MUKOPOLISAHARIDOZHO

MUKOPOLISAHARIDOZHO, mukopolisakharidho, gargoilizm, yak guruh bemorihoi irsii boftahoi payvandi, ki boisi ikhtiloli mubodilai polisakharidho meshavand. M. bo osebi dastgohi takyavu harakat, uzvhoi dokhili, ikhtiloli bosira, somea, ba takhir mondani rushdi jismoni va ravoni padid meoyand. Tanosubi M. dar bayni navzodon taqr. 1 : 400 000 ast. Dar natijai norasoii fermenthoe, ki dar mubodilai mukopolisakharidho ishtirok dorand (nig. Enzimopatiya), mukopolisakharidhoi tajziyanashuda dar boftahoi uzvhoi gunogun ba miqdori ziyod gun gashta, faoliyati onhoro taboh mesozand. Vobasta ba osebi kadomin ferment 8 navi M.-ro judo mekunand: sindromhoi Gurler, Gunter, Sanfilippo, Morkio, SHeye, Maroto-Lami, Slae, Di Ferrant.
Alomathoi M., odatan, dar soli yakumi hayoti kudak padid meoyand; sindromi SHeye dar davrai maktabkhoni paydo meshavad. Sari az had kalon (makrosefaliya), chehrai be mimikai dagal, chashmoni az ham dur (gipertelorizm) va gardani kutoh nishonahoi M. meboshand. Alomathoi mazkur khususan hangomi sindromi Gurler vozeh ba mushohida merasand. Mumkin ast kifozi qismi kamarii sutunmuhra va deformatsiyai sanduqi sina (mas., dar mavridi sindromi Morkio) ba vuqu payvandad. Bandhoi kalon kaj gashta, khub harakat namekunand; shikam kalon, qarniyai chashm tira, shunavoi zaif, kori silsilai dilu ragho vayron meshavad.
M. muolijai makhsus nadorand. Tabobati onho asosan aloimi ast.