Dzimwe nguva unonzwa sekuti mapfupa ako haana simba uye haana kusimba kupfuura mamwe here? Unotyora mapfupa ako nechinhu chidiki here? Kana kuti mazino emwana wako anobuda nguva isati yakwana here? Nhasi tichataura nezvechirwere chatisingataure zvakawanda nezvacho, asi chakakosha kuziva nezvacho. Izvi zvinonzi Hypophosphatasia, kana kuti HPP muchidimbu.
Chii chinonzi Hypophosphatasia (HPP) chaizvo?
Zvichitaurwa zviri nyore, Hypophosphatasia (HPP) chirwere chisingawanzoitiki, chinogara nhaka (majini) chinokanganisa kukura kwemapfupa nemazino edu. Kuti mapfupa edu ave akasimba uye mazino edu atibatsire kutsenga nekutsenga chikafu nemazvo, anoda maminerari akaita secalcium nephosphorus kuti awedzerwe maari. Maitiro aya anonzi "mineralization".
MuHPP, maitiro aya anonzi `mineralization` haaitike nemazvo. Nekuda kweizvozvo, mapfupa nemazino hazvina kusimba zvakakwana, asi zvinova zvakapfava uye zvinopera simba nyore nyore. Kunyange zvazvo chirwere ichi chingangobata vanhu vashoma chete , chinogona kuva chirwere chakakomba, chinoisa hupenyu panjodzi kune vamwe.
Chii chinokonzera HPP?
Iyi inyaya yesainzi, asi ngatiinzwisise zviri nyore. Fungidzira kuti muviri wedu wakaita sefekitori hombe. Zvese zvinofanira kunge zvakarongeka. Kuti mapfupa asimbiswe, tinoda mamineral ecalcium nephosphorus atataura kare.
Asi kuwanda kwemaitiro aya e "mineralization" hakuna kunakawo. Semuenzaniso, hazvina kunaka kuti zvicherwa izvi zviunganidzwe muropa. Saka tine makemikari mumiviri yedu anotonga izvi.
Asi mapfupa nemazino zvinofanira kuva nehuwandu hwakakodzera hwezvicherwa izvi. Kune enzyme yakakosha mumuviri wedu inobatsira nebasa iri rinokosha. Inonzi alkaline phosphatase (ALP) . Iyi enzyme inodzima makemikari ari mumapfupa nemazino anodzivirira kuungana kwezvicherwa izvozvo. Zvadaro zvicherwa zvinodiwa zvinounganidzwa mumapfupa nemazino, zvichiita kuti zvisimbe.
Vanhu vane HPP vane shanduko mujini inonzi `ALPL`, iyo inodzivirira enzyme yeALP kuti isagadzirwe nemazvo. Izvi zvinoita kuti makemikari anodzivirira kuungana kwemaminerari mumapfupa, zvichidzivirira mapfupa kuti asatore calcium nephosphorus yaanoda. Nekuda kweizvozvo, mapfupa anova akapfava uye asina simba panzvimbo pekuva akasimba.
Ndezvipi zviratidzo zveHPP?
Zviratidzo zveHPP zvinosiyana zvikuru. Zvinoenderana nekuti enzyme yeALP yakawanda sei mumuviri. Sezvo huwandu hweenzyme huchidzikira, zviratidzo zvinowanzowedzera kuoma. Dambudziko iri rinogona kuonekwa chero nguva kubva pakuzvarwa kusvika pakuva munhu mukuru.
Kana vamwe vakuru vakaonekwa vaine HPP, vanoyeuka kuti vaiva nezviratidzo zvakafanana kubvira vachiri vadiki, asi hazvina kuongororwa zvakanaka panguva iyoyo.
Ngationei kuti zviratidzo zvikuru ndezvipi.
| Chiratidzo | Tsananguro |
|---|---|
| Matambudziko emazino | Kurasikirwa nemazino emwana asati asvitsa makore mashanu kana kurasikirwa nemazino asingatarisirwi pazera ripi zvaro. (Iyi ndiyo fomu inonyanya kuwanikwa - fomu rekuti "odonto") |
| Kuchinja kwemapfupa | Maoko nemakumbo akakotama, mupfupi, mapfupa akapfava, asina simba kana akaremara, maoko akafara uye majoini ezvigunwe. |
| Matambudziko evana vacheche | Kukundikana kukura zvakanaka, kusangana kwemapfupa edehenya nguva isati yakwana (izvo zvinogona kukonzera kumanikidzwa kwakanyanya kuuropi), kushaya simba kwetsandanyama (hypotonia) kunoita kuti vacheche vataridzike se'vasina kusimba', uye kuoma kufema. |
| Kutyoka | Mapfupa anotyoka zviri nyore achiri mudiki, kunyanya mutsoka nemakumbo, uye anotora nguva yakareba kuti apore. |
| Zvimwe zvinhu | Kurwadziwa mumapfupa nemapfundo, kuoma kufamba, kuwedzera kwecalcium muropa (zvinogona kukonzera kurutsa, kuvimbiwa, matambudziko eitsvo), pfari, arthritis yakakomba kamwe kamwe. |
Nzira yekuziva sei chirwere ichi?
Kana iwe kana mwana wako muine zviratidzo izvi, chiremba wenyu achabvunza nezvenhoroondo yemhuri yenyu yehutano uye oita ongororo yakakwana yemuviri. Pamusoro pezvo, vacharaira kuti muongororwe X-rays uye ropa.
Chinonyanya kukosha pano ndechekuyera huwandu hweALP enzyme muropa.Kana iri HPP, huwandu uhwu hunowanzova hwakaderera.
Dzimwe nguva, bvunzo dzemajini dzinogona kuitwa kuti zvionekwe kana uine HPP. Zvisinei, bvunzo iyi haiwanikwe kwese kwese uye inogona kudhura. Zvisinei, kazhinji, chiremba wako anogona kuona chirwere ichi nemimwe miedzo.
Nekuti ichi chirwere chisingawanzoitiki, dzimwe nguva zvinotora nguva kuti chiongororwe. Saka kana iwe kana mwana wako muine zviratidzo izvi, zvakakosha kuti muve neruzivo rwakakwana uye mutaure pachena nachiremba wenyu .
Ndeapi marapirwo eHPP?
Nerombo rakanaka, ikozvino kune mishonga yeHPP. Kune HPP, kunyanya avo vakaonekwa vachiri vacheche kana kuti vari vadiki, mushonga unonzi asfotase alfa (Strensiq) unoshandiswa. Uyu ijekiseni rinopihwa pasi peganda. Unoshanda nekutsiva enzyme yeALP iyo isipo mumuviri (enzyme replacement therapy).
Kuwedzera pakurapa uku kukuru, pane zvimwe zvinhu zvakawanda zvinoitwa kudzora zviratidzo.
- Kupa mishonga inoderedza marwadzo (NSAIDs) yakaita separacetamol kana ibuprofen yekurwadziwa kwemapfupa kana majoini.
- Kana kumanikidzwa kwedehenya kwakakwira, kuvhiyiwa kuderedza.
- Kupa vamwe vanhu vhitamini B6 kuti vaderedze pfari.
- Kudzora kudya kana mishonga yekudzora huwandu hwecalcium muropa.
- Gara uchitarisira hutano hwemazino ako uye tsvaga mazano emazino.
- Kurapa kwemuviri kunosimbisa tsandanyama uye kunovandudza kufamba.
- Kuisa simbi mumapfupa anotyoka-tyoka.
- Chimwe chinhu chekurangarira zvakanyanya ndechekuti mishonga yemhando yebisphosphonate, iyo inoshandiswa kurapa zvimwe zvirwere zvemapfupa, zvakaita se osteoporosis, inogona kuwedzera HPP. Saka, inofanira kudzivirirwa.
Kurapa uku kunogona kudhura, saka zvakakosha kutaura nachiremba wako nezvezvakanakira, zvakaipa, uye mari yekurapa.
Kuwana rutsigiro paunenge uchirarama neHPP
Kutyoka, kurwadziwa, nezvimwe zviratidzo zvinogona kuita kuti hupenyu hwezuva nezuva huome. Kurarama nechirwere chisingawanzoitiki zvinoreva kuva neruzivo rwakakwana nezvechirwere chako uye kutaura uchizvidzivirira. Izvi zvinogona kushanda kwauri kana kumwana wako.
Nzwisisa uye remekedza zvipingamupinyi zvako zvemuviri. Zorora paunenge uchida. Kurapa HPP kunowanzoda timu yevashandi vane hunyanzvi hwakasiyana-siyana. Izvi zvinogona kusanganisira chiremba wevana, chiremba wemapfupa, chiremba wemazino, uye chiremba wemuviri.
Kune zvimwe zvirwere zvakajairika zvine zviratidzo zvakafanana neHPP, saka kana usina chokwadi nezvekuongororwa kwachiremba wako, usatya kuwana imwe pfungwa kubva kune imwe nyanzvi.Ikodzero yako.
Kunyange zvazvo kurapwa kuchigona kutora makore, zviratidzo zvinogona kudzorwa zvakanyanya nekufamba kwenguva.
Mharidzo Yekuenda Nayo Kumba
- Hypophosphatasia (HPP) chirwere chisingawanzoitiki, chinogara nhaka chinopedza simba mapfupa nemazino.
- Zviratidzo zvinogona kusiyana zvikuru kubva pamunhu kusvika pamunhu, kubva pakurasikirwa nezino nguva isati yakwana muvana vadiki kusvika pakuremara kwemapfupa kwakanyanya.
- Kuongororwa ropa kuti uone kana enzyme yeALP yakaderera muropa kwakakosha zvikuru pakuongororwa kwechirwere.
- Parizvino, kune mishonga inoshanda inodzora zviratidzo, kusanganisira kurapa kwekutsiva ma enzyme.
- Kana iwe kana mwana wako muine zviratidzo izvi, musavhunduka uye taurai nachiremba wenyu nekukasika. Kuwana chirwere chakakodzera uye kurapwa kwakakosha zvikuru.
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