著者

髙橋 健一郎 本間 哲 鈴木 恵子 加藤 文代 杉原 茂孝

出版者

東京女子医科大学学会

雑誌

東京女子医科大学雑誌 (ISSN:00409022)

巻号頁・発行日

vol.87, no.Extra1, pp.E118-E124, 2017-05-31 (Released:2017-07-31)

参考文献数

30

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We present a case of Kawasaki disease (KD) complicated by clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). A 2-year-old boy was brought to the emergency outpatient unit because of recurrent convulsions. He had experienced two episodes of KD at the age of 1 year. Both episodes had been treated with high dose intravenous immunoglobulin therapy (IVIG; 2 g/kg/day) and the patient had recovered from both episodes without any sequelae. He was diagnosed as having KD based on the presence of 5 out of 6 criteria. After hospitalization, the patient exhibited a persistent disturbance of consciousness and was diagnosed as having MERS based on the presence of enhanced signals in the splenium of the corpus callosum on the 3rd day of illness. Initial treatment with IVIG plus pulsed methylprednisolone (30 mg/kg/day×3 days) for MERS was started. However, a high fever recurred on the 7th day. Additional treatment with IVIG plus intravenous prednisolone (2 mg/kg/day) was started on the 8th day. The patient's body temperature normalized on the 9th day and maintenance therapy with prednisolone was continued until the 28th day. Despite some risk factors for coronary artery lesion, the patient was discharged without any sequelae because of twice IVIG and aggressive steroid therapy including steroid pulse therapy.

著者

加藤 文代 布田 伸一

出版者

東京女子医科大学学会

雑誌

東京女子医科大学雑誌 (ISSN:00409022)

巻号頁・発行日

vol.87, no.Extra1, pp.E40-E47, 2017-05-31 (Released:2017-07-31)

参考文献数

31

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Post-transplant lymphoproliferative disorders (PTLD) remain a significant cause of morbidity and mortality after pediatric heart transplantation. More than 80 % of cases are of B-cell origin and are positive for Epstein-Barr virus (EBV). The pathogenesis of EBV-positive PTLD appears to be correlated with the uncontrolled proliferation of latently EBV-infected B cells arising from a lack of EBV-specific cytotoxic T-lymphocyte function. PTLD includes a spectrum of diseases ranging from reactive lymphoid proliferation to malignant lymphoma. The risk factors underlying the development of PTLD include the degree of immunosuppression, the EBV serostatus of the recipient, the time since transplantation, and the recipient's age and ethnicity. In the pediatric age group, most PTLD cases occur in EBV-negative recipients of EBV-positive donor organs. As part of routine evaluations of patients with a high risk of PTLD, EBV monitoring is useful for the early detection of this complication. A high index of suspicion is key to an early and accurate diagnosis of PTLD. The subsequent therapeutic goals are the eradication of PTLD and the preservation of graft function. The treatment outcomes for PTLD have steadily improved over the past decade, partly because of the availability of rituximab and everolimus.