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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.

Sirs,We read with great interest the article entitled "Remission of resistant MPGN type I with mycophenolate mofetil and steroids" by De et al. [1]. We encountered a similar case and would like to comment on the efficacy of mycophenolate mofetil (MMF) for refractory membranoproliferative glomerulonephritis (MPGN) type I. A 13-year-old boy was transferred to our hospital because of steroid-resistant nephrotic syndrome with hypocomplementemia. He had been treated with 2 mg/kg of prednisolone (PSL) for 4 weeks, but systemic edema, massive proteinuria and hypertension had not improved. When he was admitted to our hospital, the results of urinalysis were: protein 2,498 mg/dl, creatinine 399 mg/dl, β2 microglobulin 5,691 μg/dl (normal <250 μg/dl), and red blood cell (RBC) count over 50 per high-power field (HPF). Those of blood biochemistry were: 4.4 g/dl of total protein, albumin 2.0 g/dl, serum creatinine 0.83 mg/dl, blood urea nitrogen 33 mg/dl, uric acid 7.3 mg/dl, total cholesterol 404 mg/dl, triglycerides 296 mg/dl, complement (C)3 21 mg/dl (86~160 mg/dl), C4 21 mg/dl (17~45 mg/dl), CH50 20 U/ml (30~40 U/ml), anti-nuclear antibody (negative), and anti-double stranded DNA antibody (negative). Renal biopsy showed diffuse and severe MPGN type I. All the glomeruli showed marked lobulation, intracapillary hypercellularity, thickening of the capillary walls and double contour. Immunofluorescence showed intense staining of all capillary walls and mesangial cells with C3 and immunoglobulin (Ig)G. An electron micrograph showed electron-dense deposits only in the subendothelial space. The patient was initially treated with three courses of methyl prednisolone pulse therapy (MPT, one course; 1 g of methyl prednisolone for 3 days) followed by PSL orally. Lisinopril and losartan were used for hypertension and proteinuria. Proteinuria was dramatically reduced after three courses of MPT, but it was still in the nephrotic range (Fig. 1). Additional cyclosporin was also insufficient to induce remission of proteinuria. In the end, he was started on MMF 3 months from the initiation of treatment. Proteinuria decreased promptly soon after he had started on MMF. Two months later the proteinuria had completely resolved, even though PSL had been withdrawn. Under treatment with PSL, cyclosporin A (CsA) and MMF, he has not had proteinuria for 8 months. Hematuria has also Pediatr Nephrol (

Objectives: The reliability of surveys on smoking habits based on questionnaires was investigated, using the urinary cotinine content as an objective index. Methods: The subjects tested were 2,849 office workers of middle age, who responded to questions concerning their smoking status, and also their urinary cotinine was measured by the HPLC method. Results: The boundary value between smokers and non-smokers, determined by the histogram independent of the questionnaire, was 63.1 and 79.4 ng/mg of creatinine for males and females, respectively. The rate of misclassification of the non-smokers and former smokers as smokers was 1.3% for males and 1.8% for females, whereas that of current smokers as non-smokers was 6.3% and 2.1%. We also assessed the effect of smoke inhalation on the urinary cotinine value, and found a significant difference for males in the cotinine value by the presence of inhalation and also its depth. Conclusions: 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.

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