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疑似免疫缺陷immunodeficiency (郭和昌醫師)
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疑似免疫缺陷immunodeficiency (郭和昌醫師)

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An introduction of Immunodeficiency by Ho-Chang Kuo from Taiwan.

An introduction of Immunodeficiency by Ho-Chang Kuo from Taiwan.

Published in Health & Medicine , Travel
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  • T cell  PCP or fungus infection  <3m/o B cell  >6m/0 S/S develp  bacterial inection and URI Neutrophil  present at any age with skin infection, abscess, lymphadenopathy Complement  general inection with encapsulated orgen 
  • T cell  PCP or fungus infection  <3m/o B cell  >6m/0 S/S develp  bacterial inection and URI Neutrophil  present at any age with skin infection, abscess, lymphadenopathy Complement  general inection with encapsulated orgen 
  • T cell  PCP or fungus infection  <3m/o B cell  >6m/0 S/S develp  bacterial inection and URI Neutrophil  present at any age with skin infection, abscess, lymphadenopathy Complement  general inection with encapsulated orgen 
  • T cell  PCP or fungus infection  <3m/o B cell  >6m/0 S/S develp  bacterial inection and URI Neutrophil  present at any age with skin infection, abscess, lymphadenopathy Complement  general inection with encapsulated orgen 
  • The microscopic nitroblue tetrazolium (NBT) slide test, used to score the ability of individual phagocytic leukocytes to produce superoxide Purified granulocyte suspensions are incubated with NBT and fixed in suspension, thereafter centrifuged on microscope slides and stained with nuclear fast red. This method precludes stimulation and selection of cells by adherence and washing. The number of formazan grains per cell can be judged in a semiquantitative way. In parallel incubations, the cells are stained with May-Grunwald/Giemsa, allowing identification of formazan-positive and -negative cells. The test discriminates well between cells from normal individuals, cells from patients with chronic granulomatous disease ( CGD ), and cells from heterozygotes for the X-linked form of CGD . Several patients and heterozygotes with an autosomal or variant form of CGD were detected with decreased NBT-reducing activity in their neutrophils and/or eosinophils. The relation between NBT-reductase activity of the phagocytes and the clinical situation is discussed.
  • 1.IgA deficiency is the most common B cell defect
  • 1.IgA deficiency is the most common B cell defect
  • 1.IgA deficiency is the most common B cell defect
  • 1.IgA deficiency is the most common B cell defect
  • 1.IgA deficiency is the most common B cell defect

Transcript

  • 1. 免 疫 缺 陷 疾 病 簡 介 郭和昌 醫師 高雄長庚兒童過敏免疫風濕科 July 2008 Waikiki beach, Hawaii
  • 2. 郭和昌醫師
    • 國立陽明大學醫學士
    • 長庚大學臨床醫學研究所博士候選人
    • 高雄長庚醫院
    • 兒童過敏氣喘免疫科主治醫師
    • 長庚醫院講師級主治醫師
    • 中華民國小兒科專科醫師
    • 台灣兒童過敏氣喘暨免疫科專科醫師
    • 中華民國免疫科專科醫師
  • 3.  
  • 4. 免疫系統
    • 原始免疫 (innate immunity)
    • 適應免疫 (adaptive immunity)
    • 吞噬細胞
    • T 細胞
    • B 細胞
    • 補體
    Source: National Cancer Institute Ab MΦ complement
  • 5. 免疫缺陷
    • 先天性
      • CGD, hyper IgE ….
    • 後天性
      • AIDS
      • KD (IVIG supplement)
      • Chemotherapy
      • Steroid
        • oral
        • Inhalation
        • IV
        • pulse
      • Immunosuppressant agent
      • Age (6 month)
      • Severe infection
  • 6. Adapted from: Laura@Medimagery.com T cell response and B cell response
  • 7. Primary vs. secondary exposure
  • 8. Question 1
    • 何時該懷疑免疫缺乏症 ?
  • 9. Q. 何時該懷疑免疫缺乏症 ?
    • 二次或以上的 systemic bacterial infections (eg: sepsis, osteomyelitis, meningitis)
    • 一年內三次或以上的 serious respiratory or documented bacterial soft tissue infection (eg: cellulitis, draining otitis media, lymphadenitis ≦ 1 y/o)
    • 不尋常部位的感染 (eg: liver or brain abscess)
    • 不尋常菌種的感染 (eg: Aspergillus, Serratia marcescens, Nocardia, Pseudomonas)
    • 尋常菌種的感染卻有不尋常的嚴重表現
    Nelson 17th edition: p.681
  • 10. 北海道 - 小樽運河
  • 11. 東京 - 皇居二重橋
  • 12. 明治神宮 , 東京
  • 13.  
  • 14.  
  • 15. 北海道 - 小樽
  • 16.  
  • 17. 北海道 - 函館山
  • 18. 北海道 - 函館山 - 百萬夜景
  • 19.  
  • 20. Question 2
    • 如何安排進一步檢查與診斷 ?
  • 21. 進一步評估分三方面
    • History taking
    • Physical examination
    • Routine laboratory examination
    • Immunological survey
  • 22. 家族及個人病史
    • History
      • Detailed family tree
        • Premature death
        • Sex of affected individuals
        • 近親結婚
      • Medical history
        • The age at onset of symptoms
        • The nature & etiology of infections, 感染菌種,侵犯的器官,使用的抗生素, 治療的反應,長期感染狀況
  • 23. 免疫系統理學檢查
    • Appearance:
        • Failure to thrive , Pale, Wasted,
        • Hair color ( 白髮 -CHS)
      • Skin:
        • Impetigo, Cellulitis, Candidiasis
        • Rash, Seborrhoic dermatitis
      • HEENT:
        • Oral ulcer, oral candidiasis , periodonitis
        • Chronic sinusitis, otitis media
        • Lymphoadenopathy, Tonsil hypotrophy or hypertrophy
        • Palpable neck LNs
  • 24.
      • Chest:
        • Lobar pneumonia , bronchiectasis, PCP
      • Abdomen:
        • Splenomegaly, Hepatosplenomegaly
        • Cholangitis, Liver abscess (CGD),
        • Chronic diarrhea (B def. ), Crohn’s disease (CGD)
        • Prolong detachment of cord , Omphalitis (LAD)
      • Extremities:
        • Palpable Inguinal LNs
        • Arthritis (B def.), Osteomyelitis (CGD)
      • Other:
        • Lymphadenopathy (B def, CGD)
    免疫系統理學檢查
  • 25. 清溪川 = 李明博 總統
  • 26. 首爾 - 雪景
  • 27. 首爾 - 民俗村
  • 28.  
  • 29.  
  • 30. 為何有兩種蛋糕 ???
  • 31. 見微知著 !
    • Laboratory diagnosis:
    • 一般性檢查:
      • CBC /DC
        • ANC  cyclic neutropenia ,
        • RBC  hemolytic anemia,
        • platelet  Wiskott-Aldrich syndrome (X linked, immunodeficiency, thrombocytopenia , dermatitis)
      • ESR
        • R/O chronic infection or inflammatory
      • Bacterial culture
        • R/O infection
    • 顯示病人可能有特別的免疫學診斷,再使用特殊檢查 
    免疫細胞的質與量均須考量 !!!
  • 32. 應該安排哪些免疫進階檢查  抽絲撥解
    • Phagocyte : chemotaxis, chemileuminence, NBT, CD11b, IL12Rb1, phagocytosis, Actin function
    • T cell : DTH, lymphocyte surface marker, lymphocyte proliferation, CD40L
    • B cell : IgG, IgM, IgE, IgA, IgD, IgG subclass, CD40
    • Complement: C3, C4, CH50
  • 33. 吞噬細胞缺損篩檢
    • 症狀
      • 反覆皮膚感染、反覆肛門膿瘍、反覆淋巴腺炎、反覆口腔潰瘍、臍帶太晚脫落、傷口不易癒合
    • 臨床評估
      • 淋巴腺大小 , 牙齦肥厚
    • 實驗室評估
      • 吞噬細胞染色劑還原試驗 (NBT):M25-016-Z
      • 吞噬血球氧化殺菌檢查 (chemiluminescence): M25-017-Z( 自費 1100)
      • 吞噬血球沾黏分子檢查 (adhesion molecule, CD11b) M32-151( 自費 1100)
      • 趨化性試驗 (chemotaxis) M32-152
  • 34. Phagocyte function Adhesion Migration Phagocytosis chemotaxis Respiratory burst
  • 35. 睡著了嗎 ?
  • 36. Phagocyte evaluation
    • Adhesion:
      • Flow cytometry to measure CD11b/18, L-selectin
    • Migration:
      • Chemotaxis assays
    • Phagocytosis: E.coli
    • Respiratory burst:
      • Nitroblue tetrazolium (NBT) test
      • Superoxide O2 assay (chemiluminescence)
  • 37. NBT slide test
    • Control
      • NBT dye is reduced to formazan , deep blue-black color
    • CGD patient
      • Unable to reduce NBT and no deep blue color
  • 38. 非洲皇冠鶴 Hyatt Regency and resort Maui, Hawaii
  • 39. 自在 Hyatt Regency and resort Maui, Hawaii
  • 40. Swam Court, Hyatt Regency and Resort Maui, Hawaii
  • 41. Swam Court, Hyatt Regency and Resort Maui, Hawaii
  • 42. 吞噬細胞缺陷 Delayed detach of umbilical cord >1 m Perianal abscess formation Granulation tissue (CGD) 反覆口腔潰瘍 , 牙齦肥厚
  • 43. Pictures of CGD
  • 44. Pictures of Hyper-IgE syndrome (Job syndrome)
  • 45. 抗體缺損篩檢 :
    • 症狀
      • 反覆呼吸道 ( 鼻竇肺炎 ) 、腸胃道感染、慢性腸病毒腦膜炎、關節炎、無法解釋的支氣管擴張
    • 臨床評估
      • 淋巴腺及扁桃腺大小
    • 實驗室評估
      • IgG L72-205
      • IgA L72-207
      • IgM L72-209
      • IgG subclass (IgG1-G4) M32-330 ( 暫定自費 1800)
  • 46. B cell 功能
    • B cell quantification ( 數量 )
      • Quantitative IgG, IgA, IgM, IgE
        • IgA  if abnormal  IgG & IgM measurement
    • B cell function ( 功能 )
      • Isohemagglutinins (anti-A & anti-B Ab)
        • 主要測 IgM 的功能
        • AB type 無法測出 , 而且 <2y/o 也通常不表現凝集
      • Antibody titer to tetanus , diphtheria , haemophilus influenzae and pneumococci
        • If low, measure the titer of antibody before & 2wk after a booster
        • check IgG function
  • 47.
    • Laboratory diagnosis:
    • Other B cell defect (other…)
      • IgG subclass measurements
      • B cell numbers in peripheral blood (CD19, 20)
      • In vitro proliferation of B cells in response to anti-CD40 and IL-4
    B cell 功能
  • 48. 抗體缺乏 1. 出生 6 個月以後發生 2. 反覆呼吸道腸胃道感染 關節炎 Recurrent sinusitis Pseudomonas infection Ecthyma gangrenosum
  • 49. T 細胞免疫缺損篩檢
    • 症狀
      • 卡介苗感染、反覆鵝口瘡、 PCP 、反覆 CMV 等感染 .
    • 臨床評估
      • 胸腺及下巴大小
    • 實驗室評估
      • 遲發性過敏反應 (DTH) M32-318
      • 淋巴球分群 M32-328
      • 淋巴球變形反應 - 分裂原刺激 M25-015-Z
      • 淋巴球變形反應 - 抗原刺激 M32-808
      • 淋巴球活化標記 (CD40L) M32-155( 自費 1100)
      • 接受器缺乏檢查 (IL-12Rb1,IFN  R1,IFNrR2…) ( 自費 1100)
  • 50.
    • T cell quantification ( 數 量 )
      • Absolute lymphocyte count
    • T cell function ( 功能 )
      • Delayed type hypersensitivity (DTH)
        • Candida, PPD (5-10 IU), mumps, tetanus, control
      • Candida albicans intradermal skin test
      • 0.1 ml of a 1:20 dilution  > 6y/o 0.1 ml of a 1:10 dilution  < 6y/o  check erythema and induration at 48, 72 hr  > 5 mm: positive  If positive, 可初步排除嚴重的 primary T-cell defects
    T cell 功能
  • 51.
    • Laboratory diagnosis:
    • T cell deficiency (other…)
      • T cell surface markers
        • CD3,CD4,CD8,CD19,CD25,CD56 by FACS flow cytometry
        • CD40L (hyper-IgM)
      • Chromosome analysis
        • 22q11  R/O DiGeorge syndrome
      • Production of cytokines by activated lymphocytes
        • Th1/Th2/Treg cytokines
    T cell 功能
  • 52. 細胞免疫缺乏 Invisible thymus Oral thrush BCG infection Disseminated varicella infection
  • 53. Delay type hypersensitivity Candida PPD Toxoid Mumps
  • 54. Waikiki beach, Hawaii
  • 55. Waikiki beach, Hawaii
  • 56. Westin Resort, Hawaii
  • 57. Kalakawa Ave. Hawaii
  • 58. Hilo Hatis, Hawaii
  • 59. 補體免疫缺損篩檢
    • 症狀
      • 皮疹、關節炎、敗血症、血管水腫、自體免疫疾病症狀
    • 臨床評估
      • 血管水腫 , 關節腫脹
    • 實驗室評估
      • C3, C4, C6, C7, C8, CH50, AH50
      • 補體 C1q esterase inhibitor
  • 60.
    • Laboratory diagnosis:
    • Complement quantification ( 數 量 )
      • C3, C4
    • Complement function ( 功能 )
      • CH50, AH50 :
        • CH50  function of the classical pathway in serum by the rate of hemolysis of sheep red cells
        • AH50  function of the alternative pathway by lysis of rabbit or guinea pig red cells
      • Analysis of quantity and function of C-components
    補體免疫缺損篩檢
  • 61. 補體功能
  • 62. 補體缺乏 淋病性敗血症, 多發關節炎 關節炎 Skin rash C2, C4 rashes C3 敗血症 C6 , 7, 8, 9 淋病關節炎
  • 63. 個案討論
    • 十歲男童
    • 診斷 : CGD
    • 住院該住何種病房 ?
    • 護理人員須注意事項
    • 干擾素施打注意事項 , 劑量換算
  • 64. 歷屆考題  牛刀小試
    • 下列有關免疫系統之敘述,何者正確? (A)IgA 及 IgM 隨年齡增加而減少  (B) 體液性免疫由 T 細胞負責  (C) 抑制性 T 細胞會溶解感染的細胞  (D). IgG 可通過胎盤,含量也最多
    • 下列免疫球蛋白中,和第一型過敏有密切關係者為: (A) IgA   (B) IgD   (C). IgE   (D) IgG 。  (‘ 96 專高)
    • 下列何者不會導致免疫功能降低而產生伺機感染? (A) 白血病 (B). 心臟病  (C) 腎臟病引起尿毒症  (D) 惡性腫瘤。  (‘ 96 專高 )
    • IgE 抗體與下列哪一類過敏反應有密切關係? (A). 第一類,異位性  (B) 第二類,細胞毒性  (C) 第三類,免疫複合體性  (D) 第四類,延遲性。  (‘ 99 高考)
    • 結核菌素試驗( tuberculin test )之皮膚變化是屬於哪一型之過敏反應? (A) 第一型  (B) 第二型  (C) 第三型  (D). 笫四型。  (‘ 02 專高)
    • 經由細胞毒殺性抗體( cytotoxic antibody )媒介引起之過敏屬哪一型過敏反應? (A) 一  (B). 二  (C) 三  (D) 四。  (‘ 04 專高)
    • DiGeorge syndrome 缺乏何種細胞? (A) B 細胞  (B) 自然殺手細胞 (C). T 細胞  (D) 組織球。  ( '04 專高 )
  • 65.  
  • 66. Thanks for your attention and comment !!!