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.

We report 2 cases of successful treatment by percutaneous catheter drainage and irrigation for methycillin-resistant Staphylococcus aureus (MRSA) prosthetic graft infection after abdominal aortic aneurysm (AAA) repair. Case 1 was a 71-year-old man in whom MRSA graft infection was diagnosed on the basis of high fever and CT-guided taps of the perigraft fluid 11 days after AAA repair, and a percutaneous catheter was inserted into the perigraft space by the CT-guided method. Case 2 was a 77-year-old man in whom MRSA graft infection was diagnosed because of high fever and purulent discharge from the wound of retroperitoneal drainage 5 days after AAA repair. A percutaneous catheter was placed into the retroperitoneal space via an extraperitoneal route. In both cases, intermittent irrigation by 0.5% Povidone-iodine solution and saline was performed as well as systemic and local antibiotic administration. The graft infection was well controlled and both patients were discharged after 4 months. Percutaneous catheter drainage and irrigation can be one of the choices for critically ill patients with graft infection after AAA repair.