慢性肉芽腫症
WordNet
- thorough; "had a good workout"; "gave the house a good cleaning"
- moral excellence or admirableness; "there is much good to be found in people" (同)goodness
- that which is pleasing or valuable or useful; "weigh the good against the bad"; "among the highest goods of all are happiness and self-realization" (同)goodness
- benefit; "for your own good"; "whats the good of worrying?"
- not forged; "a good dollar bill" (同)honest
- of moral excellence; "a genuinely good person"; "a just cause"; "an upright and respectable man" (同)just, upright
- most suitable or right for a particular purpose; "a good time to plant tomatoes"; "the right time to act"; "the time is ripe for great sociological changes" (同)right, ripe
- tending to promote physical well-being; beneficial to health; "beneficial effects of a balanced diet"; "a good nights sleep"; "the salutary influence of pure air" (同)salutary
- appealing to the mind; "good music"; "a serious book" (同)serious
- in excellent physical condition; "good teeth"; "I still have one good leg"; "a sound mind in a sound body" (同)sound
- not left to spoil; "the meat is still good" (同)undecomposed, unspoiled, unspoilt
- resulting favorably; "its a good thing that I wasnt there"; "it is good that you stayed"; "it is well that no one saw you"; "alls well that ends well" (同)well
- agreeable or pleasing; "we all had a good time"; "good manners"
- capable of pleasing; "good looks"
- generally admired; "good taste"
- having desirable or positive qualities especially those suitable for a thing specified; "good news from the hospital"; "a good report card"; "when she was good she was very very good"; "a good knife is one good for cutting"; "this stump will make a good picnic table"; "a good check"; "a good joke"; "a good exterior paint"; "a good secretary"; "a good dress for the office"
- morally admirable
- something resembling a bridge in form or function; "his letters provided a bridge across the centuries"
- make a bridge across; "bridge a river"
- a circuit consisting of two branches (4 arms arranged in a diamond configuration) across which a meter is connected (同)bridge circuit
- an upper deck where a ship is steered and the captain stands (同)bridge deck
- a denture anchored to teeth on either side of missing teeth (同)bridgework
- the link between two lenses; rests on the nose (同)nosepiece
- a structure that allows people or vehicles to cross an obstacle such as a river or canal or railway etc. (同)span
- a wooden support that holds the strings up
- any of various card games based on whist for four players
- the hard ridge that forms the upper part of the nose; "her glasses left marks on the bridge of her nose"
- connect or reduce the distance between (同)bridge over
- cross over on a bridge
- a pattern of symptoms indicative of some disease
- a complex of concurrent things; "every word has a syndrome of meanings"
- United States labor leader who organized the longshoremen (1901-1990) (同)Harry Bridges
PrepTutorEJDIC
- (物事の内容・程度などが)『よい』,りっぱな,上等の,みごとな;十分な / (道徳的に)『善良な』,徳のある,正しい / (子供が)『行儀がよい』,おとなしい / (行動・目的などが)『適している』 / 『親切な』,優しい,好意的な(kind) / 『十分な』,相当な,たっぷりの / 『じょうずな』,熟練した,有能な,鮮やかな / 『楽しい』,愉快な / 真正な,ほんものの;有効の / 腐っていない,新鮮な,口に合う,おいしい / 《話》十分に / (商取引など)信用できる,信頼できる,確実な / 《呼び掛けに添え》りっぱな / (健康・気分が)よい,元気な / 『よいこと』,善,徳 / 『利益』,幸福 / よく(well)
- 橋 / 船橋,艦橋 / 鼻柱 / (めがねの)ブリッジ(2つのレンズを接続し鼻の上にのせる部分) / (弦楽器の)こま / ブリッジ(隣接する歯に両端を固定した義歯) / 〈川・道路・線路など〉‘に'橋渡しをする,‘を'連結する
- (カードゲームの)ブリッジ
- (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
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Related Links
- 出典: meddic. 移動: メニュー, 検索. 英: chronic granulomatous disease, CGD; 同: ベレンデス-ブリッジ-グッド症候群 Berendes-Bridge-Good syndrome; 関: 原発性免疫 不全症候群. 概念. X連鎖劣性遺伝によるものが多くを占め、好中球の殺菌能低下、 幼少 ...
- 慢性肉芽腫症」. [☆]. 英: chronic granulomatous disease, CGD; 同: ベレンデス- ブリッジ-グッド症候群 Berendes-Bridge-Good syndrome; 関: 原発性免疫不全症候群 . 概念. X連鎖劣性遺伝によるものが多くを占め、好中球の殺菌能低下、幼少期からの 細菌 ...
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- 英
- chronic granulomatous disease, CGD
- 同
- ベレンデス-ブリッジ-グッド症候群 Berendes-Bridge-Good syndrome
- 関
- 原発性免疫不全症候群
概念
- X連鎖劣性遺伝によるものが多くを占め、好中球の殺菌能低下、幼少期からの細菌と真菌の反復感染、および肉芽腫形成を特徴とする原発性免疫不全症の一疾患である。
- 病原体をアレルゲンと見なせば、発症メカニズムはIV型アレルギーによるものと考えてよい?
疫学
- an incidence of 1 in 200,00 indicuduals (HIM.380)
遺伝形式
- 伴性遺伝:most often CGD is inherited as an X-linked recessive trait (HIM.380).
- 常染色体劣性遺伝:30% of patients inherit the disease in an autosomal recessive pattern (HIM.380).
病因
- 膜蛋白質シトクロムb 558の重鎖(β鎖:p91)、軽鎖(α鎖:p22)、細胞質因子p47、p67の欠損や機能異常
病態
- 食細胞(好中球・マクロファージ)に貪食された細菌を殺菌する活性酸素産生系の障害
気炎菌の特徴
- カタラーゼ陽性・過酸化水素非産生菌の感染:Salmonella typhimurium, Pseudomonas aeruginosa, Klebsiella pneumoniae, Staphylococcus aureus, Proteus mirabilis, Escherichia coli, Srratia marcescens
- ⇔カタラーゼ陰性・過酸化水素産生菌:Streptococcus pneumoniae, S. pyogenes, S.fecalis
症状
- 乳児早期から始まる全身諸臓器の反復性・難治性の感染症 (PED.1151)
- 皮膚化膿疹・膿瘍、細菌・真菌性肺炎、化膿性リンパ節炎。肝脾膿瘍・肛門周囲膿瘍
- 特徴的な症状:肉芽腫形成による尿路や消化管の通過障害
検査
- 好中球数:正常
- 白血球数:増多
- NBT色素還元能試験:陰性
治療
- 感染症:抗菌薬の静脈内投与
- 真菌以外の感染症の治療・予防:ST合剤
- インターフェロンγ:感染症の発生抑制 → アスペルギルス症を抑制できたことにより予後が改善
- 肉芽腫による通過障害:副腎皮質ステロイド薬
- 骨髄移植:造血幹細胞移植により根治療が可能
- 遺伝子治療
予後
- 平均16歳 (PED.1152)
- 深在性アスペルギルス症は致命的 → IFN-γの投与による感染症予防により予後が改善されつつある
USMLE
参考
- 1. 慢性肉芽腫症に対する造血幹細胞移植ガイドライン
- http://pidj.rcai.riken.jp/medical_guideline091221.html
===uptodate
- 1. [charged] 慢性肉芽腫性疾患:病因、臨床症状、および診断 - uptodate [1]
- 2. [charged] 慢性肉芽腫性疾患:治療および予後 - uptodate [2]
OMIM
- 1. GRANULOMATOUS DISEASE, CHRONIC, X-LINKED; CGD
- http://www.ncbi.nlm.nih.gov/omim/306400
- 2. GRANULOMATOUS DISEASE, CHRONIC, AUTOSOMAL RECESSIVE, CYTOCHROME b-POSITIVE, TYPE I
- http://www.ncbi.nlm.nih.gov/omim/233700
- 3. GRANULOMATOUS DISEASE, CHRONIC, AUTOSOMAL RECESSIVE, CYTOCHROME b-POSITIVE, TYPE II
- http://www.ncbi.nlm.nih.gov/omim/233710
- 4. GRANULOMATOUS DISEASE, CHRONIC, AUTOSOMAL RECESSIVE, CYTOCHROME b-POSITIVE, TYPE III
- http://www.ncbi.nlm.nih.gov/omim/613960
- 5. GRANULOMATOUS DISEASE, CHRONIC, AUTOSOMAL RECESSIVE, CYTOCHROME b-NEGATIVE GeneTests
- http://www.ncbi.nlm.nih.gov/omim/233690
- 6. GRANULOMATOUS DISEASE, CHRONIC, AUTOSOMAL DOMINANT TYPE
- http://www.ncbi.nlm.nih.gov/omim/138990
国試
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- 関
- excellent、superior
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