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1. ビタミンKの概要overview of vitamin k [show details]
… vitamin K deficiency can be evaluated by measuring prothrombin time (PT) and international normalized ratio (INR), both of which are prolonged in vitamin K deficiency. When the deficiency is mild,… A few foods, mainly fermented cheeses, have significant amounts of vitamin K2 (menaquinones). Special considerations apply to vitamin K requirements in the following populations: Patients on total parenteral …
2. 大顆粒リンパ球性白血病の治療treatment of large granular lymphocyte leukemia [show details]
… Large granular lymphocyte (LGL) leukemia is characterized by peripheral blood and marrow lymphocytic infiltration with clonal LGLs, splenomegaly, and cytopenias, most commonly neutropenia. The LGL is …
3. ナチュラルキラー（NK）細胞大顆粒リンパ球性白血病natural killer nk cell large granular lymphocyte leukemia [show details]
… Large granular lymphocyte (LGL) leukemia is characterized by peripheral blood and marrow lymphocytic infiltration with clonal LGLs, splenomegaly, and cytopenias, most commonly neutropenia. LGL leukemia …
4. 特別な医療が必要な小児を含めた未成年者children and youth with special health care needs [show details]
…conditions that result in special health care needs is discussed in individual topic reviews (eg, cystic fibrosis, autism spectrum disorder). Children and youth with special health care needs (CYSHCN) …
5. 大腸ポリープの概要overview of colon polyps [show details]
… laterally-spreading adenomas may have a granular appearance, which is characteristic of being benign. If the mucosa is smooth and non-granular,… and complete excision is recommended. However, due to their sessile nature and indistinct borders, special care is needed to ensure their complete removal endoscopically. There is molecular and clinical evidence …

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藤田英明 [博士（薬学）] Hideaki

★リンクテーブル★

リンク元	「免疫不全症候群」
関連記事	「欠損」「顆粒」「欠損症」「特殊顆粒」「特殊」

「免疫不全症候群」

　　[★]

英: immunodeficiency syndrome, immunodeficiency
同: 免疫不全症、免疫不全 immune deficiency、immunodeficiency disease immunodeficiency disorder ID
関: 先天性免疫不全症、原発性免疫不全症、免疫不全患者

先天性免疫不全症候群

重症複合免疫不全症 severe combined immunodeficiency SCID：重篤で主なものはX連鎖劣性遺伝。T細胞-でB細胞とNK細胞は病型ごとに欠損

1. T-B+ SCID
2. T-B- SCID
3. 特殊型：Omenn症候群、X連鎖型高IgM症候群(高IgM症候群)

抗体欠乏を主徴とする免疫不全症：B細胞の内因性欠陥またはT－B細胞相互間の異常により，抗体産生が障害された疾患群で，最も頻度が高い(原発性免疫不全症の約45%)

無ガンマグロブリン血症

X連鎖無ガンマグロブリン血症()
常染色体劣性無ガンマグロブリン血症

IgGサブクラス欠損症/IgGサブクラス欠乏症(IgG subclass deficiency)/Ig heavy-chain gene deletions
IgA欠損症

その他

1. Wiskott-Aldrich症候群 WAS：血小板減少、免疫低下、湿疹。X連鎖劣性遺伝。WASpをコードする遺伝子の変異による。T細胞における免疫シナプス形成不全が本態。IgG,IgMは正常～低下、IgE, IgAは増加。リンパ球数は漸減。
2. DNA修復の欠陥

毛細血管拡張性運動失調症 ataxia telangiectasia AT：毛細血管拡張、小脳失調症、。常染色体劣性遺伝。ATM蛋白は細胞周期制御、DNA調節に関与していると考えられ、遺伝子異常により(in vitroで？)放射線高感受性、細胞周期の異常、染色体脆弱性が認められる。T細胞減少、IgA,IgE減少。IgG2,IgG4も減少。