Umkhuhlane ojwayelekile waseMedithera

Umkhuhlane ojwayelekile weMedithera (FMF) yisifo esingajwayelekile esidluliselwa emindenini (esizuzwe njengefa). Kubandakanya ukushisa okuphindaphindiwe nokuvuvukala okuvame ukuthinta ulwelwesi lwesisu, isifuba, noma amalunga.

I-FMF ivame ukubangelwa ukuguqulwa kofuzo okuthiwa I-MEFV. Lesi sakhi sakha iphrotheni ebambe iqhaza ekulawuleni ukuvuvukala. Lesi sifo sivela kuphela kubantu abathola amakhophi amabili ohlobo olushintshiwe, elilodwa kumzali ngamunye. Lokhu kubizwa ngokuthi yi-autosomal recessive.

I-FMF ivame ukuthinta abantu bozalo lwaseMedithera. Lokhu kufaka ama-non-Ashkenazi (Sephardic) amaJuda, ama-Armenia kanye nama-Arabhu. Abantu abavela kwezinye izinhlanga nabo bangathinteka.

Izimpawu zivame ukuqala phakathi kweminyaka yobudala eyi-5 nengu-15. Ukuvuvukala kolwelwesi lwesisu, isifuba, isikhumba, noma amalunga kwenzeka kanye nemfiva ephezulu evame ukuphakama emahoreni ayi-12 kuye kwangama-24. Ukuhlaselwa kungahluka ngobukhulu bezimpawu. Abantu ngokuvamile abanazimpawu phakathi kokuhlaselwa.

Izimpawu zingafaka iziqephu eziphindiwe ze:

• Ubuhlungu besisu
• Ubuhlungu besifuba obubukhali futhi buba bubi kakhulu lapho uphefumula
• Imfiva noma ukushisa okushintshanayo nomkhuhlane
• Ubuhlungu obuhlangene
• Izilonda zesikhumba ezibomvu futhi ezivuvukile futhi zisuka ku-5 kuye ku-20 cm ububanzi

Uma ukuhlolwa kofuzo kukhombisa ukuthi unayo I-MEFV ukuguquka kwezakhi zofuzo nezimpawu zakho kufana nephethini ejwayelekile, ukuxilongwa kucishe kuqiniseke. Ukuhlolwa kwelabhorethri noma ama-x-ray kungakhipha ezinye izifo ezingaba khona ukusiza ukuxilongwa.

Amaleveli okuhlolwa kwegazi athile angaphakama kunokujwayelekile lapho kwenziwa ngesikhathi sokuhlaselwa. Ukuhlolwa kungafaka:

• Ukubalwa kwegazi okuphelele (CBC) okubandakanya ukubalwa kwamaseli amhlophe egazi
• Amaprotheni asebenza nge-C ukuhlola ukuvuvukala
• Izinga le-erythrocyte sedimentation (ESR) ukuhlola ukuvuvukala
• Ukuhlolwa kwe-Fibrinogen ukuhlola ukunqamuka kwegazi

Umgomo wokwelashwa kwe-FMF ukulawula izimpawu. IColchicine, umuthi owehlisa ukuvuvukala, ungasiza ngesikhathi sokuhlaselwa futhi ungavimbela okunye ukuhlaselwa. Kungasiza futhi ukuvimbela inkinga enkulu ebizwa nge-systemic amyloidosis, ejwayelekile kubantu abane-FMF.

Ama-NSAID angasetshenziselwa ukwelapha umkhuhlane nobuhlungu.

Alikho ikhambi elaziwayo le-FMF. Iningi labantu liyaqhubeka nokuhlaselwa, kepha isibalo nobukhulu bokuhlaselwa kuhlukile kumuntu nomuntu.

I-Amyloidosis ingaholela ekulimaleni kwezinso noma ukungakwazi ukuncela izakhamzimba ekudleni (malabsorption). Izinkinga zokuzala kwabesifazane nakwabesilisa nesifo samathambo nakho kuyinkinga.

Xhumana nomhlinzeki wakho uma wena noma ingane yakho iqala izimpawu zalesi simo.

I-paroxysmal polyserositis ejwayelekile; I-peritonitis yesikhathi; I-polyserositis ephindaphindiwe; I-Benign paroxysmal peritonitis; Izifo zesikhathi; Umkhuhlane wesikhathi; I-FMF

• Isilinganiso sethempelesha

I-Verbsky JW. Ama-syndromes we-Hereditary periodic fever nezinye izifo zesistimu ezenzakalelayo. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 188.

Umkhuhlane ojwayelekile waseMedithera kaShohat M. Ku: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, abahleli. Ukubuyekezwa kweGeneReviews [Inthanethi]. I-University of Washington, Seattle, WA: 2000 Aug 8 [ibuyekezwe ngo-2016 Dec 15]. I-PMID: 20301405 www.pubmed.ncbi.nlm.nih.gov/20301405/.