Isifo seFabry

Umxholo

• Iimpawu zesifo sikaFabry
• Ukuchongwa kwesifo seFabry
• Unyango lwezifo zeFabry

Isifo sikaFabry sisifo sokuzalwa esinqabileyo esibangela ukuqokelelwa ngokungaqhelekanga kwamafutha kwimithambo yegazi, kubangele ukukhula kweempawu ezinjengeentlungu ezandleni nasezinyaweni, ukutshintsha kwamehlo okanye amabala kulusu, umzekelo.

Ngokubanzi, iimpawu zesifo sikaFabry zibonakala ebuntwaneni, kodwa kwezinye iimeko, esi sifo sinokuchongwa kuphela ebudaleni, xa siqala ukwenza utshintsho ekusebenzeni kwezintso okanye intliziyo.

THE Isifo seFabry asinyangi, kodwa inokulawulwa ngokusetyenziswa kwamanye amayeza ukunqanda ukukhula kweempawu kunye nokubonakala kweengxaki, ezinje ngengxaki yezintso okanye ukubetha.

Iimpawu zesifo sikaFabry

Iimpawu zesifo sikaFabry zinokubonakala kwasekuqaleni ebuntwaneni kwaye zibandakanya:

• Iintlungu okanye ukutshisa imvakalelo ezandleni nasezinyaweni;
• Amabala amnyama abomvu eluswini;
• Utshintsho kwiliso elingachaphazeli umbono;
• Intlungu zesisu;
• Ukutshintsha kokuhamba kwamathumbu, ngakumbi emva kokutya;
• Umqolo obuhlungu, ngakumbi kwingingqi yezintso.

Ukongeza kwezi mpawu, isifo sikaFabry sinokubangela, ekuhambeni kweminyaka, ezinye iimpawu ezinxulumene nezilonda eziqhubekayo ezibangelwa kwamanye amalungu, anje ngamehlo, intliziyo okanye izintso, umzekelo.

Ukuchongwa kwesifo seFabry

Ukuchongwa kwesifo sikaFabry kunokwenziwa kuvavanyo lwegazi ukuvavanya isixa se-enzyme enoxanduva lokuphelisa amanqatha amaninzi aqokelelana emithanjeni. Ke, xa eli xabiso liphantsi, ugqirha unokurhanela isifo sikaFabry kwaye ayalele uvavanyo lwe-DNA ukuze kuchongwe ngokuchanekileyo esi sifo.

Unyango lwezifo zeFabry

Unyango lwesifo sikaFabry sinceda ukulawula ukuqala kweempawu kunye nokuthintela ukukhula kweengxaki, kwaye kungenziwa nge:

• ICarbamazepine: Unceda ukunciphisa intlungu okanye ukutshisa;
• Imetoclopramide: kunciphisa ukusebenza kwamathumbu, kuthintelwa utshintsho kuthutho lwamathumbu;
• Unyango lweanticoagulant, ezinje ngeAspirin okanye iWarfarin: yenza igazi libe lincinci kwaye kuthintele ukubonakala kwamahlwili anokubangela ukubetha.

Ukongeza kula machiza, ugqirha unokuyalela amayeza oxinzelelo lwegazi oluphezulu, njengeCaptopril okanye iAtenolol, njengoko zithintela ukukhula komonakalo wezintso kunye nokuthintela ukuqala kweengxaki kula malungu.