Yintoni i-familial Mediterranean fever (FMF)?

I-Family Mediterranean Fever yi-autosomal recessive hereditary disease ezibonakalisa ngezikhalazo zentlungu yesisu kunye nomkhuhlane ekuhlaselweni kwaye inokubhidaniswa ne-appendicitis enzima.

Yintoni isifo se-FMF (iFiva yeMeditera yoSapho)?

IFamilial Mediterranean Fever ibonwa rhoqo ngakumbi kumazwe akwimida yeMeditera. Ixhaphakile eTurkey, eMntla Afrika, eArmenia, eArabhu namaYuda. Ngokuqhelekileyo yaziwa njengeFamily Mediterranean Fever (FMF).

Isifo se-FMF sibonakaliswe yintlungu yesisu, intlungu kunye nokukhathazeka kwi-rib cage (plevititis) kunye nentlungu edibeneyo kunye nokudumba (i-arthritis) ngenxa yokuvuvukala kwesisu esiswini, esiphinda sihlasele kwaye sinokuhlala iintsuku ezi-3-4. Ngamanye amaxesha, ukubomvu kwesikhumba phambi kwemilenze nako kunokongezwa kumfanekiso. Ngokuqhelekileyo, ezi zikhalazo zinokuhamba ngokwazo kwiintsuku ezingama-3-4, nokuba akukho nyango lunikezelwayo. Ukuhlaselwa okuphindaphindiweyo kubangela ukuba iprotheni ebizwa ngokuba yi-amyloid iqokelele emzimbeni wethu ngokuhamba kwexesha. I-Amyloid idla ngokuqokelela kwizintso, apho inokubangela ukusilela kwezintso ezingapheliyo. Ngomlinganiselo omncinci, unokuqokelela kwiindonga ze-vascular kwaye kubangele i-vasculitis.

Iziphumo zeklinikhi zenzeka ngenxa yokuguquka kwejini ebizwa ngokuba yipyrin. Isasazwa ngokwemfuza. Nangona ubukho bemizila emibini egulayo kunye kubangela isifo, ukuthwala isifo sofuzo akubangeli isifo. Aba bantu babizwa ngokuba "ngabathwali".

Ziziphi iiMpawu zeFamily Mediterranean Fever (FMF)?

IFamily Mediterranean Fever (FMF) sisifo sofuzo esixhaphakileyo kwingingqi yeMeditera. Iimpawu ze-FMF zinokubonakalisa njengokubanjwa kwe-febrile, iintlungu ezibuhlungu zesisu, iintlungu ezidibeneyo, iintlungu zesifuba, kunye nohudo. Ukuxhuzula kweFebrile kuqala ngesiquphe kwaye kudla ngokuhlala kwiiyure ezili-12 ukuya kwezingama-72, ngelixa iintlungu zesisu zineempawu ezibukhali, ngakumbi ezijikeleze inkaba. Iintlungu ezidibeneyo zivakala ngokukodwa kumalungu amakhulu afana nedolo kunye ne-ankle, ngelixa iintlungu zesifuba zingenzeka kwicala lasekhohlo. Urhudo lunokubonwa ngexesha lokuhlaselwa kwaye ngokuqhelekileyo lunokuvakala ixesha elifutshane.

Sifunyaniswa njani isifo seFamily Mediterranean Fever (FMF)?

Uxilongo lwenziwa ngokusekelwe kwiziphumo zeklinikhi, imbali yentsapho, iziphumo zeemviwo kunye novavanyo lwebhubhoratri. Ezi mvavanyo, kunye nokuphakama okuphezulu kwe-leukocyte, ukunyuka kwe-sedimentation, ukuphakama kwe-CRP kunye nokuphakama kwe-fibrinogen, ukuxhasa ukuxilongwa kwe-Familial Mediterranean Fever. Inzuzo yovavanyo lwemfuzo kwizigulana zilinganiselwe ngenxa yokuba utshintsho oluchongiweyo ukuza kuthi ga ngoku lunokufumaneka kuphela kwi-80% yezigulane ze-Familial Mediterranean Fever. Nangona kunjalo, uhlalutyo lwezofuzo lunokuba luncedo kwiimeko ze-atypical.

Ngaba kunokwenzeka ukunyanga iFamilial Mediterranean Fever Disease (FMF)?

Kuye kwagqitywa ukuba unyango lwe-colchicine lwe-Familial Mediterranean Fever luthintela ukuhlaselwa kunye nokuphuhliswa kwe-amyloidosis kwinani elikhulu lezigulane. Nangona kunjalo, i-amyloidosis iseyingxaki enkulu kwizigulane ezingathobeli unyango okanye zilibaziseke ekuqaliseni i-colchicine. Unyango lwe-colchicine kufuneka lube ubomi bonke. Kuyaziwa ukuba unyango lwe-colchicine lunyango olukhuselekileyo, olufanelekileyo nolubalulekileyo kwizigulane ezinefiva yaseMeditera. Kunconywa ukusebenzisa nokuba isigulane sikhulelwe. I-Colchicine ayizange iboniswe ukuba ibe nefuthe elibi kumntwana. Nangona kunjalo, kucetyiswa ukuba abaguli abakhulelweyo abanefiva yeMeditera yosapho bafumane i-amniocentesis kwaye bajonge ubume bemfuza yomntwana.