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A Revised Classification of Primary Iron Overload Syndromes

Journal of Clinical and Translational Hepatology 2024年第4期12卷 346-356页

作者： yasuaki tatsumi Motoyoshi Yano Shinya Wakusawa Hiroaki Miyajima Tetsuya Ishikawa Shinsaku Imashuku Atsuko Takano Wataru Nihei Ayako Kato Koichi Kato Hisao Hayashi Kentaro Yoshioka Kazuhiko Hayashi Department of Medical Biochemistry Faculty of Pharmaceutical SciencesToho UniversityFunabashiJapan Department of Gastroenterology Yokkaichi Municipal HospitalYokkaichiJapan Department of Medical Technology Shubun UniversityIchinomiyaJapan Department of Medicine and Neurology Tenryu Kohseikai ClinicHamamatsuJapan Department of Gastroenterology and Hepatology Nagoya University Graduate School of MedicineNagoyaJapan Department of Laboratory Medicine Uji-Tokushukai Medical CenterUjiJapan Department of Medicine Saiseikai Takaoka HospitalTakaokaJapan Department of Medicine Aichi-Gakuin University School of PharmacyNagoyaJapan Department of Gastroenterology FNPS Meijo HospitalNagoyaJapan

Background and Aims:The clinical introduction of hepcidin25(Hep25)has led to a more detailed understanding of its relationship with ferroportin(FP)and divalent metal transporter1 in primary iron overload syndromes(PIOSs).In 2012,we proposed a classification of PIOSs based on the Hep25/FP system,which consists of prehepatic aceruloplasminemia,hepatic hemochromatosis(HC),and posthepatic FP disease(FP-D).However,in consideration of accumulated evidence on PIOSs,we aimed to renew the ***:We reviewed the 2012 classification and retrospectively renewed it according to new information on ***:Iron-loading anemia was included in PIOSs as a prehepatic form because of the newly discovered erythroferrone-induced suppression of Hep25,and the state of traditional FP-D was remodeled as the BIOIRON *** key molecules responsible for prehepatic PIOSs are low transferrin saturation in aceruloplasminemia and increased erythroferrone production by erythroblasts in iron-loading *** PIOSs comprise four genotypes of HC,in each of which the synthesis of Hep25 is inappropriately reduced in the *** Hep25 synthesis is adequate in posthepatic PIOSs;however,two mutant FP molecules may resist Hep25 differently,resulting in SLC40A1-HC and FP-D,*** phenotypes are diagnosed using laboratory tests,including circulating Hep25,followed by suitable *** sequencing of the candidate genes may be outsourced to gene centers when *** kits for the prevalent mutations,such as C282Y,may be the first choice for a genetic analysis of HC in ***:The revised classification may be useful worldwide.

关键词： Ethnicity Ferroportin Hemochromatosis Hepcidin25 Iron overload syndrome

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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

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Phenotypes and Chronic Organ Damage May Be Different among Siblings with Wilson's Disease

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Journal of Clinical and Translational Hepatology 2017年第1期5卷 27-30页

作者： Shinsuke Yahata Seitetsu Yung Mari Mandai Takakazu Nagahara Daisaku Kuzume Hiroshi Sakaeda Shinya Wakusawa Ayako Kato yasuaki tatsumi Koichi Kato Hisao Hayashi Ryohei Isaji Yoji Sasaki Motoyoshi Yano Kazuhiko Hayashi Masatoshi Ishigami Hidemi Goto Division of Community Medicine and Career Development Kobe University Graduate School of Medicine2-1-5Arata-choHyogo-kuKobe 652-0032Japan Department of Gastroenterology Hyogo Prefectural Kakogawa Medical CenterKakogawaHyogoJapan Department of Gastroenterology and Hepatology Tottori Prefectural Kousei HospitalKurayoshiTottoriJapan Departments of Neurology and Gastroenterology Chikamori HospitalKochiJapan Medical Laboratory Sciences Nagoya University Graduate School of MedicineHigashi-kuNagoyaJapan Department of Medicine Aichi Gakuin University School of PharmacyChikusa-kuNagoyaJapan Department of Gastroenterology Konan Kosei HospitalTakaya-choKonanAichiJapan Department of Gastroenterology Yokkaichi Municipal HospitalYokkaichiMieJapan Department of Gastroenterology and Hepatology Nagoya University Graduate School of MedicineShowa-kuNagoyaJapan

Background and Aims:Cloning of ATP7B provided evidence that Wilson's disease is a hepatic copper toxicosis with a variety of extrahepatic *** siblings with the same genetic background and exposure to similar environmental factors may be a good model for the study of genotype-phenotype ***:Twenty-three affected siblings in 11 families were selected from a *** first phenotypes were determined according to the international *** final types of chronic organ damage were re-evaluated for life-long ***:Phenotypes were identical in 5 of the families and different in 6 of the *** acute hepatic phenotype H1 was found in 3 younger siblings and 1 older *** survived an acute episode of hemolysis with underlying chronic liver *** also presented complication with neurological *** neurological phenotype N1 with neuropsychiatric symptoms and hepatic disease was found in 2 aged siblings of 1 family,in an older sibling in 3 families and in the oldest sibling in 1 *** in siblings were mainly split by either H1 occurring in random order or age-dependent *** of chronic organ damage were identical in 8 of the families and different in 3 of the *** same combination of chronic liver disease was found in 6 families and chronic liver disease complicated with neurological disease in 2 *** organ damage in siblings was found when an older sibling was complicated by neurological *** was no reverse combination of a younger sibling being complicated by neurological disease in any of the ***:Phenotype combinations of siblings were mainly modified by externally-induced hemolytic episodes,while chronic organ damage in siblings was split by age-dependent neurological complications.

关键词： Hemolysis Liver disease Neurological disease Wilson's disease

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Acute Hepatic Phenotype of Wilson Disease: Clinical Features of Acute Episodes and Chronic Lesions Remaining in Survivors

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Journal of Clinical and Translational Hepatology 2015年第4期3卷 239-245页

作者： Hisao Hayashi yasuaki tatsumi Shinsuke Yahata Hiroki Havashi Kenji Nomose Ryohei Isaji Youji Sasaki Department of Medicine Aichi-Gakuin University School of PharmacyNagoyaJapan Department of Gastroenterology Hyogo Prefectural Kakogawa Medical CenterKakogawaJapan Department of Gastroenterology Kita-Harima Medical CenterOnoJapan Department of Gastroenterology Kobe University School of MedicineKobeJapan Department of Gastroenterology Konan Kosei HospitalKonanJapan

Background and Aims:Wilson disease(WD)is an inherited disorder of copper metabolism,and an international group for the study of WD(IGSW)has proposed three phenotypes for its initial presentation:acute hepatic,chronic hepatic,and neurologic *** of the acute hepatic phenotype may improve our understanding of the ***:Clinical features of 10 WD patients with the acute hepatic phenotype and characteristics of chronic lesions remaining in survivors were assessed by the European Association for the Study of the Liver(EASL)***:All six patients younger than 30 years had survived an acute episode of hemolytic anemia with residual liver disease of cirrhosis or chronic *** acute episode was self-limiting in two of the four patients over the age of 30 years and progressed to acute liver failure in the other two *** of the two survivors had residual liver disease of chronic hepatitis,while the other had chronic hepatitis and neurologic *** disease remained in a patient who successfully received a liver *** acute episodes,serum levels of aspartate aminotransferase(AST)and alanine aminotransferase(ALT)changed rapidly along with ***-specific ALT levels were age-dependently correlated with hemoglobin(Hb)*** reduction was milder for AST than ALT which resulted in a high AST/ALT ratio in the anemic *** anemic stage in two patients transformed to acute liver ***:All survivors of an acute episode of the acute hepatic phenotype had residual liver disease or both liver and neurologic *** rapid changes in liver enzymes during the acute episode and the liver and neurologic diseases remaining in survivors may provide a better understanding of WD.

关键词： Aminotransferase Neurologic disease Liver disease Hemoglobin

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Hand Cooling Enhances the Proprioceptive Drift during Rubber Hand Illusion

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Journal of Behavioral and Brain Science 2024年第7期14卷 210-226页

作者： Masanori Sakamoto Yuta Akaike Kazuya tatsumi Hirotoshi Ifuku Division of Health and Physical Education Graduate School of Education Kumamoto University Kumamoto City Japan Lifelong Sports and Welfare Course Faculty of Education Kumamoto University Kumamoto City Japan

Background: The neural representation of the body is easily altered by integrating multiple sensory signals in the brain. The “Rubber Hand Illusion” (RHI) is one of the most popular experimental paradigms to investigate this phenomenon. During this illusion, ownership of a rubber hand is temporarily induced. It was shown that external and continuous cooling of the palm enhanced the RHI, suggesting an association between altered the autonomic nervous system regulation and altered the sense of ownership of a specific limb. Purpose: To investigate whether artificially cooling the entire hand for a short period affects the magnitude of the illusion. Methods: Participants immersed their entire hand in cool, cold, or warm water for 1 min before the RHI procedure. Results: We found that cooling the entire hand enhanced the proprioceptive drift during the RHI but not the subjective feeling of ownership. In contrast, warming and intense cooling of the entire hand did not affect the RHI strength. Conclusion: Our results suggest that transient and moderate cooling of the entire hand was sufficient in enhancing the illusory disembodiment of one’s own hand.

关键词： Hand Temperature Cooling Rubber Hand Illusion Ownership Proprioceptive Drift

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Tree diversity effects on forest productivity increase through time because of spatial partitioning

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Forest Ecosystems 2020年第3期7卷 327-333页

作者： Shinichi tatsumi Department of Biological Sciences University of Toronto ScarboroughTorontoONCanada Hokkaido Research Center Forestry and Forest Products Research InstituteHokkaidoJapan

Background: Experimental manipulations of tree diversity have often found overyielding in mixed-species plantations. While most experiments are still in the early stages of stand development, the impacts of tree diversity are expected to accumulate over time. Here, I present findings from a 31-year-old tree diversity experiment(as of2018) in ***: I find that the net diversity effect on stand biomass increased linearly through time. The species mixture achieved 64% greater biomass than the average monoculture biomass 31 years after planting. The complementarity effect was positive and increased exponentially with time. The selection effect was negative and decreased exponentially with time. In the early stages(≤ 3 years), the positive complementarity effect was explained by enhanced growths of early-and mid-successional species in the mixture. Later on(≥ 15 years), it was explained by their increased survival rates owing to vertical spatial partitioning — i.e. alleviation of self-thinning via canopy stratification. The negative selection effect resulted from suppressed growths of late-successional species in the bottom ***: The experiment provides pioneering evidence that the positive impacts of diversity-driven spatial partitioning on forest biomass can accumulate over multiple decades. The results indicate that forest biomass production and carbon sequestration can be enhanced by multispecies afforestation strategies.

关键词： Biomass productivity Complementarity effect Biodiversity and ecosystem functioning Forest dynamics Mixed plantation Plant succession Selection effect Shade tolerance Stand structure Tree diversity experiment

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Making continental crust:The sanukitoid connection

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Chinese Science Bulletin 2008年第11期53卷 1620-1633页

作者： Yoshiyuki tatsumi Institute for Research on Earth Evolution (IFREE) Japan Agency for Marine-Earth Science and Technology (JAMSTEC) Yokosuka 237-0061 Japan

The average continental crust possesses intermediate compositions that typify arc magmatism and as a result it is believed to have been created at ancient convergent plate boundaries. One possible mechanism for intermediate continental crust formation is the direct production of andesitic melts in the upper mantle. Sanukitoids, which characterize the Setouchi volcanic belt, SW Japan, include un-usually high-Mg andesites (HMA). They were generated by slab melting and subsequent melt-mantle interactions under unusual tectonic settings such as where warm lithosphere subducts into hot upper mantle. Such conditions would have existed in the Archean. Hydrous HMA magmas are likely to have solidified within the crust to form HMA plutons, which were then remelted to produce differentiated sanukitoids. At present, generation and differentiation of HMA magmas may be taking place in the Izu-Bonin-Mariana arc-trench system (IBM), because (1) HMA magmatism characterizes the initial stages of the IBM evolution and (2) the IBM middle crust exhibits Vp identical to that of the bulk conti-nental crust. Vp estimates for plutonic rocks with HMA compositions support this. However tonalitic composition for middle-crust-forming rocks cannot be ruled out, suggesting an alternative possibility that the continental crust has been created by differentiation of mantle-derived basaltic magmas.

关键词：赞岐状岩大陆壳地质学地质运动

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Prediction of Photosynthetic Carbon Assimilation Rate of Individual Rice Leaves under Changes in Light Environment Using BLSTM-Augmented LSTM

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Computer Modeling in Engineering & Sciences 2022年第12期133卷 557-577页

作者： Masayuki Honda Kenichi tatsumi Masaki Nakagawa Graduate School of Bio-Applications and Systems Engineering Tokyo University of Agriculture and TechnologyKoganei City184-8588Japan Institute of Agriculture Tokyo University of Agriculture and TechnologyFuchu City183-0054Japan Institute of Engineering Tokyo University of Agriculture and TechnologyKoganei City184-8588Japan

A model to predict photosynthetic carbon assimilation rate(A)with high accuracy is important for forecasting crop yield and *** short-term memory(LSTM),a neural network suitable for time-series data,enables prediction with high accuracy but requires mesophyll *** addition,for practical use,it is desirable to have a technique that can predict A from easily available *** this study,we propose a BLSTM augmented LSTM(BALSTM)model,which utilizes bi-directional LSTM(BLSTM)to indirectly reproduce the mesophyll variables required for *** most significant feature of the proposed model is that its hybrid architecture uses only three relatively easy-to-collect external environmental variables—photosynthetic photon flux density(Q_(in)),ambient CO_(2) concentration(C_(a)),and temperature(T_(air))—to generate mesophyll CO_(2) concentration(C_(i))and stomatal conductance to water vapor(g_(sw))as intermediate ***,A is predicted by applying the obtained intermediate outputs to the learning ***,in this study,1)BALSTM(Q_(in),C_(a),T_(air))had a significantly higher A prediction accuracy than LSTM(Q_(in),C_(a),T_(air))in case of using only Q_(in),C_(a),and T_(air);2)BALSTMC_(i),g_(sw),which had C_(i) and g_(sw) as intermediate products,had the highest A prediction accuracy compared with other candidates;and 3)for samples where LSTM(Q_(in),C_(a),T_(air))had poor prediction accuracy,BALSTMC_(i),g_(sw)(Q_(in),C_(a),T_(air))clearly improved the ***,it was found that incorrect predictions may be formed when certain factors are not reflected in the data(e.g.,timing,cultivar,and growth stage)or when the training data distribution that accounts for these factors differs from the predicted data ***,a robust model should be constructed in the future to improve the prediction accuracy of A by conducting gasexchange measurements(including a wide range of external environmental values)and by increasing the

关键词： Hybrid prediction model assimilation rate leaf internal variables recurrent neural network fluctuating light environments rice

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二甲醚的电催化氧化反应

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催化学报 2003年第9期24卷 695-700页

作者：王世忠 tatsumi ISHIHARA 厦门大学化学系福建厦门361005 大分大学应用化学系

考察了负载于镓酸镧基电解质上的镍电极与镍钐掺杂氧化铈复合电极电催化二甲醚氧化反应的特性 .结果表明 ,反应的主要产物均为CO ,H2 和CH4,同时生成少量完全氧化的产物H2 O和CO2 .在开路电位下二甲醚发生裂解反应 ,生成的CO ,H2 和CH... 详细信息

考察了负载于镓酸镧基电解质上的镍电极与镍钐掺杂氧化铈复合电极电催化二甲醚氧化反应的特性 .结果表明 ,反应的主要产物均为CO ,H2 和CH4,同时生成少量完全氧化的产物H2 O和CO2 .在开路电位下二甲醚发生裂解反应 ,生成的CO ,H2 和CH4三种主要产物的比例接近于 1.在有电泵氧存在下 ,二甲醚的电催化氧化反应强烈地依赖于阳极及电解质材料的组成 .Ni/La0 9Sr0 1Ga0 8Mg0 2 O3 界面上发生的主反应是二甲醚的部分氧化 ,且存在有严重的积碳现象 .电极中掺入SDC( 15 %Sm3 + 掺杂的CeO2 )后 ,二甲醚完全氧化性能明显增强 ;随着电流的增大 ,氢的生成速率显著减小 ,并生成大量的H2 O .采用掺钴镓酸镧基电解质后 ,Ni SDC主要表现为催化二甲醚部分氧化反应 ,且显著抑制了积碳的发生 .Ni SDC/La0 8Sr0 2Ga0 8 Mg0 11Co0 0 9O3 上二甲醚电催化氧化反应的主要产物为 1∶1的CO和H2 .掺钴电解质引起Ni SDC具有特殊的催化性能 ,可能与电解质中

关键词：二甲醚氧化电催化固体氧化物燃料电池阳极镍

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利用钐掺杂的氧化铈夹层提高燃料电池阳极的活性

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物理化学学报 2003年第9期19卷 849-853页

作者：王世忠 Ishihara tatsumi 厦门大学化学系厦门361005 Department of Applied Chemistry Oita UniversityDannoharu 700Oita 870-1192Japan

考察了Ni-钐掺杂的氧化铈(Ni-SDC)复合阳极与La_(0.9)Sr_(0.1)Ga_(0.8)Mg_(0.2)O_3(LSGM)电解质中间加入的SDC中间层对阳极及整个电池性能的影响.结果表明,SDC中间层的加入显著减小了阳极极化过电位,但同时引入了欧姆降,降低了电池的功... 详细信息

考察了Ni-钐掺杂的氧化铈(Ni-SDC)复合阳极与La_(0.9)Sr_(0.1)Ga_(0.8)Mg_(0.2)O_3(LSGM)电解质中间加入的SDC中间层对阳极及整个电池性能的影响.结果表明,SDC中间层的加入显著减小了阳极极化过电位,但同时引入了欧姆降,降低了电池的功率输出密度.氢在Ni-SDC电极的氧化主要由两个过程控制,分别对应于交流阻抗谱的两个阻抗半圆,高频环随着SDC中间层的加入显著减小,可能对应于H_2在Ni-SDC/SDC/H_2三相界的电化学氧化或氧从LSGM向SDC的传输,低频环与SDC中间层无关,可能对应于氢在电极表面的解离吸附及吸附物种的扩散过程.使用Ni-SDC/SDC夹层阳极可以明显地提高电池的稳定性.

关键词：钐掺杂氧化铈燃料电池阳极活性镍电极双层阳极极化交流阻抗固体氧化物

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