thrombophilia

raktham kattapidikkanulla saadhyatha vardhippikkunna rakthakkuzhalukalude asaadhaaranaavasthayaanu thrombophilia (chilappol hypercogulability allengil prothrombotic stettu ennu vilikkunnu). mattu kaaranangalal prakopippikkappedaathu thrombosis (kaalil deep weyin thrombosis polullava) ulla 50 sathamaanam aalukalilum atharam asaadhaaranathakal thirichariyaan kazhiyum. janasamkhyayude ganyamaaya anupaathathil kandethaavunna thrombophilic asaadhaaranathayundu, ennaal ivayil bhooribhaagavum oru adhika apakada gadakathinte saannidhyathil maathrame thrombosis vikasippikkoo.
mikka thrombophiliaykkum prathyeka chikilsayilla, pakshe thrombosisinte aavarthichulla episodukal deerghakaala prathirodha aantikoguleshante soochanayaayirikkaam. medical sayans thiricharinja thrombophiliayude aadyathe pradhana roopamaaya antithrombin kuravu 1965 ll kandethi, athesamayam ettavum saadhaaranamaaya asaadhaaranathakal (factor vii lyden ulppede) 1990 kalil vivarikkapettu.
adayaalangalum lakshanangalum.
thrombophiliayumaayi bandhapetta ettavum saadhaaranamaaya avasthakal deep weyne thrombosis (dii. vii. tii), pulmonary embolism (pii. iii) ennivayaanu, ivaye mothatthil veenas thromboempolism (vii. tii. iii) ennu vilikkunnu. kaalukalil saadhaaranayaayi sambhavikkunna dii. vii. tii. yude savisheshatha vedana, veekkam, avayavathinte chuvapp ennivayaanu. sirakalile vaalvukalkk kedupaadukal sambhavikkunnath moolam ithu deerghakaala veekkathinum bhaarathinum kaaranamaayekkam. katta potti shwaasakoshathile dhamanikalilekku maarukayum (embolyse) cheyyaam. kattayude valippatheyum sthaanatheyum aashrayich, ithu pettennulla shwasana budhimuttu, nenjuvedana, hridayamidippu ennivayilekku nayichekkaam, koodaathe thakarcha, shock, hridayasthambhanam ennivayaal sankeernamaayekkaam.
sirakalude thrombosis kooduthal asaadhaaranamaaya sthalangalilum sambhavikkaam thalachorile sirakalil, karal (portal weyin thrombosis, hepatic weyne thrombosis), mesentarik sira, vrukka (renal weyne thrombosis), kaikalile sirakal. thrombophilia arterial thrombosis (hridayaaghaathathinum strockin adisthaana kaaranamaaya) saadhyatha vardhippikkunnundo ennu nannayi sthaapichittilla. ennirunnaalum, paarambaryamaayi labhicha chila tharatthilulla thrombophilia arterial ischemic strokkinulla saadhyatha vardhippikkunnathaayi sameepakaala daatta soochippikkunnu.
thrombophilia aavarthichulla garbhachhidravumaayi bandhappettirikkunnu, koodaathe garbhaavasthayude vividha sankeernathakalaaya garbhashaya valarchaa niyanthranam, prasavam, kadinamaaya pree-eclampsia, pettennulla marupilla ennivayumaayi bandhappettirikkunnu.
protein sii yude kuravu navajaathasisuvil kattapidikkukayum tissue maranathilekkum charmmathilekkum mattu avayavangalilekkum rakthasraavam sambhavikkukayum cheiyunna gurutharamaaya rogamaaya parpura fulminansinu kaaranamakum. muthirnnavarilum eee avastha vivarichittundu. protein sii, protein ezz ennivayude kuravu enniva warferin allengil anubandha marunnukal upayogichu anticogulant chikitsa aarambhikkumbol charmma necrosis undakanulla saadhyatha vardhippikkunnathumaayi bandhappettirikkunnu.
kaaranagal.
thrombophilia janmana undakam allengil kaimaattam cheyyappedaam. "janmana undaakunna thrombophilia" ennathu thrombosis undakanulla pravanatha vardhippikkunna janmana undaakunna avasthakale (saadhaaranayaayi paarambaryamaayi, eee saahacharyathil "paarambarya thrombophilia" upayogikkaam) soochippikkunnu, maruvashathu, "aquared thrombophilia" ennathu jeevithathil pinneedu undaakunna avasthakale soochippikkunnu.
janmana.
koguleshan gadakangalude amithamaya pravartthanatthinte falamaayi undaakunnavayaan janmana undaakunna thrombophiliayude ettavum saadhaaranamaaya tharangal. avaa thaarathamyena soumyamaanu, athinaal avaye "type II" vaikalyangalayi tharamthirickunnu. factor vii lyden (1691-leeii (1691-leeii jeenil oru mutation), prothrombinile mutationaaya prothrombin prothrombin (jeeninte 3 'vivarthanam cheyyaatha mekhalayil 20210-leeii sthaanathu) ennivayaanu ettavum saadhaaranamaayath.
janmana undaakunna thrombophiliayude apoorva roopangal saadhaaranayaayi swaabhaavika aantikoagulantukalude kuravu moolamaanu undaakunnath. avaye "type I" ennu tharamthirickunnu, koodaathe thrombosisinu kaaranamakaanulla pravanathayil avaa kooduthal kadinamaanu. antithrombin III-nte kuravu, protein siyude kuravu, protein siyude kuravu ennivayaanu pradhaanappettath. xiii mutation, familial dysphybrinogenemia (oru asaadhaarana fibrinogen) ennivayaanu mithamaaya apoorva janmana undaakunna thrombophilia. fibrinolysisinte (kattakale nasippikkunna sisttam) janmana undaakunna vaikalyangal thrombosis apakadasaadhyathaykk pradhana kaaranamaakunnundo ennu vyakthamalla. udaaharanathinu, plaasminojante janmana ulla kuravu pradhaanamaayum kanninte lakshanangalkkum chilappol mattu avayavangalil prasnangalkkum kaaranamaakunnu, ennaal thrombosisumaayulla bandham kooduthal anishchithathvathilaanu.
rakthagroop oru ganyamaaya paridhivare thrombosis apakadasaadhyatha nirnnayikkunnu. oo type ozhikeyulla rakthagrooppukalullavarkk 2 muthal 4 madangu vare aapekshika apakadasaadhyathayund. rakthagroop voonn willebrand gadakathinte alavu kurayunnathumaayi bandhappettirikkunnu-vardhicha clearance kaaranam-koodaathe thrombotic apakadasaadhyathayumaayi bandhapetta ghadakam VIII.
nediyedutthu.
nediyeduttha niravadhi avasthakal thrombosis saadhyatha vardhippikkunnu. kosha stharathile gadakangalkkethiraaya aantibodikal, prathyekichu lupus anticoagulant (systemic lupus erythematosus rogamullavaril aadyamaayi kaanapedunnu, pakshe palappozhum rogamillaathavaril kandethunnu), aanti-cardiolipin aantibodikal, aanti-bii 2-glycoprotein 1 aantibodikal enniva moolamundaakunna antifospholipid syndrome oru pradhana udaaharanamaanu; athinaal ithu oru svayam rogaprathirodha rogamaayi kanakkaakkappedunnu. chila sandarbhangalil, antifospholipid syndrome damanikalkkum sirakalkkum thrombosisinum kaaranamakum. ithu garbhachhidravumaayi kooduthal shakthamayi bandhappettirikkunnu, koodaathe mattu niravadhi lakshanangalkk kaaranamakum (charmmatthinte livo reticularis, migraine enniva polullava).
heparin-induced thrombocytopenia (hitt) anticoagulant marunnaaya heparin (allengil athinte derivateevukal) nethiraaya rogaprathirodha samvidaanathinte prathikaranam moolamaanu. kuranja platelet ennathinte perilaanu ithu ariyappedunnathenkilum, sira, dhamanikalude thrombosis ennivaykkulla apakadasaadhyathayumaayi ithu shakthamayi bandhappettirikkunnu. pooraka samvidaanathil ninnu rakthaanukkalude samrakshanathil punk vahikkunna "piga" jeenile mattangal moolam undaakunna apoorva avasthayaanu paroxismal noctornal haemoglobinuria (pnh). pnh sira thrombosis saadhyatha vardhippikkunnu, pakshe ithu hemolitic anaemiyayumaayi (chuvanna rakthaanukkalude naashatthinte falamaayundaakunna anaemiya) bandhappettirikkunnu. hitt, pnh ennivaykku prathyeka chikitsa aavasyamaanu.
rakthayottathinte mandagathiyumaayi bandhapetta hematological avasthakal thrombosisinulla saadhyatha vardhippikkum. udaaharanathinu, ariwal kosha rogam (haemoglobinte mutationukal moolamundaakunna) ozhukk thakaraarilaayathukond undaakunna neriya prothrombotic avasthayaayi kanakkaakkappedunnu. athupole, asthimajja valareyadhikam rakthaanukkal ulppaadippikkunna myeloproliferative disordezhs, thrombosisilekku munkootty pookunnu, prathyekichu polycythemia vera (adhika chuvanna rakthaanukkal), avashya thrombocytosis (adhika platelettukal) ennivayil. veendum, eee avasthakal saadhaaranayaayi thirichariyumbol prathyeka chikitsa urappunalkunnu.
cancer, prathyekichu metaastaattik (shareerathile mattu sthalangalilekku vyaapikkumbol), thrombosisinulla oru amgeekritha apakada gadakamaanu. cancer koshangal vazhi kattapidikkunna sisttam sajeevamaakkukayo procogulant padaarthangalude sravam nadathukayo cheiyunnathu polulla niravadhi samvidaanangal nirddeshikkappettittund. koodaathe, prathyeka cancer chikilsakal (chemotherapic central veenas katheettarukal upayogikkunnathu polullava) thrombosis saadhyatha kooduthal vardhippikkum.
vrukka rogangal kaaranam rakthapravaahathil ninnulla protein moothrathilekku purathuvidunna nephrotic syndrome, thrombosisilekku munkootty varaam; ithu prathyekichum kooduthal kadinamaaya kesukalil (rakthathile aalbuminte alavu 25 gram/lill thaazheyaanennu soochippikkunnathu pole), membranus nephropathy avastha moolamaanu syndrome undaakunnathengil. koshajwalana malavisarjanam (ulserative colitis, krons rogam) thrombosis varaanulla saadhyatha kooduthalaanu, prathyekichu rogam sajeevamaakumbol. vividha samvidaanangal nirddeshichittundu.
garbhadharanam 2 muthal 7 madangu vare thrombosis varaanulla saadhyatha vardhippikkunnu. prasavaananthara rakthasraavathil ninnu samrakshikkunna garbhakaalathe physiological hypercogulability moolamaayirikkaam ithu. eee hypercogulability garbhakaalath sambhavikkunna uyarnna alavilulla estradiol, projestrone ennivayumaayi bandhappettirikkunnu.
eestrajanukal, samyojitha hormon janana niyanthranatthilum aarthavavirama hormon therapyilum (projastojanukalumaayi samyojippichu) upayogikkumbol, sirayile thrombosis undakanulla saadhyatha 2 muthal 6 madangu vare vardhikkunnu. upayogikkunna hormonukalude tharangal, eestrajante alavu, mattu thrombophilic apakada gadakangalude saannidhyam ennivaye aasrayichirikkunnu. protein ezz, tissue factor pathway inhibittar ennivayude kuravu polulla vividha samvidaanangalaanu ithinu kaaranamenn parayappedunnu.
ponnatthadi valarekkaalamaayi sirakalude thrombosisinulla apakada gadakamaayi kanakkaakkappedunnu. ithu niravadhi padanangalil apakadasaadhyatha erattiyaakkunnu, prathyekichu ooral garbhanirodhana maarggangalude upayogathilo shasthrakriyaykk sheshamulla kaalayalavilo. ponnathadiyullavaril vividha kattapidikkunna asaadhaaranathakal vivarichittundu. ponnathadiyullavaril uyarnna alavil fibrinolysisinte inhibittaraaya plasminogen aaktivettar inhibittar-1 kaanapedunnu. ponnathadiyulla aalukalkk tissue ghadakam vahikkunna valia ennam karangunna microvesicleukalum (kedupaadukal sambhavicha koshangalude sakalangal) undu. platelet aggregation vardhippikkaam, koodaathe voonn willebrand factor, fibrinogen, factor VII, factor VIII thudangiya uyarnna alavilulla koguleshan proteinukalum undu. ponnatthadi thrombosisinte prarambha episodinu sesham aavarthikkaanulla saadhyathayum vardhippikkunnu.
vyakthamalla.
sirakalude thrombosisumaayi bandhapetta niravadhi avasthakal janithakavum orupakshe nediyathumaanu. ivayil iva ulppedunnu ghadakam VIII, ghadakam ix, ghadakam xi, fibrinogen, 
thrombin-activatable fibrinolysis inhibittar, tissue factor pathway inhibittarinte alavu kuranju. gadakamaaya vii mutationukalkku kaaranamaakaatha sajeevamaaya protein sii prathirodham mattu gadakangal moolamaayirikkaam, ithu thrombosisinulla apakada gadakamaayi thudarunnu.
homosysteeninteyum thrombosisinteyum rakthathile alavu thammil oru bandhamund, ennirunnaalum ithu ellah padanangalilum sthiramaayi report cheythitilla. homosisteen alavu nirnnayikkunnath "emm. tii. echu. eph. aarr", "cbs" jeenukalile mutationukaliloodeyaanu, mathramalla bhakshanathe aashrayichirikkunna phoolik assid, vitaamin bii 6, vitaamin bii 12 ennivayude alavum.
samvidaanam.
oru vyakthikk thrombosis undaakunnathin palappozhum onniladhikam kaaranangalundaakaarullathil thrombosis oru multifactorial prasnamaanu. eee apakadasaadhyatha gadakangalil rakthakkuzhalukalude mathilile asaadhaaranathakal, rakthapravaahathile asaadhaaranathakal (nischalamaakkal pole), rakthathinte sthirathayile asaadhaaranathakal enniva ulppedaam. rakthathinte sthirathayile asaadhaaranathakal moolamaanu thrombophilia undaakunnath, ithu koguleshan gadakangaludeyum "koguleshan cascadil" pankedukkunna mattu raktapravaaha proteinukaludeyum alavu nirnnayikkunnu.
kedupaadukal sambhavicha tissue gadakathil ninnu tissue ghadakam purathuvidunnathiloodeyaa saadhaarana kattapidikkunnath. tissue ghadakam rakthachamkramana gadakamaaya viyayumaayi bandhippikkunnu. eee kompineshan x ghadakam xa gadakamaayum ix ghadakam ixa gadakamaayum sajeevamaakkunnu. xa ghadakam (ghadakam v-yude saannidhyathil) prothrombin thrombinilekku sajeevamaakkunnu. koguleshan prakriyayile oru kendra ensaimaanu thrombina ithu fibrinogenil ninnu fibrin uthpaadippikkukayum fibrin katta vardhippikkunna mattu niravadhi ensaimukalum coffactorukalum (factor xiii, factor xi, factor v, factor viii, taffy) sajeevamaakkukayum cheiyunnu. tfp (factor viya/tissue factor vazhi uthejippikkunna aadya ghattathe nirjjeevamaakkunnu), antithrombin (thrombin, factor aiksa, exa, xia enniva nirjjeevamaakkunnu), protein sii (protein esinte saannidhyathil gadakangalaaya vaa, viiia enniva thadayunnu), protein isad (factor exa thadayunnu) ennivayaanu prakriya thadayunnathu.
thrombophiliayil, "procogulant", "anticogulant" pravartthanangal thammilulla santhulithaavastha thadassappedunnu. asanthulithaavasthayude theevratha aarenkilum thrombosis vikasippikkanulla saadhyatha nirnnayikkunnu. proteinukalude cheria aswasthathakal, athaayath antithrombin saadhaarana nilayude <aii. dii. 1> aayi kuraykkunnathu polum thrombosis apakadasaadhyatha vardhippikkum; ithu haemophiliaykk vipareethamaayi, ithu kattapidikkunna gadakangalude alavu ganyamaayi kurayumbol maathrame undaakoo.
thrombosisile athinte falangalkku purame, hypercogulable avasthakal myocardial infarction mattu tharatthilulla hridaya sambandhamaaya rogangalum adivarayidunna dhamanikalude rogamaaya rakthapravaahathinte vikasanam thwarithappeduthiyekkaam.
roganirnayam.
thrombophiliaykkulla parisodhanakalil poornnamaaya rakthasamkhya (blud philim parishodhanaykkoppam), prothrombin samayam, bhagika thromboplastin samayam, thrombodenamics parisodhana, thrombin samayam, reptiles samayam, lupus anticoagulant, aanti-cardiolipin antibody, aanti-bii 2 glycoprotein 1 antibody, activated protein sii resistans, fibrinogen parisodhanakal, factor vii lyden, prothrombin mutation, basel homosisteen level enniva ulppedunnu. klinikkal vidhinyaayatheyum prarambha moolyanirnnayathil kandethiya asaadhaaranathakaleyum aashrayich parisodhana kooduthalo kuravo vipulamaayirikkam.
paarambarya kesukalil, rogikk kuranjathu rund asaadhaarana parishodhanakalum kudumbacharitravum undaayirikkanam.
screening.
thrombosisinte prakopanamillaattha episode ulla ellaavareyum thrombophiliaykkaayi anweshikkanamo ennathinekkurichu vyathyastha kaazhchappaadukalund. orutharam thrombophilia ullavarkku polum kooduthal thrombosis varaanulla saadhyatha illaayirikkaam, athesamayam thirichariyaan kazhiyunna thrombophilic asaadhaaranathakalillaathavaril polum munpu thrombosis baadhichavaril aavarthichulla thrombosis undakanulla saadhyatha kooduthalaanu. aavarthichulla thromboempolism, allengil asaadhaaranamaaya sthalangalil thrombosis (udha.jii. budd-chiari syndrome) leeii hepatic weyin, screening cheiyunnathinulla pothuvaayi amgeekarikkappetta soochanayaanu. thrombosisinte sakthamaaya vyakthiparamo kudumbaparamo aaya charithramulla aalukalil ithu chelavu kuranjathaayirikkaan saadhyathayund. ithinu vipareethamaayi, mattu apakadasaadhyatha gadakangalumaayi thrombophiliayude samyojanam prathirodha chikilsaykku oru soochana nalkiyekkaam, athinaalaan eee parishodhanakalude karsanamaaya maanadandangal paalikkaathavaril polum thrombophilia parisodhana nadatthuka. thrombosis oru vyakthamaaya trigger ulla rogikalil oru koguleshan asaadhaaranathaykkaayi thirayunnath saadhaaranayaayi ettedukkunnilla. udaaharanathinu, sameepakaalathe orthopedic shasthrakriyaykk sheshamulla nischalamaakkal moolamaanu thrombosis undengil, athu nischalamaakkalum sasthrakriyayum "prakopippichathaayi" kanakkaakkappedunnu, koodaathe parisodhanakal klinikkal praadhaanyamulla phalangal nalkaanulla saadhyatha kuravaanu.
oru rogikk deerghakaala chalanaseshi, sasthrakriya, aaghaatham thudangiya shanikamaaya pradhana apakada gadakangal anubhavappedumbol veenas thromboempolism sambhavikkumbol, thrombophiliaykkulla parisodhana uchithamalla, kaaranam parisodhanayude phalam rogiyude soochippicha chikitsa mattilla. 2013-ll, american sosaitti oof hemattolaji, vivekapoorvvam thiranjedukkunna prachaaranatthile shupaarshakalude bhaagamaayi, thrombophilia screening amithamaayi upayogikkunnathinethire munnariyipp nalki; parisodhanayude thettaaya positteev phalangal aalukale thrombophilia ullathayi anuchithamayi label cheiyunnathilekkum klinikkal aavasyamillaathe aantikoagulantukal upayogichu chikilsikkunnathilekkum nayikkum.
yunaittad kingdatthil, professional maargganirddeshangal thrombophilia parishodhanaykk prathyeka soochanakal nalkunnu. uchithamaaya kaunsilinginu sesham maathrame parisodhana nadathaavoo ennu shupaarsha cheiyunnu, athinaal parisodhanakal saadhaaranayaayi thrombosis roganirnayam nadakkunna samayathalla, marichu pinneedulla samayathaan nadathunnathu. rettina weyin thrombosis polulla prathyeka saahacharyangalil, thrombophilia oru pradhana apakada gadakamaayi kanakkaakkaathathinaal parisodhanaye mothatthil niruthsaahappeduthunnu. cerebral veenas thrombosis, portal weane thrombosis thudangiya hypercogulability pothuve bandhappettirikkunna mattu apoorva avasthakalil, thrombophilia screening sahaayakaramaano ennu vyakthamakkaan mathiyaya dattayilla, athinaal eee avasthakalil thrombophilia screening sambandhicha theerumaanangal thelivu adisthaanamaakkiyullathaayi kanakkakkilla. chelavu-falaprapti (chelavinu pakaramaayi gunanilavaaram krameekaricha ayuss varshangal) oru maargganirddeshamaayi edukkukayaanengil, parisodhana thiranjeduttha saahacharyangalil parimithappedutthiyittille, thrombophilia parisodhanakal palappozhum uyarnna chelavinu nyaayeekaranam nalkunnundo ennu pothuve vyakthamalla.
thrombophilia screening, prathyekichu antifospholipid aantibodikal (aanti-cardiolipin igg, igm, athupole thanne lupus anticoagulant), factor vii lyden, prothrombin mutation, activated protein sii resistans, thromboelastography ennariyappedunna anweshanathiloode koguleshante pothuvaaya vilayiruthal ennivaykkulla soochanayaanu aavarthichulla garbhachhidram.
ooral garbhanirodhana marggangal upayogikkaan padhathiyidunna sthreekalkku thrombophiliayude pathiv screening gunam cheiyunnilla, kaaranam thrombotic sambhavangalude apakadasaadhyatha valare kuravaanu. sthreeko phastt digri bandhuvino thrombosis undengil, thrombosis varaanulla saadhyatha vardhikkunnu. thiranjeduttha eee groupine parishodhikkunnath prayojanakaramaakaamenkilum negatteevu aanenkil polum avasheshikkunna apakadasaadhyathaye soochippikkaam. athinaal professional maargganirddeshangal nirddeshikkunnathu screening aasrayikkunnathinupakaram garbhanirodhanathinte badal roopangal upayogikkanamennaan.
arterial thrombosis ullavaril thrombophilia screening saadhaaranayaayi prathiphalam labhikkathathaayi kanakkaakkappedunnu, saadhaaranayaayi niruthsaahappeduthunnu, asaadhaaranamaaya cheruppakkaaraaya rogikalkkum (prathyekichu pukavali allengil eestrajan adangiya hormon garbhanirodhana marggangal upayogikkunnathu moolam) koodaathe coronary arterial baipas polulla revascularisation, graafttinte druthagathiyilulla adavu kaaranam paraajayappedunnu.
chikitsa.
oru adisthaana medical rogam (nephrotic syndrome polullava) moolamundaakunnillengil, thrombophiliaykku prathyeka chikilsayilla, avide adisthaana rogathinte chikitsa aavasyamaanu. prakopanamillaathe allengil/allengil aavarthichulla thrombosis ullavarilo allengil uyarnna apakadasaadyathayulla thrombophilia ullavarilo, kooduthal episodukalude apakadasaadhyatha kuraykkunnathinu warferin polulla anticogulation marunnukal deerghakaala adisthaanathil upayogikkano ennathaanu ettavum pradhanappetta theerumaanam. eee apakadasaadhyatha chikitsa ganyamaaya rakthasraavathinu kaaranamakumenna apakadasaadhyathaykkethire alakkendathundu, kaaranam vanthothilulla rakthasraavathinulla saadhyatha prathivarsham 3 sathamaanathiladhikamaanu, athinte falamaayi valia rakthasraavamullavaril 11 sathamaanam perr marichekkaam.
mukalil soochippicha thrombophilia roopangalkku purame, thrombosisinte oru episodinu sesham aavarthanathinulla saadhyatha nirnnayikkunnath yadhaartha thrombosisinte vyapthiyum kaadinyavum, athu prakopippichathaano (nischalamaakkal allengil garbham polullava), munbathe thrombotic sambhavangalude ennam, purusha laingikatha, thaazhnna vena kaava filterinte saannidhyam, kaansarinte saannidhyam, poost-thrombotic syndrominte lakshanagal, ponnatthadi thudangiya gadakangalaanu. kandethaavunna thrombophiliayude saannidhyathekkaalum abhaavathekkaalum theerumaanatthil eee gadakangal kooduthal pradhaanamaan.
antifospholipid syndrome ullavarkku thrombosisinte aadya prakopanamillaattha episodinu sesham deerghakaala anticogulation nalkaam. kandethappetta antibody upavibhagam, antibody titer (aantibodikalude alavu), onniladhikam aantibodikal kandethiyittundo, athu aavarthichu kandethunnundo atho orotta avasarathil maathram kandethunnundo ennathaanu apakadasaadhyatha nirnnayikkunnath.
garbhadhaaranathekkurichu chinthikkunnatho garbhinikalaayatho aaya thrombophilia ulla sthreekalkku saadhaaranayaayi garbhaavasthayil warferinu pakaramullava aavasyamaanu, prathyekichu aadyathe 13 aazhchakalil, ithu garbhastha sisuvil asaadhaaranathakal undaakkum. kuranja tanmatra bharamullah heparin (enoxaparin polulla elmdabluechu) saadhaaranayaayi oru badalaayi upayogikkunnu. mulayoottunnathil warferin, elmdabluechu enniva surakshithamaayi upayogikkaam.
sthreekalkku thrombophiliaykku sesham aavarthichulla garbhadhaarana nashtam anubhavappedumbol, kuranja tanmatra bharamullah heparin garbhachhidrathinulla saadhyatha kuraykkunnuvennu chila padanangal soochippikkunnu. ellah padanangaludeyum phalangal orumichu vishakalanam cheyyumbol, sthithivivarakkanakusaricha kaaryamaaya nettamonnum prakadippikkaan kazhiyilla.
pravachanam.
kandethaavunna thrombophilia illaathavaril, 60 vayass aakumbozhekkum thrombosis varaanulla sanjitha apakadasaadhyatha ekadesham 12 sathamaanamaanu. antithrombin kuravullavaril ekadesham 60 sathamaanam perkkum 60 vayass aakumbozhekkum oru thavanayenkilum thrombosis anubhavappettekkam, athupole thanne protein sii kuravullavaril 50 sathamaanavum protein ezz kuravullavaril moonnilonnu perum. sajeevamaaya protein sii prathirodhamulla aalukalkk (saadhaaranayaayi factor vii lyden moolam undaakunna), vipareethamaayi, thrombosis undakanulla saadhyatha alpam vardhikkunnu, 15 sathamaanam perkku arupath vayass aakumbozhekkum kuranjathu oru thrombotic sambhavamenkilum undaayittund. pothuve, sthreekale apekshichu purushanmaarkk veenas thrombosis aavarthichulla episodukal anubhavappedaanulla saadhyatha kooduthalaanu.
factor vii lyden ulla aalukalkk thrombosis varaanulla saadhyatha thaarathamyena kuravaanu, pakshe nischalamaakkal polulla adhika apakada gadakathinte saannidhyathil thrombosis vikasichekkaam. prothrombin mutation mutation ulla mikka aalukalilum orikkalum thrombosis undaakilla.
epidemiology.
pradhana ("type 1") thrombophilia apoorvamaanu. saadhaarana janasamkhyayude 0.20 sathamaanavum sirasanchar ullavaril <aii. dii. 1>% perum antithrombin kuravaanu kaanikkunnath. janasamkhyayude 0.20% aalukalil protein siyude kuravum kaanapedunnu, ithu thrombosis ullavaril <aii. dii. 1>% aalukalil kaanapedunnu. janasamkhyayil protein kuravinte krithyamaaya vyaapanam anjjaathamaanu; thrombosis ullavaril ithu <aii. dii. 1>% aayi kaanapedunnu.
cheria ("type 2") thrombophilia valare saadhaaranamaanu. vadakkan europian vamshajaraaya janasamkhyayude 5 sathamaanathil factor vii lyden kaanapedunnu, ennaal eshyan allengil aafrikkan verthirichedukkunnathil ithu valare apoorvamaanu. thrombosis ullavaril 10 sathamaanam perkku factor vii lyden undu. thrombophilia parishodhanaykk ruffer cheyyappedunnavaril, 30-50% enna takaraar undu. saadhaarana janasamkhyayil prothrombin mutation sambhavikkunnath <aii. dii. 1> enna nirakkilum thrombosis ullavaril <aii. dii. 2> enna nirakkilum 15 sathamaanam aalukalum thrombophilia parishodhanaykk vidheyaraanu. factor vii lyden pole, eee asaadhaaranatha aafrikkakkaarilum eshyakkaarilum asaadhaaranamaanu.
vyathyastha padanangal eee avasthayude vyathyastha nirvachanangal upayogikkunnathinaal antifospholipid syndrominte krithyamaaya vyaapanam ellaavarkkum ariyilla. thrombophilia parishodhanaykk vidheyamaakkunna 24 sathamaanam antifospholipid aantibodikalilum kandethiyittundu.
charithram.
jarmman doctor rudolph vircho 1856-ll raktham sthirathayil asaadhaaranathakale thrombosis vikasippikkunnathinulla oru gadakamaayi tharamthirichu. 1965-ll norwegian hemattolajist olaav ejeberg amtithrombin kuravaaya thrombophiliayude aadya roopam thirichariyunnathuvare eee asaadhaaranathakalude krithyamaaya swabhaavam avyakthamaayirunnu. 1981ll scrips research instittyoottileyum yuu. esileyum gaveshakar protein siyude kuravu vivarichu.ezz. roga niyanthrana kendrangal. 1984ll oklahoma sarvakalaashaalayile gaveshakar vivaricha protein kuravaanu ithinu kaaranamaayath.
systemic lupus erythematosus, thrombosis ennivayulla aalukalil nirddishta antibodikalekkurichulla vividha muun reportukalkku sesham 1980 kalil antifospholipid syndrome poornnamaayum vivarikkapettu. britiish rheumatologist graham aarr aanu palappozhum eee syndrome undakaan kaaranam.vii. aalinganam cheiyunnu, aaa kaaranathaal palappozhum "huggus syndrome" ennu vilikkunnu.
vilikkunnu kooduthal saadhaaranamaaya janithaka thrombophiliyakalekkurich vivarikkapettu. thrombosis ulla pala aalukalum prathirodham sajeevamakkiya protein sii kaanikkunnuvenn pala padanangalum munbu soochippichirunnu. 1994ll netherlandsile lidenile oru samgham ettavum saadhaaranamaaya adisthaana vaikalyam thiricharinju-activated protein siyude pravartthanatthe prathirodhikkunna ghadakam vii-yile oru mutation. janithaka vaikalyangal saadhaaranayaayi avaa kandethiya sthalathinte perilaanu ariyappedunnathu ennathinaal eee vaikalyathe "factor vii lyden" ennu vilichirunnu. rund varshathinu sesham, athe group prothrombin jeenile oru saadhaarana mutatione vivarichu, ithu prothrombin alavu uyarthunnathinum thrombosis apakadasaadhyathayil neriya vardhanavinu kaaranamaayi.
jeenukalil cheria maattangalkkaayi thirayunna muzhuvan janithaka kodukaludeyum padanangaliloode familial thrombosisinu adisthaanamaaya mattu janithaka asaadhaaranathakal bhaviyil kandethumennu samshayikkunnu.

