Ese hari igihe wumva amagufwa yawe afite intege nke kandi yoroshye kurusha andi? Ese uvunika amagufwa yawe n'akantu gato? Cyangwa amenyo y'umwana wawe avuka imburagihe? Uyu munsi tugiye kuvuga ku ndwara tutavugaho cyane, ariko ni ingenzi cyane kuyimenya. Iyi ndwara yitwa Hypophosphatasia, cyangwa HPP mu magambo ahinnye.
Hypophosphatesia (HPP) ni iki mu by'ukuri?
Muri make, Hypophosphatasia (HPP) ni indwara idakunze kugaragara, ivukanwa (genetique) igira ingaruka ku buryo amagufwa n'amenyo yacu akura. Kugira ngo amagufwa yacu akomere kandi amenyo yacu afashe guhekenya no guhekenya ibiryo neza, akeneye imyunyu ngugu nka kalisiyumu na fosifore kugira ngo yongerwemo. Iyi nzira yitwa "mineralization".
Muri HPP, iyi nzira yitwa `mineralization` ntabwo iba neza. Ingaruka zabyo ni uko amagufwa n'amenyo bidakomera bihagije, ariko bikoroha kandi byoroshye kwangirika. Nubwo iyi ndwara ishobora gufata abantu bake gusa , ishobora kuba ikibazo gikomeye, gishobora no gushyira ubuzima mu kaga ku bandi.
Ni iki gitera HPP?
Iki ni ikibazo cya siyansi, ariko reka tubisobanukirwe mu buryo bworoshye. Tekereza ko umubiri wacu umeze nk'uruganda runini. Ibintu byose bigomba kuba biri mu buryo bukwiye. Kugira ngo amagufwa akomere, dukeneye imyunyu ngugu ya kalisiyumu na fosifore twavuze mbere.
Ariko gukoresha cyane iyi gahunda ya "mineralization" na byo si byiza. Urugero, si byiza ko iyi myunyu ngugu yirundanya mu maraso. Bityo dufite imiti mu mibiri yacu igenzura ibi.
Ariko amagufwa n'amenyo bigomba kugira ingano ikwiye y'iyi myunyu ngugu. Hari enzyme yihariye mu mubiri wacu ifasha muri iki gikorwa cy'ingenzi. Yitwa alkaline phosphatase (ALP) . Iyi enzyme ihagarika imiti iri mu magufwa no mu menyo ibuza iyo myunyu ngugu kwirundanya. Hanyuma imyunyu ngugu ikenewe irundanywa mu magufwa no mu menyo, bigatuma ikomera.
Abantu barwaye HPP bagira impinduka muri gene yitwa `ALPL`, ituma enzyme ya ALP idakorwa neza. Ibi bituma imiti ibuza imyunyu ngugu kwirundanya mu magufwa, ikabuza amagufwa kwinjiza kalisiyumu na fosifore bakeneye. Kubera iyo mpamvu, amagufwa yoroha kandi agacika intege aho gukomera.
Ni ibihe bimenyetso bya HPP?
Ibimenyetso bya HPP biratandukanye cyane. Biterwa n'ingano ya enzyme ya ALP mu mubiri. Uko urugero rwa enzyme rugabanuka, ibimenyetso bikunze kwiyongera cyane. Iyi ndwara ishobora kugaragara igihe icyo ari cyo cyose kuva umuntu avutse kugeza umuntu akuze.
Iyo abantu bakuru bamwe basanzwe barwaye HPP, bibuka ko bagaragaje ibimenyetso nk'ibyo kuva bakiri bato, ariko icyo gihe ntibyari byasuzumwe neza.
Reka turebe ibimenyetso by'ingenzi.
| Ibimenyetso | Ibisobanuro |
|---|---|
| Ibibazo by'amenyo | Gutakaza amenyo y'abana mbere y'imyaka 5 cyangwa gutakaza amenyo mu buryo butunguranye mu myaka iyo ari yo yose. (Ubu ni bwo buryo bukunze kugaragara cyane - ubwoko bwa "odonto") |
| Impinduka mu magufwa | Amaboko n'amaguru binamye, bigufi, amagufwa yoroshye, adakomeye cyangwa afite ubumuga, ingingo nini z'ukuboko n'iz'akaguru. |
| Ibibazo by'impinja | Kunanirwa kwiyongera cyangwa gukura neza, kwivanga kw'amagufwa y'agahanga imburagihe (bishobora gutera umuvuduko mwinshi ku bwonko), intege nke z'imitsi (hypotonia) zituma abana basa n'abadafite ubushobozi bwo guhumeka neza, ndetse no kugorwa no guhumeka. |
| Kuvunika | Amagufwa avunika byoroshye akiri muto, cyane cyane mu birenge no mu maguru, kandi bifata igihe kirekire kugira ngo akire. |
| Ibindi bintu | Kubabara mu magufwa no mu ngingo, kugorwa no kugenda, kwiyongera k'umunyu wa kalisiyumu mu maraso (bishobora gutera kuruka, kuribwa mu nda, ibibazo by'impyiko), gukubita ibisebe, rubagimpande ikabije. |
Ni gute wasuzuma iyi ndwara?
Niba wowe cyangwa umwana wawe mufite ibi bimenyetso, muganga azabaza amateka y’ubuzima bw’umuryango wanyu kandi akore isuzuma ryuzuye ry’umubiri. Byongeye kandi, bazategeka ko bapimwa X-rays n’amaraso.
Ikizamini cy'ingenzi hano ni ugupima urugero rwa enzyme ya ALP mu maraso.Ku bijyanye na HPP, uru rwego rusanzwe ruba ruri hasi.
Hari igihe ikizamini cya genetiki gishobora gukorwa kugira ngo hemezwe niba ufite HPP. Ariko, iki kizamini ntikiboneka ahantu hose kandi gishobora kuba gihenze. Ariko, akenshi, muganga wawe ashobora gusuzuma indwara akoresheje ibindi bizamini.
Kubera ko iyi ari indwara idakunze kugaragara, rimwe na rimwe bishobora gufata igihe kugira ngo imenyekane. Niba wowe cyangwa umwana wawe mufite ibi bimenyetso, ni ngombwa kumenya amakuru neza no kuganira na muganga mu buryo bweruye .
Ni ubuhe buryo bwo kuvura HPP?
Ku bw'amahirwe, ubu hari uburyo bwo kuvura HPP. Kuri HPP, cyane cyane iyo basanzwemo mu bwana cyangwa mu bwana, hakoreshwa umuti witwa asfotase alfa (Strensiq) . Uru ni urushinge rutangwa munsi y'uruhu. Rukora mu gusimbuza enzyme ya ALP ibura mu mubiri (enzyme replacement therapy).
Uretse ubu buryo bw'ingenzi bwo kuvura, hari n'ibindi bintu byinshi bikorwa kugira ngo hagenzurwe ibimenyetso.
- Gutanga imiti igabanya ububabare (NSAIDs) nka parasetamol cyangwa ibuprofen ku bubabare bw'amagufwa cyangwa ingingo.
- Niba umuvuduko uri mu gahanga uri hejuru, opera ibagwe kugira ngo igabanye.
- Guha abantu bamwe na bamwe vitamine B6 kugira ngo bagabanye indwara zo mu mutwe.
- Kugenzura indyo cyangwa imiti yo kugenzura urugero rwa kalisiyumu mu maraso.
- Buri gihe yitaho ubuzima bw'amenyo yawe kandi usabe inama ku buvuzi bw'amenyo.
- Ubuvuzi bw'umubiri bukomeza imitsi kandi bugatuma imyitwarire irushaho kuba myiza.
- Gushyira inkoni z'icyuma mu magufwa akunze kuvunika.
- Ikintu kimwe ugomba kuzirikana by'umwihariko ni uko imiti yo mu bwoko bwa bisphosphonate, ikoreshwa mu kuvura izindi ndwara z'amagufwa, nka osteoporosis, ishobora kongera HPP. Bityo rero, ikwiye kwirindwa.
Ubu buryo bwo kuvura bushobora kuba buhenze, bityo rero ni ngombwa kuganira na muganga wawe ku byiza, ibibi, n'ikiguzi cy'uburyo bwo kuvura.
Kubona ubufasha mu gihe ubana na HPP
Kuvunika, ububabare, n'ibindi bimenyetso bishobora gutuma ubuzima bwa buri munsi bugorana. Kubana n'indwara idakunze kugaragara bivuze kumenya neza uko umeze no kwivugira. Ibi bishobora kuba kuri wowe cyangwa ku mwana wawe.
Sobanukirwa kandi wubahe intege nke zawe z'umubiri. Kuruhuka igihe ubikeneye. Kuvura HPP akenshi bisaba itsinda ry'abantu benshi. Harimo umuganga w'abana, umuganga w'amagufwa, amenyo, n'umuganga w'imitsi.
Hari izindi ndwara zisanzwe zifite ibimenyetso bisa na HPP, bityo rero niba utazi neza uko muganga wawe arwaye, ntutinye gusaba ubufasha bw'undi muganga w'inzobere.Ni uburenganzira bwawe.
Nubwo kuvurwa bishobora gufata imyaka myinshi, ibimenyetso bishobora kugenzurwa cyane uko igihe kigenda gihita.
Ubutumwa bwo kujyana mu rugo
- Hypophosphatasia (HPP) ni indwara idakunze kugaragara, ikwirakwizwa mu muryango, igatuma amagufwa n'amenyo bicika intege.
- Ibimenyetso bishobora gutandukana cyane bitewe n'umuntu ku giti cye, kuva ku gutakaza amenyo imburagihe ku bana bato kugeza ku busembwa bukomeye bw'amagufwa.
- Gupima amaraso kugira ngo harebwe niba enzyme ya ALP iri hasi mu maraso ni ingenzi cyane mu gusuzuma indwara.
- Muri iki gihe, hari uburyo bwiza bwo kuvura bugenzura ibimenyetso, harimo no kuvura indwara zifata imisemburo.
- Niba wowe cyangwa umwana wawe mufite ibi bimenyetso, ntimugatinye kandi muganire na muganga wanyu ako kanya. Kubona isuzuma n'ubuvuzi bikwiye ni ingenzi cyane.
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