2025/07/16 更新

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写真a

エグチ カツヒデ
江口 克秀
EGUCHI KATSUHIDE
所属
九州大学病院 小児科 助教
九州大学病院 小児科(併任)
医学部 医学科(併任)
職名
助教
連絡先
メールアドレス
電話番号
0926425421
プロフィール
先天性免疫異常症および造血不全症の患者の診療および造血細胞移植に従事している。
外部リンク

学位

  • 医学博士 ( 2025年3月 九州大学 )

  • 学士

論文

  • Adjunctive effects of eltrombopag on immunosuppressive therapy for childhood aplastic anemia 査読

    Eguchi, K; Ishimura, M; Ohga, S; Endo, S; Saito, S; Kamimura, S; Keino, D; Kato, S; Azuma, Y; Watanabe, A; Inoue, A; Higa, T; Ozono, S; Fujita, N; Watanabe, K; Takahashi, Y; Japan Childhood Aplastic Anemia Study Grp

    INTERNATIONAL JOURNAL OF HEMATOLOGY   121 ( 4 )   533 - 542   2025年4月   ISSN:0925-5710 eISSN:1865-3774

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    担当区分:筆頭著者   記述言語:英語   出版者・発行元:International Journal of Hematology  

    Eltrombopag is used with first-line immunosuppressive therapy for adult aplastic anemia, although its practical utility in childhood remains unclear. We retrospectively analyzed the outcomes of pediatric patients who received eltrombopag in Japan. Of the 27 eligible patients, 23 (85%) were previously treated, and 15 (56%) had severe or very-severe disease. Seventeen (63%) received eltrombopag with or after rabbit anti-thymocyte globulin plus cyclosporin-A. Within the first year of eltrombopag therapy, 12 patients showed a good or partial response, 15 showed no response, and 8 non-responders successfully underwent hematopoietic cell transplantation. Within the first 3 months after eltrombopag therapy, all but one of the transfusion-dependent responders became transfusion-independent. At 12 months, 6 of 12 responders were disease-free off-treatment. The one-year overall response rate was higher for severe or very-severe than non-severe cases (p = 0.006). Multivariable analysis showed that very-severe disease at the start of eltrombopag therapy was a predictor of being disease-free off-treatment (p = 0.03). No cytogenetic abnormalities developed, but myelofibrosis occurred 4 months after eltrombopag therapy in one non-responder with very-severe disease. The first 3 months’ response to adjunctive eltrombopag may guide to the safe and effective use for the cure of disease, although prospective trials are needed to determine its long-term effects.

    DOI: 10.1007/s12185-024-03903-z

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  • Successful Allogeneic Hematopoietic Cell Transplantation for Patients with IL10RA Deficiency in Japan

    Tomomasa, D; Suzuki, T; Takeuchi, I; Goto, K; Hagiwara, SI; Keino, D; Saida, S; Ishige, T; Kudo, T; Eguchi, K; Ishimura, M; Matsuda, Y; Wada, T; Ito, Y; Kato, M; Sasahara, Y; Morio, T; Arai, K; Uhlig, HH; Kanegane, H

    JOURNAL OF CLINICAL IMMUNOLOGY   45 ( 1 )   6   2025年12月   ISSN:0271-9142 eISSN:1573-2592

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    記述言語:英語   出版者・発行元:Journal of Clinical Immunology  

    Background: IL10RA (IL10 receptor subunit alpha) deficiency is an autosomal recessive disease that causes inflammatory bowel disease during early infancy. Its clinical course is often fatal and the only curative treatment is allogeneic hematopoietic cell transplantation (HCT). In Japan, only case reports are available, and there are no comprehensive reports of treatment outcomes. Methods: We retrospectively analyzed patients with IL10RA deficiency in Japan. Results: Two newly identified and five previously reported patients were included in this study. Five patients underwent HCT; one untransplanted patient survived to age 14, and one died of influenza encephalopathy before transplantation. All five HCT recipients underwent HCT at the age before 2 years. They all were conditioned with fludarabine/busulfan- or fludarabine /melphalan-based regimens. The donor source was human leukocyte antigen haploidentical donor bone marrow (BM) for two patients and unrelated umbilical cord blood (CB) for two patients. One patient experienced graft failure with unrelated CB and required a second transplant with unrelated BM. All patients who underwent HCT survived and demonstrated an improved performance status. Conclusion: In cases of IL10RA deficiency, the need for transplantation should be promptly assessed, and early transplantation should be considered. (190/250).

    DOI: 10.1007/s10875-024-01795-6

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  • Onset and Outcome of Ocular Lesions in Neonatal Severe Protein C Deficiency: Case Report and Literature Review

    Miyauchi, Y; Egami, N; Inoue, H; Sonoda, M; Eguchi, K; Ishimura, M; Ochiai, M; Tsukamoto, S; Matsumoto, S; Uchiumi, T; Sonoda, KH; Ohga, S

    PEDIATRIC BLOOD & CANCER   72 ( 3 )   e31518   2025年3月   ISSN:1545-5009 eISSN:1545-5017

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    記述言語:英語   出版者・発行元:Pediatric Blood and Cancer  

    Neonatal protein C (PC) deficiency, which presents with purpura fulminans or hemorrhagic infarction, has a poor prognosis. In a sporadic case of prenatal-onset purpura fulminans, preemptive intervention saved the patient but not her vision. Among 38 survivors from 47 cases with ocular lesions and biallelic PROC variants, 23 had total blindness and eight had unilateral blindness. Only one had intact vision. Prenatal-onset ocular lesions occurred in 11 cases (29% [corneal opacities, n = 7; bilateral persistent hyperplastic primary vitreous, n = 1; both, n = 3]). The eyes are the first organ affected by heritable severe PC deficiency. Prenatal intervention is needed to improve visual outcomes.

    DOI: 10.1002/pbc.31518

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  • Monocyte STAT1 phosphorylation and treatment response of JAK inhibitors in chronic nonbacterial osteomyelitis

    Sonoda, M; Kinoshita, K; Harada, N; Park, S; Adachi, S; Yada, Y; Eguchi, K; Fujiwara, T; Kido-Nakahara, M; Kinjo, N; Ishimura, M; Ohga, S

    PEDIATRIC RHEUMATOLOGY   23 ( 1 )   6   2025年1月   eISSN:1546-0096

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    記述言語:英語   出版者・発行元:Pediatric Rheumatology  

    Background: Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory disease of unknown cause, predominantly affecting teens and young adults. The early diagnosis and management are challenging due to the lack of reliable diagnostic markers and the occasional intractable cases despite conventional anti-inflammatory treatments. Janus kinase (JAK) inhibitors have recently shown potential utility; however, reports on their use for pediatric patients with CNO remain limited, and no established biomarkers exist to monitor disease activity. We aimed to investigate the pathophysiology of CNO and explore the rapid testing methods for accurate diagnosis and also assessing the disease activity. Methods: We assessed intracellular phosphorylation of signal transducer and activator of transcription 1 (pSTAT1) in peripheral blood monocytes or T cells following interferon-gamma (IFNγ) stimulation, using flow cytometry in 9 patients under 15 years old with CNO. The pSTAT1 expression levels were compared with those in patients with STAT1-gain of function (STAT1-GOF) mutations (n = 5), other autoinflammatory diseases (n = 7), and healthy controls. Clinical and immunological data were monitored in 4 patients with intractable CNO treated with adjunctive JAK inhibitors, focusing on scoring scales, imaging data, lymphocyte subsets, cytokine profiles, and pSTAT1 levels. Results: Monocyte pSTAT1 expression after IFNγ stimulation was elevated at diagnosis or during active CNO, similar to levels observed in STAT1-GOF cases. The pSTAT1 levels in CNO patients were significantly higher than those in other autoinflammatory diseases (p = 0.024) or controls (p < 0.001). Notably, pSTAT1 levels in CNO monocytes fluctuated with disease activity, decreasing in 5 patients during clinical remission following conventional therapies (p = 0.016). In four intractable cases, pSTAT1 levels remained high despite conventional treatments but significantly decreased after initiating JAK inhibitors (p = 0.036). This reduction correlated with improved patient pain visual analog scale (p = 0.008), CNO clinical disease activity score (p = 0.029), and better bone and joint imaging, though cytokine levels remained unchanged. Conclusions: The monocyte pSTAT1 levels after IFNγ stimulation reflect the activity of CNO, indicating the diagnostic utility as well as the monitoring effect of disease control. Adjunctive JAK inhibitors successfully controlled inflammation in treatment-resistant cases. Rapid pSTAT1 testing may help reduce osteo-articular complications, although the long-term adverse effects and resistance should be further investigated.

    DOI: 10.1186/s12969-025-01059-6

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  • Comprehensive flow cytometry-based diagnosis of XIAP deficiency

    Tomomasa, D; Nishimura, M; Ohya, A; Tanita, K; Wakatsuki, R; Watanabe, R; Miyamoto, S; Hoshino, A; Kamiya, T; Isoda, T; Kaneko, S; Shimizu, M; Hijikata, A; Eguchi, K; Ishimura, M; Maeda, Y; Izawa, K; Meguro, T; Fujimoto, K; Ishikita-Murayama, E; Suzuki, K; Okura, E; Uehara, T; Takayama, T; Okada, S; Takagi, M; Morio, T; Marsh, RA; Kanegane, H

    CLINICAL AND EXPERIMENTAL IMMUNOLOGY   219 ( 1 )   2025年   ISSN:0009-9104 eISSN:1365-2249

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    記述言語:英語   出版者・発行元:Clinical and Experimental Immunology  

    Deficiency of X-linked inhibitor of apoptosis protein (XIAP) is an X-linked recessive inborn error of immunity characterized by abnormal immune responses leading to inflammatory bowel disease and hemophagocytic lymphohistiocytosis. Although XIAP protein expression analysis by flow cytometry (XIAP flow) is commonly used to diagnose XIAP deficiency, certain variants may not affect the protein expression, thereby complicating the diagnostic process. XIAP is crucial for the nucleotide-binding and oligomerization domain 2 (NOD2) signaling pathway. In this study, we aimed to perform a comprehensive analysis of nine patients diagnosed with XIAP deficiency through genetic testing. In addition to XIAP flow, we employed a previously reported method utilizing muramyl dipeptide (MDP) stimulation, a specific agonist of NOD2, to quantitatively evaluate the downstream tumor necrosis factor-alpha (TNFα) production by flow cytometry in patient monocytes (MDP flow). The median mean fluorescence intensity in healthy controls with XIAP flow was 711 (95% confidence interval [CI], 653-815) compared to 195 (95% CI, 161-386) in patients with XIAP deficiency (P < 0.0001). The median percentage of TNFα-producing monocytes in controls with MDP flow was 29.1% (95% CI, 19.6-53.7), while in patients it was 0.34% (95% CI, 0.18-0.82) (P = 0.0008). The receiver operating characteristic curves demonstrated that both XIAP flow and MDP flow exhibited 100% sensitivity and specificity. Taken together, combining XIAP flow and MDP flow analyses allows for a highly accurate diagnosis.

    DOI: 10.1093/cei/uxaf020

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  • Epstein-Barr virus monitoring for preemptive re-hematopoietic cell transplantation in CD3δ-deficient siblings

    Park, S; Sonoda, M; Eguchi, K; Adachi, S; Kinoshita, K; Semba, Y; Ishimura, M; Ohga, S

    PEDIATRIC BLOOD & CANCER   71 ( 8 )   e31119   2024年8月   ISSN:1545-5009 eISSN:1545-5017

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    記述言語:英語   出版者・発行元:Pediatric Blood and Cancer  

    DOI: 10.1002/pbc.31119

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  • Highly sensitive detection of Epstein-Barr virus-infected cells by EBER flow FISH

    Tomomasa, D; Tanita, K; Hiruma, Y; Hoshino, A; Kudo, K; Azumi, S; Shiota, M; Yamaoka, M; Eguchi, K; Ishimura, M; Tanaka, Y; Iwatsuki, K; Okuno, K; Hama, A; Sakamoto, KI; Taga, T; Goto, K; Ota, H; Ichiki, A; Kanda, K; Miyamura, T; Endo, S; Ohnishi, H; Sasahara, Y; Arai, A; Fornier, B; Imadome, KI; Morio, T; Latour, S; Kanegane, H

    INTERNATIONAL JOURNAL OF HEMATOLOGY   120 ( 2 )   241 - 251   2024年8月   ISSN:0925-5710 eISSN:1865-3774

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    記述言語:英語   出版者・発行元:International Journal of Hematology  

    When Epstein-Barr virus (EBV) infection is suspected, identification of infected cells is important to understand the pathogenesis, determinine the treatment strategy, and predict the prognosis. We used the PrimeFlow™ RNA Assay Kit with a probe to detect EBV-encoded small RNAs (EBERs) and multiple surface markers, to identify EBV-infected cells by flow cytometry. We analyzed a total of 24 patients [11 with chronic active EBV disease (CAEBV), 3 with hydroa vacciniforme lymphoproliferative disorder, 2 with X-linked lymphoproliferative disease type 1 (XLP1), 2 with EBV-associated hemophagocytic lymphohistiocytosis, and 6 with posttransplant lymphoproliferative disorder (PTLD)]. We compared infected cells using conventional quantitative PCR methods and confirmed that infected cell types were identical in most patients. Patients with CAEBV had widespread infection in T and NK cells, but a small amount of B cells were also infected, and infection in patients with XLP1 and PTLD was not limited to B cells. EBV-associated diseases are believed to be complex pathologies caused by EBV infecting a variety of cells other than B cells. We also demonstrated that infected cells were positive for HLA-DR in patients with CAEBV. EBER flow FISH can identify EBV-infected cells with high sensitivity and is useful for elucidating the pathogenesis.

    DOI: 10.1007/s12185-024-03786-0

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  • Clinical Characteristics of Cryopyrin-Associated Periodic Syndrome and Long-Term Real-World Efficacy and Tolerability of Canakinumab in Japan: Results of a Nationwide Survey

    Miyamoto, T; Izawa, K; Masui, S; Yamazaki, A; Yamasaki, Y; Matsubayashi, T; Shiraki, M; Ohnishi, H; Yasumura, J; Kawabe, T; Miyamae, T; Matsubara, T; Arakawa, N; Ishige, T; Takizawa, T; Shimbo, A; Shimizu, M; Kimura, N; Maeda, Y; Maruyama, Y; Shigemura, T; Furuta, J; Sato, S; Tanaka, H; Izumikawa, M; Yamamura, M; Hasegawa, T; Kaneko, H; Nakagishi, Y; Nakano, N; Iida, Y; Nakamura, T; Wakiguchi, H; Hoshina, T; Kawai, T; Murakami, K; Akizuki, S; Morinobu, A; Ohmura, K; Eguchi, K; Sonoda, M; Ishimura, M; Furuno, K; Kashiwado, M; Mori, M; Kawahata, K; Hayama, K; Shimoyama, K; Sasaki, N; Ito, T; Umebayashi, H; Omori, T; Nakamichi, S; Dohmoto, T; Hasegawa, Y; Kawashima, H; Watanabe, S; Taguchi, Y; Nakaseko, H; Iwata, N; Kohno, H; Ando, T; Ito, Y; Kataoka, Y; Saeki, T; Kaneko, U; Murase, A; Hattori, S; Nozawa, T; Nishimura, K; Nakano, R; Watanabe, M; Yashiro, M; Nakamura, T; Komai, T; Kato, K; Honda, Y; Hiejima, E; Yonezawa, A; Bessho, K; Okada, S; Ohara, O; Takita, J; Yasumi, T; Nishikomori, R

    ARTHRITIS & RHEUMATOLOGY   76 ( 6 )   949 - 962   2024年6月   ISSN:2326-5191 eISSN:2326-5205

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    記述言語:英語   出版者・発行元:Arthritis and Rheumatology  

    Objective: We assess the clinical characteristics of patients with cryopyrin-associated periodic syndrome (CAPS) in Japan and evaluate the real-world efficacy and safety of interleukin-1 (IL-1) inhibitors, primarily canakinumab. Methods: Clinical information was collected retrospectively, and serum concentrations of canakinumab and cytokines were analyzed. Results: A total of 101 patients were included, with 86 and 15 carrying heterozygous germline and somatic mosaic mutations, respectively. We identified 39 mutation types, and the common CAPS-associated symptoms corresponded with those in previous reports. Six patients (5.9% of all patients) died, with four of the deaths caused by CAPS-associated symptoms. Notably, 73.7% of patients (100%, 79.6%, and 44.4% of familial cold autoinflammatory syndrome, Muckle–Wells syndrome, and chronic infantile neurological cutaneous articular syndrome/neonatal onset multisystem inflammatory disease, respectively) achieved complete remission with canakinumab, and early therapeutic intervention was associated with better auditory outcomes. In some patients, canakinumab treatment stabilized the progression of epiphysial overgrowth and improved height gain, visual acuity, and renal function. However, 23.7% of patients did not achieve inflammatory remission with crucial deterioration of organ damage, with two dying while receiving high-dose canakinumab treatment. Serological analysis of canakinumab and cytokine concentrations revealed that the poor response was not related to canakinumab shortage. Four inflammatory nonremitters developed inflammatory bowel disease (IBD)—unclassified during canakinumab treatment. Dual biologic therapy with canakinumab and anti–tumor necrosis factor-α agents was effective for IBD– and CAPS-associated symptoms not resolved by canakinumab monotherapy. Conclusion: This study provides one of the largest epidemiologic data sets for CAPS. Although early initiation of anti–IL-1 treatment with canakinumab is beneficial for improving disease prognosis, some patients do not achieve remission despite a high serum concentration of canakinumab. Moreover, IBD may develop in CAPS after canakinumab treatment. (Figure presented.).

    DOI: 10.1002/art.42808

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  • Early hematopoietic cell transplantation for familial hemophagocytic lymphohistiocytosis in a regional treatment network in Japan

    Ishimura M., Eguchi K., Sonoda M., Tanaka T., Shiraishi A., Sakai Y., Yasumi T., Miyamoto T., Voskoboinik I., Hashimoto K., Matsumoto S., Ozono S., Moritake H., Takada H., Ohga S.

    International Journal of Hematology   119 ( 5 )   592 - 602   2024年5月   ISSN:09255710

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    記述言語:英語   出版者・発行元:International Journal of Hematology  

    Familial hemophagocytic lymphohistiocytosis (FHLH) is a fatal hyperinflammation syndrome arising from the genetic defect of perforin-mediated cytolysis. Curative hematopoietic cell transplantation (HCT) is needed before development of central nervous system (CNS) disease. We studied treatment outcomes of 13 patients (FHLH2 n = 11, FHLH3 n = 2) consecutively diagnosed from 2011 to 2022 by flow cytometric screening for non-myeloablative HCT in a regional treatment network in Kyushu, Japan. One patient with a novel PRF1 variant escaped screening, but all patients with FHLH2 reached diagnosis and 8 of them received HCT until 3 and 9 months of age, respectively. The earliest HCT was conducted 65 days after birth. Three pretransplant deaths occurred in newborns with liver failure at diagnosis. Ten posttransplant patients have remained disease-free, 7 of whom had no neurological involvement. Time from first etoposide infusion to HCT was shorter in patients without CNS disease or bleeding than in patients with those factors (median [range] days: 62 [50–81] vs. 122 [89–209], p = 0.016). Six of 9 unrelated patients had a PRF1 c.1090_1091delCT variant. These results suggest that the critical times to start etoposide and HCT are within 3 months after birth and during etoposide control, respectively. Newborn screening may increase the percentage of disease-free survivors without complications.

    DOI: 10.1007/s12185-024-03721-3

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  • Pretransplant ribavirin and interferon-α therapy for rhinovirus interstitial pneumonia in a RAG1-deficient infant

    Harada, N; Sonoda, M; Ishimura, M; Eguchi, K; Kinoshita, K; Matsuoka, W; Motomura, Y; Kaku, N; Kawaguchi, N; Takeuchi, T; Ohga, S

    JOURNAL OF INFECTION AND CHEMOTHERAPY   30 ( 4 )   362 - 365   2024年4月   ISSN:1341-321X eISSN:1437-7780

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    記述言語:英語   出版者・発行元:Journal of Infection and Chemotherapy  

    Severe combined immunodeficiency (SCID) is one of the most serious inborn errors of immunity leading to a fatal infection in early infancy. Allogeneic hematopoietic cell transplantation (HCT) or elective gene therapy prior to infection or live-attenuated vaccination is the current standard of curative treatment. Even in the era of newborn screening for SCID, pretransplant control of severe infection is challenging for SCID. Multiple pathogens are often isolated from immunocompromised patients, and limited information is available regarding antiviral strategies to facilitate curative HCT. We herein present a case of successfully controlled pretransplant pneumonia after ribavirin and interferon-α therapy in an infant with RAG1-deficiency. A four-month-old infant presented with severe interstitial pneumonia due to a co-infection of rhinovirus and Pneumocystis jirovecii. The tentative diagnosis of SCID prompted to start antibiotics and trimethoprim-sulfamethoxazole on ventilatory support. Because of the progressive respiratory failure four days after treatment, ribavirin and then pegylated interferon-α were started. He showed a drastic response to the treatment that led to a curative HCT 32 days after admission. This patient received the genetic diagnosis of RAG1-deficiency. Currently, he is an active 3-year-old boy with normal growth and development. The review of literature indicated that rhinovirus had a comparable or rather greater impact on the mortality of pediatric patients than respiratory syncytial virus. Considered the turn-around time to the genetic diagnosis of SCID, prompt ribavirin plus interferon-α therapy may help to control severe rhinovirus pneumonia and led to the early curative HCT for the affected infants.

    DOI: 10.1016/j.jiac.2023.11.003

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  • Adenovirus-associated Paroxysmal Cold Hemoglobinuria as Chilly Month Hemolytic Crisis

    Harada, N; Sonoda, M; Ishimura, M; Eguchi, K; Motomura, Y; Fujino, K; Ohga, S

    PEDIATRIC INFECTIOUS DISEASE JOURNAL   43 ( 4 )   e147 - e148   2024年4月   ISSN:0891-3668 eISSN:1532-0987

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    記述言語:英語   出版者・発行元:Pediatric Infectious Disease Journal  

    DOI: 10.1097/INF.0000000000004230

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  • Non-conditioned cord blood transplantation for infection control in athymic CHARGE syndrome

    Sonoda, M; Ishimura, M; Inoue, H; Eguchi, K; Ochiai, M; Sakai, Y; Doi, T; Suzuki, K; Inoue, T; Mizukami, T; Nakamura, K; Takada, H; Ohga, S

    PEDIATRIC BLOOD & CANCER   71 ( 3 )   e30809   2024年3月   ISSN:1545-5009 eISSN:1545-5017

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    記述言語:英語   出版者・発行元:Pediatric Blood and Cancer  

    Objective: CHARGE syndrome is a congenital malformation syndrome caused by heterozygous mutations in the CHD7 gene. Severe combined immunodeficiency (SCID) arises from congenital athymia called CHARGE/complete DiGeorge syndrome. While cultured thymus tissue implantation (CTTI) provides an immunological cure, hematopoietic cell transplantation (HCT) is an alternative option for immuno-reconstitution of affected infants. We aimed to clarify the clinical outcomes of patients with athymic CHARGE syndrome after HCT. Methods: We studied the immunological reconstitution and outcomes of four patients who received non-conditioned unrelated donor cord blood transplantation (CBT) at Kyushu University Hospital from 2007 to 2022. The posttransplant outcomes were compared with the outcomes of eight reported patients. Results: Four index cases received CBT 70–144 days after birth and had no higher than grade II acute graft-versus-host disease. One infant was the first newborn-screened athymic case in Japan. They achieved more than 500/μL naïve T cells with balanced repertoire 1 month post transplant, and survived more than 12 months with home care. Twelve patients including the index cases received HCT at a median 106 days after birth (range: 70–195 days). One-year overall survival rate was significantly higher in patients who underwent non-conditioned HCT than in those who received conditioned HCT (100% vs. 37.5%, p =.02). Nine patients died, and the major cause of death was cardiopulmonary failure. Conclusions: Athymic infants achieved a prompt reconstitution of non-skewing naïve T cells after non-conditioned CBT that led to home care in infancy without significant infections. Non-conditioned CBT is a useful bridging therapy for newborn-screened cases toward an immunological cure by CTTI.

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  • Clinical Features of Pediatric Uveitis at a Tertiary Referral Center in the Western Region of Japan

    Fukuda, Y; Yawata, N; Hasegawa, E; Yamana, S; Shirane, M; Ito, T; Takeda, A; Sonoda, M; Eguchi, K; Ishimura, M; Ohga, S; Sonoda, KH

    OCULAR IMMUNOLOGY AND INFLAMMATION   31 ( 10 )   2032 - 2038   2023年11月   ISSN:0927-3948 eISSN:1744-5078

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    記述言語:英語   出版者・発行元:Ocular Immunology and Inflammation  

    Purpose: This study aimed to assess the clinical features of pediatric uveitis at a tertiary referral center in Western Japan. Methods: One hundred forty eyes of 80 patients aged <20 years at the time of uveitis onset, who visited Kyushu University Hospital between January 2010 and December 2019 were included in this study. Clinical records were retrospectively reviewed. Demographics, clinical findings, treatments, and visual prognoses were compared between the disease groups. Results: Of 80 patients, 32 were males and 48 were females. The average age of onset was 12.5 ± 4.8 (0–19) years. Tubulointerstitial nephritis and uveitis (TINU) and juvenile idiopathic arthritis (JIA) were the most frequent causes, accounting for 11.3% and 10% of cases, respectively, followed by sarcoidosis (5%), Behçet’s disease, acute anterior uveitis, Vogt-Koyanagi-Harada disease, and juvenile chronic iridocyclitis (3.8% each). Infectious uveitis accounted for 7.6% of the cases: cytomegalovirus was the most frequent agent. Of these cases, 43.8% were unclassified. Systemic therapies were administered to 87.5% of the patients with JIA, 33.3% of those with TINU, and 28.6% of the other diagnostic groups. In the unclassified group, 80% of the patients were followed up with only topical corticosteroids. LogMAR visual acuity of 0 or less accounted for more than 80% in the final examination. Conclusion: TINU and JIA were the most common causes of pediatric uveitis. Although each required systemic therapy, most unclassified cases of pediatric uveitis were managed by topical corticosteroids alone with good visual prognosis. Accurate diagnosis is important for pediatric uveitis management.

    DOI: 10.1080/09273948.2023.2273363

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  • Post-transplant <i>Schizophyllum commune</i> abscess in a pediatric patient with chronic granulomatous disease

    Yada, Y; Shiraishi, A; Ishimura, M; Eguchi, K; Motomura, Y; Kibe, Y; Kamei, K; Ohga, S

    JOURNAL OF INFECTION AND CHEMOTHERAPY   29 ( 2 )   219 - 222   2023年2月   ISSN:1341-321X eISSN:1437-7780

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    記述言語:英語   出版者・発行元:Journal of Infection and Chemotherapy  

    Schizophyllum commune is a widely distributed basidiomycete fungus that occasionally causes sinusitis or allergic bronchopulmonary mycosis. The invasive infection mostly occurs in immunocompromised adults. The number of reports on S. commune infection have increased in this decade due to the expansion of diagnostic techniques and awareness in clinical practice. However, S.commune infection in patients with primary immunodeficiencies has not been reported yet. Here, we described S. commune-abscesses developed in the brain and lung of a boy with chronic granulomatous disease (CGD) after allogenic hematopoietic cell transplantation (HCT). A 12-year-old CGD patient developed febrile neutropenia from day 4 after HCT, followed by chest pain on day 23. He had no obvious infection before HCT. Diagnostic imaging revealed disseminated lung and brain abscesses. He received administration of voriconazole, and his symptoms improved after engraftment. Chronic administration of voriconazole had also a favorable therapeutic response to brain lesion. A part of the fungus ball exhaled by the patient was cultured to develop a filamentous fungus. S. commune was identified by the analysis of the 28S rRNA gene. The catalase test was positive for S. commune, indicating that S. commune had virulence in this patient with CGD. The assessment of specific-IgG to S. commune suggested peri-transplant infection, although colonization was not excluded. This rare pediatric case of S. commune infection highlights that CGD patients are vulnerable to invasive infection, especially when undergoing HCT.

    DOI: 10.1016/j.jiac.2022.10.015

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  • High-dose immunoglobulin therapy for steroid-resistant myositis in juvenile localized scleroderma

    Sonoda, M; Ishimura, M; Eguchi, K; Shiraishi, A; Sakai, Y; Urabe, K; Ohga, S

    PEDIATRICS AND NEONATOLOGY   63 ( 5 )   542 - 544   2022年9月   ISSN:1875-9572 eISSN:2212-1692

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    記述言語:英語   出版者・発行元:Pediatrics and Neonatology  

    DOI: 10.1016/j.pedneo.2022.01.006

    Web of Science

    Scopus

    PubMed

  • Changes in bone marrow and peripheral blood lymphocyte subset findings with onset of hepatitis-associated aplastic anemia

    Kakiuchi, T; Eguchi, K; Koga, D; Eguchi, H; Nishi, M; Sonoda, M; Ishimura, M; Matsuo, M

    MEDICINE   101 ( 8 )   e28953   2022年2月   ISSN:0025-7974 eISSN:1536-5964

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    記述言語:英語   出版者・発行元:Medicine United States  

    Rationale:Hepatitis-associated aplastic anemia (HAAA) is a rare illness that results in bone marrow failure following hepatitis development. The etiological agent remains unknown in most HAAA cases. However, clinical features of the disease and immunotherapy response indicate that immune-mediated factors play a central role in the pathogenesis of HAAA. Activation of cytotoxic T cells and increase in CD8 cells could exert cytotoxic effects on the myelopoietic cells in the bone marrow.Patient concerns:A 15-month-old boy was brought to our hospital with complaints of generalized petechiae and purpura observed a week prior to hospitalization. His liver was palpated 3 cm below the costal margin, platelet count was 0 × 10<sup>4</sup>/μL, and alanine aminotransferase level was 1346 IU/L. A blood test indicated cytomegalovirus infection, and 3 bone marrow examinations revealed progressive HAAA. As the disease progressed to the 3<sup>rd</sup>, 6<sup>th</sup>, and 9<sup>th</sup>week after onset, CD4<sup>+</sup>T cells were markedly decreased, CD8<sup>+</sup>T cells were markedly increased, and the CD4/CD8 ratio was significantly decreased. The number of B cells and natural killer cells decreased with time, eventually reaching 0.0%.Diagnosis:HAAA.Interventions:Rabbit antithymocyte globulin and eltrombopag olamine (a thrombopoietin receptor agonist) were administered.Outcomes:The patient's platelet count returned to normal, and bone marrow transplantation was avoided. The peripheral blood lymphocytes (PBLs) improved as the patient's general condition recovered.Lessons:This case demonstrates that HAAA induced by cytomegalovirus infection features decreasing CD4<sup>+</sup>and increasing CD8<sup>+</sup>PBLs as the bone marrow hypoplasia progresses. The PBLs return to their normal levels with the recovery from the disease. Our case findings thus support the involvement of immunological abnormality in HAAA.

    DOI: 10.1097/MD.0000000000028953

    Web of Science

    Scopus

    PubMed

  • Case Report: Rotavirus Vaccination and Severe Combined Immunodeficiency in Japan

    Tanita, K; Kawamura, Y; Miura, H; Mitsuiki, N; Tomoda, T; Inoue, K; Iguchi, A; Yamada, M; Yoshida, T; Muramatsu, H; Tada, N; Matsui, T; Kato, M; Eguchi, K; Ishimura, M; Ohga, S; Imai, K; Morio, T; Yoshikawa, T; Kanegane, H

    FRONTIERS IN IMMUNOLOGY   13   786375   2022年2月   ISSN:1664-3224

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    記述言語:英語   出版者・発行元:Frontiers in Immunology  

    Severe combined immunodeficiency (SCID) is an inborn error of immunity that occurs in approximately 1 in 50,000 births, mainly due to impaired lymphocyte differentiation. Without curative treatment, such as hematopoietic cell transplantation (HCT) or gene therapy, severe infection in the first year of life could make this condition fatal. The results of HCT are poor when patients have active infections, thus requiring early diagnosis before onset of infection. In five cases of SCID diagnosed in Japan, the oral rotavirus vaccine had been administered before diagnosis. In this study, we demonstrated that the rotavirus from their stools was a vaccine-derived strain. In some cases, severe gastroenteritis triggered the diagnosis of SCID. However, newborn screening for SCID is available before the first rotavirus vaccination using assays for the detection of T-cell receptor excision circles (TRECs). Therefore, to improve the prognosis of patients with SCID in Japan, we should establish a screening system of TRECs for newborns throughout Japan.

    DOI: 10.3389/fimmu.2022.786375

    Web of Science

    Scopus

    PubMed

  • Successful treatment of joint and fascial chronic graft-versus-host disease with baricitinib

    Shimizu, M; Shimbo, A; Takagi, M; Eguchi, K; Ishimura, M; Sugita, J; Morio, T; Kanegane, H

    RHEUMATOLOGY   61 ( 1 )   E1 - E3   2022年1月   ISSN:1462-0324 eISSN:1462-0332

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    出版者・発行元:Rheumatology United Kingdom  

    DOI: 10.1093/rheumatology/keab599

    Web of Science

    Scopus

    PubMed

▼全件表示

講演・口頭発表等

  • 先天性溶血性貧血に対する造血細胞移植- Hydroxyurea単剤によるpreconditioningの有用性 -

    足立 俊一、江口 克秀、石村 匡崇、朴 崇娟、木下 恵志郎、矢田 裕太郎、園田 素史、中川 慎一郎、白山 理恵、高畑 靖、菅野 仁、大賀 正一

    第46回 日本造血・免疫細胞療法学会総会  2024年3月 

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    開催年月日: 2024年3月

    記述言語:日本語  

    国名:日本国  

  • Targeted busulfan-based conditioning unrelated bone marrow transplantation for Diamond-Blackfan Anemia

    Katsuhide Eguchi, Masataka Ishimura, Motoshi Sonoda, Shouichi Ohga

    第29回小児再生不良性貧血治療研究会学術集会  2023年5月 

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    開催年月日: 2023年5月

    記述言語:英語  

    国名:日本国  

  • 前処置なしでの臍帯血移植後に、細胞性免疫再構築不全に対して再移植を要したCD3δ欠損症の兄弟例

    朴 崇娟、江口 克秀、石村 匡崇、足立 俊一、木下 恵志郎、園田 素史、高田 英俊、大賀 正一

    第6回日本免疫不全・自己炎症学会学術集会  2023年2月 

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    開催年月日: 2023年2月

    記述言語:日本語   会議種別:口頭発表(一般)  

    開催地:東京   国名:日本国  

  • Clinical utility of eltrombopag for childhood aplastic anemia - a retrospective study -

    Katsuhide Eguchi, Masataka Ishimura, Saori Endo, Yoshiyuki Takahashi, Shouichi Ohga

    第28回小児再生不良性貧血治療研究会学術集会  2022年1月 

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    開催年月日: 2022年6月 - 2022年1月

    記述言語:英語   会議種別:口頭発表(一般)  

    国名:日本国  

  • Diamond-Blackfan貧血に対する用量調整ブスルファンを用いた非血縁者間骨髄移植

    江口克秀, 石村匡崇, 田村彰広, 伊藤暢宏, 平野直樹, 足立俊一, 木下恵志郎, 幸伏寛和, 矢田裕太郎, 園田素史, 白石暁, 小阪嘉之, 照井君典, 伊藤悦朗, 大賀正一

    第44回日本造血・免疫細胞療法学会学術集会  2022年5月 

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    開催年月日: 2022年5月

    記述言語:日本語   会議種別:口頭発表(一般)  

    開催地:web   国名:日本国  

  • 新生児期に診断された重症血友病Aに対するインヒビター発生抑制のための治療選択

    江口克秀、石村匡崇、江口祥美、井上普介、落合正行、大賀正一

    第62回日本小児血液・がん学会学術集会  2020年11月 

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    開催年月日: 2020年11月 - 2020年12月

    記述言語:日本語  

    開催地:web   国名:日本国  

  • A retrospective study of eltrombopag for the treatment of pediatric aplastic anemia and inherited bone marrow failure syndrome in Japan

    江口克秀、石村匡崇、大賀正一

    第27回小児再生不良性貧血治療研究会学術集会  2020年11月 

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    開催年月日: 2020年11月

    記述言語:日本語   会議種別:口頭発表(一般)  

    開催地:web   国名:日本国  

▼全件表示

MISC

  • 血液症候群(第3版)Ⅳ ―その他の血液疾患を含めて― テーマ:Ⅸ.リンパ系の腫瘍 4.組織球性疾患(1)非腫瘍性組織球症 1)遺伝性血球貪食性リンパ組織球症

    江口 克秀、石村 匡崇、大賀 正一

    2024年2月

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    記述言語:日本語   掲載種別:記事・総説・解説・論説等(学術雑誌)  

  • 第Ⅳ部 原発性免疫不全症候群 3.7 先天性角化不全症 査読

    江口 克秀、石村 匡崇

    朝倉書店   2020年11月

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    記述言語:日本語   掲載種別:記事・総説・解説・論説等(学術雑誌)  

所属学協会

  • 日本血液学会

  • 日本造血・免疫細胞療法学会

  • 日本免疫不全・自己炎症学会

  • 日本小児科学会

  • 日本小児血液がん学会

教育活動概要

  • 医学部4年生に対して、受胎成長発達「血液系の発達と疾患」の講義を担当する。(年1回、90分)
    医歯薬合同講義において、臨床医学Ⅳ「小児の血液疾患」の講義を担当する。(年1回、90分)
    医学部生の臨床実習において指導を行う。

担当授業科目

  • 医歯薬合同講義 臨床医学Ⅳ 小児の血液疾患

    2024年4月 - 現在   前期

  • 九州大学医学部4年生講義 受胎・成長・発達 血液系の発達と疾患

    2024年4月 - 現在   前期

  • 九州大学医学部4年生講義 受胎・成長・発達 血液系の発達と疾患

    2023年4月 - 2023年9月   前期

  • 医歯薬合同講義 臨床医学Ⅳ 小児の血液疾患

    2023年4月 - 2023年9月   前期

  • 医歯薬合同講義 臨床医学Ⅳ 小児の血液疾患

    2022年4月 - 2022年9月   前期

  • 九州大学医学部4年生講義 受胎・成長・発達 血液系の発達と疾患

    2022年4月 - 2022年9月   前期

  • 九州大学医学部4年生講義 受胎・成長・発達 血液系の発達と疾患

    2021年4月 - 2021年9月   前期

  • 九州大学医学部4年生講義 受胎・成長・発達 血液系の発達と疾患

    2020年4月 - 2020年9月   前期

▼全件表示

その他部局等における各種委員・役職等

  • 2020年4月 - 2022年3月   部門 輸血療法委員会

専門診療領域

  • 生物系/医歯薬学/内科系臨床医学/小児科学

臨床医資格

  • 専門医

    日本小児科学会

  • 専門医

    日本血液学会

医師免許取得年

  • 2010年

特筆しておきたい臨床活動

  • 先天性免疫異常症・造血不全症の診療、非腫瘍性疾患に対する造血細胞移植