Hemolytic uremic syndrome(hus) ver0130525
(HUS), Stx VT 3 3 1. ( Hb10g/dl ) 2. ( 10 /µl ) 3. ( 97.5 ) 1.5 ) (HUS)
D+HUS D-HUS(aHUS) HUS ahus ( 6 HUS ( ) HUS HUS ahus
O-157 9-30 HUS 15 80 5 5 10 2-3 (O-157 O-26,O-91,O-103,O-111,O-121 ) (HUS VT )
STx VT Gb3( ) Gb3
3-9 ( ) 4-15 HUS ( ) 25 4 ( ) ( )
/ ( 3-9 )
/ (PLT Na,K,Cl,Ca,P,TP,ALB,T-Bil,D-Bil,γ-GTP, LAP, Lipase AST,ALT,LDH,BUN,Cre,Amy,Glu,, IgG,A,M,C3,C4,CH50,BNP,HANP,Cys-C,β2mg, PT,APTT,AT-Ⅲ,FDP,D-Dimer,Fib O-157,VT, LPS,,, Cre BUN ( ) STEC/VTEC Xp ( (,,,,IVC ) MRI( )
HUS HUS 1) 2)HUS ( 20000 /µl) Na ( 130 meq/l) ( 5.0 g/dl) ALT(GPT) ( 100 IU/l) *HUS scre 2.0 mg/dl
(1) HUS IN ( OUT CTR IVC
(2) ( 10ml/m2/hr) ( ) (HD) (PD) (PD) < > HUS
(3) (HUS ) 1 2mg/kg/ ( ) 2-10µg/kg/min ) CKD 2009 (4) Hb 5g/dl
CKD 2009
(5) 0.3mg/kg 20 mg/kg deep sedation 1-5mg/kg/h 1-5mg/kg/h 0.1-0.5mg/kg/h (66.1 87.8%) Ccr<10ml/min 50% ) HUS
(5) ( ) MRI diffusion ADCmapping),EEG
5)DIC DIC ( ) 0.06 0.20 mg/kg/h FOY( ) 20 39mg/kg 24 ( ) 380 U/kg 30 1 1 130U/kg ( Ⅲ) 30 U/kg1 1
HUS 1) 2)γ- 3) HUS 4) PGI2) E HUS
( /Cre, β MG ) (Cre, Cys-C, ),,DMSA ( ),, CT MRI ( )
Atypical hemolytic uremic syndrome (ahus) ver0130525
ahus (thrombotic microangiopathy, TMA) 3 HUS ADAMTS13 (thrombotic thrombocy-topenic purpura, TTP) ahus HUS 10 HUS ahus 25 100 2 100 7
( ) Definite: 3 (TTP) Hb 10g/dl PLT 10 /µl 1.5 AKI Probable: 2
( ) EIA LPS-IgM (TTP) ADAMTS13 (<5%) TTP ahus ahus ADAMTS13 10 ahus ADAMTS13 10% ADAMTS13 5 10 5 ahus TTP
( ) TMA DIC,,,APS,TMA Probable ahus, HUS ahus 6 HUS ( ) HUS HUS
ahus ( ) 1 ( ) H,I,MCP(CD46),C3, B, ( ) H (ELISA ) ( ) ( ) ( ) ( ) ( ) ( )HIV ( ) ( ) ( )
ahus ( ) ( ) ( ) ( ) (5) *6 ( )SLE ( ) (6) *1 ahus
/ Na,K,Cl,Ca,P,TP,ALB,AST,ALT,LDH,T-Bil,D-Bil,γ-GTP,LAP, Amy,Lipase,Glu,, IgG,A,M,C3,C4,CH50,BNP,HANP, PT,APTT,AT-Ⅲ,FDP,D-Dimer,Fib Cre BUN ( ) Xp, (,,, ), MRI Factor H / vwf
ahus HUS ahus first line therapy ( ) 1970 50% 25% FFP 60-75 ml/kg FFP 10-20 ml/kg( ) Loirat C et al.presse Med.2012 Mar;41:e115-35 Management of hemolytic uremic syndrome. S. pneumoniae-induced HUS PE
eculizumab( ) ( ) PNH (C5) ahus C5 C5
Table III ahus ( ) CFH/I/B: complement factor H/I/B; MCP: membrane cofactor protein; THBD: thrombomodulin; ESRD: end-stage renal disease Management of hemolytic uremic syndrome Presse Med. 2012 Mar;41(3 Pt 2):e115-35 Chantal Loirat1, Jeffrey Saland2, Martin Bitzan3
HUS ( 3-9 ) 6 HUS HUS HUS Typical HUS Atypical HUS