I-Hemophilia yisifo sofuzo sokopha esivimbela igazi ukuthi lihlule, okubangela ukopha isikhathi eside ngokungavamile lapho elimele futhi ngezinye izikhathi ngaphandle kokulimala. Kubangelwa ukuguqulwa kwezakhi zofuzo okubangela ukuntuleka noma ukungabi khona kwamaprotheni okujikisa okuvimbela ihlule ukuba liqine ngokwanele ukumisa ukopha.

I-Hemophilia ithathwa njengesifo sabafana futhi ihlasela ingane eyodwa kweziyizi-1 zabafana. Kodwa-ke, amantombazane angakwazi ukuthwala ukuguqulwa kofuzo futhi athuthukise uhlobo oluncane lwalesi sifo. Esibonkeni sabantu, umuntu oyedwa kwabayi-5000 une-hemophilia. (1) Kunezinhlobo ezimbili ezivelele zesifo: i-hemophilia A kanye no-B. Ukuvama kwe-hemophilia A kungaphezulu kwalokho kwe-hemophilia B (12/000 owesilisa ngokumelene ne-1 / 1) . (6).

I-Hemophilia yayiseyisifo esithena amandla futhi esibulalayo emashumini ambalwa eminyaka edlule kusukela ebuntwaneni nasebusheni. Namuhla, ukwelashwa okusebenzayo kodwa okunemingcele kwenza kube nokwenzeka ukunqanda ukopha futhi kukhawulele ukulimala nokukhubazeka komzimba kubantu abane-hemophilia.

ingane. Ukopha isikhathi eside kwenzeka ngemva kokulimala noma ngisho nokuhlukumezeka okuncane. Zingaba ngokuzenzakalelayo (ngakho-ke zingenelele lapho kungabikho ukuhlukumezeka) ezinhlotsheni ezinzima zesifo. Ukopha kungaba ngaphakathi noma ngaphandle. Qaphela ukuthi ukopha kumuntu one-hemophilia akubi kakhulu, kodwa ukuthi isikhathi saso side. Ukopha emisipheni (imihuzuko) nasemalungeni (i-hemarthrosis), ikakhulukazi emaqakaleni, emadolweni nasezinqulwini, ngokuhamba kwesikhathi kungabangela ukuqina nokukhubazeka okukhubaza, okungaholela ekukhubazekeni.

Lesi sifo siba sibi kakhulu lapho inani lezinto ezihlanganayo egazini liphansi (1):

• Ifomu elinzima: ukopha okuzenzakalelayo nokujwayelekile (ama-50% wamacala);
• Ifomu elilingene: ukopha okude ngokungajwayelekile kulandela ukulimala okuncane kanye nokopha okuzenzakalelayo okungavamile (10 kuya ku-20% wezigameko);
• Ifomu elincane: ukopha okude ngokungajwayelekile kodwa ukungabikho kokuphuma kwegazi okuzenzakalelayo (amaphesenti angama-30 kuye kwangama-40).

Igazi liqukethe amaprotheni, abizwa ngokuthi ama-clotting factor, avumela ihlule legazi ukuba lakheke futhi ngenxa yalokho ayeke ukopha. Ukuguqulwa kofuzo kuvimbela ukukhiqizwa kwala maprotheni. Uma izimpawu ezihambisana ne-hemophilia A no-B zifana kakhulu, lezi zinhlobo ezimbili zesifo nokho zinemvelaphi ehlukile yofuzo: i-hemophilia A ibangelwa ukuguquka kofuzo lwe-F8 (Xq28) olufaka ikhodi ye-coagulation factor VIII kanye ne-l hemophilia B ngokushintshashintsha. kufuzo lwe-F9 (Xq27) olufaka i-coagulation factor IX.

I-Hemophilia ibangelwa izakhi zofuzo ezitholakala ku-X chromosome. Kuyisifo sofuzo esaziwa ngokuthi “ifa elixhunyaniswe ne-X”. Lokhu kusho ukuthi owesilisa ogulayo uzodlulisela isakhi sofuzo esiguquliwe kuphela emadodakazini akhe, angasidlulisela, ngengozi engama-50%, emadodakazini abo nakubafana. Ngenxa yalokho, lesi sifo sithinta amadoda kuphela, kodwa abesifazane bangabathwali. Cishe i-70% ye-hemophilia inomlando womndeni. (1) (3)

Ukwelashwa manje kwenza kube nokwenzeka ukuvimbela nokunqanda ukopha. Ahlanganisa ukufaka ngomthambo i-antihemophilic factor: i-factor VIII ye-hemophiliacs A kanye ne-factor IX ye-hemophiliacs B. Le mithi elwa ne-antihemophilic ikhiqizwa emikhiqizweni ethathwe egazini (imvelaphi ye-plasma), noma ekhiqizwa ubunjiniyela. izakhi zofuzo (ama-recombinants). Bajovwa ngemijovo evamile futhi ehlelekile ukuvimbela ukopha, noma ukulandela isigameko sokopha. I-Physiotherapy ivumela abantu abane-hemophilia ukuthi balondoloze ukuguquguquka kwemisipha kanye nokuhamba kwamalunga abhekene nokopha okuphindaphindiwe.