No abstract

Etanercept is a tumor necrosis factor (TNF)-alpha inhibitor that has been applied beneficially for juvenile idiopathic arthritis (JIA). We experienced long-term remission of nephrotic syndrome (NS) in a boy treated with etanercept, which was initially used for concomitant JIA. He developed NS at age 3 years 7 months and had mostly been treated with cyclosporine because of steroid dependency and frequent relapses. Cyclosporine was halted at 10 years 7 months because of nephrotoxicity, and he was subsequently treated with mizoribine. However, he had three relapses in the first year and developed JIA at 11 years 7 months. He was treated with sulfasalazine, methotrexate, and prednisolone, but his arthritis persisted. Etanercept was started at 12 years 3 months. Thereafter, his arthritis went into complete remission. Surprisingly, he has remained relapse-free for both NS and JIA for more than 3 years with etanercept and mizoribine. It is difficult to know whether the NS remission after initiating etanercept was coincidental. However, there are many reports of increased TNF-alpha or soluble TNF-alpha receptor in NS relapse. To date, there are two reports of the efficacy of TNF-alpha inhibitors against NS. It is possible that TNF-alpha inhibitors may have potential as therapeutic agents for NS.

A five-yr-old boy developed chronic liver failure and ESKD because of CHF and juvenile NPHP. He underwent sequential liver and kidney transplantation with a compatible blood type from his father, at five yr, seven months and five yr, 11 months old, respectively. Because the patient was not in ESKD, we initially performed LDLT because of significant portal hypertension. Even after LDLT, his ascites was not ameliorated, and he needed continuous drainage of ascites and daily albumin and gamma globulin infusion. Thereafter, he progressed to ESKD and needed hemodialysis for one month before LDKT. CDC crossmatch for donor B cells in the warm test, FCXM for B cell IgG, and flow PRA for donor class II were positive before LDKT. After pretreatment of three courses of plasma exchange and intravenous gamma globulin, LDKT was performed. Two weeks after LDKT, AIHA concomitant with autoimmune thrombocytopenia, also called Evans syndrome, occurred because of passenger lymphocytes from the donor; however, the patient was successfully treated with intravenous methylprednisolone. Eighteen months have passed since LDKT, and liver and kidney function in both the recipient and donor are normal.

Childhood TMA due to APS has rarely been reported. To the best of our knowledge this is the first report of pediatric TMA due to APS with positive aPS/PT. Physicians need to be aware of aPS/PT in pediatric APS and/or SLE.

The rate of misclassification in this study was considered to be comparatively low. Several studies have also assessed the reliability of the questionnaire on smoking habits, and found different misclassification rates, indicating the dependence on the race and number of subjects tested. To our knowledge, there were only a few surveys on smoking among large groups, particularly in Japan, such as this one, therefore the results obtained in this study are meaningful.