Zaj. Metachromatic leukodystrophy (MLD) yog kab mob tsis tshua muaj tshwm sim los ntawm tus kab mob Epidemiology. Nyob ib ncig ntawm 1 ntawm 50 tus neeg raug cuam tshuam los ntawmib tus kab mob paub ib leeg, thaum nyob ib ncig ntawm 1 ntawm 263 raug cuam tshuam los ntawm chromosomal disorder. Nyob ib ncig ntawm 65% ntawm cov tib neeg muaj qee yam teeb meem kev noj qab haus huv raws li qhov tshwm sim ntawm kev hloov pauv ntawm caj ces. https://en.wikipedia.org › wiki › Genetic_disorder
Kev mob caj ces - Wikipedia
tus yam ntxwv los ntawm kev sib sau ntawm cov rog hu ua sulfatides sulfatides Sulfatide yogcov khoom tseem ceeb hauv lub paj hlwbthiab pom muaj nyob hauv cov qib siab hauv myelin sheath hauv ob qho tib si peripheral paj hlwb thiab central paj hlwb. Myelin feem ntau muaj li 70-75% lipids, thiab sulfatide muaj 4-7% ntawm qhov no 70-75%. https://en.wikipedia.org › wiki › Sulfatide
Sulfatide - Wikipedia
. Qhov no ua rau kev puas tsuaj ntawm cov txheej txheem tiv thaiv rog (myelin sheath) nyob ib puag ncig cov hlab ntsha hauv nruab nrab ntawm lub paj hlwb thiab lub paj hlwb peripheral.
Cov tsos mob ntawm MLD yog dab tsi?
Cov tsos mob muaj xws li cov nqaij ntshiv thiab tsis muaj zog, cov leeg nruj, kev loj hlob qeeb, kev tsis pom kev zoo ua rau qhov muag tsis pom kev, convulsions, impaired nqos tau, tuag tes tuag taw, thiab dementia. Cov menyuam yaus tuaj yeem ua comatose. Cov menyuam feem coob uas muaj daim ntawv MLD no tuag thaum muaj hnub nyoog 5 xyoos.
Koj tuaj yeem nyob nrog MLD ntev npaum li cas?
Cov neeg raug cuam tshuam los ntawm cov neeg laus feem ntau tuag nrog 6 txog 14 xyoo tom qab pib cov tsos mob. Qhov kev cia siab rau MLD tsis zoo. Cov menyuam yaus feem ntau nyob rau hauv daim ntawv me nyuam mos tuag thaum muaj hnub nyoog 5 xyoos. Cov tsos mob ntawm daim ntawv me nyuam yaus loj hlob nrog kev tuag tshwm sim 10 txog 20 xyoo tom qab pib.
Dab tsi ua rau MLD?
MLD feem ntau yog tshwm sim los ntawm tsis muaj ib qho tseem ceeb enzyme hu ua arylsulfatase A (ARSA). Vim tias qhov enzyme no ploj lawm, cov tshuaj hu ua sulfatides tsim nyob rau hauv lub cev thiab ua rau lub paj hlwb, ob lub raum, gallbladder, thiab lwm yam kabmob.
MLD puas tuag?
Nyob rau hauv cov menyuam yaus MLD, lub neej expectancy yog 10 txog 20 xyoo tom qab kuaj mob. Yog tias cov tsos mob tsis tshwm sim txog thaum muaj hnub nyoog laus, cov neeg feem ntau nyob 20 txog 30 xyoo tom qab kuaj pom. Txawm hais tias tseem tsis tau muaj kev kho mob rau MLD, tab tom tsim kho ntau dua.
