Turner's syndrome los yog Turner-Shereshevsky syndrome yog ib qho kab mob tsis tshua muaj caj dab uas yog tshwm sim ntawm qhov kev ntseeg ntawm X chromosomes thiab tshwm sim hauv cov poj niam. Lub triad ntawm cov tsos mob ntawm no pathology raws li Shershevsky nrog sexual infantilism, pterygoid tawv folds ntawm lub caj dab thiab deformity ntawm lub luj tsho ntxiv. Cov poj niam uas raug kev txom nyem los ntawm Turner syndrome feem ntau muaj kev vwm thiab hnyuv .
Shereshevsky-Turner Syndrome - yog vim li cas
Raws li txoj cai, tus Y-chromosome txheej ntawm ib tug txiv neej muaj 46 (23 khub) ntawm chromosomes. Ntawm no, ib nkawm muaj kev sib deev chromosomes (XX rau cov poj niam lossis XY txiv neej). Yog hais tias Turner's Syndrome, ib tus X-chromosome khub yog ploj tag lossis puas. Dab tsi yog qhov ua rau ntawm cov caj ces caj dab uas ua rau Turner's syndrome tsis yog raws nraim tau tsim, txij li txawm hais tias tus kab mob tshwm sim nws tus kheej nyob rau theem ntawm fetal tsim, nws tsis yog hereditary.
Lub xub ntiag ntawm lub syndrome yog paub tseeb los ntawm kev ntsuam xyuas ntawm karyotype, uas yog, lub ntsiab lus ntawm cov txheej txheem chromosomes. Cov nram qab no chromosomal abnormalities yuav pom:
- Lub karyotype class rau Turner Syndrome yog 45X, uas yog, qhov tsis tuaj yeem ntawm ib tus X chromosome. Karyotype no tau pom muaj ntau tshaj 50% ntawm cov neeg mob, thiab hauv 80% ntawm cov neeg mob tsis muaj leej txiv X chromosome.
- Mosaic - uas yog, kev puas tsuaj rau thaj tsam ntawm ib los yog ntau dua chromosomes hauv ib hom mosaic.
- Txuj ua dua tshiab ntawm ib qho ntawm X chromosomes: Xaws Xomromos, poob ntawm chromosome ntawm luv luv los ntev caj npab.
Syshrome Shereshevsky-Turner - cov tsos mob
Feem ntau qeeb hauv kev loj hlob ntawm lub cev tseem ceeb thaum yug - qhov no yog qhov siab me me thiab qhov hnyav ntawm tus me nyuam, nws tseem tuaj yeem deform lub pob ntawm lub cev (lawv yog qhov tob hauv), o ntawm taw thiab xib teg, thiab muaj cov tawv nqaij ntawm daim tawv nqaij.
Yog tias Turner's syndrome tsis kuaj sai tom qab yug me nyuam, tom qab ntawd nws ua rau nws tus kheej tshwm sim hauv daim ntawv ntawm cov yeeb yam nram no:
- kev loj hlob ruam tuaj, feem ntau qhov kev loj hlob tus nqi poob qis tom qab peb lub xyoos;
- chamfered txo qis;
- luv luv lub caj dab, feem ntau nrog leathery folds;
- ib txoj kab uas tsis tshua muaj hau kev loj hlob hauv cheeb tsam lub cheeb tsam;
- dav hauv siab nrog dav spaced, tej zaum deformed cov txiv mis;
- tawm thiab txawv txav ntawm lub auricle, kab mob pob ntseg nquag;
- qeeb hauv puberty;
- underdeveloped mis rau thaum tiav hluas (ze li 13 xyoos);
- tsis muaj kev coj khaub ncaws.
Kwv yees li ntawm 90 feem pua ntawm cov menyuam uas muaj Turner's syndrome muaj ib lub tsev menyuam thiab cov zes qe menyuam muaj qis qis dua, thiab lawv yog cov neeg tsis paub qab hau thiab kho hormonal uas pab tua cov qeeb hauv kev loj hlob.
Kev ncua sij hawm nyob rau hauv kev txawj ntse feem ntau tsis pom, txawm hais tias muaj qhov tsis meej tsis meej yog qhov ua tau thiab, nyob rau qee zaus, muaj qee cov teeb meem cuam tshuam nrog kev paub tu nrho cov kev kawm uas yuav tsum muaj kev paub ntau ntxiv.
Syndrome Shereshevsky-Turner - kev kho mob
Lub hom phiaj tseem ceeb ntawm txoj kev kho mob muaj nyob ntawm Turner's syndrome yog kom paub meej txog kev loj hlob thiab kev sib deev ntawm tus ntxhais . Tus kab mob ntxov no tau pom thiab qhov kev kho mob tau pib, qhov muaj feem ntau rau kev loj hlob ntawm tus neeg mob.
Rau qhov no, ua ntej, hormone kho yog siv, thiab nrog rau puberty lub sij hawm, tus poj niam hormone, estrogen, ntxiv rau nws.
Tom qab ua tiav tiav puberty, ua ke nrog hormone replacement therapy lossis estrogen thiab progestin therapy yog ua tiav.
Dua li ntawm qhov tseeb hais tias thaum lub sij hawm cov neeg mob kho mob muaj peev xwm tsim tau ib txwm thiab coj tus cwj pwm coj zoo li qub, lawv feem ntau tsis ua haujlwm li. Lub peev xwm yug me nyuam rau ib tug me nyuam nrog kev siv cov kev kho yog muaj nyob hauv tsuas yog 10% ntawm cov poj niam uas muaj lub npe ntawm Turner's syndrome, thiab tom qab ntawd nrog karyotype hauv ib hom mosaic.