- 著者
- Akio Kawabe Shizuyo Tsujimura Kazuyoshi Saito Yoshiya Tanaka
- 出版者
- 一般社団法人 日本内科学会
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- vol.56, no.12, pp.1575-1580, 2017-06-15 (Released:2017-06-15)
- 参考文献数
- 15
- 被引用文献数
- 2
True renal lupus vasculitis (TRLV), a vascular lesion usually associated with proliferative lupus nephritis (LN), is resistant to conventional treatments. The expression of P-glycoprotein (P-gp) on activated lymphocytes causes drug resistance. We herein report a patient with TRLV, minimal change LN, overexpression of P-gp on peripheral B cells, and accumulation of P-gp+ B cells at the site of TRLV. High-dose corticosteroids combined with intravenous cyclophosphamide pulse therapy resulted in clinical remission and the long-term normal renal function.
- 著者
- Daijiro Suzuki Takanori Suzuki Masayuki Fujino Yumiko Asai Arisa Kojima Hidetoshi Uchida Kazuyoshi Saito Hirofumi Kusuki Yuanying Li Hiroshi Yatsuya Tsuneaki Sadanaga Tadayoshi Hata Tetsushi Yoshikawa
- 出版者
- Fujita Medical Society
- 雑誌
- Fujita Medical Journal (ISSN:21897247)
- 巻号頁・発行日
- pp.2023-001, (Released:2023-08-28)
- 参考文献数
- 26
Objectives: The Gunma score is used to predict the severity of Kawasaki disease (KD), including coronary artery aneurysm (CAA) as a cardiac complication, in Japan. Additionally, the characteristic ratio of ventricular repolarization (T-peak to T-end interval to QT interval [Tp-e/QT]) on a surface electrocardiogram reflects myocardial inflammation. This study aimed to determine whether the Tp-e/QT can be used to predict CAA in children with KD.Methods: We analyzed chest surface electrocardiograms of 112 children with KD before receiving intravenous immunoglobulin therapy using available software (QTD; Fukuda Denshi, Tokyo, Japan).Results: The Tp-e/QT (lead V5) was positively correlated with the Gunma score (r=0.352, p<0.001). The Tp-e/QT was larger in patients with CAA (residual CAA at 1 month after onset) than in those without CAA (0.314±0.026 versus 0.253±0.044, p=0.003). A receiver operating characteristic curve analysis was performed to assess whether the Gunma score and Tp-e/QT could predict subsequent CAA. The area under the curve of the Gunma score was 0.719 with the cutoff set at 5 points. The area under the curve of the Tp-e/QT was 0.892 with a cutoff value of 0.299. The fit of the prediction models to the observed probability was tested by the Hosmer–Lemeshow test with calibration plots using Locally weighted scatterplot smoothing (LOESS) fit. The Gunma score (p=0.95) and Tp-e/QT (p=0.95) showed a good fit.Conclusions: The Tp-e/QT is a useful biomarker in predicting coronary aneurysm complications in KD.
- 著者
- Keiichi Hirono Yukiko Hata Makoto Nakazawa Nobuo Momoi Tohru Tsuji Taro Matsuoka Mamoru Ayusawa Yuriko Abe Tamaki Hayashi Nobuyuki Tsujii Tadaaki Abe Heima Sakaguchi Ce Wang Asami Takasaki Shinya Takarada Mako Okabe Nariaki Miyao Hideyuki Nakaoka Keijiro Ibuki Kazuyoshi Saito Sayaka Ozawa Naoki Nishida Neil E. Bowles Fukiko Ichida
- 出版者
- The Japanese Circulation Society
- 雑誌
- Circulation Journal (ISSN:13469843)
- 巻号頁・発行日
- pp.CJ-18-0470, (Released:2018-08-18)
- 参考文献数
- 37
- 被引用文献数
- 6
Background: Left ventricular non-compaction (LVNC) is a cardiomyopathy morphologically characterized by 2-layered myocardium and numerous prominent trabeculations, and is often associated with dilated cardiomyopathy (DCM). Variants in the gene encoding tafazzin (TAZ) may change mitochondrial function and cause dysfunction of many organs, but they also contribute to the DCM phenotype in LVNC, and the clinical and echocardiographic features of children with this phenotype are poorly understood. Methods and Results: We enrolled 92 DCM phenotype LVNC patients and performed next-generation sequencing to identify the genetic etiology. Ten TAZ variants were identified in 15 male patients (16.3%) of the 92 patients, including 3 novel missense substitutions. The patients with TAZ variants had a higher frequency of early onset of disease (92.3% vs. 62.3%, P=0.0182), positive family history (73.3% vs. 20.8%, P=0.0001), and higher LV posterior wall thickness Z-score (8.55±2.60 vs. 5.81±2.56, P=0.0103) than those without TAZ variants, although the mortality of both groups was similar. Conclusions: This study provides new insight into the impact of DCM phenotype LVNC and emphasizes the clinical advantages available for LVNC patients with TAZ variants.