Puas muaj tej lub sij hawm koj xav tias koj cov pob txha tsis muaj zog thiab tawg yooj yim dua li lwm cov? Koj puas tsoo koj cov pob txha txawm tias yog ib yam me me xwb los xij? Los yog koj tus menyuam cov hniav me puas poob ua ntej lub sij hawm? Hnub no peb yuav tham txog ib qho mob uas peb tsis tham txog ntau, tab sis nws tseem ceeb heev uas yuav tsum paub txog. Qhov no hu ua Hypophosphatasia, lossis luv luv HPP.
Hypophosphatasia (HPP) yog dab tsi tiag?
Hais yooj yim xwb, Hypophosphatasia (HPP) yog ib yam mob uas tsis tshua muaj, uas tau txais los ntawm noob caj ces (genetic) uas cuam tshuam rau txoj kev uas peb cov pob txha thiab cov hniav loj hlob. Yuav kom peb cov pob txha muaj zog thiab peb cov hniav pab peb zom thiab zom zaub mov kom zoo, lawv xav tau cov zaub mov xws li calcium thiab phosphorus kom ntxiv rau hauv lawv. Cov txheej txheem no hu ua "mineralization".
Hauv HPP, cov txheej txheem no hu ua 'mineralization' tsis tshwm sim kom raug. Yog li ntawd, cov pob txha thiab cov hniav tsis muaj zog txaus, tab sis ua mos thiab yooj yim tawg. Txawm hais tias qhov mob no tsuas yog cuam tshuam rau ob peb tus neeg xwb, nws tuaj yeem yog qhov mob hnyav, txaus ntshai rau lwm tus.
Dab tsi ua rau HPP?
Qhov no yog ib qho teeb meem txog kev tshawb fawb me ntsis, tab sis cia peb nkag siab yooj yim. Xav txog tias peb lub cev zoo li lub Hoobkas loj. Txhua yam yuav tsum tau ua kom zoo. Yuav kom cov pob txha muaj zog, peb xav tau cov zaub mov calcium thiab phosphorus uas peb tau hais ua ntej.
Tiamsis cov txheej txheem 'mineralization' no ntau dhau kuj tsis zoo thiab. Piv txwv li, nws tsis zoo rau cov minerals no kom sib sau ua ke hauv cov ntshav. Yog li peb muaj cov tshuaj lom neeg hauv peb lub cev los tswj qhov no.
Tiamsis cov pob txha thiab cov hniav yuav tsum muaj cov zaub mov no kom txaus. Muaj ib qho enzyme tshwj xeeb hauv peb lub cev uas pab ua txoj haujlwm tseem ceeb no. Nws hu ua alkaline phosphatase (ALP) . Cov enzyme no ua rau cov tshuaj lom neeg hauv cov pob txha thiab cov hniav tsis ua haujlwm uas tiv thaiv kev sib sau ua ke ntawm cov zaub mov ntawd. Tom qab ntawd cov zaub mov tsim nyog tau sib sau ua ke hauv cov pob txha thiab cov hniav, ua rau lawv muaj zog.
Cov neeg uas muaj tus kab mob HPP muaj kev hloov pauv hauv cov noob hu ua 'ALPL', uas ua rau cov enzyme ALP tsis tsim tau zoo. Qhov no ua rau cov tshuaj uas tiv thaiv kev sib sau ua ke ntawm cov zaub mov kom sib sau ua ke hauv cov pob txha, ua rau cov pob txha tsis nqus tau cov calcium thiab phosphorus uas lawv xav tau. Yog li ntawd, cov pob txha mos thiab qaug zog es tsis muaj zog.
Cov tsos mob ntawm HPP yog dab tsi?
Cov tsos mob ntawm HPP sib txawv heev. Lawv nyob ntawm seb muaj pes tsawg tus ALP enzyme nyob hauv lub cev. Thaum cov enzyme txo qis, cov tsos mob feem ntau yuav hnyav dua. Tus mob no tuaj yeem tshwm sim txhua lub sijhawm txij thaum yug los txog rau thaum laus.
Thaum qee tus neeg laus raug kuaj pom tias muaj tus kab mob HPP, lawv nco qab tias lawv muaj cov tsos mob zoo sib xws txij li thaum yau, tab sis nws tsis tau raug kuaj pom zoo rau lub sijhawm ntawd.
Cia peb saib seb cov tsos mob tseem ceeb yog dab tsi.
| Cov tsos mob | Kev piav qhia |
|---|---|
| Teeb meem hniav | Poob hniav menyuam ua ntej hnub nyoog 5 xyoos lossis poob hniav tsis tau xav txog thaum muaj hnub nyoog li cas los xij. (Qhov no yog hom uas feem ntau pom - hom "odonto") |
| Kev hloov pauv hauv cov pob txha | Tes thiab ceg khoov, siab luv, pob txha mos, tsis muaj zog lossis tsis zoo, pob qij txha dab teg thiab pob luj taws dav. |
| Teeb meem menyuam yaus | Tsis tau nce lossis loj hlob zoo, kev sib koom ua ke ntawm cov pob txha taub hau ua ntej lub sijhawm (uas tuaj yeem ua rau muaj kev nyuaj siab ntxiv rau lub hlwb), cov leeg nqaij tsis muaj zog (hypotonia) uas ua rau cov menyuam mos zoo li 'fluffy', thiab ua pa nyuaj. |
| Cov pob txha tawg | Cov pob txha yooj yim tawg thaum tseem hluas, tshwj xeeb tshaj yog rau ntawm ko taw thiab ceg, thiab lawv siv sijhawm ntev heev los kho. |
| Lwm yam nta | Mob pob txha thiab pob qij txha, nyuaj taug kev, muaj calcium ntau ntxiv hauv cov ntshav (tej zaum yuav ua rau ntuav, raws plab, teeb meem raum), qaug dab peg, mob pob qij txha hnyav tam sim ntawd. |
Yuav kuaj mob li cas?
Yog koj lossis koj tus menyuam muaj cov tsos mob no, koj tus kws kho mob yuav nug txog koj tsev neeg keeb kwm kev mob nkeeg thiab ua kev kuaj lub cev tag nrho. Tsis tas li ntawd, lawv yuav xaj kom thaij duab X-ray thiab kuaj ntshav.
Qhov kev kuaj tseem ceeb tshaj plaws ntawm no yog ntsuas qib ntawm ALP enzyme hauv cov ntshav.Hauv qhov xwm txheej ntawm HPP, qib no feem ntau qis.
Qee zaum, kev kuaj noob caj ces tuaj yeem ua tau los xyuas kom meej tias koj puas muaj HPP. Txawm li cas los xij, qhov kev kuaj no tsis muaj nyob txhua qhov chaw thiab tuaj yeem kim heev. Txawm li cas los xij, feem ntau, koj tus kws kho mob tuaj yeem kuaj mob nrog lwm yam kev kuaj.
Vim tias qhov no yog ib yam kab mob tsis tshua muaj, qee zaum nws yuav siv sijhawm ntev me ntsis kom paub tseeb tias muaj mob. Yog li ntawd, yog tias koj lossis koj tus menyuam muaj cov tsos mob no, nws yog ib qho tseem ceeb kom paub zoo thiab tham nrog koj tus kws kho mob .
Cov kev kho mob rau HPP yog dab tsi?
Zoo hmoo, tam sim no muaj kev kho mob rau HPP. Rau HPP, tshwj xeeb tshaj yog cov neeg uas kuaj pom tias muaj tus kab mob no thaum yau lossis thaum yau, siv cov tshuaj hu ua asfotase alfa (Strensiq) . Qhov no yog kev txhaj tshuaj rau hauv qab daim tawv nqaij. Nws ua haujlwm los ntawm kev hloov cov enzyme ALP uas lub cev tsis muaj (enzyme replacement therapy).
Ntxiv rau qhov kev kho mob tseem ceeb no, muaj ntau yam ua los tswj cov tsos mob.
- Muab cov tshuaj tua kab mob (NSAIDs) xws li paracetamol lossis ibuprofen rau mob pob txha lossis mob pob qij txha.
- Yog tias qhov siab hauv lub taub hau siab, phais kom txo nws.
- Muab vitamin B6 los tswj kev qaug dab peg rau qee tus neeg.
- Kev tswj kev noj zaub mov lossis tshuaj kom tswj tau cov calcium hauv cov ntshav.
- Ib txwm saib xyuas koj txoj kev noj qab haus huv ntawm cov hniav thiab nrhiav kev qhia txog kev kho hniav.
- Kev kho lub cev ua rau cov leeg muaj zog thiab txhim kho kev txav mus los.
- Muab cov pas hlau ntxig rau hauv cov pob txha uas feem ntau tawg.
- Muaj ib qho uas yuav tsum nco ntsoov tshwj xeeb yog tias cov tshuaj bisphosphonate, uas siv los kho lwm yam kab mob pob txha, xws li osteoporosis, tuaj yeem ua rau HPP hnyav dua. Yog li ntawd, yuav tsum tsis txhob siv cov tshuaj no.
Cov kev kho mob no yuav kim heev, yog li nws tseem ceeb heev uas yuav tsum nrog koj tus kws kho mob tham txog qhov zoo, qhov tsis zoo, thiab tus nqi ntawm cov kev kho mob.
Tau txais kev txhawb nqa thaum nyob nrog HPP
Cov pob txha tawg, mob, thiab lwm yam tsos mob tuaj yeem ua rau lub neej txhua hnub nyuaj. Kev nyob nrog ib yam kab mob tsis tshua muaj txhais tau tias paub zoo txog koj tus mob thiab hais lus rau koj tus kheej. Qhov no yuav siv tau rau koj lossis koj tus menyuam.
To taub thiab hwm koj lub cev tsis muaj zog. So thaum twg koj xav tau. Kev kho mob HPP feem ntau xav tau pab pawg neeg ntau yam kev kawm. Qhov no yuav suav nrog kws kho mob menyuam yaus, kws phais pob txha, kws kho hniav, thiab kws kho lub cev.
Muaj lwm yam kab mob uas feem ntau muaj cov tsos mob zoo ib yam li HPP, yog li ntawd, yog tias koj tsis paub meej txog koj tus kws kho mob qhov kev kuaj mob, tsis txhob ntshai kom tau txais kev pom zoo thib ob los ntawm lwm tus kws tshwj xeeb.Nws yog koj txoj cai.
Txawm hais tias kev kho mob yuav siv sijhawm ntau xyoo los xij, cov tsos mob tuaj yeem tswj tau zoo heev raws sijhawm.
Cov Lus Qhia Txog Kev Coj Mus Tsev
- Hypophosphatasia (HPP) yog ib yam kab mob tsis tshua muaj, uas yog los ntawm noob caj ces uas ua rau cov pob txha thiab cov hniav tsis muaj zog.
- Cov tsos mob yuav txawv ntawm ib tus neeg mus rau ib tus neeg, xws li cov hniav poob ua ntej lub sijhawm ntawm cov menyuam yaus mus rau cov pob txha puas tsuaj loj heev.
- Kev kuaj ntshav kom paub seb puas muaj cov enzyme ALP qis hauv cov ntshav yog qhov tseem ceeb heev rau kev kuaj mob.
- Tam sim no, muaj cov kev kho mob zoo uas tswj cov tsos mob, suav nrog kev kho mob hloov enzyme.
- Yog koj lossis koj tus menyuam muaj cov tsos mob no, tsis txhob ntshai thiab nrog koj tus kws kho mob tham tam sim ntawd. Kev kuaj mob thiab kho mob kom raug yog qhov tseem ceeb heev.
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Tsis muaj lus teb tau muab tso tawm. Ntxiv koj cov lus tawm ntawm no thawj zaug.
Ntxiv koj cov lus