I-Hemophilia ingenye yezifo ezibucayi kakhulu, okuthuthukiswa kuyo okuhlotshaniswa nobulili. Okusho ukuthi, amantombazane ayithwala isakhi esingenasici, kodwa lesi sifo sibonakala kuphela kubafana. Lesi sifo sibangelwa ukuntuleka ngokwezakhi zofuzo ze-plasma izici eziqinisekisa igazi coagulability. Naphezu kokuthi kwaziwa isikhathi eside, igama elithi "hemophilia" lesifo lafunyanwa kuphela ngekhulu le-19.
Kunezinhlobo eziningana ze-hemophilia:
- i-hemophilia A (HA, i-hemophilia ye-classic);
- i-hemophilia B (i-GV, isifo sikaKristu);
- i-hemophilia C (HS).
Izimbangela ze-hemophilia
Ifa le-hemophilia A ne-B livela, njengoba kushiwo ngaphambili, kulayini wesifazane, ngoba abantu ababhekene nalesi sifo ngokuvamile abahlali esikhathini sokuzala. Kodwa-ke, eminyakeni yamuva nje inqubekela phambili enkulu kuye kwaphawulwa ekwelapheni, okuvumela ukwandisa isikhathi sokuphila kwabantu abagulayo. Ngaphandle kwemiphumela emihle, lokhu kwaletha nemiphumela emibi - ukwanda okuphawulekayo kwenani leziguli emhlabeni wonke. Izifo eziyinhloko zezifo (ezingaphezu kwezingu-80%) zibhekisela ezakhiweni zofuzo, okungukuthi, ezizuze abazali, amacala asele - ukuguquguquka okuncane kwesiguli. Futhi izimo eziningi ze-hemophilia ezincane zomsunguli zakhiwe kusuka kuleso gciwane lesandulela ngculazi. Futhi omdala ubaba, ephakeme amathuba okuguquka okunjalo. Amadodana amadoda aphethwe i-hemophilia aphilile, amadodakazi abathwala lesi sifo futhi adlulisele ezinganeni zabo. Amathuba okukhiqiza indodana egulayo kubathwali besifazane i-50%. Ezimweni ezingavamile, kunezifo zakudala kubantu besifazane. Njengomthetho, lokhu kwenzeka lapho indodakazi izalwa isiguli esine-hemophilia kayise nomama othwala isifo.
I-Hemophilia C izuzwa ngabantwana bobulili bobabili, futhi amadoda nabesifazane bayathinteka ngokufanayo nalolu hlobo lwesifo.
Noma yiluphi uhlobo lwe-hemophilia (i-hereditary noma i-self-spontaneous), njengoba ibonakale kanye emndenini, kamuva lizozuza njengefa.
Ukuxilongwa kwe-hemophilia
Kunezigaba eziningana zobunzima besifo: ezinzima kakhulu (ezinzima kakhulu), ezinobunzima obuphakathi, obumnene futhi obufihliwe (ususiwe noma uvaliwe). Ngakho-ke, ukuphakama kwe-hemophilia ephakeme kakhulu, lapho izimpawu zimenyezelwa ngokwengeziwe, ukuphuma kwegazi okunamandla kubhekwa njalo. Ngakho-ke, ezimweni ezinzima kukhona ukuphuma okwehlukile ngaphandle kokuxhumana okuqondile nanoma yikuphi ukulimala.
Lesi sifo singazibonakalisa kungakhathaliseki ubudala. Ngezinye izikhathi izibonakaliso zokuqala ziyabonakala kakade esikhathini sokuzalwa okusha (ukuphuma kwegciwane lesilwane, ukubola kwesisu, njll). Kodwa ngokuvamile, i-hemophilia ibonisa ngemuva konyaka wokuqala wokuphila, lapho izingane ziqala ukuhamba futhi ingozi yokulimala iyanda.
Izimpawu ezivamile kakhulu ze-hemophilia yizi:
- i-hemorrhages ejwayelekile futhi ejwayelekile;
- ama-hematomas amaningi, angenakunakekelwa isikhathi eside;
- ukuphuma kwegazi okwesikhashana kusukela emashumini, izibilini zamakhemikhali emakhaleni nasemlonyeni, kaningi emathunjini, izinso, njll. Futhi zingalethwa kunoma yikuphi ukuphathwa kwezokwelapha;
- i-hemarthroses (i-hemorrhages in the joint joint);
- ukubola emathanjeni amathambo okuholela ekunqandeni kwamathambo ne-aseptic necrosis.
Kulokhu, ukuphuma kwegazi akuqala ngokushesha ngemva kokulimala, kodwa ngemuva kwesikhathi esithile (ngezinye izikhathi ngaphezulu kwamahora angu-8-12). Lokhu kuchazwa ukuthi ngokuyinhloko ukuphuma kwegazi kuhamba ngamaplatelets, futhi nge-hemophilia, inani labo lihlala ngaphansi kwemingcele evamile.
Ukuqaphela i-hemophilia ngezivivinyo ezahlukene ze-laboratory ezithatha isikhathi se-coagulation nenani lezici eziphikisana ne-hemophilic. Kubalulekile ukuhlukanisa phakathi kwe-hemophilia ne-von Willebrand, i-thrombocytopenic purpura, ne-Glanzmann thrombastenia.
I-Hemophilia ezinganeni: ukwelashwa
Okokuqala, ingane ihlolwe udokotela wezingane, udokotela wamazinyo, i-hematologist, i-orthopedist, okungcono ukuhlolwa kofuzo nokubonisana kwengqondo kwengqondo. Bonke ochwepheshe balungisa izenzo zabo ukulungiselela uhlelo lokwelapha ngabanye, kuye ngokuthi hlobo luni kanye nesifo esiqinile.
Isimiso esiyinhloko sokwelapha i-hemophilia yindlela yokwelapha endaweni. Iziguli zijojowe ngama-anti-hemophilic amalungiselelo ezinhlobonhlobo, igazi elisha elungisiwe noma ukumpontshelwa okuqondile kwezihlobo (nge-HA).
Izindlela ezintathu zokwelashwa ezisetshenziswayo: ekwelapheni (ngegazi), ukwelapha ekhaya nokuvimbela i-hemophilia. Futhi ukugcina kwabo kuhamba phambili kakhulu futhi kubalulekile.
Njengoba lesi sifo singelapheki, imithetho yokuphila yeziguli ezine-hemophilia iyancipha ukuze igweme ukulimala, ukubhaliswa kwemali okuphoqelekile kanye nokwelashwa okwesikhashana, okuyisisekelo sokugcina igazi elingekho ngaphansi kwezinga elingaphansi kuka-5% wendabuko. Lokhu kugwema ukubola kwegazi emathangeni nasezihlangwini zomzimba. Abazali kufanele bazi izici ezikhethekile zokunakekela izingane ezigulayo, izindlela eziyisisekelo zokusiza kuqala, njll.