Ngaba ngamanye amaxesha uziva ngathi amathambo akho abuthathaka kancinci kwaye abuthathaka ngakumbi kunamanye? Ngaba uyawaphula amathambo akho nokuba yinto encinci? Okanye amazinyo omntwana wakho aphuma ngaphambi kwexesha? Namhlanje siza kuthetha ngemeko esingathethi kakhulu ngayo, kodwa kubaluleke kakhulu ukuba siyiqaphele. Oku kubizwa ngokuba yiHypophosphatasia, okanye iHPP ngamafutshane.
Yintoni kanye kanye iHypophosphatasia (HPP)?
Ngamafutshane, iHypophosphatasia (HPP) yimeko engaqhelekanga, ezuzwe njengelifa (imfuza) echaphazela indlela amathambo namazinyo ethu akhula ngayo. Ukuze amathambo ethu abe namandla kwaye amazinyo ethu asincede ekuhlafuneni nasekuhlafuneni ukutya ngokufanelekileyo, kufuneka iiminerali ezifana necalcium kunye nephosphorus zongezwe kuzo. Le nkqubo ibizwa ngokuba yi-"mineralization".
Kwi-HPP, le nkqubo ibizwa ngokuba yi-'mineralization' ayisebenzi kakuhle. Ngenxa yoko, amathambo namazinyo awanamandla ngokwaneleyo, kodwa athambile kwaye aqhekeke lula. Nangona le meko inokuchaphazela abantu abambalwa kuphela , inokuba yimeko enzima nesongela ubomi kwabanye.
Yintoni ebangela i-HPP?
Le yingxaki yesayensi kancinci, kodwa masiyiqonde ngokulula. Khawucinge ukuba imizimba yethu ifana nomzi-mveliso omkhulu. Yonke into kufuneka icwangciswe kakuhle. Ukuze amathambo omelele, sidinga iiminerali zecalcium kunye nephosphorus esizikhankanyileyo ngaphambili.
Kodwa ke ukuba le nkqubo 'yokwenza iiminerali' ininzi kakhulu nako akulunganga. Umzekelo, akulunganga ukuba ezi minerali ziqokeleleke egazini. Ngoko ke sineekhemikhali emizimbeni yethu ezilawula oku.
Kodwa amathambo namazinyo kufuneka abe nomlinganiselo ofanelekileyo wezi minerali. Kukho i-enzyme ekhethekileyo emzimbeni wethu enceda kulo msebenzi ubalulekileyo. Ibizwa ngokuba yi -alkaline phosphatase (ALP) . Le enzyme ivala iikhemikhali ezisemathanjeni nasemazinyweni ezithintela ukuqokelelana kwezo minerali. Emva koko iiminerali ezifunekayo ziqokelelwa emathanjeni nasemazinyweni, zizenze zomelele.
Abantu abane-HPP banotshintsho kwi-gene ebizwa ngokuba yi-'ALPL', ethintela i-enzyme ye-ALP ukuba iveliswe ngokufanelekileyo. Oku kubangela ukuba iikhemikhali ezithintela ukuqokelelana kweeminerali emathanjeni, zithintele amathambo ekufunxeni i-calcium kunye ne-phosphorus abayidingayo. Ngenxa yoko, amathambo ayathamba kwaye abe buthathaka endaweni yokuba aqine.
Zithini iimpawu ze-HPP?
Iimpawu ze-HPP zahlukile kakhulu. Zixhomekeke ekubeni ingakanani na i-enzyme ye-ALP emzimbeni. Njengoko amanqanaba e-enzyme ehla, iimpawu zihlala zisiba mandundu ngakumbi. Le meko inokuvela nangaliphi na ixesha ukususela ekuzalweni ukuya ebudaleni.
Xa abanye abantu abadala befunyaniswa ukuba bane-HPP, bayakhumbula ukuba babeneempawu ezifanayo ukususela ebuntwaneni, kodwa ayizange ifunyanwe ngokufanelekileyo ngelo xesha.
Makhe sibone ukuba zeziphi iimpawu eziphambili.
| Uphawu | Inkcazo |
|---|---|
| Iingxaki zamazinyo | Ukulahlekelwa ngamazinyo omntwana ngaphambi kweminyaka emi-5 okanye ukulahleka kwamazinyo okungalindelekanga nangayiphi na iminyaka. (Le yeyona ndlela ixhaphakileyo - uhlobo "loonodonto") |
| Utshintsho emathanjeni | Iingalo nemilenze egobileyo, imfutshane, amathambo athambileyo, abuthathaka okanye abuthathaka, izihlunu ezibanzi kunye namalungu amaqatha. |
| Iingxaki zeentsana | Ukungaphumeleli ukukhula okanye ukukhula ngokufanelekileyo, ukudibana kwamathambo entloko ngaphambi kwexesha (okunokukhokelela ekucinezelekeni kwengqondo), ubuthathaka bemisipha (hypotonia) okwenza abantwana babonakale 'bethambile', kunye nobunzima bokuphefumla. |
| Ukuqhekeka kwamathambo | Amathambo aphuka lula eselula, ingakumbi ezinyaweni nasemilenzeni, kwaye athatha ixesha elide ukuphola. |
| Ezinye iimpawu | Iintlungu emathanjeni nasemalungeni, ubunzima bokuhamba, amanqanaba aphezulu e-calcium egazini (kunokubangela ukugabha, ukuqhina, iingxaki zezintso), ukuxhuzula, isifo samathambo esiqatha ngequbuliso. |
Indlela yokuxilonga esi sifo?
Ukuba wena okanye umntwana wakho unezi mpawu, ugqirha wakho uza kubuza ngembali yempilo yosapho lwakho aze enze uvavanyo olupheleleyo lomzimba. Ukongeza, baza kuyalela ii-X-ray kunye novavanyo lwegazi.
Uvavanyo olubaluleke kakhulu apha kukulinganisa inqanaba le-enzyme ye-ALP egazini.Kwimeko ye-HPP, eli nqanaba lidla ngokuba liphantsi.
Ngamanye amaxesha, uvavanyo lwemfuza lunokwenziwa ukuqinisekisa ukuba unayo na i-HPP. Nangona kunjalo, olu vavanyo alufumaneki kuyo yonke indawo kwaye lunokubiza kakhulu. Nangona kunjalo, kwiimeko ezininzi, ugqirha wakho unokuxilonga isifo ngezinye iimvavanyo.
Ngenxa yokuba esi sisifo esingaqhelekanga, ngamanye amaxesha kungathatha ixesha ukufumana uxilongo. Ngoko ke ukuba wena okanye umntwana wakho ninezi mpawu, kubalulekile ukuba nolwazi olupheleleyo kwaye uthethe ngokukhululekileyo nogqirha wakho .
Ziziphi iindlela zonyango lwe-HPP?
Ngethamsanqa, ngoku kukho unyango lwe-HPP. Kwi-HPP, ingakumbi abo baxilongwe besebancinci okanye besebancinci, kusetyenziswa iyeza elibizwa ngokuba yi-asfotase alfa (Strensiq) . Eli liyeza elinikwa phantsi kolusu. Lisebenza ngokutshintsha i-enzyme ye-ALP engekhoyo emzimbeni (unyango lokutshintshwa kwe-enzyme).
Ukongeza kolu nyango luphambili, kukho nezinye izinto ezininzi ezenziwayo ukulawula iimpawu.
- Ukunika amayeza okudambisa iintlungu (ii-NSAID) afana ne-paracetamol okanye i-ibuprofen xa kuthe kwabakho iintlungu zamathambo okanye amalungu.
- Ukuba uxinzelelo olukwikhakayi luphezulu, kuya kufuneka kwenziwe utyando lokunciphisa uxinzelelo.
- Ukunika i-vitamin B6 ukulawula ukuxhuzula kwabanye abantu.
- Ulawulo lokutya okanye amayeza okulawula amanqanaba e-calcium egazini.
- Soloko unyamekela impilo yamazinyo akho kwaye ucele iingcebiso zamazinyo.
- Unyango lomzimba luqinisa izihlunu kwaye luphucula intshukumo.
- Ukufaka iintonga zesinyithi emathanjeni aqhekeka rhoqo.
- Into enye ekufuneka uyikhumbule ngakumbi kukuba amayeza ohlobo lwe-bisphosphonate, asetyenziselwa ukunyanga ezinye izifo zamathambo, ezifana ne-osteoporosis, anokwenza i-HPP ibe mandundu ngakumbi. Ngoko ke, kufuneka aphetshwe.
Olu nyango lunokubiza kakhulu, ngoko ke kubalulekile ukuthetha nogqirha wakho malunga neenzuzo, iingxaki kunye neendleko zonyango.
Ukufumana inkxaso xa uhlala ne-HPP
Ukuqhekeka kwamalungu omzimba, iintlungu, kunye nezinye iimpawu zinokwenza ubomi bemihla ngemihla bube nzima. Ukuphila nesifo esingaqhelekanga kuthetha ukuba nolwazi olupheleleyo ngemeko yakho kunye nokuzithethelela. Oku kungasebenza kuwe okanye kumntwana wakho.
Qonda kwaye uhloniphe imida yakho yomzimba. Phumla xa kufuneka. Ukunyanga i-HPP kudla ngokufuna iqela elinamacandelo amaninzi. Oku kungabandakanya ugqirha wabantwana, ugqirha wamathambo, ugqirha wamazinyo, kunye nogqirha wezonyango.
Kukho ezinye izifo eziqhelekileyo ezineempawu ezifanayo nezeHPP, ngoko ke ukuba awuqinisekanga ngokuxilongwa ngugqirha wakho, ungoyiki ukufumana uluvo lwesibini komnye uchwepheshe.Lilungelo lakho.
Nangona unyango lungathatha iminyaka, iimpawu zinokulawulwa kakhulu ngokuhamba kwexesha.
Umyalezo Wokuya Ekhaya
- I-Hypophosphatasia (HPP) sisifo esingaqhelekanga, esidluliselwa kwilifa esibuthathaka amathambo namazinyo.
- Iimpawu zingahluka kakhulu kumntu nomntu, ukususela ekulahlekelweni kwamazinyo kwangethuba kubantwana abancinci ukuya ekuphazamisekeni kwamathambo.
- Uvavanyo lwegazi lokujonga amanqanaba aphantsi e-enzyme ye-ALP egazini lubaluleke kakhulu ekuxilongweni.
- Okwangoku, kukho unyango olusebenzayo olulawula iimpawu, kuquka unyango lokutshintshwa kwee-enzyme.
- Ukuba wena okanye umntwana wakho unezi mpawu, musani ukoyika nize nithethe nogqirha wenu ngoko nangoko. Ukufumana uxilongo olufanelekileyo kunye nonyango kubaluleke kakhulu.
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