*** Topic "MICROANGIOPATIA TROMBOTICA, SINDROME UREMICO HEMOLITICO Y
PURPURA TROMBOTICA TROMBOCITOPENICA"
[22:25] *** BEPPE (BEPPE@213.217.185.205) has joined #cin
[22:25] (SCigarran> Wellcome Dr Remuzzi
[22:26] (nickMalvi> Welcome, Dr. Remuzzi from Canada
[22:26] (BEPPE> Good evening
[22:26] (SCigarran> good evening dr remuzzi
[22:26] (SCigarran> How do you do?
[22:28] (BEPPE> I'm fine why don't we start?
[22:32] (SCigarran> Well, MJ are you ready?
[22:32] (Lulusita> Buenas noches: Prof Foyaca y Dra Ibanez en linea
[22:32] (MJesus> buenas noches
[22:33] (SCigarran> Buenas noches Dra Ibañez y Dr Foyaca
[22:33] (Lulusita> Estamos listo!
[22:38] (SCigarran> Today, as , we have with us, Dr Remuzzi, very well known in the nephrology field
[22:38] (Speaker> MICROANGIOPATIA TROMBOTICA, SINDROME UREMICO HEMOLITICO Y PURPURA TROMBOTICA TROMBOCITOPENICA
[22:39] (Speaker> Piero Ruggenenti M.D.*°, Carlos Chiurchiu M.D.°, Giuseppe Remuzzi M.D.*°
[22:39] (Speaker> * Division of Nephrology and Dialysis, Ospedali Riuniti di Bergamo
[22:39] (Speaker> ° "Mario Negri" Institute for
Pharmacological Research, Negri Bergamo Laboratories and Clinical Research Center for Rare diseases "Aldo e Cele Daccò"
[22:39] (Speaker> Bergamo - Italy
[22:40] (Speaker> El término microangiopatía trombótica (MAT) se refiere a una lesión estructural de la pared vascular (principalmente arteriolas y capilares), con engrosamiento de la misma, trombosis plaquetaria intraluminal y obstrucción parcial o completa de la luz vascular. La presencia de trombocitopenia y anemia hemolítica son caracteristicas constantes de MAT y reflejan el consumo y la disrupción de plaquetas y hematíes en la
microvasculatura.
[22:40] (Speaker> La presencia de otros signos clínicos depende de la distribución de las lesiones microvasculares y la consecuente disfunción organica. Han sido descriptas, dependiendo de la prevalencia de lesiones renales o cerebrales, dos entidades patologicamente indistinguibles pero clínicamente diferentes: el Síndrome Urémico Hemolítico (SUH) y la Púrpura Trombocitopénica Trombótica (PTT). La injuria a las celulas endoteliales es el factor principal y probablemente inicial en la secuencia de eventos que llevan a MAT.
[22:41] (Speaker> La toxina-shiga y otras endotoxinas bacterianas, como así también anticuerpos, inmunocomplejos, ciertas drogas y virus (en particular HIV), son tóxicos endoteliales in vitro y pueden
causar SUH/PTT in vivo.
[22:41] (Speaker> La pérdida de la tromboresistencia fisiológica, la adhesión leucocitaria al endotelio dañado, el consumo de complememto, la anormal liberación y fragmentación del factor de von Willebrand y el aumentado shear stress vascular pueden sostener e incluso amplificar el proceso microangiopático. Las anormalidades intrínsecas del sistema de complemento y del factor de von Willembrand dan lugar a una predisposición genética para desarrollar la enfermedad que pueden jugar un rol fundamental en formas recurrentes o familiares.
[22:42] (Speaker> En niños con SUH relacionado a shiga-toxina la evolución es generalmente buena, en tanto que en formas familiares o atípicas de SUH/PTT es frecuente el desarrollo de secuelas neurológicas y renales.
[22:42] (Speaker> El recambio o infusión de plasma constituyen los únicos tratamientos con eficacia comprobada. La nefrectomía bilateral y la esplenectomía pueden servir como terapia de rescate en casos muy seleccionados con resistencia a la terapéutica con plasma.
[22:43] (Speaker> 4 it's all !
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[22:43] (MJ-usa> clap clap clap clap clap clap clap clap
[22:43] (MJ-usa> clap clap clap clap clap clap clap clap
[22:43] (MJ-usa> clap clap clap clap clap clap clap clap
[22:43] (MJ-usa> clap clap clap clap clap clap clap clap
[22:43] (SCigarran> plas plas plas plas plas plas plas plas plas plas plas plas plas plas plas plas plas
[22:43] (MJ-usa> clap clap clap clap clap clap clap clap clap
[22:43] (MJ-usa> clap clap clap clap clap clap clap clap clap
[22:43] (MJ-usa> clap clap clap clap clap clap clap clap clap
[22:43] (SCigarran> plas plas plas plas plas plas plas plas plas plas plas plas plas plas
[22:44] (MJesus> Dr, Remuzzi, thank for writing in Spanish !
[22:44] (MJesus> molte gracie
[22:45] (SCigarran> Dr Remuzzi thanks a lot!!
[22:45] (MJesus> it's also great to find you here !
[22:45] (pino> Dr. Remuzzi, le importa que le hagamos preguntas en español?
[22:45] (pino> o prefiere en inglés?
[22:46] (BEPPE> espanol es bueno
[22:46] (MJesus> do you understand Spanish ??
[22:46] (MJesus> you can also answer in italian!
[22:46] (BEPPE> Si lo entiendo muy bien
[22:46] (MJesus> questions ?
[22:46] (SCigarran> Dr Remuzzi hay diferencia entre el SHU enlos niños proveniente del Shiga toxins y las otras formas?
[22:47] (pino> Aconseja iniciar el tratamiento siempre con plasma fresco?
[22:47] (BEPPE> Quali altre forme?
[22:47] (SCigarran> Shiga vs nonshiga
[22:47] (SCigarran> associated
[22:48] (BEPPE> Il plasma va riservato alle forme
[22:48] (BEPPE> gravi in pazienti anurici e sopratutto negli adulti
[22:49] (BEPPE> però non ci sono studi controllati
[22:49] (BEPPE> le forme nonshiga dei bambini possono essere sporadiche o genetiche
[22:50] (BEPPE> di quale volete che parliamo?
[22:50] (SCigarran> della endotoxins
[22:53] (BEPPE> quali endotoxine?
[22:55] (otro-apod> Las drogas que inducen SHU actuan de la misma manera que las endotoxinas?
[22:55] (BEPPE> di preciso non si sa
[22:56] (BEPPE> credo che sia i farmaci che le tossine inducano un danno endoteliale
[22:56] (BEPPE> in questo senso si possono considerare simili
[22:58] (MJesus> ¿hay alguna medida eficaz para prevenir la aparicion del sindrome hemolitico uremico ?
[22:59] (pino> ¿debemos iniciar el tratamiento con plasma fresco lo antes posible, aún si tener un diagnóstico de certeza?
[23:00] (BEPPE> per la forma da E.coli shigatoxin la prevenzione più efficace è quella di
[23:00] (BEPPE> non consumare carne poco cotta
[23:00] (BEPPE> specie nei paesi a rischio
[23:01] (otamendi> Las alteraciones en el clivaje del FcVW:¿ son causa, o secundarias a la agresión inicial? ¿Cual es su opinión?
[23:01] (MJesus> please, could we speak English ?
[23:01] (BEPPE> un modo di diffusione della tossina è attraverso gli utensili da cucina e le mani non lavate bene che possono venire
[23:02] (BEPPE> a contatto con verdure e altri cibi. Pastosizzazione della carne macinata
[23:02] (BEPPE> attraverso irradiazione previene previene la infezione da Stx.
[23:03] (BEPPE> L'infezione da coli 0157 non conferisce immunità e al momento non ci sono vaccini
[23:05] (pino> excuse me, at this moment, Dr Malvinder is with us, would you mind if we can speak in english?
[23:05] (BEPPE> New agents targeted to prevent organ exposition to Stx are currently under evaluation
[23:06] (Malvinder> Thank you.
[23:06] (SCigarran> Abaut shear stress What role plays?
[23:07] (BEPPE> the most promising are Synsorb-PK , a resin composed of repeated synthetic carbohydrate determinants linked to collodial silica that bind Stx.
[23:09] (BEPPE> von Willebrand factor's susceptibility to fragmentation incrases in response to rising levels of share stress which induces protein unfolding
[23:10] (BEPPE> and makes vWF proteolytic cleavage site more accesible to specific plasma proteases.
[23:11] (SCigarran> How these patients respond to plasma exchange?
[23:11] (BEPPE> which patients?
[23:12] (SCigarran> with severe forms of SHU
[23:13] (SCigarran> where the kidneys are a major site of vascular bed occlussion?
[23:13] (BEPPE> before the introduction of plasma manipulation mortality for trombotic microangeopaties was 90%
[23:14] (SCigarran> and elevated shear stress
[23:14] (BEPPE> after plasma exchange infusion was introduced mortality was reduced to 20-30%
[23:15] (MJesus> Dr. Remuzzi, do you think that treatment with clopidrgel will become dangerous?
[23:15] (BEPPE> now we now that respons to plasma exchange-infusion depends on the nature of the underling deseas.
[23:18] (BEPPE> Cancer, Mitomicyn AIDS associated HUS virtually DO NOT respons to plasma, familiar and recurrent cases of HUS related to
[23:18] (SCigarran> and Ticlopidine?
[23:18] (Malvinder> Dr. Remuzzi, in TTP there is deficiency of V WF cleaving-cysteine protease where this level is not affected in HUS and that's the reason that TTP patients responsds better to plasma based therapy than HUS?
[23:19] (BEPPE> complement factor H mutations respond very well. Stx HUS of children normally do not require plasma .
[23:21] (BEPPE> It is not true, that all patients with HUS have normal vWS protesis
[23:22] (Malvinder> Antiplatelet therapies like ASA or ?newer agents (tiklid, Plavix) only shorten thrombocytopenia and not effective alone.
[23:22] (Lulusita> Dr. Remuzzi: The neurological complications are related to ischemic o haemorrhagic events?
[23:23] (BEPPE> we have just summitted to Blood a paper entitled von Willembrand factor cleaving preotease (adamts13) is deficient in thrombotic purpura and in the hemolytic uremic syndrome as well.
[23:24] (Malvinder> OK, Good to know.
[23:26] (MJesus> yes.... thanks por this advance
[23:26] (pino> any more questions?
[23:27] (Malvinder> What about the pathogenesis of seizures that occur in up to 20-30% of pateints?
[23:27] (BEPPE> The paper shows that most patients with TTP we studied had deficiency of adamts13 activity moreover a complete deficiency of Adamts13 activity was also found in 5 of 13 patients with D-HUS during the acute phase and in 5 patients with the D-HUS in during remission.
[23:28] (BEPPE> Neurological complications are due to microvascular thrombosis of brain vessels.
[23:28] (MJesus> oh! what a pity.... lulusita are dr. Foyaca and wife, fron South African travel in Cuba
[23:29] (osgarur> When plasma is infused, What are we infusing
to treat the process? Only the vWF cleaving proteasa? and What are we removing with plasma exchange?
[23:29] (Malvinder> Why antibiotic treatment of children with E coli infection, increases the risk of developing HUS?
[23:31] (BEPPE> thrombosis might be followed by focal Haemorhagies. These lesions also cause seizurres .
[23:31] (Malvinder> I mean, of course E. Coli OH157 in above question.
[23:32] (BEPPE> Brain edema may amplify the clinical picture .
[23:34] (pino> five last minutes last minutes, please, because we are on time
[23:34] (BEPPE> Several antimicrobial drugs , particularly the quinolones trimethoprim and furazolidone , are potent inducers of the expression of the Stx 2 gene amd may increase the level of toxin in the intestine.
[23:35] (pino> we announce that discussion pannels will be open till December 5th
[23:36] (BEPPE> Infusion definitly heòps in cases of factor H or vWF protease deficiency by restoring the deficient of abnormal factor
[23:36] (Malvinder> somehow got disconnected.
[23:37] (SCigarran> Yes, the line is busy
[23:37] (BEPPE> we remove volume first of all then fibrinogen degradation products cell debris and other toxins
[23:39] (MJesus> 4 thanks you very much !!!
[23:39] (pino> thank you very much to Dr.Remuzzi for accepting our invitation
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[23:40] (BEPPE> Thanks a lot to all of you
[23:40] (MJesus> your cibercurriculum will be great Dr. Remuzzi
[23:40] (Malvinder> Thank you, Dr. Remuzzi. learn from your papers alot.
[23:40] (BEPPE> it was a great pleasure
[23:40] (pino> we should know that today is our last conference closing
[23:40] (SCigarran> plas plas plas plas plas plas plas plas plas plas plas plas plas plas plas plas plas plas plas
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[23:40] (MJ-usa> clap clap clap clap clap clap clap clap clap
[23:40] (MJ-usa> clap clap clap clap clap clap clap clap clap
[23:40] (MJ-usa> clap clap clap clap clap clap clap clap
[23:40] (MJ-usa> clap clap clap clap clap clap clap clap
[23:40] (MJ-usa> clap clap clap clap clap clap clap clap
[23:40] (MJ-usa> clap clap clap clap clap clap clap clap
23:40] (MJ-usa> clap clap clap clap clap clap clap clap
[23:40] (BEPPE> I hope to have been of some help
[23:41] (SCigarran> trap traptraptraptraptraptraptraptraptraptra
ptraptraptraptraptraptraptraptraptraptraptraptraptraptrap
[23:41] (SCigarran> traptraptraptraptraptraptraptraptraptraptrap traptraptraptraptraptraptraptraptraptrap
[23:41] (SCigarran> traptraptraptraptraptraptraptraptraptraptrap
traptraptraptraptraptrap
[23:41] (pino> next days it will be published a fornefro discussion
[23:41] (SCigarran> fireworks sounds
[23:41] (pino> on this channel
[23:42] (pino> it will be a month discusion about several topics
[23:42] (ovand> clap clap clap clap
[23:42] (pino> for instance
[23:42] (ovand> thank you
[23:42] (Malvinder> Great work by the organizing committee to get nephrologist around the golbe.
[23:43] (pino> about sclerosing peritonitis, the next
[23:44] (pino> by the way, Dr Malvinder, we hope that next year you will be with us
[23:44] (SCigarran> I would like for next cin2002 all of you come back
[23:44] (Malvinder> Oh! yes, I would try my best effort.
[23:45] (SCigarran> Congratulations to all speakers and participants
[23:46] (SCigarran> Do you know that we get 790 entries around the world?
[23:46] (pedro> bye
[23:46] (Malvinder> I wish you all best of holiday season and Merry X-mas to you all.
[23:46] (SCigarran> bye
[23:46] (SCigarran> Same for you Dr malvinder. See you in Tampa
[23:47] (Malvinder> Will definitely try to meet you there.
[23:47] (MJesus> oh! you will know here
[23:47] (MJesus> pino you also travel tampa ?
[23:48] (pino> probably no, i do not will be there
[23:48] (otro-apod> Hi something wrong happened with conection
[23:48] (MJesus> scigarran :))
[23:49] (pino> thanks to everybody
[23:49] (Malvinder> Well done MJ, your support have been great.
[23:49] (SCigarran> Thanks Pino
[23:49] (MJesus> well I have dinner now
[23:49] (Malvinder> Good night to you all.
[23:49] (MJesus> I hope to see you in the next forum
[23:49] (pino> it is time to sing ... something
[23:49] (SCigarran> Good night and I see you next year!!!
[23:49] (SCigarran> Merry Christmas!!!!!!!!!!!!!!!!
[23:50] (pino> like meryy christmas..
[23:50] (Malvinder> Will definitely see you next year. Let me know the details.
[23:50] (pino> ok.
[23:50] (pino> it will be something similar like this
[23:50] (pino> adios a todos
[23:50] (SCigarran> much better!!!!
[23:51] (pino> it will be try
[23:51] (SCigarran> adios pino, gracias , muchas gracias
[23:51] (MJesus> and every month a meeting for discuss a topic
[23:51] (SCigarran> MJ not before February
[23:51] (SCigarran> we need some rest to visit Cuba
[23:52] (pino> here it is time to go to bed
[23:52] (SCigarran> and make the revolution
[23:53] (Malvinder> Cheers to CIN !!!!!!!!!!!!!!!!!!!!!!!
[23:53] (osgarur> Good night you all
[23:53] (Malvinder> I have to go now!
[23:53] (Malvinder> Thanks again. enjoyed it.
[23:53] *** Malvinder has quit IRC (Leaving)
[23:57] (SCigarran> Hasta pronto
[23:57] (SCigarran> Adios!!!!!!!!!
[23:57] *** SCigarran has quit IRC (Leaving)
