Osteopetrosis: yintoni, iimpawu kunye nonyango

Umxholo

• Iimpawu ze-Osteopetrosis
• Oonobangela be-osteopetrosis
• Unyango lwenziwa njani

I-Osteopetrosis sisifo esingaqhelekanga se-osteometabolic isifo apho amathambo axinene kunesiqhelo, okwenzeka ngenxa yokungalingani kweeseli ezinoxanduva lwenkqubo yokwakheka kwethambo kunye nokwaphuka, ukukhuthaza ukwanda ngokubanzi kokuxinana kwethambo kwaye kukhokelele ekubonakaleni ezinye iimpawu ezifana njengamathambo amancinci ngakumbi, ubunzima bokuva kunye notshintsho kuphuhliso lwe-neuronal, umzekelo.

Unyango lwe-osteopetrosis kufuneka lucetyiswe liqela lezonyango elibandakanya ugqirha wabantwana, i-hematologist kunye ne-orthopedist, kunye nokufakwa komongo wethambo kuhlala kunconyelwa ukuphucula ukusebenza kweeseli ezinxulumene nokwakheka kwethambo.

Iimpawu ze-Osteopetrosis

Iimpawu kunye neempawu ze-osteopetrosis zinokuchongwa kwakamsinya emva kokuzalwa, nanjengokuba sisifo sokuzalwa, okanye kunokubakho iimpawu kunye neempawu kuphela ebudaleni. Olona phawu luphambili lwe-osteopetrosis kukonyuka koxinano lwamathambo, olunokuthi luqatshelwe ngokuvavanya i-densitometry yethambo.

Ukongeza, kukho amathuba okuqhekeka, kuba ngenxa yokudumba kweeseli ezinoxanduva lwenkqubo yokwenziwa kwethambo kunye nokutshatyalaliswa, amathambo aya eba krwada.

Iimpawu ze-osteopetrosis zinxulumene nenyani yokuba kukho ukubekwa okukhulu kwamathambo emzimbeni, okunokubangela utshintsho emzimbeni wonke, ezona mpawu ziphambili zezi:

• Umbono ongenangqondo;
• Ubunzima bokuva;
• Usulelo oluqhubekayo lwamazinyo neentsini;
• Ukwandiswa kwesibindi kunye ne-spleen, okubangela ukuveliswa kwemveliso yeeseli zegazi;
• Utshintsho kuphuhliso lwe-neuronal;
• Ukulibaziseka ekuzalweni kwamazinyo;
• Ukunyuka koxinzelelo lwangaphakathi.

Ukuxilongwa kwe-osteopetrosis kwenziwa ngugqirha wamazinyo ngokuhlolwa kwemifanekiso efana ne-X-ray kunye ne-bone densitometry, eluvavanyo olulula nolungenabuhlungu olujolise ekuqinisekiseni ubungqingqwa bamathambo omntu, ukuvumela ukuvavanya umngcipheko wokwaphuka, umzekelo. Qonda ukuba yintoni i-osteopetrosis kwaye yenziwa njani.

Nangona kunjalo, ukuqinisekisa uhlobo kunye neengxaki ze-osteopetrosis, ugqirha unokuyalela ezinye iimvavanyo zokuqonda isifo, ezinje ngecomputer tomography okanye imaging resonance imaging yokuvavanya ubukho bezilonda kwamanye amalungu anje ngamehlo neendlebe, ukongeza kuvavanyo lwegazi.

Oonobangela be-osteopetrosis

I-Osteopetrosis ibangelwa ziziphene kuhlobo olunye okanye nangaphezulu olunoxanduva lokwenziwa kunye nophuhliso lwee-osteoclast, eziziiseli ezisusa izicwili zamathambo ezindala zize zibuyisele ezintsha, ezisempilweni. Ngokuxhomekeke kwimvelaphi yezakhi zofuzo eziguqulweyo, uhlobo lwe-osteopetrosis luyahluka:

• Ubuntwana obubi be-osteopetrosis: umntwana unesifo ukusukela ekuzalweni kwakhe ngenxa yesiphene kwimfuza esiyizuze kuyise nakunina;
• Umntu omdala osteopetrosis: I-osteopetrosis ifunyaniswa kuphela kwinqanaba lokufikisa okanye ebudaleni, kubangelwa kukutshintsha kwemfuza ezuzwe njengelifa kutata okanye umama.

Kwimeko ye-osteopetrosis yabantu abadala, ukutshintsha kwemfuza kunokubangelwa kukutshintsha, ngaphandle kokufumana ilifa lotshintsho kubazali.

Unyango lwenziwa njani

Unyango lwe-osteopetrosis kufuneka lukhokelwe liqela leengcali ezininzi zempilo, ezinje ngugqirha wabantwana, ugqirha wamathambo, ugqirha wegazi, ugqirha we-endocrinologist kunye nethambo lomzimba.

Okwangoku, olona nyango lusebenzayo lwe-osteopetrosis kukufakelwa komongo wethambo, kuba iiseli ezirhoxisiweyo ziveliswa kwelo lungu. Ke, xa usenza ukufakelwa, kunokwenzeka ukuba ulawule ukusebenza kweeseli ezinoxanduva lokwenziwa kunye nokutshatyalaliswa kwamathambo, ukulwa ne-osteopetrosis. Qonda ukuba ukufakelwa komongo wethambo kwenziwa njani.

Nangona ukufakelwa umongo wethambo kunyango olucetyiswayo lokunyanga esi sifo, olunye unyango lunokucetyiswa ukukhuthaza ukukhululeka kwempawu, ezinje:

• Inaliti nge-Interferon gamma-1bEli chiza elinakho ukulibazisa ukukhula kwesi sifo;
• Ukutya kweCalcitriol, eluhlobo olusebenzayo lwevithamin D elinceda ukukhuthaza iiseli zethambo ukuba zikhule ngesiqhelo kunye nokunciphisa uxinano lwethambo;
• Ukungena kwePrednisone, eyihormoni efana necortisone enokuphucula imveliso yeeseli zokhuselo emzimbeni, eziveliswa emathanjeni;
• Iiseshoni zePhysotherapy, njengoko bephucula amandla omzimba wesigulana, bencedisa ukuthintela ukwaphuka kwamathambo kunye nokuphucula ukuzimela kwezinye izinto zemihla ngemihla.

Ugqirha unokukucebisa ukuba uqhagamshelane nesazi sezondlo ukulungelelanisa indlela otya ngayo ukuze ubandakanye ukutya okunceda ukuququzelela ukukhula komzimba kunye nethambo, ngakumbi ngexesha lobuntwana.

Ukongeza, kubalulekile ukuba undwendwele rhoqo kwi-ophthalmologist, i-otolaryngologist kunye nogqirha wamazinyo ukuvavanya ukukhula kunye nokubonakala okunokwenzeka kwezinye izilonda okanye ukungalunganga emehlweni, amazinyo, impumlo, iindlebe kunye nomqala, umzekelo.