WordNet

the means of connection between things linked in series (同)nexus
a unit of length equal to 1/100 of a chain
an interconnecting circuit between two or more locations for the purpose of transmitting and receiving data (同)data link
a fastener that serves to join or connect; "the walls are held together with metal links placed in the wet mortar during construction" (同)linkup, tie, tie-in
(computing) an instruction that connects one part of a program or an element on a list to another program or list
the 24th letter of the Roman alphabet (同)x, ex
a disease of the intestinal tract

PrepTutorEJDIC

(鎖の)『輪』,環 / 鎖のようにつながったソーセージの一節 / (…と)つなぐ物(人),(…との)きずな,つながり《+『with』(『to』)+『名』》 / 《複数形で》=cuff links / …‘を'『つなぎ合わせる』,連結する;(…と)…‘を'つなぐ《+『名』+『with』(『to』)+『名』》 / (…と)結合する,つながる《+[『up』(『together』)]『with』+『名』》
たいまつ
《語・句・節を対等に結んで》…『と』…,…や…,および / 《数詞を結合して》…『足す』…,…に加えて / …『しかも』…,…して,同時に… / 《時間的に》『そして』,それから,すると(and then) / 《当然の帰結として》…『ので』,…だから(and so) / 《おもに話》《命令文などの後で》『そうすれば』,そうしたら / 《追加・強調して》それに,しかも / 《対照的な内容を導いて》ところが,しかし(but) / 《譲歩的に》それなのに,それでいながら / 《A and Bで一体の関係を表して》《単数扱い》 / 《同一語を反復して多数・継続などを表して》 / 《同一の複数名詞を結合して変化・種類などを表して》 / 《話》《「形容詞+and+形容詞」の形で前の形容詞が後に副詞的に働く》 / 《話》《「動詞come, goなど+and+動詞」の形でandとその後の動詞が不定詞の働きをして》 / 《「動詞+and+動詞」の形で後の動詞が現在分詞の意を表して》 / 《話》《文頭において前の質問・意見などに同意を示して》そうとも,そして(Yes!and) / 《話》《文頭に置いて驚き・疑念・非難などを表して》ほんとうに;…なのに
(次にくる語の発音が母音で始まるときに用いる) / (子音[h]で始まり第1音節に強勢のない語の場合はanを用いることがある.ただし,この場合は[h]を発音しない)
Christ / Christian

UpToDate Contents

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1. IPEX：免疫不全，多腺性内分泌障害、腸疾患、X連鎖 ipex immune dysregulation polyendocrinopathy enteropathy x linked
2. 小児における慢性下痢の原因の概要 overview of the causes of chronic diarrhea in children
3. IgEの生物学 the biology of ige
4. 原発性免疫不全症の診断のためのフローサイトメトリー flow cytometry for the diagnosis of primary immunodeficiencies
5. 自己免疫の概要 overview of autoimmunity

English Journal

Single centre experience of haematopoietic SCT for patients with immunodysregulation, polyendocrinopathy, enteropathy, X-linked syndrome.

Nademi Z1, Slatter M1, Gambineri E2, Mannurita SC2, Barge D3, Hodges S4, Bunn S4, Thomas J4, Haugk B5, Hambleton S1, Flood T6, Cant A1, Abinun M1, Gennery A1.
Bone marrow transplantation.Bone Marrow Transplant.2014 Feb;49(2):310-2. doi: 10.1038/bmt.2013.181. Epub 2013 Nov 25.
PMID 24270390

Anti-CD4 treatment inhibits autoimmunity in scurfy mice through the attenuation of co-stimulatory signals.

Mayer CT1, Tian L, Hesse C, Kühl AA, Swallow M, Kruse F, Thiele M, Gershwin ME, Liston A, Sparwasser T.Author information 1Institute of Infection Immunology, TWINCORE, Centre for Experimental and Clinical Infection Research, Feodor-Lynen-Str. 7, 30625 Hannover, Germany(1).AbstractA major concept in autoimmunity is that disruption of Foxp3+ regulatory T cells (Tregs) predisposes to breach of tolerance. This is exemplified by the Foxp3-linked disorder termed IPEX (immunodysregulation, polyendocrinopathy, enteropathy, X-linked) which affects newborn children. There has been considerable clinical interest in the role of non-depleting anti-CD4 antibodies as a means of upregulating the function of Foxp3+ Tregs in order to control detrimental inflammatory responses such as transplant rejection. However, according to the paradigm of a Treg-dependent mechanism of action, the effectiveness of anti-CD4 antibodies as a therapy for human autoimmune diseases is unclear considering that Treg function might be intrinsically impaired. Specifically, anti-CD4 therapy is expected to fail in patients suffering from the IPEX syndrome due to the lack of functional Foxp3+ Tregs. Taking advantage of natural Foxp3 mutant scurfy (sf) mice closely resembling the IPEX syndrome, and genetically engineered mice depleted of Foxp3+ Tregs, we report here that anti-CD4 treatment induces tolerance independent of Foxp3+ Tregs. This so far undefined mechanism is dependent on the recessive non-infectious tolerization of autoreactive T cells. Treg-independent tolerance alone is powerful enough to suppress both the onset and severity of autoimmunity and reduces clinically relevant autoantibody levels and liver fibrosis. Mechanistically, tolerance induction requires the concomitant activation of autoreactive T cells and is associated with the down-regulation of the co-stimulatory TNF-receptor superfamily members OX40 and CD30 sustaining CD4+ T cell survival. In the light of ongoing clinical trials, our results highlight an unexpected potency of anti-CD4 antibodies for the treatment of autoimmune diseases. Particularly, CD4 blockade might represent a novel therapeutic option for the human IPEX syndrome.
Journal of autoimmunity.J Autoimmun.2013 Sep 25. pii: S0896-8411(13)00116-9. doi: 10.1016/j.jaut.2013.08.010. [Epub ahead of print]
A major concept in autoimmunity is that disruption of Foxp3+ regulatory T cells (Tregs) predisposes to breach of tolerance. This is exemplified by the Foxp3-linked disorder termed IPEX (immunodysregulation, polyendocrinopathy, enteropathy, X-linked) which affects newborn children. There has been con
PMID 24075450

Assessment of intracellular cytokines and regulatory cells in patients with autoimmune diseases and primary immunodeficiencies - novel tool for diagnostics and patient follow-up.

Osnes LT1, Nakken B, Bodolay E, Szodoray P.Author information 1Institute of Immunology, Rikshospitalet, Oslo University Hospital, Norway.AbstractSerum and intracytoplasmic cytokines are mandatory in host defense against microbes, but also play a pivotal role in the pathogenesis of autoimmune diseases by initiating and perpetuating various cellular and humoral autoimmune processes. The intricate interplay and fine balance of pro- and anti-inflammatory processes drive, whether inflammation and eventually organ damage will occur, or the inflammatory cascade quenches. In the early and late, as well as inactive and active stages of autoimmune diseases, different cellular and molecular patterns can dominate in these patients. However, the simultaneous assessment of pro- and anti-inflammatory biomarkers aids to define the immunological state of a patient. A group of the most useful inflammatory biomarkers are cytokines, and with increasing knowledge during the last decade their role have been well-defined in patients with autoimmune diseases and immunodeficiencies. Multiple pathological processes drive the development of autoimmunity and immunodeficiencies, most of which involve quantitative and qualitative disturbances in regulatory cells, cytokine synthesis and signaling pathways. The assessment of these biomarkers does not aid only in the mechanistic description of autoimmune diseases and immunodeficiencies, but further helps to subcategorize diseases and to evaluate therapy responses. Here, we provide an overview, how monitoring of cytokines and regulatory cells aid in the diagnosis and follow-up of patients with autoimmune diseases and immunodeficiencies furthermore, we pinpoint novel cellular and molecular diagnostic possibilities in these diseases.
Autoimmunity reviews.Autoimmun Rev.2013 Aug;12(10):967-71. doi: 10.1016/j.autrev.2013.02.003. Epub 2013 Mar 26.
Serum and intracytoplasmic cytokines are mandatory in host defense against microbes, but also play a pivotal role in the pathogenesis of autoimmune diseases by initiating and perpetuating various cellular and humoral autoimmune processes. The intricate interplay and fine balance of pro- and anti-inf
PMID 23541481

Japanese Journal

IPEX症候群と腎疾患

橋村裕也,野津寛大,神田杏子,早川晶,竹島泰弘,金兼弘和,宮脇利男,飯島一誠,松尾雅文
日本小児腎臓病学会雑誌 = Japanese journal of pediatric nephrology 22(2), 131-135, 2009-11-15
　IPEX症候群は，FOXP3遺伝子の変異が制御性T細胞の機能異常を引き起こすまれな自己免疫異常症である。また，IPEX症候群患者は無治療で経過すると2歳までに感染や栄養障害から死亡する予後不良な疾患である。しかし，特異的な治療法がなく，免疫抑制剤の併用療法が行われてきたが根治療法とはならない。造血幹細胞移植が，近年，根治術として報告され良好な成績を挙げている。腎合併症は膜性腎症や尿細管間質性腎炎 …
NAID 10026411780