{"id":10215,"date":"2020-04-23T04:59:53","date_gmt":"2020-04-23T01:59:53","guid":{"rendered":"https:\/\/www.synevo.ro\/product\/alfa-talasemie-testare-genetica-hba1\/"},"modified":"2024-10-31T10:19:58","modified_gmt":"2024-10-31T08:19:58","slug":"alfa-talasemie-testare-genetica-hba1","status":"publish","type":"product","link":"https:\/\/old.synevo.md\/ru\/shop\/alfa-talasemie-testare-genetica-hba1\/","title":{"rendered":"\u0410\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u044f - \u0433\u0435\u043d\u0435\u0442\u0438\u0447\u0435\u0441\u043a\u043e\u0435 \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u0435 (HBA1)"},"content":{"rendered":"

\u041e\u0431\u0449\u0430\u044f \u0438\u043d\u0444\u043e\u0440\u043c\u0430\u0446\u0438\u044f<\/em><\/strong><\/p>\n

Alfa-talasemiile reprezinta un grup de afectiuni cu transmitere genetica autozomal-recesiva caracterizate printr-o rata \u00a0scazuta de sinteza a lanturilor \u03b1 ale globinei.<\/p>\n

Clusterul genelor \u03b1 este localizat in apropierea telomerului cromozomului 16, se intinde pe o lungime de 28 kb si contine in urmatoarea ordine: o gena zeta (\u03b6), o pseudogena \u03b6 (\u03c8\u03b6), o pseudogena \u03b1 (\u03c8\u03b1) si doua gene \u03b1, care codifica \u03b12<\/sub>-globina \u00a0si \u03b11<\/sub>-globina, <\/sub>denumite HBA2 si respectiv HBA11<\/sup>. Fiecare gena prezinta 3 secvente care codifica proteine (exoni) si 2 regiuni care aparent nu au nici un rol in sinteza proteinelor (introni = intervening sequences), flancate de secvente 5\u2019 si\u00a0 3\u2019, care de asemenea nu intervin in procesul de translatie. La capatul 5\u2019 al fiecarei gene se gaseste promotorul<\/em>, o secventa necesara pentru initierea transcriptiei. Promotorul genei \u03b1 include 2 secvente: TATA box si CCATT box. Atat exonii cat si intronii sufera fenomenul de transcriptie a ADN-ului in ARN mesager; acest proces este controlat de factori de transcriptie care se leaga de promotori si de elementele de control situate in amonte<\/em> \u2013 intensificatori (enhancers) \u2013 care cresc foarte mult viteza de transcriptie. \u00a0Astfel, genele \u03b1-globinei se afla sub influenta unui element intensificator major, denumit HS-40 (vezi figura<\/em>)1;3<\/sup>.<\/p>\n

\"-\"<\/p>\n

Adaptare dupa J. Clin. Invest<\/em> 117(4): 850-858 (2007)<\/p>\n

Fenomenul de transcriptie include urmatoarele etape:<\/p>\n

- transcriptia propriu-zisa<\/em>: rezulta o copie ARN a genei, ce include secventele complementare atat pentru exoni cat si pentru introni;<\/p>\n

- incapsularea si poliadenilarea<\/em> (capping + polyadenilation): atasarea unor nucleotide speciale la capetele 5\u2019 si\u00a0 3\u2019 ale ARN-ului;<\/p>\n

- innadirea<\/em> (splicing): indepartarea intronilor din ARNm; secventele ARN corespunzatoare exonilor sunt apoi \u201cinnadite\u201d impreuna3<\/sup>.<\/p>\n

Alfa-talasemiile se produc atunci cand una sau mai multe gene care codifica lanturile \u03b1 ale globinei lipsesc sau sunt afectate. Severitatea defectului este variabila; una din extreme este conditia complet asimptomatica ce rezulta din deletia sau disfunctia uneia din cele 4 gene \u03b1; la polul opus este situat sindromul Hemoglobinei Bart<\/em><\/strong>, produs prin deletia celor 4 gene \u03b1, caracterizat prin hidrops fetal, de obicei incompatibil cu viata (edeme generalizate, efuziuni pleurale si pericardice, hepatosplenomegalie, anemie hipocroma severa in absenta unei incompatibilitati de grup sangvin si Rh).<\/p>\n

Unele din manifestarile clinico-patologice ale sindroamelor alfa-talasemice rezulta ca urmare a absentei lantului \u03b1, in timp ce altele se produc prin afectarea precursorilor eritropoietici de catre lanturile non-\u03b1 aflate in exces. Astfel, lanturile \u03b2 si \u03b3\u00a0 sintetizate in exces formeaza tetrameri, rezultand hemoglobinele H (\u03b24<\/sub>) si Bart (\u03b34<\/sub>).<\/p>\n

Atunci cand ambele gene \u03b1 de pe un singur cromozom sufera o deletie sau o inactivare completa a transcriptiei conditia rezultata este denumita \u03b10<\/sup> talasemie<\/em><\/strong> (alfa-zero talasemie); atunci cand se mentine o functie reziduala a genei si o anume productie a lantului \u03b1, de exemplu in cazul deletiei unei singure gene \u03b1 de pe un cromozom, se utilizeaza termenul de \u03b1+ talasemie<\/em><\/strong> (alfa-plus talasemie). Deletia sau disfunctia a trei gene \u03b1 genereaza boala HbH<\/em><\/strong>.<\/p>\n

Din punct de vedere al defectului genetic subiacent alfa-talasemiile sunt impartite in talasemii deletionale<\/em> si non-deletionale<\/em>. Talasemiile deletionale vor genera \u03b10<\/sup> talasemie sau \u03b1+ talasemie, in functie de natura si lungimea deletiei. Talasemiile nedeletionale conduc de obicei la \u03b1+ talasemie si rezulta prin:<\/p>\n

-mutatii ale codonului de initiere;<\/p>\n

-mutatii care afecteaza clivajul, innadirea, poliadenilarea sau stabilitatea ARN-ului;<\/p>\n

-productia unui lant \u03b1 structural anormal, care este fie sintetizat la o rata redusa, fie este instabil1<\/sup>.<\/p>\n

Testarea genetica<\/em><\/strong><\/p>\n

Testarea prin tehnici de biologie moleculara a demonstrat ca HBA1<\/sub> si HBA2<\/sub> prezinta deleti<\/span>i<\/em> (ca urmare a unor evenimente de recombinare aberanta) in 90% din cazuri si mutatii punctiforme<\/em> la aproximativ 10% dintre indivizii afectati.<\/p>\n

Metodele bazate pe reactia de polimerizare in lant<\/span> (PCR) ce utilizeaza primeri specifici care flancheaza punctele de ruptura (breakpoints), detecteaza deletia unei singure gene de \u03b1-globina<\/p>\n

(\u03b1+<\/sup>-talasemie) sau deletia ambelor gene, \u03b11<\/sub> si \u03b12,<\/sub> de pe un singur cromozom (\u03b10<\/sup>-talasemie). Prin tehnica MLPA<\/span> (multiple ligation-dependent probe amplification) se pot detecta deletii mai putin intalnite. Cele mai obisnuite deletii care afecteaza o singura gena \u03b1 sunt cele denumite -\u03b13.7<\/sup> si -\u03b14.2<\/sup>; in plus fata de acestea au mai fost raportate 3 deletii mai rare6<\/sup>. (-\u03b14.2 <\/sup>) este o deletie de 4.2 kb la nivelul genei \u03b12; <\/sub><\/p>\n

(-\u03b13.7<\/sup>) desemneaza un grup de 3 deletii usor diferite cu lungimea de 3.7 kb produse la capatul 3\u2019 al genei \u03b12\u00a0 <\/sub>si la capatul 3\u2019 al genei \u03b11 <\/sub>cu formarea unei gene de fuziune \u03b12<\/sub>\u03b11. <\/sub>Ambele deletii au ca rezultat reducerea cu 50% a sintezei lantului \u03b1 de pe cromozomul afectat1<\/sup>.<\/p>\n

In ceea ce priveste \u03b10<\/sup> talasemia, sunt cunoscute mai mult de 20 de deletii diferite, de la 6 kb la 300 kb, ce conduc la inlaturarea ambelor gene de \u03b1-globina (uneori si la deletia genei zeta). Cele mai cunoscute sunt \u00a0\u2013 \u2013SEA <\/sup>, \u2013 \u2013FIL<\/sup>, \u2013 \u2013THAI<\/sup>, \u2013 \u2013MED<\/sup>, \u2013 \u201320.5 <\/sup>care in cazul homozigotiei provoaca sindromul hemoglobinei Bart5<\/sup>. Atunci cand oricare dintre aceste alele apare in combinatie cu o alta alela purtatoare a deletiei unei singure gene \u03b1 (ca de exemplu, -\u03b13.7<\/sup>) rezulta boala Hb H, caracterizata prin prezenta tetramerului Hb Bart la nastere, inlocuit ulterior de tetramerul \u00a0Hb H2<\/sup>.<\/p>\n

Deletia elementului reglator major care include intensificatorul HS-40 induce scaderea marcata sau anularea expresiei ambelor gene \u03b1 cu structura normala (\u03b10<\/sup> talasemie)1<\/sup>.<\/p>\n

Tehnica de secventiere<\/span> este aplicata in cazurile in care nu a fost depistata nici o deletie a lanturilor \u03b1-globinei in prezenta suspiciunii clinice de alfa-talasemie.\u00a0 Pot fi detectate astfel mutatiile punctiforme:6<\/sup><\/p>\n

\u25cf Mutatiile codonului de terminalizare <\/em>interfera cu fenomenul de translatie a ARN-ului\u00a0 prin producerea unui lant \u03b1 instabil alungit. Cele mai cunoscute exemple sunt hemoglobina Constant Spring (intalnita in sudul Chinei, Tailanda, Cambodgia, Vietnam, Laos, bazinul mediteranean) si hemoglobina Koya Dora (India).<\/p>\n

\u25cf Mutatii care afecteaza clivajul, splicing-ul si poliadenilarea ARN-ului.<\/em> De exemplu IVSI-117(G\u2192A) la nivelul genei \u03b11<\/sub> este o mutatie a jonctiunii de splicing intalnita in India care conduce la o gena nefunctionala. \u03b1TSaudi<\/sup>\u03b1, intalnita in mod obisnuit in regiunea mediteraneana, este o mutatie a semnalului de poliadenilare<\/em> care conduce la o reducere marcata a sintezei lantului \u03b11<\/sup>.<\/p>\n

\u25cf Mutatii care determina instabilitatea posttranslationala a unui lant \u03b1 anormal<\/em>, de exemplu hemoglobina Quong Sze (\u03b1125Leu\u2192Pro<\/sup>\u03b1 sau \u03b1QS<\/sup>\u03b1), intalnita la evreii kurzi si in Asia de Sud-Est, si hemoglobina Agrinio (\u03b129Leu\u2192Pro<\/sup>\u03b1 sau \u03b1Agr<\/sup>\u03b1), intalnita in regiunea mediteraneana si Asia de sud-Est1<\/sup>.<\/p>\n

In zona mediteraneana \u03b1+ talasemia (cauzata de deletia -\u03b13.7<\/sup> ) este obisnuita, cu frecventa cea mai mare in Sardinia si cea mai scazuta in Spania. Pe de alta parte, \u03b10<\/sup> talasemia este foarte rara, ceea ce explica si incidenta extrem de scazuta a hidropsului fetal. O caracteristica importanta a \u03b1- talasemiei in populatia mediteraneana este heterogenicitatea mutatiilor, in special a celor nedeletionale6<\/sup>.<\/p>\n

Recomandari pentru testarea genetica<\/em><\/strong><\/p>\n

-confirmarea diagnosticului la persoanele cu suspiciune clinica;<\/p>\n

-depistarea statusului de purtator asimptomatic;<\/p>\n

-diagnosticul prenatal6<\/sup>.<\/p>\n

Specimen recoltat<\/em><\/strong> \u2013 sange venos4<\/sup>.<\/p>\n

Recipient de recoltare<\/em><\/strong>\u00a0\u2013 vacutainer ce contine EDTA ca anticoagulant4<\/sup>.<\/sub><\/p>\n

Cantitate recoltata <\/em><\/strong>\u2013 cat permite vacuumul4<\/sup><\/p>\n

Cauze de respingere<\/em><\/strong> a probei <\/em><\/strong>\u2013 folosirea heparinei ca anticoagulant; probe coagulate sau hemolizate4<\/sup>.<\/p>\n

Stabilitate proba<\/em><\/strong> - 7 zile<\/em> la 2-8\u00baC4<\/sup>.<\/p>\n

Metoda<\/em><\/strong> - secventiere completa HbA1<\/sub> si HbA2<\/sub>; analiza duplicatiilor\/deletiilor prin metoda MLPA<\/span>4<\/sup>.<\/p>\n

Interpretarea \u00a0rezultatelor<\/em><\/strong><\/p>\n

Alfa plus-talasemia <\/span><\/p>\n

Tara de \u03b1+ talasemie este cea mai frecventa afectiune monogenica din lume. Mutatiile cele mai obisnuite sunt sunt -\u03b13.7 <\/sup>si -\u03b14.2<\/sup>. Aproximativ un sfert din persoanele de origine africana sunt heterozigote pentru \u03b1+ talasemie (avand genotipul -\u03b13.7<\/sup>\/\u03b1\u03b1), in timp ce 1-2% sunt homozigoti (genotip -\u03b13.7<\/sup>\/-\u03b13.7<\/sup>). Alte grupuri etnice care pot asocia \u03b1+ talasemie deletionala sunt grecii, cipriotii, turcii, sarzii, libanezii, sauditii, indienii, tailandezii, filipinezii, indonezienii, melanezienii si polinezienii. Mai putin frecvent, \u03b1+ talasemia rezulta ca urmare a unei mutatii punctiforme a genei \u03b12<\/sub>, denumita \u03b1\u0423<\/sup>\u03b1 (intalnita in Orientul Mijlociu, dar si in Cipru si Sardinia). Alfa-plus talasemiile non-deletionale care afecteaza gena \u03b11<\/sub>, denumite \u03b1\u03b1\u0423<\/sup>, sunt forme mai usoare in comparatie cu \u03b1\u0423<\/sup>\u03b1, fiind de regula intermediare ca severitate intre -\u03b1\/\u03b1\u03b1 si \u2013 -\/\u03b1\u03b1. Anumite variante de lant \u03b1 pot da nastere fenotipului de \u03b1+ talasemie. Acestea includ: Hb Constant Spring, Hb Q, Hb Icaria, Hb Seal Rock si Hb Koya Dora. Cea mai obisnuita forma de \u03b1-talasemie nedeletionala este hemoglobina Constant Spring, cu o frecventa de 1-8% in Tailanda. Aceasta varianta este datorata unei mutatii a codonului de terminalizare al genei \u03b12<\/sub>, in urma careia se adauga la lantul \u03b1 o secventa de 31 aminoacizi; ARN-ul mesager este foarte instabil, iar rata sintezei lantului \u03b1 este redusa la aproximativ 1%.<\/p>\n

Alfa plus-talasemia este in majoritatea cazurilor nesemnificativa clinic, persoanele afectate fiind asimptomatice. Conditia prezinta insa semnificatie genetica deoarece heterozigotia compusa pentru \u03b1+ talasemia deletionala si \u03b10<\/sup> talasemie genereaza boala HbH. Homozigotii pentru formele mai severe de \u03b1+ talasemie nedeletionala (\u03b1\u0423<\/sup>\u03b1 \/ \u03b1\u0423<\/sup>\u03b1) pot prezenta de asemenea caracteristicile clinice ale bolii HbH. Homozigotia pentru hemoglobina Constant Spring conduce la o anomalie mai severa comparativ cu cea intalnita in statusul homozigot pentru \u03b1+ talasemie; pacientii prezinta de regula anemie, iar in unele cazuri pot sa apara icter moderat si hepatosplenomegalie.<\/p>\n

Heterozigotii pentru \u03b1+ talasemie (-\u03b1\/\u03b1\u03b1) pot prezenta fie un tablou hematologic normal, fie o anemie discreta, cu reducerea\u00a0 usoara a VEM si HEM. In medie, valoarea Hb este cu 1g\/dL mai mica decat la persoanele cu cele 4 gene \u03b1 intacte. Electroforeza Hb este normala, la fel ca si separarea fractiunilor prin cromatografie lichida sub inalta presiune (HPLC). Homozigotii pentru \u03b1+ talasemie (-\u03b1\/-\u03b1) prezinta de obicei un tablou hematologic similar cu cel intalnit in beta-talasemia minora. Heterozigotii pentru Hb Constant Spring prezinta un grad mai mare de anemie decat cel intalnit in tara de \u03b1+ talasemie, insa VEM nu este redus in mod proportional; punctatiile bazofile sunt de regula proeminente. Homozigotii pentru aceasta varianta sunt anemici, cu o reducere marcata a HEM; VEM este mai putin scazut datorita\u00a0 hidratarii celulare excesive (consecutiva afectarii membranei eritrocitare de catre lanturile \u03b1 si\u00a0 \u03b1CS<\/sup> oxidate).<\/p>\n

Diagnosticul tarei de alfa-talasemie este suspectat in conditiile unei microcitoze persistente care nu este explicata prin deficitul de fier sau prin existenta tarei de \u03b2 sau \u03b4\u03b2 talasemie. Numai testarea genetica poate stabili diagnosticul de certitudine.<\/p>\n

Alfa 0-talasemia<\/span><\/p>\n

Aceasta conditie rezulta de obicei prin deletia ambelor gene \u03b1; rareori se produce numai prin deletia genei \u03b11 <\/sub>si a secventelor in amonte<\/em> cu inactivarea genei \u03b12 <\/sub>restante. Este relativ comuna in sud-estul Chinei, precum si in Tailanda, Vietnam, Laos, Cambodgia, Malaezia si Filipine. Este intalnita cu o frecventa mai mica in zona mediteraneana (Grecia, Cipru, Turcia, Israel, Sardinia). Trei tipuri de deletie sunt mai frecvente in sud-estul Asiei: \u2013 \u2013SEA <\/sup>, \u2013 \u2013FIL<\/sup>, \u2013 \u2013THAI<\/sup> (ultimele doua constituie deletii mari care includ si gena \u03b6). In regiunea mediteraneana cea mai cunoscuta deletie este\u00a0 \u2013 \u2013MED<\/sup>, care nu include si gena \u03b6. In statusul heterozigot nu exista diferente ale tabloului clinic sau hematologic intre diferitele deletii care genereaza \u03b10<\/sup> talasemia.<\/p>\n

Tara de \u03b10<\/sup> talasemie conduce la o anemie foarte usoara insotita de un numar crescut de eritrocite si de reducerea indicilor VEM si HEM. Diagnosticul este suspectat in prezenta acestor modificari aparute la persoane cu origine etnica sugestiva si avand o electroforeza a Hb normala. Confirmarea se efectueaza prin testare genetica.<\/p>\n

Boala hemoglobinei H<\/span><\/p>\n

Este un sindrom clinic rezultat dintr-o varietate mare de anomalii genetice. Cauza cea mai obisnuita este heterozigotia compusa pentru \u03b1+ talasemie si \u03b10<\/sup> talasemie, cum ar fi \u2013 \u2013SEA<\/sup>\/-\u03b14.2 <\/sup>in sud-estul Asiei sau \u2013\u03b120.5<\/sup>\/-\u03b13.7<\/sup> ori \u2013 \u2013MED<\/sup> \/-\u03b13.7<\/sup> in regiunea mediteraneana. Un mecanism alternativ il constituie heterozigotia compusa pentru \u03b10<\/sup> talasemie si o \u03b1-talasemie nedeletionala, cum ar fi hemoglobina Constant Spring sau tipul Saudi (- \u2013SEA<\/sup>\/\u03b1CS<\/sup>\u03b1 sau \u2013 \u2013MED<\/sup>\/ \u03b1TSaudi<\/sup>\u03b1); mai este posibila si homozigotia pentru \u03b1-talasemia nedeletionala, in principal \u03b1TSaudi<\/sup>\u03b1\/ \u03b1TSaudi<\/sup>\u03b1.<\/p>\n

Boala HbH este caracterizata printr-o reducere semnificativa a ratei de sinteza a lanturilor \u03b1, avand drept rezultat o anemie microcitara hipocroma moderat-severa (Hb 7-10 g\/dL, VEM 50-65 fL, HEM 15-20 pg, CHEM 25-30 g\/dL) insotita de reticulocitoza. Electroforeza Hb sau HPLC indica prezenta hemoglobinei H cu migrare rapida, intr-un procent de 2-40% (de regula 8-10%). Procentul de HbA2<\/sub> este redus, iar cel de HbF poate fi usor crescut (1-3%). Pe frotiul de sange colorat supravital pot fi observate incluziile HbH \u00a0(precipitatele de lanturi \u03b2), intr-un procent ridicat de eritrocite (35-90%), cu aspect caracteristic de \u201eminge de golf\u201d. Atunci cand boala HbH este cauzata de \u03b1-talasemii nedeletionale, hemoglobina H nu este detectata intotdeauna pe electroforeza Hb, iar incluziile HbH sunt inconstante; aceasta se datoreaza probabil faptului ca lanturile \u03b2 formeaza impreuna cu un lant \u03b1 anormal dimeri tranzitorii si instabili care sunt distrusi rapid.<\/p>\n

Sindromul hemoglobinei Bart<\/span><\/p>\n

Homozigotia pentru \u03b10<\/sup> talasemie conduce la imposibilitatea sintezei lanturilor \u03b1, cu absenta producerii hemoglobinelor F, A sau A2<\/sub>. Aceasta conditie este de obicei incompatibila cu viata extrauterina.<\/p>\n

Deletia tuturor celor 4 gene \u03b1, dar cu una sau ambele gene \u03b6 intacte (- \u2013SEA<\/sup>\/- \u2013SEA<\/sup>, \u2013 \u2013MED<\/sup> \/- \u2013MED<\/sup>,<\/p>\n

\u2013 \u2013SEA<\/sup>\/- \u2013THAI<\/sup> sau \u2013 \u2013SEA<\/sup>\/- \u2013FIL<\/sup>) are drept rezultat hidropsul fetal al hemoglobinei Bart.\u00a0 Majoritatea hemoglobinei prezente este constituita de Hb Bart, ce este alcatuita din 4 tetrameri \u03b3, este instabila si incapabila sa furnizeze oxigen tesuturilor. Restul hemoglobinei este reprezentat de Hb Portland 2 (\u03b62<\/sub>\u03b32<\/sub>) care, fiind capabila sa livreze oxigen tesuturilor, reuseste sa mentina fatul in viata pana in trimestrul trei de sarcina. Se dezvolta o anemie severa insotita de hematopoieza extramedulara ce conduce la hepatosplenomegalie.<\/p>\n

Atunci cand exista o deletie mare ce include genele \u03b6 nu poate fi produsa nici o forma de hemoglobina functionala. Singurele Hb sintetizate sunt Hb Bart si un tetramer \u03b54<\/sub>. Moartea fatului se inregistreaza precoce in sarcina, cu avort consecutiv. Acest sindrom poate rezulta ca urmare a homozigotiei pentru \u2013 \u2013FIL <\/sup>sau \u2013 \u2013THAI<\/sup>.<\/p>\n

La electroforeza hemoglobinei si HPLC se constata Hb Bart intr-un procent de 70-100% si, uneori, cantitati mici de Hb Portland 1, Portland 2 si HbH. Cazurile produse ca urmare a homozigotiei pentru \u2013 \u2013FIL <\/sup>sau \u2013 \u2013THAI<\/sup> nu prezinta Hb Portland 1 sau 2.<\/p>\n

Datorita consecintelor letale asupra fatului, este esential ca aceasta conditie sa fie depistata precoce in sarcina prin identificarea tuturor cuplurilor purtatoare ale tarei de \u03b10<\/sup> talasemie. Atunci cand ambii parinti prezinta tara de \u03b10<\/sup> talasemie este necesara analiza ADN-ului fetal. Pentru diagnostic se utilizeaza tesut fetal obtinut prin prelevarea de vilozitati coriale in primul trimestru sau din lichid amniotic in trimestrul doi de sarcina1<\/sup>.<\/p>\n

\u00a0<\/span><\/p>\n

\u0411\u0438\u0431\u043b\u0438\u043e\u0433\u0440\u0430\u0444\u0438\u044f<\/span><\/p>\n

1. Barbara J. Bain. Haemoglobinopathy Diagnosis. Blackwell Science, 2001, 7-9; 49-73.<\/span><\/p>\n

2. BridgesK R, Thalassemia Overview [Online]. Available from <\/span>http:\/\/sickle.bwh.harvard.edu\/menu_thal.html<\/span><\/a>. Reference Type: Internet Communication.<\/span><\/p>\n

3. Cornel Mironel Niculae. Transcrierea (transcriptia). Bioinformatica. fpce9.fizica.unibuc.ro\/bioinfo\/transcrierea.htm. Reference Type: Internet Communication.<\/span><\/p>\n

4. Laborator Synevo. Referintele specifice tehnologiei de lucru utilizate 2010. Ref Type: Catalog.<\/span><\/p>\n

5. Orathai Tangvarasittichai1, Chanthonrat Sitthiworanan, Warong Dechgitvigrom, Torpong Sanguansermsri, Rattima Jeenapongsa, Prevalence and Haematological Parameters of Thalassaemia in Lower Northern Thailand, In Haema 2005; 8(2): 241-244.<\/span><\/p>\n

6. Renzo Galanello, Antonio Cao. Alpha-Thalassemia. Gene Reviews, 2008. <\/span>www.ncbi.nlm.nih.gov<\/span><\/a>. ReferenceType: Internet Communication.<\/span><\/p>","protected":false},"excerpt":{"rendered":"

\u0410\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u044f \u2013 \u044d\u0442\u043e \u0433\u0435\u043d\u0435\u0442\u0438\u0447\u0435\u0441\u043a\u043e\u0435 \u0437\u0430\u0431\u043e\u043b\u0435\u0432\u0430\u043d\u0438\u0435, \u0445\u0430\u0440\u0430\u043a\u0442\u0435\u0440\u0438\u0437\u0443\u044e\u0449\u0435\u0435\u0441\u044f \u0440\u0430\u0437\u043b\u0438\u0447\u043d\u044b\u043c\u0438 \u043d\u0430\u0440\u0443\u0448\u0435\u043d\u0438\u044f\u043c\u0438 \u0432 \u0433\u0435\u043d\u0430\u0445 HBA1 \u0438 HBA2, \u043a\u043e\u0434\u0438\u0440\u0443\u044e\u0449\u0438\u043c\u0438 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d \u0410 (HbA, \u043e\u0441\u043d\u043e\u0432\u043d\u043e\u0439 \u043a\u043e\u043c\u043f\u043e\u043d\u0435\u043d\u0442 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 \u0432\u0437\u0440\u043e\u0441\u043b\u043e\u0433\u043e \u0447\u0435\u043b\u043e\u0432\u0435\u043a\u0430), \u0438 \u043f\u0440\u043e\u044f\u0432\u043b\u044f\u044e\u0449\u0435\u0435\u0441\u044f \u0430\u043d\u0435\u043c\u0438\u0435\u0439 \u0440\u0430\u0437\u043b\u0438\u0447\u043d\u043e\u0439 \u0442\u044f\u0436\u0435\u0441\u0442\u0438.<\/p>\n

\u0412 \u043d\u043e\u0440\u043c\u0435 \u0443 \u0432\u0437\u0440\u043e\u0441\u043b\u043e\u0433\u043e \u0447\u0435\u043b\u043e\u0432\u0435\u043a\u0430 \u0432 \u043a\u0440\u043e\u0432\u0438 \u043f\u0440\u0438\u0441\u0443\u0442\u0441\u0442\u0432\u0443\u0435\u0442 \u0442\u0440\u0438 \u0440\u0430\u0437\u043b\u0438\u0447\u043d\u044b\u0445 \u0444\u043e\u0440\u043c\u044b \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430: HbA (2 \u0430\u043b\u044c\u0444\u0430- \u0438 2 \u0431\u0435\u0442\u0430- \u0446\u0435\u043f\u0438), HbA2 (2 \u0430\u043b\u044c\u0444\u0430- \u0438 2 \u0434\u0435\u043b\u044c\u0442\u0430-) \u0438 HbF (2 \u0430\u043b\u044c\u0444\u0430- \u0438 2 \u0433\u0430\u043c\u043c\u0430-). \u0421\u0438\u043d\u0442\u0435\u0437 \u0430\u043b\u044c\u0444\u0430-\u0446\u0435\u043f\u0435\u0439, \u044f\u0432\u043b\u044f\u044e\u0449\u0438\u0445\u0441\u044f \u043a\u043e\u043c\u043f\u043e\u043d\u0435\u043d\u0442\u0430\u043c\u0438 \u043a\u0430\u0436\u0434\u043e\u0439 \u0438\u0437 \u043e\u0441\u043d\u043e\u0432\u043d\u044b\u0445 \u0444\u043e\u0440\u043c \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 \u0432\u0437\u0440\u043e\u0441\u043b\u043e\u0433\u043e \u0447\u0435\u043b\u043e\u0432\u0435\u043a\u0430, \u043e\u0441\u0443\u0449\u0435\u0441\u0442\u0432\u043b\u044f\u0435\u0442\u0441\u044f \u043f\u043e\u0434 \u043a\u043e\u043d\u0442\u0440\u043e\u043b\u0435\u043c \u0433\u0435\u043d\u043e\u0432 HBA1 \u0438 HBA2. \u0412 \u0441\u0432\u044f\u0437\u0438 \u0441\u043e \u0441\u043d\u0438\u0436\u0435\u043d\u043d\u043e\u0439 \u043f\u0440\u043e\u0434\u0443\u043a\u0446\u0438\u0435\u0439 \u0430\u043b\u044c\u0444\u0430-\u0446\u0435\u043f\u0435\u0439 \u043f\u0440\u043e\u0438\u0441\u0445\u043e\u0434\u0438\u0442 \u043a\u043e\u043c\u043f\u0435\u043d\u0441\u0430\u0442\u043e\u0440\u043d\u0430\u044f \u0433\u0438\u043f\u0435\u0440\u043f\u0440\u043e\u0434\u0443\u043a\u0446\u0438\u044f \u0433\u0430\u043c\u043c\u0430-\u0446\u0435\u043f\u0435\u0439. \u0412 \u0434\u0435\u0442\u0441\u043a\u043e\u043c \u0432\u043e\u0437\u0440\u0430\u0441\u0442\u0435 \u044d\u0442\u043e \u0432\u0435\u0434\u0435\u0442 \u043a \u043e\u0431\u0440\u0430\u0437\u043e\u0432\u0430\u043d\u0438\u044e \u0442\u0435\u0442\u0440\u0430\u043c\u0435\u0440\u043e\u0432 \u0438\u0437 \u0433\u0430\u043c\u043c\u0430-\u0446\u0435\u043f\u0435\u0439 (\u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d \u0411\u0430\u0440\u0442\u0430, HbBart\u2019s), \u0430 \u0431\u0435\u0442\u0430-\u0442\u0435\u0442\u0440\u0430\u043c\u0435\u0440\u043e\u0432 \u2013 \u0438\u0437 \u0431\u0435\u0442\u0430-\u0446\u0435\u043f\u0435\u0439 (\u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d H, HbH). \u0421\u0432\u043e\u0431\u043e\u0434\u043d\u044b\u0435 \u0446\u0435\u043f\u0438, \u043d\u0435 \u0432\u043e\u0448\u0435\u0434\u0448\u0438\u0435 \u0432 \u0441\u043e\u0441\u0442\u0430\u0432 \u043d\u043e\u0440\u043c\u0430\u043b\u044c\u043d\u044b\u0445 \u0444\u043e\u0440\u043c \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430, \u043e\u0431\u0440\u0430\u0437\u0443\u044e\u0442 \u043f\u0440\u0435\u0446\u0438\u043f\u0438\u0442\u0430\u0442\u044b, \u043a\u043e\u0442\u043e\u0440\u044b\u0435 \u043e\u0442\u043a\u043b\u0430\u0434\u044b\u0432\u0430\u044e\u0442\u0441\u044f \u0432 \u0441\u043e\u0437\u0440\u0435\u0432\u0430\u044e\u0449\u0438\u0445 \u044d\u0440\u0438\u0442\u0440\u043e\u0446\u0438\u0442\u0430\u0445 \u0432 \u043a\u043e\u0441\u0442\u043d\u043e\u043c \u043c\u043e\u0437\u0433\u0435. \u041f\u0430\u0442\u043e\u043b\u043e\u0433\u0438\u0447\u0435\u0441\u043a\u0438\u0435 \u0432\u0430\u0440\u0438\u0430\u043d\u0442\u044b \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 \u043d\u0435 \u0441\u043f\u043e\u0441\u043e\u0431\u043d\u044b \u0430\u0434\u0435\u043a\u0432\u0430\u0442\u043d\u043e \u0432\u044b\u043f\u043e\u043b\u043d\u044f\u0442\u044c \u0444\u0443\u043d\u043a\u0446\u0438\u044e \u043f\u0435\u0440\u0435\u043d\u043e\u0441\u0430 \u043a\u0438\u0441\u043b\u043e\u0440\u043e\u0434\u0430, \u043a\u0440\u043e\u043c\u0435 \u0442\u043e\u0433\u043e, \u0438\u0445 \u043f\u0440\u0438\u0441\u0443\u0442\u0441\u0442\u0432\u0438\u0435 \u0432\u0435\u0434\u0435\u0442 \u043a \u043f\u0440\u0435\u0436\u0434\u0435\u0432\u0440\u0435\u043c\u0435\u043d\u043d\u043e\u043c\u0443 \u0440\u0430\u0437\u0440\u0443\u0448\u0435\u043d\u0438\u044e \u043d\u043e\u0440\u043c\u0430\u043b\u044c\u043d\u044b\u0445 \u044d\u0440\u0438\u0442\u0440\u043e\u0446\u0438\u0442\u043e\u0432. \u042d\u0442\u0438 \u0444\u0430\u043a\u0442\u043e\u0440\u044b \u0432 \u0441\u043e\u0432\u043e\u043a\u0443\u043f\u043d\u043e\u0441\u0442\u0438 \u0432\u0435\u0434\u0443\u0442 \u043a \u043d\u0430\u0440\u0443\u0448\u0435\u043d\u0438\u044e \u044d\u0440\u0438\u0442\u0440\u043e\u043f\u043e\u044d\u0437\u0430 \u0438 \u0444\u043e\u0440\u043c\u0438\u0440\u043e\u0432\u0430\u043d\u0438\u044e \u0433\u0435\u043c\u043e\u043b\u0438\u0442\u0438\u0447\u0435\u0441\u043a\u043e\u0439 \u0430\u043d\u0435\u043c\u0438\u0438. \u0414\u043b\u044f \u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438 \u0445\u0430\u0440\u0430\u043a\u0442\u0435\u0440\u043d\u0430 \u0433\u0438\u043f\u043e\u0445\u0440\u043e\u043c\u043d\u0430\u044f \u043c\u0438\u043a\u0440\u043e\u0446\u0438\u0442\u0430\u0440\u043d\u0430\u044f \u0430\u043d\u0435\u043c\u0438\u044f, \u0441\u043d\u0438\u0436\u0435\u043d\u043d\u044b\u0439 MCH-\u043f\u043e\u043a\u0430\u0437\u0430\u0442\u0435\u043b\u044c, \u043d\u043e\u0440\u043c\u0430\u043b\u044c\u043d\u044b\u0439 \u0438\u043b\u0438 \u043f\u043e\u043d\u0438\u0436\u0435\u043d\u043d\u044b\u0439 \u0443\u0440\u043e\u0432\u0435\u043d\u044c HbA2 \u0438 HbF \u0438 \u043d\u0430\u043b\u0438\u0447\u0438\u0435 HbH \u0432 \u0440\u0430\u0437\u043d\u043e\u043c \u043a\u043e\u043b\u0438\u0447\u0435\u0441\u0442\u0432\u0435 \u0432 \u0437\u0430\u0432\u0438\u0441\u0438\u043c\u043e\u0441\u0442\u0438 \u043e\u0442 \u0444\u043e\u0440\u043c\u044b \u0437\u0430\u0431\u043e\u043b\u0435\u0432\u0430\u043d\u0438\u044f.<\/p>\n

\u0414\u043b\u044f \u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438 \u0432 \u0431\u043e\u043b\u044c\u0448\u0435\u0439 \u0441\u0442\u0435\u043f\u0435\u043d\u0438 \u0445\u0430\u0440\u0430\u043a\u0442\u0435\u0440\u043d\u044b \u0434\u0435\u043b\u0435\u0446\u0438\u0438 \u0432 \u0433\u0435\u043d\u0430\u0445 HBA1 \u0438\u043b\u0438 HBA2, \u0430 \u0442\u0430\u043a\u0436\u0435 \u0434\u0435\u043b\u0435\u0446\u0438\u0438 \u0438\u043b\u0438 \u0442\u043e\u0447\u0435\u0447\u043d\u044b\u0435 \u043c\u0443\u0442\u0430\u0446\u0438\u0438 \u0432 \u0440\u0435\u0433\u0443\u043b\u044f\u0442\u043e\u0440\u043d\u044b\u0445 \u0440\u0435\u0433\u0438\u043e\u043d\u0430\u0445 \u044d\u0442\u0438\u0445 \u0433\u0435\u043d\u043e\u0432 (\u043d\u0430\u043f\u0440\u0438\u043c\u0435\u0440, \u0432 HS-40 \u0438\u043b\u0438 Constant Spring). \u0412 \u0437\u0430\u0432\u0438\u0441\u0438\u043c\u043e\u0441\u0442\u0438 \u043e\u0442 \u0442\u043e\u0433\u043e, \u043a\u0430\u043a\u043e\u0435 \u043a\u043e\u043b\u0438\u0447\u0435\u0441\u0442\u0432\u043e \u0433\u0435\u043d\u043e\u0432 \u0438\u0437 4 (2 HBA1 \u0438 2 HBA2) \u043e\u0441\u0442\u0430\u043b\u0438\u0441\u044c \u0444\u0443\u043d\u043a\u0446\u0438\u043e\u043d\u0430\u043b\u044c\u043d\u044b\u043c\u0438, \u0432\u044b\u0434\u0435\u043b\u044f\u044e\u0442 4 \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u0438\u0445 \u0444\u043e\u0440\u043c\u044b \u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438: \u0431\u0435\u0441\u0441\u0438\u043c\u043f\u0442\u043e\u043c\u043d\u043e\u0435 \u043d\u043e\u0441\u0438\u0442\u0435\u043b\u044c\u0441\u0442\u0432\u043e, \u043c\u0430\u043b\u0430\u044f \u0444\u043e\u0440\u043c\u0430, HbH-\u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u043e\u043f\u0430\u0442\u0438\u044f \u0438 \u0431\u043e\u043b\u044c\u0448\u0430\u044f \u0444\u043e\u0440\u043c\u0430 \u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438, \u043a\u043e\u0442\u043e\u0440\u0430\u044f \u043f\u0440\u0438\u0432\u043e\u0434\u0438\u0442 \u043a \u0432\u043d\u0443\u0442\u0440\u0438\u0443\u0442\u0440\u043e\u0431\u043d\u043e\u0439 \u0433\u0438\u0431\u0435\u043b\u0438 \u043f\u043b\u043e\u0434\u0430 \u0438\u043b\u0438 \u0433\u0438\u0431\u0435\u043b\u0438 \u0432 \u043f\u0435\u0440\u0432\u044b\u0435 \u0447\u0430\u0441\u044b \u043f\u043e\u0441\u043b\u0435 \u0440\u043e\u0436\u0434\u0435\u043d\u0438\u044f (\u0432\u043e\u0434\u044f\u043d\u043a\u0430 \u043f\u043b\u043e\u0434\u0430, hydrops fetalis). \u0423\u0432\u0435\u043b\u0438\u0447\u0435\u043d\u0438\u0435 \u0447\u0438\u0441\u043b\u0430 \u043a\u043e\u043f\u0438\u0439 \u0433\u0435\u043d\u043e\u0432 HBA1 \u0438 HBA2, \u043a\u0430\u043a \u043f\u0440\u0430\u0432\u0438\u043b\u043e, \u043d\u0435 \u0441\u043e\u043f\u0440\u044f\u0436\u0435\u043d\u043e \u0441 \u0440\u0430\u0437\u0432\u0438\u0442\u0438\u0435\u043c \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u043e\u0439 \u043a\u0430\u0440\u0442\u0438\u043d\u044b, \u043e\u0434\u043d\u0430\u043a\u043e \u043f\u0440\u0438 \u0441\u043e\u0447\u0435\u0442\u0430\u043d\u0438\u0438 \u0442\u0430\u043a\u043e\u0433\u043e \u0433\u0435\u043d\u043e\u0442\u0438\u043f\u0430 \u0441 \u0433\u0435\u0442\u0435\u0440\u043e\u0437\u0438\u0433\u043e\u0442\u043d\u044b\u043c\u0438 \u043d\u0430\u0440\u0443\u0448\u0435\u043d\u0438\u044f\u043c\u0438 \u0432 \u0433\u0435\u043d\u0435 HBB \u043c\u043e\u0436\u0435\u0442 \u0443\u0442\u044f\u0436\u0435\u043b\u044f\u0442\u044c \u0442\u0435\u0447\u0435\u043d\u0438\u0435 \u0431\u0435\u0442\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438.<\/p>\n

\u0414\u043b\u044f \u0447\u0435\u0433\u043e \u0438\u0441\u043f\u043e\u043b\u044c\u0437\u0443\u0435\u0442\u0441\u044f \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u0435?<\/strong><\/p>\n

    \n
  1. \u0414\u0438\u0430\u0433\u043d\u043e\u0441\u0442\u0438\u043a\u0430 \u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438.<\/li>\n
  2. \u0412\u044b\u044f\u0432\u043b\u0435\u043d\u0438\u0435 \u043f\u0440\u0438\u0447\u0438\u043d \u043d\u0430\u0440\u0443\u0448\u0435\u043d\u0438\u044f \u0441\u043e\u043e\u0442\u043d\u043e\u0448\u0435\u043d\u0438\u044f \u0440\u0430\u0437\u043b\u0438\u0447\u043d\u044b\u0445 \u0442\u0438\u043f\u043e\u0432 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 \u0438 \u043f\u043e\u044f\u0432\u043b\u0435\u043d\u0438\u044f \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 H.<\/li>\n
  3. \u0414\u0438\u0444\u0444\u0435\u0440\u0435\u043d\u0446\u0438\u0430\u043b\u044c\u043d\u0430\u044f \u0434\u0438\u0430\u0433\u043d\u043e\u0441\u0442\u0438\u043a\u0430 \u043f\u0440\u0438\u0447\u0438\u043d \u0433\u0435\u043c\u043e\u043b\u0438\u0442\u0438\u0447\u0435\u0441\u043a\u0438\u0445 \u0430\u043d\u0435\u043c\u0438\u0439.<\/li>\n
  4. \u0412\u0435\u0440\u0438\u0444\u0438\u043a\u0430\u0446\u0438\u044f \u043f\u0440\u0438\u0447\u0438\u043d \u0445\u0440\u043e\u043d\u0438\u0447\u0435\u0441\u043a\u043e\u0439 \u0430\u043d\u0435\u043c\u0438\u0438 (Hb < 110 \u0433\/\u043b, MCV < 80 \u0444\u043b, MCH < 27 \u043f\u0433).<\/li>\n
  5. \u0412\u0435\u0440\u0438\u0444\u0438\u043a\u0430\u0446\u0438\u044f \u043f\u0440\u0438\u0447\u0438\u043d \u044d\u0440\u0438\u0442\u0440\u043e\u0446\u0438\u0442\u043e\u0437\u043e\u0432.<\/li>\n
  6. \u0414\u0438\u0444\u0444\u0435\u0440\u0435\u043d\u0446\u0438\u0430\u043b\u044c\u043d\u0430\u044f \u0434\u0438\u0430\u0433\u043d\u043e\u0441\u0442\u0438\u043a\u0430 \u043f\u0435\u0440\u0435\u0433\u0440\u0443\u0437\u043a\u0438 \u043e\u0440\u0433\u0430\u043d\u0438\u0437\u043c\u0430 \u0436\u0435\u043b\u0435\u0437\u043e\u043c.<\/li>\n
  7. \u041e\u0446\u0435\u043d\u043a\u0430 \u0440\u0438\u0441\u043a\u0430 \u0440\u0430\u0437\u0432\u0438\u0442\u0438\u044f \u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438 \u043f\u0440\u0438 \u043d\u0430\u043b\u0438\u0447\u0438\u0438 \u0431\u043b\u0438\u0437\u043a\u0438\u0445 \u0440\u043e\u0434\u0441\u0442\u0432\u0435\u043d\u043d\u0438\u043a\u043e\u0432 \u0441 \u0432\u044b\u044f\u0432\u043b\u0435\u043d\u043d\u044b\u043c \u0437\u0430\u0431\u043e\u043b\u0435\u0432\u0430\u043d\u0438\u0435\u043c.<\/li>\n<\/ol>\n

    \u0427\u0442\u043e \u043e\u0437\u043d\u0430\u0447\u0430\u044e\u0442 \u0440\u0435\u0437\u0443\u043b\u044c\u0442\u0430\u0442\u044b?<\/strong><\/p>\n

    \u0418\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u0435 \u0438\u043d\u0442\u0435\u0440\u043f\u0440\u0435\u0442\u0438\u0440\u0443\u0435\u0442\u0441\u044f \u043d\u0430 \u043e\u0441\u043d\u043e\u0432\u0430\u043d\u0438\u0438 \u043a\u043e\u043b\u0438\u0447\u0435\u0441\u0442\u0432\u0430 \u043a\u043e\u043f\u0438\u0439 \u0433\u0435\u043d\u043e\u0432 HBA1 \u0438 HBA2 (\u0432 \u043d\u043e\u0440\u043c\u0435 \u0438\u0445 4 \u2013 \u0433\u0435\u043d\u043e\u0442\u0438\u043f aa\/aa) \u0438 \u043d\u0430\u043b\u0438\u0447\u0438\u0438 \u0430\u0431\u0435\u0440\u0440\u0430\u0446\u0438\u0439 \u0432 \u0440\u0435\u0433\u0443\u043b\u044f\u0442\u043e\u0440\u043d\u044b\u0445 \u0440\u0435\u0433\u0438\u043e\u043d\u0430\u0445 HS-40 \u0438\u043b\u0438 Constant Spring.<\/p>\n

    \u041e\u0431\u043d\u0430\u0440\u0443\u0436\u0435\u043d\u0438\u0435 3 \u043a\u043e\u043f\u0438\u0439 \u0433\u0435\u043d\u0430 (\u0433\u0435\u043d\u043e\u0442\u0438\u043f \u0430\u0430\/a-) \u0441\u0432\u0438\u0434\u0435\u0442\u0435\u043b\u044c\u0441\u0442\u0432\u0443\u0435\u0442 \u043e \u0431\u0435\u0441\u0441\u0438\u043c\u043f\u0442\u043e\u043c\u043d\u043e\u043c \u043d\u043e\u0441\u0438\u0442\u0435\u043b\u044c\u0441\u0442\u0432\u0435 \u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438 \u0438, \u043a\u0430\u043a \u043f\u0440\u0430\u0432\u0438\u043b\u043e, \u043d\u0435 \u0430\u0441\u0441\u043e\u0446\u0438\u0438\u0440\u043e\u0432\u0430\u043d\u043e \u0441 \u0440\u0430\u0437\u0432\u0438\u0442\u0438\u0435\u043c \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u043e\u0439 \u043a\u0430\u0440\u0442\u0438\u043d\u044b \u0430\u043d\u0435\u043c\u0438\u0438.<\/p>\n

    \u041e\u0431\u043d\u0430\u0440\u0443\u0436\u0435\u043d\u0438\u0435 2 \u043a\u043e\u043f\u0438\u0439 \u0433\u0435\u043d\u0430 (\u0433\u0435\u043d\u043e\u0442\u0438\u043f aa\/– \u0438\u043b\u0438 a-\/a-) \u0441\u043e\u043f\u0440\u044f\u0436\u0435\u043d\u043e \u0441 \u0440\u0430\u0437\u0432\u0438\u0442\u0438\u0435\u043c \u043c\u0430\u043b\u043e\u0439 \u0444\u043e\u0440\u043c\u044b \u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438, \u043c\u0438\u043a\u0440\u043e\u0446\u0438\u0442\u0430\u0440\u043d\u043e\u0439 \u0430\u043d\u0435\u043c\u0438\u0438 \u043b\u0451\u0433\u043a\u043e\u0439 \u0438\u043b\u0438 \u0443\u043c\u0435\u0440\u0435\u043d\u043d\u043e\u0439 \u0441\u0442\u0435\u043f\u0435\u043d\u0438 \u0442\u044f\u0436\u0435\u0441\u0442\u0438.<\/p>\n

    \u041e\u0431\u043d\u0430\u0440\u0443\u0436\u0435\u043d\u0438\u0435 1 \u043a\u043e\u043f\u0438\u0438 \u0433\u0435\u043d\u0430 (\u0433\u0435\u043d\u043e\u0442\u0438\u043f a-\/–) \u0430\u0441\u0441\u043e\u0446\u0438\u0438\u0440\u043e\u0432\u0430\u043d\u043e \u0441 \u0442\u044f\u0436\u0435\u043b\u043e\u0439 \u0444\u043e\u0440\u043c\u043e\u0439 \u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438, \u043d\u0430\u043b\u0438\u0447\u0438\u0435\u043c \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 H \u0438 \u0432 \u043d\u0435\u043a\u043e\u0442\u043e\u0440\u044b\u0445 \u0441\u043b\u0443\u0447\u0430\u044f\u0445 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 \u0411\u0430\u0440\u0442\u0430.<\/p>\n

    \u0423\u0432\u0435\u043b\u0438\u0447\u0435\u043d\u0438\u0435 \u0447\u0438\u0441\u043b\u0430 \u043a\u043e\u043f\u0438\u0439 HBA1\/HBA2 (\u0433\u0435\u043d\u043e\u0442\u0438\u043f \u0430\u0430\u0430\/\u0430\u0430), \u043a\u0430\u043a \u043f\u0440\u0430\u0432\u0438\u043b\u043e, \u043d\u0435 \u0432\u0435\u0434\u0435\u0442 \u043a \u0440\u0430\u0437\u0432\u0438\u0442\u0438\u044e \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u043e\u0439 \u043a\u0430\u0440\u0442\u0438\u043d\u044b \u0430\u043d\u0435\u043c\u0438\u0438, \u043d\u043e \u043c\u043e\u0436\u0435\u0442 \u0441\u043b\u0443\u0436\u0438\u0442\u044c \u043e\u0442\u044f\u0433\u043e\u0449\u0430\u044e\u0449\u0438\u043c \u0444\u0430\u043a\u0442\u043e\u0440\u043e\u043c \u0442\u0435\u0447\u0435\u043d\u0438\u044f \u0431\u0435\u0442\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438, \u0432 \u0441\u0432\u044f\u0437\u0438 \u0441 \u0447\u0435\u043c \u0442\u0430\u043a\u0438\u043c \u043f\u0430\u0446\u0438\u0435\u043d\u0442\u0430\u043c \u0440\u0435\u043a\u043e\u043c\u0435\u043d\u0434\u043e\u0432\u0430\u043d\u043e \u0441\u043e\u0432\u043c\u0435\u0441\u0442\u043d\u043e\u0435 \u043d\u0430\u0437\u043d\u0430\u0447\u0435\u043d\u0438\u0435 \u0433\u0435\u043d\u0435\u0442\u0438\u0447\u0435\u0441\u043a\u043e\u0433\u043e \u043e\u0431\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u044f \u043d\u0430 \u043e\u0431\u0435 \u0444\u043e\u0440\u043c\u044b \u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438.<\/p>\n

    \u041d\u0430\u043b\u0438\u0447\u0438\u0435 \u0434\u0435\u043b\u0435\u0446\u0438\u0439 \u0432 \u0440\u0435\u0433\u0438\u043e\u043d\u0435 HS-40 \u0438\u043b\u0438 \u0442\u043e\u0447\u0435\u0447\u043d\u044b\u0445 \u043c\u0443\u0442\u0430\u0446\u0438\u0439 \u0432 \u0440\u0435\u0433\u0438\u043e\u043d\u0435 Constant Spring \u0432\u0435\u0434\u0435\u0442 \u043a \u0444\u043e\u0440\u043c\u0438\u0440\u043e\u0432\u0430\u043d\u0438\u044e \u0444\u0435\u043d\u043e\u0442\u0438\u043f\u0430 \u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438.<\/p>\n

    \u041e\u0442\u0441\u0443\u0442\u0441\u0442\u0432\u0438\u0435 \u043a\u043e\u043b\u0438\u0447\u0435\u0441\u0442\u0432\u0435\u043d\u043d\u044b\u0445 \u0438 \u043a\u0430\u0447\u0435\u0441\u0442\u0432\u0435\u043d\u043d\u044b\u0445 \u0438\u0437\u043c\u0435\u043d\u0435\u043d\u0438\u0439 \u0432 \u0433\u0435\u043d\u0430\u0445 HBA \u0438 HBA2 \u0441\u0443\u0449\u0435\u0441\u0442\u0432\u0435\u043d\u043d\u043e \u0441\u043d\u0438\u0436\u0430\u0435\u0442 \u0432\u0435\u0440\u043e\u044f\u0442\u043d\u043e\u0441\u0442\u044c \u0434\u0438\u0430\u0433\u043d\u043e\u0437\u0430 “\u0430\u043b\u044c\u0444\u0430-\u0442\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u044f”. \u041e\u0434\u043d\u0430\u043a\u043e \u043d\u0435\u043e\u0431\u0445\u043e\u0434\u0438\u043c\u043e \u0443\u0447\u0438\u0442\u044b\u0432\u0430\u0442\u044c, \u0447\u0442\u043e \u0437\u0430\u0431\u043e\u043b\u0435\u0432\u0430\u043d\u0438\u0435 \u043c\u043e\u0436\u0435\u0442 \u0432\u044b\u0437\u044b\u0432\u0430\u0442\u044c\u0441\u044f \u0442\u043e\u0447\u0435\u0447\u043d\u044b\u043c\u0438 \u0430\u0431\u0435\u0440\u0440\u0430\u0446\u0438\u044f\u043c\u0438 \u0432 \u0438\u0441\u0441\u043b\u0435\u0434\u0443\u0435\u043c\u044b\u0445 \u0433\u0435\u043d\u0430\u0445, \u043a\u043e\u0442\u043e\u0440\u044b\u0435 \u043d\u0435 \u0432\u044b\u044f\u0432\u043b\u044f\u044e\u0442\u0441\u044f \u043f\u0440\u0438 \u0438\u0441\u043f\u043e\u043b\u044c\u0437\u043e\u0432\u0430\u043d\u0438\u0438 \u0434\u0430\u043d\u043d\u043e\u0433\u043e \u0442\u0435\u0441\u0442\u0430.<\/p>\n

    \u0427\u0442\u043e \u043c\u043e\u0436\u0435\u0442 \u0432\u043b\u0438\u044f\u0442\u044c \u043d\u0430 \u0440\u0435\u0437\u0443\u043b\u044c\u0442\u0430\u0442?<\/strong><\/p>\n

    \u041d\u0435\u0441\u043c\u043e\u0442\u0440\u044f \u043d\u0430 \u0442\u043e \u0447\u0442\u043e \u0433\u0435\u043d\u0435\u0442\u0438\u0447\u0435\u0441\u043a\u0438\u0435 \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u044f \u044f\u0432\u043b\u044f\u044e\u0442\u0441\u044f \u0442\u043e\u0447\u043d\u044b\u043c \u043c\u0435\u0442\u043e\u0434\u043e\u043c \u043b\u0430\u0431\u043e\u0440\u0430\u0442\u043e\u0440\u043d\u043e\u0439 \u0434\u0438\u0430\u0433\u043d\u043e\u0441\u0442\u0438\u043a\u0438, \u0432\u0440\u0435\u043c\u044f \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u0438\u0445 \u043f\u0440\u043e\u044f\u0432\u043b\u0435\u043d\u0438\u0439 \u0437\u0430\u0431\u043e\u043b\u0435\u0432\u0430\u043d\u0438\u044f (\u043f\u0435\u043d\u0435\u0442\u0440\u0430\u043d\u0442\u043d\u043e\u0441\u0442\u044c \u0431\u043e\u043b\u0435\u0437\u043d\u0438) \u0437\u0430\u0432\u0438\u0441\u044f\u0442 \u043e\u0442 \u0432\u043d\u0435\u0448\u043d\u0435\u0439 \u0441\u0440\u0435\u0434\u044b, \u0444\u043e\u0440\u043c\u044b \u0437\u0430\u0431\u043e\u043b\u0435\u0432\u0430\u043d\u0438\u044f \u0438 \u0438\u043d\u0434\u0438\u0432\u0438\u0434\u0443\u0430\u043b\u044c\u043d\u044b\u0445 \u0433\u0435\u043d\u0435\u0442\u0438\u0447\u0435\u0441\u043a\u0438\u0445 \u0444\u0430\u043a\u0442\u043e\u0440\u043e\u0432.<\/p>\n

    \u0412\u0430\u0436\u043d\u044b\u0435 \u0437\u0430\u043c\u0435\u0447\u0430\u043d\u0438\u044f<\/strong><\/p>\n

    \u0414\u043b\u044f \u043f\u043e\u043b\u0443\u0447\u0435\u043d\u0438\u044f \u0437\u0430\u043a\u043b\u044e\u0447\u0435\u043d\u0438\u044f \u043f\u043e \u0440\u0435\u0437\u0443\u043b\u044c\u0442\u0430\u0442\u0443 \u043e\u0431\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u044f \u043d\u0435\u043e\u0431\u0445\u043e\u0434\u0438\u043c\u043e \u043f\u0440\u043e\u043a\u043e\u043d\u0441\u0443\u043b\u044c\u0442\u0438\u0440\u043e\u0432\u0430\u0442\u044c\u0441\u044f \u0443 \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u043e\u0433\u043e \u0433\u0435\u043d\u0435\u0442\u0438\u043a\u0430.<\/p>","protected":false},"featured_media":0,"comment_status":"closed","ping_status":"closed","template":"","meta":[],"product_brand":[],"product_cat":[969],"product_tag":[],"class_list":{"0":"post-10215","1":"product","2":"type-product","3":"status-publish","5":"product_cat-teste-de-genetica-umana","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\/10215","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=10215"}],"wp:attachment":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/media?parent=10215"}],"wp:term":[{"taxonomy":"product_brand","embeddable":true,"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/product_brand?post=10215"},{"taxonomy":"product_cat","embeddable":true,"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/product_cat?post=10215"},{"taxonomy":"product_tag","embeddable":true,"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/product_tag?post=10215"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}