Ukuvuvukala Komzimba Okudluliselwa Ngokuzalwa (Familial Mediterranean Fever)

I-Familial Mediterranean fever (FMF) yisifo esidluliselwa ngezofu esibangela ukuvuvukala okuphindaphindayo nobuhlungu esiswini, esifubeni nasezinhlangothini. I-Familial Mediterranean fever (FMF) luyisifo oluzuzwe oluvamise ukubangelwa abantu abavela emazweni aseMedithera – okuhlanganisa labo abavela e-Juda, e-Arab, e-Armenia, eTurkey, eNyakatho Afrika, eGrisi noma e-Itali. Kodwa lingathinta abantu banoma yiluphi uhlobo. I-FMF ivame ukuxilongwa ebuntwaneni. Nakuba kungekho ukwelapha lesi sifo, ungase ukwazi ukunciphisa noma uvikele izimpawu zesifo se-FMF ngokulandela uhlelo lwakho lokwelapha.

Izimpawu nezimpawu ze-familial Mediterranean fever ngokuvamile ziqala ebuntwaneni. Zivelela ngezigaba ebizwa ngokuthi ukuhlaselwa okuphela izinsuku ezingu-1-3. Ukuhlaselwa kwe-Arthritic kungathatha amasonto noma izinyanga.

Izimpawu nezimpawu zokuhlukunyezwa kwe-FMF ziyahluka, kodwa zingafaka:

• Ukuqina
• Ubuhlungu besisu
• Ubuhlungu esifubeni, okungenza kube nzima ukuphefumula ngokujulile
• Izinhlungu ezibuhlungu, eziqabukile, ngokuvamile emadoyeni, ezinqenqemeni nasemadolweni
• Isikhumba esibomvu emilenzeni yakho, ikakhulukazi ngaphansi kwamadolo akho
• Ubuhlungu bemisipha
• I-scrotum ebuhlungu, ebomvu

Ukuhlaselwa ngokuvamile kuqeda ngokwakho ngemva kwezinsuku ezimbalwa. Phakathi kokuhlasela, cishe uzizwa uphenduke impilo yakho evamile. Izikhathi ezingenazo izimpawu zingaba mfushane njengezinsuku ezimbalwa noma iminyaka eminingi.

Kwabanye abantu, isici sokuqala se-FMF yi-amyloidosis. Ngo-amyloidosis, i-protein amyloid A, engatholakali ngokuvamile emzimbeni, yakha ezihlabathini - ikakhulukazi izinso - ibangele ukuvuvukala futhi iphazamise umsebenzi wazo.

Bona uhlinzeki wakho wezempilo uma wena noma ingane yakho inesifo esiqala ngokuzumayo esiqondene nobuhlungu esiswini, esifubeni nakwihlombe.

I-Familial Mediterranean fever ibangelwa ukuguquka kwe-jini (mutation) okuthathwa kubazali bayo kubantwana. Ukuguquka kwe-jini kuthinta umsebenzi we-proteni yesistimu yokuvimba ukugula ebizwa nge-pyrin, kubangela izinkinga ekulawuleni ukuvuvukala emzimbeni.

Kubantu abane-FMF, ukuguquka kwenzeka ku-jini ebizwa nge-MEFV. Izinguquko eziningi ezahlukene ku-MEFV zihlanganiswe ne-FMF. Ezinye izinguquko zingabangela izimo ezinkulu kakhulu, kanti ezinye zingabangela izimpawu ezilula.

Akucaci ukuthi yini ngempela eqala ukuhlaselwa, kodwa kungenzeka kwenzeke ngenxa yokucindezeleka ngokomzwelo, ukuya esikhathini, ukubhekana nobuqandayo, nokucindezeleka komzimba njengokugula noma ukulimala.

Izici ezingase zikhulise ingozi yomkhuhlane waseMedithera wasekhaya zihlanganisa:

• Umlando womndeni wesifo. Uma unomlando womndeni we-FMF, unethuba elikhulu lesifo.
• Ubuzwe baseMedithera. Uma umndeni wakho ukwazi ukuthola umlando wawo endaweni yaseMedithera, ingozi yakho yesifo ingase ikhuphuke. I-FMF ingathinta abantu kwanoma yiluphi uhlobo lwabantu, kodwa ingase ibe nethuba elikhulu kubantu abangamaJuda, ama-Arab, ama-Armenian, ama-Turkish, ama-North African, amaGreki noma ama-Italian.

Izinkinga zingase zenzeke uma ubuhlungu beMediterranean family lungaphathwa. Ukuvuvukala kungabangela izinkinga ezinjengalezi:

• Amyloidosis. Ngesikhathi sezimpawu ze-FMF, umzimba wakho ungase ukhiqize iprotheni ebizwa nge-amyloid A, engavamile ukuba khona emzimbeni. Ukubunjwa kwale protheni kubangela ukuvuvukala, okungalimaza izitho.
• Ukonakala kwesibindi. I-Amyloidosis ingalimaza izinkulungwane, ibangele i-nephrotic syndrome. I-Nephrotic syndrome ivela uma izinhlelo zokucwecwa zezinso zakho (i-glomeruli) zilimele. Abantu abane-nephrotic syndrome bangase baphelelwe yiprotheni eningi emathunjini abo. I-Nephrotic syndrome ingabangela ukubunjwa kwegazi ezinkulungwaneni zakho (i-renal vein thrombosis) noma ukuhluleka kwesibindi.
• Ubuhlungu bezingqondo. I-Arthritis ivamile kubantu abane-FMF. Izingqondo ezithintekayo kakhulu yizinkulungwane, izingalo nezinqindi.
• Ukungaqali. Ukuvuvukala okungaphathwayo okubangelwa yi-FMF kungathinta izitho zokuzala, kubangele ukungaqali.
• Ezinye izinkinga. Lezi zingafaka ukuvuvukala enhliziyweni, emaphashini, e-spleen, ebuchosheni nasemithanjeni ebonakalayo.

Izivivinyo nezinqubo ezisetshenziswa ekutholakala kokutheleleka kwe-Familial Mediterranean fever zihlanganisa:

Ukuhlolwa kwe-genetic kwe-FMF kungase kunconywe kubantu bakho abaseduze, njengabazali, abafowabo noma abantwana, noma kubanye abantu abasemncintisweni. Ukuqondisa nge-genetic kungakusiza ukuqonda izinguquko ze-gene nemiphumela yazo.

• Ukuhlolwa komzimba. Umhlinzeki wakho wezempilo angase akusize ngezimpawu zakho kanye nezimpawu bese enza ukuhlolwa komzimba ukuze aqoqe olunye ulwazi.
• Ukubuyekezwa komlando wakho wezokwelapha womndeni. Umlando womndeni we-Familial Mediterranean fever (FMF) wandisa amathuba akho okukhula ngalesi simo ngoba lesi shintsho se-genetic sidluliselwa kubazali kubantwana babo.
• Izivivinyo zelebhu. Ngesikhathi sokuhlaselwa, ukuhlolwa kwegazi nokwelutho kungabonisa amazinga aphezulu ezikhombisi ezithile ezibonisa isimo sokuvuvukala emzimbeni wakho. Izinga eliphezulu lamaseli amhlophe egazi, alwa nokutheleleka, yisikhombisi esinye esinjalo. Iprotheni eluthweni elingabonisa i-amyloidosis enye.
• Ukuhlolwa kwe-genetic. Ukuhlolwa kwe-genetic kungase kunqume ukuthi i-MEFV gene yakho inenqubo ye-gene ehlobene ne-FMF. Izivivinyo ze-genetic azithuthukisiwe ngokwanele ukuze zivivinye zonke izinguquko ze-gene ezihlobene ne-FMF, ngakho kunokwenzeka ukuthi kube khona imiphumela engalungile. Ngenxa yalesi sizathu, abahlinzeki bezempilo ngokuvamile abasebenzisi izivivinyo ze-genetic njengendlela kuphela yokuxilonga i-FMF.