Koj cov hniav puas zoo li tsis muaj zog? Lawv puas ploj xim? Qhov no yuav yog Dentinogenesis Imperfecta!

Koj puas tau pom tias qee tus neeg cov hniav muaj xim txawv txawv me ntsis, tej zaum yog xim xiav-grey, lossis xim daj-xim av? Los yog koj puas muaj cov hniav uas tsis muaj zog heev uas lawv tawg lossis hnav txawm tias koj noj ib yam dab tsi me me? Tej zaum koj yuav xav tias qhov ntawd yog qhov ib txwm muaj. Txawm li cas los xij, qhov no kuj tseem yuav yog vim muaj ib qho mob tsis tshua muaj uas tshwm sim thaum lub sijhawm tsim cov hniav hu ua '(Dentinogenesis Imperfecta)'. Cia peb tham txog qhov no me ntsis hnub no, puas yog?

`(Dentinogenesis Imperfecta)` yog dab tsi? Cia peb nkag siab yooj yim, puas yog?

Hais yooj yim xwb, '(Dentinogenesis Imperfecta)' yog ib qho teeb meem uas tshwm sim thaum cov hniav tsim, uas yog, thaum cov hniav loj hlob. Qhov tshwm sim hauv qhov no yog tias koj cov hniav yuav tsis muaj zog thiab yooj yim tawg . Ib yam li lub ncuav qab zib, yog tias koj tsoo nws me ntsis, nws yuav tawg. Tsis tas li ntawd, cov hniav ntawm cov neeg uas muaj tus mob no yog me ntsis translucent . Xim kuj yog, raws li kuv tau hais ua ntej , xiav-grey lossis daj-xim av .

Qhov mob no, hu ua "Dentinogenesis Imperfecta," tuaj yeem cuam tshuam rau koj cov hniav tseem ceeb thiab koj cov hniav tas mus li . Qhov no txhais tau tias cov tsos mob tuaj yeem pib thaum koj tseem hluas, lossis lawv tuaj yeem tshwm sim tom qab hauv koj lub neej.

Tam sim no saib, thaum peb rho ib tug hniav, nws muaj ntau txheej. Qhov ci ntsa iab, muaj zog nyob rau sab saum toj hu ua '(Enamel)'. Hauv qab ntawd '(Enamel)' muaj lwm txheej, uas hu ua '(Dentin)'. Qhov '(Dentin)' no yog qhov uas feem ntau muab lub zog thiab xim rau cov hniav. Qhov tshwm sim hauv '(Dentinogenesis Imperfecta)' yog tias qhov '(Dentin)' tsis loj hlob zoo. Tom qab ntawd yuav ua li cas? Vim tias '(Dentin)' tsis muaj zog, '(Enamel)' nyob rau sab saum toj tsis txuas nrog cov hniav zoo. Yog vim li cas '(Enamel)' yooj yim tawg thiab hnav tawm.

Tseem Ceeb: `(Dentinogenesis Imperfecta)` txawv ntawm `(Amelogenesis Imperfecta)`. Hauv `(Amelogenesis Imperfecta)`, `(Enamel)` ntawm cov hniav raug cuam tshuam ncaj qha. Hauv `(Dentinogenesis Imperfecta)`, qhov teeb meem nyob hauv `(Dentin)`.

Qhov no yog ib qho mob tsis tshua muaj . Feem ntau, qhov mob no tshwm sim rau li ib ntawm 6,000 txog 8,000 tus neeg.

Puas muaj hom kab mob '(Dentinogenesis Imperfecta)'?

Yog, peb tab tom tham txog peb hom tseem ceeb ntawm `(Dentinogenesis Imperfecta)`. Cia peb saib seb lawv yog dab tsi.

1. `(Dentinogenesis Imperfecta)` Hom 1 (Hom I): Qhov no yog qhov tshwj xeeb. Vim tias hom no tshwm sim nrog ib yam kab mob hu ua `(Osteogenesis Imperfecta)`. `(Osteogenesis Imperfecta)` tsuas yog ib yam kab mob uas ua rau pob txha tsis muaj zog heev (kab mob pob txha tawg). Yog li, nrog rau cov teeb meem hniav, cov neeg no kuj muaj teeb meem pob txha. Qhov laj thawj rau qhov no yog tias muaj qee qhov kev hloov pauv hauv `(Genes)` ntawm `(COL1A1)` lossis `(COL1A2)` noob. Ob qho noob no pab ua cov protein `(Collagen)` hauv peb lub cev.

2. `(Dentinogenesis Imperfecta)` Hom 2 (Hom II): Hom no feem ntau tshwm sim ntau dua li cov hniav tas mus li.Nws feem ntau tshwm sim rau ntawm cov hniav menyuam yaus. Cov neeg no feem ntau muaj tsawg dua lwm yam mob uas tau los ntawm noob caj noob ces. Txawm li cas los xij, nws tau pom tias qee zaum muaj qhov ua tau ntawm kev hnov ​​​​​​lus tsis zoo uas cuam tshuam nrog hnub nyoog .

3. `(Dentinogenesis Imperfecta)` Hom 3 (Hom III): Qhov no yog hom tsis tshua muaj . Nws cuam tshuam rau cov hniav tas mus li ntau dua li cov hniav menyuam. Cov kws tshawb fawb tau tshaj tawm tias hom no feem ntau ntawm cov neeg Yudais hu ua `(Ashkenazi Jewish)`, thiab hauv ntau tsev neeg nyob rau sab qab teb Maryland.

Cov tsos mob ntawm `(Dentinogenesis Imperfecta)` yog dab tsi? Yuav ua li cas thiaj paub nws?

Zoo li kuv tau hais ua ntej lawm, cov tsos mob tseem ceeb yog cov xim tsis zoo thiab cov hniav tsis muaj zog, tawg yooj yim . Cov hniav feem ntau pom tseeb, nrog rau xim daj-xim av lossis xiav-grey .

Tsis tas li ntawd xwb, koj kuj tseem yuav pom lwm cov tsos mob xws li:

• Cov hniav menyuam poob lig: Cov hniav menyuam poob lig dua li qub.
• Kev puas tsuaj ntawm cov hniav cuav: Qhov no txhais tau tias cov txheej enamel ntawm sab saum toj ntawm cov hniav tau ploj mus txaus lawm.
• Kev Mob Hlwb Ntau Dhau: Ib txhia neeg tuaj yeem khoov lawv cov pob qij txha, xws li lawv cov ntiv tes thiab lub luj tshib, rov qab thiab tawm ntau dhau.
• Hniav Agenesis: Qhov no txhais tau tias yug los tsis muaj qee cov hniav.
• Cov hniav enamel tsis muaj zog: Cov enamel tsis muaj zog thiab lwj sai sai.

Xav txog seb, yog tias cov hniav menyuam yaus ntawm ib tug menyuam yaus muaj xim txawv txawv, muaj xim xiav, lossis tawg yooj yim, koj yuav tsum txhawj xeeb txog lawv.

Vim li cas qhov '(Dentinogenesis Imperfecta)' no thiaj tshwm sim? Dab tsi yog qhov ua rau?

Qhov laj thawj tseem ceeb rau qhov no yog kev hloov pauv hauv peb cov noob caj noob ces. Tshwj xeeb, kev hloov pauv hauv cov noob caj noob ces hu ua '(DSPP) ' ua rau '(Dentinogenesis Imperfecta). Cov noob caj noob ces '(DSPP)' no muab cov lus qhia los ua '(Dentin)', txheej nruab nrab ntawm peb cov hniav. Yog li thaum muaj teeb meem nrog cov noob caj noob ces no, '(Dentin)' tsis tsim kom zoo.

Qhov kev hloov pauv caj ces no tuaj yeem tau txais los ntawm niam txiv , lossis nws tuaj yeem tshwm sim yam tsis muaj kev cuam tshuam .

Cov neeg uas muaj Dentinogenesis Imperfecta hom 1, raws li kuv tau hais ua ntej, muaj kev hloov pauv hauv cov noob caj noob ces COL1A1 lossis COL1A2. Cov no yog cov kev hloov pauv caj ces zoo ib yam li cov neeg uas muaj Osteogenesis Imperfecta (ib yam kab mob uas ua rau pob txha tawg).

Cov kws kho mob kuaj mob li cas?

Yog koj lossis koj tus menyuam muaj cov tsos mob no, koj yuav tsum mus ntsib kws kho hniav ua ntej. Nws yuav kuaj lub cev ua ntej thiab nug seb puas muaj leej twg hauv koj tsev neeg tau muaj cov mob no..

Tsis tas li ntawd xwb, yog tias koj xav tias koj muaj Dentinogenesis Imperfecta, cov kev kuaj hauv qab no tuaj yeem ua tau los xyuas kom meej qhov mob:

• Thaij duab X-ray ntawm cov hniav.
• Tej zaum koj tuaj yeem ua CT scan .

Los ntawm kev saib cov duab no, tus kws kho mob tuaj yeem tau txais lub tswv yim zoo txog qhov xwm txheej ntawm sab hauv ntawm cov hniav, qhov xwm txheej ntawm "Dentin".

Qee zaum, koj tus kws kho mob yuav pom zoo kom kuaj xyuas cov noob caj noob ces kom paub tseeb tias qhov kev hloov pauv dab tsi hauv koj cov DNA ua rau dentinogenesis imperfecta.

Cov kev kho mob rau qhov no yog dab tsi? (Kev Kho Mob)

Lub hom phiaj tseem ceeb ntawm kev kho mob rau Dentinogenesis Imperfecta yog ua kom cov hniav uas tsis muaj zog muaj zog . Cov kws kho hniav siv ntau yam txheej txheem kho kom zoo dua qub los ua qhov no.

Piv txwv li:

• Kev txhaws hniav: Sau cov qhov khoob thiab cov kab nrib pleb hauv cov hniav.
• Cov hniav cuav: Cov no yog cov plhaub nyias nyias uas muab tso rau ntawm sab xub ntiag ntawm cov hniav. Lawv kuj tseem tuaj yeem txhim kho qhov tsos ntawm cov hniav.
• Cov kaus mom hniav: Lub kaus mom hniav zoo li lub hau uas muab tso rau ntawm ib tug hniav. Nws npog tag nrho cov hniav thiab ua kom nws muaj zog.

Cov kev kho mob no siv cov khoom siv xim zoo li cov hniav, yog li koj tuaj yeem ua kom koj lub luag nyav zoo nkauj.

Thaum koj muaj Dentinogenesis Imperfecta, koj yuav muaj feem poob hniav ntau dua thaum koj laus zuj zus. Yog tias koj poob hniav, koj tuaj yeem hloov lawv nrog cov hniav cog lossis cov hniav cuav .

Koj muaj kev cia siab li cas thaum koj nyob nrog qhov xwm txheej no? (Outlook)

Koj txoj kev xav nyob ntawm ntau yam:

• Koj muaj hnub nyoog thaum koj raug kuaj pom tias muaj tus kab mob.
• Koj pib kho mob sai npaum li cas.
• Koj muaj hom kab mob Dentinogenesis Imperfecta twg?

Qhov tseem ceeb yog tias Dentinogenesis Imperfecta tsis kho tau . Txawm li cas los xij, nrog kev saib xyuas hniav kom zoo thiab kev tu cev qhov ncauj zoo, koj tuaj yeem pab tiv thaiv kev puas tsuaj ntxiv rau koj cov hniav thiab ua kom lawv muaj zog. Qhov sai dua qhov mob raug kuaj pom thiab kho, qhov tshwm sim zoo dua.

Puas tiv thaiv tau qhov teeb meem Dentinogenesis Imperfecta?

Tu siab kawg li, Dentinogenesis Imperfecta tsis tuaj yeem tiv thaiv tau vim nws yog los ntawm kev hloov pauv caj ces uas peb tsis tuaj yeem tswj tau. Txawm li cas los xij, kev pib kho sai li sai tau tuaj yeem txo qhov kev puas tsuaj rau cov hniav mus sij hawm ntev.

Kuv yuav saib xyuas kuv cov hniav li cas? (Kev Saib Xyuas Tus Kheej)

Yog koj muaj tus kab mob "(Dentinogenesis Imperfecta)", kev tu cev qhov ncauj kom huv yog qhov tseem ceeb heev.Kev saib xyuas yog qhov tseem ceeb. Yuav kom txo tau qhov lwj thiab kev puas tsuaj ntawm cov hniav, ua cov hauv qab no:

• Nug koj tus kws kho hniav txog kev siv tshuaj fluoride los ua kom koj cov hniav khov kho.
• Txhuam koj cov hniav ob lossis peb zaug hauv ib hnub nrog txhuam hniav mos mos thiab tshuaj txhuam hniav uas muaj fluoride .
• Txwv cov khoom noj qab zib thiab cov khoom noj uas muaj kua qaub.
• Yaug koj lub qhov ncauj nrog cov tshuaj fluoride ntxuav qhov ncauj ib hnub ib zaug.
• Mus ntsib kws kho hniav tsis tu ncua kom tau kev kuaj xyuas thiab ntxuav hniav .

Thaum koj muaj "(Dentinogenesis Imperfecta)" noj thiab haus dab tsi zoo?

Qhov zoo tshaj plaws uas yuav tsum ua yog xaiv cov khoom noj thiab dej haus uas muaj qab zib thiab kua qaub tsawg . Qhov no txhais tau tias yuav tsum txo cov dej haus xws li dej qab zib, txiv hmab txiv ntoo xws li txiv qaub ntsuab, thiab cov kua txiv lws suav kom ntau li ntau tau. Nws kuj yog ib lub tswv yim zoo kom txo cov khoom noj uas yuav tsum tau zom thiab tawv lossis nkig .

Nov yog qee cov khoom noj zoo rau ib tus neeg uas muaj Dentinogenesis Imperfecta noj:

• Ntses ci
• Hom Casseroles
• Cov zaub siav lawm
• Qos yaj ywm
• Pasta nrog cov kua ntses cream
• Qe qe kib
• Cov dej qab zib

Cov no yog cov khoom noj uas yooj yim noj thiab tsis ua rau koj cov hniav puas ntau.

Kuv yuav tsum mus ntsib kws kho mob thaum twg?

Yog tias koj lossis koj tus menyuam pom muaj dab tsi txawv txawv lossis txawv txog lawv cov hniav, nws yog qhov zoo tshaj plaws los qhia rau koj tus kws kho mob tam sim ntawd . Nco ntsoov tias, cov tsos mob ntawm DGI tuaj yeem tshwm sim rau txhua lub hnub nyoog. Yog tias koj tus menyuam thawj cov hniav menyuam tshwm xiav, grey, lossis xim av , nco ntsoov mus ntsib kws kho hniav menyuam yaus kom tau txais lus qhia.

Koj yuav tsum nug tus kws kho mob li cas?

Yog tias koj raug kuaj pom tias muaj Dentinogenesis Imperfecta, tej zaum koj yuav xav nug koj tus kws kho mob cov lus nug xws li:

• Kuv muaj hom '(DGI)' dab tsi?
• Kuv xav tau kev kho mob hom twg?
• Cov kev kho mob yuav siv sijhawm ntev npaum li cas? Cov kev kho mob yuav kav ntev npaum li cas?
• Kuv yuav tsum tau ntxuav kuv cov hniav ntau npaum li cas?
• Kuv yuav ua li cas thiaj li tiv thaiv tau cov hniav kom tsis txhob lwj thiab puas tsuaj?

Thaum kawg muaj ib yam dab tsi... (Cov Lus Coj Mus Tsev)

Muaj cov hniav tsis muaj zog thiab yooj yim tawg ua rau koj ntxhov siab heev. Nws kuj ua rau koj nkees heev uas yuav tsum ceev faj txhua zaus koj noj mov. Thaum koj muaj '(Dentinogenesis Imperfecta)', koj yuav txaj muag txog qhov zoo li cas ntawm koj cov hniav. Vim li no, koj yuav tsis xav ntsib zej zog.

Tiamsis nco ntsoov, koj tsis yog tib leeg xwb.Muaj cov kws kho mob uas tuaj yeem pab koj. Lawv tuaj yeem pom zoo cov kev kho mob kom ua rau koj cov hniav muaj zog, txhim kho koj lub luag nyav, thiab txhawb koj txoj kev ntseeg siab rau koj tus kheej. Yog li, tsis txhob txhawj txog dab tsi, thiab ua raws li koj tus kws kho mob cov lus qhia.

Los ntawm kev kuaj mob thaum ntxov, tau txais kev kho mob kom zoo, thiab ua raws li kev coj ua zoo ntawm kev tu cev qhov ncauj, koj tuaj yeem ua neej noj qab nyob zoo thiab zoo siab txawm tias muaj tus mob '(Dentinogenesis Imperfecta)'.

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