Isifo sePaget kwithambo

Isifo sePaget sisifo esichaphazela ukubhujiswa kwethambo ngokungaqhelekanga kunye nokuphinda ubuye. Oku kubangela ukukhubazeka kwamathambo achaphazelekayo.

Isizathu sesifo sePaget asaziwa. Isenokuba ngenxa yemfuza, kodwa ikwanokubangelwa sisifo sentsholongwane kwintsholongwane.

Esi sifo senzeka kwihlabathi liphela, kodwa sixhaphake kakhulu eYurophu, e-Australia, nase-New Zealand. Esi sifo siye saxhaphaka kakhulu kule minyaka ingama-50 idlulileyo.

Kubantu abanesifo sePaget, kukho ukuphazamiseka okungaqhelekanga kwethambo leethambo kwiindawo ezithile. Oku kulandelwa kukwenziwa kwamathambo ngokungaqhelekanga. Indawo entsha yethambo inkulu, kodwa buthathaka. Ithambo elitsha lizaliswe yimithambo yegazi emitsha.

Ithambo elichaphazelekayo linokuba kwindawo enye okanye ezimbini kumathambo, okanye kumathambo ahlukeneyo emzimbeni. Ihlala ibandakanya amathambo eengalo, ikhola, imilenze, isinqe, umqolo kunye nokakayi.

Uninzi lwabantu abanemeko abanazo iimpawu. Isifo sePaget sihlala sifunyaniswa xa kwenziwa iX-reyi ngesinye isizathu. Inokufunyanwa kwakhona xa uzama ukufumana unobangela wamanqanaba aphezulu egazi le-calcium.

Ukuba ziyenzeka, iimpawu zinokubandakanya:

• Intlungu yethambo, iintlungu ezidibeneyo okanye ukuqina, kunye nentlungu yentamo (iintlungu zinokuba nzima kwaye zibekhona ixesha elininzi)
• Ukuguqa kwemilenze kunye nokunye ukubonakala okubonakalayo
• Ukwandiswa kwentloko kunye nokukhubazeka kwekhanda
• Ukophuka
• Intloko ebuhlungu
• Ukuphulukana nokuva
• Ukuncipha kobude
• Ulusu olufudumeleyo phezu kwethambo elichaphazelekayo

Uvavanyo olunokuthi lubonise isifo sePaget kubandakanya:

• Ukuskena amathambo
• I-X-ray yethambo
• Amanqaku aphakamileyo okuphuka kwamathambo (umzekelo, i-N-telopeptide)

Esi sifo sinokuchaphazela iziphumo zolu vavanyo lulandelayo:

• I-alkaline phosphatase (ALP), ithambo elithile isoenzyme
• Isalcium calcium

Akunabo bonke abantu abanezifo zePaget abafuna unyango. Abantu abangenakudinga unyango baquka abo:

• Yiba novavanyo lwegazi olungaqhelekanga
• Akukho zimpawu kwaye akukho bungqina besifo esisebenzayo

Isifo sePaget sihlala siphathwa xa:

• Amathambo athile, anje ngamathambo athwala ubunzima, ayabandakanyeka kwaye umngcipheko wokuqhekeka uphezulu.
• Utshintsho lweBony luya lusiba mandundu ngokukhawuleza (unyango lunokunciphisa umngcipheko wokuqhekeka).
• Iziphene zeBony zikhona.
• Umntu uneentlungu okanye ezinye iimpawu.
• Ukhakhayi luyachaphazeleka. (Oku kukuthintela ukulahleka kwendlebe.)
• Amanqanaba e-calcium aphakanyisiwe kwaye abangela iimpawu.

Unyango lweziyobisi luyanceda ukuthintela ukonakala ngakumbi kwamathambo kunye nokwakheka. Okwangoku, kukho iindidi ezininzi zamachiza asetyenziselwa ukunyanga isifo sePaget. Oku kubandakanya:

• I-Bisphosphonates: La machiza lunyango lokuqala, kwaye anceda ukunciphisa ukulungiswa kwamathambo. Amayeza aqhelekileyo athathwa ngomlomo, kodwa anokunikwa ngomthambo (ngaphakathi).
• I-Calcitonin: Le hormone ibandakanyeka kwimethambo ye-metabolism. Inganikwa njenge-nasal spray (Miacalcin), okanye ngenaliti phantsi kolusu (iCalcimar okanye iMithracin).

I-Acetaminophen (iTylenol) okanye i-nonsteroidal anti-inflammatory drug (NSAIDs) nayo inokunikwa iintlungu. Kwiimeko ezinzima, utyando lwamathambo lunokufuneka ukulungisa ukukhubazeka okanye ukwaphuka.

Abantu abakule meko banokuzuza ngokuthatha inxaxheba kumaqela enkxaso kubantu abanamava afanayo.

Uninzi lwexesha, imeko inokulawulwa ngamayeza. Inani elincinci labantu linokuphuhlisa umhlaza wethambo elibizwa ngokuba yi-osteosarcoma. Abanye abantu baya kufuna utyando oludibeneyo lokutshintsha.

Iingxaki zinokubandakanya:

• Ukuqhekeka kwamathambo
• Izithulu
• Iziphene
• Ukumelwa yintliziyo
• Hypercalcemia
• Iparaplegia
• Umqolo wesisu

Fowunela umboneleli wakho wezempilo ukuba uvelisa iimpawu zesifo sePaget.

Osteitis deformans

• X-reyi

URalston SH. Isifo sePaget samathambo. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl 233.

Imvumi FR. Isifo sePaget samathambo. Ku: Jameson JL, De Groot LJ, de Kretser DM, et al, ii-eds. Endocrinology: Abantu abadala kunye nabantwana. Umhla wesi-7. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 72.