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Liver cirrhosis in a child associated with Castleman's disease: A case report

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World Journal of Clinical Cases 2020年第9期8卷 1656-1665页

作者： Soya Kobayashi Ayano Inui Tomoyuki Tsunoda Syuichiro Umetsu Tsuyoshi Sogo Masaaki Mori Masato Shinkai tomoo fujisawa Department of Pediatric Hepatology and Gastroenterology Saiseikai Yokohama City Tobu HospitalYokohama 230-8765Japan Department of Lifetime Clinical Immunology Graduate School of Medical and Dental SciencesTokyo Medical and Dental UniversityTokyo 113-8519Japan Department of Surgery Kanagawa Children’s Medical CenterYokohama 232-8555Japan

BACKGROUND Castleman's disease(CD)is a lymphoproliferative *** syndrome is classified as a variant of CD based on its key clinical manifestations of thrombocytopenia,anasarca(generalized edema and pleural effusion),fever(pyrexia),reticulin fibrosis in the bone marrow and the proliferation of megakaryocytes,and organomegaly(such as hepatosplenomegaly and multiple lymphadenopathies);TAFRO syndrome is mainly reported in Japanese *** our knowledge,this is the first pediatric case report detailing a CD-associated disorder progressing to *** SUMMARY A 10-year old male patient presented with fever and *** months before hospitalization,he had remarkable abdominal ***,he visited a clinic for a fever that lasted 5 *** physical findings were marked hepatosplenomegaly and cervical lymphadenopathy.A blood test revealed leukocytosis,microcytic anemia,aspartate aminotransferase-dominant transaminase elevation,high levels of C-reactive protein,polyclonal hypergammaglobulinemia,and high levels of interleukin-6 and vascular endothelial growth *** contrast computed tomography and magnetic resonance imaging suggested cirrhosis,which was confirmed by liver *** findings in the enlarged hepatic lymph nodes revealed both hyperplasia and atrophy of lymphoid follicles with some vascular hyperplasia and moderate plasmacytosis between the lymphoid follicles,which is compatible with lymph node histology in TAFRO *** was not effective in reducing the patient’s symptoms;therefore,the patient was prescribed *** date,the patient remains free of fever and continues to receive *** We described the clinicopathological features of TAFRO syndrome to highlight the clinical presentation of this rare disease in a pediatric case.

关键词： Castleman disease Case report Multi-centric Castleman’s disease Liver cirrhosis Tocilizumab Child

Alanine Aminotransferase as the First Test Parameter for Wilson's Disease

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Journal of Clinical and Translational Hepatology 2019年第4期7卷 293-296页

作者： Hisao Hayashi Kazumasa Watanabe Ayano Inui Ayako Kato Yasuaki Tatsumi Akihiko Okumura tomoo fujisawa Koichi Kato Department of Medicine Aichi Gakuin University School of PharmacyChikusa-kuNagoyaJapan Department of Gastroenterology Kainan HospitalYatomiJapan Department of Pediatric Gastroenterology and Hepatology Saiseikai Yokohama Toub HospitalYokohamaKanagawaJapan

Background and Aims: The liver is the first organ affected by toxic copper in the classical and severe hepatic forms of Wilson's disease(WD).Because their associated chronic liver damage is mostly asymptomatic,an intervention using a special test including serum alanine aminotransferase(ALT)activity is needed for detecting ***: Using the modified international criteria for the diagnosis of WD,45 patients were selected from the collective databases of our institutions,and 7 infants were reviewed from the *** patients had the severe hepatic form,with nor-moceruloplasminemia and no mutations in *** rapid ALT change during hemolytic anemia was adjusted for a *** diagnostic potential of the ALT test was assessed from the age-dependent natural course of the liver damage of ***: The natural course had three *** were still low in some infants younger than 4 *** were high in all children between the ages of 4 and 8 years-old;then,they reduced to low levels in some patients over 9 years of *** high ALT stage represents chronic active hepatitis,and the sub-sequent low ALT stage is due to silent *** hepatic cop-per content is a reliable but invasive test,while urinary copper secretion is an alternative,non-invasive test for copper toxicosis of *** serum ceruloplasmin and ATP7B analyses are sub-type tests of *** response to anti-copper regimens is the final test ***: ALTcould be the first parameter to test to detect WD in children between the ages of 4 and 8 years.

关键词： Alanine aminotransferase ATP7B Ceruloplasmin Chronic active hepatitis Wilson's disease

Safety and utility of capsule endoscopy for infants and young children

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World Journal of Gastroenterology 2013年第45期19卷 8342-8348页

作者： Manari Oikawa-Kawamoto Tsuyoshi Sogo Takeshi Yamaguchi Tomoyuki Tsunoda Takeo Kondo Haruki Komatsu Ayano Inui tomoo fujisawa Department of Pediatric Hepatology and Gastroenterology and Endoscopy Center Saiseikai Yokohama City Tobu Hospital Yokohama Kanagawa 230-0012 Japan Department of Pediatric Hepatology and Gastroenterology Saiseikai Yokohama City Tobu Hospital Yokohama Kanagawa 230-0012 Japan

AIM:To assess the safety and utility of capsule endoscopy(CE)for children who are unable to swallow the capsule ***:The medical records of all of the children who underwent CE between 2010 and 2012 were retrospectively *** patients were divided into 2groups:group A included patients who were unable to swallow the capsule endoscope,and group B included patients who were able to swallow *** the patients who were unable to swallow the capsule endoscope,it was placed in the duodenum *** small bowel transit time,endoscopic diagnosis and complications of the 2 groups were ***:During the study period,28 CE procedures were performed in 26 *** A included 11patients with a median age of 2 years(range 10 mo-9years),and group B included 15 patients with a median age of 12 years(range 8 years-16 years).The lightest child in the study weighed 7.9 *** detection rates did not differ between the 2 *** median small bowel transit time was 401 min(range 264-734 min)in group A and 227 min(range 56-512 min)in group B(P=0.0078).No serious complications,including capsule retention,*** significant mucosal trauma occurred in the pharynx,esophagus,stomach or duodenum when the capsule was introduced using an ***:CE is a safe and useful procedure for infants and young children who are unable to swallow the capsule endoscope.

关键词： Capsule endoscopy Retention Infants Children Small bowel transit time Complications

First cases of MPV17 related mitochondrial DNA depletion syndrome with compound heterozygous mutations in p.R50Q/p.R50W:a case report

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Hepatoma Research 2020年第1期6卷 1-10页

作者： Shuichiro Umetsu Ayano Inui Sohya Kobayashi Masaru Shimura Tomoko Uehara Hajime Uchida Rie Irie Tsuyoshi Sogo Haruki Komatsu Takako Yoshioka Kei Murayama Kenjiro Kosaki MureoKasahara tomoo fujisawa Department of Pediatric Hepatology and Gastroenterology Saiseikai Yokohama City Tobu HospitalKanagawa 230-0012Japan Department of Metabolism Chiba Children’s HospitalChiba 266-0007Japan Center for Medical Genetics Keio University School of MedicineTokyo 160-8582Japan Department of Transplantation Surgery National Center for Child Health and DevelopmentTokyo 157-8535Japan Department of Pathology Keio University School of MedicineTokyo 160-8582Japan Department of Pediatrics Toho University Sakura Medical CenterChiba 285-8741Japan Department of Pathology National Center for Child Health and DevelopmentTokyo 157-8535Japan

Mutations in MPV17 lead to severe mitochondrial DNA depletion syndrome(MTDPS).All known p.R50W variants in MPV17 are *** homozygous variant p.R50Q in MPV17 among patients with Navajo neurohepatopathy is known to allow longer survival,although heterozygous variants p.R50Q have not been *** is the first clinical report in compound heterozygosity MPV17 mutation(p.R50W/p.R50Q).Three siblings were admitted due to multiple hepatic nodules;none presented neurological ***,they suffered from severe hypoglycemia and cyclic *** diagnosis of MPV17-related MTDPS was confirmed by detection of a compound heterozygous MPV17 mutation(p.R50W/p.R50Q),and striking reduction of hepatic mitochondrial *** patient developed pediatric-onset of hepatocellular ***,all patients survived for extended periods,including two patients who received liver transplantation,which contrasted the high mortality rate associated with p.R50W mutations,as previously *** p.R50Q mutation might be associated with longer survival and improved liver transplantation outcomes.

关键词： Mitochondrial DNA depletion syndrome MPV17 compound heterozygous mutation liver transplantation

Intestinal ascariasis at pediatric emergency room in a developed country

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World Journal of Gastroenterology 2014年第38期20卷 14058-14062页

作者： Shuichiro Umetsu Tsuyoshi Sogo Kentaro Iwasawa Takeo Kondo Tomoyuki Tsunoda Manari Oikawa-Kawamoto Haruki Komatsu Ayano Inui tomoo fujisawa Department of Pediatric Hepatology and Gastroenterology Saiseikai Yokohama City Tobu Hospital Department of Pediatrics Toho University Medical CenterSakura Hospital

Ascaris lumbricoides infection is rare among children in developed *** large numbers of adult Ascaris in the small intestine can cause various abdominal symptoms,this infection remains asymptomatic until the number of worms in the intestine considerably increases in most *** causing bilious vomiting suggesting ileus is rare,especially in developed countries.A 6-year-old boy who lived in Japan,presented with abdominal colic,bilious vomiting at the pediatric emergency *** appeared pale,and had no abdominal distention,tenderness,palpable abdominal mass,or findings of *** experienced bilious vomiting again during a physical *** tests showed mild elevation of white blood cells and C-reactive protein *** of adenovirus,rotavirus,and norovirus were not detected from his stool,and stool culture showed normal *** showed multiple,round-shaped structures within the small intestine,and a tubular structure in a longitudinal scan of the small *** endoscopy showed a moving worm of Ascaris in the *** ascariasis should be considered as a cause of bilious vomiting in children,even at the emergency room in industrial *** examination and capsule endoscopy are useful for diagnosis of pediatric intestinal ascariasis.

关键词： Ascaris lumbricoides Paralytic ileus Cap-sule endo

Behavior of Vaneless Diffuser Stall in a Centrifugal Compressor

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Journal of Thermal Science 2022年第1期31卷 3-12页

作者： AGARI Yuki YAMAO Yoshifumi fujisawa Nobumichi OHTA Yutaka Department of Applied Mechanics and Aerospace Engineering Waseda UniversityTokyo 169-8555Japan

The rotating stall in a centrifugal compressor with a vaneless diffuser was investigated both experimentally and numerically with focus on the effect of the internal flow field within the impeller on the diffuser *** numerical analysis,the boundary layer separation at the impeller outlet was found to play an important role in the expansion and rotation processes of the diffuser *** particular,the expanded boundary layer separation near the hub side at the outlet of the main blade(M.B.)suction surface passage was considered to be the main cause of the expansion and rotation processes.A longitudinal vortex existed at the throat of the ***,and the mass flow rate in the *** was significantly reduced by the blockage *** addition,the longitudinal vortex induced the rolling up flow near the hub side at the impeller ***,the boundary layer separation expanded.

关键词： centrifugal compressor vaneless diffuser pressure and velocity measurement CFD rotating stall

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十字路口上的日本动产担保法

苏州大学学报（法学版） 2024年第1期11卷 149-160页

作者： [日]藤泽治奈徐洁燕颖川(译) 立教大学法学部西南政法大学民商法学院

日本动产担保法的修改正面临着艰难的抉择。担保的法律形式的一元主义和多元主义的选择以及动产担保标的物范围的规定最为棘手。关于法律形式,无论是以美国为代表的一元主义,还是以法国为代表的多元主义实际都不是绝对的,日本担保法制... 详细信息

日本动产担保法的修改正面临着艰难的抉择。担保的法律形式的一元主义和多元主义的选择以及动产担保标的物范围的规定最为棘手。关于法律形式,无论是以美国为代表的一元主义,还是以法国为代表的多元主义实际都不是绝对的,日本担保法制部会的两种法案也不是对立的。所以,或许在法律形式上采用多元主义,而对任何形式的担保交易都采用同样的规则是实现实质性的一元主义的最好路径。关于担保标的物范围的扩张,其核心问题是,集合动产概念扩张的范围,如何处理因缓和集合动产的特定性要件而产生的一系列法律问题,以及企业营业资产成为担保客体的问题。同时,还要考虑一般实体法与破产法关于动产担保制度的协调。

关键词：动产担保法担保的法律形式担保标的物的范围

Geometric-phase-induced arbitrary polarization and orbital angular momentum generation in helically twisted birefringent photonic crystal fiber

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Photonics Research 2020年第8期8卷 1278-1288页

作者： TAKESHI fujisawa KUNIMASA SAITOH Graduate School of Information Science and Technology Hokkaido UniversityJapan

The evolutions of polarization and orbital angular momentum(OAM)states of light in helically twisted birefringent photonic crystal fibers(TB-PCFs)are *** is shown that a circular polarization(CP)component(S3 of a Stokes parameter)is periodically excited when usual linearly polarized(LP)modes of PCF are *** excitation originates from a geometric phase in *** S3 excitation is larger for larger linear birefringence for a fixed twisting *** the linear birefringence is large enough,a CP filtering behavior can be seen in addition to the S3 *** the analytical consideration of the sign of the geometric phase,the TB-PCF with periodical inversion of twisting is proposed to generate arbitrary polarization state on the Poincaré***,an OAM state generation in multimode TB-PCFs is shown for higher-order LP mode *** observing a far-field interference pattern from TB-PCF mixed with LP01 mode,a vortex associated with the OAM state can be *** to the single-mode case,by using periodical twisting inversion,efficient OAM generation is *** results indicate that by simply launching fiber’s LP mode into TB-PCF,arbitrary polarization and OAM states can be generated,leading to a novel mechanism for the manipulation of the spatial state of light.

关键词： polarization fiber momentum

Unsteady Behavior and Control of Vortices in Centrifugal Compressor

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Journal of Thermal Science 2014年第5期23卷 401-411页

作者： Yutaka Ohta Nobumichi fujisawa Department of Applied Mechanics and Aerospace Engineering Waseda University3-4-1OkuboShinjukuTokyo169-8555Japan Graduate School of Fundamental Science and Engineering Waseda University3-4-1OkuboShinjukuTokyo169-8555Japan

Two examples of the use of vortex control to reduce noise and enhance the stable operating range of a centrifugal compressor are presented in this *** the case of high-flow operation of a centrifugal compressor with a vaned diffuser,a discrete frequency noise induced by interaction between the impeller-discharge flow and the diffuser vane,which appears most notably in the power spectra of the radiated noise,can be reduced using a tapered diffuser vane(TDV) without affecting the performance of the *** longitudinal vortices produced by leakage flow passing through the tapered portion of the diffuser vane induce secondary flow in the direction of the blade surface and prevent flow separation from the leading edge of the *** use of a TDV can effectively reduce both the discrete frequency noise generated by the interaction between the impeller-discharge flow and the diffuser surface and the broadband turbulent noise *** the case of low-flow operation,a leading-edge vortex(LEV) that forms on the shroud side of the suction surface near the leading edge of the diffuser increases significantly in size and blocks flow in the diffuser *** formation of an LEV may adversely affect the performance of the compressor and may cause the diffuser to *** a one-side tapered diffuser vane to suppress the evolution of an LEV,the stable operating range of the compressor can be increased by more than 12 percent,and the pressure-rise characteristics of the compressor can be *** results of a supplementary examination of the structure and unsteady behavior of LEVs,conducted by means of detailed numerical simulations,are also presented.

关键词：压缩机控制不稳定离心式行为涡流叶片表面扩散器离散频率