著者

Kenichi Watanabe Hiroshi Suzuki Meizi Jiang Shinya Tsukano Satoshi Kataoka Sueshi Ito Takatsugu Sakai Toru Hirokawa Hisanori Haniu Fujito Numano Satoshi Hoshina Satoshi Hasegawa Masamichi Matsunaga Kousei Chiba Naka Saito Hiroshi Yoshida Satoru Takami Soichiro Okubo Harunobu Hirano Akihiko Saitoh Hideaki Bujo

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-20-1271, (Released:2021-09-16)

参考文献数

23

被引用文献数

1

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Background:Intimal smooth muscle cells (SMCs) play an important role in the vasculitis caused by Kawasaki disease (KD). Lipoprotein receptor 11 (LR11) is a member of the low-density lipoprotein receptor family, which is expressed markedly in intimal vascular SMCs and secreted in a soluble form (sLR11). sLR11 has been recently identified as a potential vascular lesion biomarker. sLR11 is reportedly elevated in patients with coronary artery lesions long after KD, but there is no description of sLR11 in acute KD. Our aim was to determine the sLR11 dynamics in acute KD and to assess its usefulness as a biomarker.Methods and Results:106 acute KD patients and 18 age-matched afebrile controls were enrolled. KD patients were classified into the following subgroups: intravenous immunoglobulin (IVIG) responders (n=85) and non-responders (n=21). Serum sLR11 levels before IVIG therapy were higher in non-responders (median, 19.6 ng/mL; interquartile range [IQR], 13.0–24.9 ng/mL) than in controls (11.9 ng/mL, 10.4–14.9 ng/mL, P<0.01) or responders (14.3 ng/mL, 11.7–16.5 ng/mL, P<0.01). Using a cutoff of >17.5 ng/mL, non-responders to initial IVIG therapy were identified with 66.7% sensitivity and 78.8% specificity.Conclusions:sLR11 can reflect the state of acute KD and might be a biomarker for patient response to IVIG therapy.

著者

Rohi Shima Kentaro Sawano Nao Shibata Hiromi Nyuzuki Sunao Sasaki Hidetoshi Sato Yohei Ogawa Yuki Abe Keisuke Nagasaki Akihiko Saitoh

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.29, no.3, pp.105-110, 2020 (Released:2020-07-11)

参考文献数

13

被引用文献数

2

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Newborn screening (NBS) can detect 21-hydroxylase deficiency (21-OHD), allowing for early treatment initiation. However, many patients present with adrenal crises or hyponatremia at their first visit. Age (in days) of hyponatremia development in infants with salt-wasting (SW)-type 21-OHD remains unclear. Therefore, we determined the earliest age of hyponatremia diagnosis in this retrospective observational study using medical records of 40 patients with classic 21-OHD in Niigata Prefecture, Japan, from April 1989 to March 2019. We determined the earliest diagnosis of hyponatremia (serum sodium levels < 130 mEq/L) and created a sodium decrease rate model to estimate hyponatremia development age. Of 23 patients with SW-type 21-OHD, 10 (43.5%) were identified during NBS; the earliest case to present with hyponatremia was at day 7. Serum sodium levels were significantly and negatively correlated with age in days, and hyponatremia was estimated to develop at 6.6 d after birth. Genotype or serum 17-hydroxyprogesterone levels were not associated with sodium decrease rate. Thus, hyponatremia development age is earlier (within 7 d) than the previously described time-point (10–14 d) in infants with SW-type 21-OHD. Efforts to reduce the time lag from obtaining results to consultation may be required in patients with high 17-hydroxyprogesterone levels on NBS.