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2005 1 25 1 Basic & Clinical Medicine January 2005 Vol. 25 No. 1, 2005, : 1 00126325 ( 2005) 012086204 (1), (, 100730) 1,,48, 2, 9 5 7,, 1116 10 9 L - 1 8711 % 101 g/ L, 910 mg/ dl ( 796 mol/ L ) 6411 mg/ dl (2219 mmol/ L) 319 mmol/ L, ph 71354 Pa2 111 CO 2 1617 mmhg ( 1 mmhg = 1331322 Pa ) PaO 2-2002 7,3 4 / 8515 mmhg HCO 3 911 mmol/ L, 0 2/,300 450 ml/, HP 6 10/ HP ( + ),,,,,,, (ARF), 2 15 kg, 2003 8, 117 mmol/ L, ( 112 ),, 3716, 64 /, 20 /,, 210 mmol/ L, 100/ 60 mmhg, 2004 2 28,7 12 /,150 200 ml/,,, 4 mm, 119 mmol/ L, 66 mol/ L, 24 h 1 500, 2 000 ml, 1 000 2 000 ml/,,,, 410 mmol/ L 134 mmol/ L 111 mmol/ L,,, ( + ) CT, 3 1, 5 /,200 ml/ ;3 2, 113 3 000 5 000mL/ d, 20 mg/ d ;, 1 000mL/ d ;3 4 114 (VIP ) 100 mg 1 / 8 h, (3 11 ) 100 ml/ d, 3 7 400mL,,3 6,,, 180 mg/ d,,,,3 640 mol/ L, 18109 mmol/ L, ph 71261 HCO - 31 1 600mL/ d, 3 712 mmol/ L 3 :2004-10 - 06 :2004-11 - 03 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005125 (1) Basic & Clinical Medicine 87, C4 1 619 %, 2 1412 %,, C,ANA dsdna 3 15,, ENA ANCA ( - ) CA19-9 CEA,, CA50 CA242 ; CA125 8112 U/ ml B 1, B 12 3 21 37 mmhg,,,, 116, 2 L/ min ph 714, PaCO 2 3814 mmhg, PaO 2 6811 mmhg, HCO 3-2318 mmol/ L D2 ( D2dimer ) 015 g/ L V/ Q,,V/ Q, 3 26 115 11511 : : (1116 8159) 10 9 L - 1, 8711 %, 101,T2, 82 g/ L, (295 407) 10 9 L - 1 : MRI, 11015 110 310 g/ L,, : 10 15/ HP, 100 %, CT 10 15/ HP, : CT, 40 g/ L, 3134 mmol/ L, 9142 mg/ dl ( 833 mol/ L ), 26 mg/ dl ( 913 mmol/ L ), 4 cm CT, 1011 mg/ dl (2153 mmol/ L) : (1) :, (2) 11512 : : 50 %, ( - ) (ACTH), 3214 ng/ L 25 ng/ L 219 mu/ L, (PTH) 1115 g/ L, MRI,MRI B CT, CT (3) :,,, MRI 212 ( ), 612 cm 310 cm, 11513 :, B CT CT,,,, (ATN) ATN 11514 :3, :, ; ATN : ; CT, ;,,, 11515 : 57 mm/ 1h CH50 5718 U/ ml,c3 ; ; ; Adie s? ; ; ; ; 2 211 ( ) MRI T1,, 515 cm ATN 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

88 Basic & Clinical Medicine 2005125 (1) 213 ( ), VIP 1958 Vern2 (1) :ARF, ATN, er Morrison, : ; (2) : 2 ( /,, 7 000mL/ d), ph 210,, achlorhydria syndrome, WDHA) 50 ; (3) :,,,80 % 90 %,V/ Q ; (4),50 % 70 % :,10 % VIP ; (5) : :, :, (1) : 7 400mL/ d ; (2) : 15 kg ; (3),, ;, (4) : 612 cm 310 cm ARF,,CT, ARF,,,,, (1) VIP : ; (2) : Whip2 ple, ; (3) :,, ( ACTH ; CT ), MRI, 214 ( ),,,,,MEN2 MEN,, : (1) < 100 ml/ d, : > 1 000 ml/ d, 50 150 g, 2 ( 48 h, > 500 ml/ d) 3 (1) 20 (2), (2) (3) 52Fu, (4),,CT, (5) : VIP VIP, ;,, ;,,40 % 50 %, 3, VIP 14,,,, ph 210,,, 30 %,,, 216 ( ), : ;, 215 ( ) ) ( watery diarrhea hypokelamia, PTH, ; ;,,, ; VIP, PHM( ; 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005125 (1) Basic & Clinical Medicine 89, F MRI,,, 219 ( ) ( + ), (, ), PaCO 2 3916 mmhg,, CT,,,,UCG : (1),, V/ Q, SLE (2), 40 %, V/ (3) (4) Q 50 %,UCG (,, ),, : (1) (2) :, (3), 217 ( ), 2110 ( ),,,,, - CT 218 ( ),, (CCK ),CCK,,,,,, : 90 db, 55 db ; 100 db, 55 db : B, 2111 ( ) CT:,, ATN,,, :, B CT, ;, :, ;, ( ), 3 2004 5 14, 1, ;,,, ;,, 300, 1 500 ml/ d, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005 2 25 2 Basic & Clinical Medicine February 2005 Vol. 25 No. 2 : 100126325 (2005) 0220185204 (2) 1, 2 2, ( 11 ; 21, 100730) 1,,18, 1, ( 3), 3 111 1,, 1, 3,,,, CT MRI 10 ANA ( + ) 1 : 160 ( ), dsdna : ( + ) 48199 % ( Farr ), ( - ) ( IF ), 65 mm/ h, C HIV2Ab ANCA ENA ( - ) 2, 70 %, : PaO 2 264 mmhg ( 1mmHg = 1331322Pa ), PaCO 2 61 mmhg, 100 % : ( 59 mmhg), EF 70 % CT :, 5 cm 7 cm, ;, 300 mg/ d, 15 kg 112 T 3711, P 120 /, R 26 /, BP 90/ 60 mmhg,, ( 1) ( 2) ( + ),, 1 2,, 3 :2005-01 - 12

186 Basic & Clinical Medicine 2005125 (2) 113,, :,, : (PNP), :, ; PNP ;, (BO),, PaCO 2 102 mmhg, IVIG 20 g/ 300 mg/ BiPAP,, 4 CT,,,, ; ; CT,, ;,, 114 115 :WBC (5117 7175) 10 9 L - 1, PNP 4218 4816 %, 911 1815 %, 113 133 g/ L, 318 381 10 9 L - 1 ( - ), ( PaCO 2 6312 102 mmhg ) : 30 mm/ 1h ANA : ( + ) 211 ( ) 1 160 dsdna : ( + ) 48199 % ( Farr ), ( - ) ( IF ) C3 C4 CH50, ScL270 Jo21 1, ; 3, ( - ), ;, ( - ) CT:, 1, ; CT, ( 4) CT + ; : ( ) : ( + ) 1 1280 ( IIF, / / AIDS (SLE ) : ),,, PNP, PNP, PNP, : (1) : ; PNP : ; ( IP) ; (2) IgG IgM IgA C3 2 BO : ; : ( IIF) ;

2005125 (2) Basic & Clinical Medicine 187 (DIF) IgG C3 ;, ; (3), :3 2 + 2, IIF PNP PNP PNP, :desmoplakin I BP Ag1 desmoplakin envoplakin Castleman, periplakin HD1/ Plectin PNP, PNP 5 PNP,,, IVIG,, 212 ( ) CT: ;,,, ;,, CT,,, ; Castleman,, Castle2 man,,,, PNP,,, : (1) : Castleman 213 ( ),, ( ) PNP, Anhalt ; (2) :, 1990 5, PNP : PNP : (1) ;, : (90 %),,,, ; (2) : 214 ( ) : (1) (1) : ; (2), ( PaCO 2 ; (2),PaO 2 ) ; (3) ; (4) ; (3) ; (4), (1) : IgG C3 Castleman,Castleman PNP ; (2) : IgG BO BO, PNP ; Dsg3 IgG Dsg3, Plakin Plakin IgG, (DP I&,ENV,PER,BP230 ) 5 PNP, 1 2

188 Basic & Clinical Medicine 2005125 (2) ;, ;, (,, ), ; BO, 014 019 mm, Castleman (60 % 90 %), 217 ( ),,, ; 1 6 mm, Sm, 10 %,,, 3 % 5 %, BO, SLE dsdna,,,20 %PNP 60 % 80 %, SS MCTD AIH,, SSA SSB Takahashi 1, SLE PNP,, ANA, BO, ANA PNP BO dsdna ssdna,, B BO,,,,, - 1, (Dsg - 1) 23 (Dsg23), 215 ( ) PNP,, ANA, Castle2, : man Castleman (1), ; (2) ANA,, SLE,, ; (3) dsdna Farr, IF, T, IF SLE ; (4) B Ig,, ; (5) (CTD) SLE 216 ( ) 218 ( ), Castleman,, CTD,,, Castleman, CTD, dsdna,,

2005 3 25 3 Basic & Clinical Medicine March 2005 Vol. 25 No. 3 : 100126325 (2005) 0320285204 (3), ( 100032) 1 B :, 3 19, CEA CA199 ; 111 : (,,27 ), : 3 4 27 37 cm 2001 2,,,, ;, CT 2001 4 B Castleman 5173 cm 5143 cm,,, 3147 cm 2197 cm, 1175 cm, 3 25 kg 117 cm,, 112, MRI, 110/ 60 mmhg(1 mmhg = 1331322 Pa), 2001 4 27,, 1 cm, 1 cm,,,,, 3 cm 3 cm, ;, 3 cm, ; ( - ),, 113, 2003 6, ; Hb Castleman 54 g/ L 2004 2 114, 2 10,, 2 h,,, B,, 400 ml,, 115, 11511 : : RBC ( 2171 3141) 10 12 L - 1,Hb 52 70 g/ L, WBC (3186 5101) 10 9 CT,, L - 1,PLT(160 207) 10 9 L - 1, 2102 %,,, :BIL 1 + PRO UBG 66 mol/ L LEU :2004-11 - 18 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

286 Basic & Clinical Medicine 2005125 (3) + OB ( - ) 40 mm/ h (5, 26 ) : ALT 45 U/ L AST 45 U/ L TP 619 g/ dl ALB 310 g/ dl A/ G 018 TBIL 2131 mg/ dl DBIL 1117 mg/ dl GGT 177 U/ L ALP 479 U/ L TBA 1317 mol/ L, Cr 0183 mg/ dl BUN 1411 mg/ dl, ( 5 31 ) : ALT 41 U/ L AST 41 U/ L TP 612 g/ dl ALB 218 g/ dl TBIL 1170 mg/ dl DBIL 0196 mg/ dl GGT 163 U/ L ALP 437 U/ L TBA 3714 mol/ L : HbsAb ( + ), ( - ) HIV HCV( - ) CRP AFP, 11512 : : 18 g/ dl,, 392 g/ dl 5 %, WBC 4 ng/ ml :517 ng/ ml B12 :253 pg/ ml ESR CRP ; :, ;CT,,, : (1) : ( 11513 : ANA ds2dna ENA ANCA 70 % 90 % ), ( - ), : AMA 1 : 80, ; ;CT ( - ) Ig : IgA 4111 g/ L IgM IgG ;, 11514 : CT ;,, 7, 4 4 11515 : 4 3 CT 4,, 5 (B ) (2) :, 116 : Castleman, (3) : 2 211 ( ) Weber2Christian,,,,,, (4) : (5) : AMA 1 80, (6) :, :,,, Castleman ( ) ; Castleman : Hb 52 g/ L, ESR,, 40 mm/ h ;, ;, ;, B :,,,,, Castleman, ; CT:,,, :,,,, ( 30 %), : : (1) ; 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005125 (3) Basic & Clinical Medicine 287 (2),,, ; (3), TCR IgH, Castleman,90 % ; (4) ; (5),,,,, ;10 % 212 ( ) : ;,,, ; 2001 4 CT, Castleman :, CT,,,,,, ; 2 % 20 % ; Ig M,, ESR,ANA RF Coombs ;,, (CT, Castleman ),, Castleman,, ; ( ) AIDS Castleman Castleman ;, Caseleman ;,, + ;, ASCT,,,,, ;,,,, ;,, Castleman 213 ( ), 214 ( ), ;,,,,,,,, :Castleman,, :,,, Castleman, 2 : (1) ( 80 % 90 %),,, 215 ( ), ;, (2) ( 10 % 20 %),,,, SS TTP ; POEMS,,,,,, ;, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

288 Basic & Clinical Medicine 2005125 (3) Castleman,,,,, 216 La wrence ( ),, ; 3 ;,, 200 mg, ; 1, 012 mg 2,,, 10, 217 ( ) 014 mg,, 10 mg 2,, (, 100005) 2004,, 2 : ( RNA ) /,, 90 %,, :,,,, ( ),,, : ( ),,,,,, ( ) 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005 4 25 4 Basic & Clinical Medicine April 2005 Vol. 25 No. 4 : 100126325 (2005) 0420376205 (4),, (, 100730) : R5 :A 1 : WBC 25/ L RBC 25/ L : 103mm/ 1h ANA ( - ) : ANCA 1 : 40 Ig ( - ) :, C5 7 2004 4 22,, C5 6 6 7, 5 kg / :, 112 37, 130/ 75 mmhg,,,, 68,, 1 111, ( ) ( - ), 2004 2,,, CT MRI, III, ( ), 113 3718 39,,, ( Gaint Cell Arteritis, GCA)?, 114 ( ), ( : WBC ( 8139 10131) 10 9 L - 1, N (75 8014) %, RBC (3139 3177) ),, 3 20 (100 116) g/ L, Plt (306 471), 10 12 L - 1, Hb 10 9 L - 1 ;, ; : RBC (2 4) / HP, WBC (2, 4) / HP ;, + : ; 219 317 g/ dl,,, ; : ; PT + A : PT 1211 s, 015g, 2 / PT % 8715 %, INR 1105, Fbg 812 mg/ dl, APTT 2818 s ; ENA ANA dsd2,, : Hb 100g/ L, Plt 471 10 9 NA ACL / L ; ( - ) ; ANCA 1 : 40 ( ) ; RPR HIV2Ab HCV2Ab ( - ) ; B :2005-01 - 10

2005125 (4) Basic & Clinical Medicine 377 115 11 ( GCA), 68, 1 ;? ;,, ; 21 GCA? 31,? 41? 2 211 ( ),, ;,,,,, 10 GCA! 1 1, :? 2, ( - ),? 31,, ( - ),? 41 (50 %, -,, )? 51, : 103 mm/ 1 h, CRP 5193 mg/ dl, ANA ENA dsdna ( - ), AN2 CA 1 40 : ( GCA)? 81 : (PMN) 3 : 11? 91 ; 21 ;? 101 : 31, : 11GCA,? 21 GCA DMARDs,,? 31GCA ANCA? 41 Narvaez,? 51 GCA10 : PMR 23, GCA? 40 60 mg/ d 1 2,, ( Johns Hopkins http : / / MTX) 2002, INSSV ( vasculitis1med1jhu1edu/ index1html, ) 4 212 ( ), MRA +, + MTX ), GCA 213 ( ) ACR GCA : 11 > 50 ; 9112 % WBC Plt CRP 21 ; 31, ; 41ESR 50 mm/ h ; 51 : 214 ( ) ; 103 mm/ 1 h GCA, : ( ) ; CTD : RA SLE ; : ; : NHL MM ; GCA :? 61,? 71 : TIA,,, 56 : 98 GCA, 2 (,,,, 3, :, GCA 9315 %, 2 :

378 Basic & Clinical Medicine 2005125 (4),,, ; :, ;, (1) :, Babinski ( + ) : : (1) > 50 (2), (3),, (4) :, (5),, (6) :,,, (2) : 3, (, :,, ),, :,, (1) : ; (2), : GCA, ; 10 % 15 %,, ;,,,,, (10 % 80 %) (30 %, 50 %), : ; (3) :,,, (4) : Wegener :,?,,, (5)? (80 %) :,,, (6) :, GCA (7) :, 215 ( ),, GCA, (8) 40 60 mg/ d, (3) : GCA,,,, GCA 33 % 50 %, 1 2,, 40 % 50 %, 10 % 60 %

2005125 (4) Basic & Clinical Medicine 379 50 %, 64 (51 75 ) : 8/ 9 ; 50 % 2/ 9 ; > 50 mm/ 1 h 9/ 9 ; AION (anterior ischemic optic neuropathy), 5/ 9 ; 3/ 9 : 20, ; 70 mg/ d ; ( CTX/ MTX) ;, ) Takayasuπs GCA, 217 ( ) 7/ 9 ; 3/ 9 ; 5/ 9 ;, GCA,, 30,, 7, 4, GCA, ESR 27 48 mm/ h :, > 50, GCA,,, AION, GCA,, GCA,?, 218 ( ) : 1 GCA,, GCA 2 mg/ (kg d) (120 140 mg/ d)? ( 1 2,, 2004, 27 (2) : 105-107) + + 219 ( ) : GCA,,,,? 2110 ( ), GCA GCA :, 70 % 80 %, 90 %,,,, GCA,! 2! AION PION, 2111 ( ) CRAO GCA!,,,,,,!,,, GCA, 2112 ( ) 216 ( ) 1, GCA, GCA, 6 mm, GCA,,, ( ) ( 2113 ( ),, GCA, 9, : (3 6 ) ;, GCA 1

380 Basic & Clinical Medicine 2005125 (4),??,,? :,,, GCA?, 2114 ( ) GCA ( ),,, GCA 30/ 10, 80 Kelly (2002 ) GCA 300/ 10 50,,, 2 GCA 80,,, GCA 13, 28, 48,,, GCA GCA,,,, PMN, GCA, (Sandra L. Dabora) 3 ( Genes Chromosomes Cancer, 2005 ;42 :213-227), (TSC) (Ramona O. Hopkins) 2 15 (Am J Respir Crit Care Med,2005 ;171 :340-347), (ARDS), NCI BIOCOMPARE (2005/ 3/ 7),, ( ) 2005 3 Genome Research http :/ / home. ccr. cancer. gov/ oncology/ oncogenomics 18,927 (Hashem El2Serag) 4 ( Gut,2005 ;54 :533-539), (HCC) 3 X (Am J Roentgen,2005 ;184 :926-930),, 8, (Jonathan S. T. Sham) 2 20 (J Clin Oncol,2005 ;23 :1118-1124), (NPC)

2005 5 25 5 Basic & Clinical Medicine May 2005 Vol. 25 No. 5 : 100126325 (2005) 0520475206 ( 5),, (, 100730) 1 30 mgπd,5 6,,15 6, 4, 4 111 5 10 9 L - 1,WBC 017 10 9 L - 1 1998 5 (8 ) B,, ( PLT) 40 10 9 114 cm, ;, ( - ) ΠL, ( WBC) (, 2004 Hb), ( + ) 2 6, 800 ml ;B, ; 1, C, 150 ml, 2000,,,,, 800 1 000 ml,,,, PLT 8 32 10 9, 2 8, Hb 18 L - 1,Hb 67 100 gπl,wbc 115 416 10 9 L - 1 47 gπl,plt 8 14, 10 9 L - 1,WBC 017 117 10 9 L - 1, (Ret) 112 % 5 %,, ( PT) 1616 s, ( PT %) ; (ALT) 84 UΠL, (AST) 6214 %,, 78 UΠL, ( GGT ) 91 UΠL, 5 ( - ) : 6 7, 3 ;, 6 B,, 019 cm ; ; CT,,1,,, ; 10 5 6 25 28, 20, MDS, : 60 mgπd ( ), 112 C PLT 20 10 9 L - 1 2000 72 Πmin, 105Π55 mmhg ( 1 mmhg = 11 1 000 ml, 13313 Pa),,,,,,, 2001,,,, 30 mgπd, ( - ), 72 2002 11, 1 500 ml, Πmin,, 2 3Π6, 2003 1,,,,,, :2005-02 - 28 :2005-03 - 20 ( 50 ), PLT 8 9 10 9 L - 1 13 10 9 L - 1, 2003 9 12, 16,Hb 18 gπl, PLT 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

476 Basic & Clinical Medicine 2005125 (5) 4 cm,, 3 cm, ( + ),, 113 : 71 8215 gπl : 819 gπl ; B12 : PT + A :PT 1616 13 s, PT % 6214 % 77 %, (APTT) 3318 2611 s :25 42 mmπ1h C ( CRP) : 8189 mgπl 5 211 ( ) :HBsAb, HBcAb ;, CT : ( - ) ; IV :175 ngπml ( < 140), : 0124 ( ) B :, ; 1017 cm, ;,, 519 cm, 215 cm ;, 310 cm CT + + :,,,,,,,,, ;, : 111 cm, 212 ( ) 280 mπs, 114 cm, 015 cm,, :,, : :16183 molπl, : 49123 molπl, : 34 % 812 gπl Rouπs, Coomb s, Ham, : ( - ) :,,,?,, 114 3, ; MDS - RA :,, 2 19,, 3 2 3 5, MDS, ( - ) 3, 500 ml,, :,,,,, : ANA ds DNA ENA ( - ) 115 : 3315 8217 s IgG:34 ngπ10 7 PA : WBC 0139 117 10 9 L - 1, Hb 18 ( < 128 ngπ10 7 PA) : ; : 69 gπl,plt 7 21 10 9 L - 1 Ret :114 % 212 %, : K2F ( - ) 2314 3111 10 9 L - 1 : 2Π 116 50, 25Π50, 18Π50, 5Π50 ; : ( - ) : ( - ) : ( + ) : ( TP) 54 72 gπl, (ALB) 29 42 gπl, 102 mgπl ; IgG 1118 gπl, IgA 4189 gπl, IgM 0198 gπl ; : ALB 5617 %, 1 4 %, 2 915 %, 1014 %, 1914 %? 2,, :,,, :, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005125 (5) Basic & Clinical Medicine 477, ( - ) ; ;,,, ( ITP),, (5) :,, 3, :,,,,,,,,, 12 %,, 10 %,,,24 %,, ;, Budd2Chiari, 12 %,,8 %,,,,, (6) :,,,, : (1), : MDS,10, ;, : (1), ;,,,,,,, ;,, (2), (2) :,,,,,,, 2, 2, (3), 2, : (1), 2,? (2), (3),? : (1) 7 8 : Π, 10 9 L - 1, 3 % 5 %, ; (2) MDS, 20 %,? (3),20 %,,?, 213 ( ),,, 6, (4) (VOD) : ( ),,,, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

478 Basic & Clinical Medicine 2005125 (5),,,,,,, HCT,, MCV ; ; (6), ; (DC) :85 % X,,, ;,,,,,,, Π,,DC 50 %,70, 10 % (1) ITP : ITP,60 % 80 % 20 % 10,, IVIG, MDS AML HD DC,,, ITP MDS,, DC VW, 2002, 146 MDS2RA 9 % PLT,8Π 11, ITP, DC MDS (2), :,,,,, : (1), + Π +, ( + ),,,,,,, PLT ( - ), (3) : 20 10 9 L - 1,,, VII,,,, 20,, MDS (2) ;90 %, MDS, A, ; > 500 gπl, > 80 %, 214 ( ) ;, 15 000 ml, VWF (3) DC,, > 200 gπl, (4), 2 : ( IPH) ( ), ( ) ( ),,,, VOD IPH,,,,, (5) MDS :, ; IPH ;,,,,,,,MDS 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005125 (5) Basic & Clinical Medicine 479, ;,, ;, (5) ( ) :,, ;,, ; ;,,,, 216 ( ), (DC) DC, :,CTA,, 215 ( ) ;,, :15,,, ;,,,, MDS ; ( + ), IgA Fanconi ( ) DC : : (1) IPH :,,,:,, DC, IPH SLE, IPH ;DC, 1 MDS ( 2) DC, (DC) :,, DC,,, 217 ( ), : (1),,,, ; IPH,, DC (3) : IPH,IPH,,, (2),MDS, ( 4) Wiskott2,, Aldrich : X,,,,,,,, IVIG,5 %, +, IVIG,,, MDS,,,, ;, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

480 Basic & Clinical Medicine 2005125 (5), 2111 ( ) ; 70 80 gπl, 1 2, ;, + +,, MDS,,, 2112 ( ), 3 : (1) MDS DC? (2) CTA,, +,? (3),? 218 ( ) ;, 2114 ( ),? 219 ( ), 10 %, 3,, 2110 ( ), 2113 ( ), 30 %, 3 IVIG 1 2 IVIG, 100 g,, 2004 3 23, 1 2 Phemister, 3 4,,,,, 1 10 % 20 %,3 70 % : ; :,,,, ; 2 3, CD15 +, CD20 +, CD3 +, CD68 +, PC +,,2 SMA +, (2004 4 5 ) WBC 4101 10 9 L - 1, NEU % 6215 %, Hb,, 74 gπl,plt 53 10 9 L - 1 : 80 gπl, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005 6 25 6 Basic & Clinical Medicine June 2005 Vol. 25 No. 6 : 100126325 (2005) 0620572205 ( 6) 1, 1, 1 2, ( 11 ; 21, 100005) 1,, :,,54 18 ( ) 20, 1 112, 6 T 3616, R 19 Π,BP 105Π80 mmhg 111 18,,,,, ( - ), 4,, 82 Π,, Π6, P2 > A2,, 3,,, 3 5 ( - ), 1 cm,, ( - ) ( ) 113, 95 mmhg, ( )?,,, MRI,,, 114, :, 122Π50 mmhg,,, 15 mg :O 2 CP 69160 mmhg,co 2 CP 2618 mmhg, Tid, 1125 mg Qd 115, 11511, OB, ( - ) 24 h :01310 g,, 11512 ALT,TBil 2117 3155 gπl,dbil 1117, B 1172 gπl,alb 39 gπl, K Na Cl Cr BUN,,,,UA 816 1918 gπl, 11513 :ANA + dsdna Jo21 1, RF ANCA ESR CRP 5 kg : D2Dimer AcL ENA LA( - ), :2005-04 - 01

2005125 (6) Basic & Clinical Medicine 573 11514 : X :,, CT:, ;, CT VΠQ, CTPA CTV :, ;,, ; ; : (1) :, ; (2), ;, COPD, - : FEV1 FVC (, FEV1ΠFVC : ) ; (3),, :, HIV, 1990 2000,, ( 44, 44, ) ( ),, B :,,, B :, 1814 % 116 BP 95 105Π70 85 mmhg,,,, 3 kg,, 82 CT 92 bpm,, VIΠ6, VΠQ, P2 > A2,,, : (1) 90,,,, 1 cm,,? (2) ; (3) CT 119 mmhg 85 mmhg,ct 2 211 ( ),,, -,,,,,? :, 1,,,,,, 3 : ;,,, ;4 5,?,, 3,,, ;,,,,

574 Basic & Clinical Medicine 2005125 (6),,,, ACEI,,,,, 3 214 ( ) 1, 119 mmhg 85 212 ( ) 2003, ;, ;,,, ( ) CT ( COPD -, ),CT 14, FEV1 FVC FEV1ΠFVC CT (,, ),, COPD, MRI T1,CT T1, T2,,, ; VΠQ? CT?, CT,,,, CTPA,,,, ( 80 mmhg, ),,, 50 mmhg,, CT MRI, 1 %, VΠQ CTA,, VΠQ, ; CT,,,, ;, 213 ( ),, ;, HIV 3 VΠQ : 30,,,,,,, : ;,,,,, Q :

2005125 (6) Basic & Clinical Medicine 575 20 %,, ( 25 % 217 ( ) ),,?,?,,,?, 215 ( )? 218 ( ),, COPD,,,, < 85 %,,,, :, 219 ( ) : VΠQ, COPD,, VΠQ, ; 2110 ( ), 1 ( ), ;SLE, ( 100 mmhg ),,, :, (10 CT,VΠQ ) ;,, ;, ;, 40 mmhg,,, 2,, C2,, 216 La wrence ( ),,, TNF, oxldl ( 1, ), HIV,,,, statin ACEI,,, ;,,,

576 Basic & Clinical Medicine 2005125 (6) 2111 ( ),,?,?,,Lawrence?, :,,,,?, T, T 4 (Diabetes,2005,54 :1048-1055),, T :, (Robert I. Lechler), CTLA42Ig :,,,, BNP CHF CHF, (Robert P. Frantz) 3 (Am Heart J,2005,149 :541-547), (CHF) (BNP) BNP BNP, BNP CHF,,, BNP, DNA BIOCOMPARE (2005Π4Π20), DNA Ecole Normale Superieure Sloan2Kettering 2005 3 31 Natrue Nature TopIB DNA DNA, IB2 (TopIB) DNA,, TopIB,, DNA, DNA DNA, DNA CT 4 27 (JAMA,2005,293 :2012-2017), CT CT,, CT

22000055 77 2255 77 BBaassiicc && CClliinniiccaall MMeeddiicciinnee JJUULLYY 22000055 VVooll.2255 NNoo.77 11000011--66332255 22000055 0077--00667700--0033 77 11 22 22 11.. 22.. 110000773300 11 11..11 2244 11 11 4400 1100 55 ~~ 66 // 22 WWBBCC 99..33 ~~ 2200..22 1100 99 LL -- 11 00..228866 ~~ 99..99 1100 99 LL -- 11 HHbb 8822 ~~ 9922 gg // LL PPLLTT 220099 ~~ 336644 1100 99 LL -- 11 AALLTT 228888 UU // LL AASSTT 223322 UU // LL TTBBIILL 2244..88 mmgg // LL DDBBIILL 1188..55 mmgg // LL AALLBB 2255 gg // LL EESSRR 7777 mmmm // hh BB 55 ccmm 11 88 HHIIVV--AAbb -- HHCCVV--AAbb HHCCVV--RRNNAA -- TTOORRCCHH RRVV--IIggGG 11 :: 2200 ++ HHSSVV--IIggGG 11 :: 6644 ++ HHSSVV-- IIggMM ++ 11 CCMMVV--IIggGG 11 :: 6644 ++ EESSRR 2222 ~~ 22..00 ~~ 44..00 1100 99 LL -- 11 9955 mmmm // hh PPPPDD -- AAllbb 2200..33%% ~~ -- CCEEAA CCAA1199--99 CCAA112255 CCTT 3366 %% 5500 mmgg // VVPP 3322..88%% γ 5533..88%% ~~ 6688..88%% IIgg IIggGG 5500..55 ~~ 5566..77 GG // LL 77 ~~ 1177 IIggEE 663355..33 IIUU // mmll 00 ~~ 110000 TT BB NNKK TT CCDD88 ++ TT AANNAA ++ ddssddnnaa EENNAA -- SSMMAA 11 :: 116600 ++ CCHH 5500 99..88 UU // mmll 2266 ~~ 5555 CC 33 << 5588..33 mmgg // LL 6600 ~~ 115500 CCssAA CCTTXX MMTTXX 2255 kkgg 11..22 TT 3377..66 BBPP 114400 // 9900 mmmmhhgg 11 ccmm -- 1100 ccmm 1122 ccmm 11..3 WWBBCC 55..33 ~~ 1122..9933 1100 99 LL -- 11 00..441188 ~~ 22..9988 1100 99 LL -- 11 HHbb 9988 ~~ 111122 gg // LL PPLLTT 222288 ~~ 770000 1100 99 LL -- 11 AALLBB 2200 ~~ 2277 gg // LL 33 -- 22 -- 55 %% 6611 %% 2222 %% 44 %% 66 %% 11 %% 11 %% -- 11 22 -- CCMMVV--PPPP6655 BBCCRR // AABBLL -- UUCCGG EEFF 6622 %% CCTT CCTT 22000055 -- 0044 -- 2200

22000055..2255 77 BBaassiicc && CClliinniiccaall MMeediicciinnee 667711 22 1100 %% ~~ 1177 %% 11..44 11..55 CCssAA 220000 mmgg // 2200 mmgg // 4400 ++ ++ ++ 3377 ~~ 3399..33 2200 ~~ 3300 mmll // 55 ccmm 22 22..11 CCTT CCTT CCTT 11 22 33 44 22..22 11 VVCCRR CCTTXX MMTTXX CCMMLL HHDD NNHHLL CCMMLL IIHHEESS IIHHEESS 11..55 1100 99 LL -- 11 >> 55 %% << 1199 %% 22..3 11 22 HHSSVV--IIggMM HHCCVV--AAbb HHCCVV--RRNNAA -- HHCCVV--AAbb 33 TTBB--AAbb PPPPDD 11 44 55

667722 BBaassiicc & CClliinniiccaall MMeeddiicciinnee 22000055..2255 77 22..44 11 CChhuurrgg--SSttrraauussss ssyynnddrroommee CCSSSS 11 22 CCSSSS 33 CCSSSS PP--AANNCCAA ++ AANNCCAA -- 44 CCSSSS 22 22..55 22..66 11 550000 gg CCDD4433 ++ ++ CCDD33 ++ ++ NNKK--11 ++ CCDD2200 CCDD7799 ±± CCDD11 CCDD2233 CCDD1100 -- TT TT TT ppllaassmmaaccyyttooiidd TT--cceellll llyymmpphhoommaa << 1100 %% TT 22..7 11 00..44 1100 99 LL -- 11 00..44 ~~ 11..55 1100 99 LL -- 11 11..55 ~~ 55..00 1100 99 LL -- 11 55..00 1100 99 LL -- 11 -- CCMMLL -- // TT IILL--22 IILL--44 IILL--55 γ IIFFNN IILL--55 --889 PPeetteerr SSttrraanngg 44 JJ PPaaiinn SSyymmppttoomm MMaannaaggee22000055 2299 335522 -- 335577 --8899

22000055 88 2255 88 11000011--66332255 22000055 0088--00776644--0055 BBaassiicc && CClliinniiccaall MMeeddiicciinnee AAuugguusstt 22000055 VVooll..2255 Noo..88 88 11 22 22 11.. 22.. 110000773300 11 4411 44 33 11 11..11 22000000 22000011 55 2200 CCTT 3388 PPPPDD ++ EESSRR CCTT 1100 CCTT 22000033 55 22000033 1111 11..4 BB 11..44..11 553300 1100 99 LL -- 11 22000044 88 BB 660000 mmll 66 kkgg 33 2266 PPCCOOSS 3300 11 3300 dd 4400 ~~ 6600 dd 22000044 55 CCTT 11..22 TT 3377..33 00..55 ~~ 00..88 ccmm ++ 33 ccmm Ⅱ SSMM 33 ccmm ++ ++ 55 ccmm -- 11..33 3399 gg // LL PPTT ++ AAPPTTTT HHbbssAAbb HHbbccAAbb ++ CC AAFFPP CCAA112255 CCAA1199--99 CCEEAA 11..44..22 -- PPPPDD 2255 mmmm 7722 hh 441100 // mmmm 33 112200 // mmmm 33 7788 %% 2222 %% -- 11..001155 2200 gg // LL 5522 uu // LL 1188 uu // mmll -- 22000055 -- 0055 -- 1166

22000055..2255 88 BBaassiicc & CClliinniiccaall MMeeddiicciinnee 776655 11..44..33 11555500 // mmmm 33 11..44..44 IIggAA 66..8877 gg // LL IIggGG IIggMM IIggDD IIggEE IIggAA γγ2222..00%% MM kk λ 775533 229988 ~~ 666655 mmgg // ddll 2244 hh 11..55 22 ccmm 22 // 550044 22..55%% -- -- 11..44..55 EEKKGG UUCCGG EEFF 7722 %% 1188 ccmm HH 22 OO 1199..55 ccmm HH 22 OO 11..44..66 AANNAA ddssddnnaa EENNAA AANNCCAA -- AAMMAA 11 :: 8800 -- 11..44..77 ppoollyynneeuurrooppaatthhyy oorrggaannoommeeggaallyy eennddooccrriinnooppaatthhyy MM pprroo-- TTSSHH 88..99 UU // mmll 00..3388 ~~ 44..3344 tteeiinn sskkiinn cchhaannggeess 1177 2244 hh CCTT 116600 ppgg // mmll << PPOOEEMMSS 4466 22..6622 110088 ppmmooll // LL PPOOEEMMSS 00..3388 gg // LL 00..331188 nnmmooll // LL 88..44 mmiiuu // mmll 99 mmiiuu // ddll 2244 hh NNaa KK CCll CCaa PP BB 11..44..88 226655 mmmm HH 22 OO 66 // mmmm 33 00 // mmmm 33 00..9900 gg // LL -- 11..44..99 CCTT CCTT CCTT MMRRAA 4400 // mmmm 33 3399 11 -- 11..001100 1144 gg // LL 3300 UU // LL SSAAAAGG 22..55 ++ MMRRII 1100..55 UU // mmll -- BB 11..55 225500 mmll QQ88hh 22 22..11 11 22 33 44 MM IIggAAλ 55 AACCTTHH FF 66 77 PPOOEEMMSS PPOOEEMMSS PPOOEEMMSS 22..22 CCTT CCTT MMRRAA 22..3

776666 BBaassiicc & CClliinniiccaall MMeeddiicciinnee 22000055..2255 88 22..44 PPCCOOSS 11 22 BB 33 TT LLHH // FFSSHH >> 22..55 33 22 PPCCOOSS PPCCOOSS PPCCOOSS PPCCOOSS PPCCOOSS 22..55 11 22 33 44 55 -- cchhrroonniicc iinnffllaammmmaattoorryy ddeemmyyeelliinnaattiinngg ppoollyynneeuurrooppaatthhyy CCIIDDPP 66 77 HHMMSSNN 8899 PPOOEEMMSS PPOOEEMMSS PPOOEEMMSS PPOOEEMMSS 118800 ~~ 440000 mmmmhh 22 OO PPOOEEMMSS 11CCIIDDPP CCIIDDPP 22 33 BB11 BB66 BB1122 AA AATTPP 33 ~~ 66 22..66 AAddddiissoonn FF TTSSHH AACCTTHH TTSSHH FF AACCTTHH ⅠⅠ Ⅱ// ⅠⅠ PPOOEEMMSS 22..7 55 ~~ 1122 2244 %% ~~ 3322 %% 9900 %% SSAAAAGG ++

22000055..2255 88 BBaassiicc && CClliinniiccaall MMeediicciinnee 776677 22..88 PPOOEEMMSS PPOOEEMMSS VVEEGGFF ++ // 22..99 DDiissppeennzziieerrii 22000011 PPOOEEMMSS 11 2 CCaassttlleemmaann PPOOEEMMSS MM IIggAA PPOOEEMMSS PPOOEEMMSS PPOOEEMMSS 2200 %% CCPPAAPP ++ ++ 22..1100 PPOOEEMMSS PPOOEEMMSS 22..1111 22..1122 PPOOEEMMSS PPOOEEMMSS 33 22..1133 PPPPDD 1100 PPOOEEMMSS 22..1144 1100 22..1155 00 PPPPDD 1188 ccmmhh 22 OO PPPPDD 22..116 PPOOEEMMSS PPOOEEMMSS 33 33..11 PPPPDD 1177 mmmm

776688 BBaassiicc && CClliinniiccaall MMeeddiicciinnee 22000055.2255 88 1144 ccmmhh 22 OO Ⅳ 222211 nngg // mmll -- XX -- -- 33..22 >> 3300 ccmmhh 22 OO >> 22 ccmm 22 << 2200 ccmmhh 22 OO 33..33 PPOOEEMMSS PPOOEEMMSS ++ 33..4 PPOOEEMMSS 4 6600 mmgg qqdd ++ 22mmgg qqdd MMPP 11 33 ccmm 1100 22000055 77 99 MMPP BB -- 11 DDiissppeennzziieerrii AA KKyyllee RRAA LLaaccyy MMQQ eett aal.. PPOOEEMMSS ssyynnddrroommee ddeeffiinniittiioonnss aanndd lloonngg--tteerrmm oouuttccoommee JJ.. BBlloooodd 22000033 110011 77 22449966 -- 550066.. 22 DDiissppeennzziieerrii AA GGeerrttzz MMAA.. TTrreeaattmmeenntt ooff PPOOEEMMSS ssyynnddrroommee JJ.. CCuurrrr TTrreeaatt OOppttiioonnss OOnnccooll 22000044 55 33 224499 -- 225577.. DDaann TThheeooddoorreessccuu 66 UUrroollooggyy22000055 6655 11114466 -- 11115511 2 AALLLLHHAATT PPaauull KK.. WWhheellttoonn 66 2299 AArrcchh IInntteerrnn MMeedd 22000055 116655 11440011 -- 11440099 22 AACCEE 33 AALLLLHHAATT 22 55 2266 NN EEnnggll JJ MMeedd 22000055 335522 22119933 -- 22220011 GGMM--CCSSFF JJoosshhuuaa RR.. KKoorrzzeenniikk

22000055 99 2255 99 11000011--66332255 22000055 0099--00886622--0055 BBaassiicc && CClliinniiccaall MMeeddiicciinnee SSeepptteemmbbeerr 22000055 VVooll.2255 NNoo.99 99 11 22 11.. 22.. 110000773300 11 5511 22 11 11..11 22 4400 mmgg // dd 2200 1155 mmgg ttiidd 11..55 mmgg bbiidd 3388 11 ~~ 22 55 33 ccmm 11 PPIIPP MMCCPP EESSRR 5522 mmmm // hh CCRRPP 44..9944 mmgg // ddll AANNAA ++ 11 :: 8800 EENNAA RRFF AAPPFF AAKKAA -- 2200 ~~ 3300 00..7755 mmgg 2200 55 mmgg 22..55 mmgg 3322..55 mmgg // dd 33 1100 mmgg // 55 1100 mmgg // 11 ~~ 22 1100 00..33 qqdd 11 77..55 kkgg 1155 mmgg qqww 1100mmgg qqdd 2200mmgg ttiidd 11..2 CCuusshhiinngg TTmmaaxx 3399 WWBBCC 1100..4477 GG // LL GGRR 7755..11%% HHGGBB 111144 gg // LL PPLLTT 334444 GG // LL JJoo--11 -- 55mmgg qqdd 11 TTmmaaxx 3388 ~~ 4400 22 ccmm 55 mmgg // dd 2200 mmgg // dd 44 ++ VV Ⅳ BBaabbiinnsskkii -- 11 ~~ 22 22 mmgg 11 1100 mmgg // dd 11 22000055 -- 0044 -- 0077

22000055..2255 99 BBaassiicc && CClliinniiccaall MMeddiicciinne 886633 11..44 RRAA CCTTXX 00..44 // MMTTXX 1155 mmgg // SSCCLLEE BB CCTT TTmmaaxx 3399..44 66 NNSSAAIIDDss 22 3377..33 PPPPDD ++ ++ 3377..33 ~~ 3388..33 2200 mmgg qqdd 11..5 11..55..11 WWBBCC 55..5566 ~~ 77..7733 1100 99 LL -- 11 NNEEUUTT 6666..11%% ~~ 9911..22%% EEOOSS 00..55%% ~~ 11..55%% LLYYMMPP 66..66%% ~~ 1199..33%% RRBBCC 22..5577 ~~ 33..9911 1100 1122 LL -- 11 HHGGBB 7788 ~~ 112211 gg // LL -- 11 PPLLTT 8822 ~~ 223366 1100 99 LL -- 11 -- ~~ 11..00 gg // LL -- -- 2244 hh 00..1144 gg -- -- -- AALLTT 1111 ~~ 5500 uu // LL AALLBB 22..9911 ~~ 44..3333 gg // ddll AASSTT 88 ~~ 6699 UU // LL CCKK 99 ~~ 2222 UU // LL LLDD 113355 ~~ 442244 UU // LL HHBBDD 110055 ~~ 335511 UU // LL GGGGTT 3355 ~~ 553366 UU // LL AALLPP 6600 ~~ 117744 UU // LL KK 22..3377 ~~ 44..0066 mmmmooll // LL BBUUNN 1111..77 ~~ 2200..00 mmgg // ddll CCRR 00..5555 ~~ 00..6699 mmgg // ddll 2233 ~~ 113355 mmmm // hh CCRRPP 00..773344 ~~ 3333..66 mmgg // ddll 669977..00 nngg // ddll >> 1144..00 PPTT 1111..11 ss IINNRR 00..9944 AAPPTTTT 2211..11 ss FFbbgg 332277..44 mmgg // ddll 11..55..22 AANNAA -- --ddssddnnaa -- EENNAA -- AANNCCAA -- RRFF -- AAKKAA AAKKFF -- -- AALLBB 5511..11%% γγ 1155..55%% αα1144..77%% 1155..55%% IIggGG 99..7788 gg // LL IIggAA 22..1166 gg // LL β IIggMM 11..1188 gg // LL IIggEE 11..2277 IIUU // mmll 00 ~~ 110000..00 CCHH5500 6666..66 UU // mmll 2266..00 ~~ 5555..00 CC33 7700..44 mmgg // ddll CC44 2233..99 mmgg // ddll 11..55..33 HHBBssAAbb ++ HHbbccAAbb ++ -- HHCCVV--AAbb -- HHIIVV--AAbb -- RRPPRR -- EEBBVV IIggGG // VVCCAA ++ 11 :: 4400 IIggAA // VVCCAA -- IIggAA // EEAA -- IIggMM // VVCCAA -- -- PPPPDD ++ ++ 22 -- 22 -- 11 ++ 11 :: 116600 22 ++ 11 :: 8800 BB NNKK TT 22 -- 33 -- -- TTOORRCCHH RRVV--IIggGG ++ 11 :: 4400 CCMMVV--IIggGG ++ 11 :: 225566 HHSSVV--IIggGG ++ 11 :: 551122++ 11..55..44 AAFFPP 33..8833 nngg // mmll CCAA112255 88..88 UU // mmll CCAA -- 11..55..55 TTSSHH 77..664444 UU // mmll 00..3388 ~~ 11..0099 --TTPPOO AAbb 114411..00 UU // mmll 00 ~~ 6600..00 -- 11..55..66 MM == 7744 %% EE == 2233 %% MM :: EE == 33..2222 :: 11 11..55..77 221100 ccmmhh 22 OO CCSSFF -- ++ 00 CCSSFF 5533..11 mmgg // ddll 6611 mmgg // ddll 9922 mmmmooll // LL CCSSFF -- -- -- -- CCSSFF 11..55..88 XX--rraayy RRAA CCTT CCTT CCTT BB

CCTT CCTT EEFF 5533..77%% 1100 BB 66..88 ccmm 22..11 ccmm 44..88 ccmm 11..66 22 886644 BBaassiicc && CClliinniiccaall MMeeddiicciinnee 22000055..2255 9 CCTTDD RRAA PPMMRR TTBB XX--rraayy CCTT CCTT CCTT CCTT 11 22 CCTTDD 22..11 11 CCTTDD SSCCLLEE 22..3 22 CCTTDD SSCCLLEE CCTTDD 11 CCuusshhiinngg Ⅲ EESSRR CCRRPP CCTTDD RRAA PPPPDD ++ ++ ++ ++ 22..22 XX--rraayy XX--rraayy

22000055..2255 99 BBaassiicc && CClliinniiccaall MMeediicciinnee 886655 RRAA RRFF AAKKAA AAPPFF RRAA RRSS33PPEE 2200 8800 HHLLAA--BB77 RRFF -- EESSRR PPMMRR 11 CCKK 22 PPMMRR EESSRR PPMMRR 33 44 Ⅱ 55 EESSRR CCRRPP EESSRR CCRRPP 44 11 22 33 SSSS 44 CCTTXX MMTTXX 22..44 MMRRII 22..55 11 -- 22 33 33 ++ QQ 44 EESSRR CCRRPP PPPPDD ++ ++ CCTT 1100 22 22..6 CCTT TT CCTT

886666 BBaassiicc && CClliinniiccaall MMeeddiicciinnee 22000055.2255 99 22..77 XX--rraayy CCTT PPPPDD 22..8 XX--rraayy RRAA DDNNAA 11998855 HHIIVV 1133%% AAIIDDSS 1122 DDoouuggllaass LLoowwrriiee PPoohhaanngg UUnniivveerrssiittyy ooff SScciieennccee aanndd TTeecchhnnoollooggyy YYoouunnggcchhuull SSuunngg GGeennee TThheerraappyy SSuunngg DDNNAA LLoowwrriiee LLoowwrriiee TTuubbeerrccuulloossiiss ccaann bbee ttaacckklleedd wwiitthh aa ccoocckkttaaiill ooff aannttiibbiioottiiccss bbuutt uupp ttoo 1122 mmoonntthhss ooff ttrreeaattmmeenntt aarree nneeeeddeedd.. 1122 ttaacckkllee hhttttpp // // wwwwww.. nnaattuurree.. ccoomm // nneewwss // 22000055 // 005500113311 // ffuullll // 005500113311 -- 1122.. hhttmmll

2005 10 25 10 Basic & Clinical Medicine October 2005 Vol. 25 No. 10 : 100126325 (2005) 1020960204 ( 10),,,,, (, 100730) 1,,,28 2,, 1, 111 3 d : ph 71467, PO 2 64 mmhg, PCO 2 3, 4 d 3815 mmhg, SO 2 93 % ; WBC 3418 10 9 L - 1, GR, T 3815,, 93 %,,, RCU,, 1, Raynaud, (WBC) 1613 10 9 L - 1,, 1 Πd, ; ( GR) 7719 %, ;, 3 d, WBC 1418 10 9 L - 1, GR 84 %,, 2 d,, ;,,, WBC 2513 10 9 L - 1, GR 90 %,, 309 112, + + + 3915, 100 Π, 34 Π,, 120Π80 mmhg,, 38 39,,,,,,, 5 d, CT,, 100 Π,,, ; ( HIV),, ( ESR) 42 mmπhr, (ALT),,4 Π 47 UΠL, (AST) 4018 UΠL, (TBIL) ( ), 3313 molπl, (ALB) 2318 gπl, 113, :ph 71517,PO 2 47 mmhg,pco 2 2915 mmhg,so 2 88 % 12 30pm?, 10, ;3 00pm,,, : 120Π60 mmhg, 150 160 Π min, 35 Πmin,, :2005-06 - 20

2005125 (10) Basic & Clinical Medicine 961 114 +, 2 10 g 4 d ; 40,, 211 ( ) 10 LΠmin, PO 2 40 60 mmhg CT, ;, ;, 1 B, 3, 1600 ml, 1400 ml,, CT 38 39, 2, 3 LΠmin,,WBC,, HRCT 3,,, 400 mg qd,, 250 mg qd po, 3 HRCT, ;,,, ;, ( - ), 3712 3812,, 2 18, CT,,,, 1119 10 9 L - 1,, ALT 61 UΠL 115 : WBC 4516 10 9 L - 1, HB 10 9 gπl, Plt 136 10 9 L - 1 3 : RBC 2 4,, ; ΠHPF, WBC 1 3 ΠHPF 24 h : ;, ; 01860 g ( 2 000 ml), ESR 46 125 mmπh C 9712mgΠL, HIV2 ( - ),CMV2IgG( + ) 1 16 1 64, HSV2 IgG( + ) 256 1 512 1 16, 6 d,, ( - ), 6 d, ( - ), ( - ),, : :ALT 237 398 UΠL,, WBC : ; :,, ; T :,, ( ALT 380 UΠL, ) : 8 1 100,1 400, 116,,, 212 ( ), ;,, 3 d, 2 d, 5 d 39, 400 mg qd 250 mg qd, 37,

962 Basic & Clinical Medicine 2005125 (10), CT,,,ALT, ALT, ALT ;WBC 111 10 9 L - 1 213 ( ),,,2,,,,,,,,,,,,, CMV2IgG( + ) 216 ( ) 1 16 1 64,4,,,, CMV ( - ),,, ( ) ( - ), IgM CMVpp65 ( - ), - ( - ),,,, 6 ;,,, 1,,,,,,,, 2,,,,,,,, IFA,,, Lp8, 1 100, 1 400,4 214 ( ), Lp8, CMV, CMV2Ig 4, CMV, CMV,, HIV, Still,,,,, CMV 215 ( ), ;,,, 217 ( ),,

2005125 (10) Basic & Clinical Medicine 963,,, 1 d,, 6,, 1 d, CMV,,,, CMV,,CMV,,, 219 ( ), 218 ( ),,,,?, BCYE,,, CMV, ( - ),,,,,,,, WBC, CMV, ESR 125 mmπh,crp, CMV 2110 ( ), CMV,, CMV2IgG 4, CMV, CMV,, CMV,, CMV, IPF Lp8, 4, IFA,,,,, BIOCOMPARE (2005Π8Π30),, 1 (SCA1), Purkinje Ataxin21 1,,, 2005 8 26 (Cell), Purkinje Ataxin21,

2005 11 25 11 Basic & Clinical Medicine November 2005 Vol. 25 No. 11 : 100126 3 2 5 ( 2 0 0 5 ) 1 121 0 7 420 5 (11),, (, 100730) 1 (DB IL ) 1512 mol/ L, ( K) 3129 mmol/ L,, 70 /,,,,,,,54 3, 9 113 : WBC 519 10 9 / L, N 3016 %, L 111 5919 %, RBC 4137 10 12 / L, Hb 117 g/ L, PL T 231 10 9 / L : AL T 66U/ L, ALB 216 g/ dl, 3, TBiL 1165 mg/ dl, DBil 0166 mg/ dl, AL P 49 U/ L,,, Cr 0169 mg/ dl, Ca 718 mg/ dl, 9 ( PT + A) : PT 1515 S,APTT,, + + 3617 S, INR 1129 : ALB 4213 %,,, (1211 %, 41 %, IgA 5184 g/ L, Ig G 3112 g/ L, IgE ;, ;, : 37513 U/ ml : T3 0169 ng/ ml, T4,, 5137 g/ dl, TSH 1101733 U/ ml, FT3,, 0173 pg/ ml, Fr T4 0145 ng/ dl TG2Ab >,,, 4 000 IU/ ml ( < 115), TPO2Ab 6716 U/ ml, : ( AL T) ( < 34) 108 U/ L, ( TB IL) 3215 mol/ L, 420 ng/ ml, ESR 30 mm/ h ANA + dsdna: ANA1 : 640 ( ), 1 160 ( ) ( T3) 1112 pmol/ L, : SMA 1 160, AMA 1 160, AMA2M2 : ( T4) 5144 pmol/ L : 203 RU/ ml RF 413 IU/ ml, LA 2416 S, ACL :, ( - ),ANCA ( - ), ENA ( - ),APF ( - ),A KA ( - ), 0154 CT :, ( - ),HCV ( - ) :1984, :, :, AL T PO 2 4113 mmhg, PCO 2 3016 mmhg,p H 71504, SO 2 1985,2001 8116 %, A2aDO 2 7115 mmhg : PO 2, 3414 mmhg, PCO 2 2815 mmhg, p H 71518, SO 2 112 7318 %,A2aDO 2 8017 mmhg : BP 100/ 70 mmhg,, PO 2 45918 mmhg,pco 2 2812 mmhg,p H 71477,SO 2,, 9918 %,A2aDO 2 21719 mmhg : : 2005-06 - 05

2005125 (11) Basic & Clinical Medicine 1075 PO 2 392 mmhg, PCO 2 2913 mmhg, p H 71484, SO 2 9919 %,A2aDO 2 28414 mmhg Qs/ Qt = 19159 % : ( ) : 212 ( ),LV EF 70 % 2 211 ( ) ( TIPS), 99m Tc2 : CT : ( 99m Tc2macroaggregated albumin, MAA),, CTPA :, CT :,,,, : = ( - :, ) / 100 % : -, 1913 %,, : 1913 %,,, 21/ 18 mmhg, 114,,,, 10 L/ min,, 98 % 100 % :,,,, 213 ( ),,,,, 1984, AL T 9, CT :,,,, : :,,,, : - :, : CT :,,, : ( ), CT (V/ Q) : :AMA 1 160,M2,SMA 1 160,ANA1 :, 1913 % 640 ( ),1 160 ( ), Ig G + + : ( ) CT Krowka, : 2 : ( ) ;, ( ) ; / (, ( ),, ) ; ( ) ; (,,,

1076 Basic & Clinical Medicine 2005125 (11), ) 215 ( ),,V/ Q,,,,,,,, :1986 10,,,7 1, 1989 6 4 3, 16 75, 40 %, 15 % 5016 5, 1 :,, 1 1 4,,,, ;, ;,,A2aPO 2, : (100 %), 15 mmhg ; UCG ( ) V/ Q (8416 %), (1412 %) ; ( ) (1412 %), ( 8416 %), ;, (8416 %), (7014 %) : ( 1111 %), + : (1) (1111 %),CT (3313 %),, ; ( 6617 %), (2) 214 ( ),,,2, 216 ( ), : (1), (AIH) : ANA 1 640 ( ) SMA 1 160 Ig G 3112 g/ L, 41 % ; (2) ( PBC) : AMA 1 160 AMA2M2 203 RU/ ml IgM 4164 g/ L, AL P GGT, ; (3) (AIH + PBC) : M - 2,, 90 % 95 %,,,, ; ;, 50 %,,,, PaO 2 < 50 mmhg,,, > 30 %,,UCG (100 %), (7718 %), (1617 %) 100 %, 22 % 57 % 3,1,,

2005125 (11) Basic & Clinical Medicine 1077 GP210,, PBC 25 % ; SP100, 218 ( ) PBC 20 % 30 %, +, PBC, PBC?, PBC 50 %,, PBC 80 %,,,,, 219 ( ), 1,, 20,, 5, 5, +, 80,,,,,,,, ANCA,,,,,,, 2110 ( ),, B,,,, SSA SSB, Ig G,,,, Ig G 2111 ( ), 20,,,, 217 ( ), 60 mmhg,, 15 h,,

1078 Basic & Clinical Medicine 2005125 (11) 2112 ( ),,,, 5 %, 500 mmhg,, 459 mmhg,, V/ Q 5 6,, ;,,, V/ Q,,,,,,, ACE ( Alzheimer disease) (2004110), (ACE) (captopril) (perindopril) Takashi Ohrui Ohrui 162 ACE (MMSE),, ACE ( 3715 mg/ d 2 mg/ d) MMSE, 016 ACE ( enalapril 5 mg/ d imidapril 5 mg/ d ; 416 ) ( nifedipine 20 mg/ d nil2 vadipine 4 mg/ d ; 419 ) MMSE, ACE, Ohrui, MMSE ACE Ohrui ACE Neurology, 2004 ;63 :1145,1324-1325

2005 12 25 12 Basic & Clinical Medicine December 2005 Vol. 25 No. 12 : 1 00126325 ( 2 0 0 5 ) 1 221 1 7 920 6 ( 12),, (, 100730) 1, ( + ),,10 20 Πmin, 113,,22 3 111 114 3,,,,, ;,, 0 2 Πd,,3 7 Πd,,,,, 4 6 Πd (,, ),, 1 h,, 1 Πd, 1 115 ;; ( ) : WBC7141 1012 10 9 L - 1,N 5318 %, 6518 % ; Hb114 135 gπl ;PLT198 310 10 9 L - 1,, ; + ;, : ANCA ASCA ENA ; : ALT74 133 UΠL, AST50, 115 UΠL ; ;AKP GGT BIL TP ALB :TG316 626 mgπdl (40 150),CHO128 216 mgπdl,, (110 220),HDL2C 25 37 mgπdl (36 70),LDL2C 76,, 113 mgπdl (80 140),APOA187 150 mgπdl,, (100 160),APOB,, Lp (a) Ig :, 1 CRP ESR ; PT + A ; 347 ngπml ( < 140 ngπml) ;ANA AMA ENA ASCA (AGA) ( EMA) 112 (ARA) OGTT BP 110Π60 mmhg,,bmi HBA1C ; 1612, K2F HIV HCV HEV EBV CMVIgM, 1 cm, (, ( - ) - ), 4 cm, ; ( 313 cm), :2005-07 - 02

1180 Basic & Clinical Medicine 2005125 (12),, 414 cm, 212 cm,,, CT +,,, :, CT,,, ;,PR > 1, ; D2 :1106 g( > 112 gπ5 h) ; PABA :41 %( > 60 %Π6 h) :12 h, 0 % ;24 h 10 %( 75 % ( IBS),15 % ) ;48 h 75 %(, ) 5 h :0 % : 212 ( ), 2 h MMC :, 3 ; 1, 8 min, 53 min, :, 40 min 4611 ; 22418 mmhg, 310 710 s,, 55 min, 6813 ; ( 15913 mmhg, 212 710 s ), ( : (1) : ) : 112 cm 75 %,,,, Mallory, (2) :, ALTΠAST, > 1 ; ;,, (NASH) : (NASH) ; (3) :, : ( TG),, : :CD68 ( + ) :PAS, ( - ) 116, 2 211 ( ) ; ( ) 3,,? MEDLINE,?, 3,, 4 ; VIP,,,,, ;,,, ;

2005125 (12) Basic & Clinical Medicine 1181,,,NASH :,,,,,,,, Gaucher Niemann2Pick, ;, ; (, ) (,,, ) ( ), 213 ( ) ;SLE,,, ( TG),, ds2dna, ENA SM, A B C E,, ApoA1 ApoB100 ApoE TG,,,,,,, : : PABA, ;, ; :, (LPL), (LCAT), TG,, 4, ;,, ; TG,, CM VLDL ApoC2,, LPL ; LPL HL,, 214 ( ) (nonalcoholic steatohepatitis,nash) NASH LDL HDL VLDL, (1),75 %,25 % : TG, NASH B48 NASH, (CM),,, HDL ApoE B2 C3, CM NASH LPL, CM TG, NASH,, TG,,, CM

1182 Basic & Clinical Medicine 2005125 (12), TG LPL, CM,,,, ApoE, :, (2) 3, ( : ),,, ; TG, TG ; ApoB100 VLDL, VLDL 3, VLDL TG (275 629 mgπdl), LDL TG :,, ; htg ( Type ) ( Type ), LPL ApoC2 TG ( Type ) TG, TG 3 : TG, TG, ; TG, LPL ApoC2 LPL LPL,, LPL TG < 1Π100 000 LPL,, TG, 1 000 mgπdl, LPL,,,, : TG TG, TG, TG,, LPL, LPL LPL, LPL, 215 ( ) TG NASH, TG, TG NASH? TG LPL,,, ApoC2, ApoC2 LPL, LPL, ApoC2,TG > 1 000 mgπdl,,,, HDL, TG LPL, ApoC2 LPL, VLDL (htg), TG, (VLDL) (CM),,,, TG, TG,,, TG : :,, SLE,

2005125 (12) Basic & Clinical Medicine 1183 :,, TG TG, TG, ; ; 218 ( ), TG LPL,, 250 750 mg tid HL, VLDL HL 216 ( ),,, TG,, 219 ( ), Gaucher Niemann2Pick,, 2110 ( ), TG,,, ApoC2 ;,, LPL, LPL, TG,, 1 000 mgπdl, LPL,, TG ; LPL ApoC2 LPL TG, 217 ( ) ApoA5 TG,,, 75 %, 2111 ( ),,, TG, TG,, TG? Mallory Mallory,,,,,,, 3, LPL (, 1) (, ) NASH :,,,,, LPL :,, >, TG, TG? LPL, LPL, LPL 1 000 mgπdl, : 013 cm LPL,,,, LPL TG NASH LPL TG

1184 Basic & Clinical Medicine 2005125 (12) 1 LPL HL LPL 151198 112101 39197 192138 97189 941493 211139 153125 58114 ( ) 180 350 90 200 90 150 ( :muπml ) :,,,,,, Paris25 Xavier Jouven 10 ( Eur Heart J,2005 ;26 :2142-2147),,,, Terje R. Pedersen (JAMA,2005 ;294 :2437-2445,2492-2494) LDL, BIOCOMPARE (2005Π11Π16),Laurence Livermore 11 11 Molecular Cell, (FERT) RNA DNA RNA BIOCOMPARE (2005Π11Π18), 11 15 (American Journal of Respiratory and Critical Care Medicine,),, (COPD) Stamford Tracy Shevell 11 (Obstet Gynecol,2005 ;106 :1039-1045), (ART), ( IVF), ART,, ART

2006 1 26 1 Basic & Clinical Medicine January 2006 Vol. 26 No. 1 : 1 00126325 ( 2 0 0 6 ) 0 120 1 0 620 7 (13) 1 2, ( 11 ; 21, 100730) 1,73 2, 111,, 90, 3918, /,,,,,,,,Mur2,, phy,, 12 d, 3712, 113 3 d, 38 39,, 3815, 40 d : WBC 513 10 9 L - 1, 6913 %, Hb 102 g/ L, PLT 8 6 10 9 L - 1 ; ASO 400IU/ ml ; ; ; 114 B, ; :, 38, ; CT:, 3816,, ; CT:,,,,1 d 1,,, 3918,,,, 20,,, 40, : 6 6,, 22 10 9 L - 1, 1 U, 200 mg/, 40 60 10 9 / L :, 112, 1 110 cm,,, ; T 36, BP 105/ 70 mmhg,, 2, :2005-02 - 28

2006126 (1) Basic & Clinical Medicine 107, 11513 : : HBcAb, ANA, 1 : 1280 640,AMA 1 : 320, AMA2M2 166 RU/ ml,aca 1 640, 1 64 HSV2Ab : IgM, IgG 1 256 :, CTD, 2 1 HIV2Ab HCV2, 40 mg Qd Ab TB2Ab PPD RPR 1 mg/, 2,, 11514 :AFP CA CA125, 6,, 11515 : :,, CT:, CT:,,,,,,, CT ;,, CT ;, ; 115 11511 : : WBC, Hb 80 106 g/ L,pH 22 129 10 9 L - 1, : 116 % : 013 g/ L,RBC 1 3/ HPF, 116 : WBC 6 8/ HPF, RBC / HPF ( ) 2,PT +? A : PT 1212 s, FBG 62316 mg/ dl,aptt 3419 s ESR : 82 20 mm/ h ; CRP :1318 14 mg/ L :Alb 4317 %, 1 411 %, 2 1019 %, 2818 %,A/ G 0178 Ig : IgG 1917 g/ L, IgA 4171 g/ L, IgM 315 g/ L > 500 g/ L : LAM 11 g/ L, KAP 1611 g/ L : ; 260/ mm 3, 1/ mm 3, 1/ mm 3, 0/ mm 3, ; TP 2019 g/ L, ALB 814 g/ L, LD 48 U/ L, Cl 11316 mmol/ L, GLU 3 990 mg/ L ; ADA 715 U/ ml ; ; CT Kwart, ; DIC : FDP 5 10 g/ L,D2dimer 0155 mg/ L, IgG: 766 ng/, 10 7 PA : 11512 :ANA : 1 :1280 640 ;AMA 1 320 ; AMA2M2 166 RU/ ml ; ACA 1 640 ; : C3 5 550 g/ L, C4 595 g/ L dsdna ENA ANCA RF ACL :,,, : 0, 2 2 < 011 :, 7 7 : Schirmer : R 3 mm,l 4 mm BUT: R 2 s,l 3 s :R,L EBV2Ab : IgM 1 80,IgG 1 20 CMV2Ab : IgM,IgG, ; B :,, B : UCG ECG? 2 211 ( ), CT, CT,, CT:, 8 25 :,, 8

108 Basic & Clinical Medicine 2006126 (1) 29 :, 6,,, 9 19, ( :, PLT ) ( ) 7 21 CT( ) : ( ),,,,,,, 9 9, CT( + ) :,, ;,, ;,,,, ;,,, ;,,,,,, CT, ;,, 10 15, ;,, PPH,, (2) :? : :,, ; :,,, ; :, :,,,,?,,, 212 ( ),,, : (1) :,,,, 2? 4, 5 10mL,,,,,,, :, 6, ; :,,, B,,,,, (3) : CT,,,,

2006126 (1) Basic & Clinical Medicine 109,, ACA,,,, ACA SSA SSB,,, SSA SSB,,,,, AMA2M2, CT : ( PBC),,,, GGT ALP, B,, PBC,,,,,,,, 213 ( ),,, 4, SLE,,,,, CT, PBC,,,,,,,,,,,,,,, CT,,,,,,,,,,, CT,, 2 PPD,,,, : ANA 1 214 ( ) 640,ACA 1 640,AMA2M2, PBC,,,,,, PBC,

110 Basic & Clinical Medicine 2006126 (1) PBC, PBC, PLT AMA2M2, 40 %, PBC, 5 % 10 %,,, : :, B, T ; :,,, PT APTT,, 40 mg,,,,, B, MTX, IVIG,,,, ACL 2GP1,,,,,, 215 ( ), PBC,,,, 40,,,,ANA 500 mg 3 d,,,,,, SS,2002 216 ( ), CT, SSA SSB,,,,, 6,,, AMA2M2,, PBC,AMA M2, 2 3,, IgM,, CT,,,,,,, PBC,,,,,, PBC CT,, GP210 AMA,

2006126 (1) Basic & Clinical Medicine 111,,,,,,,, CT,,, CT,, 217 ( ), :, ;,,,,,,,,,, EDTA,,,,,,,,,,,,,, ;,, : 218 ( ),,, PBC, ;, 3 : DIC :,, : 20 2 CT,,,,,, ; ; TTP :,, :, TTP, :,, 2147 g/ dl,,,, ;, :

112 Basic & Clinical Medicine 2006126 (1), ; :, CREST,, 80 % 98 %,, 1/ 4, PBC,, ;,PBC,20 %, M2,ANA, SSA,,,,,,, PBC, PBC, 2110 ( ) 219 ( ) (AMA), ; (ACA),, ;M2,AMA PBC, 90 % 95 %,, 73,,

2006 2 26 2 Basic & Clinical Medicine February 2006 Vol. 26 No. 2 : 1 00126325 ( 2 0 0 6 ) 0 220 2 1 520 5 (14),,, (, 100730) 1,74 2, 220 RU/ ml,iif2anca( 2 9 111 2, :,,, 2, (70 %) HRCT( CT) :, ;,,, 114 9 d Scr 33418 mol/ L, BUN 21120mmol/ L,ESR 140 mm/ hr,crp 794 mg/ L 20 mg Qd),11 2, : 140/ 70mmHg, CTX( ) 200 mg Qod( 2 ),, :,24 h 0156 g, Scr 35118 mol/ L,BUN 2115 mol/ L,MPO2ANCA ( + ) 7 d ACS( ),,10 ml, 6 2004 11 15 ; 5 10 / 30, 112 : 130/ 90 mmhg, ; 115, ;, 72 min - 1,, ; ( - ), ; ;,,,? 113 : WBC 10103 10 9 L - 1, HgB 84 g/ L, Plt 295 10 9 L - 1, : ( - ) ( + ),, ;24 h 0169 g Scr 31013 34912 mol/ L, BUN 1911 3214 mol/ L, K 419 5168 mmol/ L, Ccr ( ) 13 ml/ min ; hs2crp > 100 mg/ L ; MPO2ANCA( 2 ) ) > 1 80 : :, 10 29 65 mg Qd 7 d ( : (SV2PDA, SV2OM3, LIMA2LAD), 40814 mol/ L, CRRT,6 d,?? :2005-09 - 02 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

216 Basic & Clinical Medicine 2006126 (2) 116 ;,Scr 34912 mol/ L,? : 2 211 ( ) (1) (CABG),, CT (RPGN),,, 36512 mol/ L,,,, 60 mg/ d 1, (2), CTX 012 g/ (2 ), : 3 ml/ kg,,,,,, 24 72 h, >, 4412 mol/ L 25 %, 10 ;, 2 % 2 3 ;,,, ; 2 3, 90 : 120 ml, (Scr < 26512 mol/ L), 80 %,Scr 48 h,4 5 d 212 ( ),,,,, ;, 2,, ;,,CRP ),MPO2ANCA( + ),IIF2ANCA( + ) > 1 2,,,,CRP,MPO2ANCA ( + ), ANCA,,,2 3, 80,2004 8,,, 018 % 110 % ; 412 %, 515 % ; 0106 %, 0118 % : 8 1213 % (n = 57), 77 % (n, Scr 9712 mol/ L, = 22), 27 % (n = 71), : > 60 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (2) Basic & Clinical Medicine 217 CABG 1, (Scr > 17618 mol/ L), :, ;, 150, > 70 Scr > 200 m,, 22918 mol/ L, ;,, : ;, 41 %, 22 % : 50 %, :,28 % 61 %,30 %,, 54 % :,,,,,,,ANCA( - ), 1/ 3, > 60, :,,, ;,, ; CABG,,, ;, 1, 64 % 87 %,,1 79 % :, 2, 2, 214 ( ),, ;,,,,, ;,, ; MPO2ANCA, 220 RU/ ml, MPO2ANCA, ;, 65 mg, qd 1 ; 4, 213 ( ),, p2anca, ANCA : 717 %, 114 %, 019 %, 19 %,, 63 % CABG :, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

218 Basic & Clinical Medicine 2006126 (2), ( ),, 216 ( ),, avf V5 V6 ST,,,,,,,,, 70 %,,,,,,, IVIg,,,,,, 215 ( ), 217 ( ),CT,,,,, ;, ;, ARDS,,, ANCA MPN Wegener, Churg2Strause Wegener,, ;, ; MPA,,, Churg2Strause,,,,, CT,,, 218 ( ),, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (2) Basic & Clinical Medicine 219,,, 2110 ( ),,,, ANCA,MPA,,,,,,,,, MPA RPGN,,, 2111 ( ),, 219 ( )? 2112 ( ),,MPA,,,,,,,,, 25 %,, 2113 ( ),,,,, 2,,,,, 60 mg qd,,, BIOCOMPARE (2005/ 12/ 27), tetrahydrocannabinol,, CB1 rimonabant ( AcompliaTM) 2005 12 22 (Neuron), CB1 (leptin) (LH),,CB1, (DSI), leptin DSI,leptin CB1,leptin,, leptin 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006 3 26 3 Basic & Clinical Medicine March 2006 Vol. 26 No. 3 : 1 00126325 ( 2 0 0 6 ) 0 320 3 3 320 4 (15),,, ( ) 1, ; ( 1 7) : :WBC(35191 69152) 10,25 5, 9 L - 1, EO 7018 % 8313 %,Hgb 74 103 g/ L,PLT(600 990) 10 9 L - 1 111 :ALB 119 310 g/ dl 5 ( - ), ( - ), ( - ), : 29 mm/ h ANA dsdna ANCA ENA,, ( - ) IgE 590 IU/ ml :,, 2 ( EO),, ( + ) CT:, 10 cm 10 cm 1 : (WBC) CT:,, 1918 10 9 L - 1,EO 6413 %, (Hb) 128 g/ L, : ; M E = (PLT) 373 10 9 L - 1 4173 1 43 % ; : : : : HLA ( - ), DR CD11b CD15 CD10 CD13 40 % ( + ), 10 mg Qd 200 mg Qod NAP : 26 %, 38 2,, EO : bcr/ abl ( - ) :,, 12 %, 4414 %,,, 45 mg Qd,,,,, 20 cm 10 cm,, :,,,, :,,,, 3 cm 1 cm,, ; ( - ) + 014 Bid ; 110 / ; 2 cm, 2 cm,,, ; 014 Qd,, : 10 20 210 Bid,, 10 cm 20 cm 8 cm 75 mg Qd 112 15 cm 3 5 cm,, ;,, :2005-07 - 03

334 Basic & Clinical Medicine 2006126 (3) 1 ( ) 5 ( ) 2 ( ) 6 ( ) 3 ( ) 7 ( ) 4 ( ) 43 % ; :, : > 7 %, > 0144 10 9 L - 1 ; : < 15 %, < 115 10 9 L - 1 ; 15 % 50 %, ( 115 510) 10 9 L - 1 ; 50 % 90 %, > 510 10 9 L - 1, > 10 10 9 L - 1, : (HES) / (CEL) :20 50 2,, 211 ( ), :, 5, ; CEL, HES, EO, HES,Ph CEL 2/ 3, ; > 50 10 9 L - 1, 60 % ( - ), bcr/ abl : 85 % BM 2 % 20 %,

2006126 (3) Basic & Clinical Medicine 335 : >,, : 115 10 9 L - 1, 6 ;,,, ;,,, ;,, IL25, IgE ;, ;, < 5 % ;, ;,, ; IgE ;, > 70 %,,,, (HD) :,,, 213 ( ),,, Churg2Strauss (CSS) :, T,,,,,,, ; :,,, ANCA,, :, :, ( ), ( CML) 2M4EO (AML2, M4EO) (ALL) Wegener : :,,C2ANCA, : :,,,,, :, :, Churg2Strauss (CSS), CSS, ;, :,,,,, :,,, 214 ( ), : 212 ( ),, T :CD3 ( + ),CD20, ( - ),MPO ( + ),CD30 ( - ),granzymeb

336 Basic & Clinical Medicine 2006126 (3) ( - ) 10 29 : abl, CML,, T, Langer2, : han s T AE1/ AE3 ( - ),S2100 ( + ), CD34 ( + ),CD3 ( + ) T,, Langerhan s,, Langerhan s, ; 215 ( ),,,,,,,,, HES,, 3, Sezary,,,,, CML WHO, 2004 10 30 CHOP HES CEL, > 20 %, (CTX 112 D1, EPI 110 mg D1, VCR 2 mg D1, Pred ; 2 % 20 %, 40 mg D1 5), CEL,, bcr/ BIOCOMPARE (2006/ 1/ 23), 2006 1 19 Neuron, strychnine BIOCOMPARE (2006/ 1/ 23),, 1 19 (Nature), 2 5 2AMP, 5π2AMP, 5π2AMP,,5π2AMP 2 1 Insubria Anne M. Grandi 1 (Am J Cardiol,2006 ;97 :71-76), 1,,

2006 4 26 4 Basic & Clinical Medicine April 2006 Vol. 26 No. 4 : 1 00126325 ( 2 0 0 6 ) 0 420 4 4 420 5 ( 16) 1, 2 3, ( 11 ; 21 ; 31, 100730) 1 ( 1,2),,51 43, 3, 3 8 mm, 5 111 7 cm, 7,,,,, 2 5 mm,, 10, ; :,, ;, 16, :,,,3, ; 1,, ;,,,, 2 3 Π 5 6 Π,, ;, 1 2 Π,,, 3 ; 15 kg : ;1961,2001 ; 15, 180Π 90 mmhg, ; 1963 ;1988 112, 1 2 :2006-03 - 06

2006126 (4) Basic & Clinical Medicine 445, ; : ;, : 3 5 cm,, : ( ) ( - ),, ( - ), :, ; Murphy ( - ), AE1ΠAE3 ( + ),AFP( + ),CgA ( + ),Syn ( + + ), EMA :, 3 5 mm, ( + ),SMA( - ),CEA( - ),Ki267 ( - ), 113 ; OB + III( - ) ; D2 :115 gπ5 h ( 260 ml) ; ANA dsdna ( - ) ; ESR 15 mmπh ; CRP 3164 mgπl ; : ; CEA 4 ugπl, CA 50 1 UΠmL, CA 199 15 UΠmL, CA 242 27 UΠmL ; ECG ; :, ; : B :, ; :, 114, B : ;,, ;,, 1 2 Π, CT :,,,, ; 2, ; ;?? 211 ( ) ; :, ; : ( 3) : ( ) :, ; ( ) ;,, ; ( ) : ( :, 4) :, ; :,,:, ; 3, 3 4, ;,, : Gardner Peutz2Jeghers Cronkhite2Canada Cowden, :Cowden Cowden, 2 8 mm,,,

446 Basic & Clinical Medicine 2006126 (4), ;33 % 45 % ; ( HER22Πneu ras PS22 ), PTEN (10q23), 5 7 8 Cowden,, : Cowden : 5 Cowden??? 212 ( ) 213 ( ) : 3 1, : ; 2,, : ; ;, Gardner Turcot,,,,Cowden Peutz2Jeghers,, ( 5), Cronkhite2Canada,, : Peutz2Jeghers, :, (FAP) :, Gardner : 3 : Cowden Corn2 (FAP) khite2 Canada, Turcot : (20 30,, < 100 ), > 115 3 cm, 3 B : 10,,,,,, Peutz2Jeghers :,,, Ki67 ( ), Cowden,,1963 :,, AE1ΠAE3 EMA ( + ), ( PTENΠMMAC1 ; CgA ( + ) Syn ( + + ), ), ;,,,AFP ( + ), AFP,,,

2006126 (4) Basic & Clinical Medicine 447, Cowden, Cronkhite2Canada (CCS),,,, 2 2 ( ), CCS,,, 31 86,80 % > 50, : 1129 1 ; : ; den, ; ; ; 1Π3 ( 92 %) ( 80 %),, Π PTENΠ ( 100 %) MMAC1 ( ), 216 ( ) 91 % 90 % 87 %, ( ), CCS CCS, 1715 %,, Cowden,,, ;, ;,,,,,,, : ;, ;,,, :,, Cronkhite2Canada ; Cowden Cowden ;, : : ( 11 ;21 ), ;, (B ) ( ) (B ), (CT), 214 ( ),, ; Cow2,, den,,,? PTEN,, 215 ( ) Cowden, Cow2 Cowden ( ) : Cowden,,, 2 PTEN, Cowden,, Lhermitte2Duclos (LD) (, Π ) Bannayan2Riley2Ruvalcaba,, (BRR) (, 1 ),

448 Basic & Clinical Medicine 2006126 (4) CT 217 ( ) Cowden :,, (, ) ; ; Cowden + ( 90 100 %) 20 219 ( ),,, (50 67 %) ( 3, 10 %) ; Π (76 %, Π ) (25 50 %) ; (40 %),, ; (38 %) ; 2, ; (44 % ) (, AFP? ), :,, 80 % PTEN,10q23,2Π3 5 7 8, 40 % 5,, 3, 1973,,,,, Π? Cowden, 10 15 %,,, 7 8 cm ; 50 %,, ;, 3 cm, Cowden,,,,, Peutz2Jeghers 40 cm, : Crnkhite2Canada, 218 ( ) ;, :CgA NSE Syn AE1ΠAE3 ( + ), 1,, Vikram Patel Martin Prince 3 (Br J Psychiatry,2006 ;188 :284-285),, 3, John D1 Fernstrom 3 (Arch Surg,2006 ;141 :276-283),,

2006 5 26 5 Basic & C linical M edicine May 2006 Vol. 26 No. 5 : 1 00 1 263 25 ( 2 0 0 6 ) 0 5 20 5 5 6 20 5 (17) 1, 2, 1 ( 11 ; 21, 100730) 1, 14 3, 2, 1 111 3, 38, 28 10 9 L - 1,, 5 mg/ 3,, Tmax 39, 5 7 pm, ANA (1 80),, 24 h 11443 g, (ACL ) ; PLT 70 10 9 L - 1, 24 10 9 L - 1, 60 mg/qd 8 /qd,,,, ( 7 ), A ( 12 ), 1,, : : 112,, ( + ),,, 120 /,,,,,, ; 2 cm,,,, ( + ) ; ( + ), 9 /, 113 : :, ITP,,, 300 mg/qd 2, CT ; 192 mm H 2 O, 9115 mg/l; MR I,, 10 d,, ICP > 340 mm H 2 O, 6511 mg/l,, 2 4 h ;,,,,,,,,,, ; 1,,,, 200 mg/, 800 mg/,,, 2 MTX 10 mg : 2005-09 - 09 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (5) Basic & ClinicalMedicine 557 2005 2 5,,,, ;,, 2005 2 14, CT, ACL ( + + + ),,, 2 3 m in, 10 20 m in,, 114 11411 : CT, MRV MRA,,,, 2005 2 14 CT MR I ( 1) ; MRV,, 2125 2165 kpa,,, ; PET 1 M R I 11412 : H IV ( - ), TB 2Ab ( - ) ; ESR 36 mm /h; CRP 1217 mg/dl 11413 :DNA (ANA + ds2dna ) ( - ) ENA,, 550 ng/10 7 /L, ( ANCA ) ( - ), ( + + + ), ;, SMA 1 80; CH50 415 ku /L, C3 1614 g/l, C4 < 01167 g/l 11414 : CT,,, ; : ( + ), 1150 /mm 3, 110 /mm 3, 2%, 98% ; : TP 31 g/l, ALB 113 g/l, LDH 211 U /L, GLU 0114 g/l; : ( - ), 280 /mm 3, 190 /mm 3, 60%, 40% ; : TP 217 g/l, ALB 11 g/l, LDH 95 U /L, GLU 1144 10 3 mg/l 2 211 ( ), 8, :, ; : ; :,, ; :,, ; : ; : ; ANA, ACL, : ( SLE) :,, ACR ( 1982 ) SLE ACR SLE, ANA SLE99% ANA, SLE : :,,, : ; 8,, ; PET,, : NP2SLE; ; ; ( ) ( reversible pos2 terior leukoencephalopathy syndrome, RPLE ) H inchey 1996, 15:,, (1 17 ) CSF 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

558 Basic & ClinicalMedicine 2006126 (5), ;,,,,, : ; ; ; RPLE A FK506, IV IG, CHOP RPLE RPLE, A, A 5, A RPLECTX MTX, CTX MTX RPLE? : SLE? SLE?????,? 212 ( ) : ; ( ), ;,, ;,, 213 ( ), ( ),, :,,, ; ;,,, ;,,, 214 ( ) : ; ( ) ; ( ),,,, ;,, ( ) 215 ( ),, :,, ; ;, PET, ACL, ;, 216 ( ),, SLE, Sm ds2dna, ANA, ANA SLE ANA SLE,, SLE ANA SLE ANA 97%, 90% ANA SLE 4% 13%, : SSA ; SLE ANA ; ; 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (5) Basic & ClinicalMedicine 559 ANA ANA SLE: ; ANA : a ; b ; c,, ANA SLE ( 10% 3% ), 1 /3 ACL, 1 /3, 1 /3 ANA, 1 /3 ANAITP, SLE ITP, ( ) ( ), ANA, SSA, SLE ( ) (NPSLE), SLE, 24% 51%, SLE, SLE NPSLE, : : ; : ; : NPSLE, ACL ( + + + ), NPSLE, ( - ), 217 ( ) : ; ; NPSLE,, : ( ) MRV, CTX,,, (DSA), A,,, ( ),,, PET,, 218 ( ),,, SLE SLE SLE, ANA Sm ANA, ACL, ACL, ANA, SSA ANA, SSA SLE ANA, ACL,, ANA, SLE, SLE ANA,,, 219 ( ),,,,,?? 2110 ( ) NPSLE, rrnp CTX, CTX, CTX, ( 200 mg/ ),, 2111 ( ) 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

560 Basic & ClinicalMedicine 2006126 (5),, RPLE MRV,, DSA, DSA 2112 ( ) SLE,,?? 2113 ( ),, 2114 ( ) SLE,, ;, PET,,,,,, DSA 3, 10% 715%, MDS2RAEB : 3%, 5%, 19%, RBC, RBC, 46, XX; MDS : ;,, ;,, MDS, rrnp, ;, 24 h 1111 g,, MR I ( 2) : RPLE, CTX,,, RPLE;, 50 mg/qd 20 mg/ tid,, 4 2 M R I I (MRSA ) 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006 6 26 6 Basic & C linical M edicine June 2006 Vol. 26 No. 6 : 1 00 1 263 25 ( 2 0 0 6 ) 0 6 20 6 6 8 20 6 (18),,,,, ( 11 ; 21, 100730) 1, 19, 6 111 6, 1,,,, 15 m in,, :WBC 1217 10 9 L - 1, N 83%, Hb 116 g /L, PLT 100 10 9 L - 1 CT, Tmax 3911, : WBC 1319 10 9 L - 1, N 9217%, Hb PLT CRP 4514 mg/l, TB 2Ab ( - ) ANA ( + ) SN 1: 160, PPD ( + + + ), CXR ( - ) CSF, 210 mm H 2 O, : ( + ), 79 / L, WBC 76 / L, 58 / L, 18 / L; GLU 58 m g / dl, PRO 85 m g / dl, C l 11619 mmol/l; TORCH ( - ) MR I T1 T2,,,, 3715,,, 1 1,, 4 h, : 1, 6 :, 112 T 3714,,,, ; I,, 2, 1 1 cm 2 cm,, ; 2 3, ( - ), V, ( - ), Babinski ( ), ( - ), 113 114, 4 d Tm ax 3715 3818,, 1 8,,,,,,,, Tm ax 3919 11 B,,, 115 11511 : WBC 9102 10 9 L - 1, N 7510%, L 1914%, E 015%, Hgb 128 g /L, PLT 141 10 9 L - 1 +OB ( - ) 11512 : ALT 14 U /L; ALB 44 g /L, TB IL : 2006-05 - 29

2006126 (6) Basic & ClinicalMedicine 669 0136 m g /dl, Cr 0174 m g / dl, K 3196 mmol/l 11513 CSF : 210 mm H 2 O, WBC 76 / L, 58 / L, GLU 58 mg/dl, PRO 58 m g / dl, Cl11619mmol/L, ( - ), ( - ), ( - ), TORCH ( - ), ( - ), 1 512, OB ( - ), MBP 4179 nmol/l, ( - ) 11514 : H IV HCV RPR EBV 2Ab CMVpp65 ( - ) TORCH: RV 2IgG ( + ) 1 40, CMV 2IgG ( + ) 1 128, HSV 2IgG ( + ) 1 256, ( - ) ( ) ( - ) 11515 : ESR 7 54 mm / h, CRP 1182 m g /dl : A lb 5218%, 1 318%, 2 1119%, 918%, 2117% Ig : IgG 1615 g /L, IgA 2157 g /L, IgM 110 g /L, IgE 11182 IU /ml ANA ( + ) SN 1 160 1 80; SMA AMA ( - ) ENA ANCA RF ACL rrnp ( - ) : CH 50 4913 U /ml, C 3 9318 m g / dl, C 4 1318 mg/dl ( - ) HCY 13165 mol/l (5 15) T : B T NK, T (CD4 + T 107 /mm 3, CD8 + T 79 /mm 3 ), CD8 + T 11516 : EKG T UCG EF 61%, TCD, CXR ( - ) BUS, ; CT, ;, CT, MR I, ; MR I,,,,,, ; : : 015, 016; :, ; : ( ) ; PAS ; ; ( - ) : ( ),,,,,, : PAS TB 116 ( - ) (?) 2 211 ( ) :,, 1, :,,,,,, 2 /6 SM, : 1, 6,,,,, CSF, WBC,, CSF, CSF TORCH : PAS, ( - ) :,, PAS ( - ),,,,, CSF WBC,, CSF, : ( 1) :,,,, CSF,,,,,,

670 B asic & C linicalm edicine 2006126 (6), T, CD4 + T, CD8 + T, :,, 20% 75%,,,,,,,, CT,,, :,,,, CNS,, CSF,,,,,,,, :,,,, 7 8 9,,,, CSF,, CSF, ( 2) :, 6 4,, CT,, CSF,,, CT, ( 3 ) :,, CSF WBC < 100 / L,,,, ; CSF ( 4) :,, UCG,, IE ( 5) CNS : :,,,,, :,,, CSF :,,, ( 6 ) CNS : ( 7 ) : :,,, CSF,,,, :,,,,, ( + ), ( + ), :,,, CT;, 45 : 2 2,,,,,, :,,,,,,, 2 2 2 ;,,,

2006126 (6) Basic & ClinicalMedicine 671 :,, 7 10 d, 80% 90%: 80%, 2, ph ;,, ; : B, 2 4 g,, B, ( posaconazole), 1983, 2, ( ), CNS, 2, 2,,, M EDL INE : 1960 2005 34, = 3 2, (32 11) (16 68 ) 34, 22 (6417% ), 12 ( 3513% ) 20 B 12,, 40% ; 8 14 B 13, 9219%, B 2, 34 1 :,,, T CD4 ;, ;, ;,, CNS,,,,, CSF, CNS : SLE:,, 2 2, SLE, :, : CNS,?, : :?? : 4, 200 mg/d B 25 mg/d, 556 m g? 212 ( ), 1 ; CT, CT CT,, CT, CT MR I CT T1 T2, MR I + MRS T1 T2 ;, ( T1, T2 ), ;, ;,, MR I ;,, MR I, ; CT,, : :,,, :,, MR I,, :, :

672 B asic & C linicalm edicine 2006126 (6), 213 ( ), :,, :,,,, CSF,,,,,, CSF,,,,, CSF,,,,, CSF,, CNS :, ; 2 CNS, (1, 1 ), 1, 1,,,, ;, PAS,, CNS 214 ( ) :,,,, ;, ;,,,,, ;,,,,,,,,,,,,,,, 215 ( ) ; PAS; ( ) ( - ),,,,, 6 10 m,,, GM S,,,,,,,, B 216 ( ) MR I,, CT,,,, 217 ( ) CNS,,,, 10,,, T,

2006126 (6) Basic & ClinicalMedicine 673, CD4 + T CD8 + T, CNS 218 ( ) :, 1 CNS,,, 60%,,,,,,?,,? :,,,,

2006 7 26 7 Basic & C linical M edicine July 2006 Vol. 26 No. 7 : 1 00 1 263 25 ( 2 0 0 6 ) 0 7 20 7 9 8 20 5 (19),,, (,, 100730) 1 B 5 d,,, 2,,,, 51, 1 112 T: 3911 P: 120 /m in R: 22 /m in Bp: 160 / 111 80 mmhg 1, ( 3814 ),,, < 1, cm,,,, Pro2 + Ery5 +,, CRP, ALT 48 U /L ( - ), 120 /m in,, SM Murphy s ( - ) UCG B ( - ) 113 Still, 114,, 39 ( Tmax 41 ),, WBC, 2015 10 9 /L, NEUT%, Hb ( 117 87g/L ), PLT CRP 195 mg, ESR 112 mm /h ENA ASO RF ( - ) 3 ( + ) PPD ( - ) 11411 : Hb 90 g/l, WBC 20125 10 9 L - 1, N 8812%, E 415%, L 415%, PLT 337 10 9 L - 1, RET 1122% 11412 :, 76% 84%, 2% 11%, 1% 11413 : 0 - TRACE ( - ) ALT 302 79 U /L, ALB 24 g/l 24 h 0115 016 g 5,, 1 2 /d,, 3 kg : 1980 11414 +OB ( - ) 11415 : ALT 89 U /L, AST 96 U /L, GGT 81 U /L, ALP 177U /L, LDH 933 U /L, ALB 26g/L, Cr 0183 mg/dl, +, 11416 PT + A: PT 1316 s, INR 1115R, Fbg 1999 3 4, 150 /80 mmhg, 49318 mg/dl, APTT 4012 s, TT 3410 s 11417 ESR 109 11 mm /h, CRP 17 6190 mg/dl : 2006-04 - 10

2006126 (7) Basic & ClinicalMedicine 799 11418 : AST 97 166 U /L, LD 1076 1422 U /L, HBD 602 776 U /L, CK 100% CK2MM 11419 : SI 23 ug/dl, TIBC 391 ug/dl, TS 6%, SF 1000 ng/ml 114110 IV 260 ng/ml 114111 : ANA dsdna ENA ANCA RF LA ( - ) ACL ( + ) - ( + + + ) : 1 SMA ( + ) 1 80, 1, ( - ) : ALB38%, a1 814%, a2 1612%, 2513% Ig : CH50 6015 U /ml, C3 165 mg/dl, C4 ( - ) 114112 : HBV H IV HCV RPR ( - ) PPD ( - ) TB 2Ab ( - ) EBV: IgG/VCA 01377 ( ) CMVpp65 ( - ) TORCH1 RV 2IgG ( + ) 11 160, CMV2IgG ( + ) 11 256, HSV2IgG ( + ) 11 64 - ( - ) 3 ( - ) 1 MRSE, 2012 h, 114113 : CA ( - ) CA125 65 1 U /L 114114 : B : UCG: (56 39 mmhg) ECG ; CT ( 6-16) :, ; ; CT ( 7-8) :, 114115 : : ( ) ; : 114116 : 1, 27%, 2,,, ( - ) : O 95%, I 5% ; + (2 ) :,, / 114117 :,,,,,,,, :, : CD3 CD20 CD79 CD68 MPO ( + ) 114118 : ( ),,,,,,, : ( + ) 114119 : 0106 ml /m in, 2 8197 mg/l 115 1 /3 q12 q8h,,,,,, 60 g/l, 2u, 10 g 13, 1,,, 5 d,, 15 mg q 12 h, 3912,, 25 mg q 12 h, 116 : :?? 2 211 ( ) CT,,, CT,,,, 212 ( ), 2,,

800 Basic & ClinicalMedicine 2006126 (7) +,, NSA ID s, Hb,,,, NSA ID s, 11 Still :,WBC,,, 21 : :, ANA, SMA ACL, 31 : WBC,,,, LDH > 1000 U /L,,, 41?,, ; 51 : +,,,? 2,? : 11? 21,?? 31? 41? 213 ( ), 2, :, (,,, ),, Hb, A lb, ACL ( + ), ANCA ( - ), Still 11, Still 30 21, 2, Still,,,,,?,,,, ( ),, 11, 21, V Gotten,,,,,,, Jo - 1,,,, MTX 214 ( ),,,,,,,,, T,, 215 ( ), CT,,,?,,,,?,,,, CT, 216 ( ), 2,,, (CRP ESR SF D - dimer ),

2006126 (7) Basic & ClinicalMedicine 801 LDH, LDH,,,,,,,,,,,,,,,, 11,,21 PCR - HH? 31, PET 41,,,,,,, 2110 ( ),,,, Hb 8 9 g/dl,, LDH,,,, EPO,, EPO,, EPO 2111 ( ) 217 ( ), ACL ( + ),, CT,,,?,,,, 10 15%,,, MR I,,,, MTX,,, 2112 ( ) DNA,,,,,,,,,,,, 2113 ( ),,, 218 ( ),,,, 219 ( ),,,

802 Basic & ClinicalMedicine 2006126 (7) ( ),,, ( - ),,,, ( ) 2114 ( ),, : ;, ;, ;, ;, ;,,, (,,,,,,,,,,,?,,, Hb PLT WBC Hb,WBC,,,, EPO,,,,,, Hb 2115 ( ),, Still,,,,,,,,,, Still ), 150 Still,, 3 300 mg Qd 10, CTX 2, WBC 1018 10 9 L - 1, Hb 93 g/l, PLT 336 10 9 L - 1, : ALT 26 U /L, ALB 317 g/dl, GGT 49 U /L, ALP 99 U /L, LDH 256 U /L CTX 110 iv drip, 1 /,, 1 WBC 12129 10 9 /L, Hb 99 g/l, PLT 314 10 9 /L,

2006 9 26 9 Basic & C linical M edicine Sep tember 2006 Vol. 26 No. 9 : 1 00 1 263 25 ( 2 0 0 6 ) 0 9 21 0 4 1 20 6 (20) 1, 2, 2 ( 11 ; 21, 100730),, 39 5, 3 1 111 5, 3 5 d,, 3, 3815, ;, ;,, 3915,,, CT, B,???,,, 41, 1, T,,,,, : UCHL21 ( + ), CD3 ( + ), CD20 ( + ) CHOP ( CTX 800 mg d1, 600 mg d8 + EP I 70 mg d1 + VP216 100 mg d12d5 + Pred 60 mg d12d5), 4, CT, ( IFO 2g d123 + DDP 40 mg d123 + VCR 2 mg d1, d8), CT IFO 2 g d123 +DDP 40 mg d123 +VCR 1 mg d1, d8) 3,, CT 2 N IH / NC I,,, 3 CT CT 2 ( IFO 2 g d123 +DDP 40 mg d123 + VCR 1 g d1, d8) 3, TCR: 128 /A ( + ), 10 ( - ), 11 ( - ), ( - ) ; IgH: VH ( - ), R3A ( - ), R2 ( - ) ; IgK ( - ),,, ; ; ;, 9 kg : 1992 EB 1998 : 2002 5,, : 112,,,, 80m in - 1,,,, : 2005-10 - 29

1042 Basic & ClinicalMedicine 2006126 (9),,, 113 7 114 : ESR 33 mm /h, CRP : ALB 5318%, 2318%, A /G 1116 ANA + S 1 160, SMA + 1 80, ds2dna ENA ACL LA ANCA RF Ig 24 ( - ) T : CD4 + T, CD8 + T, CD8 + T : CMV2PP65 ( - ), TORCH: RV2IgG ( + ) 1 80, CMV2IgG ( + ) 1 128, HSV2IgG ( + ) 1 64 EBV2IgA /VCA 01630 ( < 0129) : WBC 3169 6103 10 9 /L, N 4813% 5516%, Hb PLT : N 39%, L 44% V itb12 :M E 1182,: Hu Yo R i ( - ) :, PET :, CT :, 3,, CT :,, : ( ),,, ( ),, : UCHL21 ( + + ), CD20 ( + ), CD68 ( + ), EBER ( - ) : CD3 ( + + ), CD20 ( + ), CD79 ( + ), TTF - 1 ( - ), CD34 ( + ) 2 211 ( ) CT :,,, CT, 212 ( ) CT,,,,,,,,, W egener, W egener,,,, PET 3,, 213 ( PET ) PET: 11, 21, 112 1215,,, PET,, 214 ( ) :,,, CT PET 3,, N IH /NC I,, CHOP,,

2006126 (9) Basic & ClinicalMedicine 1043,,, 3 : 11, ; 21,, ; 31,,,,, ( 5% 8% ),,,,,,, CD4 + T, ( B ), EB,, 4, 38 88% 4,,,,, 3,,, 2,, 500 600,,, 150 30 50 215 ( ) (215 85 ) ( : 1, 615 1) 90% : 9, ( 6 3),, (40 60 ), 30% 70%, (,, 80% ),, (6616% ), 3313% PaO 2 6616%, 1 67 mmhg(40 100 mmhg) 80%,,, (1 8 cm),,,,, 40%, 25% (5, ( 36% 53% ), 3, 1 ) 10% 35%, 41, 2000 8,,, CT,,, CT, ( ), CD4 +, CD20 +, CD +, SMA - CTX +VCR + Pred, 21 d 1, 4 CT,, 1, CT L iebow

1044 Basic & ClinicalMedicine 2006126 (9) 1972, 30,,,,,,,, ; 1 /4,, ;,,, (,, ) W egener,,,,,,,, 1982 Fauci, [ 1 mg/ ( kg d), 2, 2 ] ; ; [ 2 mg/ ( kg d), 37 ], 5 ; IgM IgG 46%,, 17, 5 2, 1113, 65% 90%,, 50%,, ( ) 15% 25%, 9 1 10, 1,,, 216 ( ),, CT,, W egener,,,, PET,, ;,, ;,,,,,,,

2006126 (9) Basic & ClinicalMedicine 1045,,,, :,,,,, PET, ;, 3, ;,,,,,,, N IH /NC I,, B, EB RNA, T B B ; B,, ; B,, B, ;, ;, 5,,,,,,,, 217 ( ), 80,,,,,,, 3-5cm, 3 3,1,,, 10,,, CHOP, 5,,,,, CHOP,,,,,,,, 2,,, :,,,,,,,,, 5,,,,,, 218 ( ), : ;;,,, 3, 1, 2,, 2,

1046 Basic & ClinicalMedicine 2006126 (9) 1,, 2,,,,,,,, 219 ( ) 1,, EBV 2,, 1, EBV, 5 20 3,,, EBV 2110 ( ),,,, W egener,,ct,, 2110 ( PET ) PET PET,, CT B, 2111 ( ),,, 3, ( ),,, B IOCOMPARE (2006 /8 /3), Scripp s ( an2 ti2obesity vaccine),,, ( yo2yo dieting), 8 4 PNAS, 2ghrelin Janda : ghrelin,, 1999 ghrelin,,,, ghrelin,,, ghrelin,,,, ( Immunopharmacotherapy), Ghr1, Ghr2 Ghr3,, Ghr1 Ghr3 ghrelin, ghrelin Ghr1 Ghr3,, ghrelin ghrelin,,,,

2006 11 26 11 Basic & C linical M edicine November 2006 Vol. 26 No. 11 : 1 00 1 263 25 ( 2 0 0 6 ) 1 1 21 2 8 6 20 7 (21),,, (, 100730) 1 111 1, 69 1, B ( - ), 3 2 cm 017 cm,, PT + A, B + 2, 34 /m in, 130 m in - 1,, 86% CT (CTPA ), ICU,,, APTT2R (2 ) 2 3 11 d 3 mg Qd 14 d,, PT2INR ( 2 ) 2 3 25 d, 3, 112 2, 731, 115 110 cm, 115 210 cm; : 2, : 30 ; 5 ; 4 : ( - ) PT + A, UCG:, ; ; ; ; + + 13,,, 10 L /m in, : 89%, BP: 150 /70 mmhg, : 120 m in - 1, 3075 IU 2, 15 m in 47%, 35 m in, 2 3 311 ( ) 1985 2006, ICU / 28, / (76251 ) 01037% 28 : 14 5010% 5 1719% 4 1413% 3 1017% 1 316% 1 316% : 2006-06 - 18 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (11) Basic & ClinicalMedicine 1287 : 26 85, : 6315 11, 17, 1 1155 10, 3517% 1 30, 615 d : 9219% 90% ; 6719% /, / ; 25% / ; 711%,, : 6 D2D immer (D2 ), 4 2 FDP ( ) 13 V /Q, 12,92% 5 CTPA, 4 1, : 5711%; 2510% + ; 711% ; + 316%, 2, : 7510%, 2510% /, 5 /7 (pulmonary embolism, PE),, 90% PE ( pulmonary thromoembolism, PTE) PE ( deep vein thrombosis, DVT) ( venous thromboembolism, VTE) PTE DVT, DVT ) ; DVT 1) : 20% ( 2) : 44% ; 3)(LDUH) : 68% ; 4) (LMWH) : 76%,,, DVT 40% 60%,,, 312 ( ), VTE > 1 /1000, 50% PE 3 15% 25% 75% VTE 1 ( ET PV) ;APC ( FV Leiden ) G20210A ( PC PS AT2III) VTE, DVT 80%, DVT PE, 50% PE DVT, PE PE, 70% DVT, DVT ; DVT PE, VTE PE, W ells ( Thromb Haemost, 2000, 83: 4181) PE, PE,, 3 :, < 4% ; ( PE) :, 5% 10% ; ( PE) :, 30% PE CTPA /, D2 VTE 98%, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

1288 Basic & ClinicalMedicine 2006126 (11) 99% VTE, FDA PE r2tpa (LMWH ), LMWHAPTT,,, LMWH 5 d, PT2INR 210 310, LMWH, (ACCP),, 3 ;, 6 ;, 12 313 ( ) / (V /Q) ( PE), ( 7 9 m ),,,,,,, ( 99 Tcm2DTPA 99 Tcm 2GP ), < 110 m,,,,,,,,,, PE V /Q PE PE PE, PE 100% 90% 50% 10% 0% PE PE, ;, CTA,,, ;; PE, CTA PE, CTA V /Q 314 ( ) CT1992,, CT,, 30 m in, 5 mm, 1998 4 CT, CT, 16 64 CT,,, 16 CT, 1 mm 016 mm 93%, 572%, 1998 CT ( combined multislice CT venography and pulmonary angiography, CTVPA ), 170 s,,,, (multip lanar reconstruction, MPR) (maximum intensity p rojection, M IP) ( volume rendering technique, VRT) ( curve p lanar reconstruction, CPR ),,,, MPR,,,,, 7% 12%, PE,, CTPA,, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (11) Basic & ClinicalMedicine 1289,,M IP,,,, 70%, 6215% CTV,, CT,,, X, CTPA, 87%, CT X, 86% 98%, 66%, X CT, CT,, CT,, CTPA, X CTPA,,,, CTPA,, CT CT,, CT DVT,,,,,,,, ACCP,,,,,,, - DVT, 100%, X, CT, PE CTPA CTV, 5% DVT PE, DVT, CTVPA,, X, CT,, CT,, 315 ( ), 1996 1 2004 5 303, 50, 1615%, 27 79 ( 57 ), 50 14, 28% : 16%, 14%, 12%, 10%, 8%, 4%, 6%,, 8%,,,,, DVT, : 1) 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

1290 Basic & ClinicalMedicine 2006126 (11) 2),, PO 2 3), 4)1 14, 3 4,,, 50 44, 8 6,, 6 : 1),,,,, 2),, 3), 4),, : 1),, 2), 3) DVT,, 316 ( ICU) ( PE) (DVT),, (VTE) DVT PE Geerts 2001 Chest DVT ( ) 29%, PE 116%, PE 019%, 80% DVT, VTE 11VTE (1) (2), ( GCS) ( ICP) (3), (LDUH ) (ADH) (LMWH) VTE, VTE : ) ( 1 2 3 5 ( > 45 m in) ( > 45 m in) > 72h 2 2 2 : : 41 60 (1 ) > 60 (2 ) DVT/PE (3 ) (1 ) 1 (1 ) (2 ) (1 ) (BM I > 25) (1 ) (1 ), : 20210A Leiden ( C ) S C 3,, VTE 1 2 3 4 5 GCS ICP GCS ICP GCS ICP LDUH /LMWH LDUH /LMWH LDUH /LMWH, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (11) Basic & ClinicalMedicine 1291 VTE, 2004 7 ACCP: :,,,, : VTE < 40,,, 40 60,,, 40, 5000 U, 2, 3400 U, 1 > 60, > 40, 5000 U, 3, 3400 U, 1, ( 5000 U, 3, 3400 U /d),,,, 21VTE (1) : VTE, : 14 d : UK ( ) 2 U / kg, 2 h, rtpa 50 100 mg 2 h 2 PT APTT, 2,,, 7 ACCP,,, VTE,,,, : 5 d, PE, 10 d 1,, INR 2, ICU 3, 3 5, INR,,, : Xa IIa, ;,,, APTT, (, ), 4 ( PF4 ),, (H IT), (Ccr < 25 ml /m in) ( > 150 kg), (UFH ), (2) VTE,, VTE,,, PE, PE,, 3 ;, 6 12 ; 2,,, 2 2 PE, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

1292 Basic & ClinicalMedicine 2006126 (11),,, INR 210 310, (3),, INR 210 310,, LMWH UFH UFH VTE 3%, APTT2R 115 215,,, 2( H IT),, 4 d,, 100 150 10 9 /L, 5 d, IgG, 100 10 9 /L, 1 /3,,,,,,,,,,, VTE VTE VTE, 317 ( ICU),,,, VTE,,,,,,,,,, adalimumab EvanstonKenneth B. Gordon 10 (J Am Acad Der2 matol, 2006; 55: 598-606. ),, G1 adalimumab 3 Gideon Koren 9 ( Fertil Steril, 2006; 86: 658-663. ), 3( PCOS),, 9 (J Clin Psychiatry, 2006; 67: 1422-1427. ), Kuop io Tuula H. Heiskanen,, 1 /3 Heiskanen,,, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006 12 26 12 Basic & C linical M edicine December 2006 Vol. 26 No. 12 : 1 00 1 263 25 ( 2 0 0 6 ) 1 2 21 4 0 2 20 7 (22),,,, (, 100730) 1 2006 2 B MR I ; ( ) ;, 3,,,, 24,,, G 0 P 0 1, 10, 112 10 BP: 120 /80 mmhg, ( - ) ;, 111 ;, 12 4 5 /30, 15 cm;, ( - ), ; 2003,,, 2003 12 113 4, 10 cm, :,, ( ),, ;, ( 1 2 /10HPF) 114 2005 5 B, 11411 : ( ) : (76 81 35 mm ), ( ) :,,, + + 6 6 5 cm 3 11412 :MR I( ) :,,,,,, ;;,, 5 5 ( 1) CT :, 4 cm 3 : ( ),,, ( 4 5 ; /10HPF), ( ), ( 2) B : 612 418 3,,, B 319 cm, 014 cm, 1016 919 ; 4 814 cm,, CDF I B ; 316 215 cm; 410, 118 cm;, 619 212 cm, 2005 9 : ; DDP 50 mg + EP I 40 mg, ( ), CDF I,; 1011 611 : 2006-06 - 18 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (12) Basic & ClinicalMedicine 1403 514 cm, (119 cm), (017 cm ), 1019 419 316 cm,, : ; ; ; 116? ( ) 2 1 M R I 2 211 ( ),,,, 14,, ;, CT MR I ;,,,,,, 212 ( ) 1,,,, ;, ;,,, ; 7,,,,,,, 11413 : : ; W arm2like,, 48 mmhg, :, ( ), :,,, ;, > 5,, ;, GFR: 10814 ml /m in, 2012 ml /m in,, 8812 ml /m in;, 7 2,, 5,, : CA125 CA199 CEA AFP, uncertain low2 CEA grade ( ) 115 213 ( ), MR I,, CT, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

1404 Basic & ClinicalMedicine 2006126 (12), CT,, CT :;,,,,,, 214 ( ),,,,, DJ,, 215 ( ),,,,,,,,,,,,GnRH2a; GnRH2a GnRH2a 216 ( ) 4 200, 7, 2 21, 72,,,,,,,,,, 217 ( ) 1, ;,,,,, 45,,,,,,,,,,,, 218 ( ), ( 48 mmhg),, UCG,, 219 ( ),,,,,, 2110 ( ),,,, 60 70%, CD117 CD34, 2111 ( ), 2,,,, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (12) Basic & ClinicalMedicine 1405,, 2112 ( ) :,, 2,,,,,,,,,,,,,, IVP, 5000 ml 3, ( 4), ( 5), 8 8 5 cm, 70 /30 mmhg, 20 g/l,, 6 cm,, 13 cm,,, 10000 ml, 6000 ml,, 311 :, ; ( 3), IVP:, :, 4 3 312,,,,, 313 5,, 1 2 /10HPF; ( BV0 /2) ; ; 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

1406 Basic & ClinicalMedicine 2006126 (12) ;;, : CD34 ( + ), CD117 ( - ), Desm in ( + ), ER ( - ), PR ( + ), Ki267 ( + ) 6 314 6,, 5 cm 3 cm 250 mg B id 4 13 8, 7 1: 43, 218, 113 7 4, 1, 2 1,,,, 4, 1, 2 : 4, 2, 1, 2 13, 1,,, 1896, 1 / 1 36 3 /1 12 12, TAH + RSO 9 5 5 cm 2, IVL 2 38 3 /1 6 cm 10 10 cm TAH + IVL 5 3 56 4 /2 4 cm 4 cm TAH = BSO + IVL 6. 5 4 58 2 /1 10 5 31 3 /2,, 16 TAH +BSO IVL 8 5 015 cm, TAH +BSO, 18 cm, 13 IVL 6 40 3 /1 6 TAH + IVL 9 cm 10 cm 415 7 43 2 /1 5 10 TAH +BSO 5 IVL 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2006126 (12) Basic & ClinicalMedicine 1407, 200, 50 28 80, 45, 90% 24, 30%, 10 30% 8 2,, : ; [ 1 3 ],, [ 4 ], 2,,,, ;,, MR I CT,,, [ 1 ],, [ 5 ],, 4, 1, 2,, 0,, :, /,, ;,,,, 1,,, 1, 3, 1,,,,, CT MR I, 5,,,,,,,, 7, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

1408 Basic & ClinicalMedicine 2006126 (12) : [ 1 ] Merchant S, Malp ica A, DeaversMT, et al. Vesselswithin vessels in the myometrium [ J ]. Am J Surg Pathol, 2002, 26 (2) : 32-36. [ 2 ] Quade BJ, Dal Cin P, Nesky DM, et al. Intravenous leio2 myomatosis: molecular and cytogenetic analysis of a case [ J ]. Mod Pathol, 2002, 15 (3) : 351-356. [ 3 ] Dal Cin P, Quade BJ, Nesky DM, et al. Intravenous leio2 myomatosis is characterized by a der (14) t (12; 14) ( q15; q24) [ J ]. Genes Chromosomes Cancer, 2003, 36 ( 2) : 205-206. [ 4 ] Kokawa K, Yamoto M, Yata C, et al. Postmenopausal in2 travenous leiomyomatosis with high levels of estradiol and estrogen recep tor[ J ]. Obstet Gynecol, 2002, 100 ( 5p t2) : 1124-1126. [ 5 ] Merchant S, Malp ica A, DeaversM T, et al. Vessels with in vessels in the myometrium [ J ]. Am J Surg Pathol, 2002, 26 (2) : 32-36.,, 22 212 262 [ 4, 52b ] ( Ph IP),, (BaP), Ph IP,,, Ph IP 137 BaP ( ) Ph IP 29361 1338,, 868, 520 ( III IV ), 10 g,1. 4 ( 95% : 1. 0521. 92),,, Ph IP Cancer Res, 2005, 65 (24) In conclusion, very well done meat was positively associated with p rostate cancer risk., 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2007 3 27 3 Basic & ClinicalMedicine March 2007 Vol. 27 No. 3 : 1 00 1 263 25 ( 2 0 0 7 ) 0 3 20 3 5 3 20 5 (23) 1, 2 ( 11 ; 21, 100730), 34 4 1 111 4, 3815,, 10, 1 /, 1,, 112, ( ),,,,,, 2 d, 170 /110 mmhg,, 3 mm,, PaO 2 4418 mmhg (, 1 mmhg = 13313 Pa), SaO 2 87%, 113 39 40, : : WBC ( 7146 ( ),, 911) 10 9 L - 1, N 65% 6918%, Hb 120 g/l; ALT 27 U /L, AST 30 U /L, GGT 17 U /L, ALP, 50 U /L, TP 69 g/l, A lb 41 g/l, Cr 141 mol/l, CHO 20 d,, 315 mmol/l, TG 211 mmol/l, LDL 212 mmol/l; (ARDS), ; ; PT +A ( - ) ; PaO, 500 mg/6 h, 75 mg, 2 300 mg, 40 mg WBC ( 311 9019 mmhg, SaO 2 9612% ; ESR 14 mm /h; CRP 1011 ) 10 9 L - 1 3158 mg/l; HB, Hb 80 108 g/l, Cr 0192 sab HBcAb ( + ), H IV HCV ( - ) ; RPR ( - ) ; ( - ) ; PPD ( - ) ; Ig 2129 mg/dl, HAV HCV H IV ( - ) ; ( - ) ; ANCA ( - ) ; 2 + ; 2 ( - ) EBV CMV ( IgG + ) :, ESBL ( - ) ; 1 + HB sab HBeAb HBcAb ( + ) ; ( - ), MRSA; : MRSA canca panca ANA dsdna SM ( - ) ; CH50 C3 C4 1,,, 3 d,, 10 d, 30 mg/d 3 5 mg 5 mg, 300 mg/d, 2, CT CRP 16 mg/l; ESR 26 mm /hr; ( - ) ; ANA dsdna ENA Jo1 Scl ( - ) ; ; 1997 : 112 10 3 L - 1, 10 3 L - 1, > 100, 4 ( - ), ( - ) : B : ; : 2006-09 - 09 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

354 Basic & ClinicalMedicine 2007. 27 (3) ; ECG ; : : :, ; : ( ), 114,, 1, 3719 3810,,,, 3711 3715 :,, : 2, 10 cm 10 cm 5 cm,,,, 1 cm,, : 10 cm 5 cm,??,,, (CTX) 200 mg 2 211 ( ) 11 d,, 22 d, 1,1 CT,,, 3 CT,, CT, CT, ( 1) 212 ( ) :,,,,,,, 1 CT,,,,,, ( DAH) DAH : ( 1): (APS) Goodpasture ( HSP ) IgA (MPA) ( SLE) (W G) ; (2) : D IC : ( 1) : W G? MPA? (2) Goodpasture? ( 3 ) :? : W G W G : ; ;,, canca DAH W G : Travis 87 W G, DAH 7% (Am J Surg Pathol, 1991, 15) DAH 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2007. 27 (3) Basic & ClinicalMedicine 355 W G : DAH ; ;;, (CTX) 66% W G, DAH,, canca, W G,, (MPA) : MPA, 50,, DAH,, 80% ; ANCA,, MPA Goodpasture :, DAH,, GBM, : ( IgG) GBM 27%, :, DAH, :, CT, ; ; :,,?,? CTX? 213 ( ),,, 6 5 mg, 214 ( ),,,,,, CT,,,,, 215 ( ),,, S ( ) K ( ) L ( ) E ( ) N ( ),,,,MPA, 18, 216 ( ) : IgA + +, IgG C3 +,,,,,,,,,, IgA, 217 ( ),,,,,, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

356 Basic & ClinicalMedicine 2007. 27 (3), Churg2Strsuss,,,,, IgA, IgA, IgA,,, IgA, IgA, IgA 218 ( ),,,, 219 ( ),,,,,,,,,,,,,, 2110 ( ),,,,,,,,, 2111 ( ),, + CTX,,, 2112 ( ),,, ARDS,,,,,,, 2113 ( ),,,, 2114 ( ),,,, 2115 ( ),,,,,, T, 3 60 mg/d, CTX 600 mg : ( ), ; ( ), : ( - ), ( - ), AB /PAS ( - ) ; CTX,, CTX800 mg, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2007. 27 (3) Basic & ClinicalMedicine 357 2 CTX CT ( 159112 mol/l 114192 mol/l ), CTX 1 g 2, 20 d CT:( 2),20 mg/d, CTX 514 g, 8814 mol/l,,, ( growth2stimulatory pathway) RT2PCR W estern (NSCLC) 86%, EL ISA, ( anastrozole),,, A549, 21,, Cancer Res, 2005, 65 (24) Treatment of tumor cells in vitro with anastrozole led to significant supp ression of tumor cell groath. 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2007 4 27 4 Basic & C linical M edicine Ap ril 2007 Vol. 27 No. 4 : 1 00 1 263 25 ( 2 0 0 7 ) 0 4 20 4 7 2 20 4 (24),,, ( 100730),, 25, 1, 3, 1 1 111 1,,,, 38,,,,,,,, 3715, ; RBC 317 10 12 /L 2101 10 12 /L; Hb 117 g/l 59 g/l; PLT 231 10 9 /L 93 10 9 /L, ESR 103 mm /h CRP 6122 mg/dl CH 50 6216 u /ml C 3 214 mg/dl LDH 960 u /L 3 d,,,,, 10 kg : 3 : 112 T: 3710, P: 90 /, R: 21 /, BP: 130 / 60 mmhg,,,, 3 4 cm,, ; ( - ) 3 ; Kernig ( - ) ; ( - ) 113 11311 : : Hb 31 g/l RBC 1118 10 12 /L WBC 3104 10 9 /L, PLT 28 10 9 /L + OB: : GGT 91 u /L ALP 894 1314 u /L AST 122 133 u /L LDH 784 1514 u /L OB ( + ) : > 330 mmh 2 O 35 /mm 3 1 /mm 3 10512 mg/dl, ( - ) 11312 : :, ; (8% ) (2% ) (1% ) ( ), :, 11313 : : CH 50 5912 U /ml, C 3 C 4 ; IgG 6148 g/l IgM 01506 g/l; ESR 40 mm /h; ANA dsdna ENA ANCA ACL ( - ) 11314 : TORCH: CMV2IgM ( + ) ; H IV EB 2Ab CMVpp65 ( - ) 11315 : CA125 4216 U /ml, AFP CA192 9 CEA 11316 : ; CT: B : CT: ; MR I:, :, :,, : 2006-10 - 17 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2007127 (4) Basic & ClinicalMedicine 473 114, 38,,, CT,, CNS, ( 5 mg) ( 250 ml Q 4 h + 250 ml Q 12 h) ( ) 200 mg Qd,,, WBC, PLT > 5 /mm 3,,, : BP 130 /75 mmhg,, 3, ( + ), ( - ) : Hb 52 g/l, PLT 21 10 9 /L 2 211 ( ),, CT,,,, :,, (MM ), 218 MM 30, MM ( acute myelofibrosis, AMF) : (MF),,,, MF AMF,,,,, 1 AMF ( AML2M7), MF ; AMF, :,, LDH, 212 ( ),,,,,,,, ( ),,, : (1),,, ( 2 ), (3),, ( 4),, : (1),, (2), (3),,,, 213 ( ), 3,, :,,,,,,?? MRA, CT,,,, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

474 Basic & ClinicalMedicine 2007127 (4) 214 ( ),, ( 1),, ( 2), AE1 /AE3 ( + ), : 1 F ig 1 Large ma lignan t cells in bone marrow w ith little hem opo ietic tissue and hyperpla stic f ibrous tissue 2 F ig 2 Imm unoh istochem istry of bone marrow AE1 /AE3 ( + ) 215 ( ),, :,,, :,,,, :, KPS,, :, :,,,, :( ), ; 177, 1195: 1, 4617 ( 4 81 ) 8311%, 5519% 4011% 4512%,, 216 ( ),,;,,, ;,,,,,,,,,, 3 : ( 1), ( 2) (3) 217 ( ),, :,, 1 /3, 2 /3 5 85 90%, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2007127 (4) Basic & ClinicalMedicine 475,, : (1) :, (2) : (3), 50%,,, 8, 2 3, 2148,, 2148 : (1) :, ( 2) (3) : 3 9,, 50%,, 218 ( ), : (1), ; (2), ; (3) (MF), MF, MF MR I, ECT,,,,, Anthony R. Green 2 1 (N Engl J Med, 2007; 356: 459-468. ),, Janus (JAK) 2, JAK2 V617F, V617F,, V617F,, ADHD, Gelderse Vallei Marcel G. Sm its 2 (J Am Acad Child Adolesc Psychiatry2007; 46: 233-241. ), ( sleep2onset insomnia) (ADHD),, Sm its,, B IOCOMPARE (2007 /2 /6),,,,,, :,,,,,,,, Giovanna Mallucci, 2007 2 1 (Neuron) 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2007 5 27 5 Basic & C linical M edicine May 2007 Vol. 27 No. 5 : 1 00 1 263 25 ( 2 0 0 7 ) 0 5 20 5 9 3 20 4 (25) 1, 1, 1, 1, 1, 1, 1, 2 ( 11 ; 21, 100730) 1, 17 3 120 mmhg; 4174 mmol/l; ; ; ; CT ; CT ;, 111, 2,, 1 /,, ( ) 2003,,, : 1 2 ( 1),,, 112,,,, BP: 140 /100 mmhg, H t: 130 cm ( 3 3 ( ), W t: 3715 kg ), 14, ( 3 ), BM I: 2212 kg/m 2, 2 B,, ( + ), ( + ), ( + ),,,,,,,, 2006 5, BP: 160 / ( 2),, ( - ) 1 : 2007-01 - 31

594 Basic & ClinicalMedicine 2007127 (5) 2 113 : WBC 10121 10 9 /L, N 8018%, Hb 145 g/l, PLT 326 10 9 /L; + OB ( - ) ; ( - ) ; : ALT 42 39 U /L, ALP 209 149 U /L, K 4125 mmol/l, Cr 57 mol/l, GLU 412 mmol/l, TG 1168 mmol/l, CHO 6142 mmol/l, LDL2C 3143 mmol/l, HDL2C 2167 mmol/l; 24 h 0109 g; : FT3 FT4 TSH ; PRL 017 nmol/l; : E 2 17179 pmol/l, FSH 417 IU /L, LH 014 IU /L, T 213 nmol/l; GH < 0135 g/l; 18512 ng/l; 59 g/l; 3 h OGTT: FBG 411 mmol/l, 2 h PBG 818 mmol/l, 9182 m IU /L, 2 > 300 m IU /L, IGT ; : 1032124 nmol/l ( 8 am ), 771114 nmol/l (4 pm), 639136 nmol/l (0 am ) ; ACTH < 212 pmol/l (2 ) ; : 24 h UFC 45316 g/d, 450184 g/d, 24 h UFC 352192 g/d, 24 h UFC 414172 g/d; ECG:, ; UCG: ; B :, ; X :, ; : T2score 514 (L224) ; : 16 ; CT: ; B : ; B : ; B : ; MR I: ( 3) ; CT: ( 4),, 4 CT 114 10 mg q 8 h 120 / 80 mmhg, ( p rimary p igmented nodular adrenal disease, PPNAD ),, Carney ( Car2 ney comp lex, CNC), PPNAD 2006 9 18, : ( 5) ; : CgA ( + ), 2inhibin ( + ) 5

2007127 (5) Basic & ClinicalMedicine 595 115 Carney ( PPNAD) 2 211 ( ),, 3,,,,, ; 3, ; 2006 5 : 1 2 : BP 140 /100 mmhg, BM I 2212 kg/m 2,,,,,,,,,?? ( - ) :, ; ; ACTH < 10 ng/ml, ACTH :MR I ; CT, Carney comp lex, PPNAD Carney comp lex, 1985 J1 A idan Carney, ( PPNAD ) 40% 60% camp A ( PRKAR1A ) ( 17q22224) ; 2p16 Carney comp lex : ; : PPNAD ( GH ) ; :, ; : N IH ( 1998 ) Carney comp lex : PPNAD L iddle Sertoli (LCCSCT) B ( ) ; : PRKR1A, + 1 CNC : : IGT, ; : X2ray:, ; : T2score2511 (L224) ; B :, ; : CHO ; ; : PRL ; E2, T ; GH, 120 /80 mmhg ; ; ; Carney,,,, 212 ( ) Carney PPNAD,,?,,,,,,? 213 ( ) PTH, 214 ( ), ACTH < 10 ng/l,,,, PPNAD,

596 Basic & ClinicalMedicine 2007127 (5),,50 mg, 215 ( ) 2001 Carney,,,,,,, Carney,, 216 ( ), ACTH, PPNAD,,,,,,, 217 ( ),,, 3,, 218 ( ),,,,, 2,, 3,,, 115 kg, 110 /70 mmhg,,2006 10 8 : 295 nmol/l ( 8 am ), 281152 nmol/l (4 pm ), 26714 nmol/l ( 0 am ) ; 24 h 2818 g,, 4 4, 7 10,,10,, 24, 2007 7 7, 1500 / :38, : 100083 : ( suncw@ bjmu. edu. cn, 010-82801746) (phsiology@ bjmu. edu. cn, 010-82801455) :,

2005 1 25 1 Basic & Clinical Medicine January 2005 Vol. 25 No. 1 : 1 00126325 ( 2005) 012090203 1, 1, 1, 2 2, ( 11 ; 21, 100730) 1 (WBC) 712 918 10,,39, 31, 9 / L, ( PLT) 218 265 10 4 9 / L ; B : 510 cm, 5, 8 115 cm 2 6,, : 1 2 mm, ( - ), : :, 3 4,, : ( ) : K2F B 12,, :7,, 70 cm, :, 1, : (Crohn s disease) 10 mg 1/,2,,,,1 :,,, :,,,,,,,,,, 30, 1 7 1, B,Hb 50 60 g/ L, PLT 50 10 9 / L,WBC 3, 5 20 / d,,,,,b :, B 12,,, 1, : Hb 60 g/ L, ( 2 211, : (Hb) 73 111 g/ L, : ), 5 mg 2 2,, 10 /, 2003, :Hb 23 g/ L,WBC 217 10 9 / L,Plt 14 (1979 ) 1, :2004-06 - 14 :2004-07 - 14 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005125 (1) Basic & Clinical Medicine 91 47 10 9 L - 1 : (ALT) (713 % 12115 %),Alb 5312 % (5410 % 6517 %), 23 U/ L ( < 40 U/ L ), ( ALB ) 2415 g/ L ( 35 55 g/ L ), ( TBIL ) 0193 mg/ dl ( 011 110 mg/ dl ) 1 ( Cr ) 1175 mg/ dl ( < 115 mg/ dl) : FT 4 TSH : 20 g/ dl (80 180 g/ dl), 187 g/ dl (200 400 g/ dl), 1110 % (30 % 5 %) : B 12 :, 0102 ( > 0112) : 1215 umol/ L (1110 2210 umol/ L) :,WBC 78/ ul, ( + ), 11010 ; 018 g/ dl, LDH,, Hb 23 g/ L 47 U/ L, ALB 29 g/ L ( 5 g/ L),, ( - ) ( - ) ( - ), : :HbcAb HBeAb ( + ), ; 212 HIV2Ab HCV2Ab TB2Ab ( - ) ( - ) 21211 :,,, : AFP ( - ) CA125 102149 U/ ml ( <,, 35 U/ ml),ca199 CA242 CA50 CEA ( - ) :,,,,, 0153 ( <,, 0150) :,,HR :80 / min, II, BUS :,,, 117 cm[ (1117,,, ( + ) ( - ) 0113) cm],,, : CT( + ) :,,,,,,,,, :,,Hp ( - ), :, : 10 cm,,, : ( ), :,M : E = 1139 :1, 1, 21212 :Hb 16 35 g/ L,WBC (1157 319) 10 9 L - 1, PLT (23 49) 10 9 L - 1, ( + ) ; K2F ( - ) 2 2163 % : 3181 % ( 015 % 115 %), ( - ), 5 3 :, : ALT AST, 0193 2132 mg/ dl (011 110 mg/ dl), 0157 1138 mg/ dl (0 0120 mg/ dl), ( TP) 414-4172 g/ dl (610 810g / dl), ( ALB ) 212 217 g/ dl ( 315 515 g/ dl), A/ G, Cr 1175 1138 mg/ dl ( < 115 mg/ dl) ( ESR) : 24 mm/ 1 ( < 15) : 1 614 % (114 % 115 %), 2 1015 % 1812 %(1016 % 2315 %) :320 g/ L ( < 140 g/ L) T3 < 014 mg/ L (0166 1192 mg/ L), FT 3 118 pmol/ L (118 411 pmol/ L), T 4 :,, : UCG ECG : K2F (1),,, (2), ALB, CT, A/ G,, 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

92 Basic & Clinical Medicine 2005125 (1), (wilson disease, WD) 3 (3) WD :WD ;WD 20,, : 39, WD ; (1) :,,, WD K2F :,,, (4) :,, ( (2) : ),,, ( ), (5) : DNA,, WD ;, ( ) ( ) ( ) ( ) 2. 213 :2003 10 30 (3) WD : WD,, ( 3 000 ml) K2F,,,, WD 1 5 cm,,, K2F, 115 cm,, 190 cm,,, WD 7 9 cm, 7 8 cm ( - ), WD 20 cm, K2F,,, WD 112 m, (4) :,, 2,, 39,, 1, 3715 11 29, :WBC 1511X10 9 L - 1,Hb 50 g/ L,PLt 214X10 9 L - 1 (5) : 21214 : ( ),,,,,, (6) :, ( - ), ;, 4,,, 3,, ( ),,, 5 1994-2007 China Academic Journal Electronic Publishing House. All rights reserved. http://www.cnki.net

2005 11 25 11 Basic & Clinical Medicine November 2005 Vol. 25 No. 11 : 100126 3 2 5 ( 2 0 0 5 ) 1 121 0 7 120 3,,, (, 100005) 1 (1),,, 55 2, 1,,, 2,,,, 2 4, 400 500 ml/ d, :,,,,, CT, ;,, 1 / 5 ; 6 d,, 1,,,,,,, 1 2 / 10 d, ; :,, B (BUS) ; CT ; 30, %,,,,,, X, AL T 36 U/ L, TP 615 g/ dl, ALB 317 g/ dl, TBil 2171 mg/ dl, DBil 1124 mg/ dl ; (OB) ( - ) ; ; BUS,, ; CT, ;, ; ; ( ) ( 9 00 CT, 9 00 ) : 10, 2 (2) :,, ; : AL P, ( - ),,, ( - ), ( - ), : : :,,, /,,, ( ), ; : 65 U/ L, TB IL 4172 mg/ dl, DB IL 2166 mg/ dl,,,, GGT 108 U/ L, AST 69 U/ L, K 218 mmol/ L, Na :2005-06 - 17 :2005-06 - 22

1072 Basic & Clinical Medicine 2005125 (11) 13215 mmol/ L, CL 7317 mmol/ L ; (CA) ( PSA), ; : BUS 0163 cm, 6 15 ( 25 ( PSV) 3517 cm/ s ( ),, 60 ) ;, 36 ; 2 8 mm ( 10 28 mm) ; 914 cm 416cm,, < 10 cm ;,,,,,, ( 1), CT :,,, ; ( 2) : 1 2 CT ( superior mesenteric, ; 4 6 h ; ( ) ; X,, SMAS,,,, SMAS, SMAS, : ; ;,, HBV,, 6 cm 515 cm,,, Roux - Y :,,,, SMAS, artery syndrome, SMAS),,, 6 cm,, CT?,,,

2005125 (11) Basic & Clinical Medicine 1073, ( 2),,,, 1 CT, Diana O. Perkins 10 (Am J Psychiatry, 2005 ; 162 : 1785-1804),, Perkins,,,,, Certolizumab Christian2Albrechts Stefan Schreiber 9 ( Gastroenterology, 2005 ; 129 : 807-818, 1114-1116), TNF, Certolizumab Certolizumab II, Certolizumab pegol Tufts Brigitte T. Huber 9 (J Rheumatol, 2005 ; 32 : 1821-1831), (RA) HERV2K18 HERV2K18 HERV2 K18, EB,, T, RA Young Yull Koh 10 (Allergy, 2005 ; 60 : 1327-1330), BIOCOMPARE (2005/ 10/ 13),, (SIDS),,,,,,, 10 10 ( Pediatrics)