西山 慶(にしやま けい) | データ更新日:2023.11.22 |
助教 /
九州大学病院
小児科
1. | Iwaya Y, Sanefuji M, Nishiyama K, Sonoda Y, Hamada N, Suga R, Ochiai M, Shimono M, Kusuhara K, Ohga S; Japan Environment and Children's Study Group., Prenatal metal levels and congenital anomalies of the kidney and urinary tract: The Japan Environment and Children's Study., Science of The Total Environment, 10.1016/j.scitotenv.2023.164356., 2023.09. |
2. | Nishiyama K, Sanefuji M, Kurokawa M, Iwaya Y, Hamada N, Sonoda Y, Ogawa M, Shimono M, Suga R, Kusuhara K, Ohga S; Japan Environment and Children’s Study Group., Maternal Chronic Disease and Congenital Anomalies of the Kidney and Urinary Tract in Offspring: A Japanese Cohort Study., American Journal of Kidney Diseases, 10.1053/j.ajkd.2022.03.003., 80, 5, 619-628, 2022.11. |
3. | Eguchi H, Kakiuchi T, Nishi M, Kojima-Ishii K, Nishiyama K, Koga Y, Matsuo M., Case Report: Juvenile Myelomonocytic Leukemia Underlying Ornithine Transcarbamylase Deficiency Safely Treated Using Hematopoietic Stem Cell Transplantation, FRONTIERS IN PEDIATRICS, 10.3389/fped.2022.898531, 10, 2022.05. |
4. | Noguchi H, Nishiyama K, Kaku K, Okabe Y, Nakamura M., Factors Associated With Height Among Pediatric Kidney Transplant Recipients Aged ≤16 Years: A Retrospective, Single-Center Cohort Study of 60 Transplants., Exp Clin Transplant., 20(1):35-41., 2022.01. |
5. | Miura K, Ando T, Kanda S, Hashimoto T, Kaneko N, Ishizuka K, Hamada R, Hataya H, Hotta K, Gotoh Y, Nishiyama K, Hamasaki Y, Shishido S, Fujita N, Hattori M., Response to steroid and immunosuppressive therapies may predict post-transplant recurrence of steroid-resistant nephrotic syndrome., Pediatr Transplant. , 26:e14103., 2021.01. |
6. | Nishiyama K, Watanabe Y, Ishimura M, Tetsuhara K, Imai T, Kanemasa H, Ueki K, Motomura Y, Kaku N, Sakai Y, Imadome KI, Ohga S, Parvovirus B19-Infected Tubulointerstitial Nephritis in Hereditary Spherocytosis., Open Forum Infect Dis, 21(1):80, 2020.07. |
7. | Imai T, Nishiyama K, Ueki K, Tanaka T, Kaku Y, Hara T, Ohga S, Involvement of activated cytotoxic T lymphocytes and natural killer cells in Henoch-Schönlein purpura nephritis., Clin Transl Immunology, 9(11):e1212., 2020.11. |
8. | Imai T, Shiraishi A, Nishiyama K, Ishimura M, Ohga S, Lipopolysaccharide-induced monocyte death in a novel ZnF7 domain mutation of TNFAIP3., J Allergy Clin Immunol Pract, 8(6):2071-2074., 2020.06. |
9. | Mari Kurokawa, Kei Nishiyama, Yuhki Koga, Katsuhide Eguchi, Takashi Imai, Utako Oba, Akira Shiraishi, Hazumu Nagata, Noriyuki Kaku, Masataka Ishimura, Satoshi Honjo, Shouichi Ohga, Hyperferritinemia and acute kidney injury in pediatric patients receiving allogeneic hematopoietic cell transplantation, PEDIATRIC NEPHROLOGY, 10.1007/s00467-020-04619-y, 2020.06, Background: Acute kidney injury (AKI) often occurs in pediatric patients who received allogeneic hematopoietic cell transplantation (HCT). We evaluated the risk and effect of HCT-related AKI in pediatric patients. Methods: We retrospectively studied the survival and renal outcome of 69 children 100 days and 1-year posttransplant in our institution in 2004-2016. Stage-3 AKI developed in 34 patients (49%) until 100 days posttransplant. Results: The 100-day overall survival (OS) rates of patients with stage-3 AKI were lower than those without it (76.5% vs. 94.3%, P = 0.035). The 1-year OS rates did not differ markedly between 21 post-100-day survivors with stage-3 AKI and 29 without it (80.8% vs. 87.9%, P = 0.444). The causes of 19 deaths included the relapse of underlying disease or graft failure (n = 11), treatment-related events (4), and second HCT-related events (4). Underlying disease of malignancy (crude hazard ratio (HR) 5.7; 95% confidence interval (CI), 2.20 to 14.96), > 1000 ng/mL ferritinemia (crude HR 4.29; 95% CI, 2.11 to 8.71), stem cell source of peripheral (crude HR 2.96; 95% CI, 1.22 to 7.20) or cord blood (crude HR 2.29; 95% CI, 1.03 to 5.06), and myeloablative regimen (crude HR 2.56; 95% CI, 1.24 to 5.26), were identified as risk factors for stage-3 AKI until 100 days posttransplant. Hyperferritinemia alone was significant (adjusted HR 5.52; 95% CI, 2.21 to 13.76) on multivariable analyses. Conclusions: Hyperferritinemia was associated with stage-3 AKI and early mortality posttransplant. Pretransplant iron control may protect the kidney of pediatric HCT survivors.. |
10. | Vlad Tocan, Kazuhiro Okubo, Kanako Higashi, Naoko Toda, Kanako Kojima-Ishii, Kei Nishiyama, masataka ishimura, Hidetoshi Takada, Osamu Sakamoto, Fusako Sasaki, Kazuko Yoshimura, Shinichi Hirose, Shoichi Ohga, Reappraising newborn screening for cobalamin C disorder, Pediatrics and Neonatology, 10.1016/j.pedneo.2017.11.002, 59, 4, 415-417, 2018.08, [URL]. |
11. | Mitsuru Arima, Shouko Tsukamoto, Rumi Akiyama, Kei Nishiyama, Ri ichiro Kohno, Takashi Tachibana, Akira Hayashida, Miwa Murayama, Toshio Hisatomi, Kandai Nozu, Kazumoto Iijima, Shoichi Ohga, Kohei Sonoda, Ocular findings in a case of Pierson syndrome with a novel mutation in laminin ß2 gene, Journal of AAPOS, 10.1016/j.jaapos.2018.03.016, 22, 5, 401-403.e1, 2018.10, [URL], Pierson syndrome, an autosomal recessive disorder caused by a mutation in laminin ß2 (LAMB2) gene, is characterized by congenital nephrotic syndrome and various ocular abnormalities. The ocular findings in Pierson syndrome are not well understood, because the incidence of this syndrome is very rare. We report ocular findings in a 5-month-old boy with Pierson syndrome with a novel mutation in LAMB2. We performed a pupilloplasty for his microcoria. Ophthalmic examinations after surgery revealed that he had cataract, severe retinal degeneration, and high myopia. Optical coherence tomography showed the collapse of retinal layer structures and a marked decrease of choroidal thickness. Immunohistochemistry and electron microscopy examinations revealed abnormal iris differentiation and thinning or defect of basal membranes. These results suggest that the development of the iris, lens, retina, and choroid are affected in this type of mutation.. |
12. | Hiroe Itami, Shigeo Hara, Masanori Matsumoto, Shin Imamura, Rie Kanai, Kei Nishiyama, masataka ishimura, Shoichi Ohga, Makiko Yoshida, Ryojiro Tanaka, Yoshiyuki Ogawa, Yujiro Asada, Yoko Sekita-Hatakeyama, Kinta Hatakeyama, Chiho Ohbayashi, Complement activation associated with ADAMTS13 deficiency may contribute to the characteristic glomerular manifestations in Upshaw-Schulman syndrome, Thrombosis Research, 10.1016/j.thromres.2018.08.020, 170, 148-155, 2018.10, [URL], Introduction: Upshaw-Schulman syndrome (USS) is a congenital form of thrombotic thrombocytopenic purpura (TTP) associated with loss-of-function mutations in the ADAMTS13 gene, possibly leading to aberrant complement activation and vascular injury. However, USS is extremely rare, and there have been no systematic studies correlating histopathological severity with local ADAMTS13 expression and complement activation. Materials and methods: Here, we compared histopathological features, ADAMTS13 immunoreactivity, and immunoreactivity of complement proteins C4d and C5b-9 among renal biopsy tissues from five USS cases, ten acquired TTP cases, and eleven controls. Results: Pathological analysis revealed chronic glomerular sclerotic changes in the majority of USS cases (4 of 5), with minor glomerular pathology in the remaining case. In two of these four severe cases, more than half of the glomerular segmental sclerosis area was localized in the perihilar region. The average number of ADAMTS13-positive cells per glomerulus was significantly lower in USS cases than controls (p |
13. | 西山 慶, 生体腎移植の適応判断に苦慮しているメチルマロン酸血症の1例, 腎移植・血管外科, 29, 1, 57-60, 2019.05, メチルマロン酸血症(MMA)の長期合併症として慢性腎不全が知られ、腎機能の低下により急性発作の頻度が増加する。このため早期の腎代替療法導入が推奨されており、なかでも献腎移植はcell therapyとしても有用である。今回、腎機能が低下しているMMA患児に対しヘテロ保因者である両親からの生体腎移植を検討した。これまで9例の腎単独移植の報告があり、5例が献腎、3例が生体腎、1例は腎種不明であった。腎種によらず短期の腎予後は良好であった。今後はヘテロ保因者であるドナーへの影響、cell therapyとしての有用性、移植腎への原病再発について明らかにしていく必要がある。. |
14. | 西山 慶, 今井 崇史, 高田 英俊, 小児慢性腎疾患における尿中L型脂肪酸結合蛋白測定の意義, 日本小児腎不全学会雑誌, 2016.07, 【研究目的】尿中L型脂肪酸結合蛋白(liver type fatty acid binding protein:L-FABP)の小児慢性腎疾患における測定意義を検討する。【方法】2015年3月から7月までの期間に当科外来受診した小児腎疾患患児の尿L-FABPを測定した。尿L-FABP/Crと年齢、身長、尿β2ミクログロブリン(BMG)/Cr、尿蛋白(TP)/Cr、推定GFR(eGFR)との相関について検討した。高値をとった症例とその臨床的意義を考察した。【結果】対象は51症例であった。相関分析および重回帰分析において尿L-FABP/Crは尿BMG/Crと強い正の相関を認めた。尿L-FABP/Cr高値の症例は11例であり、高度蛋白尿を有する糸球体疾患5例と薬剤性腎障害2例を含んでいた。残りの4例は先天性腎尿路奇形(congenital anomalies of kidney and urinary tract:CAKUT)の症例であり、うち3例はcaudal regression syndrome(CRS)であった。【結論】小児慢性腎疾患において尿L-FABP/Crは尿BMG/Crと強い相関を示した。尿L-FABP/Cr高値の症例のうち3症例はCRSであり、尿LFABP/Crが小児慢性腎疾患においても尿細管・間質病変の程度を反映している可能性がある。. |
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九州大学病院
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