Adrenoleukodystrophy: nws yog dab tsi, cov tsos mob thiab kev kho mob

Zoo Siab

• Cov tsos mob ntawm adrenoleukodystrophy
• Yuav kho tus mob li cas

Adrenoleukodystrophy yog qhov tsis tshua muaj caj ces txuas nrog rau X chromosome, nyob rau hauv uas muaj adrenal tsis txaus thiab tsub zuj zuj ntawm cov tshuaj hauv lub cev uas txhawb nqa demyelination ntawm cov axons, uas yog ib feem ntawm cov neuron lub luag haujlwm rau lub teeb liab hluav taws xob, thiab tej zaum yuav koom nrog piv txwv, pom kev los yog hauv kev nqes tes thiab so ntawm cov leeg, piv txwv.

Yog li, zoo li hauv adrenoleukodystrophy, kev ceeb toom tsis txaus siab tej zaum yuav tsis muaj peev xwm, nws yog qhov ua tau rau cov cim thiab cov tsos mob ntsig txog qhov xwm txheej no yuav tshwm sim dhau sijhawm, nrog kev hloov ntawm kev hais lus, kev nyuaj rau nqos thiab taug kev, thiab kev hloov pauv tus cwj pwm, piv txwv.

Tus kab mob no muaj ntau rau cov txiv neej, vim tias txiv neej tsuas muaj 1 x chromosome, thaum poj niam yuav tsum muaj ob qho chromosomes kho kom tus mob muaj. Ntxiv mus, cov paib thiab cov tsos mob tuaj yeem tshwm sim rau txhua lub hnub nyoog, nyob ntawm qhov siv ntawm caj ces hloov pauv thiab qhov nrawm uas tau teev tseg.

Cov tsos mob ntawm adrenoleukodystrophy

Cov tsos mob ntawm adrenoleukodystrophy muaj feem xyuam rau kev hloov pauv ntawm kev ua haujlwm ntawm adrenal qog thiab demyelination ntawm axons. Cov qog adrenal ua haujlwm nyob sab saud lub raum thiab cuam tshuam txog kev tsim cov tshuaj uas pab tswj cov leeg hlwb tsis zoo, txhawb kev tswj hwm qee qhov haujlwm ntawm lub cev, xws li kev ua pa thiab kev zom zaub mov, piv txwv. Yog li, thaum muaj dysregulation lossis poob adrenal ua haujlwm, kev hloov pauv ntawm cov hlab ntsha poob siab kuj tseem pom.

Tsis tas li ntawd, vim kev hloov caj ces, nws muaj peev xwm khaws ntau yam tshuaj lom rau hauv lub cev, uas tuaj yeem ua rau kom tsis muaj cov myelin sheath ntawm cov axons, tiv thaiv kev sib kis ntawm cov pa hluav taws xob thiab ua rau cov xeeb ceem cim thiab cov tsos mob ntawm adrenoleukodystrophy.

Yog li, cov tsos mob ntawm adrenoleukodystrophy yog pom raws li tus neeg txhim kho thiab tuaj yeem paub meej:

• Poob ntawm cov qog adrenal ua haujlwm;
• Tsis muaj peev xwm hais lus thiab tham tau;
• Kev coj cwj pwm hloov;
• Strabismus;
• Kev nyuaj hauv kev taug kev;
• Kev nyuaj hauv kev pub mis, thiab kev pub mis los ntawm cov raj kuj yuav tsim nyog;
• Nyuaj noj nyuaj;
• Poob peev xwm ntawm kev txawj ntse;
• Qhov pom tseeb.

Nws yog ib qho tseem ceeb tias adrenoleukodystrophy tau txheeb xyuas txoj cai thaum yug los, vim nws muaj peev xwm kom txo qis ntawm cov tsos mob tshwm sim, txhawb tus menyuam lub neej zoo.

Yuav kho tus mob li cas

Txoj kev kho rau adrenoleukodystrophy yog hloov hlwb pob txha, uas tau pom zoo thaum cov tsos mob twb dhau los lawm thiab muaj kev hloov loj rau lub hlwb. Hauv qee qhov mob me me, kws kho mob yuav pom zoo kom hloov cov tshuaj hormones los ntawm cov qog adrenal, ntxiv rau kev kho lub cev kom tsis txhob mob leeg.