Umkhuhlane oqhelekileyo weMeditera

Umkhuhlane oqhelekileyo weMeditera (FMF) sisifo esinqabileyo esidluliswa ziintsapho (ezizuzwe njengelifa). Kubandakanya imikhuhlane ephindaphindiweyo kunye nokudumba okuhlala kuchaphazela ulusu lwesisu, isifuba, okanye amalungu.

I-FMF ihlala ibangelwa kukutshintsha kwemfuza enegama I-MEFV. Olu luhlobo ludala iproteni ebandakanyekayo kulawulo lokudumba. Esi sifo sibonakala kuphela kubantu abafumene iikopi ezimbini zemfuza eguqulweyo, omnye kumzali ngamnye. Oku kubizwa ngokuba yi-autosomal recessive.

I-FMF ihlala ichaphazela abantu bohlanga lweMeditera. Oku kubandakanya ama-non-Ashkenazi (Sephardic) amaYuda, amaArmenian, kunye nama-Arabhu. Abantu abavela kwezinye izizwe banokuchaphazeleka.

Iimpawu zihlala ziqala phakathi kweminyaka emi-5 ukuya kweli-15. Ukudumba kwilaphu esiswini, kwisifuba, kulusu, okanye kumalungu kwenzeka kunye nefiva ephezulu edla ngokufikelela kwiiyure ezili-12 ukuya kwezingama-24. Uhlaselo lungahluka ngokobunzima beempawu. Abantu bahlala bengenazo iimpawu phakathi kokuhlaselwa.

Iimpawu zinokubandakanya iziqendu eziphindaphindwayo ze:

• Intlungu zesisu
• Intlungu yesifuba ebukhali kwaye iba mandundu xa uthatha umphefumlo
• Umkhuhlane okanye ubushushu obutshintshanayo kunye nomkhuhlane
• Intlungu edibeneyo
• Izilonda zesikhumba ezibomvu nezidumbileyo kwaye ziqala ukusuka kwi-5 ukuya kwi-20 cm ubukhulu

Ukuba uvavanyo lwemfuzo lubonisa ukuba une I-MEFV Ukutshintsha kwemfuza kunye neempawu zakho zihambelana nomzekelo oqhelekileyo, ukuxilongwa kuphantse kuqiniseke. Uvavanyo lweLabhoratri okanye i-x-ray inokulawula ezinye izifo ezinokubakho ukunceda ukufumanisa isifo.

Amanqanaba ovavanyo oluthile lwegazi anokuphakama kunesiqhelo xa usenziwa ngexesha lokuhlaselwa. Uvavanyo lunokubandakanya:

• Gcwalisa inani legazi (CBC) elibandakanya inani leeseli ezimhlophe
• C-protein esebenzayo ukukhangela ukudumba
• Ireyithi ye-erythrocyte sedimentation (ESR) ukujonga ukudumba
• Uvavanyo lwe-Fibrinogen ukukhangela ukujiya kwegazi

Injongo yonyango lwe-FMF kukulawula iimpawu. IColchicine, iyeza elinciphisa ukudumba, linokunceda ngexesha lokuhlaselwa kwaye linokuthintela uhlaselo oluthe kratya. Inokukunceda ekuthinteleni ingxaki enkulu ebizwa ngokuba yi-systemic amyloidosis, exhaphakileyo kubantu abane-FMF.

Ii-NSAID zingasetyenziselwa ukunyanga umkhuhlane kunye nentlungu.

Akukho lunyango lwe-FMF. Uninzi lwabantu luyaqhubeka nokuhlaselwa, kodwa inani kunye nobukhali bohlaselo bohlukile kumntu kumntu.

I-Amyloidosis inokukhokelela ekonakaleni kwezintso okanye ukungabinakho ukufunxa izakhamzimba kukutya (malabsorption). Iingxaki zokuchuma kwabasetyhini nakumadoda kunye nesifo samathambo zikwanengxaki.

Nxibelelana nomboneleli wakho ukuba wena okanye umntwana wakho uvelisa iimpawu zale meko.

Usapho oluqhelekileyo lwe-paroxysmal polyserositis; Peritonitis ngamaxesha; Ukuphindaphindeka kwepolyserositis; I-Benign paroxysmal peritonitis; Isifo sexesha; Umkhuhlane wexesha; I-FMF

• Umlinganiselo wobushushu

Iilwimi JW. I-Hereditary syndromes periodic fever kunye nezinye izifo zesifo somzimba. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahl. 188.

Umkhuhlane oqhelekileyo weMeditera. Ku: UAdam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, eds. Uphononongo lweGene [Intanethi]. IYunivesithi yaseWashington, Seattle, WA: 2000 Agasti 8 [ihlaziyiwe ngo-2016 Dec 15]. IINKCUKACHA: 20301405 www.pubmed.ncbi.nlm.nih.gov/20301405/.