I-Hemophilia yenye yezona zifo ezifa kakhulu, ukuphuhliswa kwayo ehambelana nesini. Okokuthi, amantombazana ayithwala i-gene defective, kodwa eso sifo sibonakalisa kuphela kubafana. Esi sifo sibangelwa ukuphelelwa komzimba kweengxaki ze-plasma eziqinisekisa igazi lokuguqula igazi. Nangona eyaziwa ngokuba ixesha elide, igama elithi "hemophilia" lafunyanwa kuphela ngekhulu le-19.
Kukho iintlobo ezininzi ze-hemophilia:
- i-hemophilia A (HA, i-hemophilia ye-classic);
- i-hemophilia B (i-GV, isifo sikaKristu);
- i-hemophilia C (HS).
Iimbangela ze-hemophilia
Ifa le-hemophilia A ne-B likhoyo, njengoko sele likhankanywe, kunye nomgca wesifazane, ekubeni abantu abahluphekileyo kwesi sifo bahlala bephila kwiminyaka yokuzala. Nangona kunjalo, kwiminyaka yamuva nje inkqubela phambili iye yaphawulwa kwonyango, evumela ukwandisa ixesha lokuphila labantu abagulayo. Ukongezelela kwimpembelelo emihle, oku kwazisa nemiphumo emibi-ukwanda kwenyameko kwinani lezigulane emhlabeni wonke. Ipesenteji ephezulu yezifo (ngaphezulu kwe-80%) ibhekisela kwimvelaphi yezofuzo, oko kukuthi, ifa elivela kubazali, amacala asele - ukutshintshwa kwentsholongwane yentsholongwane. Kwaye iimeko ezininzi ze-hemophilia zentsholongwane zenziwa ngumama ezivela kwi-gene gene. Kwaye umdala ubaba, ophakamileyo amathuba okuguquka okunjalo. Oonyana babantu abanesifo se-hemophilia banempilo, iintombi zithwele isifo kwaye zidlulisela kubantwana babo. Ubunokwenzeka bokuvelisa unyana ogulayo kwizithuthi zabasetyhini ngu-50%. Kwiimeko ezinqabileyo, kukho isifo seklasi kubasetyhini. Njengomthetho, oku kwenzekayo xa intombi ibelelwa kwisigulane nge-hemophilia kayise kunye nomama othwala isifo.
I-Hemophilia C izuzwe ngabantwana besini, kunye namadoda nabasetyhini bachaphazeleka ngokufanayo nangolu hlobo lwesifo.
Naluphi na uhlobo lwe-hemophilia (ifa okanye uqobo), evelelwe kanye kwintsapho, iya kuzuza ilifa.
Ukuxilongwa kwe-hemophilia
Kukho amadidiyure amanyathelo obunzima besifo: ezinzima (kwaye zinzima kakhulu), ezinobunzima obuphakathi, obunobuncwane kunye obufihlakeleyo (ususiwe okanye ufihliweyo). Ngako oko, ukuphakama kwe-hemophilia ephezulu, ngokubhekiselele kwiimpawu, ukuphuma kwegazi ngokugqithisileyo kudla rhoqo. Ngoko, kwiimeko ezinzima kukho ukuphuma kwamanzi ngaphandle kokuxhamla ngqo naluphi na ukulimala.
Isifo singazibonakalisa kungakhathaliseki ubudala. Ngamanye amaxesha iimpawu zokuqala ziyabonakala sele sele zityalwa (ukuphuma kwegazi ukusuka kwilonda elixhambileyo, i-hemorrhage engaphantsi, njl.). Kodwa ngokuqhelekileyo, i-hemophilia ibonakalisa emva komnyaka wokuqala wokuphila, xa abantwana beqala ukuhamba kwaye ingozi yokulimala iyanda.
Iimpawu eziqhelekileyo ze-hemophilia zi:
- eziqhekezayo;
- ii-hematomas ezinde, ezingapheliyo;
- ukuphuma kwegazi kwimilo, iimbumba zomlomo zomlomo kunye nomlomo, kaninzi kunamathambo, izintso, njl. Kwaye ziyakwazi ukuziswa nakuphi na ukuphathwa kwezokwelapha;
- i-hemarthroses (i-hemorrhages kwi-joint joint);
- ukubola kwiisithambo zethambo ezikhokelela ekuhlahleni kwamathambo kunye ne-aseptic necrosis.
Kule meko, ukuphuma kwegazi kuqale ngokukhawuleza emva kokulimala, kodwa emva kwexesha (ngamanye amaxesha ngaphezulu kweeyure eziyi-8-12). Oku kuchazwa yinyaniso yokuba ukuphuma kwegazi kuhamba ngeeplatelets, kunye ne-hemophilia, inani labo lihlala kumyinge oqhelekileyo.
Ukufumanisa i-hemophilia kunye neemvavanyo ezahlukeneyo zebhubhoratory ezichonga ixesha lokuguquka kunye nenani lezinto ezichasene ne-hemophilic. Kubalulekile ukuhlukanisa phakathi kwe-hemophilia kunye nesifo se-von Willebrand, purpura ye-thrombocytopenic, kunye ne-Glanzmann thrombastenia.
I-Hemophilia kubantwana: unyango
Okokuqala, umntwana uhlolwe ngumntwana wezilwanyana, udokotela wamazinyo, isifo semfuyo, isifo semfuyo, ngokukhethekileyo ukuhlolwa kofuzo kunye nokubonisana kwengcali yengqondo. Zonke iingcali zilungelelanisa izenzo zabo ukulungiselela inkqubo yonyango, kuxhomekeke kwimeko kunye nobuhlungu besifo.
Umgaqo oyintloko wokonyango lwe-hemophilia yindlela yokwelapha endaweni. Izigulane zijojowe ngamalungiselelo anxamnye ne-hemophilic zeentlobo ezahlukeneyo, igazi elilungiswe ngokutsha okanye ukuxilongwa ngokuthe ngqo kwizihlobo (kunye ne-HA).
Izindlela ezintathu zonyango zonyango zisetyenziswa: kwonyango (ngegazi), unyango lwasekhaya kunye nokuthintela i-hemophilia. Kwaye okokugqibela kubo kuhamba phambili kwaye kubalulekile.
Ekubeni isifo asiphilwanga, imigaqo yobomi bezigulane ezine-hemophilia iyancitshiswa ukuze ikhusele ukulimala, ukubhaliswa kwemibandela yonyango kunye nonyango olufike ngexesha, oko kubalulekile ukuba kugcinwe igazi elingekho phantsi kwezinga elingaphantsi kwe-5% yesiqhelo. Oku kugwema ukubola kwesisu kwiisisipha kunye namalungu. Abazali bafanele bazi iziganeko zokunyamekela abantwana abagulayo, iindlela zokuqala zokunceda, njl.