sindroame hemoragipare 2016

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Sindroame hemoragipare

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  • HEMOSTAZA NORMALAI. Etapa vascularaII. Etapa trombocitaraIII. Etapa coagulareIV. Etapa fibrinoliza

  • HEMOSTAZA NORMALAI. Endoteliul- functia 1. bariera de permeabilitate2. secretie:- F. Willebrand, - trombomodulin - ataseaza trombina, proteina C, S - fibronectina inhibitorul activatorului tisular al plasminogenului - PGI2 , Endotelina

  • - adeziunea prin receptori Gp I - colagenul subendotelial - fibronectin + f. Willebrand prin Gp IIb IIIa - fibrinogenII. Etapa trombocitara- agregarea - ADP subendotelial - Tx A2- secretia - Ca+2 , Factor 3, 4, serotonina

  • COAGULAREA - intrinseca - trombocite activatecolagenul subendotelial factorul XII - XIIXII a - IX IX a - X XaCa, F3plachetar- fosfolipide,VIII

  • COAGULAREA - extrinseca - tromboplastina tisularaFT - X XaProteina C- VII

  • - fibrinogen fibrina + fibrinopeptid A,BCOAGULAREA - calea comuna - X Xa - II IIaCa, F3plachetar,VProteina C

  • COAGULAREA

  • CLASIFICARE- Etapa vasculara - Purpura vasculara- congenitala - dobandita- Etapa trombocitara - Purpura trombocitara - trombocitopenie - trombopatii

  • - Etapa coagulare - Purpura deficienta factorilor de coagulare- congenitala - dobandita- Etapa reologica - Purpura ortostatica- Etapa fibrinolizei - Purpura fibrinolitica- Etapa activare sistem monocit-macrofag - Purpura CID

  • Purpure vasculare- congenitaleEhler Danlos osteogeneza imperfectaRendu - Osler- dobanditescorbutsenilaSindrom Henoch Schonleininfectioasa - meningococcica, viraledisglobulinemii - Waldenstrom, mielom, disglobulinemie mixta

  • Boala Rendu Osler- congenitalaautosomal dominantadefect perete venos(endoteliu, membrana bazala, adventice) fistule arteriovenoase(mucoasa digestiva, plaman, ficat, splina, cerebral)

  • Telangiectazia ereditara

  • Telangiectazia ereditara

  • Manifestari clinice1. telangiectazii cutanate, mucoase 2. epistaxis, hemoptizii, melena 3. anemie feripriva cr4. anevrisme arteriovenoase hepatice cu sindrom hiperkinetic cardiovascular 5. AHC sugestive boala Rendu

  • Diagnostic diferential1. Telangiectaziile sclerodermie (CREST)2. Pete telangiectatice3. B Fabry (Angiokeratoma corporis difusum) - boala a metabolismului glicolipidic (ceramide intramacrofagic) - leziuni nodulare inchise la culoare ( aproape negre) periombilical, scrotal, cardiac, sistem nervos

  • CREST

  • Boala Fabry

  • Pete telangiectatice

  • Tratament1. Suportiv: tamponament nazal, unguente ( dermatol), cauterizari contraindicate2. Trat anemiei : fier, ac folic, vit B12 la 3 luni

    3. Trat chirurgical al fistulelor mari greu de abordat

  • 2. Purpure vasculare anomalii dobandite ale tesutului conjunctiv

  • Purpura de stazaPurpura senila

  • Scorbutul Situatii etiologice- alcoolism, copii hraniti exclusiv cu lapte de vaca - patogenie: - deficit vit C deficit de colagen (hidroxiprolina) - reduce adeziunea, agregarea plachetelor, - clinic : - purura in jurul foliculului pilos, durere - hipercheratoza- rar: HDS, hemoragii musculare - tratament: Vit C iv

    * Patients with scurvy (vitamin C deficiency) develop painful episodes of perifollicular skin bleeding as well as bleeding into muscles and, occasionally, into the gastrointestinal and genitourinary tracts. The diagnosis is confirmed by the presence of hyperkeratosis of skin, gum swelling, and low levels of the vitamin in leukocytes. Vitamin C is needed to synthesize hydroxyproline, an essential constituent of collagen. Thus, collagen synthesis is impaired by scurvy. Patients with Cushing's syndrome, an excess production of glucocorticoids, or patients on large doses of glucocorticoids develop generalized protein wasting and may show skin bleeding or easy bruising due to atrophy of the supporting connective tissue around blood vessels. Aging causes a similar atrophy of perivascular connective tissue on the extensor surfaces of the hands and arms, leading to senile purpuradark purple, irregularly shaped hemorrhagic areas due to abnormal skin mobility that tears small blood vessels.

  • 3. Purpure vasculare dobandite - boli infectioase - boli de tesut conjunctiv - hiper/disglobulinemii - Henoch Schonlein -cauze rare

  • Purpure vasculare dobandite boli inflamatorii- etiopatogenie- complexe imune circulante - post infectii bacteriene, virale, rickettsi- medicamente penicilina, sulfamide, allopurinol

  • Purpura infectioasa

    - infectii (virusuri, meningococ, rickettsi) - patogenie: complexe imune circulante - clinic : - purura bule hemoragice purpura fulminans - tratament antiinfectios

    *

  • Sindromul hemolitic uremic- microtromboze exclusiv in vase renale- etiologie: infectioasa - patogenie: - copii, adolescenti- dupa infectii (E Coli, Shigella) - leziune de celule endoteliale, microtromboze a. aferenta, glomerul

    * HEMOLYTIC-UREMIC SYNDROME

    HUS is a disease of infancy and early childhood that closely resembles TTP. Patients present with fever, thrombocytopenia, microangiopathic hemolytic anemia, hypertension, and varying degrees of acute renal failure. In many cases, onset is preceded by a minor febrile or viral illness, and an infectious or immune complex-mediated cause has been proposed. Epidemics related to infection with a specific strain of Escherichia coli (O157:H7) have been documented. As in TTP, disseminated intravascular coagulation is not found. In contrast to TTP, the disorder remains localized to the kidney, where hyaline thrombi are seen in the afferent arterioles and glomerular capillaries. Such thrombi are not present in other vessels, and neurologic symptoms, other than those associated with uremia, are uncommon. No therapy is proven effective; however, with dialysis for acute renal failure, the initial mortality is only 5%. Between 10 and 50% of patients have some chronic renal impairment.

  • Sindromul hemolitic uremic- microtromboze exclusiv in vase renale- etiologie: infectioasa - clinic: - purpura - insuf renala acuta - HTA - AH microangiopatica- evolutie : - deces prin IRA - 10- 50% cazuri IRC - tratament - dializa in IRA

    * HEMOLYTIC-UREMIC SYNDROME

    HUS is a disease of infancy and early childhood that closely resembles TTP. Patients present with fever, thrombocytopenia, microangiopathic hemolytic anemia, hypertension, and varying degrees of acute renal failure. In many cases, onset is preceded by a minor febrile or viral illness, and an infectious or immune complex-mediated cause has been proposed. Epidemics related to infection with a specific strain of Escherichia coli (O157:H7) have been documented. As in TTP, disseminated intravascular coagulation is not found. In contrast to TTP, the disorder remains localized to the kidney, where hyaline thrombi are seen in the afferent arterioles and glomerular capillaries. Such thrombi are not present in other vessels, and neurologic symptoms, other than those associated with uremia, are uncommon. No therapy is proven effective; however, with dialysis for acute renal failure, the initial mortality is only 5%. Between 10 and 50% of patients have some chronic renal impairment.

  • Meningococcemie

  • Purpura fulminans post zona zoster

  • Endocardita bacteriana

  • Vasculita poliartrita reumatoida

  • Manifestari clinice1. purpura2. hemoragii retiniene3. nevrita periferica Tratament 1. Tratatamentul mielom, etc2. PlasmaferezaPurpura hiperglobulinemica IgA+IgG - boala Waldenstrom, mielom

  • Purpura hiperglobulinemica

  • Hemoragii subunghiale - crioglobulinemie

  • Purpura Henoch Schonlein- etiopatogenieinfectie streptococica (faringiana 50%cazuri, respiratorie)-alergii alimentare, medicamentoase (chinidina, sulfamide) membrana bazala capilara - complexe imune IgA2

  • Purpura Henoch SchonleinManifestari clinice

  • Manifestari clinice1. purpura2. poliartralgii3. colica abdominala, melena, rar perforatia