Hemofilija s pridruženo pridobljeno motnjo koagulacije. Irena Preložnik Zupan Dušan Andoljšek
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1 Hemofilija s pridruženo pridobljeno motnjo koagulacije Irena Preložnik Zupan Dušan Andoljšek
2
3 Primer 1
4 Posthepatitična HCV pozitivna jetrna ciroza D.U., 1963, hemofilija A, FVIII <1%, 0 BE Pri 11 letih zbolel za hepatitisom kasneje ugotovljen hepatitis C Od 25 leta - samozdravljenje na domu po potrebi ( on demand ) 3-5 krvavitev mesečno Huda hemofilična poliartropatija Do 35 let - zdravljen na Pediatrični kliniki
5 Posthepatitična HCV pozitivna jetrna ciroza 1998, 35 let - zaplet Dan pred sprejemom preko IPP na KOH Febrilno stanje, 38,5 C, bruhanje Bolečina in oteklina levega kolena Tremor Epileptični napad po opisu Prisotni žolčni kamni (UZ trebuha -1997), ko je preboleval holecistitis
6 Posthepatitična HCV pozitivna jetrna ciroza Sprejem KOH Prizadet, bled, tremorozen, TT 38 C Glava bp, tilnik mehak, dihanje pospešeno Srčna akcija redna, tahikardna 99/min, brez šumov Trebuh mehak, neobčutljiv Levo koleno oteklo, boleče, gibljivost omejena Grand mal epileptični napada med čakanjem na izvide
7 Posthepatitična HCV pozitivna jetrna ciroza TH na KOH Redno nadomeščanje koncentrata FVIII Širokospektralni antibiotiki DD: vnetje kolena, vnetje žolčnika, meningitis Punkcija kolena, CT glave ARI, sepsa in premestitev v KOIM
8 KOIM SEPSA, ARDS, intubacija, um.ventilacija CRP 220; L 8.5, Hb 109, Tr 165 Bilirubin-cel 59, dir 39; AST 1.9; ALT 0.5; LDH 7.6; gama GT 4.7 Amilaza 13, lipaza 34 PČ 0.5, INR 1.66, PTČ 30, fibrinogen 7.3 CT glave: stare spremembe, ni sveže krvavitve UZ trebuha: difuzna parenhimska okvara jeter, zadebeljena in razslojena stena žolčnika, ni sprememb pankreasa Punkcija L kolena- EColi
9 Posthepatitična HCV pozitivna jetrna ciroza KOH ABD KRG Holecistektomija + biopsija jeter 3 dan po op KOH Melena+stresne razjede na sluznici želodca Dekompenzirana jetrna ciroza z ascitesom Ponovno febrilno stanje (Ent. fecium) Nadomestno je prejemal- FVIII, SZP, traneksamično kislino
10 Jetrna bolezen in motnja v koagulaciji Nagnjenost h krvavitvam nastajanje koagulacijskih F Trombocitopenija+spremenjena f trombocitov Spremembe fibrinogena Hiperfibrinoliza Nagnjenost h trombozam sinteza proteina C, S raven AT plazminogen FVIII in vwf (iz endotelnih celic)
11 Jetrna bolezen in motnja v koagulaciji možni ukrepi ni jasnih priporočil Sveža zmrznjena plazma, krioprecipitat Trombocitna plazma rfvii Antifibrinolitiki Koncentrat protrombinskega kompleksa (od vit K odvisne faktorje: II,IX,X, VII Dezmopresin
12 Primer 2
13 Hemofilija A in jatrogene spremembe v hemostazi V.A., 1957, F VIII <1%, 0 BE krvavitev/leto, sklepi, mišice (1994/95 38 krvavitev) 1972 d. ileopsoas pareza n. fem. dx d. podleht pareza n. uln. dx invalidska upokojitev (34 let!)- poliartropatija
14 Hemofilija A in jatrogene spremembe v hemostazi proktoragije, op. hemoroidov 2002 neuspešno zdravljenje HCV 2005 endoskopska holecistektomija (kamni) 2006 melena MW zg. rob hiatusne kile krioprecipitat, Hemofil M, Octanate Ped. klinika, SB Jesenice, ZD Škofja Loka, 1991samozdravljenje, Interna klinika pozna pravice, ne pa dolžnosti
15 Hemofilija A in jatrogene spremembe v hemostazi 4/2008 AMI sprednje stene, brez predhodnih stenokardij/ ob naporu urgentno koronarografija - trožilna koronarna bolezen: LAD 90-95%, LCX 60%, RCA 90% zožitev dilatacija LAD, vstavljeni neprevlečeni opornici
16 Perkutana koronarna intervencija Balonček postavimo na mesto zožitve Z napihom balončka razširimo žilno svetlino in razpremo žilno opornico Žilna opornica učvrsti žilno razširitev
17 Hemofilija A in jatrogene spremembe v hemostazi koronarografija : inf. heparina+ klopidogrel+hemofil M ( VIII 0.9) po posegu aspirin+ klopidogrel+ inf. Hemofil M ( VIII 0.8) pred odpustom Hemofil M bolus ( VIII 0.6, 0.3)+ aspirin+ klopidogrel po odpustu koncentrat FVIII vsak drugi dan ( VIII 0.3)+ aspirin+ klopidogrel
18
19 Treatment of ischemic heart disease IHD Stable AP ASC Unstable AP / NSTEMI STEMI Antithrombotic treatment - UFH: bolus 70 U/kg, followed by 400 U/kg/d for 48 hours - Aspirin: loading dose 325 mg, followed by 80 mg - Clopidogrel: loading dose 600 mg, followed by 75 mg for 4 weeks - GPIIb/IIIa inhibitors: abciximab bolus 0.25 mg/kg followed by µg/kg/min for 12 hours Clotting factor correction - Peak level 0.8 U/l for 48 hours - Trough level 0.3 U/l as long as dual antiplatelet therapy is given < 12 hours Yes Primary PCI indicated Schutgens, et al. Haemophilia. 2009
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