記述言語：英語   掲載種別：研究論文（学術雑誌）  

AMeD syndrome is characterized by aplastic anemia, mental retardation, short stature, and microcephaly and is caused by digenic mutations in the aldehyde dehydrogenase 2 (ALDH2) and alcohol dehydrogenase 5 (ADH5) genes. We have successfully performed hematopoietic stem cell transplantation in two patients with AMeD syndrome and isochromosome 1q. AMeD syndrome with myelodysplastic syndrome or acute myeloblastic leukemia generally has a poor prognosis; however, early diagnosis may improve treatment response. Although the gain of 1q has been considered as a form of early clonal evolution in Fanconi anemia, it may be an equally important finding observed in AMeD syndrome.

DOI： 10.1016/j.lrr.2024.100476

PubMed

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Background. Staphylococcus lugdunensis is one of the clinically important coagulase-negative staphylococci. The purpose of this study was to elucidate the microbiological features of S. lugdunensis in hospitalized children. Methods. From January 2012 to December 2019, all isolates were retrospectively screened for S. lugdunensis. Results. Twenty-five children were eligible for study. Nineteen and six children were classified into a critical care unit group (Group A) and a general medical ward group (Group B), respectively. The prevalence of methicillin-resistant S. lugdunensis was significantly higher in Group A than in Group B (68.4% vs 0%; P < .01). Eleven children (44%) had S. lugdunensis infections, while the remaining children were colonized. Six of the 11 infected children (55%) had healthcare-associated infections. Moreover, 3 isolates exhibited the methicillin resistance. Conclusions. The bacteriological characteristics of S. lugdunensis differ depending on patient background. Selection of antibiotic treatment should in part rely on patient background data.

DOI： 10.1177/2333794X211044796

PubMed

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：一般社団法人 日本周産期・新生児医学会  

<p> 小児の心臓腫瘍は非常に稀である．病型としては横紋筋腫が64％を占め最も頻度が高い．心横紋筋腫は無症状で自然退縮することも多いが，一方重度の心機能障害や流入路・流出路閉塞，不整脈を来たしうる．近年，mTOR阻害剤であるエベロリムスが有効とする報告が散見される．症例は在胎34週に胎児心臓超音波検査により左室流出路狭窄を合併した心横紋筋腫と診断された．期外収縮を頻発し突然死のリスクがあると考え，日齢0よりエベロリムスの使用を開始した．その結果，超音波検査上の腫瘍断面積は投与前で最大3.0cm²だったが日齢14には1.4cm²まで縮小した．腫瘍の縮小に伴い心室性期外収縮も消失し，循環不全を来さず良好な経過を得た．日齢32にエベロリムスを中止後，心臓腫瘍はわずかに増大したが左室流出路狭窄は来たさず経過した．出生前からの慎重な経過観察と準備により，早期の治療介入が可能であった．</p>

DOI： 10.34456/jjspnm.56.1_148

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1002/gcc.22734

PubMed

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記述言語：英語  

We evaluated the efficacy of linezolid treatment in 6 children with health care-associated meningitis or ventriculitis (HCAMV) caused by gram-positive cocci. All children were diagnosed and treated at the Ehime University Hospital between January 2010 and December 2017. Of these, 5 were treated with linezolid as an empirical therapy. In these 5 patients, vancomycin was initially used but was changed to linezolid because of cerebrospinal fluid (CSF) culture positivity (n = 3) and a high minimum inhibitory concentration of vancomycin (n = 2). The most common HCAMV pathogens were methicillin-resistant coagulase-negative staphylococci (n = 3). In 3 patients, vancomycin concentration was low in CSF but reached the target concentration in serum, while linezolid concentration was high in both CSF and serum. HCAMV treatment using antimicrobial agents with poor CSF penetration may increase the likelihood of therapy failure. Linezolid is more susceptible as the first-line treatment for HCAMV compared with vancomycin.

DOI： 10.1177/0009922818803399

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記述言語：日本語   出版者・発行元：(株)日本小児医事出版社  

【緒言】肋骨骨髄炎は全骨髄炎の中で約1%と非常に稀であり、骨成長期に多く発症するとされている。【症例】生来健康な5歳男児。1ヵ月前から左側胸部痛が出現し、左第6肋骨に腫瘤を触知した。血液検査でWBC 7,700/μL(Neutro 58.5%)、CRP 3.87mg/dLと炎症反応の上昇を認めた。胸部CT検査で左第6肋骨に骨融解を伴う軟部腫瘤影、MRI T1強調画像でlow intensity、T2強調画像でhigh intensityを示す13mm×14mm×15mmの腫瘤を認めた。切開生検術を施行し、病理検査、細菌検査結果からStaphylococcus aureus(MSSA)による左第6肋骨骨髄炎と診断した。掻爬術と合計6週間の抗菌薬治療により治癒した。【結語】肋骨骨髄炎では、発熱といった全身症状がなく局所症状のみの症例も存在することから、小児の肋骨部腫瘤では鑑別すべき疾患の一つと考えられる。(著者抄録)

J-GLOBAL

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DOI： 10.1002/ccr3.1506

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY  

BackgroundChildhood cancer survivors' (CCSs') readiness for adult care has not been evaluated in Japan. We conducted a survey to examine transition barriers and facilitators in CCSs and compared the results with those of CCSs in Canada.
MethodsParticipants were selected from the Heart Link mutual-aid health insurance membership directory and the Millefeuille Childhood Cancer Frontiers. We conducted a cross-sectional survey (self-report questionnaire) via mail, using the Transition Scales.
ResultsIn total, 268 questionnaires were collected by January 2016 (response rate, 42.5%). After confirming the reliability and validity of the Transition Scales, we analyzed 242 questionnaires. After excluding questionnaires for CCSs younger than 15 or older than 26years, we compared scales scores between Japanese and Canadian CCSs. Relative to that of Japanese CCSs, Canadian CCSs showed greater cancer-related worry for 4 items (P&lt;.001) and preference for self-management in 3 items (P&lt;.001). Japanese CCSs showed greater preference for self-management, relative to that of Canadian CCSs, in 5 items (P&lt;.001). In the expectation scale, Japanese CCSs showed lower levels of expectation concerning adult care in 6 of 12 items (P&lt;.001). Relative to that of Canadian CCSs, a significantly higher number of Japanese CCSs preferred to visit the same doctor for long-term care as adults (P&lt;.001).
ConclusionsThe results confirmed the reliability and validity of the Transition Scales and showed that Japanese CCSs expressed fewer cancer concerns, but a higher number of Japanese CCSs preferred to visit the same doctor for long-term care as adults.

DOI： 10.1002/pon.4276

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記述言語：日本語   出版者・発行元：日本小児血液・がん学会  

<p>小児のフィラデルフィア染色体陽性急性リンパ性白血病（Ph+ ALL）の予後はチロシンキナーゼ阻害薬（TKI）の使用により改善しつつあるが，副作用や合併症など解決すべき課題も残っている．症例は13歳男児．Ph+ ALLと診断されimatinib併用による化学療法を開始したが，再寛解導入療法開始day 14から発熱性好中球減少症と血圧低下を認めた．抗菌薬を開始したが敗血症性ショックに至り，急性腎不全，腸管浮腫，急性呼吸窮迫症候群の進行により再寛解導入療法day62に死亡した．剖検所見からは敗血症性ショックからの多臓器不全が死因と考えられたが，血液培養で大腸菌が検出され，敗血症の機序は腸管を通じたbacterial translocation （BT） の可能性が示唆された．Ph+ ALLの治療中に遷延する骨髄抑制時にはimatinib併用によってBTが増強する可能性があり，速やかな抗菌薬の使用が必要である．</p>

DOI： 10.11412/jspho.54.15

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

Background:Myeloid/NK cell precursor acute leukemia (MNKL) is a rare type of leukemia, and ocular complications have not previously been reported. We now report a patient with MNKL who developed intraocular infiltrates during follow-up.Methods and Results:A 13-year-old boy diagnosed with MNKL developed left eye pain 3 months after starting treatment. Examination of the left eye revealed a visual acuity of counting fingers at 20cm, ciliary hyperemia, small corneal keratic precipitates, hypopyon, grade 4 vitreous opacities, and an obscured fundus. The differential diagnosis was between an opportunistic infection associated with immunodeficiency and an intraocular leukemic cell infiltrate. Therefore, a sample of aqueous humor was aspirated. Multiplex PCR/broad-range PCR of the aqueous humor was below detection limits for viruses, bacteria, and fungi. Flow cytometry (FCM) detected NK-related CD56-positive cells, thus leading to a diagnosis of ocular infiltrates due to MNKL. With treatment of the ocular infiltrates by consolidation systemic chemotherapy including intrathecal methotrexate (MTX), there was clearing of the vitreous opacities; and optic disc swelling, retinal hemorrhages, exudates, and protuberant lesions were now seen. With the addition of local radiation therapy to the eye, there was a dramatic treatment response, with regression of the optic disc findings and retinal lesions, and an improved visual acuity of 1.5.Conclusion:We encountered the first case of MNKL in which ocular infiltrates developed during follow-up. Multiplex PCR and FCM of the aqueous humor were useful in rapidly distinguishing leukemic cell infiltrates from an opportunistic infection. This case highlights the usefulness of intrathecal MTX and local radiotherapy in treating ocular infiltrates in patients with MNKL.

DOI： 10.1097/MD.0000000000004967

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PubMed

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記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：文光堂  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

Outcome of children with acute lymphoblastic leukemia (ALL) has improved over the years, but not for those with multiple recurrences because of high therapy resistance and heavily pretreated history that potentially cause physical damages. We describe the case of an 11-year-old boy with a third relapse of ALL and a history of 2 allogeneic bone marrow transplantations. He was successfully treated with clofarabine combination chemotherapy and achieved a fourth remission at 16 months following haploidentical bone marrow transplantation with conditioning regimen of clofarabine and busulfan. Clofarabine/busulfan conditioning might be a preferable option for children with multiple recurrent ALL, and warrants further investigation.

DOI： 10.1097/MPH.0000000000000454

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PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Lippincott Williams and Wilkins  

Background: Myeloid/NK cell precursor acute leukemia (MNKL) is a rare type of leukemia, and ocular complications have not previously been reported. We now report a patient with MNKL who developed intraocular infiltrates during follow-up. Methods and Results: A 13-year-old boy diagnosed with MNKL developed left eye pain 3 months after starting treatment. Examination of the left eye revealed a visual acuity of counting fingers at 20cm, ciliary hyperemia, small corneal keratic precipitates, hypopyon, grade 4 vitreous opacities, and an obscured fundus. The differential diagnosis was between an opportunistic infection associated with immunodeficiency and an intraocular leukemic cell infiltrate. Therefore, a sample of aqueous humor was aspirated. Multiplex PCR/broad-range PCR of the aqueous humor was below detection limits for viruses, bacteria, and fungi. Flow cytometry (FCM) detectedNK-relatedCD56-positivecells,thusleadingtoadiagnosisofocularinfiltratesduetoMNKL.Withtreatmentoftheocularinfiltrates by consolidation systemic chemotherapy including intrathecal methotrexate (MTX), there was clearing of the vitreous opacities
and optic discswelling,retinalhemorrhages,exudates,andprotuberantlesionswerenowseen.Withtheadditionoflocalradiationtherapytotheeye, there was a dramatic treatment response, with regression of the optic disc findings and retinal lesions,and an improved visual acuity of 1.5.

DOI： 10.1097/MD.0000000000004967

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

Infantile hemangioma (IH) is the most common tumor of infancy, and it sometimes associated with Kasabach-Meritt syndrome (KMS) characterized by anemia, intraperitoneal hemorrhage secondary to rupture, coagulopathy, jaundice, and vascular malformations involving the brain, skin, gut, and other organs. Here, we report two newborn patients having IH with KMS at birth. The first patient had a giant hemangioma in the liver, which was successfully treated with i.v. corticosteroid and coil embolization. The second patient had a large hemangioma of the right axillary region, which was also successfully treated with i.v. corticosteroid, beta-blocker, coil embolization and local irradiation. All symptoms were controlled without any side-effects in both patients. According to these findings, combination therapy including coil embolization and corticosteroid is effective for IH patients with KMS. The indications for and timing of coil embolization should be determined further cases have been accumulated.

DOI： 10.1111/ped.12618

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PubMed

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記述言語：日本語   出版者・発行元：(一社)日本てんかん学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

BackgroundSerum procalcitonin (PCT) increases in various respiratory disorders such as acute respiratory distress syndrome. Elevated PCT is also observed in healthy neonates. In this study, we investigated whether PCT is a good marker of respiratory disorder in neonates.
MethodsA total of 155 neonates with or without respiratory disorder, were eligible for the study. PCT was measured on electrochemiluminescence immunoassay. Each neonate was allocated to the non-respiratory disorder (control) group (n = 95), or a respiratory disorder group (n = 60). PCT was compared between the groups, and association with other markers, including C-reactive protein (CRP) and white blood cell (WBC) count, was analyzed.
ResultsOf the 60 neonates in the respiratory disorder group, 39, 10, five, one, two, two, and one neonates had transient tachypnea of the newborn, respiratory distress syndrome, air leak syndrome, meconium aspiration syndrome, 18-trisomy, neonatal asphyxia, and congenital diaphragmatic hernia, respectively. Mean PCT, CRP and WBC count in the respiratory disorder group were 9.01ng/mL, 0.26mg/dL, and 16100 cells/L, respectively. The area under the curve obtained for PCT in distinguishing between the respiratory disorder and control groups was 0.85 (sensitivity, 66.7%; specificity, 93.0%; optimum cut-off, 3.73ng/mL), that for CRP was 0.72 (sensitivity, 75.0%; specificity, 64.6%; optimum cut-off, 0.14mg/dL), and for WBC it was 0.44 (sensitivity, 60.0%; specificity, 29.6%; optimum cut-off, 15000cells/L).
ConclusionsPCT is more susceptible, as a diagnostic parameter of infection, to the effect of respiratory disturbance than CRP and WBC.

DOI： 10.1111/ped.12505

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PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

We encountered a case of neonatal acute megakaryoblastic leukemia not associated with Down syndrome (DS). Molecular cytogenetic analysis of leukemic blast cells indicated that increased blast cell status was caused by transient abnormal myelopoiesis with trisomy 21 and GATA1 mutation. Based on these molecular cytogenetic data, intensive chemotherapy was avoided, and the patient was successfully cured with low-dose cytarabine. Morphologically, leukemic blast cells of acute megakaryoblastic leukemia in a non-DS neonate are indistinguishable from a blast cell of transient abnormal myelopoiesis. The possibility of transient abnormal myelopoiesis should be carefully considered before intensive chemotherapy is adopted.

DOI： 10.1111/ped.12500

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その他リンク： http://orcid.org/0000-0003-1520-7007

記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本てんかん学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：NATURE PUBLISHING GROUP  

Hematopoietic cell transplantation (HCT) is used for treatment of hematopoietic diseases. Assessment of T-and B-cell reconstitution after HCT is crucial because poor immune recovery has a major effect on the clinical course. In this study, we retrospectively analyzed T-cell receptor excision circles (TRECs) as well as signal and coding joint kappa-deleting recombination excision circles (sjKRECs and cjKRECs, respectively) as markers of newly produced lymphocytes in 133 patients (56 primary immunodeficient and 77 malignant cases, median (range): 12 (0-62) years old). We analyzed the kinetics of TREC and KREC recovery and determined the factors that contributed to better immune recovery. KRECs became positive earlier than TRECs and increased thereafter. Younger recipient age had a favorable effect on recovery of sjKRECs and cjKRECs. Compared with BM and peripheral blood, our data suggested that cord blood (CB) provided rapid B-cell recovery. CB also provided better B-cell neogenesis in adult HCT recipients. Chronic GVHD was associated with low TRECs, but not increased sjKRECs/cjKRECs. Finally, positive sjKRECs 1 month after HCT were associated with fewer infectious episodes. Monitoring of TRECs and KRECs may serve as a useful tool for assessment of immune reconstitution post HCT.

DOI： 10.1038/bmt.2014.123

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(公社)日本薬学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児感染症学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   出版者・発行元：(一社)日本癌学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：日本小児血液・がん学会  

再寛解導入療法中に肺動脈血栓症（PTE）を発症したB-前駆細胞性急性リンパ性白血病の3歳男児を経験した．寛解導入療法直前に末梢挿入式中心静脈カテーテル（PICC）を挿入し，以後約6か月間使用していた．PTE発症前にD-ダイマー上昇とカテーテル閉塞徴候が認められていた．未分画ヘパリン持続点滴後にPICCを抜去し，ワーファリンによる抗凝固療法を行ったところ，2週後に臨床症状は改善した．ワーファリンを継続しながら2回目の再寛解導入療法前にPICCを再挿入したが，血栓症の再燃は認めず，予定の化学療法を継続しえた．本症例のPTEはカテーテル関連血栓症と考えられた．

PICCは従来の中心静脈カテーテルと比較して血栓症の頻度が高いとされるため，カテーテルの選択や留置位置，留置が長期化する場合の管理に細心の注意を払うことが重要である．

DOI： 10.11412/jspho.56.46

CiNii Research

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記述言語：日本語   出版者・発行元：日本小児血液・がん学会  

小児がん経験者の成人期医療移行への準備状況や長期フォローアップ （FU） 支援ツールの周知状況について調査した．方法：研究デザインは，自記式の紙を媒体とした横断研究で，対象はハートリンク共済保険加入者/問い合わせした方またはミルフィーユ小児がんフロンティアーズ登録者で研究に同意した小児がん経験者である．結果：解析対象の小児がん経験者は，男性132人，女性110人で，調査時年齢の中央値は26歳（12歳～47歳） であった．「ある程度～十分わかる」 と答えたのは，病気説明では74%，病期・リスクと治療内容列挙では51～52%，晩期合併症リスクに関しては31%であり，生活習慣注意点や病院に連絡すべき場合についても45～49%であった．一方抗癌剤の種類は50%が，個々の抗癌剤使用や総量は80%が「全くわからない」と答えた．支援ツールについて「ある程度～十分わかる」と答えたのは，治療サマリーで43%，FU手帳の活用では18%，長期FUガイドラインの存在の周知は13%であった．それぞれの理解・周知度に関連していた因子の検討では，年少期発症/小児固形腫瘍（芽腫）や主観的健康度良好なものの認知が低く，高学歴のものは認知が高かった．結論：長期FU支援ツールの認知度は予想以上に低く，小児がん経験者でこれらの支援ツールの周知や活用は不十分である．年少期発症や低学歴では周知に特別の配慮が必要である．

DOI： 10.11412/jspho.53.436

CiNii Research

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記述言語：日本語   出版者・発行元：(一社)日本小児神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語  

J-GLOBAL

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