Qhov txawv ntawm Ehlers Danlos thiab Marfan syndrome yog tias Ehlers Danlos syndrome feem ntau cuam tshuam rau ntawm daim tawv nqaij, pob qij txha, thiab cov hlab ntsha, thaum Marfan syndrome feem ntau cuam tshuam rau lub plawv, qhov muag, cov hlab ntsha, thiab pob txha.
Ehlers Danlos thiab Marfan syndrome yog ob qhov sib txawv ntawm cov kab mob sib txuas. Muaj nyob ib ncig ntawm 200 qhov sib txawv nyob rau hauv cov kab mob sib txuas ntawm cov nqaij mos. Qee qhov kev paub zoo tshaj plaws ntawm cov kab mob sib txuas ua ke yog Ehlers Danlos syndrome, Marfan syndrome, Loeys Dietz syndrome, cutis laxa, Stickler syndrome, thiab thoracic aortic artery dilatation thiab dissection.
Ehlers Danlos Syndrome yog dab tsi?
Ehlers Danlos syndrome yog ib qho kev sib txuas ntawm cov ntaub so ntswg uas feem ntau cuam tshuam rau ntawm daim tawv nqaij, pob qij txha, thiab cov hlab ntsha. Muaj 14 subtypes ntawm qhov teeb meem no. Cov ntaub so ntswg sib txuas yog cov sib xyaw ua ke ntawm cov protein thiab cov khoom uas muab elasticity rau cov qauv hauv qab hauv tib neeg lub cev. Ib hom mob hnyav ntawm tus kab mob no hu ua vascular Ehlers Danlos syndrome, uas ua rau cov phab ntsa ntawm cov hlab ntsha, txoj hnyuv, thiab lub tsev menyuam tawg. Cov tsos mob thiab cov tsos mob ntawm Ehlers Danlos Syndrome muaj xws li cov pob qij txha yooj yooj yim, tawv nqaij tawv, tawv nqaij tawv, cov ntsej muag txawv xws li lub qhov ntswg nyias, nyias daim di ncauj, pob ntseg me me, thiab lub qhov muag tseem ceeb, cov tawv nqaij tawv nqaij uas bruises, tsis muaj zog aorta uas zoo li rupture., thiab cov phab ntsa tsis muaj zog ntawm lub tsev menyuam thiab cov hnyuv loj uas zoo li tawg.
Daim duab 01: Ehlers Danlos Syndrome
Kev hloov pauv hauv tsawg kawg 20 cov noob, suav nrog COL5A1, COL5A2, thiab tsis tshua muaj COL1A1 cov noob, tau cuam tshuam ua rau Ehlers Danlos syndrome. Ntxiv mus, Ehlers Danlos syndrome tuaj yeem kuaj tau los ntawm tsev neeg keeb kwm, kuaj lub cev, thiab kuaj caj ces. Tsis tas li ntawd, kev kho mob rau Ehlers Danlos syndrome muaj xws li tshuaj (mob mob (acetaminophen), ntshav siab), kho lub cev, thiab phais thiab lwm yam txheej txheem.
Marfan Syndrome yog dab tsi?
Marfan syndrome yog ib qho kev sib txuas ntawm cov ntaub so ntswg uas feem ntau cuam tshuam rau lub plawv, qhov muag, cov hlab ntsha, thiab pob txha. Cov neeg uas raug kev txom nyem los ntawm Marfan syndrome yog ib txwm siab thiab nyias nrog caj npab ntev, ceg, ntiv tes, thiab ntiv taw. Cov kev puas tsuaj uas tshwm sim los ntawm Marfan syndrome tuaj yeem mob me lossis hnyav. Marfan syndrome yog tshwm sim los ntawm qhov tsis xws luag lossis tshem tawm hauv FBN1 (fibrillin-1) noob. Cov tsos mob thiab cov tsos mob ntawm Marfan Syndrome muaj xws li qhov siab thiab qhov siab, qhov ntev ntawm caj npab, ob txhais ceg, thiab ntiv tes, pob txha caj dab uas feem ntau protrudes sab nraud los yog dips sab hauv, lub siab arched palate, neeg coob coob cov hniav, lub plawv yws yws, qhov ze ze ze, ib qho txawv txav ntawm tus txha nraub qaum, thiab pav ca.
Daim duab 02: Marfan Syndrome
Marfan syndrome tuaj yeem kuaj tau los ntawm tsev neeg keeb kwm, kuaj lub cev, kuaj lub plawv, kuaj qhov muag (slit teeb kuaj, ntsuas qhov muag), thiab kuaj caj ces. Tsis tas li ntawd, cov kev kho mob rau Marfan syndrome yog cov tshuaj (cov tshuaj txo cov ntshav siab), kev kho qhov muag, kev phais, thiab lwm yam khoom (kev kho aortic, phais scoliosis, kho pob txha, phais qhov muag).
Dab tsi zoo sib xws ntawm Ehlers Danlos thiab Marfan Syndrome?
- Ehlers Danlos thiab Marfan syndrome yog ob yam sib txawv ntawm cov kab mob sib txuas.
- Ob leeg yog ntau yam kab mob.
- Lawv feem ntau cuam tshuam rau cov ntaub so ntswg mos ntawm tib neeg lub cev.
- Ob qho kev mob tshwm sim vim kev hloov pauv lossis hloov pauv ntawm cov noob tshwj xeeb.
- Lawv tuaj yeem kho tau los ntawm kev siv tshuaj thiab kev phais.
Qhov txawv ntawm Ehlers Danlos thiab Marfan Syndrome yog dab tsi?
Ehlers Danlos Syndrome yog ib qho kev sib txuas ntawm cov ntaub so ntswg uas feem ntau cuam tshuam rau ntawm daim tawv nqaij, pob qij txha, thiab cov hlab ntsha, thaum Marfan syndrome yog cov kab mob sib txuas uas cuam tshuam feem ntau cuam tshuam rau lub plawv, qhov muag, cov hlab ntsha, thiab pob txha. Yog li, qhov no yog qhov sib txawv tseem ceeb ntawm Ehlers Danlos thiab Marfan syndrome. Tsis tas li ntawd, Ehlers Danlos syndrome yog tshwm sim los ntawm kev hloov pauv hauv cov noob xws li COL5A1, COL5A2, thiab tsis tshua muaj hauv COL1A1. Ntawm qhov tod tes, Marfan syndrome yog tshwm sim los ntawm kev hloov pauv hauv cov noob FBN1 (fibrillin-1).
Cov ntawv qhia hauv qab no nthuav tawm qhov sib txawv ntawm Ehlers Danlos thiab Marfan syndrome hauv daim ntawv teev npe rau kev sib piv ntawm ib sab.
Summary – Ehlers Danlos vs Marfan Syndrome
Ehlers Danlos thiab Marfan syndrome yog ob hom kab mob sib txuas sib txuas (HCTDs). Ehlers Danlos Syndrome feem ntau cuam tshuam rau ntawm daim tawv nqaij, pob qij txha, thiab cov hlab ntsha, ua rau cov pob qij txha yooj yooj yim, tawv nqaij tawv, tawv nqaij tawv, tshwj xeeb ntawm lub ntsej muag xws li nyias lub qhov ntswg, nyias daim di ncauj, pob ntseg me me, lub qhov muag zoo nkauj, daim tawv nqaij tawv nqaij uas bruises, tsis muaj zog aorta, phab ntsa. ntawm lub tsev menyuam, thiab cov hnyuv loj uas zoo li rupture. Ntawm qhov tod tes, Marfan syndrome feem ntau cuam tshuam rau lub plawv, qhov muag, cov hlab ntsha, thiab lub cev pob txha, ua rau lub siab thiab lub cev muaj zog, cov caj npab ntev ntev, ob txhais ceg thiab cov ntiv tes, cov pob txha caj dab uas feem ntau protrudes sab nraud los yog dips sab hauv, lub siab arched palate, cov hniav ntau., lub plawv yws, qhov ze ze, qhov txawv txav ntawm tus txha nraub qaum, thiab cov ko taw tiaj tus. Yog li, qhov no qhia txog qhov sib txawv ntawm Ehlers Danlos thiab Marfan syndrome.
