Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

AIMS: Although shortening of the corrected QT interval (QTc) is a key finding in the diagnosis of short QT syndrome (SQTS), there may be overlap of the QTc between SQTS patients and normal subjects in childhood and adolescence. We aimed to investigate electrocardiographic findings for differentiation of SQTS patients. METHODS AND RESULTS: The SQTS group comprised 34 SQTS patients <20 years old, including 9 from our institutions and 25 from previous reports. The control group comprised 61 apparently healthy subjects with an QTc of <360 ms who were selected from 13 314 participants in a school-based screening programme. We compared electrocardiographic findings, including QT and Jpoint-Tpeak intervals (QT and J-Tpeak, respectively), those corrected by using the Bazett's and Fridericia's formulae (cB and cF, respectively) and early repolarization (ER) between the groups. QT, QTc by using Bazett's formula (QTcB), QTc by using Fridericia's formula (QTcF), J-Tpeak, J-Tpeak cB, and J-Tpeak cF were significantly shorter in the SQTS group than in the control group. On receiver operating characteristic curve analysis, the area under the curve (AUC) was largest for QTcB (0.888) among QT, QTcB, and QTcF, with a cut-off value of 316 ms (sensitivity: 79.4% and specificity: 96.7%). The AUC was largest for J-Tpeak cB (0.848) among J-Tpeak, J-Tpeak cB, and J-Tpeak cF, with a cut-off value of 181 ms (sensitivity: 80.8% and specificity: 91.8%). Early repolarization was found more frequently in the SQTS group than in the control group (67% vs. 23%, P = 0.001). CONCLUSION: A QTcB <316 ms, J-Tpeak cB < 181 ms, and the presence of ER may indicate SQTS patients in childhood and adolescence.

DOI： 10.1093/europace/euab097

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

Marfan症候群(MFS)に心室中隔欠損(VSD)を合併し、急速に心不全が進行し、手術前後で心機能が低下した乳児例を経験した。症例は2ヵ月の女児で、VSDによる高肺血流性心不全と診断され、当院を紹介となった。家族歴、身体所見からMFSが疑われた。高肺血流に心機能低下も伴うため、段階的に修復術を施行された。肺動脈絞扼術直後と心内修復術直後にそれぞれ心機能は低下したが、徐々に回復した。3歳時に水晶体脱臼を認め、父がMFSであることから改訂Ghent基準を満たした。MFSは潜在的な心機能障害を有する可能性があり、治療介入が必要な先天性心疾患を合併する例では、術前の心不全の進行や術後管理に注意し、慎重な経過観察や治療戦略を考慮すべきである。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2020&ichushi_jid=J02003&link_issn=&doc_id=20200817180011&doc_link_id=10.9794%2Fjspccs.36.159&url=https%3A%2F%2Fdoi.org%2F10.9794%2Fjspccs.36.159&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Authorship：Lead author,　Corresponding author   Language：Japanese   Publisher：(NPO)日本小児循環器学会  

遺伝性不整脈は、心筋活動電位を形成するイオンチャネルとこれに関連する蛋白などをコードする遺伝子変異によって発症する疾患の総称である。1957年にQT延長症候群が初めて報告され、現在ではカテコラミン誘発多形性心室頻拍、Brugada症候群、QT短縮症候群、早期再分極症候群、進行性心臓伝導障害も遺伝性不整脈とみなされている。若年突然死の主要な原因であるが、早期発見と介入により予防しうる。遺伝子解析の進歩により、原因不明であった失神、突然死に遺伝性不整脈の診断がつき、個々により適した管理、治療が行われるようになってきている。近年、日本循環器学会のガイドラインも改訂された。これも踏まえて本稿では、QT延長症候群、QT短縮症候群、カテコラミン誘発多形性心室頻拍、Brugada症候群について述べる。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2019&ichushi_jid=J02003&link_issn=&doc_id=20200409160006&doc_link_id=%2Fee7shoni%2F2019%2F003504%2F008%2F0249-0263%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fee7shoni%2F2019%2F003504%2F008%2F0249-0263%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Japanese Circulation Society  

DOI： 10.1253/circj.cj-18-0984

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Authorship：Lead author   Language：Japanese   Publishing type：Research paper (scientific journal)  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)  

Importance: Few studies with sufficient statistical power have shown the association of the z score of the coronary arterial internal diameter with coronary events (CE) in patients with Kawasaki disease (KD) with coronary artery aneurysms (CAA). Objective: To clarify the association of the z score with time-dependent CE occurrence in patients with KD with CAA. Design, Setting, and Participants: This multicenter, collaborative retrospective cohort study of 44 participating institutions included 1006 patients with KD younger than 19 years who received a coronary angiography between 1992 and 2011. Main Outcomes and Measures: The time-dependent occurrence of CE, including thrombosis, stenosis, obstruction, acute ischemic events, and coronary interventions, was analyzed for small (z score, <5), medium (z score, ≥5 to <10; actual internal diameter, <8 mm), and large (z score, ≥10 or ≥8 mm) CAA by the Kaplan-Meier method. The Cox proportional hazard regression model was used to identify risk factors for CE after adjusting for age, sex, size, morphology, number of CAA, resistance to initial intravenous immunoglobulin (IVIG) therapy, and antithrombotic medications. Results: Of 1006 patients, 714 (71%) were male, 341 (34%) received a diagnosis before age 1 year, 501 (50%) received a diagnosis between age 1 and 5 years, and 157 (16%) received a diagnosis at age 5 years or older. The 10-year event-free survival rate for CE was 100%, 94%, and 52% in men (P < .001) and 100%, 100%, and 75% in women (P < .001) for small, medium, and large CAA, respectively. The CE-free rate was 100%, 96%, and 79% in patients who were not resistant to IVIG therapy (P < .001) and 100%, 96%, and 51% in patients who were resistant to IVIG therapy (P < .001), respectively. Cox regression analysis revealed that large CAA (hazard ratio, 8.9; 95% CI, 5.1-15.4), male sex (hazard ratio, 2.8; 95% CI, 1.7-4.8), and resistance to IVIG therapy (hazard ratio, 2.2; 95% CI, 1.4-3.6) were significantly associated with CE. Conclusions and Relevance: Classification using the internal diameter z score is useful for assessing the severity of CAA in relation to the time-dependent occurrence of CE and associated factors in patients with KD. Careful management of CE is necessary for all patients with KD with CAA, especially men and IVIG-resistant patients with a large CAA.

DOI： 10.1001/jamapediatrics.2018.0030

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1111/ped.13552

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Language：Japanese   Publisher：新潟医学会  

症例は10ヵ月の男児。発熱とけいれんを主訴に当院に入院した。Haemophilus influenzae type b(Hib)ワクチンおよび肺炎球菌ワクチンは接種済み。健診で異常を指摘されたことはなかった。入院時、多核球優位の髄液細胞数増多を認め、細菌性髄膜炎と診断された。セフトリアキソン、バンコマイシン、デキサメタゾンで治療を開始され、一旦は解熱したが、入院4日目から再度発熱した。腰仙部中央に痂皮形成を伴う発赤があり、痂皮下に軽微な陥凹を認めたため腰部MRIを施行した。皮膚洞、硬膜内に連続する嚢状構造物を認め、先天性皮膚洞と硬膜内膿瘍が疑われた。入院8日目の髄液検査では再度細胞数の増多を認めた。標準的な抗菌薬治療でも解熱せず、外科的介入が必要と判断された。入院11日目に高次医療機関に転院し、皮膚洞と嚢胞を摘出された。転院後の髄液培養でStaphylococcus lugdunensisが検出された。病理の結果、先天性皮膚洞、類皮嚢胞と診断された。術後経過は良好で転院26日目に退院した。術後の運動発達は良好で、1歳で独歩を獲得している。本症例は髄膜炎が先天性皮膚洞発見の契機となった。非感染時の皮膚症状が軽微で健診では確認が困難であったが、入院時の診察で皮膚異常を認めたことで診断に至った。先天性皮膚洞は早期発見と介入が重要である。皮膚所見が軽微でも、臀裂内にない陥凹等のリスクのある症例を見逃さないことが大切と考えられた。Hib、肺炎球菌ワクチン接種済みの症例や通常想定されない起因菌の細菌性髄膜炎では、解剖学的異常を含めた基礎疾患の可能性を特に考えるべきである。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2018&ichushi_jid=J00990&link_issn=&doc_id=20181107050007&doc_link_id=%2Fdg3nigta%2F2018%2F013205%2F007%2F0203-0207%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2018%2F013205%2F007%2F0203-0207%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Blackwell Publishing  

Aim: We aimed to assess the accuracy and effectiveness of fetal cardiac screening for congenital heart disease (CHD) during the second trimester by general obstetricians in a non-selected population. Methods: In this multicenter, prospective cohort study of fetal cardiac screening, four-chamber and three-vessel views were recorded by obstetricians at 18–21 gestational weeks (GW). A total of 3005 fetuses that were scheduled for delivery at our institution were included. Results: Thirty-seven newborns were born with CHD (1.2%). On excluding 23 cases of ventricular septal defects, the prenatal detection rate of CHD was 42.8%. Although six cases (75.0%) of severe structural abnormality were diagnosed prenatally, the prenatal detection rate of valvular abnormalities was 0%. Conclusion: One-point ultrasound screening of the fetal heart using a combination of four-chamber and three-vessel views at 18–21 GW by general obstetricians in a non-selected population may be useful for detecting severe structural abnormalities but not valvular abnormalities. However, this limitation may be improved by conducting another fetal cardiac screening at approximately 30 GW along with the routine use of color Doppler.

DOI： 10.1111/jog.13472

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© 2018, Japanese Circulation Society. All rights reserved. Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) has been often misdiagnosed as long QT syndrome (LQTS) type 1 (LQT1), which phenotypically mimics CPVT but has a relatively better prognosis. Methods and Results: The derivation and validation cohorts consisted of 146 and 21 patients, respectively, all of whom had exercise-or emotional stress-induced cardiac events. In the derivation cohort, 42 and 104 patients were first clinically diagnosed with CPVT and LQTS, respectively. Nine of 104 patient who had initial diagnosis of LQTS were found to carry RYR2 mutations. They were misdiagnosed due to 4 different reasons: (1) transient QT prolongation after cardiopulmonary arrest; (2) QT prolongation after epinephrine test; (3) absence of ventricular arrhythmia after the exercise stress test (EST); and (4) assumption of LQTS without evidence. Based on genetic results, we constructed a composite scoring system by modifying the Schwartz score: replacing the corrected QT interval (QTc) at 4 min recovery time after EST >480 ms with that at 2 min, or with ∆QTc (QTc at 2 min of recovery−QTc before exercise) >40 ms and assigning a score of −1 for ∆QTc <10 ms or documented polymorphic ventricular arrhythmias. This composite scoring yielded 100% sensitivity and specificity for the clinical differential diagnosis between LQT1 and CPVT when applied to the validation cohort. Conclusions: The modified Schwartz score facilitated the differential diagnosis between LQT1 and CPVT.

DOI： 10.1253/circj.CJ-17-1032

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Language：Japanese   Publisher：新潟医学会  

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J00990&link_issn=&doc_id=20171214040016&doc_link_id=%2Fdg3nigta%2F2017%2Fs13108%2F007%2F0513-0513%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2017%2Fs13108%2F007%2F0513-0513%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier Ltd  

Mutation of the BMPR2 gene is the most common genetic cause of pulmonary arterial hypertension (PAH). Although there have been some reports of BMPR2 mutation carriers among PAH patients with congenital heart disease, there have been few reports of their treatment. Here, we describe a 13-year-old female BMPR2 mutation carrier who presented with heritable PAH and atrial septal defect (ASD). She complained of fatigue, and cardiac catheterization showed a mean pulmonary artery pressure (PAP) of 56 mmHg, a pulmonary vascular resistance (PVR) of 8 Wood units and a pulmonary to systemic blood flow ratio (Qp/Qs) of 1.3. Following 2 years of medication therapy, the mean PAP had decreased to 30 mmHg, the Qp/Qs had increased to 2.7, and her symptoms persisted. We closed the ASD interventionally, and her symptoms improved after closure. Medication therapy was continued. Four years after closure, the PAH had improved with a mean PAP of 20 mmHg and a PVR of 3.1 Wood units. To the best of our knowledge, this is the first report of PAH improvement following medication and ASD closure in a BMPR2 mutation carrier with heritable PAH. ASD closure following medication appears to be effective in some ASD patients with heritable PAH. &lt
Learning objective: Mutation of the BMPR2 gene is the most common genetic cause of pulmonary arterial hypertension (PAH). Heritable PAH with BMPR2 mutations has been reported to have a poorer prognosis once PAH has developed. Recent reports have described treatment of atrial septal defect (ASD) patients with PAH by surgical or interventional ASD closure following medication therapy. This case suggests that medication followed by ASD closure could also be effective for BMPR2 mutation carriers with ASD and heritable PAH.&gt

DOI： 10.1016/j.jccase.2017.03.005

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Language：Japanese   Publisher：(株)東京医学社  

5歳9ヵ月女児。生来健康であったが、発熱、意識障害、けいれんを発症し、項部硬直を認め救急搬送された。髄液検査で細胞数2/3μL、髄液糖67mg/dL、髄液蛋白10mg/dLであり、セフォタキシム300mg/kg/日およびデキサメタゾン0.6mg/kg/日の静脈内投与を開始した。尿定性で白血球反応2+、亜硝酸塩1+、尿沈渣で白血球数5〜9/μLを認めたが、細菌性髄膜炎と誤診していたため尿培養は行わなかった。入院後深夜までに意識清明となり、2日目以降は解熱が維持され、髄液検査で細胞数増加を認めなかった。血液・髄液培養48時間までに有意な菌は同定されなかったが、入院4日目の全身造影CTにて右腎に区域性の血流欠損像を認め、急性巣状細菌性腎炎と診断した。セフォタキシムを200mg/kg/日に減量して10日間治療を行い、セファクロル内服へ移行し退院した。その後外来にて排尿時膀胱尿道造影で右腎の膀胱尿管逆流II度を認めたためセファクロル予防内服を継続した。

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：新潟県医師会  

1973年に学校保健法施行規則の改定がなされ、定期健康診断として学校心臓検診の実施が義務付けられた。1975年には省略4誘導心電図+心音図と調査票方式の学校心臓検診が理想的と決まり、心臓管理指導表が制定された。さらには学童集団検診用の心電図判定基準も制定され、実施基準が整っていった。法整備や診断・管理基準等の策定の経緯、さらに先進的に取り組んできた新潟市での歩みを中心に述べた。

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Language：Japanese   Publisher：新潟医学会  

日本での小児心臓突然死は減少傾向にあるが、いまだ発生している。さらなる抑制には心事故の予防と事後対応が重要である。予防は心疾患の早期発見と介入であり、学校心臓検診が柱となる。一方事後対応では、目撃者によるAED使用を含む適切な心肺蘇生が求められる。既報と自験の院外心停止症例から見えてくる課題と、心臓突然死ゼロを目指した学校心臓検診での我々の取り組みを紹介する。(著者抄録)

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：ELSEVIER IRELAND LTD  

Objective: Coronary artery lesions (CALs) and a risk for early onset of atherosclerosis are major concerns following Kawasaki disease (KD). Intimal smooth muscle cells (SMCs) have an important role in vascular lesions in KD. It is known that soluble LR11 (sLR11) is a novel biomarker for vascular lesions and LR11 is markedly expressed in intimal SMCs in atherosclerotic lesions. In this study, we hypothesized that sLR11 reflects the presence of vascular lesions late after KD.
Methods: Twenty-three age-matched controls (group 1) and 59 patients with a history of KD were enrolled; 36 with KD had normal coronary arteries or regressed aneurysms (group 2), and 23 had CALs (group 3).
Results: Serum sLR11 levels in group 3 (median, interquartile range (IQR): 11.1 ng/mL, 9.3-13.9 ng/mL) were significantly higher than those in groups 1 (8.4 ng/mL, 7.1-10.2 ng/mL, p &lt; 0.001) and 2 (9.0 ng/mL, 7.7-10.1 ng/mL, p &lt; 0.01). Levels of sLR11 were positively correlated with levels of high-sensitivity C-reactive protein (r = 0.480, p &lt; 0.01) and lipoprotein (a) (r = 0.486, p &lt; 0.01).
Conclusion: These findings suggest that sLR11 reflects the development of vascular lesions in patients with serious CALs. (C) 2015 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.atherosclerosis.2015.12.035

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：JAPANESE CIRCULATION SOC  

Background: In children with long QT syndrome (LQTS), risk factors for cardiac events have been reported, but age-, gender-and genotype-related differences in prognosis remain unknown in Asian countries.
Methods and Results: The study examined clinical prognosis at age between 1 and 20 years in 496 LQTS patients who were genotyped as either of LQT1-3 (male, n=206). Heterozygous mutations were observed in 3 major responsible genes: KCNQ1 in 271, KCNH2 in 192, and SCN5A in 33 patients. LQTS-associated events were classified into 3 categories: (1) syncope (n=133); (2) repetitive torsade de pointes (TdP, n=3); and (3) cardiopulmonary arrest (CPA, n=4). The risk of cardiac events was significantly lower in LQT1 girls than boys &lt;= 12 years (HR, 0.55), whereas LQT2 female patients &gt;= 13 years had the higher risk of cardiac events than male patients (HR, 4.60). Patients in the repetitive TdP or CPA group included 1 LQT1 female patient, 1 LQT2 male patient, and 5 LQT2 female patients. All LQT2 patients in these groups had TdP repeatedly immediately after the antecedent event. In addition, all 5 female LQT2 patients in these groups had the event after or near puberty.
Conclusions: Female LQT2 children might have repeated TdP shortly after prior events, especially after puberty.

DOI： 10.1253/circj.CJ-15-0933

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Language：Japanese   Publisher：公益財団法人 日本心臓財団  

<p> 症例は1歳 男児. 啼泣後呼吸を止め, 生後4カ月に前医でBrugada症候群と診断. Holter心電図で784回/日のVfを認めた. Vfはイソプロテレノールとアトロピン投与によりほぼ消失. 硫酸キニジン等内服開始したが, Vfは完全に消失せず, ICD植込み適応と判断. 当院紹介され, 1歳1カ月でICD植込み. 経静脈的リード挿入は困難で, 心外膜内側に心外膜パッチを留置. その後Vf減少し, ICD作動はなかった. 1歳11カ月で呼吸困難を認めた. 心外膜パッチによる両心室拡張障害と判断し, ICD再植込み. 3歳1カ月頃より両心室の拘束性障害が進行し, 心不全入院を繰り返したが, Vfはほとんどなかった. 5歳過ぎに意識消失あり, VfのためICDが作動. また蛋白漏出性胃腸症と診断. その後も心不全入院を繰り返した. 8歳2カ月よりICD作動が増加. キニジン増量等を行った. 8歳5カ月の早朝にelectrical stormとなり, ICD作動したがPEAとなり, 永眠.</p>

DOI： 10.11281/shinzo.48.S1_164

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

Background-Electrical storms (ESs) in patients with Brugada syndrome (BrS) are rare though potentially lethal.
Methods and Results-We studied 22 men with BrS and ES, defined as &gt;= 3 episodes/d of ventricular fibrillation (VF) and compared their characteristics with those of 110 age-matched, control men with BrS without ES. BrS was diagnosed by a spontaneous or drug-induced type 1 pattern on the ECG in the absence of structural heart disease. Early repolarization (ER) was diagnosed by J waves, ie, &gt;0.1 mV notches or slurs of the terminal portion of the QRS complex. The BrS ECG pattern was provoked with pilsicainide. A spontaneous type I ECG pattern, J waves, and horizontal/descending ST elevation were found, respectively, in 77%, 36%, and 88% of patients with ES, versus 28% (P&lt;0.0001), 9% (P=0.003), and 60% (P=0.06) of controls. The J-wave amplitude was significantly higher in patients with than without ES (P=0.03). VF occurred during undisturbed sinus rhythm in 14 of 19 patients (74%), and ES were controlled by isoproterenol administration. All patients with ES received an implantable cardioverter defibrillator and over a 6.0 +/- 5.4 years follow-up, the prognosis of patients with ES was significantly worse than that of patients without ES. Bepridil was effective in preventing VF in 6 patients.
Conclusions-A high prevalence of ER was found in a subgroup of patients with BrS associated with ES. ES appeared to be suppressed by isoproterenol or quinidine, whereas bepridil and quinidine were effective in the long-term prevention of VF in the highest-risk patients.

DOI： 10.1161/CIRCEP.114.001806

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：WILEY-BLACKWELL  

We report on an asymptomatic 10-year-old boy who had a short QT interval (corrected QT interval, 260ms). Short QT syndrome (SQTS) was detected in a school screening program for heart disease and the patient was subsequently diagnosed as having N588K mutation in the KCNH2 gene. Quinidine prolonged the QT interval, but not the QU interval. During treadmill exercise stress test, QT and QU intervals responded differently to heart rate changes, suggesting a mechanoelectrical hypothesis for the origin of the U wave. Although rare, attention should be paid to SQTS, which is associated with potential fatal arrhythmias.

DOI： 10.1111/ped.12308

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：新潟医学会  

学校検診受診者のQT短縮者を抽出し、QT短縮症候群(SQTS)の有無とQT短縮例に関する特徴と不整脈素因の有無を検討した。SQTSと診断された例はなかった。QT短縮例で、運動負荷心電図、ホルター心電図で問題のある不整脈が検出された例はなかったが、抽出者の中に心臓関連死を疑われる若年突然死の家族歴を認めた。一般小児でSQTSが判明することはまれであるが、今回の検討ではQT短縮者には、心拍数とQT時間関係がSQTSと類似の所見を示す例や、不整脈関連死を示唆する家族歴もあり、不整脈素因については更なる検討が必要と考えられた。(著者抄録)

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：SPRINGER  

Two cases of coronary aneurysm developed in the late period after Kawasaki disease (KD). Case 1 involved a 13-year-old boy who had aneurysms develop after a diagnosis of complete regression. Case 2 involved a 29-year-old man who had a new aneurysm develop after he was older than 20 years. Physicians need to be aware that coronary aneurysms can develop in patients with antecedent KD even after regression or in adulthood.

DOI： 10.1007/s00246-012-0543-x

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Language：Japanese   Publisher：(公社)日本小児科学会  

症例1:6ヵ月女児。4ヵ月時に心雑音を指摘され、心エコーおよび心臓カテーテル検査で大動脈弁閉鎖症(AR)と診断し、心不全がコントロール不能となった場合に手術を施行する方針とした。しかし、経過観察中に大動脈左室トンネル(ALVT)の疾患概念を知り、改めて心エコーを施行しALVTと診断した。Dual source CTでトンネルの部位を明らかにし、1歳4ヵ月時に心内修復術を施行した。術後経過は良好で、残存シャントはなく、ARの合併も認めず、術後1年半の現在も経過良好である。症例2:16歳女。10歳時に心雑音を指摘され、前医でARの診断のもと経過観察されていたが、徐々に易疲労感が出現した。心エコー、心臓カテーテル検査、Dual source CTでALVTと診断し、心不全の症状を認めたことから閉鎖術を施行した。術後経過は良好であったが、ALVTのshuntが残存し、ARも軽度残存した。易疲労感も残存し、利尿薬とアスピリンで経過観察している。

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：日本小児高血圧研究会  

症例は14歳男子で、学校心電図健診で左室肥大を指摘され受診した。肥大型心筋症を疑い、更に検査を勧めたところ、安静時血圧は124/62mmHgであったが、トレッドミル負荷により血圧は206/98mmHgまで上昇し、運動中止後の回復も時間を要した。24時間血圧でも収縮期血圧が150mmHgを超えるところがあり、日中平均血圧は130/85mmHgと高値であった。高血圧の精査を行ったところ、血漿レニン活性は正常で、他の内分泌学的検査も正常範囲であった。腹部CTで左腎に多発する小嚢胞が認められた。家族歴がなく、片側性であった常染色体優性多発性嚢胞腎(ADPKD)が疑われADPKDが高血圧の原因と考えられた。アンジオテンシン受容体拮抗薬内服を開始し、血圧コントロールを行い、経過観察を続けている。治療開始後1年経過し、収縮期血圧は120mmHg前後で推移し、左心肥大の所見もやや改善が認められた。

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：JAPAN SOC INTERNAL MEDICINE  

An 8-year-old boy suffered from an unconsciousness attack and torsade de pointes arrhythmia during sleep or at rest. His electrocardiogram showed prolonged QT intervals, but the T wave morphology was atypical for type 1, 2 or 3 congenital long-QT syndrome (LQTS). Intravenous epinephrine slightly prolonged the QT interval whereas mexiletine infusion shortened the QT interval. Although these clinical characteristics might suggest type 3 LQTS, a genetic analysis identified the G604S-KCNH2 mutation (type 2 LQTS). Because mismatches between the genotype and phenotype of LQTS are possible, genetic analysis of LQTS is important to identify the most appropriate therapeutic option and risk stratification.

DOI： 10.2169/internalmedicine.51.7494

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：WILEY-BLACKWELL  

Background:
The second derivative of the photoplethysmogram (SDPTG) has been verified as a useful method for analyzing pulse wave form in adults; however, there have been few studies on the SDPTG in children.
Methods:
We examined age-related alteration of SDPTG (study 1) and the SDPTG response to a vasodilator (study 2). The subjects in study 1 were 36 healthy children aged 0.9-16.0 years. The subjects in study 2 were 13 children aged 5.4-18.9 years with a history of Kawasaki disease. Subjects received an i.v. infusion of 0.568 mg/kg dipyridamole. We assessed the SDPTG by d/a ratio, b/a ratio and aging index (AGI).
Results:
The d/a ratio increased with advance of age (r = 0.636, P &lt; 0.001), and the b/a ratio and AGI decreased with advance of age (r = -0.343, P &lt; 0.05 and r = -0.678, P &lt; 0.001, respectively). The d/a ratio and AGI were correlated with height (r = 0.523, P &lt; 0.01 and r = -0.623, P &lt; 0.001, respectively), but the b/a ratio was not significantly correlated with height. In study 2, the d/a ratio increased significantly (P &lt; 0.05), but the b/a ratio and AGI did not alter.
Conclusions:
The SDPTG indices in children show characteristic alterations with advance of age and react to a vasodilator.

DOI： 10.1111/j.1442-200X.2010.03235.x

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：JOHN WILEY & SONS INC  

Purpose: To compare magnetic resonance imaging (MRI) with transthoracic echocardiography (TTE) in the diagnosis of coronary cusp prolapse (CCP) as a complication of ventricular septal defect (VSD).
Materials and Methods: Twenty-three patients (10 males, 13 females, mean age 9 years) with clinically suspected CCP were included in a retrospective study. Long and short axis cine images and electrocardiogram (ECG)-gated axial T1-weighted image MRI images were evaluated for deformity and protrusion of right and noncoronary cusps. We compared MRI and TTE results. We compared the agreement between MRI and TTE in outlet VSD and membranous VSD.
Results: On MRI, 19 patients had right coronary cusp prolapse (RCCP), three had noncoronary cusp prolapse (NCCP), and one patient had RCCP and NCCP on MRI. Twenty-two patients had RCCP on TTE and none had NCCP. Seventeen patients had outlet defects and six patients had a membranous defect at operation. All patients with an outlet VSD had RCCP on both MRI and TTE. The agreement between MRI and TTE in outlet VSD was better than in membranous VSD.
Conclusion: MRI results corresponded with TTE results in patients with outlet VSDs, but discordant results were observed in patients with membranous VSDs.

DOI： 10.1002/jmri.22370

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Authorship：Corresponding author   Language：Japanese   Publisher：新潟医学会  

症例は1歳7ヵ月の男児。主訴は浮腫。在胎中に先天性完全房室ブロックと胎児水腫と診断され、出生日に開胸下で永久ペースメーカーを植え込まれた(VVI、リードは右室前壁)。その後順調に経過していたが、1歳7ヵ月時に浮腫を認め、当科に入院した。胸部エックス線写真で心胸郭比69%、心エコーで左室拡張末期径42.1mm(139%of normal)、左室駆出率27%、BNP901pg/mlと拡張型心筋症様であった。心電図はペーシング調律で心拍数120/分、QRS時間136msecond、左脚ブロックであった。保存的治療で改善せず、1歳8ヵ月時に開胸下に心再同期療法を施行された。術中経食道心エコーによる他のペーシング法との比較で最も有効と判断された。右室リードは既存のものを利用され、新たに心房リードを左心耳に、左室リードを心尖部側壁に装着された。心再同期療法後1年で症状はなく、胸部レントゲンで心胸郭比60%心エコーで左室拡張末期径34.1mm(109%of Normal)左室駆出率71.2%BNP16.7pg/mlと著明に改善した。先天性完全房室ブロックの右室ペーシング後の重症心不全に、心再同期療法は有効な治療と考えられた。(著者抄録)

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Other Link： http://search.jamas.or.jp/link/ui/2011125224

Language：Japanese   Publisher：(一社)日本体外循環技術医学会編集委員会  

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Language：Japanese   Publisher：日本小児高血圧研究会  

3歳女。健診で尿潜血を指摘され、近医小児科を受診した。微小血尿のみであったため経過観察されていたところ収縮期血圧が140mmHgと高値を示し、精査目的で当科に紹介された。心エコーや造影検査で第4大動脈弓の離断と遺残第5大動脈弓による大動脈縮窄が確認され、これが高血圧の原因であると判断した。治療は大動脈縮窄解除術の方針とし、現在手術待機中である。

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(一社)日本内分泌学会  

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Language：Japanese   Publisher：日本小児高血圧研究会  

小児肥満検診の受診者男子131名(年齢10.5±2.0歳)を肥満群とし、年齢をマッチさせた正常体格男子20名(年齢9.6±2.0歳)を正常群として脳性ナトリウム利尿ペプチド(BNP)を測定して肥満度、血圧および肥満関連因子との関連を検討した。年齢、身長、体重、肥満度、血圧においては肥満群が有意に高値であったが、脈拍では有意差は認めなかった。BNPは正常群14.76pg/ml、肥満群11.38pg/mlで肥満群が有意に低かったが、レプチンは有意に高く、アディポネクチンでは有意に低かった。BNPと各検査項目(肥満度、脈拍、収縮期血圧、レプチン、インスリン)との相関においては負の相関を認めた。以上よりBNPの低下が脈拍上昇を伴う収縮期高血圧の発生に影響している可能性を考えたが、さらなる関連の検討が必要であると考えられた。

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Authorship：Lead author,　Corresponding author   Language：Japanese   Publisher：(NPO)日本小児循環器学会  

新生仔ウサギのランゲンドルフ心に様々な血清を灌流させてWenckebach cycle lengthを測定し、血清が引き起こす急性の房室伝導障害を定量的に評価して様々な血清を比較した。第1の実験では、1)抗Ro、抗La抗体陽性で先天性完全房室ブロック(CHB)児の母親、2)抗Ro、抗La抗体陽性だがCHB児をもたない母親、3)抗Ro、抗La抗体陰性の自己免疫疾患患者、4)健常者の血清を比較した結果、CHB児の母親の血清だけが急性の房室伝導障害を起こし、それは不可逆的であった。一方、第2の実験ではCHB児の臍帯血清とその母親の血清を、それぞれIgG成分とそれ以外の成分に分けて比較したところ、CHB児の臍帯の血清成分が房室伝導に及ぼす影響は、母親の血清成分に比べて少なかった。また、臍帯血と母親血ともにIgG成分だけでなくIgG以外の成分でも房室伝導を障害した。すなわち、CHB児の母親の血清が特異的に房室伝導の急性障害を引き起こすことが示唆された。

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Language：Japanese   Publisher：日本小児高血圧研究会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：ELSEVIER IRELAND LTD  

The aim of this study was to assess whether platypnea and orthodeoxia occur in Fontan patients. We divided 14 Fontan patients into 2 groups: 8 patients who had pulmonary arteriovenous fistulas and/or intra-atrial shunts (group A) and 6 patients who had neither pulmonary arteriovenous fistulas nor intra-atrial shunts (group B). They were compared with 9 controls (group Q. Arterial oxygen saturation, minute ventilation per body weight and ventilatory equivalent for carbon dioxide were measured in the supine and then sitting positions. In group A, I patient had platypnea and 3 patients had orthodeoxia (changes in the saturation from the supine position to the sitting position were -4% to -7%) accompanied with slight hyperpnea, and all 4 patients had both pulmonary arteriovenous fistulas and intra-atrial shunts. Contrary, patients in group B had neither platypnea nor orthodeoxia. The saturation was significantly lower and the minute ventilation was significantly higher in the sitting position than in the supine position in group A (p&lt;0.05). The other groups showed no significant difference in the saturation or the minute ventilation between the 2 positions. All groups showed the ventilatory equivalent was significantly higher in the sitting position than in the supine position (p &lt;0.05 to 0.01). We demonstrated platypnea and orthodeoxia in Fontan patients with pulmonary arteriovenous fistulas and intra-atrial shunts. We believe platypnea and orthodeoxia should be regard as a complication in Fontan patients with pulmonary arteriovenous fistulas and/or intra-atrial shunts. (c) 2005 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.ijcard.2005.01.044

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：NATURE PUBLISHING GROUP  

The pathogenesis of congenital heart block (CHB) remains unclear. The occurrence rate of neonatal CHB is low, even in murine models of lupus erythematosus. The assessment of heart block in murine maternal lupus models by measuring atrioventricular conduction in neonatal offspring is potentially confounded by fetal wastage. We therefore sought to develop a murine CHB model with a superior immune response and to use embryonic Doppler echocardiography to observe conduction system damage in the fetus. Mature 8-wk-old female C3H/HeJ mice (n = 43) were immunized with 60 kD Ro, 48 kD La, or recombinant calreticulin autoantigens. ELISA confirmed that significant serum autoantibodies developed in all three immunized groups when compared with controls. Starting at 13 d of gestation, a significantly lower fetal heart rate (HR) and a higher percentage of fetal bradycardia/atrioventricular block (AVB, nonadvanced second degree) were observed in all immunized groups, compared with controls. There was 9-18% nonadvanced second-degree AVB in immunized groups and 0% in controls at &lt; 18 d of gestation. Neonatal electrocardiograms demonstrated only 1 degrees AVB in immunized groups. Maternal immunization with 60 kD Ro, 48 kD La, or recombinant calreticulin autoantigens resulted in AVB in a significant percentage of fetuses, however, lesser degrees of AVB were seen at birth. Significant fetal bradycardia and AVB may be missed by assessment only at birth in murine models of CHB due to fetal wastage.

DOI： 10.1203/01.PDR.0000155947.82365.E4

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Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

DOI： 10.1007/s100670200129

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Other Link： http://link.springer.com/article/10.1007/s100670200129/fulltext.html

Authorship：Lead author,　Corresponding author   Language：Japanese   Publisher：新潟市民病院  

1ヵ月男児.呼吸困難を主訴とし,上室性頻拍を疑いATPの静注,電気的除細動等を施行したが改善せず,人工呼吸管理を要しジゴキシンの投与などで徐々に改善した.標準12誘導心電図では異所性P波の先行する期外収縮や先行するP波の確認できない幅の広いQRSの頻拍を認めた.既製のリードを工夫して用いた食道誘導心電図により幅の広いQRSに先行する異所性P波を確認し,心房の異常自動能亢進による期外収縮と頻拍と診断し,更に入院時の頻拍の原因も同一の可能性が高いと考えられた.現在インデラルとメキシレチンの内服で経過順調であった

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Language：Japanese   Publisher：(株)日本小児医事出版社  

1ヵ月の女児.1ヵ月健診で体重増加不良を指摘された.数日前から呼吸困難があり,心臓超音波検査および逆行性橈骨動脈造影により,重度の大動脈縮窄症を考え,同日,緊急に大動脈弓形成術を施行した.術中所見よりA型大動脈離断と第5大動脈弓遺残と診断した.術後経過は良好である

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2002&ichushi_jid=J00643&link_issn=&doc_id=20020527170020&doc_link_id=%2Fag1snrsd%2F2002%2F005506%2F022%2F1033-1036%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fag1snrsd%2F2002%2F005506%2F022%2F1033-1036%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Mary Ann Liebert Inc.  

Hypothyroid status is believed to cause various metabolic changes in infants. However, it is interesting that even severely hypothyroid neonates, detected during mass neonatal screening, rarely show bradycardia, hypothermia, or inactivity. To study cardiac functions of screen-detected neonates with congenital hypothyroidism (CH), we recorded the electrocardiograms (ECG) of 53 screen-detected CH neonates before levothyroxine (LT4) replacement therapy, and 15 age-matched normal neonates for controls. The 53 CH neonates were divided into two groups according to initial serum thyroid hormone levels: a mildly hypothyroid group (n = 37), serum thyroid-stimulating hormone (TSH) less than 100 μU/mL and free thyroxine (FT4) 0.6 ng/dl or more
and a severely hypothyroid group (n = 16), TSH 100 μIU/mL or more and FT4 less than 0.6 ng/dL. TSH, FT4, and other blood chemicals were measured on an autoanalyzer (Hitachi 7170). After blood sampling, the ECG was recorded during induced sleep by oral administration of triclofos sodium syrup. ECG parameters, including HR, PR, QRS, QT time and corrected QT time (QTc) were automatically obtained, using an auto-ECG analyzing system. The following results were obtained. No CH patients showed abnormal ECG findings. There was no significant difference of the mean heart rates (HRs) between the mildly hypothyroid (147.5 ± 16.3 beats per minute) and the control group (148.3 ± 12.1 beats per minute). The mean HR in the severely hypothyroid group (134.0 ± 17.9 beats per minute, p = 0.007) was significantly low compared with the normal control group. However, all values were within normal ranges. QTc in the severely hypothyroid group (0.414 ± 0.015, p = 0.033) was significantly shorter than in the control group (0.440 ± 0.052). No statistical differences of PR, QRS, and QT time were noted among the three groups. All ECG parameters were within normal ranges. HR positively correlated with FT4 and log (FT4), and negatively with TSH and log (TSH). From these results we conclude that the deficiency of thyroid hormones does not affect ECG findings of congenitally hypothyroid neonates. This may be consistent with the unexpectedly mild signs and symptoms of screen-detected hypothyroid neonates.

DOI： 10.1089/10507250152484600

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Language：English   Publishing type：Research paper (scientific journal)  

Background: Children with orthostatic dysregulation (OD) appear to have hypodynamia, as well as the symptoms described in the OD criteria. Hypodynamia, which is greatly influenced by motivation, volition and concentration, is unexceptionally recognized in their everyday life. It has been suggested that the symptoms and hypodynamia aggravate considerably the quality of life (QOL) of children with OD. The purpose of this study was to distinguish the characteristics of contingent negative variation (CNV) and post imperative negative variation, which may reflect the level of attention and motivation in children with OD. Methods: Twelve patients with OD aged 10-15 years and 23 age-matched healthy children were included. The CNV was recorded from Fz, Cz and Pz linked to earlobes during 30 trials consisting of a warning stimulus and an imperative stimulus with an interstimulus interval (ISI) of 2 s and an intertrial interval (ITI) of 10 s. The imperative stimulus of each trial required a button to be pressed. Results: The untreated children with OD did not have a significantly smaller CNV amplitude than healthy children. Children with OD treated with midodrine and autonomic training had a significantly larger CNV amplitude than the untreated children, in the area of early, late and total CNV at the three sites. Conclusion: The present study confirms that children with OD have diminished motivation and deterioration of concentration, which cause hypodynamia in everyday life. Treatment for OD improves the symptoms, diminished motivation and deterioration of concentration, consequently restoring dynamia. Treatment for OD should be recommended to ameliorate QOL of children with OD.

DOI： 10.1046/j.1442-200X.2001.01439.x

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Language：English   Publishing type：Research paper (scientific journal)  

We report the successful implantation of a cardioverter defibrillator (ICD) in a 12-month-old infant. A single-lead ICD using an epicardial patch and a cathodal pulse-generator titanium shell electrode was very useful for implantation in this infant. © 2001 by The Society of Thoracic Surgeons.

DOI： 10.1016/S0003-4975(00)02589-3

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Lippincott Williams and Wilkins  

Background - There are few studies of cardiac autonomic nervous activity (CANA) in patients with congenital heart disease. Methods and Results - We evaluated CANA in 54 patients after closure of an atrial/ventricular septal defect (group A), 54 patients after successful right ventricular outflow tract reconstruction (RVOTR) (group B1), 35 RVOTR patients with residual stenosis (group B2), and 47 controls. Cardiac parasympathetic nervous activity (PSNA) was estimated by heart rate (HR) change after cholinergic blockade, HR variability, and arterial baroreflex sensitivity (BRS). Cardiac sympathetic nervous activity was estimated by the heart-to-mediastinum 123I-metaiodobenzylguanidine activity ratio (H/M) and HR increase after isoproterenol infusion (β). HR response (ΔHR) and peak oxygen uptake (Vo2) were measured by exercise test. There was no difference in β among study groups. Group A exhibited mildly impaired PSNA, which recovered 1 year after surgery, and no change in H/M. Impaired PSNA and low H/M were found in groups B1 and B2 compared with controls (P &lt
0.001), although the PSNA tended to recover 1 year after re-RVOTR. In group B1, PSNA and β were related to AHR, and BRS correlated inversely with the number of surgical procedures and age at RVOTR and positively correlated with the follow-up period, whereas ΔHR correlated with peak Vo2 (P &lt
0.01 to 0.001). Conclusions - After RVOTR, postsynaptic β-sensitivity is maintained and is important in ΔHR during exercise, as is PSNA, although ventricular sympathetic denervation is common. Impaired PSNA immediately after RVOTR improves with improved ΔHR and results in future amelioration of aerobic capacity, whereas ventricular sympathetic reinnervation is uncertain.

DOI： 10.1161/01.CIR.102.22.2732

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：NATURE PUBLISHING GROUP  

To investigate the difference in heart rate (HR) recovery after exercise between children and young adults, we administered a constant load of light exercise intensity and progressive treadmill exercise tests to nine children (aged 9 to 12 y, group A) and eight young adults (six male and two female, aged 17 to 21 y, group B) who had a history of Kawasaki disease without significant coronary arterial lesions. HR after both exercise protocols was analyzed. The low-frequency (LF) and high-frequency (HF) components of HR variability were measured, and LF/HF was calculated (log LF, log HF, log L/H). Arterial baroreflex sensitivity was assessed by the phenylephrine method. There were no differences between groups A and B in resting HR, peak HR, peak oxygen uptake, and decreases in systolic blood pressure during the recovery period. HR 1 and 2 min after peak exercise and 1 min after constant-load exercise was significantly lower in group A than in group B (p &lt; 0.05), and the changes in HR from peak values after both exercise tests were also greater in group A than in group B (p &lt; 0.05-0.01). Although no difference in arterial baroreflex sensitivity was observed, log HF was significantly higher in group A than in group B (p &lt; 0.01), and log L/H was significantly lower in group A than in group B (p &lt; 0.05). The value of log HF correlated inversely with the decrease in HR immediately after both exercise protocols (p &lt; 0.05-0.01). Although log L/H correlated with the decrease in HR after peak exercise (p &lt; 0.05-0.0005), the early decline in HR after constant-load exercise did not correlate with log L/H. Arterial baroreflex sensitivity did not correlate with the decrease in HR at any recovery time. These data suggest that the early phase of HR recovery after light to severe exercise is influenced by the cardiac parasympathetic nervous activity at rest and that the greater central cholinergic modulation of HR in children than in young adults may be responsible in part for children's faster HR recovery after exercise.

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Futura Publishing Company Inc.  

We report a 6-month-old Japanese infant with a malignant form of Brugada syndrome, who had frequent episodes of ventricular fibrillation (VF) and nonsustained polymorphic ventricular tachycardia (VT). To the best of our knowledge, this infant is the youngest patient reported to have Brugada syndrome. Continuous infusion of a β-adrenergic agonist and intravenous injection of a parasympathetic antagonist suppressed the electrical storm of polymorphic VT and VF. Combined oral administration of a β1-adrenergic agonist, a parasympathetic antagonist, and quinidine has successfully suppressed recurrences of VT or VF for 6 months, and the combination may have the potential to decrease the incidence of VT or VF as an adjunctive therapy with prophylactic placement of an implantable cardioverter defibrillator.

DOI： 10.1046/j.1540-8167.2000.01277.x

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Language：Japanese   Publisher：日本小児腎不全学会  

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Language：Japanese   Publisher：新潟県立病院医学会  

2歳小児ドナーから8歳小児への献腎移植を行い良好な経過を得た.強力かつ厳密な初期の免疫抑制を行ったこと,積極的に血栓形成予防治療をしたこと,移植腎の状態が良好で,体格的には大差なく移植血管のサイズが同等で,腎のサイズも相応でありより生理的であったことなどが好結果をもたらした

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Language：Japanese   Publisher：(株)日本小児医事出版社  

尿路感染症と診断された乳児18例に画像検査を施行した.全例が初発尿路感染症であったが,18例中5例に腎尿路奇形を認め,3例に手術適応があった.しかし,腎エコー検査では,腎尿路奇形5例中2例,手術適応3例中1例は異常を認めなかった.腎尿路奇形と腎瘢痕の検索は,初回尿路感染症乳児全てを対象とすべきであり,また,腎エコー検査のみでは不十分と考えられた

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Authorship：Lead author   Language：Japanese   Publisher：(株)東京医学社  

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溶連菌感染後急性糸球体腎炎においてもPodocyte障害が認められ,多数のPodocyteが基底膜より剥離すれば管外性病変が形成される

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Language：Japanese   Publisher：日本小児腎不全学会  

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Language：Japanese   Publisher：金原出版(株)  

3歳7ヵ月男児.アミノフィリン使用時にはエチレンジアミン過敏症の存在を念頭に置くべきである.アミノフィリン中のエチレンジアミン過敏症には白血球遊走阻止試験が有効と考えられた

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12歳の若年性関節リウマチ,全身型の女児でスリンダク投与を引き金としたDICを経験した.スリンダクを中止,プレドニゾロンを増量し,更に,ヘパリン,メシル酸ガベキサート,AT IIIを投与しDICは軽快した.経過中,出血症状,臓器症状は認めなかった.スリンダクに対する白血球遊走試験が陽性であり,JRAを基礎に,スリンダクに対する薬剤アレルギーが引き金となってDICが起こったと考えられた

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肥満小児を対象にNT-proBNPとBNPと肥満の代謝異常の指標との関連を検討した。肥満小児108例を対象に、肥満度、腹囲、血圧、脈拍、体脂肪率、腹部エコーによる最大腹壁前脂肪厚(Pmax)および最小皮下脂肪厚(Smin)を測定した。男子+58.6%、女子+55.8%であり、高度肥満小児が多かった。BNP、NT-proBNPは男子が女子に比し高値傾向であった。体脂肪率、インスリン、HOMA-Rは女子が男子に比し高値であった。BNP(log変換)、NT-proBNP(log変換)と各因子との性別で補正した回帰分析で、BNPは、腹囲、Pmax、インスリン、HOMA-R、アルドステロンと負の相関、Totalアディポネクチンと正の相関があった。NT-proBNPは、Totalアディポネクチンと負の相関がみられた。

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