{"id":10010,"date":"2020-04-23T03:53:52","date_gmt":"2020-04-23T01:53:52","guid":{"rendered":"https:\/\/www.synevo.ro\/product\/factorul-ix-al-coagularii\/"},"modified":"2025-02-04T16:30:38","modified_gmt":"2025-02-04T14:30:38","slug":"factorul-ix-al-coagularii","status":"publish","type":"product","link":"https:\/\/old.synevo.md\/ru\/shop\/factorul-ix-al-coagularii\/","title":{"rendered":"\u0424\u0430\u043a\u0442\u043e\u0440 \u0441\u0432\u0435\u0440\u0442\u044b\u0432\u0430\u043d\u0438\u044f \u043a\u0440\u043e\u0432\u0438 IX"},"content":{"rendered":"<p><span style=\"font-size: medium;\"><strong><em>Informatii generale si recomandari privind efectuarea testului<\/em><\/strong><\/span><\/p>\n<p><span style=\"font-size: medium;\">Factorul IX (factorul antihemofilic B sau factorul Christmas) este o glicoproteina sintetizata in hepatocit in prezenta vitaminei K si compusa din doua fractiuni: FIX-C si FIX \u2013C Ag<sup>1;3;7<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\">FIX-C este responsabil de activitatea coagulanta si se sintetizeaza sub inductia genelor de pe cromozomii X. FIX-C Ag constituie elementul de suport proteic inactiv al fractiunii coagulante si detine proprietati antigenice<sup>8<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\">Din punct de vedere functional reprezinta o serin-proteaza in complexul intrinsec al tenazei, interactionand cu cofactorul sau FVIIIa in prezenta ionilor de calciu. Conform modelului actual al coagularii, acest complex intervine in etapa a doua (de propagare), fiind activatorul major al factorului X (de 50 ori mai activ decat complexul factor VIIa-factor tisular). Astfel, peste 90% din cantitatea totala de FXa este produsa de catre complexul tenazei. La randul sau, factorul IX este activat de catre complexul \u00a0VIIa-factor tisular in prima etapa a coagularii (de initiere). In etapa de propagare se genereaza o cantitate suplimentara de FIXa de catre FXIa, pentru a amplifica in continuare producerea de FXa si trombina<sup>2<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\">Deficitul de FIX-C, denumit hemofilie B sau boala Christmas, este o afectiune cu transmitere genetica recesiva X-linkata (genele pentru FIX sunt situate pe cromozomul X)<sup>8<\/sup>. Sunt descrise numeroase mutatii, cele mai multe fiind punctiforme<sup>9<\/sup>. Majoritatea cazurilor de boala se dezvolta la baietii ale caror mame sunt purtatoare ale defectului genetic (heterozigote). Un subtip de hemofilie B \u2013 hemofilia B Leiden \u2013 se caracterizeaza printr-o expresie alterata a factorului IX, astfel incat in perioada copilariei nivelurile pot fi &lt;1% si dupa pubertate pot creste treptat pana la 70%. Sunt descrise si cazuri de hemofilie B rezultate in urma unor mutatii spontane ale genei pentru FIX. Femeile purtatoare au un nivel de factor IX mai redus decat persoanele normale, insa suficient pentru a asigura hemostaza si de aceea sangerarile excesive sunt rar intalnite (numai dupa traumatisme si interventii chirurgicale majore). In schimb femeile cu sindrom Turner (cariotip X0) pot fi simptomatice, la fel ca si persoanele de sex feminin purtatoare care prezinta un grad crescut de lionizare (inactivarea unui cromozom X)<sup>6<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\">Tabloul clinic include manifestari hemoragice care debuteaza cel mai adesea la sfarsitul primului an de viata:<\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\">\u2013 sangerari externe, variate ca localizare dar reduse ca incidenta si gravitate, care apar dupa o perioada de latenta de cateva ore de la traumatismul cauzal;<\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\">\u2013 sangerari interne care sunt mult mai frecvente si cu indice de gravitate mult mai mare (hematoame profunde, intra si interviscerale, intracavitare: abdomen, pleura, pericard, articulatii, spatiu subarahnoidian).<\/span><\/p>\n<p><span style=\"font-size: medium;\">Caracteristice bolii si cu incidenta crescuta sunt hemartrozele si hematoamele<sup>7<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\">In functie de severitatea sindromului hemoragipar sunt descrise 3 forme clinice de hemofilie B:<\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\"><em>\u25cf\u00a0 severa<\/em>: hemoragiile apar spontan (nivel FIX &lt;1%)<\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\"><em>\u25cf\u00a0 medie<\/em>: hemoragiile apar dupa traumatisme minore (nivel de factor IX: 1-5%)<\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\"><em>\u25cf\u00a0 usoara<\/em>: hemoragiile apar dupa traumatisme mari si interventii chirurgicale (nivel de factor IX: 5-25%).<\/span><\/p>\n<p><span style=\"font-size: medium;\">Deficitul dobandit de factor IX apare in conditiile asociate cu deficit de vitamina K. Pacientii cu afectiuni hepatice grave pot prezenta o scadere generalizata a factorilor coagularii, inclusiv factorul IX.<\/span><\/p>\n<p><span style=\"font-size: medium;\">Pacientii cu hemofilie B care primesc tratament substitutiv pot dezvolta inhibitori ai factorului IX in 3% din cazuri, ca urmare a producerii de aloanticorpi. Exista si cazuri rare de hemofilie B dobandita prin aparitia de autoanticorpi anti-FIX in cadrul unor afectiuni autoimune. Acesti pacienti prezinta manifestari clinice similare celor din forma congenitala<sup>6<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\">In cazul deficitului de factor IX testele de hemostaza prezinta urmatoarele modificari: timpul de tromboplastina partiala activat (APTT) este mult alungit si se corecteaza dupa administrarea de plasma normala, in timp ce timpul Quick, timpul de trombina si timpul de singerare sunt normale. Determinarea specifica a activitatii factorului IX \u2013 % din normal \u2013 stabileste diagnosticul de hemofilie B si forma clinica (severa\/medie\/usoara)<sup> 1;4<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Pregatire pacient<\/em><\/strong> <em>- <\/em>\u00e0 jeun (pe nemancate)<sup>5<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Specimen recoltat<\/em><\/strong> <em>- <\/em>sange venos<sup>5<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Recipient de recoltare<\/em><\/strong> \u2013 vacutainer cu citrat de Na 0.105M (raport citrat de sodiu \u2013 sange=1\/9). Presiunea realizata de garou trebuie sa fie intre valoarea presiunii sistolice si cea a presiunii diastolice si nu trebuie sa depaseasca 1 minut. Daca punctia venoasa a esuat, o noua tentativa pe aceeasi vena nu se poate face decat dupa 10 minute<sup>5<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Cantitate recoltata<\/em><\/strong> \u2013 cat permite vacuumul; pentru a preveni coagularea partiala a probei se va asigura amestecul corect al sangelui cu anticoagulantul, prin miscari de inversiune a tubului<sup>5<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Prelucrare necesara dupa recoltare <\/em><\/strong>\u2013 proba va fi centrifugata 15 minute la 2500g, urmata imediat de separarea plasmei si congelarea acesteia; probele recoltate in afara laboratorului vor fi transportate in recipientul destinat probelor congelate<sup>5<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Cauze de respingere a probei<\/em><\/strong> <em>- <\/em>vacutainer care nu este plin; proba de singe hemolizata sau coagulata; plasma care nu a ajuns congelata la laborator<sup>5<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Stabilitate proba<\/em><\/strong> \u2013 plasma separata este stabila <em>4 ore<\/em> la temperatura camerei; <em>3 saptamani<\/em> la\u00a0 \u2013 20\u00baC; <em>&gt; 1 an<\/em> la \u2013 70\u00baC<sup>5<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Metoda <\/em><\/strong>- <span style=\"text-decoration: underline;\">coagulometrica<\/span> (consta in masurarea timpului de coagulare a probei de investigat in prezenta cefalinei + activator si a unei plasme care contine in exces toti factorii plasmatici ai coagularii cu exceptia factorului IX<sup>5<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Valori de referinta <\/em><\/strong><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\">&lt; 7 zile: 35-60%<\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\">8 zile \u2013 1 an: 45-110%<\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\">&gt; 1 \u2013 10 ani: 60-100%<\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\">&gt; 10 ani: 70-120%<sup>5<\/sup>.<\/span><\/p>\n<p><span style=\"font-size: medium;\"><strong><em>Limite si intreferente <\/em><\/strong><\/span><\/p>\n<p><span style=\"font-size: medium;\">Determinarea activitatii factorului IX nu trebuie utilizata pentru depistarea femeilor purtatoare ale tarei hemofilice \u2013 pentru aceste cazuri sunt indicate teste genetice.<\/span><\/p>\n<p><span style=\"font-size: medium;\">In prezenta inhibitorilor se pot obtine niveluri fals scazute ale factorului IX<sup>6<\/sup><\/span>.<\/p>\n<p><span style=\"font-size: small;\">\u00a0<\/span><\/p>\n<p><span style=\"font-size: small;\">\u0411\u0438\u0431\u043b\u0438\u043e\u0433\u0440\u0430\u0444\u0438\u044f<\/span><\/p>\n<p><span style=\"font-size: small;\">1. Daniela Badea. Veronica Sfredel. Homeostazia sangelui. Editura Medicala Universitara Craiova Ed. 2003, 217-233.<\/span><\/p>\n<p><span style=\"font-size: small;\">2. Denise O\u2019 Shaughnessy, Michael Makris, David Lilicrap. Basic principles underlying coagulation system. In Practical Hemostasis and Thrombosis, Balckwell Publishing, 2008, 4-6.<\/span><\/p>\n<p><span style=\"font-size: small;\">3. Florica Enache. Maria Stuparu. Diagnosticul de laborator in hemostaza. Editura ALL, 1998, pag.155-167.<\/span><\/p>\n<p><span style=\"font-size: small;\">4. Jacques Wallach. Interpretarea testelor de diagnostic. Editura Stiintelor Medicale, Romania, 7 ed., 2001, 639-641.<\/span><\/p>\n<p><span style=\"font-size: small;\">5. Laborator Synevo. Referintele specifice tehnologiei de lucru utilizate 2010. Ref Type: Catalog<\/span><\/p>\n<p><span style=\"font-size: small;\">6. Laboratory Corporation of America. Directory of Services and Interpretive Guide. Factor IX Activity.www.labcorp.com 2010. Ref Type: Internet Communication.<\/span><\/p>\n<p><span style=\"font-size: small;\">7. Radu Paun. Tratat de medicina interna . Hematologie. Partea I. Ed.Medicala 1997, 353-404.<\/span><\/p>\n<p><span style=\"font-size: small;\">8. Radu Paun. Tratat de medicina interna . Hematologie. Partea II. Ed. Medicala 1999, 779-854.<\/span><\/p>\n<p><span style=\"font-size: small;\">9. Richard A. McPetersen, Matthew R. Pincus. Coagulation and Fibrinolysis. In Clinical diagnosis and management by laboratory methods. 2007, 729-743.<\/span><\/p>","protected":false},"excerpt":{"rendered":"<p>\u0418\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u0435 \u043f\u0440\u0438\u043c\u0435\u043d\u044f\u044e\u0442 \u0434\u043b\u044f \u0434\u0438\u0430\u0433\u043d\u043e\u0441\u0442\u0438\u043a\u0438 \u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u0438 \u0442\u0438\u043f\u0430 B (\u0431\u043e\u043b\u0435\u0437\u043d\u0438 \u041a\u0440\u0438\u0441\u0442\u043c\u0430\u0441\u0430), \u043f\u0440\u0438\u043e\u0431\u0440\u0435\u0442\u0435\u043d\u043d\u044b\u0445 \u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u0447\u0435\u0441\u043a\u0438\u0445 \u0440\u0430\u0441\u0441\u0442\u0440\u043e\u0439\u0441\u0442\u0432, \u0432 \u043e\u0431\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u044f\u0445 \u043f\u0430\u0446\u0438\u0435\u043d\u0442\u043e\u0432 \u0441 \u0443\u0434\u043b\u0438\u043d\u0435\u043d\u043d\u044b\u043c \u0410\u0427\u0422\u0412.<\/p>\n<p>\u0424\u0430\u043a\u0442\u043e\u0440 \u0441\u0432\u0435\u0440\u0442\u044b\u0432\u0430\u043d\u0438\u044f IX (FIX), \u043d\u0430\u0437\u044b\u0432\u0430\u0435\u043c\u044b\u0439 \u0442\u0430\u043a\u0436\u0435 \u0430\u043d\u0442\u0438\u0433\u0435\u043c\u043e\u0444\u0438\u043b\u044c\u043d\u044b\u043c \u0433\u043b\u043e\u0431\u0443\u043b\u0438\u043d\u043e\u043c B \u0438\u043b\u0438 \u0444\u0430\u043a\u0442\u043e\u0440\u043e\u043c \u041a\u0440\u0438\u0441\u0442\u043c\u0430\u0441\u0430, \u043e\u0442\u043d\u043e\u0441\u0438\u0442\u0441\u044f \u043a \u0432\u0438\u0442\u0430\u043c\u0438\u043d \u041a-\u0437\u0430\u0432\u0438\u0441\u0438\u043c\u044b\u043c \u0441\u0435\u0440\u0438\u043d\u043e\u0432\u044b\u043c \u043f\u0440\u043e\u0442\u0435\u0430\u0437\u0430\u043c. \u0423\u0447\u0430\u0441\u0442\u0432\u0443\u0435\u0442 \u0432 \u043a\u0430\u0441\u043a\u0430\u0434\u0435 \u0441\u0432\u0435\u0440\u0442\u044b\u0432\u0430\u043d\u0438\u044f \u043f\u043e \u0432\u043d\u0443\u0442\u0440\u0435\u043d\u043d\u0435\u043c\u0443 \u043f\u0443\u0442\u0438, \u0430\u043a\u0442\u0438\u0432\u0438\u0440\u0443\u044f \u0444\u0430\u043a\u0442\u043e\u0440 \u0425 (\u043f\u0435\u0440\u0435\u0432\u043e\u0434\u0438\u0442 \u043d\u0435\u0430\u043a\u0442\u0438\u0432\u043d\u0443\u044e \u0444\u043e\u0440\u043c\u0443 \u0444\u0430\u043a\u0442\u043e\u0440\u0430 \u0425 \u0432 \u0425\u0430), \u0438 \u0442\u0435\u043c \u0441\u0430\u043c\u044b\u043c \u043f\u0440\u0438\u043d\u0438\u043c\u0430\u044f \u0430\u043a\u0442\u0438\u0432\u043d\u043e\u0435 \u0443\u0447\u0430\u0441\u0442\u0438\u0435 \u0432 \u0444\u043e\u0440\u043c\u0438\u0440\u043e\u0432\u0430\u043d\u0438\u0438 \u0442\u0435\u043d\u0430\u0437\u043d\u043e\u0433\u043e \u043a\u043e\u043c\u043f\u043b\u0435\u043a\u0441\u0430. FIX \u0441\u0438\u043d\u0442\u0435\u0437\u0438\u0440\u0443\u0435\u0442\u0441\u044f \u0432 \u043f\u0435\u0447\u0435\u043d\u0438, \u0432\u0440\u0435\u043c\u044f \u043f\u043e\u043b\u0443\u0436\u0438\u0437\u043d\u0438 \u0432 \u043f\u043b\u0430\u0437\u043c\u0435 \u043a\u0440\u043e\u0432\u0438 \u0441\u043e\u0441\u0442\u0430\u0432\u043b\u044f\u0435\u0442 18-24 \u0447\u0430\u0441\u0430. \u041d\u0435 \u043e\u0442\u043d\u043e\u0441\u0438\u0442\u0441\u044f \u043a \u0440\u0435\u0430\u043a\u0442\u0430\u043d\u0442\u0430\u043c \u043e\u0441\u0442\u0440\u043e\u0439 \u0444\u0430\u0437\u044b.<br \/>\n\u0412\u0440\u043e\u0436\u0434\u0435\u043d\u043d\u044b\u0439 \u0434\u0435\u0444\u0438\u0446\u0438\u0442 FIX \u044f\u0432\u043b\u044f\u0435\u0442\u0441\u044f \u043f\u0440\u0438\u0447\u0438\u043d\u043e\u0439 \u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u0438 \u0412 (\u0431\u043e\u043b\u0435\u0437\u043d\u044c \u041a\u0440\u0438\u0441\u0442\u043c\u0430\u0441\u0430).<\/p>\n<p>\u0413\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u044f \u2013 \u0433\u0435\u043d\u0435\u0442\u0438\u0447\u0435\u0441\u043a\u0438 \u043e\u0431\u0443\u0441\u043b\u043e\u0432\u043b\u0435\u043d\u043d\u043e\u0435 \u0433\u0435\u043c\u043e\u0440\u0440\u0430\u0433\u0438\u0447\u0435\u0441\u043a\u043e\u0435 \u0437\u0430\u0431\u043e\u043b\u0435\u0432\u0430\u043d\u0438\u0435, \u0432\u043e\u0437\u043d\u0438\u043a\u0430\u044e\u0449\u0435\u0435 \u0432\u0441\u043b\u0435\u0434\u0441\u0442\u0432\u0438\u0435 \u0434\u0435\u0444\u0438\u0446\u0438\u0442\u0430 \u0444\u0430\u043a\u0442\u043e\u0440\u0430 \u0441\u0432\u0435\u0440\u0442\u044b\u0432\u0430\u043d\u0438\u044f VIII (\u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u044f \u0410) \u0438\u043b\u0438\/\u0438 IX (\u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u044f \u0412) \u043f\u0440\u0438 \u043d\u043e\u0440\u043c\u0430\u043b\u044c\u043d\u043e\u0439 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\u0441\u0442\u043e\u043c\u0430\u0442\u043e\u043b\u043e\u0433\u0438\u0447\u0435\u0441\u043a\u0438\u043c\u0438 \u043e\u043f\u0435\u0440\u0430\u0446\u0438\u044f\u043c\u0438 (\u0443\u0434\u0430\u043b\u0435\u043d\u0438\u0435 \u0437\u0443\u0431\u0430), \u043e\u0431\u043d\u0430\u0440\u0443\u0436\u0438\u0432\u0430\u0435\u0442\u0441\u044f \u043e\u0431\u044b\u0447\u043d\u043e \u0432 \u0440\u0430\u043d\u043d\u0435\u043c \u0432\u043e\u0437\u0440\u0430\u0441\u0442\u0435. \u0421\u043e\u043e\u0442\u043d\u043e\u0448\u0435\u043d\u0438\u0435 \u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u0438 \u0410 \u0438 \u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u0438 \u0412 \u0441\u043e\u0441\u0442\u0430\u0432\u043b\u044f\u0435\u0442 \u0432 \u0441\u0440\u0435\u0434\u043d\u0435\u043c 4:1 (\u0441\u043c. \u0442\u0430\u043a\u0436\u0435 \u0442\u0435\u0441\u0442 \u2116 1409 \u0424\u0430\u043a\u0442\u043e\u0440 VIII, \u0430\u043a\u0442\u0438\u0432\u043d\u043e\u0441\u0442\u044c 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X).<\/li>\n<\/ol>","protected":false},"featured_media":0,"comment_status":"closed","ping_status":"closed","template":"","meta":[],"product_brand":[],"product_cat":[951],"product_tag":[],"class_list":{"0":"post-10010","1":"product","2":"type-product","3":"status-publish","5":"product_cat-teste-de-hemostaza","7":"first","8":"instock","9":"sold-individually","10":"shipping-taxable","11":"purchasable","12":"product-type-simple"},"_links":{"self":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/product\/10010","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/product"}],"about":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/types\/product"}],"replies":[{"embeddable":true,"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/comments?post=10010"}],"wp:attachment":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/media?parent=10010"}],"wp:term":[{"taxonomy":"product_brand","embeddable":true,"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/product_brand?post=10010"},{"taxonomy":"product_cat","embeddable":true,"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/product_cat?post=10010"},{"taxonomy":"product_tag","embeddable":true,"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/product_tag?post=10010"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}