Ngaba ufumana umkhuhlane rhoqo ngaphandle kwesizathu? Ngaba unezinye iingxaki kunye nawo? Oku kusenokuba yi-'Familial Mediterranean Fever (FMF)'!

Ngaba ngequbuliso ufumana umkhuhlane ngaphandle kwesilumkiso? Ngaba uneentlungu zesisu, ukuziva uxinezelekile esifubeni sakho, okanye iintlungu zamalungu? Ngamanye amaxesha ezi mpawu ziyaphela emva kweentsuku ezimbalwa, kodwa ziphinde zivele emva kwexesha elithile? Kuqhelekile ukuziva usoyika kakhulu xa oku kusenzeka. Namhlanje siza kuthetha ngesifo esibangela ezi mpawu, esingaqhelekanga kancinci, kodwa kubaluleke kakhulu ukwazi ngaso.

Yintoni i-'Familial Mediterranean Fever (FMF)'?

Ngamafutshane, iFamilial Mediterranean Fever, okanye i-FMF ngamafutshane, yimeko yobomi bonke evela kwiijini zethu. Okwenzekayo koku kukuba, ngaphandle kwesizathu esicacileyo, ngequbuliso sifumana umkhuhlane, kwaye kwangaxeshanye, kukho ukudumba kwiindawo ezahlukeneyo zomzimba. Ukuchaneka, oku kwenzeka ngenxa yengxaki encinci kwinkqubo yethu yokuzikhusela. Yiyo loo nto ikwabizwa ngokuba sisifo sokudumba okuzenzakalelayo .

Ichaphazela kakhulu abantu abahlala kumazwe aseMeditera. Ibangela ukudumba kwee-membranes ezibhityileyo ezibizwa ngokuba yi-'serous membranes' (ezi zigubungela iindawo ezifana nomgodi wesifuba kunye nomgodi wesisu), ezibizwa ngokuba yi-'serositis'. Inokubangela nokudumba kwee-'synovial membranes' ezigubungela amalungu ethu, ezibizwa ngokuba yi-'synovitis'. Iimpawu zihlala iintsuku ezimbalwa zize ziphele.

Zithini iimpawu ze-FMF?

Iimpawu ze-FMF zihlala ziqala ebuntwaneni, ngaphambi kokuba zibe neminyaka engama-20 ubudala. Ezi mpawu zinokuba nzima ekuqaleni, kodwa zinokuphucuka kancinci kancinci njengoko ukhula. Wakuba unesifo, usenokungabi nazimpawu kangangeeveki, iinyanga, okanye iminyaka. Nangona kunjalo , ukufunyaniswa kwangethuba kunye nonyango kunokunceda ukunciphisa iimpawu kunye nokuthintela iingxaki zexesha elide ezifana ne-amyloidosis.

Ngoku makhe sibone ukuba zeziphi ezi mpawu:

• Umkhuhlane ophezulu ofikelela kwi-40.6 degrees Celsius (105 Fahrenheit), ngamanye amaxesha uhamba nokubanda nokungcangcazela.
• Isenokuza nentloko ebuhlungu kunye nentamo eqinileyo.
• Intlungu yesisu kunye nokudumba (ukudumba).
• Izihlunu zesisu ziyaqina kwaye ukuqhinwa kusenokubakho.
• Intlungu yesifuba , eyandayo xa uphefumla.
• Intlungu kunye nokudumba kwamalungu (ngokuqhelekileyo kwilungu elinye).
• Ukudumba kunye nentlungu emasendeni kumadoda.
• Iintlungu zemisipha , ingakumbi kumzimba ongezantsi.
• Isikhumba esibomvu nesiphakanyisiweyo sinokukhula emilenzeni .

Ezi mpawu zinokuvela ngequbuliso kwiiyure ezimbalwa. Nangona kunjalo, ayinguye wonke umntu oza kuzibona zonke ezi mpawu.. Umkhuhlane lolona phawu luqhelekileyo. Ebuntwaneni, umkhuhlane usenokuba lolona phawu kuphela. Esi sifo sihlala iintsuku ezimbalwa.

Iimpawu zeProdromal ze-FMF

Abanye abantu baqala ukuziva iimpawu ezincinci kwiintsuku ezimbalwa ngaphambi kokuba lo mkhuhlane uqale. Njengokuba abanye abantu beziva ngaphambi kokuba "umhlaza" okanye abafazi beziva ngaphambi kokuba "baye exesheni". Ezi mpawu sizibiza ngokuba " ziimpawu zokukhulelwa". Xa uziva ezi, ufumana umbono wokuba "Ah... ngoku loo ngxaki iyeza".

Ziziphi ezi mpawu zibalulekileyo?

• Ixhala, ukungazinzi
• Ukucaphuka
• Intloko ebuhlungu
• Isicaphucaphu
• Intlungu yomzimba
• Ukungabi namdla ( ukungabi namdla, ukungabi namdla )

Kutheni le nto isenzeka le `FMF`?

Oku kungenxa yotshintsho kwijini ye-'MEFV' emizimbeni yethu. Le jini ye-'MEFV' inceda ekwenzeni iproteni ebaluleke kakhulu, oko kukuthi iproteni ebizwa ngokuba yi-'pyrin'. Inkqubo yethu yokuzikhusela yomzimba - oko kukuthi, inxalenye yenkqubo yokuzikhusela yomzimba esebenza ngokuchasene nezingozi ezifana nosulelo olukhawulezileyo - ixhomekeke kule protein ye-'pyrin'. Ngoko ke, xa ijini ye-'MEFV' ingakwazi ukwenza i-'pyrin' ngokufanelekileyo, loo protein ayinakusebenza ngokufanelekileyo ngaphakathi kwenkqubo yokuzikhusela yomzimba. Kulapho ke le ngxaki ivele khona.

Kuye kwachongwa malunga nama-30 eenguqu zemfuza (`utshintsho`) ezibangela i-`FMF`. Kwezi, ezixhaphakileyo yi-`V726A`, `M680I`, `E148Q`, `M694V` kunye ne-`M694I`. Ezi zixhaphake kakhulu kubemi baseMeditera naseMbindi Mpuma. Umzekelo:

• Ama-Arabhu
• AmaArmenia
• AmaGrike
• Ama-Iran
• AmaTaliyane
• AmaJuda
• AmaKurd
• AmaTurkey

Izinto ezibangela i-FMF

Nangona i-FMF ibonakala ngathi ivela ngequbuliso kwaye ngaphandle kwesizathu esicacileyo, kwabanye abantu ibangelwa zizinto ezithile. Kwabanye abantu, le meko inokubangelwa luxinzelelo lomzimba okanye lwengqondo. Abantu bathi i-FMF inokubangelwa zizinto ezifana nezi:

• Ukuvezwa kumkhuhlane
• Ukuzilolonga okanye ukudinwa kakhulu
• Usulelo lwakutshanje
• Ukulimala okanye utyando lwakutshanje
• Uxinzelelo olukhulu (njengengxaki enkulu ebomini)
• Ixesha apho abafazi besiya exesheni okanye besiya kwi-ovulation

Iingxaki ezinokubakho ze-FMF

Nangona umkhuhlane we-FMF uphela emva kweentsuku ezimbalwa, ukuvuvukala okuqhubekayo emzimbeni kunokuchaphazela imizimba yethu ngokuhamba kwexesha. Yiyo loo nto oogqirha becebisa unyango oluqhubekayo koku. Xa kukho inkqubo yokuvuvukala okuqhubekayo efana nale, inkqubo yethu yomzimba yokuzikhusela ifakwa phantsi koxinzelelo olukhulu, kwaye nezinye izifo zinokuvela.

Eyona ngxaki inzima yoku yimeko ebizwa ngokuba yi-'secondary amyloidosis' . Oku kunokukhokelela nasekungasebenzini kwezintso. Yiyo loo nto kubaluleke kakhulu ukuba uqaphele oku.

Ezinye iingxaki zingabandakanya:

• I-Splenomegaly (i-splenomegaly)
• Ukungazali
• Ezinye izifo ezihlasela umzimba (`Isifo sesibini esihlasela umzimba`)

Ifunyaniswa njani i-FMF?

Ugqirha uyixilonga njani i-FMF? Benza izinto ezinje:

• Baza kumamela ngenyameko iimpawu zakho, ukuba zivela kangaphi, kwaye zikho ixesha elingakanani .
• Baza kubuza ukuba kukho umntu kusapho lwakho okhe waba nesi sifo kwaye yintoni ilifa lakho lemfuza.
• Xa ugula, baza kukuhlola ukuze babone ukuba akukho na ukudumba okanye ukurhawuzelelwa emzimbeni wakho .
• Ngexesha lokugula , kwenziwa uvavanyo lwegazi ukuze kubonwe ukuba kukho naziphi na iimpawu zokudumba .
• Uvavanyo lwemfuza luyacetyiswa ukujonga utshintsho kwi-MEFV gene, edla ngokubangela i-FMF.
• Baza kujonga nendlela osabela ngayo kunyango lwe-FMF.

Unyango lwe-'FMF`

Unyango oluphambili lwe-FMF liyeza elilwa nokuvuvukala i-colchicine. Oku kusebenza ngokunciphisa umsebenzi wohlobo lweseli emhlophe yegazi ebizwa ngokuba yi-neutrophils, ebandakanyeka ekuvuvukeni. I-Colchicine inokunceda ukuthintela okanye ukunciphisa ukuvela rhoqo kwe-FMF, kwaye inciphise umngcipheko weengxaki. Abantu abangaphezu kwama-90% abane-FMF bayaphiliswa . Uninzi lwabantu kufuneka bathathe eli yeza yonke imihla ubomi babo bonke.

Nangona kunjalo, inani elincinci kakhulu labantu alifumani ziphumo ezifunwayo kwi-colchicine, okanye basenokungakwazi ukulithatha eli yeza ngenxa yezinye izizathu zempilo. Kubantu abanjalo, oogqirha bacebisa ezinye iintlobo zamayeza. Ezinye zazo zezi:

• `Anakinra` (`Anakinra`)
• `I-Rilonacept`
• `I-Canakinumab`

Kwenzeka ntoni ukuba une-`FMF`?

Nangona kungekho nyango lwe-FMF, inokulawulwa kakuhle ngamayeza . Kwabaninzi abantu, umkhuhlane kunye neentlungu zomzimba ziyaphela ngokupheleleyo xa beqala ukuthatha amayeza. Kwabanye, ukuphindaphindeka kunye nokuphindaphindeka kokuqhambuka kwesifo kuyancipha. Nangona kunjalo, ukuze la mayeza asebenze kakuhle, kufuneka athathwe rhoqo, nokuba akanazo iimpawu.Ukusela eli yeza xa sele ugula akuyi kuyiphelisa loo nto ngoko nangoko. Eli yeza lithintela kuphela ukuba isifo singabikho.

Ngaba i-FMF ingathintelwa?

Abantu abaninzi abazi nokuba bane-gene variant ebangela i-FMF. Ukuze ufumane i-FMF, kufuneka ufumane iikopi ezimbini ze-gene variant kumama wakho nakutata wakho. Ukuba unekopi enye kuphela, awuyi kuba nazimpawu ze-FMF. Kodwa ungadlulisela loo gene variant kubantwana bakho. Ukuba iqabane lakho nalo linayo loo gene variant, abantwana bakho banokuba ne-FMF. Uvavanyo lwe-genetic lunokukunceda ufumanise ukuba ungumthwali we-gene variant.

Bekukho iingxelo zokuba kukho imikhuhlane exhaphakileyo ukususela mandulo. Mhlawumbi esi sifo sibizwa ngokuba yi-'FMF' sidala kakhulu. Abanye abaphandi bacinga ukuba le meko isenokuba yavela njengendlela yokunceda ukulwa nosulelo olukhulu olufana 'nesifo sofuba' olwaluxhaphakile ngelo xesha. Enyanisweni, kukho iingenelo ezithile zokuba nokudumba emzimbeni, kodwa kuphela xa kuba nzima kakhulu apho kuba yingxaki. Ngethamsanqa, ngoku kukho amayeza alungileyo okulawula le meko. Ngoko ke nokuba une-'FMF', ungaphila ubomi obuqhelekileyo.

Eyona nto ibalulekileyo ekufuneka uyikhumbule! (Umyalezo oya nawo ekhaya)

Kulungile, ngoku uyaqonda ngcono into esithethe ngayo namhlanje, `FMF`. Eyona nto ibalulekileyo kukuba ukuba wena okanye umntu kusapho lwakho unomkhuhlane ngaphandle kwesizathu, isisu esibuhlungu, iintlungu zamalungu, njl.njl., musa ukuwuthatha njengomkhuhlane nje, kodwa bona ugqirha ukuze ufumane ukuba kwenzeka ntoni kanye kanye. Ukuba uyasiqonda esi sifo kwaye uqale unyango kwangethuba, unokuthintela iingxaki ezininzi kwaye uphile ubomi obuqhelekileyo. Khumbula, kubaluleke kakhulu ukusebenzisa amayeza achazwe ngugqirha wakho ngqo, njengoko kuyalelwe. Ungalibali ukuthetha nogqirha wakho ngayo nayiphi na imibuzo okanye iinkxalabo onokuba nazo.

I- FMF, isifo somkhuhlane wosapho, umkhuhlane oqhelekileyo, iintlungu zesisu, iintlungu zamalungu, izifo zofuzo, i-colchicine, i-amyloidosis