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Fibrinogen yetishmovchiligi - Vikipediya
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</ul> </li> <li id="toc-Manbalar" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Manbalar"> <div class="vector-toc-text"> <span class="vector-toc-numb">5</span> <span>Manbalar</span> </div> </a> <ul id="toc-Manbalar-sublist" class="vector-toc-list"> </ul> </li> </ul> </div> </div> </nav> </div> </div> <div class="mw-content-container"> <main id="content" class="mw-body"> <header class="mw-body-header vector-page-titlebar"> <nav aria-label="Mundarija" class="vector-toc-landmark"> <div id="vector-page-titlebar-toc" class="vector-dropdown vector-page-titlebar-toc vector-button-flush-left" > <input type="checkbox" id="vector-page-titlebar-toc-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-vector-page-titlebar-toc" class="vector-dropdown-checkbox " aria-label="Toggle the table of contents" > <label id="vector-page-titlebar-toc-label" for="vector-page-titlebar-toc-checkbox" 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.ambox+.mw-empty-elt+link+link+.ambox{margin-top:-1px}html body.mediawiki .mw-parser-output .ambox.mbox-small-left{margin:4px 1em 4px 0;overflow:hidden;width:238px;border-collapse:collapse;font-size:88%;line-height:1.25em}.mw-parser-output .ambox-speedy{border-left:10px solid #b32424;background-color:#fee7e6}.mw-parser-output .ambox-delete{border-left:10px solid #b32424}.mw-parser-output .ambox-content{border-left:10px solid #f28500}.mw-parser-output .ambox-style{border-left:10px solid #fc3}.mw-parser-output .ambox-move{border-left:10px solid #9932cc}.mw-parser-output .ambox-protection{border-left:10px solid #a2a9b1}.mw-parser-output .ambox .mbox-text{border:none;padding:0.25em 0.5em;width:100%}.mw-parser-output .ambox .mbox-image{border:none;padding:2px 0 2px 0.5em;text-align:center}.mw-parser-output .ambox .mbox-imageright{border:none;padding:2px 0.5em 2px 0;text-align:center}.mw-parser-output .ambox .mbox-empty-cell{border:none;padding:0;width:1px}.mw-parser-output .ambox .mbox-image-div{width:52px}html.client-js body.skin-minerva .mw-parser-output .mbox-text-span{margin-left:23px!important}@media(min-width:720px){.mw-parser-output .ambox{margin:0 10%}}</style><table class="box-Merge plainlinks metadata ambox ambox-move" role="presentation"><tbody><tr><td class="mbox-image"><div class="mbox-image-div"><span typeof="mw:File"><span><img alt="" src="//upload.wikimedia.org/wikipedia/commons/thumb/5/52/Merge-arrows.svg/50px-Merge-arrows.svg.png" decoding="async" width="50" height="20" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/5/52/Merge-arrows.svg/75px-Merge-arrows.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/5/52/Merge-arrows.svg/100px-Merge-arrows.svg.png 2x" data-file-width="50" data-file-height="20" /></span></span></div></td><td class="mbox-text"><div class="mbox-text-span"><b>Ushbu sahifani <i><a href="/wiki/Fibrinogen" title="Fibrinogen">Fibrinogen</a></i> bilan birlashtirish taklif etiladi. (<a href="/wiki/Munozara:Fibrinogen_yetishmovchiligi" title="Munozara:Fibrinogen yetishmovchiligi">munozara</a>)</b></div></td></tr></tbody></table> <table class="infobox" cellspacing="1" style="width:270px;"><tbody><tr><th colspan="2" class="" style="text-align:center; font-size:120%; font-weight:bold; background:#ccc">Fibrinogen yetishmovchiligi</th></tr> <tr class=""><th scope="row" style="text-align:left;">Sinonimlari</th> <td class="" style=""> I omil yetishmovchiligi, I omil immunodefitsiti</td></tr><tr class=""><td colspan="2" class="" style="text-align:center;"> <span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/Fayl:Autosomal_recessive_-_en.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/f/f1/Autosomal_recessive_-_en.svg/300px-Autosomal_recessive_-_en.svg.png" decoding="async" width="300" height="522" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/f/f1/Autosomal_recessive_-_en.svg/450px-Autosomal_recessive_-_en.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/f/f1/Autosomal_recessive_-_en.svg/600px-Autosomal_recessive_-_en.svg.png 2x" data-file-width="738" data-file-height="1283" /></a></span></td></tr><tr class=""><td colspan="2" class="" style="text-align:center;"> Carrier parent (tashuvchi ota va ona) dan farzandlarning tugʻilish ehtimolligi. Chapda Uneffected child (sogʻlom bola), oʻrtadagi 2ta carrier (tashuvchi bolalar), oʻngda affected child (fibrinogen defitsiti bilan tugʻilgan bola)</td></tr><tr class=""><th scope="row" style="text-align:left;"><a href="#Specialty_(medicine)">Mutaxassislik</a></th> <td class="" style=""> Gemotologiya; ichki kasalliklar</td></tr><tr class=""><th scope="row" style="text-align:left;">Turlari</th> <td class="" style=""> afibrinogenemiya, gipofibrinogenemiya, disfibrinogenemiya, gipodisfibrinogenemiya</td></tr><tr class=""><th scope="row" style="text-align:left;">Tashxis usullari</th> <td class="" style=""> Biokimyoviy qon tahlili: umumiy oqsil tahlili va fibrinogen fraksiyasi</td></tr><tr class=""><th scope="row" style="text-align:left;">Prognoz</th> <td class="" style=""> turiga koʻra har 1000 boladan 0,5-3 tagacha</td></tr> </tbody></table> <p><a href="/wiki/Fibrinogen" title="Fibrinogen">Fibrinogen</a> yetishmovchiligi (shuningdek, I omil yetishmovchiligi) <a href="/wiki/Qon_ivish_kasalliklari" title="Qon ivish kasalliklari">qon ivish</a> "kaskadi"dagi fibrinogen funksiyasi yetishmovchiligi bilan bogʻliq kamyob <a href="/wiki/Irsiy_kasalliklar" title="Irsiy kasalliklar">irsiy kasallik</a> hisoblanadi. Fibrinogen yetishmovchiligi oʻz navbatida bir-biridan farqli toʻrt guruhga boʻlinadi: afibrinogenemiya, gipofibrinogenemiya, disfibrinogenemiya va gipodisfibrinogenemiya.<sup id="cite_ref-1" class="reference"><a href="#cite_note-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Afibrinogenemiya">Afibrinogenemiya</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&veaction=edit&section=1" title="Boʻlimni tahrirlash: Afibrinogenemiya" class="mw-editsection-visualeditor"><span>tahrir</span></a><span class="mw-editsection-divider"> | </span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&action=edit&section=1" title="Edit section's source code: Afibrinogenemiya"><span>manbasini tahrirlash</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Afibrinogenemiya qonda fibrinogen miqdorining yetishmasligi, klinik jihatdan <a href="/wiki/Plazma_(qon)" title="Plazma (qon)">plazma</a> tarkibida fibrinogen <20 mg/dl holatida boʻlishi. Ushbu patologik holatning uchrash chastotasi million kishi orasida 0,5 dan 2 tagacha baholanadi.<sup id="cite_ref-:0_2-0" class="reference"><a href="#cite_note-:0-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup>  <a href="/wiki/Amerika_Qo%CA%BBshma_Shtatlari_tarixi" title="Amerika Qoʻshma Shtatlari tarixi">Amerika Qoʻshma Shtatlarida</a> fibrinogenning barcha irsiy anomaliyalarining 24% ni afibrinogenemiya, gipofibrinogenemiya va disfibrinogenemiya esa 38% ni tashkil qiladi.<sup id="cite_ref-:1_3-0" class="reference"><a href="#cite_note-:1-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Gipofibrinogenemiya">Gipofibrinogenemiya</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&veaction=edit&section=2" title="Boʻlimni tahrirlash: Gipofibrinogenemiya" class="mw-editsection-visualeditor"><span>tahrir</span></a><span class="mw-editsection-divider"> | </span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&action=edit&section=2" title="Edit section's source code: Gipofibrinogenemiya"><span>manbasini tahrirlash</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Tugʻma gipofibrinogenemiya fibrinogenning qisman yetishmovchiligi boʻlib, klinik jihatdan plazmada 20-80mg/dl ni tashkil qiladi. Taxminiy uchrash chastotasi million kishida <0,5 dan 3 tagacha.<sup id="cite_ref-:0_2-1" class="reference"><a href="#cite_note-:0-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:1_3-1" class="reference"><a href="#cite_note-:1-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Disfibrinogenemiya">Disfibrinogenemiya</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&veaction=edit&section=3" title="Boʻlimni tahrirlash: Disfibrinogenemiya" class="mw-editsection-visualeditor"><span>tahrir</span></a><span class="mw-editsection-divider"> | </span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&action=edit&section=3" title="Edit section's source code: Disfibrinogenemiya"><span>manbasini tahrirlash</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Disfibrinogenemiya qondagi fibrinogenning notoʻgʻri ishlashi yoki ishlamasligi bilan tavsiflanadi, garchi fibrinogen miqdori normal konsentratsiyalarda boʻlsa ham: yaʼni plazmada 200-400 mg/dl boʻlsa ham. Disfibrinogenemiya kelib chiqishiga koʻra ikki guruhga ajratiladi: irsiy boʻlishi mumkin (shuning uchun tugʻma disfibrinogenemiya) va shuningdek, boshqa kasallikdan keyin kelib chiqqan ikkilamchi darajali disfibrinogenemiya (orttirilgan disfibrinogenemiya deb ataladi). Tugʻma uchrash chastotasi har million boladan 1 yoki 3 tasi orasida uchraydi.<sup id="cite_ref-:0_2-2" class="reference"><a href="#cite_note-:0-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:1_3-2" class="reference"><a href="#cite_note-:1-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-4" class="reference"><a href="#cite_note-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Gipodisfibrinogenemiya">Gipodisfibrinogenemiya</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&veaction=edit&section=4" title="Boʻlimni tahrirlash: Gipodisfibrinogenemiya" class="mw-editsection-visualeditor"><span>tahrir</span></a><span class="mw-editsection-divider"> | </span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&action=edit&section=4" title="Edit section's source code: Gipodisfibrinogenemiya"><span>manbasini tahrirlash</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Gipodisfibrinogenemiya fibrinogenning qisman yetishmovchiligi boʻlib, mavjud boʻlgan fibrinogen ham notoʻgʻri ishlaydi.  Gipodisfibrinogenemiya juda kam uchraydigan irsiy kasallikdir.<sup id="cite_ref-5" class="reference"><a href="#cite_note-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p><p>Klinik jihatdan bu holatlar, odatda, diatezning kuchayishi, ya'ni oʻz-oʻzidan qon ketish epizodlari, kichik toʻqimalarning shikastlanishi va operatsiyalaridan keyin ham koʻp qon ketishining rivojlanishiga moyillik bilan bogʻliq. Ushbu kasalliklarni davolash, odatda, ixtisoslashgan markazlarni va har bir shaxsning shaxsiy va oilaviy tarixidan kelib chiqqan holda, oldingi qon ketish va tromboz epizodlarining chastotasi va ogʻirligi, shuningdek, ayrim hollarda, genetik mutatsiyalarning taxminiy moyilligi asosida ishlab chiqilgan profilaktika choralarini oʻrnatishni oʻz ichiga oladi. Fibrinogen yetishmovchiliklari qon ketishi va trombozga sabab boʻladigan buzilishlar asosida yotadi.<sup id="cite_ref-6" class="reference"><a href="#cite_note-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Manbalar">Manbalar</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&veaction=edit&section=5" title="Boʻlimni tahrirlash: Manbalar" class="mw-editsection-visualeditor"><span>tahrir</span></a><span class="mw-editsection-divider"> | </span><a href="/w/index.php?title=Fibrinogen_yetishmovchiligi&action=edit&section=5" title="Edit section's source code: Manbalar"><span>manbasini tahrirlash</span></a><span class="mw-editsection-bracket">]</span></span></div> <ol class="references"> <li id="cite_note-1"><span class="mw-cite-backlink"><a href="#cite_ref-1">↑</a></span> <span class="reference-text">"What is factor I (fibrinogen) deficiency?".</span> </li> <li id="cite_note-:0-2"><span class="mw-cite-backlink">↑ <sup><a href="#cite_ref-:0_2-0">2,0</a></sup> <sup><a href="#cite_ref-:0_2-1">2,1</a></sup> <sup><a href="#cite_ref-:0_2-2">2,2</a></sup></span> <span class="reference-text">"Factor I Deficiency".</span> </li> <li id="cite_note-:1-3"><span class="mw-cite-backlink">↑ <sup><a href="#cite_ref-:1_3-0">3,0</a></sup> <sup><a href="#cite_ref-:1_3-1">3,1</a></sup> <sup><a href="#cite_ref-:1_3-2">3,2</a></sup></span> <span class="reference-text">Acharya, Suchitra S (2019-02-02). Tebbi, Cameron K (ed.). "Inherited Abnormalities of Fibrinogen". <i>medscape.com</i>.</span> </li> <li id="cite_note-4"><span class="mw-cite-backlink"><a href="#cite_ref-4">↑</a></span> <span class="reference-text">Casini A, Neerman-Arbez M, Ariëns RA, de Moerloose P (2015). "Dysfibrinogenemia: from molecular anomalies to clinical manifestations and management". <i>Journal of Thrombosis and Haemostasis</i>. <b>13</b> (6): 909–19. doi:10.1111/jth.12916. PMID 25816717.</span> </li> <li id="cite_note-5"><span class="mw-cite-backlink"><a href="#cite_ref-5">↑</a></span> <span class="reference-text">Casini A, Brungs T, Lavenu-Bombled C, Vilar R, Neerman-Arbez M, de Moerloose P (2017). "Genetics, diagnosis and clinical features of congenital hypodysfibrinogenemia: a systematic literature review and report of a novel mutation". <i>Journal of Thrombosis and Haemostasis</i>. <b>15</b> (5): 876–888. doi:10.1111/jth.13655. PMID 28211264.</span> </li> <li id="cite_note-6"><span class="mw-cite-backlink"><a href="#cite_ref-6">↑</a></span> <span class="reference-text">Neerman-Arbez M, de Moerloose P, Casini A (2016). "Laboratory and Genetic Investigation of Mutations Accounting for Congenital Fibrinogen Disorders". <i>Seminars in Thrombosis and Hemostasis</i>. <b>42</b> (4): 356–65. doi:10.1055/s-0036-1571340. 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