the right renal artery. Although the right renal artery was affected by the dissecting false lumen, all other abdominal branches were intact. He was treated with VIABAHN via occlusion of the re-entry of the dissection and reconstruction of the right renal artery. The patient recovered uneventfully and was discharged 10 days after the operation. Postoperative enhanced CT scanning showed that the aortic false lumen was completely thrombosed, and the right renal arterial flow had significantly improved. Although TEVAR is the standard treatment in acute complicated type B dissections, its role in chronic type B dissections remains controversial. Our technique of using VIABAHN for the reconstruction of the right renal artery showed promising results for patients with chronic type B dissections.

The objective of this case report was to evaluate the efficacy of the Plug Attachment Technique (PAT) with the Amplatzer Vascular Plug (AVP) in endovascular aneurysm repair (EVAR) in a case of ruptured abdominal aortic aneurysm (rAAA). An 84-year-old woman was taken by ambulance to our hospital. The enhanced CT scan showed an rAAA of 90 mm (Fitzgerald classification 3). The patient was immediately transferred to the operation room and treated with EVAR followed by the closing of the rupture cite using AVP, the Plug Attachment Technique (PAT). The total operation time was 158 min. The patient recovered uneventfully after the operation and was discharged 30 days after the onset. EVAR has been recognized as an efficient acute therapy in cases of rAAA internationally. However, in comparison with the conventional open surgery, we are often facing the critical complications after EVAR in case of rAAA, continuous bleeding thorough the rupture cite and acute compartment syndrome. Our Plug Attachment Technique (PAT) with the Amplatzer Vascular Plug (AVP) may not cause such complications and lead to improved results for EVAR in case of rAAA.

OBJECTIVE: Interest in the "at-risk mental state" (ARMS) for psychosis has increased because early intervention is expected to delay or prevent the onset of schizophrenia. However, the optimum intervention strategy remains controversial, especially with regard to antipsychotics. Although administration of antipsychotic medications is often associated with adverse effects and raises ethical considerations, recent studies have shown that some novel antipsychotics are safer and more tolerable for young people than conventional antipsychotics. We investigated whether administration of perospirone, a combined serotonin (5-HT)/dopamine antagonist and 5-HT1A receptor agonist, could alleviate prodromal symptoms and be well tolerated by clinical high risk patients. METHODS: The participants were outpatients seeking help. The Structured Interview for Prodromal Symptoms was performed in patients identified as being at clinical high risk. The Scale of Prodromal Symptoms (SOPS) was also completed and changes of subjective experience were assessed with the Subjective Well-being under Neuroleptics, short version. The incidence of akathisia was recorded by using the Barnes Akathisia Scale. Subjects were monitored for 26 weeks after starting medication. RESULTS: SOPS scores improved significantly after 26 weeks of perospirone therapy, while BAS scores did not show deterioration. No serious adverse events occurred during the study. CONCLUSION: This trial suggests that perospirone therapy provides a clinical benefit for clinical high risk subjects without causing serious adverse events. Although further placebo-controlled studies are needed for confirmation, perospirone might be one of optimum treatments for individuals at imminent risk of psychosis.
Antipsychotic Agents ; Early Intervention (Education) ; Humans ; Incidence ; Outpatients ; Prodromal Symptoms ; Psychomotor Agitation ; Psychotic Disorders ; Receptor, Serotonin, 5-HT1A ; Schizophrenia ; Serotonin

Differential diagnosis of a so-called false aneurysm of the left ventricle from the true type after a myocardial infarction is important because the risk of rupture of the false aneurysm is high. Two cases of ventricular aneurysms with false type-like shape underwent surgical repair. Preoperative left ventriculography in Case 1 (male, 77) showed an aneurysm of 40×40×35mm in size with a narrow neck at the postero-inferior wall. The aneurysm of Case 2 (male, 61) was 20×20×10mm in size with a narrow neck at the inferior wall. These ventriculographic findings suggested a false type of aneurysm, but operative findings and pathological examination revealed that these were“true”aneurysms in which wall myocardial cells were observed. Left ventriculography and echocardiography were not sufficient to differentiate false left ventricular aneurysm from true aneurysm, particularly at the posterior and inferior wall.