Neonatal hemochromatosis with epsilon gamma delta beta-thalassemia: a case report and analysis of serum iron regulators

Background Neonatal hemochromatosis causes acute liver failure during the neonatal period, mostly due to gestational alloimmune liver disease (GALD). Thalassemia causes hemolytic anemia and ineffective erythropoiesis due to mutations in the globin gene. Although neonatal hemochromatosis and thalassemia have completely different causes, the coexistence of these diseases can synergistically exacerbate iron overload. We report that a newborn with epsilon gamma delta beta-thalassemia developed neonatal hemochromatosis, which did not respond to iron chelators and rapidly worsened, requiring living-donor liver transplantation. Case presentation A 1-day-old Japanese boy with hemolytic anemia and targeted red blood cells was diagnosed with epsilon gamma delta beta-thalassemia by genetic testing, and required frequent red blood cell transfusions. At 2 months after birth, exacerbation of jaundice, grayish-white stool, and high serum ferritin levels were observed, and liver biopsy showed iron deposition in hepatocytes and Kupffer cells. Magnetic resonance imaging scans showed findings suggestive of iron deposits in the liver, spleen, pancreas, and bone marrow. The total amount of red blood cell transfusions administered did not meet the criteria for post-transfusion iron overload. Administration of an iron-chelating agent was initiated, but iron overload rapidly progressed to liver failure without improvement in jaundice and liver damage. He underwent living-donor liver transplantation from his mother, after which iron overload disappeared, and no recurrence of iron overload was observed. Immunohistochemical staining for C5b-9 in the liver was positive. Serum hepcidin levels were low and serum growth differentiation factor-15 levels were high prior to living-donor liver transplantation. Conclusions We reported that an infant with epsilon gamma delta beta-thalassemia developed NH due to GALD, and that coexistence of ineffective erythropoiesis in addition to erythrocyte transfusions may have exacerbated iron overload. Low serum hepcidin levels, in this case, might have been caused by decreased hepcidin production arising from fetal liver damage due to neonatal hemochromatosis and increased hepcidin-inhibiting hematopoietic mediators due to the ineffective hematopoiesis observed in thalassemia

ニホンブンポウロン ト ハイゼ ノ ドイツ ブンテン

山田孝雄は『日本文法論』において、西洋文典の模倣による日本文典の不備を的確に指摘しつつ、自らは西洋文典の綿密な研究を踏まえ、「國語の本性に適合」した独自の文法体系を構築したと言われる。この文脈で必ず言及されるのがスウィートの英文典とハイゼの獨逸文典、そしてヴントの心理学である。スウィートやヴントについては、『日本文法論』に見られる引用文の出典を容易に特定できるため、引用の妥当性も含め、彼らの著作と『日本文法論』の関係を原典にあたって検証することができる。しかし、「ハイゼの獨逸文典」と呼ばれるものが正確に何を指し、計175 行にも及ぶドイツ語の文章が、どの文典のどの箇所から引用されているのかは、これまでの研究でも正確には特定されていない。ハイゼの氏名についてさえ、文献には若干の混乱が見られる。　こうした現状に鑑み、本稿は『日本文法論』が引くハイゼの原典を特定しようと試みるものである。ハイゼが残した3 系統の文典は、それぞれに極めて複雑な来歴があり、これが原典の特定を妨げてきた大きな要因であると思われるが、本稿ではこれらを1 系統に絞り込んだ上で、この系統の第28 版までの中から山田が参照した版を特定し、すべての引用文の出所を原典にあたって確認することができた。これにより、「ハイゼの獨逸文典」と『日本文法論』の関係を具体的に検証するための基礎が確立されたことになる。Yoshio Yamada’s Nihonbunporon（ “Theory of Japanese Grammar,” hereafter TJG） is widely recognized as a truly monumental work in the history of Japanese linguistics. TJG is known for its penetrating criticism of the previous grammars of Japanese which were modeled on the 19th century grammars of Western languages. As Yamada rightly stresses, one cannot hope to gain any genuine insights into the “essence” of the Japanese language by a naive application of the framework of Western grammars（ i.e., Western “parts of speech,” etc.）. 　Yamada did not ignore Western grammars, however. He explicitly says in TJG that there is much to learn from Western scholars especially on the nature of language in general; in fact, he quotes extensively from “Sweet’s English Grammar” and “Heyse’s German Grammar” as well as from Wundt’s psychological writings. To determine how these Western scholars influenced Yamada, it is essential that one examine the original sources of the passages quoted in TJG. So far, however, this has not been done for “Heyse’s German Grammar” because the exact original sources of Yamada’s quotes from Heyse have yet to be traced. TJG contains a total of 175 lines quoted from what Yamada refers to as “Heyse’s German Grammar” but no bibliographical information on this work is given in TJG. This, together with the fact that Heyse published three different books which can all be called “Heyse’s German Grammar,” and that each of them has a complex history of continued revisions by different editors, has made it difficult to determine exactly which edition of which book Yamada actually consulted. The present paper identifies the original text, thereby providing a solid basis for further research on how Heyse’s German Grammar influenced Yamada’s Theory of Japanese Grammar.研究論文(Article

Long-term Remission of Hepatitis-associated Aplastic Anemia Possibly due to Immunosuppressive Therapy after Liver Transplantation

Hepatitis-associated aplastic anemia (HAAA) is an acquired bone marrow failure syndrome that develops after seronegative fulminant hepatitis. Abnormal cytotoxic T-cell activation with cytokine release is a possible pathophysiology. We present the case of a 16-month-old Japanese male who developed HAAA following living-donor liver transplantation for fulminant hepatitis. His aplastic anemia was successfully treated with immunosuppressive therapy. He had been administered tacrolimus for prophylaxis against hepatic allograft rejection. Ten years after the HAAA onset, the patient’s bone marrow was found to be slightly hypoplastic. Tacrolimus may be effective in controlling abnormal immune reactions that can cause recurrent impaired hematopoiesis

1. Introduction Secondary Predication in Mongolian

The goal of this paper is to answer the desriptive question of what semanti

Lijdend/Passive ノ ヤクジュツ キゲン

大槻文彦は「和蘭字典文典の譯述起原」において、「受身」という用語をオランダ語の“Lydende Werk Woorden” に相当する「今譯」と位置づけ、『訂正蘭語九品集』の「被動詞」から続く、蘭学の系譜に連なる訳語として提示している。　文法用語としての「受身」は、大槻が考案したものであると大槻自身が述べていることもあり、一般にそう認定されている。専門的な文献だけではなく、『日本国語大辞典』などの信頼できる辞書も、「受身」の初出例として「語法指南」の一節を挙げている。しかし、大槻が用いた意味での「受身」は、実際には大槻以前の文献にも見出せるため、「訳述起原」に関する従来の記述には修正が必要となる。　大槻の「語法指南」はまた、現代的な意味での「助動詞」を日本文法の用語として用いた嚆矢とされるが、「助動詞」という表現自体は大槻以前に使用例があり、これまでに指摘されているところでは『附音插図英和字彙』が最も古い。しかし、これについても、さらに古い使用例を指摘することができる。『日本国語大辞典』は「助動詞」の初出例をヘボンの『和英語林集成』第3 版に求めているが、この選択には考えるべき問題があると思われるので、上の「受身」と併せて検討する。Otsuki’s grammar known as Gohoshinan（1889）is widely believed to be the first work which used ukemi ‘passive’ as a technical term for Japanese grammar. For instance, Shogakkan’s Grand Dictionary of the Japanese Language cites a passage from Otsuki’s grammar as the earliest example in which the term is used. It is also generally believed that Otsuki himself devised the grammatical term ukemi, adopting it from the traditional Japanese martial arts where it had long been used with the meaning of ‘defensive’. The present paper claims based on comprehensive documentation that these accepted views on the origin of the grammatical term ukemi are incorrect. Similar issues arise as to the origin of the grammatical term jodoshi ‘auxiliary verb’, which are also discussed in this paper.研究論