Efficacy, pharmacokinetics, and safety of adalimumab in pediatric patients with juvenile idiopathic arthritis in Japan.

The objective of this study was to evaluate the efficacy, pharmacokinetics, and safety of adalimumab in patients with polyarticular juvenile idiopathic arthritis (JIA) in Japan. Patients aged 4 to 17 years were enrolled in a single-arm, open-label, multicentre study of adalimumab. Patients weighing <30 kg received 20 mg every other week (eow), and those ≥30 kg received 40 mg eow. Concomitant methotrexate (MTX) was allowed (≤10 mg/m(2) per week). The primary efficacy outcome was the percent of patients with American College of Rheumatology Pediatric 30 response (ACR Pedi 30) at week 16. JIA core variables, serum adalimumab concentrations, and anti-adalimumab antibodies (AAAs) were analysed. Patients were monitored for adverse events (AEs). Twenty-five patients (20 with concomitant MTX at baseline and 5 without) were enrolled: 24 patients completed 16 weeks of therapy and 22 patients completed 60 weeks. At week 16, 90 % of patients with MTX and 100 % without MTX achieved ACR Pedi 30; response rates were maintained through week 60 in 94 and 80 % of patients, respectively. Each JIA core variable improved over time. Six patients became AAA positive (two each at weeks 8, 16, and 60), some of which were transient. All six AAA-positive patients achieved ACR Pedi 30 at week 16, and four maintained that response at week 60. Six patients (all with MTX) experienced nine serious AEs (JIA, pyrexia, arthralgia, pneumonia, hepatitis B infection, pharyngitis, dehydration, pharyngeal pain, and pneumonia). In pediatric patients with polyarticular JIA in Japan, adalimumab was safe and effective for reducing disease activity for up to 60 weeks.

Residential proximity to high-voltage power lines and risk of childhood hematological malignancies.

BACKGROUND: Epidemiologic studies of electromagnetic fields and childhood cancers have focused on home exposure. The authors investigated whether residence in districts near high-voltage power lines is associated with childhood hematological malignancies, using small area analysis. METHODS: Among 50,000 children in a city in Japan, 14 cases aged younger than 15 years were diagnosed with these malignancies in the period from 1992 through 2001. A total of 294 districts constituting this city were classified according to their proximity to high-voltage power lines (either 66 kV or 220 kV). Mantel-Haenszel rate ratio is used to calculate incidence rate ratio and its 95% confidence interval (CI). RESULTS: Compared to districts of which no area fell within 300 m of high-voltage power lines, districts in which at least 50% of the area fell within 300 m of high-voltage power lines demonstrated an increased risk (incidence rate ratio: 2.2; 95% CI: 0.5-9.0). The association was strengthened for homes in which patients had resided for the longest interval of their lives (incidence rate ratio: 3.4; 95% CI: 0.9-13.2). Point-in-time measurements showed no increase in magnetic field levels for patient homes in districts near the lines. CONCLUSION: An increased, albeit nonsignificant, risk of childhood hematological malignancies associated with residential proximity to high-voltage power lines warrants further investigations.

Successful unrelated cord blood transplantation for Epstein-Barr virus-associated lymphoproliferative disease with hemophagocytic syndrome.

We report a case of successful umbilical cord blood transplantation (CBT) for Epstein-Barr virus (EBV)-associated lymphoproliferative disease (LPD) in a 6-year-old girl. The patient had hemophagocytic syndrome with excessive circulating levels of EBV DNA that was refractory to immunochemotherapy. Multiple hepatosplenic lesions favored the diagnosis of EBV-associated LPD, although the aggressive course precluded the histopathologic diagnosis. Unrelated CB cells mismatched at 1 HLA locus were infused after patient conditioning with 900 mg/m2 etoposide, 2 g/m2 cytarabine, 16 mg/kg busulfan, and 200 mg/kg cyclophosphamide. Complete chimeric status was obtained on day 19 posttransplantation. Drug fever and acute graft-versus-host disease of the skin (grade II) were the major complications. A transient increase of EBV DNA 1 year after CBT indicated a primary EBV infection of the donor cells. The patient is alive with no evidence of disease 27 months after CBT. There has been no previous report of successful CBT for EBV-related LPD/lymphoma. CBT can be a curative treatment for the disease, even if no viral memory has been set in the stem cell source.