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GLIKOGENOZHO

glikogenezGLIKOGENOZHO (Glycogenosis), bemorii glikogeni, guruhi bemorihoi irsii mubodilai karbogidratho. G. binobar norasoii fermenthoe, ki dar jarayoni tajziya yo sintezi glikogen ishtirok mekunand, ba vujud meoyand. Vobasta ba ikhtiloli faoliyati fermentho 13 navi G.-ro farq mekunand, ki 9 navi onho nisbatan purra omukhta shudaand.
Hangomi glikogenozi navi I kudakon biduni alomathoi zohirii bemori tavallud meshavand. Qay, tashannuj va sianoz dar zaminai gipoglikemiya badi chand soati ba dunyo omadan yo mohhoi avvali hayoti u ba vujud meoyad. Kudak az sabzish memonad, ruy farbeh (misli «rui lukhtak»), badan (khususan qismi poyoni shikam) kharob, dastu po borik, gardan kutoh meshavad.
Dar asnoi G.-i navi II glikogen dar uzvu boftaho (khususan mushaki dil) ba miqdori ziyod gird omada, boisi kalon shudani andoza va inkishofi nuqsi dil meshavad. Alomathoi avvalini bemori badi chand soat yo ruzi tavallud padid meoyand. Sianoz, damkutahi, beqarori, beholi, kalon shudani zabon nishonahoi marizi meboshand. Alomati bolinii in navi G. ba miokardit monand ast.
Alomathoi bolinii G.-i navi III shabehi G.-i navi I buda, faqat dar mavridi tadqiqi biokhimiyavii makhsus muqarrar karda meshavand.
Navhoi digari G. bisyor kam duchor meoyand. Baroi tasdiqi tashkhisi G. biopsiyai jigar va mushakho zarur ast. Mavodi biopsiya ba tadqiqi gistologi firistoda meshavad.
Rohhoi peshgirii G. malum nest; muolijai makhsusi onho daryoft nashudaast.

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