
beetaa thalassemia

beetaa thalassemia (β thalassemia) paarambaryamaayi labhikkuna raktha vaikalyangalude oru koottamaanu. haemoglobinte beetta shringalakalude synthesis kurayukayo illaathirikkukayo cheiyunnathu moolamundaakunna thalassemiayude roopangalaanu avaa, ithu gurutharamaaya vilarcha muthal clinically lakshanangalillaatha vyakthikal vareyulla variable falangalkku kaaranamaakunnu. aagola vaarshika sambhavangal 100,000 ll onnu aayi kanakkaakkappedunnu. haemoglobin subunit beetta allengil echbibiyile thakaraarukal moolamaanu beetta thalassemia undaakunnath. rogathinte theevratha mutationte swabhaavathe aasrayichirikkunnu.
kaalakramena echbibi thadayal beetta-cheyin synthesis kurayunnathilekku nayikkunnu. puthiya beetaa cheyinukal nirmmikkanulla shareerathinte kazhivillaayma echu. bii. eiii (muthirnna haemoglobin) yude kuranja ulppaadanathilekku nayikkunnu. chuvanna rakthaanukkal niraykkunnathinu mothatthil labhyamaya echu. bii. eyude kuravu micrositic anaemiyayilekku nayikkunnu. mathiyaya chuvanna rakthaanukkalude pravarthanatthinu aparyapthamaaya echbibi proteinumaayi bandhappett micrositic anaemiya aathyanthikamayi vikasikkunnu. eee ghadakam kaaranam, beetaa-cheyinukalile thadassam nikathaan rogikk rakthappakarcha aavashyamaayi vannekkaam. 
aavarthichulla rakthappakarcha irumbinte amithabharavumaayi bandhapetta gurutharamaaya prashnangalkk kaaranamaakunnu.
adayaalangalum lakshanangalum.
moonnu pradhana roopangal vivarichittundu rogalakshanamillaathatho neriyatho aaya lakshanangalil ninnu aajeevanantha rakthappakarcha aavasyamulla kadinamaaya anaemiya vare vyathyaasappedunna thalassemia minor, thalassemia intermedia, thalassemia mejar. beetaa thalassemia mejar ulla vyakthikal (thalassemia mutationukalkku homocygus ullavar, allengil 2 mutationukal paarambaryamaayi labhikkunnavar) saadhaaranayaayi jeevithathinte aadya rund varshathinullil rogalakshanamulla gurutharamaaya vilarcha, mosham valarcha, asthikooda asaadhaaranathakal ennivayumaayi prathyakshappedunnu. chikilsikkaattha thalassemia mejar oduvil maranathilekku nayikkunnu, saadhaaranayaayi hridayasthambhanam; athinaal, prasavapoorva parisodhana valare pradhaanamaan. beetaa thalassemia intermedia ullavar (beetaa thalassemia myootteshante samyuktha hetterosaigottukallur) saadhaaranayaayi pinneedu jeevithathil vilarchayude neriyatho mithamaayatho aaya lakshanangalumayi prathyakshappedunnu. beetaa thalassemia swabhaavam (beetaa thalassemia minor ennum ariyappedunnu) beetaa thalassemia myootteshante heterosaigas anantharaavakaasham ulkkollunnu, koodaathe rogikalkku saadhaaranayaayi borderline micrositic, hypochromic anaemiya enniva undaakunnu, avaa saadhaaranayaayi lakshanangalillaathatho neriya lakshanangalullatho aanu. beetaa thalassemia mainarinu beetaa thalassemia nishabda vaahakarayum avatharippikkan kazhiyum; beetaa thalassemia mutation paarambaryamaayi labhichavarkku ennaal hematological asaadhaaranathakalo lakshanangalo illa. thalassemia ulla chila aalukal pleeha (hypersplenism), pitthasanchi (peripheral haemolysisil ninnulla hyperbilirubinemia kaaranam) enniva ulppedunna aarogyaprashnangalkk vidheyaraanu. thalassemia mejar, intermedia rogikalilaanu eee sankeernathakal kooduthalum kaanappedunnath.
adikamaaya irumbu (haemolysis allengil traansfushanukalil ninnu) karal, hridayam, endocrine grandhikal ennivaykkullil gurutharamaaya sankeernathakal srishtikkunnu. karal sirosis, karal fibrosis, angeyattatthe kesukalil karal cancer ennivayaanu gurutharamaaya lakshanagal. beetaa thalassemia mejar moolamundaakunna jeevan apakadappeduthunna avasthakalaanu hridayasthambhanam, valarchaa vaikalyam, prameham, austioporosis enniva. idathu ventricular systolic, diastolic disfungshion, pulmonary hypertension, valvuloppathi, aritmiyas, pericarditis ennivayaanu beetaa thalassemiayudeyum irumbinteyum amithabhaarathinteyum falamaayi kaanapedunna pradhana hridaya vaikalyangal. dahananaalathile irumbinte agiranam ellah tharatthilumulla beetaa thalassemiayilum kaanapedunnu, koodaathe phalapradamallaatha erythropoisis kaaranam pleehayude chuvanna rakthaanukkalude nasham vardhikkukayum rakthapravaahathilekk adhika irumbu purathuvidukayum cheiyunnu.
beetaa thalassemia mejarinteyo intermediayudeyo adhika lakshanangalil ksheenam, valarcha, kuttikkaalatthe vikasana kaalathamasam, kaalinte alsar, avayavangalude parajayam ennivayulppede mithamayathum kadinavumaaya vilarchayude classic lakshanagal ulppedunnu. phalapradamallaatha erythropoisis (chuvanna rakthaanukkalude uthppaadanam) nashtaparihara asthi majjayude vikaasathilekku nayichekkaam, ithu asthi mattangal/vaikalyangal, asthi vedana, craniophasial asaadhaaranathakal ennivayilekku nayichekkaam. erythropoisis undakaan saadhyathayulla karal, pleeha thudangiya extramedullary avayavangal sajeevamaakukayum hepatosplenomegaly (karalinteyum pleehayudeyum valarcha) yilek nayikkukayum cheiyunnu. shareerathile mattu koshangalum erythropoyisisinte sthalangalaayi mariyekkaam, ithu extramedullary hematopoyattik seudotumerukalilekku nayikkunnu, ithu thorassic arayilo sushumna canalilo sambhavikkukayaanenkil compress lakshanangalkk kaaranamakum.
kaaranam.
mutationukal.
mutationukalude rund pradhana grouppukale verthirichariyaan kazhiyum
β globin shringalakalude roopeekaranam thadayukayaanenkil mutationukal (βo) aayi visheshippikkappedunnu, chila β globin shringhala roopeekaranam sambhavikkaan anuvadikkukayaanenkil mutationukal (β +) aayi visheshippikkappedunnu.
marnah assembly.
haemoglobin badhikkunna oru paarambarya rogamaanu beetaa thalassemia. paarambarya rogangalil pakuthiyolam varunnathupole, paarambaryamaayi vanna oru parivartthanam oru chromosomil ninnu pakarthiya sandeshavahaka-tharam rna (mrna) yude assembly nasippikkunnu. amino aasidukale proteinukalaayi bandhippikkunnathinulla nirddeshangalum (jeenukal) uthppaadippikkunna protein alavu niyanthrikkunnathil pradhana punk vahikkunna dnaude strechukalum dnal adangiyirikkunnu.
thalassemiayil, oru adhika, thudarchayaaya neelam allengil noonn-coding nirddeshangalude oru thudarchayaaya bhagam emrnal ulppeduthiyittundu. dna templettinte intronic, exonic bhagangal thammilulla athirthi mutation illaathaakkunnathinaal ithu sambhavikkunnu. ellah coding vibhaagangalum ippozhum undakaamennathinaal, saadhaarana haemoglobin ulppaadippikkappedukayum, kootticherttha janithaka vasthukkal, athu pathology uthppaadippikkukayaanel, pakaram vilarcha undakkaan paryapthamaaya niyanthrana pravartthanangale thadassappeduthukayum cheiyunnu. haemoblogininte saadhaarana alfa, beetta subunitukalkku oronninum irumbu adangiya oru central paars (heam) undu, athu oru sabyoonittinte protein cheyin athine chutti madakkaan anuvadikkunnu. saadhaarana praayapoorthiyaayavaril haemoglobinil 2 alfa 2 beetta sabyoonittukalum adangiyirikkunnu. thalassemia saadhaaranayaayi beetaa shringalakalude ulppaadanathinulla emrnakale maathrame baadhikkukayulloo (athinaal eee peru). mutation orotta aditharayile (single-nucleotide polymorphism) maattamaayirikkaamennathil, aaa orotta thiruthal nadatthaan jeane theraappikal thedaanulla sramangal thudarukayaanu.
apakadasaadhyatha gadakangal.
kudumbacharitravum vamshaparambarayumaanu beetaa thalassemiayude saadhyatha vardhippikkunna gadakangal. kudumbacharithrathe aashrayich, oru vyakthiyude maathapithaakkalkko muthasshimaaro beetaa thalassemia mejar allengil intermedia undengil, parivartthanam cheyyappetta jeane oru santhathikk paarambaryamaayi labhikkanulla 75 sathamaanam (4 ll 3) saadhyathayund (pejinte mukalil anantharaavakaasha chart kaanuka). oru kuttikk beetaa thalassemia mejaro intermediayo illengilum, avarkk oru vaahakaraakaan kazhiyum, ithu avarude santhathikalil bhaavi thalamurakalk beetaa thalassemia undaakunnathin kaaranamakum.
mattoru apakada gadakamaanu vamshaparambara. itaaliyan, greak, middle eestten, dakshineshyan, aafrikkan vamshajarilaan beetaa thalassemia kooduthalum kaanappedunnath.
roganirnayam.
hypersplenism moolamundaakunna vayaruvedana, pleeha infarction, pitthasanchi moolamundaakunna valath-mukalile kwaadrant vedana ennivayaanu pradhana klinikkal prakadanangal. ennirunnaalum, rogalakshanangalil ninnu maathram thalassemia nirnnayikkunnath aparyapthamaanu. eee rogathinte sankeernnatha kaaranam eee adayaalangal anubandhamaanennu doctormar rekhappeduthunnu. thaazhepparayunna anubandha adayaalangalkku phenotypinte theevratha sthireekarikkaan kazhiyum pallore, mosham valarcha, aparyapthamaaya bhakshanam kazhikkal, pleanomagaly, manjappitham, maxillary hyperplasia, dental maloclusion, colelithiasis, gurutharamaaya vilarchayudeyum rogalakshanangalude odivukaludeyum saannidhyathil systolic ejection pirupirukkal. rogalakshanangalude adisthaanathil, oru deferential diagnosisinaayi parishodhanakalkk utharavidunnu. eee parisodhanakalil poornnamaaya rakthasamkhya; haemoglobin electrophoresis; serem transferrin, ferritin, motham irumbu bandhippikkal sheshi; yoorin eurobilin, eurobilogen; kodosaittukal allengil taargett cellukal kaanichekkaavunna peripheral blud smear; hematokritt; serem bileerubin enniva ulppedunnu. haemoglobin eiii2-nte alavu vardhikkukayum haemoglobin eph-nte alavu cheruthaayi vardhikkukayum cheiyunnathaanu beetaa-thalassemia ullavaril haemoglobin electrophoresisinecture prathekshikkunna reethi. haemoglobin electrophoresis allengil uyarnna prakadanamulla liqvid chromatography vazhiyaan roganirnayam sthireekarikkunnathu.
asthimajjayude vikaasavumaayi bandhapetta asthikooda mattangal
dna vishakalanam.
ellah beetaa thalaseemiyakalum asaadhaaranamaaya chuvanna rakthaanukkal prakadippichekkaam; oru kudumbacharithrathe thudarnnu dna vishakalanam nadathunnu. alfa, beetaa globin uthppaadippikkunna jeenukalile illaathaakkalukalum mutationukalum anweshikkaan eee parisodhana upayogikkunnu. kudumbatthile mattu amgangalil kaanapedunna parivartthanangalude tharangalum carrier nilayum vilayiruthunnathin kudumba padanangal nadatthaam. dna parisodhana pathivallengilum thalassemia kandethaanum carrier nila nirnnayikkaanum sahayikkum. mikka kesukalilum chikilsikkunna doctor vilarchayude lakshanagal vilayiruthunna oru klinikkal prediaragnosis upayogikkunnu ksheenam, shwaasathadassam, mosham vyayaama sahishnutha. pathiv electrophoresis budhimuttaanennu theliyikkappettal kooduthal janithaka vishakalanathil echu. pii. ell. sii ulppedaam.
prathirodham.
beetaa thalassemia ennathu oru paarambarya rogamaanu, ithu carrier screening, prasavapoorva roganirnayam ennivayiloode prathirodha chikitsa anuvadikkunnu. oru rakshakarthaavin saadhaarana jeenukal undengil ithu thadayaan kazhiyum, ithu saadhaarana haemoglobin ulla pankaalikale thiranjedukkaan vaahakanmare shaaktheekarikkunna parishodhanakalkk kaaranamaakunnu. sickle sell rogamulla santhathikalkku kaaranamakunna jeenukal kandethukayenna lakshyathodeyulla oru patanam. beetaa thalassemia baadhicha rogikalkku emm. sii. echu ≤26 pii. jii. yum 10,148 rogikalil aarr. dii. dablyu <19 umm undu, 1,739 rogikalkku haemoglobin phenotype aarr. dii. dablyu. beetaa thalaseemiyayumaayi poruthappedunnu. rogikalude ennam kuranjathinushesham, beetaa thalassemia baadhicha 77 rogikale kaanikkunna echu. bii. eiii 2 level pareekshichu. eee screening nadapadikramam padinjaaran aafrikkan vamshajaraaya janasamkhyayil samvedanakshamathayillaathayi thelinju, kaaranam soochakangalil alfa thalassemiayude uyarnna vyaapanamund. haemoglobinopathy rogangalude vaahakarumaayi bandhapetta prathyulpaadana apakadasaadhyathakalekkurichu vivarangal vitharanam cheiyunna paripaadikal raajyangalkkund. skoolukalilum sayudha senayilum bahujana maadhyamangaliloodeyum vidyaabhyaasa paripaadikalum carrierukalkkum carrier dambathikalkkum kaunsiling nalkunnathum thalassemia carrier screening prograamukalundu. screening sambhavangal kuranju; 1995 aayappozhekkum italiyile vyaapanam 1:250 ll ninnu 1:4000 aayi kurayukayum aaa pradeshathu 95 sathamaanam kurayukayum cheythu. rakthathinte aavasyam kuranjathinaal thalassemia baadhichavarkku rogathinte thoth kuranjathu gunam cheythu, athinaal chikilsayude vitharanam mechappeduthi.
chikitsa.
beetaa thalassemia mejar.
rogabadhitharaaya kuttikalkk pathivayi aajeevanantha rakthappakarcha aavasyamaanu. asthimajja maattivaykkal chila kuttikalkk rogashanti nalkum. rogikalkku idaykkide rakthappakarcha labhikkunnu, ithu irumbinte amithabhaarathilekku nayikkukayo shakthamaakkukayo cheiyunnu. irumbu amithabharamulla kesukalil aantharika avayavangalkk kedupaadukal sambhavikkunnath thadayaan irumbu cheleshan chikitsa aavasyamaanu. irumbu cheleshan chikilsakalile purogathi thalassemia mejar ulla rogikalkku sariyaaya chikitsa labhyamaakkikkond deerghaayussu jeevikkaan anuvadikkunnu. deferoxamine, deferipron ennivayaanu janapriya chelettarukalil ulppedunnathu.
chila rajyangalil 2005ll ooral chelettar deferasirox upayogathinu amgeekaaram labhichu. kadinamaaya thalassemia rogikalaaya rogikalkku soochippikkunna oreyoru prathividhi asthimajja maattivaykkalaanu. transplantation vazhi oru rogikk rakthappakarchaye aashrayikkunnath illaathaakkaan kazhiyum. oru poruthappedunna daathaavinte abhaavathil, rogathil ninnu mukthamayirikkaanum sweekarthaavinte human leukosite aantijan (echla) tharavumayi poruthappedaanum pree implaanteshan janithaka roganirnayathiloode (pgd) oru rakshaka sahodarane sangalppikkaan kazhiyum.
irumbinte amithabhaarathinte alavu nirnnayikkaan beetaa thalassemia ullavaril serem ferritin (irumbinte sambharana roopam) pathivayi alakkunnu; vardhicha ferritin alavu irumbu cheleshan therapy upayogathe nayikkunnu. moonnu irumbu chelettarukal; subcutaneous deferoxamine, ooral deferripron, ooral deferrasirox enniva monotherapy allengil samyojippichu upayogikkaam, avayellaam serem/systemic irumbinte alavu, hepatic, cardiac irumbinte alavu kuraykkukayum hridaya aritmiya, hridayasthambhanam, maranam ennivayude saadhyatha kuraykkukayum cheiyunnu. hepatic, myocardial emm. aarr. aii enniva lakshya avayavangalile, prathyekichu hridayathilum karalilum irumbu nikshepam alakkaan upayogikkunnu.
wheel cornell medical kolejile shaasthranjar beetaa-thalassemia, sickle sell rogam enniva chikilsikkaan kazhiyunna oru jeane therapy thanthram vikasippicheduthittundu. jeane transcription vivarthanavum mechappeduthunnathinum β-globin ulppaadanathinte alavu vardhippikkunnathinam manushyante β-globin jean oru aankirin insulettarum vahikkunna lentiviral vekttarinte vitharanathe adisthaanamaakkiyullathaanu eee saankethikavidya.
2022 juun 10 nuu yuu.ezz. beetaa thalassemia upayogikkunnathinaayi oru jeane therapy chikilsaykku fda amgeekaaram nalkanamennu federal upadeshaka samiti shupaarsha cheythu. nirmmathaavaaya bluebird bio athinte ottathavana chikilsayaaya synteglo (beettibeglojen autotemsal) kku amerikkayil 28 laksham dollar eedaakkunnu.
beetaa thalassemiayil garbhapindatthile haemoglobin uthppaadanam vardhippikkunnathinum bcl11eiii jeenine thadayunnathiloode sickle sell anaemiyaykkum lakshyamitulla jeane editting theraappikalum paryavekshanam cheyyappedunnu.
sasthrakriya.
thalassemia mejar ulla rogikalkku splenectomy cheyyaan kooduthal thaalpparyamund. mathiyaya thothil rakthappakarcha cheytha rogikalil hypersplenisathinte vyaapanam kuranjathinaal sameepa varshangalil splenectomikalude upayogam kurayunnu. pleeha shareerathil ninnu rogalakshanamo asaadhaaranamo aaya chuvanna rakthaanukkale ozhivakkaan scavenchingle earppedunnathinaal splinectomy anubaadhaykkulla saadhyathayum vascular rogam moolamulla rogaavasthayum vardhippikkunnathumaayi bandhappettirikkunnu. hypersplenism ulla rogikalkku avarude shareerathil aarogyakaramaya rakthaanukkalude alavu saadhaaranayekkaal kuravaayirikkaanum vilarchayude lakshanagal velippeduthaanum saadhyathayund. ooppan, laparoscopic reethikalaanu vyathyastha shasthrakriya reethikal. laparoscopic reethikk kooduthal pravartthana samayam aavasyamaanu, ennaal cheruthum kuravullathumaaya shasthrakriyayude vadu ulla oru hrasvamaaya veendedukkal kaalayalav aavasyamaanu. pleeha muzhuvan neekkam cheiyunnathu anaavashyamaanengil oru bhagika pleehamatta sasthrakriya sambhavikkam; eee reethi hypersplenisathinte saadhyatha kuraykkumbol rogaprathirodha pravartthanatthinte chila bhagangal samrakshikkunnu. pleehamatta shasthrakriyaykk vidheyaraakunnavarkku shasthrakriyaykk kuranjathu orazhcha munbengilum (munganana moonnu aazhchakal) uchithamaaya pneumococcal vaxine nalkanam.
chikitsa.
deerghakaala transfusion therapy (transfusion ashrita beetaa thalassemia ullavaril) haemoglobin alavu 9-10.5 g/dl (11-12 g/dl) enna transfusion pree-haemoglobin levelil nilanirthaan upayogikkunna oru chikilsayaanu. gunanilavaaramulla rakthappakarcha urappakkaan, pothinja chuvanna rakthaanukkal leukoredues cheyyanam. leucoreduced blud paakkattukal ullathiloode, malinamaaya velutha koshangal prathikoola prathikaranangal undakkunnathinum platelet aloi immunisation thadayunnathinum rogikk apakadasaadhyatha kuravaanu. allergy kaimatta prathikaranangalo asaadhaaranamaaya chuvanna kosha aantibodikaloo ulla rogikalkku kazhukiya chuvanna koshangalo cryopreserved chuvanna koshangalo labhikkanam. kazhukiya chuvanna koshangal plasma proteinukal neekkam cheythittundu, athu rogiyude aantibodikalude lakshyamaayi maarukayum rakthappakarcha surakshithamaayi nadatthaan anuvadikkukayum cheyyum. asaadhaaranamaaya chuvanna kosha aantibodikaloo allengil saadhaarana chuvanna kosha aantijanukalo illaatha rogikalkku apoorva daathaakkalude yoonittukalude vitharanam nilanirthaan cryopreserved chuvanna koshangal upayogikkunnu. eee pathiv rakthappakarcha saadhaarana valarchayum shaareerika pravartthanangalum prolsaahippikkukayum asthi majjayude hyper aaktivitti adichamarthukayum cheiyunnu.
farmaceutical.
saadhaarana irumbu homeostasis samayat rakthachamkramanam nadathunna irumbu transferrin aayi bandhikkappettirikkunnu. ennaal amithamaya irumbu (pathivayi rakthappakarcha polullava) ullathinaal, transferrin irumbine bandhippikkanulla kazhivu kaviyukayum transferrin ithara bandhita irumbu shekharikkappedukayum cheiyunnu. eee aniyanthrithamaaya irumbu ooksijan enangale prerippikkanulla uyarnna pravanatha kaaranam vishaamshamullathaan, ithu sellular naashathinu kaaranamaakunnu. irumbinte amithabharam thadayunnathu rogaavasthayil ninnum marananirakkil ninnum rogikale samrakshikkunnu. shareerathil ninnu irumbumaayi bandhippikkukayum neekkam cheyyukayum cheyyuka ennathaanu praadhamika lakshyam, transfusional irumbu inputtinte nirakkin thulyamo irumbu inputtinekkaal kooduthalo aanu nirakk. irumbu amithabhaarathinte sankeernathakal thadayaan kazhiyunna oru medical theraappiyaan ayann cheleshan. rakthathinte oro yoonittilum <aii. dii. 1> milligram irumbu adangiyittundu, adhika irumbu neekkam cheyyaan sareerathinu swaabhaavika samvidaanamilla. irumbu chelettarukal (deferoxamine, deferripron, deferrasirox) upayogichu adhika irumbu neekkam cheyyaan kazhiyum.
transfusion ashrita beetaa thalassemia ulla muthirnnavaril chikilsayaayi upayogikkunna oru recombinent fusion proteinaan laspaattersept (easu-536). human aaktivin risapttar type aii. aii. biyude parishkaricha extra sellular domaine ithil adangiyirikkunnu, ithu human aii. jii. jii 1 antibody eph. sii bhagavumaayi bandhippichirikkunnu. smad2,3 signalingukal thadayunnathinaayi valarchaa ghadakam beetta suuppar famili ligandukal thiranjedukkunnathinu thanmatra bandhippikkunnu, angane erythroid pakwata vardhippikkunnu. placeboyumaayi thaarathamyappeduthumbol rakthappakarchaye aasrayikkunna beetaa thalassemia ulla muthirnnavaril eee marunnu rakthappakarchayude bharam 33 sathamaanam kuraykkumennu theliyikkappettittund, koodaathe ferritin alavu kurayunnathumaayi (karalinteyo hridayathile irumbinteyo alavil kaaryamaaya kuravilla) bandhappettirikkunnu.
beetaa thalassemia intermedia.
beetaa thalassemia intermedia ulla rogikalkku rakthappakarcha aavasyamilla allengil chila saahacharyangalil (anubadha, garbham, sasthrakriya) episodic rakthappakarcha aavashyamaayi vannekkaam. idaykkide rakthappakarcha nadakkunna rogikalkku amithamaya irumbu undaakukayum cheleshan therapy aavashyamaayi varikayum cheyyum. transmission autosomal resessive aanu; ennirunnaalum, prabalamaaya mutationukalum samyuktha heterosaigottukalum report cheyyappettittund. janithaka kaunsiling shupaarsha cheyyukayum prasavapoorva roganirnayam vaagdaanam cheyyukayum cheyyaam.
beetaa thalassemia minor.
beetaa thalassemia minor ulla rogikal saadhaaranayaayi rogalakshanangalillaathavar palappozhum chikilsayillaathe nireekshikkappedunnavar. beetaa thalassemia minor vittumaaraatha heppattaitis bii, vittumaaraatha heppattaitis sii, noonn-alcoholic phaatti liver disease, alcoholic liver disease thudangiya mattu avasthakalumaayi sahavarthichekkaam, ithu samyojippikkumbozho sahavarthikkumbozho oru vyakthikk karalil irumbinte amithabharavum kooduthal gurutharamaaya karal rogavum undakaan kaaranamakum.
epidemiology.
thalassemiayude beetta roopam medittareniyan janathayil prathyekichum prachaaratthilundu, eee bhoomishaasthraparamaaya associationaanu athinte perinu utharavaadikal "thalassa" ("θάλασσα") kadalinte greak padavum "haima" ("ατμα") rakthathinte greak padavumaanu. europil, rogathinte ettavum uyarnna saandrata grease, turkey theerapradeshangalilaanu kaanappedunnath. sisili, sardinia, corsica, cypress, malta, kreett thudangiya pradhana medittareniyan dveepukale (baliarics ozhike) prathyekichum saaramaayi badhikkunnu. mattu medittareniyan janathayilum medittareniyan pradeshangalkk sameepamullavarilum pashchimeshya, vadakke aphrica ennividangalil ninnullavar ulppede uyarnna rogavyaapananirakkund. greak, turkish cypriyattukalil 15 sathamaanam beetaa-thalassemia jeenukalude vaahakaraanennum janasamkhyayude 10 sathamaanam alfa-thalassemia jeenukal vahikkunnuvennum daatta soochippikkunnu.
parinamaparamaaya poruthappedutthal.
thalassemia swabhaavam maleriyaykkethire oru paridhivare samrakshanam nalkiyekkaam, ithu swabhaavam saadhaaranamaaya pradeshangalil prachaaratthilundo allengil nilavilundo, angane kaariyarukalkk (heterosaigas aanukoolyam ennariyappedunnu) thiranjeduttha athijeevana nettam nalkunnu, angane mutation nilanilkkunnu. aaa kaaryatthil, vividha thalassemia haemoglobin, ariwal kosha rogathe badhikkunna mattoru janithaka thakararumaayi saamyamullathaan.
sambhavam.
chila vamseeya vibhaagangalilum praaya vibhaagangalilum eee vaikalyam kooduthalaayi kaanapedunnu. sub-saharan aafrikkayile pradeshangal ulppedunna "thalassemia belttil" beetaa thalassemia ettavum kooduthal kaanapedunnu, medittareniyan middle eest, thekkukizhakkan eshya ennividangalilekku vyaapichukidakkunnu. eee bhoomishaasthraparamaaya vitharanam maleriyaykkethire prathirodham nalkunna beetaa-thalassemia carrier stettu (beetaa thalassemia minor) moolamaanennu karuthappedunnu. amerikkayil 272,000 allengil 1,000 peril ekadesham oraalaan thalassemiayude vyaapanam. 2002ll englandil 4,000 perkku ashupathriyil praveshippikkappedukayum thalassemiayude 9,233 kansalttant episodukal undaakukayum cheythittundu. ashupatri kansalttant kesukalil 53 sathamaanavum purushanmaarum 47 sathamaanavum sthreekalum aanu. sharashari rogi praayam 23 aanu, kansalttantukalil oru sathamaanam maathrame 75 vayassinu mukalilulloo, 69 sathamaanam perr <aii. dii. 1> aanu. loka janasamkhyayude 1.5 sathamaanam rogavaahakaraanennum prathivarsham 40,000 rogabadhitha sisukkal eee rogavumaayi janikkunnuvennum kanakkaakkappedunnu. rakthappakarcha udan aarambhichillenkil beetaa thalassemia mejar saadhaaranayaayi shaishavathil maarakamaan.

