Vol.9 No.2 June, 2008

100 6 5 5081 9 0 8 1 6 6 1

T e l (02) 2311-4670Fax (02) 2311-4732ht tp: / /www.ai r .org. tw

19391392T e l (02) 2311-4670Fax (02) 2311-4732ht tp: / /www.asthma.org. tw

( ----1000-1500 )

Vol.9 No.2 June 2008 1

(Review of immuno-compromised system) ..... 2................................................ 8

PRACTALL ............................................... 9

(PRACTALL, 2008) ........................ 13Interferon gamma

pathway defect ....................... 14....................................... 16

...................................................... 18

......................................... 19....................................... 20

................................................................. 22Job's syndrome ........ 23

A

1. 7 12 7

2. 7 12 13

3. 7 19

4. 97 7 20 14 00-16 00

9

5. 8 2 3

6. 10 17 20

FIMSA

B

1. 22nd Congress of the European Rhinologic Society and the 27th

International Symposium of Infection & Allergy of the Nose, June 15,

2008 - June 20, 2008. Heraklion Greece. a.papavassiliou@frei.gr

2. Glucocorticoids & Mood: Clinical Manifestations, Risk Factors &

Molecular Mechanisms, June 20, 2008 - June 21, 2008. San Diego

United States. ocme@ucsd.edu

3. 9th Iranian Congress of Immunology and Allergy. June 28, 2008 -

June 30, 2008. Tehran Iran, Islamic Republic of. info@isiairan.net

4. XXXII Annual Congress of the Argentinean Assciation of Allergy &

Clinical Immunology - Interasma Regional Chapter. August 14,

2008 - August 16, 2008. Buenos Aires Argentina. aaaei@speedy.

com.ar

Vol.9 No.2 June 200822222

50 365 250

(Immune system)

(protozoa) (nonreplicant

agents)

(inherited primary) (acquired

secondary) ( ) (1, 2)

1952 Dr. Bruton

(X-linked agammaglobulinemia) 150

(primary immunodeficiency)

1960(2, 3)

(1) (humoral immunodeficiency)

(2) (cellular immunodeficiency)

(3) (combined immunodeficiency)

(4) (((((phargocytic defects)

(5) (complement deficiency)

(Review of immuno-compromised system)1 1 1

1 2

1 2

(opportunistic microorganisms)

(4)

(failure to thrive)

(persistent candidiasis) (absolute

lymphocyte count) 2,000/mm3

(2, 4)

(

mycobacteria salmonella)

(herpesvirus)

(natural killer cell dysfunction)

(Neisserial infection)

( t e r m i n a l c o m p l e m e n t c o m p o n e n t

deficiency) Pneumocystis

carinii (cellular immune

dysfunction) IgM (hyper-IgM

syndrome) (severe combined

immunodeficiency SCD) (deep-

seated abscess) (catalase-positive

organism . )

(chronic granuloma-

tous disease CGD)(2)

Vol.9 No.2 June 2008 33333

(major components)(1,2,5) B- T-

(complement)(3,4,6,7)

1. B- (B-cell mediated immunity)

(humoral immunity)

(B-cell mediated immunodeficiency) 50%

IgA (selective

IgA deficiency hypogammaglobulinemia)

(transient hypogammaglobuinemia

of infancy(THGI) (IgG sub-

class deficiency)

2. T- ((T-cell mediated immunity)

(cellular immunity)

(T-cell mediated immunodeficiency) 10%(2)

3. B- T- (combined

immunodeficiency) 20%

(severe combined immunodefi-

ciency SCID)

4. (Phagocytosis)

20% (chronic

ganulomatous disease CGD)

(Leukocytes adhesion defect LAD) IgE

(hyer IgE syndrome)(5, 6)

5. (complement system)

1-2%(4, 5)

(5)

B-

(plasma cell) IgG, IgA, IgM, IgE IgD

IgG 75% IgA 15% IgM 10% IgD

0.2% IgE 0.004% IgG IgA

IgM IgE

IgD (4)

IgG

8 IgG

IgG

5-10% (4) IgG

(heat-stable opsonin)

G(+) (4)

IgG (subclass) IgG1(70%) IgG2

(20%) IgG3(7%) IgG4(3%) IgG1 IgG3

(vaccine) IgG2 Ig4

(capsular polysaccharide)

IgG2

(Hemophilus influenzae) (S. pneumoniae)(8) IgG2

(4, 9)

IgM

75%

(heat-stable opsonin)IgM

G(-)

IgM IgM

IgM

IgA

IgA

( ) (1, 2, 5)

20%(4)

IgD IgM B- (a

membrane-bound antigen receptor on the surface of

B lymphocyte) IgD (4,

5)

IgE

(cytotoxicity)

(adenoid)

(half life) 2-3

14 (10)

T-

(helper function) B-

(macrophage)

(dentritic cells) (pathogen)

( ) (degrade) T-cells

(cell-surface) T- B-

cytokines B- T-

(defect) T- B-

T-

Vol.9 No.2 June 200844444

(aggressive opportunistic pathogens)

(virus) (fungus)(4, 10)

1) Cytomegalovirus(CMV) Epstein-

Bar virus(EBV) (severe varicella)

(chronic infec-

tion with respiratory & intestinal viruses)

2) (Candida)

3) (Protozoa) Pneumocystis carinii

T-

(sinopulmonary

infections)

1) (encapsulated)

(Streptococcus)

2) (Enteroviruses)

(Giardiasis)

(phagocytosis)

(phagocytic or granulocytic

defects)

(phargocytosis)

(granulocytes) superoxide-mediated killing

G( - )

(Pseudomonas)

(protozoal infection)

(Aspergilus), (Candida) (Norcardia)

(10)

(neutrophile)

(leukocyte adhesion defi-

ciency LAD) (1) ( 30

)

(flow cytometry) CD11a CD11b

markers for leukocyte adhesion molecule(6)

(complements defect)

(complement system)(

)

1) C1 qrs C4 C2

(lupus)

2) (pivotal pathway) C3

C5-9 C3

G(+) (encapsulated bacterial

infection) (S. pneumoniae)

(Hemophius. influenzae)

. C3

(SLE) C5,

C6,C7,C8 C9

(Neisseria)

(meningococcal meningitis)

(invasive gonococcal disese)(4,5)

3) (alternate pathway) C3b B

D

C3b(heat-labile)

(1) (2)

(3) (4) (angioedema)(5)

2-3

6-8 2

10 6-8

(failure to

thrive)

Vol.9 No.2 June 2008 55555

(4,11)

(IgG) 21-

23 (4,5,10) 3-6

(5)

(5,12)

Dr. Hollander(4),

1.

2.

3.

4.

5. (failure to thrive)

6. (recurrent deep

skin or organ abscesses)

7.

8.

9.

10. (primary immunodefi-

ciency)

(6,11)

1. CBC, ESR( )

(1, 4,

10) (absolute neutrophile

count)

(congenital or acquired neutropenia)

(leukocyte adhesion defect)

(absolute lymphocytes)

T- (severe T-cell defect)(1) Howell-Jolly

(congenital asplenism)

Wiskott-

Aldrich syndrome(1)

2.

(immunoglobulins) IgG, IgA, IgM IgE

3. IgG

(subclass) IgG2 encapsulated bacteria

4.

(titer)

2-3

B - (immunity)

(panhypogamma-

globulinemia) B T-

T- B

( X - l i n k e d

agammaglobulinemia) B- T-

(transient hypogammaglobulinemia THGI) com-

mon variable immunodeficiency(CVID) T-

B-

(severe combined immunodefi-

ciency SCID) CD4

(human immunodeficiency HIV) (4,8,13)

CBC

T-

B-

B- Ig, IgM IgG IgM

(AB AB

) IgG

2-3

B-

Vol.9 No.2 June 200866666

IgG 200-300 mg/dL(

100 mg/dL) IgG (2,5)

T-

T-

(Candida)

0.1 ml 1:1,000

(Candida extract) 24,

48 72 10mm

T-cell 1:100 0.

1 ml

1:100 T T- subpopula-

tions (flow cytometry)

CD2 CD3(mature T-cell) CD4(mature T-helper

cell) CD8(mature cytotoxic T-cell) CD11

(leukocytes) CD56(natural killer cell)

T- X-

1,500 cells/mL3

(lymphopenia) T-

(cell immunity deficiency)(1,4)

NBT(nitoblue

t e t r a z o l i u m ) ( 4 , 6 , 8 , 1 1 , 1 4 ) f l o w

cytometry assessment of respiratory burst using

rhodamine dye(repiratory burst assay)

G(-)

(phagocytosis)

(chronic granulomatous dis-

ease CGD) (leukocyte adhe-

sion deficiency LAD)(6)

CH50 (assay)

CH50 C3 C4 C4

(hereditary angioedema)

C1,C2,C3; factor 1 & factor H

(autoimmune disease)

(glomerulonephritis)

(systemic lupus erythromatosus)

(scleroderma) C5,C6,

C7,C8 (recurrent

neisserial disease)(4)

CBC

IgG IgA IgM IgE C1qrs C2 C3 C4 CH50

(((((total complement level)

90%

B-cell T-cell(1,2,5,10,

14)

B-cell

(Screening tests)IgG, IgM, IgA (Preexisting Ab)

(isoagglutinins)

(Advanced Tests)B cells (CD19 enumeration )

X-

T-cell (Screening tests)

X-

(Advanced Tests)T-cell (subset) CD3, CD4, CD8, CD40, CD16

mitogens

Cytokine

HIV

(Screening tests)

NBT

Neutrophile oxidation activity)

IgE

(Advanced Tests)(Flow cytometry) CD18, CD15,

(myeloperoxidase)

Vol.9 No.2 June 2008 77777

10. DeMera RS: Primary Immunodeficiencies, In

Naguwa SM, Gershwin ME. Allergy and Immu-

nity Secrets, Hanley & Belfus, Inc., Philadelphia

211-24, 2001

11. Kidon MI, Handzel ZT, Schwartz R, Altboum I,

S t e i n M & I s r a e l Z B . S y m p t o m a t i c

hypogammaglobulinemia in infancy and

childhoo-clinical outcome and in vitro immune

response. BMC Family Practice 5 23-9, 2004

12. Yasuno T, Yamasaki A, Maeda Y, Fujiki A &

Yagyu S. Atopic dermatitis and transient

hypogammaglobulinemia of infancy improved

simultaneously. Pediatrics international 49

406-8, 2007

13. Wang LJ, Yang YH, Lin YT, Chiang BL. Immu-

nological and clinical features of pediatric pa-

tients with primary hypogammaglobulinemia in

Taiwan. Asian Pacific Journal of Allergy and im-

munology 22 1-31, 2004

14. Wang HC, Whelan MA, McGeady SJ, Yousdf E.

A 5-month-old boy with recurrent respiratory

infections, failure to thrive, and borderline el-

evated sweat chloride levels. Allergy & Asthma

Proceedings 27 285-8, 2006

Cytokine assay(interferon gamma)

(biopsy)

(Screening tests) Total (CH50) activity, C3, C4 level

(Advanced Tests)C2 ,C4, C5, C6 levels

(opsonization)

Reference

1. Buckley RH: The immunologic System and

Disorders. Nelson Textbook of Pediatrics 17th ed.,

Philadelphia, Sander 681-90, 2004

2. Morimoto Y, Routes JM: Immunodeficiency

Overview. Primary Care: Clinics in Office Practice

35(1) 159-73, 2008

3. Stiehm RE. The four most common pediatric

immunodeficiencies. Adv Exp Med Biol 601 15-

26, 2007

4. Hollander GA, Fasth A :Immunity, In: Polin R.A,

Ditmar M.F. Pediatric Secrets 4th ed, Philadelphia,

Elsevier Mosby 295-390, 2005

5. Fireman P: Primary Immunodeficiency Disease.

Mosby Elsevier, Philadelphia 329-49, 2006

6. Verma S, Sharman PK, Sivanandan S, Rana N, et.

All. Spectrum of Primary Immune Deficiency at

Tertiary Care Hospital. Indian Journal of Pediat-

rics 75 53-8, 2008

7. Whelan MA, Hwan WH, Beausoleil J, Hauck

WW, McGeady SJ. Infants presenting with recur-

rent infections and low imunoglobulins: charac-

teristics and analysis of normalization. Journal of

Clinical Immunology 26 7-11, 2006

8. Hsueh KC, Chiu HH, Lin HC, Hsu CH, & Tsai

FJ. Transient hypogammaglobulinemia of infancy

presenting as Staphyylococcus aureus sepsis with

deep neck infection. J Microbiol Immunol infect

38(2) 141-4, 2005

9. Atkinson AR, Roifman CM. Low Serum Immuno-

globulin G2 Levels in Infancy Can Be Transient.

Pediatrics 120 e543-e547, 2007

Fireman P: Complements casade. Atlas

of Allergies & Clinical Immunology 3rd edi., Mosley

Elsevier 344, 2006.

Vol.9 No.2 June 200888888

(

)

:

relievers

controllers

morning meeting

subspecialist training

fainting

location

(

fainting)

berotec inhalation

bosmin intubation

favor regular follow-up

compliance in-

different

outcome factors

intention just do our job

3S(serotide, symbicort, singulair),

(for mild ~ moderate persistent asthma)

117A 110A

data

aging

Vol.9 No.2 June 2008 99999

(

correlation) GI subspecialist

index

made right choice

( )

10-15

5-6 .Reference

1.

2. Ken blog

(emollients)

( )

(PH 5.5

~6.0)

calcineurin inhibitor corticosteroid

PRACTALL

cyclosporin A( )

Vol.9 No.2 June 20081010101010

stepwise management of patients with

AD

polidocanol

urea

(prednicabate, mometasone furoate,

fluticasone and methylprednisolone aceponate)

:

fluticasone

TCIsTCIs(topical calcineurin inhibitors):

pimecrolimus(1%) tacrolimus(0.03%)

Tacrolimus(0.1%)

0.1% tacrolimus

1%pimecrolimus pimecrolimus

tacrolimus

TCIs

0.03%tacro l imus 1%

pimecrolimus

TCIs

TCIs

triclosan

chlorhexidine

Triclosan

fusidic acid

MRSA

mupirocin yeast dermatophyte

streptococci

(

)

clidamycin

Vol.9 No.2 June 2008 1111111111

fusidic acid

MRSA

(maintenace therapy)

acyclovir valcyclovir

Cyclosporin ATCIs, cyclosporin A calcineurin-de-

pendent pathyway IL-2

IFN- Cyclosporin A

Cyclosporin A

(3~5mg/kg/day) (2.5mg/

kg/day)

AzathioprineAzathioprine

azathioprine thiopurine

methyltransferase

Azathioprine

1~3

Azathioprine

2~3

UVB(280~320 ) UVB

(311~313 ) UVA(320~400 ) UVA1

(340~400 ) UVB UVB

UVA UVA1

12

( TNF inhibitor)

T (alefacept

efalizumab)

IgE

50%

( )

pimecrolimus

1. Akdis et al. Diagnosis and treatment of atopic der-

matitis in children and adults: European Academy

of Allergology and Clinical Immunology/American

Academy of Allergy Asthma and Immunology/

PRACTALL Consensus Report. J Allergy Clin

Immunol 118 152-69, 2006

Vol.9 No.2 June 2008 1313131313

(PRACTALL, 2008)

(European

Academy of Allergy and Clinical Immunology)

(American Academy of

Allergy Asthma and Immunology)

PRACTALL consensus

Natural History

wheezing

1. Transient wheezing 2 3

3

2. Nonatopic wheezing

3. Persistent asthma (

)

total IgE

IgE

4. Severe intermittent wheezing

Diagnosis and Treatment

IgE

(reliever) (controller)

(reliever)2

(controller) (ICS)

(leukotriene receptor

antagonist, LTRA)

2 (LABA)( ICS )

theophylline

IgE

Cromolyn sodium

PRACTALL

Inhaled corticosteroid (ICS) 30

Vol.9 No.2 June 20081414141414

(

)

ICS(beclomethasone 800 g

)

(hypothalamic-pituitary-adrenal axis)

(LTRA)

2 (LABA) ICS

(allergen immunotherapy)

PRACTALL

1. 2 10

20 2 4

2. 2 2.5 5 mg salbutamol

20 30

3.

4. prednisolone 1-2 mg kg

3

5. 2

6.

aminophylline 20 6 mg

kg

1. Bacharier et al. Diagnosis and treatment of

asthma in childhood: a PRACTALL consensus

report. Allergy 63 5-34, 2008

Interferon gammapathway defect

Vol.9 No.2 June 2008 1515151515

(mastoiditis)

Rifampin

Ethambutol Isoniazid

Clarithromycin

X

Alkaline-P

methicillin-resis-

tant Vancomycin

Gentamicin

(Acid fast stain)

B T

partial interferon gamma re-

ceptor 1 deficiency Myco-

bacterium avium complex

T

(1)

(phagocyte)

Interferon gamma

(INF- )/IL-12 pathway

(1,2)

IFN- /IL-12 deficiency

( )

(Histoplasma capsulatum)(3)

Interferon- pathway defect

INF- R1 deficiency INF- R2 deficiency

STAT1 deficiency IL-12 p40 deficiency IL-

12RINF- 1 deficiency(4-6) Interferon- pathway defect

Mendelian Susceptibility to Mycobacterial Disease

(MSMD)(4)

INF- R1 INF- R2

INF- R1 4 base

818 (6)

IFN- R1 IL-

12RINF- 1 and IL-12p40 ,

IFN-

azithromycin clarithromycin(1)

References:1. Rosenzweig SD, Holland SM. Phagocyte immuno-

deficiencies and their infections. J Allergy Clin

Immunol 113 620-6, 2004

2. Han JY, Rosenzweig SD, Church JA, Holland SM,

Ross LA. Variable presentation of disseminated

nontuberculous mycobacterial infections in a fam-

Vol.9 No.2 June 20081616161616

ily with an interferon-gamma receptor mutation.

Clin Infect Dis 39 868-70, 2004

3. Zerbe CS, Holland SM. Disseminated histoplas-

mosis in persons with interferon-gamma receptor

1 deficiency. Clin Infect Dis 41 e38-41, 2005

4. R, Altare F, Casanova JL. Genetic het-

erogeneity of Mendelian susceptibility to mycobac-

terial infection. Microbes Infect 2 1553-7, 2000

5. Remus N, Reichenbach J, Picard C, et al. Im-

paired interferon gamma-mediated immunity and

susceptibility to mycobacterial infection in

childhood. Pediatr Res 50 8-13, 2001

6. Jouanguy E, Lamhamedi-Cherradi S, Lammas D,

et al. A human IFNGR1 small deletion hotspot

associated with dominant susceptibility to myco-

bacterial infection. Nat Genet 21 370-8, 1999

( )

?

(Intrauterine sensitization)

[1]

Derp 1 [2]

IgE[3] total IgE

[4] allergen-specific IgE

allergen-specific T

[5][6]

[7]

4

6 9

2008 3 Journal of Allergy and

Clinical Immunology Sensitization does not

develop in utero[8]

Klaus

specific IgE

IgE [8]

411 243

ImmunoCAP

total IgE specific IgE ( Milk

egg Dermatophagoides pteronyssinus cat

dander dog dander birch timothy mugwort

peanut) IgA

243

milk egg specific IgE 34(14%)

mixed allergens specific IgE

mixed specific IgE

22 36 single-allergen

IgE (35 )

single-allergen IgE

22 21

Specific IgE

specific IgE

specific

IgE (a) specific

Vol.9 No.2 June 2008 1717171717

IgE (b) specific IgE

(c) total/specific IgE

1:1 specific

IgE IgE

IgE

specific IgE IgA

IgA

IgA IgE

IgA IgE

IgE

TH2

[9] TH

[10]

37% specific IgE

specific IgE [11]

4 5

total IgE [12]

specific IgE

1. C.A. Jones, J.A. Holloway and J.O. Warner, Does

atopic disease start in foetal life?, Allergy 55 2-

10, 2000

2. J.A. Holloway, J.O. Warner, G.H. Vance, et al.,

Detection of house-dust-mite allergen in amniotic

fluid and umbilical-cord blood, Lancet 356

1900-2, 2000

3. J.O. Lima, L. Zhang, T.P. Atkinson, J. Philips, et

al . . , Early expression of iepsi lon, CD23

(FcepsilonRII), IL-4Ralpha, and IgE in the hu-

man fetus, J Allergy Clin Immunol 106 911-7,

2000

4. G. Edenharter, R.L. Bergmann, K.E. Bergmann,

et al., Cord blood-IgE as risk factor and predictor

for atopic diseases, Clin Exp Allergy 28 671-8,

1998

5. M. Nambu, N. Shintaku and S. Ohta, Relation-

ship between cord blood level of IgE specific for

Dermatophagoides pteronyssinus and allergic

manifestations in infancy, Biol Neonate 83 102-

6, 2003

6. S.L. Prescott, C. Macaubas, T. Smallacombe, et

al., Development of allergen-specific T-cell

memory in atopic and normal children, Lancet

353 196-200, 1999

7. J.O. Hourihane, R. Aiken, R. Briggs, et al., The

impact of government advice to pregnant mothers

regarding peanut avoidance on the prevalence of

peanut allergy in United Kingdom children at

school entry, J Allergy Clin Immunol 119

1197-202, 2007

8. Klaus , Christian Bressen Pipper, Hans

Bisgaard, et al., Sensitization does not develop in

utero, J Allergy Clin Immunol 121 646-51, 2008

9. J. Rowe, M. Kusel, B.J. Holt, et al., Prenatal ver-

sus postnatal sensitization to environmental aller-

gens in a high-risk birth cohort, J Allergy Clin

Immunol 119 1164-73, 2007

10. C.A. Thornton, J.W. Upham, M.E. Wikstrom, et

al., Functional maturation of CD4+CD25+

CTLA4+CD45RA+ T regulatory cells in human

neonatal T cell responses to environmental anti-

gens/allergens, J Immunol 173 3084-92, 2004

11. E. Bertino, C. Bisson, C. Martano, et al., Rela-

tionship between maternal- and fetal-specific

IgE, Pediatr Allergy Immunol 17 484-8, 2006

12. G. Lilja, S.G. Johansson, E. Kusoffsky , et al.,

IgE levels in cord blood and at 4-5 days of age:

relation to clinical symptoms of atopic disease

up to 18 months of age, Allergy 45 436-44,

1990

Vol.9 No.2 June 20081818181818

(autoantibodies)

(antinuclear

antibodies) 95% 99%

syndrome

scleroderma mixed connective-tissue disease

Tan 1:40

20-60 31.7%

13.3% 1:80 5.0% 1:160 3.3%

1:320 1:40

97.4% 68.

3% 1:160

94.7%

95.0%

< 1:40

1:160

Arbuckle 88%

3.3 9.4

78% ( 1:120) 55%

DNA 47% anti-Ro 34%

anti-La 32% anti-Sm 26% anti-

nuc lea r r i bonuc leopro te in 18%

antiphopholipid

anti-Ro anti-La

antiphopholipid

( 3.4 ) anti-Sm anti-nuclear

ribonucleoprotein

DNA

Nielen

(49%) 0.1-13.8 ( :

4.5 ) IgM rheumatic factor

anticyclic-citrullinated peptide (anti-CCP)

75%

1.1% IgM rheumatic factor

0.6% anti-CCP

DNA anti-Sm

anti-CCP

islet

cell antibodies(ICAs) insulin autoantibodies

(IAAs) anti-glutamic acid decarboxylase antibodies

(GADAs) anti-tyrosine phosphatase-like protein

autoantibodies (IA-2As)

50%

Vol.9 No.2 June 2008 1919191919

DNA anti-nucleosome anti-

C1q

1. Tan EM, Feltkamp TE, Smolen JS, et al. Range of

antinuclear antibodies in "healthy" individuals. Ar-

thritis Rheum 40 1601-11, 1997

2. Kupila A, Muona P, Simell T, et al. Feasibility of

genetic and immunological prediction of type I

diabetes in a population-based birth cohort.

Diabetologia 44 290-7, 2001

3. LaGasse JM, Brantley MS, Leech NJ, et al. Suc-

cessful prospective prediction of type 1 diabetes in

schoolchildren through multiple defined

autoantibodies: an 8-year follow-up of the Wash-

ington State Diabetes Prediction Study. Diabetes

Care 25 505-11, 2002

4. Arbuckle MR, McClain MT, Rubertone MV, et al.

Development of autoantibodies before the clinical

onset of systemic lupus erythematosus. N Engl J

Med 349 1526-33, 2003

5. Rantapaa-Dahlqvist S, de Jong BA, Berglin E, et

al. Antibodies against cyclic citrullinated peptide

and IgA rheumatoid factor predict the develop-

ment of rheumatoid arthritis. Arthritis Rheum

48 2741-9, 2003

6. Nielen MM, van Schaardenburg D, Reesink HW,

et al. Specific autoantibodies precede the symp-

toms of rheumatoid arthritis: a study of serial

measurements in blood donors. Arthritis Rheum

50 380-6, 2004

7. Scofield RH. Autoantibodies as predictors of

disease. Lancet 363 1544-6, 2004

8. Reveille JD. Predictive value of autoantibodies for

activity of systemic lupus erythematosus. Lupus

13 290-7, 2004

9. Yurasov S, Tiller T, Tsuiji M, et al. Persistent ex-

pression of autoantibodies in SLE patients in

remission. J Exp Med 203 2255-61, 2006

peak expiratory flow

1 2

1 2

2004

97 (control) 99

(intervention)

6 12

Vol.9 No.2 June 20082020202020

(Peak flow meter)

( ) (

)

USB

(http://140.116.58.191/asthma/first.php)

12 4

12

Pediatric Asthma Quality of Life

Childhood Asthma Control Test

5

5

DPI MDI Spacer

88

76

reliever

well-controlled

88

12

1. Ren-Long Jan, Jiu-Yao Wang, Mei-Chih Huang,

Shih-Mu Tseng, Huey-Jen Su, Li-Fan Liu. An

Internet-based interactive telemonitoring system

for improving childhood asthma outcomes in

Taiwan. Telemed J E-Health 13 257-68, 2007

(guideline)

4 12

7

(Childhood asthma control test,

1 2

1 2

CACT) 0 27

19

(inadequately controlled asthma)

Chinese CACT

C-CACT(http://www.gsk.tw/YourHealth/

health-8-2.shtml)

Vol.9 No.2 June 2008 2121212121

(reliability) (validity)

C-CACT

0 3

0 5 27

4 11

4

8

PEFR IgE MAST

GINA severity

(not controlled, poorly controlled,

somewhat controlled, well controlled and completely

controlled) C-CACT

physician evaluation score

(PES) GINA se-

verity Intermittent

group persistent group C-CACT

reliability internal consistency test-re-

test method criterion validity

PES percent predicted PEFR C-CACT

correlations discriminate validity C-

CACT PES percent predicted PEFR

ratio therapy recommendation

C-CACT

baseline 247

follow-up 209

intermittent group 84 persistent group 125

Total IgE Der

P C-CACT PES

Predicted percentage of PEFR persistent group

baseline reliabil-

ity 0.741 follow-up 0.759 test-retest

reliability r2=0.618(P<0.001) C-CACT

PES (r2=0.610, P<0.001)

persistent group C-CACT PES

(r2=0.699, P<0.001) PEFR C-CACT

(r2=-0.02, P=0.756)

PEFR

Discriminate validity

(1)not controlled; (2)poorly controlled;

(3)somewhat controlled; (4)well controlled; (5)com-

pletely controlled

C-CACT F statistic 17.10(P<0.01)

therapy recommen-

dation (1)stepped down; (2)no change; (3)

stepped up C-

CACT Fstatistic 39.61(P<0.001)

percent predicted PEFR

(1)<60%; (2)60-79%; (3)80-100%; (4)>

100% C-CACT

F statistic 0.88

receiver-operating characteristic curve

cut-point score 20 persistent group

sensitivity 43.2% specificity 81.1%

follow-up

(1)worse; (2)same; (3)better by one rating level; (4)

better by two rating levels follow-

up percent predicted PEFR <10% improve-

ment >=10% improvement C-

CACT correlation

C-CACT (r=0.536, P<0.

001) percent predicted PEFR (r=0.

097, P=0.44)

C-CACT

(F=15.76, P<0.001) percent predicted

PEFR (F=3.46, P=0.048)

C-CACT reliability validity

PEFR

12 C-CACT

C-CACT

1. Hsin-Hung Chen, Jiu-Yao Wang, Ren-Long Jan,

Yi-Hsun Liu, Li-Fan Liu. Reliability and validity of

childhood asthma control test in a population of

Chinese asthmatic children. Qual Life Res 17

585-93, 2008

Vol.9 No.2 June 20082222222222

Dr. Galant Dr.

Galant

E-mail linkoala@COX.NET

Eosnophilia Esophagitis( )

Gastro-Esophageal Reflux Disease, GERD(

)

..

E type T type IV

prick patch

prick patch (

patch

)

(

)

~

Dr. Spergel

Clinical Gastroenterology and Hepatology 2005 3

1198-1206

(

)

Vol.9 No.2 June 2008 2323232323

So went Satan forth from the presence of the

Lord, and smote Job with sore boils from the sole of

his foot unto his crown (Job 2:7).

( 2 7)

Job's syndrome

STAT3

STAT(Signal Transducer and Acti-

vator of Transcription) Janus kinases(JAKs)

(cytokines)

JAK STAT

STAT3 IL-6 IL-

10

STAT3

STAT3

(2006)

hyper IgE syndrome

JAK TYK2

TYK2 STAT3

NIH Dr. Holland

STAT3 dominant negative hyper IgE syn-

drome 1966

Job's syndrome

STAT3 dominant negative

Job's syndrome TYK2-STAT3

( ) 1960

IgE ( )

(Job ' s s yndrome, hyper IgE

syndrome)

B (IgE )

Vol.9 No.2 June 20082424242424

hyper IgE syndrome

NIH

STAT3 Th17

(IgE T

)

Th17 Th1 Th2 CD4

T Th17

(co ld

abscess) Th17

Job's syndrome

IL-6

Th17

( )

References

1. Milner JD, Brenchley JM, Laurence A, Freeman

AF, Hill BJ, Elias KM, Kanno Y, Spalding C,

Elloumi HZ, Paulson ML, Davis J, Hsu A, Asher

AI, O'Shea J, Holland SM, Paul WE, Douek DC.

Impaired T(H)17 cell differentiation in subjects

with autosomal dominant hyper-IgE syndrome.

Nature 452(7188) 773-6, 2008 Apr 10

2. Holland SM, DeLeo FR, Elloumi HZ, Hsu AP,

Uzel G, Brodsky N, Freeman AF, Demidowich A,

Davis J, Turner ML, Anderson VL, Darnell DN,

Welch PA, Kuhns DB, Frucht DM, Malech HL,

Gallin JI, Kobayashi SD, Whitney AR, Voyich JM,

Musser JM, Woellner C, Schaffer AA, Puck JM,

Grimbacher B.STAT3 mutations in the hyper-IgE

syndrome.N Engl J Med 357(16) 1608-19,

2007 Oct 18

3. Bustamante J, Boisson-Dupuis S, Jouanguy E,

Picard C, Puel A, Abel L, Casanova JL.Novel pri-

mary immunodeficiencies revealed by the investi-

gation of paediatric infectious syndromes.Curr

Opin Immunol 20(1) 39-48, 2008 Feb

. Dr. Steve Holland NIH

Hyper IgE syndrome

STAT3 Th17

(CGD) NIH

Dr. Steve Holland

NIH STAT3 ( )

Th17

Th17

STAT3 Job's syndrome