{"id":9222,"date":"2020-04-23T04:31:48","date_gmt":"2020-04-23T01:31:48","guid":{"rendered":"https:\/\/www.synevo.ro\/product\/electroforeza-hemoglobinei\/"},"modified":"2025-07-01T07:56:37","modified_gmt":"2025-07-01T04:56:37","slug":"electroforeza-hemoglobinei","status":"publish","type":"product","link":"https:\/\/old.synevo.md\/ru\/shop\/electroforeza-hemoglobinei\/","title":{"rendered":"\u042d\u043b\u0435\u043a\u0442\u0440\u043e\u0444\u043e\u0440\u0435\u0437 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430"},"content":{"rendered":"<h6><strong>* Electroforeza hemoglobinei se efectueaz\u0103 \u00eempreun\u0103 cu testele <a href=\"https:\/\/www.synevo.md\/shop\/viteza-de-sedimentare-a-hematiilor-vsh\/\" rel=\"nofollow noopener\" target=\"_blank\">Viteza de sedimentare a hematiilor(VSH)<\/a> \u0438 <a href=\"https:\/\/www.synevo.md\/shop\/hemograma-cu-formula-leucocitara-cu-hb-ht-si-indici\/\" rel=\"nofollow noopener\" target=\"_blank\">Hemograma cu formul\u0103 leucocitar\u0103 cu Hb, Ht \u0219i indici\u00a0<\/a><\/strong><\/h6>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\"><b><i>\u041e\u0431\u0449\u0430\u044f \u0438\u043d\u0444\u043e\u0440\u043c\u0430\u0446\u0438\u044f<\/i><\/b><b><\/b><\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Hemoglobina reprezinta elementul esential pentru principala functie fiziologica a eritrocitului: functia respiratorie. Hemoglobina este o cromoproteina porfirinica care contine fier, in structura sa intrand o parte proteica \u2013 globina \u2013 si o parte prostetica \u2013 hemul. Globina difera ca structura de la o hemoglobina la alta, ea imprimand specificitate diverselor hemoglobine cunoscute. <\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">In cursul dezvoltarii organismului de la embrion la adult se succeda mai multe tipuri de Hb; hemoglobinele embrionare Gower 1, Gower 2 si Portland sunt inlocuite incepand cu cea de-a 3-a luna de gestatie de catre Hb fetala (HbF), care reprezinta 70-80% din totalul Hb la nou-nascut, dupa care sinteza sa scade rapid, ajungand la sfarsitul primului an de viata la valori sub 2% din totalul Hb. Hb A si Hb A<sub>2<\/sub> sunt caracteristice perioadei adulte de dezvoltare; sinteza lor incepe inca\u00a0 din perioada fetala, iar dupa nastere ele inlocuiesc rapid Hb F. La adultul normal, Hb A reprezinta 97-98%, HbA<sub>2<\/sub> 2-3%, iar HbF sub 1%<sup>1<\/sup>.<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Lanturile polipeptidice \u03b1, \u03b2, \u03b3 si \u03b4 ale globinei alcatuiesc<i> <\/i>hemoglobinele fiziologice (fiecare molecula de hemoglobina contine doua perechi de lanturi polipeptidice), lantul \u03b1 fiind comun celor trei hemoglobine:<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\"><span style=\"color: #000000;\">HbA = \u03b12 \u03b22\u00a0 \u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\"><span style=\"color: #000000;\">HbA2 = \u03b12 \u03b42<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"font-size: medium;\"><span style=\"color: #000000;\">HbF = \u03b12 \u03b32.<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Ca si in cazul celorlalte proteine structura spatiala a hemoglobinei precum si alte proprietati moleculare depind de tipul si secventa de aminoacizi care intra in alcatuirea hemoglobinei. Substitutia unor aminoacizi din lanturile polipeptidice ca urmare a mutatiilor genetice genereaza <i>variante anormale de hemoglobina<\/i> ce prezinta o incarcare electrica diferita si in consecinta mobilitati electroforetice diferite. Pe de alta parte, scaderea ratei de sinteza a unui tip de lant al globinei da nastere la anomalii cantitative denumite <i>sindroame talasemice<\/i>.<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Metodele electroforetice sunt utilizate de rutina in screening-ul anomaliior hemoglobinei. Dintre acestea cea mai cunoscuta este electroforeza Hb in mediu alcalin (pH 8.2-8.6) folosind ca suport de migrare gelul de agaroza. La pH alcalin hemoglobina prezinta o sarcina electrica negativa si va migra catre anod. Prin aceasta metoda pot fi separate si identificate hemoglobinele fiziologice HbA, HBA2, HbF si migreaza de asemenea o serie de variante de hemoglobina, cum ar fi HbS, HbD, HbG, Hb Lepore, HbC, HbO-Arab, care genereaza majoritatea hemoglobinopatiilor cunoscute. <\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">La rularea fiecarui set de probe este folosit un control ce contine hemoglobinele A, F, S si C. Deoarece este utilizata o coloratie pentru proteine si nu una pentru hem, anhidraza carbonica (enzima cu un continut crescut in eritrocite) va fi vizualizata suplimentar in spatele benzii A2. Pentru identificarea fractiunilor Hb pozitia benzilor obtinute este comparata cu cea a benzilor standard furnizate de materialul de control pozitia benzilor obtinute fiind comparata cu cea a benzilor standard furnizate de materialul de control (<i>vezi fig.1<\/i>). Cuantificarea relativa a benzilor obtinute se face prin scanarea densitometrica a gelului electroforetic<sup>1;3<\/sup>.<\/span><\/span><\/p>\n<p><a class=\"shutterset_2613 cboxElement\" title=\"hb_1\" href=\"https:\/\/www.synevo.md\/wp-content\/uploads\/2009\/08\/hb_1.jpg\" rel=\"wpdevart_lightbox lightbox[2613] nofollow noopener\" data-rel=\"lightbox-gallery-juomPC5B\" data-rl_title=\"\" data-rl_caption=\"\" target=\"_blank\"><img decoding=\"async\" class=\"alignnone size-medium wp-image-13497\" src=\"\/wp-content\/uploads\/2009\/08\/hb_1.jpg\" alt=\"hb_1\" width=\"353\" height=\"404\" \/><\/a><b><i>\u00a0<\/i><\/b><\/p>\n<p><b><i>\u00a0<\/i><\/b><\/p>\n<p><em>Fig. 1 Migrarea fractiunilor Hb la electroforeza in gel de agaroza la pH alcaline<\/em><\/p>\n<p><b><i><span style=\"color: #000000; font-size: medium;\">\u00a0<\/span><\/i><\/b><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Cu toate acestea electroforeza hemoglobinei in gel de agaroza la pH alcalin prezinta limitari importante:<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"color: #000000;\"><span style=\"font-size: medium;\">-<\/span><span style=\"font-size: medium;\">variatii mari in cuantificarea HbA2, ceea ce afecteaza identificarea corecta a beta-talasemiilor;<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"color: #000000;\"><span style=\"font-size: medium;\">-<\/span> <span style=\"font-size: medium;\">imposibilitatea separarii HbS de HbD si a HbC de HbE si HbA2 (<i>vezi fig.1<\/i>).<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Din aceste motive in laboratorul nostru se utilizeaza in prezent <span style=\"text-decoration: underline;\">electroforeza capilara<\/span> pentru separarea fractiunilor Hb. Prin aceasta tehnica, complet automatizata, moleculele incarcate electric sunt separate pe baza mobilitatii electroforetice proprii intr-o solutie tampon alcalina. Separarea se produce de asemenea si in functie de pH-ul solutiei electrolitice si de fluxul electroosmotic.<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Sistemul electroforetic este alcatuit din tuburi capilare de silica (SiO2) cu d&lt;100 \u03bc care functioneaza in paralel ceea ce permite 7 analize simultane pentru cuantificarea unei fractiuni de Hb. Proba diluata cu solutia hemolizanta este injectata la capatul anodic al capilarului; se aplica apoi un voltaj inalt pentru separarea fractiunilor Hb care vor fi apoi detectate la 415 nm de catre detectorul de absorbanta plasat la capatul catodic al capilarului. Astfel, fractiunile Hb sunt detectate direct si specific la lungimea de unda caracteristica Hb. In final, pe ecranul analizorului vor fi afisate pozitiile potentiale ale fractiunilor Hb obtinute (identificate in zonele denumite Z1 \u2192Z15) precum si cuantificarea acestora. Prin utilizarea unui tampon cu pH alcalin fractiunile Hb normale sau anormale vor fi detectate in urmatoarea ordine de la catod la anod (Z1 \u2192Z15): \u03b4A\u20192 (varianta de HbA2), C, A2\/O-Arab, E, S, D, G-Philadelphia, F, A, Hope, Bart, J, N-Baltimore si H.<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Electroforegramele rezultate vor fi inspectate vizual pentru a stabili daca s-a obtinut un profil normal sau modificat (<i>vezi fig.2,3<\/i>).<\/span><\/span><\/p>\n<p><a class=\"shutterset_2613 cboxElement\" title=\"hb_2\" href=\"https:\/\/www.synevo.md\/wp-content\/uploads\/2009\/08\/hb_2.jpg\" rel=\"wpdevart_lightbox lightbox[2613] nofollow noopener\" data-rel=\"lightbox-gallery-juomPC5B\" data-rl_title=\"\" data-rl_caption=\"\" target=\"_blank\"><img decoding=\"async\" class=\"alignnone size-medium wp-image-13498\" src=\"\/wp-content\/uploads\/2009\/08\/hb_2.jpg\" alt=\"hb_2\" width=\"404\" height=\"345\" \/><\/a><\/p>\n<p><em>Fig. 2\u00a0 Electroforegrama Hb cu aspect normal (adult)<\/em><\/p>\n<p><b><i>\u00a0<\/i><\/b><\/p>\n<p><b><i>\u00a0<a class=\"shutterset_2613 cboxElement\" title=\"hb_3\" href=\"https:\/\/www.synevo.md\/wp-content\/uploads\/2009\/08\/hb_3.jpg\" rel=\"wpdevart_lightbox lightbox[2613] nofollow noopener\" data-rel=\"lightbox-gallery-juomPC5B\" data-rl_title=\"\" data-rl_caption=\"\" target=\"_blank\"><img decoding=\"async\" class=\"alignnone size-medium wp-image-13499\" src=\"\/wp-content\/uploads\/2009\/08\/hb_3.jpg\" alt=\"hb_3\" width=\"404\" height=\"354\" \/><\/a><\/i><\/b><\/p>\n<p><em>Fig. 3\u00a0 Electroforegrama Hb cu aspect normal (copil de 3 saptamani)<\/em><\/p>\n<p><b><i><span style=\"color: #000000; font-size: medium;\">\u00a0<\/span><\/i><\/b><span style=\"color: #000000; font-size: medium;\">\u00a0<\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Avantajele utilizarii electroforezei capilare sunt urmatoarele:<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"color: #000000;\"><span style=\"font-size: medium;\">-<\/span><span style=\"font-size: medium;\">cuantificare mai buna a HbA2 pentru identificarea beta-talasemiilor minore (datorita mai\u00a0<\/span><\/span><span style=\"font-size: medium;\"><span style=\"color: #000000;\">multor analize simultane);<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"color: #000000;\"><span style=\"font-size: medium;\">-<\/span><span style=\"font-size: medium;\">diferentierea HbS de HbD precum si a HbC de HbE si HbA2 (aceste fractiuni co-migreaza la electroforeza Hb in gel de agaroza in mediu alcalin);<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"color: #000000;\"><span style=\"font-size: medium;\">-<\/span><span style=\"font-size: medium;\">deoarece nu este vizualizata anhidraza carbonica pe eletroforegrama este posibila identificarea variantelor de HbA2 in zona Z1 (detectarea unei variante de HbA2 este importanta pentru diagnosticul corect al beta-talasemiilor minore deoarece se insumeaza la HbA2 normala)<sup>2<\/sup>.<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><b><i>Recomandari pentru efectuarea electroforezei de hemoglobina <\/i><\/b><i>- <\/i>diagnosticul hemoglobinopatiilor si sindroamelor talasemice a caror suspiciune este indicata de:<b><i><\/i><\/b><\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"color: #000000;\"><span style=\"font-size: medium;\">\u2022 anemie hemolitica cronica;<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"color: #000000;\"><span style=\"font-size: medium;\">\u2022 criza ocluziva vasculara de cauza necunoscuta la un pacient provenit din zone cu niveluri crescute ale Hb S si\/sau Hb C;<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"color: #000000;\"><span style=\"font-size: medium;\">\u2022 sindrom de hidrops fetal de cauza necunoscuta<sup>3<\/sup>;<\/span><\/span><\/p>\n<p style=\"padding-left: 30px;\"><span style=\"color: #000000;\"><span style=\"font-size: medium;\">\u2022 aspect sugestiv al hemogramei, cu microcitoza si hipocromie mai severe decat ar fi de asteptat pentru gradul anemiei si numar de eritrocite la limita superioara a normalului sau chiar crescut, asociate cu un numar mare de hematii \u201ein tinta\u201d si punctatii bazofile pe frotiul de sange. In aceasta situatie, pentru diferentierea de deficitul de fier, sunt utile indicele Mentzer si indicele RDW: <\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><i>Indicele Mentzer<\/i> = VEM\/nr. de eritrocite (x10<sup>6<\/sup>)<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\">\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 &gt;14 \u00a0\u00a0\u00a0\u00a0\u00a0 sugestiv pentru deficit de fier;<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\">\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 12-14\u00a0\u00a0\u00a0 echivoc<b><\/b><\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\">\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 &lt;12 \u00a0\u00a0\u00a0\u00a0\u00a0 sugestiv pentru tara talasemica.<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\">Indicele RDW = Indicele Mentzer x RDW (largirea distributiei eritrocitare).<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\">\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 &lt; 220:\u00a0\u00a0\u00a0 sugestiv pentru talasemie;<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\">\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 \u2265 220:\u00a0\u00a0\u00a0 sugestiv pentru anemia feripriva<sup>4<\/sup>.<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><b><i>Pregatire pacient <\/i><\/b>-<b><i> <\/i><\/b>\u00e0 jeun (pe nemancate) sau postprandial (dupa mese)<sup>2<\/sup>.<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><b><i>Specimen recoltat <\/i><\/b>-<b><i> <\/i><\/b>sange venos<sup>2<\/sup>.<b><i><\/i><\/b><\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><b><i>Cauze de respingerea probei<\/i><\/b><b> <\/b>-<b> <\/b>specimen coagulat sau care a depasit perioada de stabilitate<sup>2<\/sup>.<b><\/b><\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><b><i>Recipient de recoltare <\/i><\/b><i>- <\/i>vacutainer cu K3 EDTA<sup>2<\/sup>.<b><i><\/i><\/b><\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><b><i>Cantitate recoltata <\/i><\/b>\u2013 cat permite vacuumul<sup>2<\/sup>.<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><b><i>Stabilitate proba <\/i><\/b><i>-<b> <\/b><\/i>sangele integral este stabil <i>7 zile<\/i> la 2-8\u00b0C; in nici un caz vacutainerul cu sange recoltat pe K3 EDTA nu se introduce in congelator!!! (se produce hemoliza)<sup>2<\/sup>.<\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><b><i>Metoda<\/i><\/b><i> <\/i>-<b> <\/b><span style=\"text-decoration: underline;\">electroforeza capilara<\/span>; la fiecare rulare a probelor se utilizeaza un control HbA2 normal<sup>2<\/sup>.<b><i><\/i><\/b><\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\"><b><i>Valori de referinta<\/i><\/b> <\/span><\/span><\/p>\n<p><span style=\"color: #000000;\"><span style=\"font-size: medium;\">Pentru cele 3 fractiuni fiziologice se inregistreaza variatii in functie de varsta<sup>2<\/sup>:<\/span><\/span><\/p>\n<table class=\"wp-block-table w-100\">\n<tbody>\n<tr>\n<td width=\"174\"><strong>Varsta<\/strong><\/td>\n<td width=\"167\"><strong>Hb A %<\/strong><\/td>\n<td width=\"162\"><strong>Hb F %<\/strong><\/td>\n<td width=\"162\"><strong>Hb A2 %<\/strong><\/td>\n<\/tr>\n<tr>\n<td width=\"174\"><strong>&lt;1 luna<\/strong><\/td>\n<td width=\"167\">8.1 \u2013 62.7<\/td>\n<td width=\"162\">49.9 \u2013 98.4<\/td>\n<td width=\"162\">0.0 \u2013 1.8<\/td>\n<\/tr>\n<tr>\n<td width=\"174\"><strong>1 \u2013 3 luni<\/strong><\/td>\n<td width=\"167\">37.3 \u2013 88.8<\/td>\n<td width=\"162\">10.3 \u2013 64.6<\/td>\n<td width=\"162\">0.3 \u2013 2.4<\/td>\n<\/tr>\n<tr>\n<td width=\"174\"><strong>3 \u2013 6 luni<\/strong><\/td>\n<td width=\"167\">67.0 \u2013 96.7<\/td>\n<td width=\"162\">5.2 \u2013 34.5<\/td>\n<td width=\"162\">1.7 \u2013 2.7<\/td>\n<\/tr>\n<tr>\n<td width=\"174\"><strong>6 \u2013 9 luni<\/strong><\/td>\n<td width=\"167\">82.4 \u2013 98.8<\/td>\n<td width=\"162\">1.1 \u2013 28.5<\/td>\n<td width=\"162\">2.0 \u2013 3.0<\/td>\n<\/tr>\n<tr>\n<td width=\"174\"><strong>9 \u2013 15 luni<\/strong><\/td>\n<td width=\"167\">91.9 \u2013 98.7<\/td>\n<td width=\"162\">0.2 \u2013 9.9<\/td>\n<td width=\"162\">2.2 \u2013 3.2<\/td>\n<\/tr>\n<tr>\n<td width=\"174\"><strong>15 luni \u2013 2 ani<\/strong><\/td>\n<td width=\"167\">94.9 \u2013 97.8<\/td>\n<td width=\"162\">0.0 \u2013 5.5<\/td>\n<td width=\"162\">2.4 \u2013 3.1<\/td>\n<\/tr>\n<tr>\n<td width=\"174\"><strong>2 \u2013 6 ani<\/strong><\/td>\n<td width=\"167\">95.9 \u2013 97.9<\/td>\n<td width=\"162\">0.0 \u2013 1.6<\/td>\n<td width=\"162\">2.2 \u2013 3.2<\/td>\n<\/tr>\n<tr>\n<td width=\"174\"><strong>6 \u2013 17 ani<\/strong><\/td>\n<td width=\"167\">96.4 \u2013 98.2<\/td>\n<td width=\"162\">0.0 \u2013 0.0<\/td>\n<td width=\"162\">1.9 \u2013 3.3<\/td>\n<\/tr>\n<tr>\n<td width=\"174\"><strong>\u226518 ani<\/strong><\/td>\n<td width=\"167\">96.7 \u2013 97.8<\/td>\n<td width=\"162\">\u22640.5<\/td>\n<td width=\"162\">2.2 \u2013 3.2<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p><span style=\"color: #000000; font-size: medium;\">\u00a0<\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\"><b><i>Interpretarea rezultatelor<\/i><\/b><b><\/b><\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 in <i>\u03b2<\/i><i>-talasemia minima si minora<\/i> valorile\u00a0 Hb A<sub>2 <\/sub>\u00a0sunt crescute (3.2%- 7%), iar Hb F este usor crescuta (0.5-6%) in peste 50% din cazuri, restul fiind reprezentat de Hb A (<i>vezi fig.4<\/i>);<\/span><\/span><\/p>\n<p><a class=\"shutterset_2613 cboxElement\" title=\"hb_4\" href=\"https:\/\/www.synevo.md\/wp-content\/uploads\/2009\/08\/hb_4.jpg\" rel=\"wpdevart_lightbox lightbox[2613] nofollow noopener\" data-rel=\"lightbox-gallery-juomPC5B\" data-rl_title=\"\" data-rl_caption=\"\" target=\"_blank\"><img decoding=\"async\" class=\"alignnone size-medium wp-image-13500\" src=\"\/wp-content\/uploads\/2009\/08\/hb_4.jpg\" alt=\"hb_4\" width=\"404\" height=\"351\" \/><\/a><\/p>\n<p><em>Fig. 4\u00a0 Electroforegrama unui pacient cu beta-talasemie minor<\/em><\/p>\n<p><span style=\"color: #000000;\">\u00a0<\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 in <i>\u03b2<\/i><i>-talasemia intermedia<\/i>, procentul de Hb A<sub>2 <\/sub>este variabil, iar Hb F = 20-80%;<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 in <i>\u03b2<\/i><i>-talasemia majora<\/i>, valorile de\u00a0 Hb F sunt foarte crescute, situate obisnuit intre 20-90% din totalul Hb, restul fiind format de\u00a0 Hb A (valori foarte scazute) si HbA<sub>2 <\/sub>(valori normale sau crescute); se caracterizeaza clinic prin tabloul clasic al anemiei Cooley;<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 in afara de tipurile amintite, mai exista forme de \u03b2- talasemie cu valori normale de Hb A<sub>2<\/sub> si Hb F <\/span><\/span><span style=\"font-size: medium;\"><span style=\"color: #000000;\"><i>\u201csilent thalassemia<\/i>\u201d cu indici eritrocitari normali si <i>\u201calmost silent thalassemia<\/i>\u201d cu indici eritrocitari <\/span><\/span><span style=\"font-size: medium;\"><span style=\"color: #000000;\">modificati) care pot fi puse in evidenta prin tehnici de genetica moleculara<sup>3;5<\/sup>;<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\"><i>\u2022 \u03b4\u03b2-talasemia <\/i>rezulta din deletia genelor \u03b4<i> <\/i>si \u03b2 cu mentinerea integritatii genelor \u03b3, in timp ce in <i><sup>\u0410<\/sup>\u03b3\u03b4\u03b2-talasemia<\/i> se inregistreaza deletia genelor <sup>\u0410<\/sup>\u03b3<i>, <\/i>\u03b4<i> <\/i>si \u03b2 cu mentinerea integritatii genei <sup>\u0416<\/sup>\u03b3; heterozigotii prezinta un tablou hematologic similar \u03b2-talasemiei minore dar la electroforeza Hb, procentul de Hb A2 tinde sa fie redus la jumatate iar Hb F este persistent crescuta intr-o proportie de 5-20%; homozigotii prezinta un fenotip similar \u03b2-talasemiei intermedia iar electroforeza evidentiaza Hb F intr-un procent de aproape 100%; testarea genetica confirma diagnosticul<sup>3<\/sup>;<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 <i>Hb Lepore<\/i> este o hemoglobina anormala, in care lanturile \u03b1 sunt normale, iar lanturile non-\u03b1 au o structura asemanatoare lanturilor \u03b4 la capatul N-terminal si lanturilor \u03b2 la capatul C-terminal. Sindromul Lepore, foarte asemanator clinic in forma sa heterozigota cu\u00a0 \u03b2-talasemia minora, se caracterizeaza prin prezenta Hb Lepore intr-un procent de 5-10 %, cu o pozitie asemanatoare HbD la electroforeza capilara (in zona Z6); in forma homozigota, asemanatoare clinic cu anemia Cooley,\u00a0 Hb Lepore reprezinta 10-20%, restul fiind format de HbF (Hb A si Hb A<sub>2<\/sub> lipsesc in totalitate)<sup>1;2<\/sup>;<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\"><i>\u2022 persistenta ereditara de Hb F<\/i> este o entitate asimptomatica clinic si hematologic, caracterizata prin valori crescute de Hb F, la heterozigoti Hb F = 15-30%, iar la homozigoti se poate ajunge pana la 100% Hb F, in afara oricarui semn de boala<sup>5<\/sup>;<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 in <i>forma heterozigota de<\/i> <i>siclemie (\u201csickle cell trait\u201d)<\/i>, electroforeza capilara evidentiaza o Hb anormala in Z5, Hb S, in proportie de 25-45%, Hb A<sub>2<\/sub> fiind in cantitate normala, iar restul fiind reprezentat de Hb A (<i>vezi fig.5<\/i>); cei mai multi pacienti cu tara de siclemie sunt asimptomatici, cu un frotiu de sange normal sau usor modificat (microcitoza sau hematii \u201cin tinta\u201d); coexistenta tarei Hb S cu anumite tipuri de talasemii sau alte hemoglobinopatii modifica tabloul clinic si hematologic precum si electroforeza Hb<sup>2;3<\/sup>;<\/span><\/span><\/p>\n<p><a class=\"shutterset_2613 cboxElement\" title=\"hb_5\" href=\"https:\/\/www.synevo.md\/wp-content\/uploads\/2009\/08\/hb_5.jpg\" rel=\"wpdevart_lightbox lightbox[2613] nofollow noopener\" data-rel=\"lightbox-gallery-juomPC5B\" data-rl_title=\"\" data-rl_caption=\"\" target=\"_blank\"><img decoding=\"async\" class=\"alignnone size-medium wp-image-13501\" src=\"\/wp-content\/uploads\/2009\/08\/hb_5.jpg\" alt=\"hb_5\" width=\"404\" height=\"364\" \/><\/a><\/p>\n<p><em>Fig. 5 Eletroforegrama unui pacient cu forma heterozigota de siclemie<\/em><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 in <i>forma homozigota de siclemie (\u201csickle cell anemia\u201d)<\/i>, electroforeza Hb indica prezenta Hb S in proportie de 90-95%, restul fiind reprezentat de Hb A2 si Hb F; tabloul hematologic este normal la nastere si incepe sa se modifice dupa varsta de 6 luni, pe masura ce Hb F este inlocuita de Hb S<sup>3<\/sup>; <\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 in <i>hemoglobinopatia C<\/i>, la electroforeza Hb in gel de agaroza in mediu alcalin, HbC se izoleaza\u00a0 ca o fractie anormala, cu migrare foarte lenta, pe linia Hb A<sub>2<\/sub>, in aceeasi pozitie\u00a0 cu Hb E si Hb O<sub>Arabia<\/sub>, intr-un procent de 38-44%laheterozigoti; pentru diferentierea Hb C de Hb E si Hb O<sub>Arabia <\/sub>este necesara efectuarea electroforezei la un pH acid (6-6.2); la electroforeza capilara HbC este identificata separat in Z2 (<i>vezi fig.6<\/i>)<sup>1;2<\/sup>; prezenta tarei Hb C nu prezinta semnificatie clinica insa este importanta in consilierea genetica a cuplurilor; homozigotii pentru Hb C (boala hemoglobinei C) prezinta urmatoarele caracteristici: anemie usoara\/medie cu microcitoza marcata, numeroase hematii \u201cin tinta\u201d pe frotiul de sange periferic, iar la electroforeza hemoglobinei se izoleaza Hb C in proportie de 95%, restul fiind reprezentat de Hb A2 si Hb F<sup>3<\/sup>;<\/span><\/span><\/p>\n<p><a class=\"shutterset_2613 cboxElement\" title=\"hb_6\" href=\"https:\/\/www.synevo.md\/wp-content\/uploads\/2009\/08\/hb_6.jpg\" rel=\"wpdevart_lightbox lightbox[2613] nofollow noopener\" data-rel=\"lightbox-gallery-juomPC5B\" data-rl_title=\"\" data-rl_caption=\"\" target=\"_blank\"><img decoding=\"async\" class=\"alignnone size-medium wp-image-13502\" src=\"\/wp-content\/uploads\/2009\/08\/hb_6.jpg\" alt=\"hb_6\" width=\"404\" height=\"351\" \/><\/a><\/p>\n<p><em>\u00a0Fig. 6 Electroforegrama unui pacient cu forma heterozigotade hemoglobinopatie C<\/em><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 in <i>\u03b1<\/i><i>\u2013 talasemia minora <\/i>\u00a0nu exista modificari procentuale ale Hb A, Hb A<sub>2 <\/sub>\u00a0si Hb F, iar electroforeza de Hb apare normala la subiectul adult heterozigot, insa dublu heterozigotii realizeaza \u201cboala Hb H\u201d, cu aspect clinic de talasemie intermedia; la electroforeza Hb in gel de agaroza la pH alcalin se poate evidentia\u00a0 o fractiune anormala cu migrare rapida (cea mai apropiata de anod) in timp ce la electroforeza capilara se detecteaza o fractiune anormala in pozitia Z15 (<i>vezi fig.7<\/i>) corespunzatoare Hb H (5-30%). 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<sup>1;<\/sup><sup>2<\/sup>. Genetica moleculara asigura insa diagnosticul de certitudine al tuturor formelor de <i>\u03b1<\/i><i>\u2013 talasemie<\/i><sup>3<\/sup><i>.<\/i><\/span><\/span><\/p>\n<p><a class=\"shutterset_2613 cboxElement\" title=\"hb_7\" href=\"https:\/\/www.synevo.md\/wp-content\/uploads\/2009\/08\/hb_7.jpg\" rel=\"wpdevart_lightbox lightbox[2613] nofollow noopener\" data-rel=\"lightbox-gallery-juomPC5B\" data-rl_title=\"\" data-rl_caption=\"\" target=\"_blank\"><img decoding=\"async\" class=\"alignnone size-medium wp-image-13503\" src=\"\/wp-content\/uploads\/2009\/08\/hb_7.jpg\" alt=\"hb_7\" width=\"404\" height=\"354\" \/><\/a><\/p>\n<p><em>Fig. 7 Electroforegrama unui pacient cu \u201cboala Hb H\u201d<\/em><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\"><b><i>Limite si interferente<\/i><\/b><\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Transfuziile de sange pot dilua o eventuala hemoglobina anormala, pentru o perioada de 3-4 luni, interferand cu rezultatele electroforezei<sup>5<\/sup>.<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">Hiposideremia poate determina obtinerea de valori fals scazute pentru Hb A2; daca exista o neconcordanta intre rezultatul obtinut pentru HbA2 si suspiciunea clinica de betatalasemie minor, pacientul va fi tratat cu fier, pana la normalizarea sideremiei, apoi se va repeta electroforeza de hemoglobina<sup>3<\/sup>.<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">\u2022 Interferente analitice<\/span><\/span><\/p>\n<p><span style=\"font-size: medium;\"><span style=\"color: #000000;\">In probele de sange\u00a0 care au depasit perioada de stabilitate se produce o degradare progresiva a fractiunilor Hb ceea ce poate afecta profilul electroforetic<sup>2<\/sup>.<\/span><\/span><\/p>\n<p><span style=\"font-size: small;\">\u0411\u0438\u0431\u043b\u0438\u043e\u0433\u0440\u0430\u0444\u0438\u044f<\/span><\/p>\n<p><span style=\"font-size: small;\">1. Predescu C. Sindroamele talasemice. In Tratat de Medicina Interna, Hematologie, partea I (Coordonator Dan<\/span><\/p>\n<p><span style=\"font-size: small;\">Colita). Ed. Med. Buc., 1997; 779-802.<\/span><\/p>\n<p><span style=\"font-size: small;\">2. Laborator Synevo. Referintele specifice tehnologiei de lucru utilizate 2013. Ref Type: Catalog.<\/span><\/p>\n<p><span style=\"font-size: small;\">3. Bain B, J. Haemoglobinopathy Diagnosis. Blackwell Science, 2001, 20-25.<\/span><\/p>\n<p><span style=\"font-size: small;\">4. Glader B. Anemia: General Considerations. In Wintrobe`s Clinical Hematology. Lippincott Williams &amp; Wilkins,<\/span><\/p>\n<p><span style=\"font-size: small;\">Philadelphia, 11th edition, 2004, 962.<\/span><\/p>\n<p><span style=\"font-size: small;\">5. Thomas L. Hemoglopinopathies. In Clinical Laboratory Diagnostics-Use and Assessment of Clinical Laboratory<\/span><\/p>\n<p><span style=\"font-size: small;\">Results. TH-Books Verlagsgesellschaft mbH, Frankfurt \/Main, Germany, 1 ed., 1998; 489-493.<\/span><\/p>","protected":false},"excerpt":{"rendered":"<p>\u0413\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d \u2013 \u0431\u0435\u043b\u043e\u043a, \u043a\u043e\u0442\u043e\u0440\u044b\u0439 \u0441\u043e\u0434\u0435\u0440\u0436\u0438\u0442\u0441\u044f \u0432 \u044d\u0440\u0438\u0442\u0440\u043e\u0446\u0438\u0442\u0430\u0445 \u043a\u0440\u043e\u0432\u0438 \u0438 \u0432\u044b\u043f\u043e\u043b\u043d\u044f\u0435\u0442 \u0444\u0443\u043d\u043a\u0446\u0438\u044e \u043f\u0435\u0440\u0435\u043d\u043e\u0441\u0430 \u043a\u0438\u0441\u043b\u043e\u0440\u043e\u0434\u0430 \u043e\u0442 \u043b\u0435\u0433\u043a\u0438\u0445 \u043a \u043e\u0441\u0442\u0430\u043b\u044c\u043d\u044b\u043c \u043e\u0440\u0433\u0430\u043d\u0430\u043c \u0438 \u0442\u043a\u0430\u043d\u044f\u043c \u043e\u0440\u0433\u0430\u043d\u0438\u0437\u043c\u0430. \u0415\u0433\u043e \u043c\u043e\u043b\u0435\u043a\u0443\u043b\u0430 \u0441\u043e\u0441\u0442\u043e\u0438\u0442 \u0438\u0445 \u0447\u0435\u0442\u044b\u0440\u0435\u0445 \u043f\u043e\u043b\u0438\u043f\u0435\u043f\u0442\u0438\u0434\u043d\u044b\u0445 \u0446\u0435\u043f\u0435\u0439, \u043a\u0430\u0436\u0434\u0430\u044f \u0438\u0437 \u043a\u043e\u0442\u043e\u0440\u044b\u0445 \u0441\u043e\u0434\u0435\u0440\u0436\u0438\u0442 \u0443\u0447\u0430\u0441\u0442\u043e\u043a \u0441\u0432\u044f\u0437\u044b\u0432\u0430\u043d\u0438\u044f \u043a\u0438\u0441\u043b\u043e\u0440\u043e\u0434\u0430 \u2013 \u0433\u0435\u043c (\u043d\u0435\u0431\u0435\u043b\u043a\u043e\u0432\u0443\u044e \u043f\u0440\u043e\u0441\u0442\u0435\u0442\u0438\u0447\u0435\u0441\u043a\u0443\u044e \u0433\u0440\u0443\u043f\u043f\u0443 \u0441 \u0430\u0442\u043e\u043c\u043e\u043c \u0436\u0435\u043b\u0435\u0437\u0430 \u0432 \u0446\u0435\u043d\u0442\u0440\u0435). \u0420\u0430\u0437\u043b\u0438\u0447\u0430\u044e\u0442 \u0432\u0430\u0440\u0438\u0430\u043d\u0442\u044b \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430, \u043e\u0442\u043b\u0438\u0447\u0430\u044e\u0449\u0438\u0435\u0441\u044f \u043f\u043e \u0441\u043e\u0441\u0442\u0430\u0432\u0443 \u043f\u043e\u043b\u0438\u043f\u0435\u043f\u0442\u0438\u0434\u043d\u044b\u0445 \u0446\u0435\u043f\u0435\u0439. \u041a \u043d\u043e\u0440\u043c\u0430\u043b\u044c\u043d\u044b\u043c \u0432\u0430\u0440\u0438\u0430\u043d\u0442\u0430\u043c \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 \u0447\u0435\u043b\u043e\u0432\u0435\u043a\u0430 \u043e\u0442\u043d\u043e\u0441\u044f\u0442:<\/p>\n<p><strong>HbA (Hemoglobin A \u2013 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d \u0410)<\/strong> \u2013 \u0441\u043e\u0441\u0442\u043e\u0438\u0442 \u0438\u0437 \u0434\u0432\u0443\u0445 \u03b1- \u0438 \u0434\u0432\u0443\u0445 \u03b2-\u0446\u0435\u043f\u0435\u0439, \u0432 \u043d\u043e\u0440\u043c\u0435 \u0443 \u0432\u0437\u0440\u043e\u0441\u043b\u044b\u0445 \u043b\u044e\u0434\u0435\u0439 \u044d\u0442\u0430 \u0444\u043e\u0440\u043c\u0430 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 \u044f\u0432\u043b\u044f\u0435\u0442\u0441\u044f \u043e\u0441\u043d\u043e\u0432\u043d\u043e\u0439 \u0438 \u0441\u043e\u0441\u0442\u0430\u0432\u043b\u044f\u0435\u0442 \u0431\u043e\u043b\u0435\u0435 96% \u0432\u0441\u0435\u0433\u043e \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430;<\/p>\n<p><strong>HbA2 (Hemoglobin A2 \u2013 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d \u04102)<\/strong>, \u0441\u043e\u0441\u0442\u043e\u0438\u0442 \u0438\u0437 \u0434\u0432\u0443\u0445 \u03b1- \u0438 \u0434\u0432\u0443\u0445 \u03b4-\u0446\u0435\u043f\u0435\u0439, \u043e\u0431\u044b\u0447\u043d\u043e \u0441\u043e\u0441\u0442\u0430\u0432\u043b\u044f\u0435\u0442 2,5-3,0% \u043e\u0431\u0449\u0435\u0433\u043e \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430;<\/p>\n<p><strong>HbF (Hemoglobin F \u2013 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d F)<\/strong> \u2013 \u0444\u0435\u0442\u0430\u043b\u044c\u043d\u044b\u0439 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d, \u0441\u043e\u0441\u0442\u043e\u0438\u0442 \u0438\u0437 \u0434\u0432\u0443\u0445 \u03b1- \u0438 \u0434\u0432\u0443\u0445 \u03b3-\u0446\u0435\u043f\u0435\u0439. \u042d\u0442\u0430 \u0444\u043e\u0440\u043c\u0430 \u043f\u0440\u0435\u043e\u0431\u043b\u0430\u0434\u0430\u0435\u0442 \u0432\u043e \u0432\u0440\u0435\u043c\u044f \u0440\u0430\u0437\u0432\u0438\u0442\u0438\u044f \u043f\u043b\u043e\u0434\u0430, \u043d\u043e \u0435\u0435 \u0441\u043e\u0434\u0435\u0440\u0436\u0430\u043d\u0438\u0435 \u0431\u044b\u0441\u0442\u0440\u043e \u0441\u043d\u0438\u0436\u0430\u0435\u0442\u0441\u044f \u0432 \u0442\u0435\u0447\u0435\u043d\u0438\u0435 \u043f\u0435\u0440\u0432\u043e\u0433\u043e \u0433\u043e\u0434\u0430 \u0436\u0438\u0437\u043d\u0438, \u0441\u043e\u0441\u0442\u0430\u0432\u043b\u044f\u044f \u0443 \u0432\u0437\u0440\u043e\u0441\u043b\u044b\u0445 \u0432 \u043d\u043e\u0440\u043c\u0435 \u043c\u0435\u043d\u0435\u0435 2%.<\/p>\n<p>\u0421\u0443\u0449\u0435\u0441\u0442\u0432\u0443\u044e\u0442 \u0432\u0440\u043e\u0436\u0434\u0435\u043d\u043d\u044b\u0435 \u0432\u0438\u0434\u044b \u043f\u0430\u0442\u043e\u043b\u043e\u0433\u0438\u0438 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430, \u0441\u0432\u044f\u0437\u0430\u043d\u043d\u044b\u0435 \u0441 \u0438\u0437\u043c\u0435\u043d\u0435\u043d\u0438\u0435\u043c \u0441\u0442\u0440\u0443\u043a\u0442\u0443\u0440\u044b \u0435\u0433\u043e \u043f\u043e\u043b\u0438\u043f\u0435\u043f\u0442\u0438\u0434\u043d\u044b\u0445 \u0446\u0435\u043f\u0435\u0439 \u0438\u043b\u0438 \u043d\u0430\u0440\u0443\u0448\u0435\u043d\u0438\u0435\u043c \u0438\u0445 \u043f\u0440\u043e\u0434\u0443\u043a\u0446\u0438\u0438.<\/p>\n<p><strong>\u0421 \u043a\u0430\u043a\u043e\u0439 \u0446\u0435\u043b\u044c\u044e \u043f\u0440\u043e\u0432\u043e\u0434\u044f\u0442 \u042d\u043b\u0435\u043a\u0442\u0440\u043e\u0444\u043e\u0440\u0435\u0437 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430<\/strong><\/p>\n<p>\u0422\u0435\u0441\u0442 \u0438\u0441\u043f\u043e\u043b\u044c\u0437\u0443\u044e\u0442 \u0432 \u043a\u0430\u0447\u0435\u0441\u0442\u0432\u0435 \u0441\u043a\u0440\u0438\u043d\u0438\u043d\u0433\u043e\u0432\u043e\u0433\u043e \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u044f \u043f\u0440\u0438 \u043f\u043e\u0434\u043e\u0437\u0440\u0435\u043d\u0438\u0438 \u043d\u0430 \u043f\u0430\u0442\u043e\u043b\u043e\u0433\u0438\u044e \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430, \u043d\u0430\u043f\u0440\u0438\u043c\u0435\u0440, \u043d\u0435 \u0438\u043c\u0435\u044e\u0449\u0438\u0435 \u0438\u043d\u044b\u0445 \u043e\u0431\u044a\u044f\u0441\u043d\u0435\u043d\u0438\u0439 \u0430\u043d\u043e\u043c\u0430\u043b\u044c\u043d\u044b\u0435 \u0440\u0435\u0437\u0443\u043b\u044c\u0442\u0430\u0442\u044b \u043e\u0431\u0449\u0435\u0433\u043e \u0430\u043d\u0430\u043b\u0438\u0437\u0430 \u043a\u0440\u043e\u0432\u0438 \u0438\/\u0438\u043b\u0438 \u043f\u0440\u043e\u0441\u043c\u043e\u0442\u0440\u0430 \u043e\u043a\u0440\u0430\u0448\u0435\u043d\u043d\u043e\u0433\u043e \u043c\u0430\u0437\u043a\u0430 \u043a\u0440\u043e\u0432\u0438; \u043f\u0440\u0438\u0437\u043d\u0430\u043a\u0438 \u0433\u0435\u043c\u043e\u043b\u0438\u0442\u0438\u0447\u0435\u0441\u043a\u043e\u0439 \u0430\u043d\u0435\u043c\u0438\u0438; \u0441\u0435\u043c\u0435\u0439\u043d\u044b\u0439 \u0430\u043d\u0430\u043c\u043d\u0435\u0437. \u0412\u0430\u0440\u0438\u0430\u043d\u0442\u043d\u044b\u0435 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u044b \u043e\u0442\u043b\u0438\u0447\u0430\u044e\u0442\u0441\u044f \u043f\u043e \u0441\u0432\u043e\u0435\u0439 \u043f\u0440\u043e\u0441\u0442\u0440\u0430\u043d\u0441\u0442\u0432\u0435\u043d\u043d\u043e\u0439 \u0441\u0442\u0440\u0443\u043a\u0442\u0443\u0440\u0435, \u043f\u043e\u0432\u0435\u0440\u0445\u043d\u043e\u0441\u0442\u043d\u043e\u043c\u0443 \u0437\u0430\u0440\u044f\u0434\u0443 \u0438 \u044d\u043b\u0435\u043a\u0442\u0440\u043e\u0444\u043e\u0440\u0435\u0442\u0438\u0447\u0435\u0441\u043a\u043e\u0439 \u043f\u043e\u0434\u0432\u0438\u0436\u043d\u043e\u0441\u0442\u0438, \u0444\u0440\u0430\u043a\u0446\u0438\u0438 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 \u043c\u043e\u0433\u0443\u0442 \u0431\u044b\u0442\u044c \u0440\u0430\u0437\u0434\u0435\u043b\u0435\u043d\u044b \u0438 \u043e\u0446\u0435\u043d\u0435\u043d\u044b \u0441 \u043f\u0440\u0438\u043c\u0435\u043d\u0435\u043d\u0438\u0435\u043c \u044d\u043b\u0435\u043a\u0442\u0440\u043e\u0444\u043e\u0440\u0435\u0437\u0430.<\/p>\n<p><strong>\u0420\u0430\u0437\u043b\u0438\u0447\u0430\u044e\u0442 \u0441\u043b\u0435\u0434\u0443\u044e\u0449\u0438\u0435 \u0432\u0430\u0440\u0438\u0430\u043d\u0442\u044b \u043f\u0430\u0442\u043e\u043b\u043e\u0433\u0438\u0438 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430:<\/strong><\/p>\n<p>\u0413\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u043e\u043f\u0430\u0442\u0438\u0438 \u2013 \u043d\u0430\u0440\u0443\u0448\u0435\u043d\u0438\u044f \u0441\u0442\u0440\u0443\u043a\u0442\u0443\u0440\u044b \u043c\u043e\u043b\u0435\u043a\u0443\u043b\u044b \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 (\u0441\u0432\u044f\u0437\u0430\u043d\u044b \u0441 \u0434\u0435\u0444\u0435\u043a\u0442\u043e\u043c \u0433\u0435\u043d\u043e\u0432, \u043e\u0442\u0432\u0435\u0442\u0441\u0442\u0432\u0435\u043d\u043d\u044b\u0445 \u0437\u0430 \u0441\u0438\u043d\u0442\u0435\u0437 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430, \u0432 \u0442\u043e\u043c \u0447\u0438\u0441\u043b\u0435 \u0442\u043e\u0447\u0435\u0447\u043d\u044b\u043c\u0438 \u043c\u0443\u0442\u0430\u0446\u0438\u044f\u043c\u0438). \u041e\u043f\u0438\u0441\u0430\u043d\u044b \u0441\u043e\u0442\u043d\u0438 \u0432\u043e\u0437\u043c\u043e\u0436\u043d\u044b\u0445 \u0432\u0430\u0440\u0438\u0430\u043d\u0442\u043e\u0432 \u043f\u043e\u0434\u043e\u0431\u043d\u044b\u0445 \u043d\u0430\u0440\u0443\u0448\u0435\u043d\u0438\u0439, \u043d\u0435\u0440\u0435\u0434\u043a\u043e \u0432\u044b\u044f\u0432\u043b\u044f\u0435\u043c\u044b\u0445 \u0441\u043b\u0443\u0447\u0430\u0439\u043d\u043e, \u043d\u0430\u043f\u0440\u0438\u043c\u0435\u0440, \u0437\u0430\u043f\u043e\u0434\u043e\u0437\u0440\u0435\u043d\u043d\u044b\u0445 \u043f\u0440\u0438 \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u0438 \u0433\u043b\u0438\u043a\u0438\u0440\u043e\u0432\u0430\u043d\u043d\u043e\u0433\u043e \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430. \u0418\u0437 \u043d\u0438\u0445 \u043b\u0438\u0448\u044c \u043d\u0435\u043a\u043e\u0442\u043e\u0440\u044b\u0435 \u0438\u043c\u0435\u044e\u0442 \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u043e\u0435 \u0437\u043d\u0430\u0447\u0435\u043d\u0438\u0435. \u041f\u0440\u0438\u043c\u0435\u0440\u043d\u043e\u043c \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u0438-\u0437\u043d\u0430\u0447\u0438\u043c\u043e\u0439 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u043e\u043f\u0430\u0442\u0438\u0438 \u044f\u0432\u043b\u044f\u0435\u0442\u0441\u044f \u0441\u0435\u0440\u043f\u043e\u0432\u0438\u0434\u043d\u043e-\u043a\u043b\u0435\u0442\u043e\u0447\u043d\u0430\u044f \u0430\u043d\u0435\u043c\u0438\u044f (\u0432\u0430\u0440\u0438\u0430\u043d\u0442\u043d\u044b\u0439 \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d S).<\/p>\n<p>\u0422\u0430\u043b\u0430\u0441\u0441\u0435\u043c\u0438\u0438 \u2013 \u043d\u0430\u0440\u0443\u0448\u0435\u043d\u0438\u044f \u043f\u0440\u043e\u0434\u0443\u043a\u0446\u0438\u0438 \u0446\u0435\u043f\u0435\u0439 \u043d\u043e\u0440\u043c\u0430\u043b\u044c\u043d\u043e\u0433\u043e \u0433\u0435\u043c\u043e\u0433\u043b\u043e\u0431\u0438\u043d\u0430 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