Background: Therapeutic outcomes for childhood malignancy have dramatically improved. However, secondary malignancies are a major concern, as they greatly affect the quality of life of survivors. This retrospective study evaluated the cumulative incidence, clinical features, and outcomes of secondary malignancies at Nippon Medical School Hospital. Methods: We examined data from 275 cases of primary childhood malignancy diagnosed between 1980 and 2014. Information regarding treatment of the primary malignancy, including irradiation dose, site, and cumulative dose of anticancer drugs, was assessed. We also collected data on secondary malignancy, including patient sex, age at diagnosis, malignancy site, time from primary to secondary malignancy, and outcomes. Results: Secondary malignancies developed in 11 patients and included acute myeloid leukemia (AML) (4), meningioma (4), Ewing sarcoma (1), germ cell tumor (1), and malignant parotid gland tumor (1). The primary malignancies included acute lymphoblastic leukemia (ALL) (9), non-Hodgkin lymphoma (1) and brain tumor (1). In 7 of the 9 ALL patients, chemoradiotherapy was the primary treatment. The meningiomas and 1 solid tumor developed within the radiation field. All AMLs and meningiomas developed within 5 years and after 20 years, respectively, of the primary diagnosis. The 10-and 20-year cumulative incidence rates for secondary malignancy in our hospital were 1.9% and 5.8%, respectively. Conclusions: Our results revealed that the type of secondary malignancy depends on the interval after the end of treatment for primary malignancy. Meningioma, notably, develops many years after completion of primary malignancy treatment. Early detection during long-term follow-up is therefore essential.

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IgA vasculitis (IgAV) is the most frequent form of vasculitis in childhood which classically presents with purpura of the lower extremities, joint pain or swelling and abdominal pain. Though it is a self-limiting disease, and its prognosis is generally good, glomerulonephritis is one of the most important complications. IgAV is classified as a small vessel vasculitis, and though glomerulonephritis develops in IgAV, necrotizing arteritis is rarely seen. Here, we present a case of a 13-year-old girl with IgAV, glomerulonephritis, and necrotizing arteritis in the small renal arteries. There have been only a few reports of adult cases of IgAV with necrotizing arteritis in the kidneys, but there have been no pediatric cases. Some previous reports showed a high mortality rate and implied the possibility of overlap with other vasculitides. In the current report, a rare case of IgAV is described which exhibited necrotizing arteritis rather than overlap with another vasculitis, with a relatively typical clinical course for IgAV and laboratory tests.

Objectives Although epidemiological surveys of paediatric rheumatic diseases in Japan have been conducted they were single surveys with no continuity. This is the first report of the Pediatric Rheumatology Association of Japan (PRAJ) registry database, which was established to continuously collect data for paediatric rheumatic diseases. Methods ‘PRICUREv2’ (Pediatric Rheumatology International Collaborate Unit Registry version 2) is a registry database established by the PRAJ. The registry data were analysed for the age of onset, time to diagnosis, sex differences, seasonality, and other factors. Results Our data showed the same trend regarding rates of paediatric rheumatic diseases reported in Japan and other countries. The age of onset was lower in juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM) and higher in systemic lupus erythematosus (SLE) and Sjögren’s syndrome (SS). The time to diagnosis was relatively short in JIA and SLE but longer in JDM and SS. Rheumatoid factor-positive polyarticular JIA showed a seasonality cluster with regard to onset. Conclusion PRICUREv2 aided the retrieval and evaluation of current epidemiological information on patients with paediatric rheumatic diseases. It is expected that the data collection will be continued and will be useful for expanding research in Japan.

Nephrotic syndrome is known to cause hypercoagulation due to various factors. Steroid therapy is considered the main treatment for nephrotic syndrome. However, this can increase the risk of developing venous thromboembolism, prompting caution in its use. Here, we report a case of venous thromboembolism in a 13-year-old girl with acute renal failure. This developed during catheter insertion into the right internal jugular vein for hemodialysis. We initiated direct oral anticoagulant therapy. Ultrasound was used to monitor the jugular vein.Throughout the course of treatment, the venous thrombosis gradually disappeared. This study highlights the importance of considering prophylactic anticoagulant therapy in patients with nephrotic syndrome who have an increased risk of venous thromboembolism during central venous catheter placement.