| 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. |
| 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, 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, 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 |
