We report two unique cases of persistent fifth aortic arch with a systemic-to-pulmonary connection. All previously reported cases with such a connection in the literature have either been cases of pulmonary atresia or an aortic arch anomaly, and the existence of a fifth aortic arch was a benefit to the underlying great vessel anomaly. However, our two cases did not have this associated great vessel anomaly, and the fifth arch resulted in a large left-to-right shunt with severe pulmonary hypertension and heart failure. The first case was misdiagnosed preoperatively; an accurate diagnosis was made after cardiac surgery. Because of its rerity and complexity, a persistent fifth aortic arch is often ignored and misdiagnosed.

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A persistent fifth aortic arch was recognized in a 4-yearold boy by echocardiography, magnetic resonance imaging and angiocardiography. The case was unique in at least two respects: right-sided aortic arch, and systemic-topulmonary connection without intracardiac lesion. This rare type of anomaly was correctly diagnosed and successfully repaired by patch closure of the arch. This is the first report of this combination of lesions in the Orient.

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症例は1歳7カ月女児．在胎36週6日，体重1,351 gで出生．Cornelia de Lange症候群・Fallot四徴症の診断に加え，大動脈弓小弯側から主肺動脈へのシャント血流が確認され，異所性動脈管開存と診断．頭部超音波検査で前大脳動脈の拡張期血流の途絶と逆行性血流を呈することから，異所性動脈管による盗血現象と診断し，日齢25で外科治療介入．手術は胸骨正中切開でアプローチし，シャント血管を結紮すると，経皮的酸素飽和度が90%台後半から70%台前半へと低下するため，いったん結紮を解除．80%台後半を維持できるように絞扼し，手術を終了した．術後頭部超音波検査で前大脳動脈圧波形改善を認めた．外来観察中に体重増加に伴う酸素需要増大を認め，1歳7カ月に1弁付きパッチを用いた心内修復術を施行した．希少な背景を有するファロー四徴症に合併した異所性動脈管開存に対し，動脈管絞扼を先行し心内修復術に達した1例を経験したので報告する．

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症例は1ヵ月,男児.肺動脈閉鎖をともなったファロー四徴症,総肺静脈還流異常,右側大動脈弓,動脈管開存,pulmonary artery sling(PA sling)の診断で手術を行った.胸骨正中切開により体外循環下,左肺動脈を右肺動脈より離断し気管前面で肺動脈幹に吻合しPA slingを解除した.ついで左総頸動脈より肺動脈幹までのcentral shunt(3.5mm PTFE graft)を吻合した.術中所見より動脈管と診断した血管は正常動脈壁の構造をみとめ第5大動脈弓遺残と診断した.このため術前の3D-CT所見と発生学的な検討から左鎖骨下動脈が左第4大動脈弓より発生するきわめて希な大動脈弓分枝と考えられた.Arch anomalyの診断では形態の正確な把握と発生学的な見地からの検討が必要である.

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第5大動脈弓は稀な血管異常であり, 無症状であることが多い. 第4大動脈弓離断を合併した第5大動脈弓縮窄に対する手術を2例経験した. 1例目は生後9日(女児)で動脈管閉鎖によるショックで発症し, 同日緊急手術を行った. 再建は拡大大動脈弓吻合法で第4大動脈弓と下行大動脈の直接吻合を行った. 2例目は2歳1カ月の女児で, 心雑音で当院へ紹介された. 第4大動脈弓と第5大動脈弓を側々吻合して, そこへ下行大動脈を吻合した. 術後それぞれ1年11カ月および2年6カ月が経過し, 2例ともに吻合部狭窄を認めず経過良好である.

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Persistent fifth aortic arch (PFAA) associated with interruption of the fourth aortic arch is a rare malformation, usually requiring surgical intervention in childhood. A 51-year-old woman developed a distal aortic arch aneurysm associated with PFAA after patch aortoplasty for coarctation of the aorta at the age of 18. To our knowledge, this is the first reported case of a distal aortic arch aneurysm associated with PFAA. Residual tissues of the ductus and PFAA, which is not a definitive aortic arch, and the implanted synthetic patch may have been related to the development of the aneurysm. Graft interposition between the proximal border of PFAA and the descending aorta was performed because severe adhesions impeded the application of the fourth aortic arch as a proximal anastomotic site of the graft. Accurate diagnosis and sufficient understanding of PFAA allow an appropriate surgical treatment in childhood with a good result over the long term.

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This paper reports a toe speed control approach to achieving complex gaits with the Jansen linkage mechanism. In order to achieve complex gaits, delicate control of the toe is required. Since, the Jansen linkage mechanism is a closed loop linkage mechanism, the trajectory of the toe is defined uniquely by the set of link lengths. Hence, by controlling the toe speed, the locomotion of the toe can be controlled arbitrarily in response to intended purposes of its gait pattern. In this paper, we proved that the norm of the toe speed bears a proportionate relationship to the angular velocity of the driving link in a Jansen mechanism based robot platform. Using this relationship as basis, we derived the angular trajectory that results in a constant toe speed in the robot platform. Numerical simulations were performed to demonstrate the efficacy and validity of the proposed approach.

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Intravascular papillary endothelial hyperplasia (IPEH) is a benign non-neoplastic lesion histopathologically characterized by papillary proliferation of endothelial cells in dilated vascular lumen, and occurrence in the oral region is relatively rare. In this study, we performed clinical, histopathological, and immunohistochemical evaluations in 13 cases of oral IPEH, and examined the pathogenetic mechanism, pathophysiology, and differentiation from other vascular anomalies. The upper lip was the most common lesion site (5 cases), followed by the lower lip (3 cases) and the tongue (2 cases). The most common clinical diagnosis was hemangioma in 7 cases, followed by benign tumor in 4 cases. On gross examination, the tumor was dark red or mucosal color, similar to a hemangioma, and the surface was smooth, elastic and soft. Histological classification was mixed form in 11 cases and pure form in 2 cases. In immunohistochemical staining, CD31 was expressed in the cytoplasm and on cell membrane of vascular endothelial cells in all 13 cases; ERG was expressed in the nuclei of vascular endothelial cells in all 13 cases; α-SMA was expressed on cell membrane of smooth muscle cells in the blood vessel wall in all 13 cases; CD105 was expressed in the cytoplasm of vascular endothelial cells in 10 of 13 cases; and VEGF-A was expressed in the cytoplasm of vascular endothelial cells in all 13 cases. D2-40 was expressed in the cytoplasm and on cell membrane of endothelial cells within the papillary structures in only 2 cases, but Glut-1 expression was not detected in any of the cases. IPEH was easily differentiated from angiosarcoma, Kaposi’s sarcoma, mucous cyst, and pyogenic granuloma. The pathogenetic mechanism of this disease suggested by this study is that thrombus formation induces a hypoxia and low glucose state at the lesion site, and VEGF produced by endothelial cells stimulates reactive proliferation of the endothelial cells to form IPEH.

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症例は40歳代女性．風俗店勤務．外陰部腫瘤，潰瘍，体幹部丘疹，手掌足底に紅斑を認め梅毒を疑った．梅毒反応陽性，胆道系優位の肝胆道系酵素上昇を認めた．腹部超音波検査で門脈域に高エコー像を認めた．造影CTでは動脈相で肝内胆管の一部拡張と壁肥厚，肝実質に不均一な造影所見を認め，以上より胆管炎も疑われた．MRIではT1強調画像opposed-phaseで門脈域周囲低信号域を認め，MRCPで胆管の枯れ枝状変化を認めた．肝生検ではグリソン鞘と周囲組織にリンパ球浸潤と線維化を認め実質に脂肪変性を認めた．AMPC内服を開始し梅毒反応は陰性化し，肝胆道系酵素は約11カ月の経過で低下し画像所見も改善を認めた．早期梅毒肝炎の過去報告例では画像上胆道系異常を指摘した報告は少ない．この所見は胆道系優位の肝障害という早期梅毒性肝炎の特徴とも合致し，特徴的な画像所見を呈した1例として報告する．

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　近年、臨床研究における研究倫理審査の受審・承認に関する虚偽記載をめぐる問題事案が国内外において少なからず散見されるようになってきている。しかし、報告される事案の数自体が少ないことに加えて、主たる「研究活動の不正行為」とされる捏造、改竄、盗用の陰に隠されてきたことから、本問題の倫理的な深刻さや何がそもそも問題であるかということについて、研究公正を含む研究倫理学の中でもほとんど検討されていない。そこで本論では、過去十年余りの間に国際的及び国内的に問題となった、研究倫理審査の受審・承認に関する虚偽記載が確認されたいくつかの臨床研究事案を取り上げつつ、本問題について研究倫理学的観点から考察した。

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The study was aimed to find out the prevalence of non-classical congenital adrenal hyperplasia (NC-CAH) due to 21-hydroxylase deficiency (21-OHdef) among Greek women with hirsutism and polycystic ovary syndrome (PCOS) and to compare the results of ACTH stimulated 17-hydroxyprogesterone 60 min (17-OHP60) values, with human leukocyte antigens (HLA) phenotypes, in any patient diagnosed as having NC-CAH. One hundred and seven women with hirsutism and PCOS were included in the study. All were presented at the Reproductive Endocrinology Outpatient Clinic with hirsutism and PCOS. After ACTH stimulation test, 10 women were diagnosed as having NC-CAH because of high 17-OHP60 values ≥36 nmol/l, and 97 as having PCOS. Ten (10.3%) of the 97 women presented hormonal findings compatible with adrenal hyper-response due to ACTH testing, because of hyperstimulated 17-OHP60 values ≥21 nmol/l and <32 nmol/l. The HLA typing of 10 patients with NC-CAH revealed the phenotypes B14, DR1, B35, B7 and B44 which present positively genetic linkage disequilibrium with 21-OHdef, as reported in the literature. In conclusion: In Greek women with hirsutism and PCOS we have found that: a. The prevalence of NC-CAH among these women is relatively high and reaches at 10%. b. The HLA phenotypes B₁₄, DR₁, B₃₅, B₇ and B44 were found in high frequency in these NC-CAH patients. c. Adrenal NC-CAH due to 21-OHdef as well as adrenal hyperactivity, revealed after ACTH testing, constitutes an important reason of hirsutism and PCOS in these Greek women and both reach a rate of 20%.

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An 80-year-old man was admitted to our hospital with a diagnosis of primary hepatic actinomycosis determined based on a percutaneous aspiration biopsy. The abscesses and state of liquefaction were easily and effectively visualized on contrast-enhanced ultrasonography. Ampicillin/sulbactam was administered; however, lesions of hepatic actinomycosis suddenly infiltrated the diaphragm and right thorax six months later. A drainage tube was inserted into the right thoracic space, and the pleural effusion gradually decreased. The patient received continuous antibiotic therapy for nearly two years and remained free of hepatic actinomycosis on follow-up more than one year later.

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Background:The prognostic factors in Japanese patients with wild-type transthyretin amyloidosis (ATTRwt) have not been elucidated.

Methods and Results:In this study we retrospectively analyzed the clinical characteristics and outcomes of 47 patients with ATTRwt (mean (±SD) age at diagnosis 80.3±4.6 years; 41 males). Fifteen patients died within 2 years of their diagnosis. Receiver operating characteristic and Kaplan-Meier analyses revealed that the best predictors of 2-year mortality were low serum albumin (≤3.75 g/dL), elevated high-sensitivity cardiac troponin T (hs-cTnT; >0.086 ng/mL), and reduced left ventricular ejection fraction (LVEF; <50%). According to the total number of these 3 risk factors, patients were stratified into 4 subgroups: low risk (no risk factors; n=15), intermediate-low risk (1 risk factor; n=15), intermediate-high risk (2 risk factors; n=7), and high risk (3 risk factors; n=10). The estimated 2-year survival rate of patients classified as low risk, intermediate-low risk, intermediate-high risk, and high risk was 93%, 80%, 83%, and 11%, respectively (P<0.001).

Conclusions:Low serum albumin, elevated hs-cTnT, and reduced LVEF are associated with a worse prognosis in Japanese patients with ATTRwt. The combination of these factors may be useful for predicting medium-term mortality in patients with ATTRwt.

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