I-Osteopetrosis: kuyini, izimpawu nokwelashwa

-Delile

• Izimpawu ze-Osteopetrosis
• Izimbangela ze-osteopetrosis
• Yelashwa kanjani

I-Osteopetrosis isifo esingajwayelekile esivela ku-osteometabolic lapho amathambo acinene kunokujwayelekile, okwenzeka ngenxa yokungalingani kwamangqamuzana abhekele inqubo yokwakheka kwamathambo nokwephuka, okukhuthaza ukwanda okujwayelekile kokuqina kwamathambo futhi okuholele ekubukekeni kwezinye izimpawu ezifana njengamathambo amaningi aqhekekile, ukuzwa okunzima kanye nezinguquko ekukhuleni kwe-neuronal, ngokwesibonelo.

Ukwelashwa kwe-osteopetrosis kufanele kunconywe yithimba lezokwelapha elibandakanya udokotela wezingane, i-hematologist nodokotela wamathambo, futhi ukufakelwa umnkantsha wethambo kuvame ukunconywa ukuze kuthuthukiswe ukusebenza kwamaseli ahlobene nokwakheka kwamathambo.

Izimpawu ze-Osteopetrosis

Izimpawu nezimpawu ze-osteopetrosis zingabonakala ngemuva nje kokuzalwa, njengoba kuyisifo sokuzalwa, noma kungaba nezimpawu nezimpawu lapho usumdala. Isici esiyinhloko se-osteopetrosis ukukhula kwamathambo, okungabonwa ngokuhlola i-bone densitometry.

Ngaphezu kwalokho, kunamathuba amakhulu okuqhekeka, ngoba ngenxa yokwehla kwamaseli abhekele inqubo yokwakheka kwamathambo nokubhujiswa, amathambo ayaqina.

Izimpawu ze-osteopetrosis zihlobene neqiniso lokuthi kukhona ukubekwa okukhulu kwamathambo emzimbeni, okungaholela ekuguqukeni kuwo wonke umzimba, izimpawu eziyinhloko yilezi:

• Ukubona okufiphele;
• Ukuzwa okunzima;
• Ukutheleleka okuvamile kwamazinyo nezinsini;
• Ukwandiswa kwesibindi nobende, okuholela ekukhiqizeni okushintshiwe kwamaseli egazi;
• Shintsha ekukhuleni kwe-neuronal;
• Ukubambezeleka kokuzalwa kwamazinyo;
• Ukwanda kwengcindezi yokungasebenzi.

Ukuxilongwa kwe-osteopetrosis kwenziwa i-orthopedist ngokuhlolwa kwezithombe ezifana ne-X-ray ne-bone densitometry, okuwukuhlolwa okulula nokungenazinhlungu okuhlose ukuqinisekisa ubukhulu bamathambo omuntu, okuvumela ukuhlola ubungozi bokuphuka, isibonelo. Qonda ukuthi yini i-osteopetrosis nokuthi yenziwa kanjani.

Kodwa-ke, ukuqinisekisa uhlobo nezinkinga ze-osteopetrosis, udokotela angaphinde a-oda ezinye izivivinyo zokuxilonga, njenge-computed tomography noma i-magnetic resonance imaging yokuhlola ubukhona bezilonda kwezinye izitho ezinjengamehlo nezindlebe, ngaphezu kokuhlolwa kwegazi.

Izimbangela ze-osteopetrosis

I-Osteopetrosis ibangelwa ukukhubazeka kuhlobo olulodwa noma ngaphezulu olubhekele ukwakheka nokuthuthuka kwama-osteoclast, okungamaseli asusa izicubu zamathambo ezindala bese efaka okusha, okunempilo esikhundleni sako. Ngokuya ngemvelaphi yezakhi zofuzo eziguquliwe, uhlobo lwe-osteopetrosis lungahluka:

• I-osteopetrosis ebuntwaneni enobungozi: ingane inesifo selokhu yazalwa ngenxa yokukhubazeka kuzakhi zofuzo ezizuzwe ngubaba nomama;
• I-osteopetrosis yabantu abadala: I-osteopetrosis itholakala kuphela ebusheni noma ebudaleni, ibangelwa izakhi zofuzo eziguquliwe ezitholwe kubaba noma kumama kuphela.

Endabeni ye-osteopetrosis yabantu abadala, ukuguqulwa kwezakhi zofuzo nakho kungabangelwa ukuguqulwa, ngaphandle kokuthola ifa lokuguqulwa kubazali.

Yelashwa kanjani

Ukwelashwa kwe-osteopetrosis kumele kuqondiswe yithimba lochwepheshe bezempilo abaningana, njengodokotela wezingane, udokotela wamathambo, i-hematologist, i-endocrinologist kanye ne-physiotherapist.

Njengamanje, ukwelashwa okuphumelela kakhulu kwe-osteopetrosis ukufakelwa umnkantsha wethambo, ngoba amangqamuzana ancishisiwe akhiqizwa kuleso sitho. Ngakho-ke, lapho kwenziwa ukufakelwa, kungenzeka ukuthi kulawulwe ukusebenza kwamaseli abhekele ukwakheka nokubhujiswa kwamathambo, elwa ne-osteopetrosis. Qonda ukuthi ukufakelwa umnkantsha wenziwa kanjani.

Yize ukufakelwa umongo wethambo kuyindlela yokwelashwa enconywayo yokwelapha lesi sifo, ezinye izindlela zokwelashwa zinganconywa ukuze kuthuthukiswe ukukhululeka kwezimpawu, njenge:

• Imijovo nge-Interferon gamma-1b, okuwumuthi okwazi ukubambezela ukukhula kwesifo;
• Ukudla kweCalcitriol, okuwuhlobo olusebenzayo lukavithamini D osiza ukukhuthaza amangqamuzana amathambo ukuba akhule ngokujwayelekile futhi anciphise ukuminyana kwethambo;
• Ukungeniswa kwePrednisone, eyi-hormone efana ne-cortisone engathuthukisa ukukhiqizwa kwamaseli wokuzivikela emzimbeni, akhiqizwa emathanjeni;
• Izikhathi ze-Physiotherapy, njengoba zithuthukisa amandla omzimba wesiguli, zisiza ukuvimbela ukuphuka kwamathambo nokwenza ngcono ukuzimela kweminye imisebenzi yansuku zonke.

Udokotela futhi angakweluleka ukuthi uthintane nesazi sokudla okunempilo ukuze uvumelanise indlela odla ngayo ukufaka ukudla okusiza ukwenza ukukhula komzimba namathambo, ikakhulukazi ngesikhathi sobuntwana.

Ngaphezu kwalokho, kubalulekile ukuthi uvakashele njalo kudokotela wezifo zamehlo, i-otolaryngologist nodokotela wamazinyo ukuhlola ukukhula kanye nokubonakala okungenzeka kwezinye izilonda noma ukukhubazeka emehlweni, emazinyweni, ekhaleni, ezindlebeni nasemphinjeni, ngokwesibonelo.