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Cas Clinque Commenté

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Cas Clinque Commenté

  1. 1. Cas Clinique Commenté<br />Congrès National de d’Hépato-Gastroentérologie3ème Journée commune SAHGE-AFEFHCA 9-10 décembre 2009N.DEBZICHU MUSTAPHA-ALGERnabildebzi@yahoo.fr<br />
  2. 2. 0bservation (1) <br />Mr D . B âgé de 41 ans - Suivi à l’hôpital de Tiaret depuis 2001 pour cavernome porte , il reçoit un TRT Bbloquant pour prophylaxie primaire , pas d’anti-coagulation , le bilan étiologique n’a pas été fait - Naissance à domicile .<br />
  3. 3. Observation (2) <br />1er hospitalisation dans le service 4/12/2004- Ictère cutanéo-muqueux franc associé à un prurit , précédé de douleurs de l’HCD , pas de trouble du transit , ni d’hémorragie -Il ne fume plus depuis 3 ans ( 22pqts/année)Il n’a jamais consommé de l’alcool-Pas d’ATCD de chirurgie (foie et voies biliaires) -A l’examen physique : EG conservé , TA 110/70 , pouls 55 , il n’est pas fébrile , BMI 18 , pas d’hépatomégalie , splénomégalie II , pas d’ascite <br />
  4. 4. Observation (3)<br />BiologiqueFNS : GB 7100 , Hb 13. 3 , Plaquettes 415.000TP 78% EFH : ALAT 98 , ASAT 82 , BT 63 ( BC 35) PAL (2N) , GGT 5N ,Alb 34 .<br />Morphologique Echodoppler : Foie ne présente pas d’anomalies , Dilatation de la VBP = 12 mm , pas d’obstacle en son sein , avec dilatation des VBIH , VB multilithiasique , Cavernome porte avec dilatations veineuses monstrueuses , SPMG 218 mm , la VS est thrombosée , la VMS et la VCI sont libres Bilan : Sérologie VHC (-), VHB (-) , Bilan martial (Nle) , bilan cuprique (-) , Bilan d’auto-immunité (-)<br />
  5. 5. Quel est l’étiologie la plus probable de cette cholestase<br />A : Lithiase Biliaire : LVBP<br />B : Cholangiopathie portale <br />C : Cholangite sclérosante <br />D : Cholangiocarcinome<br />
  6. 6. Quel est l’étiologie la plus probable de cette cholestase<br />A : Lithiase Biliaire : LVBP<br />B : Cholangiopathie portale <br />C : Cholangite sclérosante <br />D : Cholangiocarcinome<br />
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  9. 9. ThrombosePortaleChronique - Complications<br />% Pt-an<br />n = 136<br />20<br />12<br />6<br />2.5<br />2<br />0<br />Hémorragie<br />Biliaire<br />Thrombose<br />SMP <br />Condat. Gastroenterology 2001 & Hepatology 2003. ChaitBr J Haematol 2005<br />
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  11. 11. Mécanisme - Infection- Compression- Cholangiopathie ischémiqueSémiologie Rx 90% = Symptômes 25% <br />
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  13. 13. Quelle serait l’étiologie la plus probable de la thrombose porte ?<br />A : cause locale<br />B : Cause générale ( S. Myéloprolifératif)<br />C : A + B <br />
  14. 14. Quelle serait l’étiologie la plus probable de la thrombose porte ?<br />A : cause locale<br />B : cause générale ( S. Myéloprolifératif)<br />C : A + B<br />
  15. 15. Bilan étiologique<br />Arguments pour un SMP - Thrombocytose relative (415 Giga) , absence d’hypersplénisme malgré HTP - Protéine S , C , AT III , RPCA , homocystéînémie , APLsans anomaliesPBO : pas de SMP Mutation Jak 2 indisponible .<br />Arguments pour une cause locale - Naissance à domicile ( EHPVO)FOGD , Iléo coloscopie , TG , scanner sans anomalies <br />
  16. 16. Etiologie de la thrombose portaleCauses générales (60 %)<br />Syndrome myéloprolifératif35%<br />Déficit en protéine S, C et AT 3 à 20 %<br />Anticorpsantiphospholipides10 %<br />Mutation du facteurII 10 %<br />Facteur V Leiden 5 %<br />Denninger et al. Hepatology 2000;31:587-91.<br />
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  19. 19. Quelle est votre attitude vis-à-vis de sa cholangiopathie portale <br />A : Traitement endoscopique<br />B : Acide ursodésoxycholique<br />C : Shunt porto-systémique <br />D : TIPS <br />
  20. 20. Quelle est votre attitude vis-à-vis de sa cholangiopathie portale <br />A : Traitement endoscopique<br />B : Acide ursodésoxycholique<br />C : Shunt chirurgical porto-systémique <br />D : TIPS <br />
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  27. 27. Prise en Charge <br />Le patient est classé CHILD B 7 - TRT endoscopique indisponible - Chirurgie shunt porto-systémique : Spléno-rénal ( VS) Thrombosée , une dérivation bilio-digestive première(mauvaise indication ) ,les conditions opératoires n’ont permis qu’une cholecystostomie avec extraction de quelques calculs .<br />L’hématologiste a estimé qu’il ne fallait pas débuter un traitement par l’Hydrea®<br />Le patient est sortant : Ursolvan®, Avlocardyl®<br />
  28. 28. Quels sont les risques évolutifs chez notre patient ?<br />A : Rupture de VO<br />B : Accidents thrombotiques<br />C : Récidive de l’angiocholite<br />
  29. 29. Quels sont les risques évolutifs chez notre patient ?<br />A : Rupture de VO<br />B : Accidents thrombotiques<br />C : Récidive de l’angiocholite<br />
  30. 30. ThrombosePortaleChronique - Complications<br />% Pt-an<br />n = 136<br />20<br />12<br />6<br />2.5<br />2<br />0<br />Hémorragie<br />Thrombose<br />Biliaire<br />SMP <br />Condat. Gastroenterology 2001 & Hepatology 2003. ChaitBr J Haematol 2005<br />
  31. 31. Evolution <br />2005 : 1er épisode d’hémorragie digestive par RVO , il bénéficie de ligatures élastiques jusqu’à eradication<br />Il est perdu de vue , 2006 – 03/2009 , il ne prend plus l’ursolvan® ni les AVK .- Pas d’accidents thrombotiques - Ictère conjonctival – prurit intermittent- Pas de récidive hémorragique <br />
  32. 32. Hospitalisation 03/2009<br />A l’examen l’ EG est conservé , ictère conjonctival , rate type II . <br />BiologiqueNFS : GB 10200 , Hb 11.7 , Plaquettes 835.000TP 71% EFH : ALAT (64) ASAT (86) , BT 15 (BC 10) ,PAL 2N GGT 5N , Alb 33 <br />La PBO : Megacaryocytes dystrophiques « clusters » <br />Dg SMP : thrombocytémie essentielle , enfin le patient est mis enfin sous Hydréa® Mutation Jak 2 disponible ( 30.000 DA ) <br />
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  36. 36. Quelle est votre attitude vis-à-vis de sa cholangiopathie portale <br />A : Traitement endoscopique<br />B : Acide ursodésoxycholique<br />C : Shunt porto-systémique <br />D : TIPS <br />
  37. 37. Quelle est votre attitude vis-à-vis de sa cholangiopathie portale <br />A : Traitement endoscopique<br />B : Acide ursodésoxycholique<br />C : Shunt chirurgical porto-systémique <br />D : TIPS <br />

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