Ingabe ingane yakho ivame ukuba nomkhuhlane ngaphandle kwesizathu? Ake sifunde ngalezi zifo ze-SAID (Systemic Autoinflammatory Diseases)!

Ingabe ingane yakho encane ngezinye izikhathi ivele ibe nomkhuhlane ngaphandle komkhuhlane noma olunye ukugula? Ingabe umkhuhlane uyafika, uphele ezinsukwini ezimbalwa, bese uphinda uvele ezinsukwini ezimbalwa kamuva? Abanye abazali babhekana nokuhlangenwe nakho okunjalo. Ngakho-ke namuhla sizokhuluma ngembangela yomkhuhlane evame kakhulu futhi okunzima ukuyithola. Lokhu kubizwa ngokuthi ama-SAID (Izifo Ezizivuvukalayo Ezisemzimbeni) . Esikhathini esidlule, lokhu kwakubizwa ngokuthi `(Periodic Fever Syndromes)` noma `(Recurrent Fever Syndromes)`.

Ziyini lezi SAID? Masiqonde kalula!

Kalula nje, ama-SAID ayiqembu lezifo ezibangelwa ukungasebenzi kahle noma inkinga yokulawula ohlelweni lokuzivikela lomzimba esizalwa nalo . Kulesi simo, ngaphandle kokutheleleka kwangaphandle (njengegciwane noma amagciwane), umzimba wethu umane udale ukuvuvukala. Kunjengokungathi uhlelo lokuzivikela lomzimba wethu luya ngokweqile.

Cabanga ngakho, sineqembu lamasosha ngaphakathi emizimbeni yethu (lokho kuyisimiso somzimba sokuzivikela). Umsebenzi wabo ukulwa namagciwane abangela izifo uma engena. Kodwa emzimbeni womuntu one-SAIDs, la masosha ngezinye izikhathi ayaphazamiseka futhi abangele izinkinga ngaphakathi emzimbeni.

Manje ungase uzibuze ukuthi lokhu kufana yini nokuthi `(izifo ezizimele)` (okungukuthi, izifo ezizimele). Isibonelo, `(i-rheumatoid arthritis)` noma `(i-lupus)` ziyafana. Cha, lezi ezimbili zihlukile kancane. Ezifweni ezizimele, okwenzekayo ukuthi isimiso somzimba wethu sokuzivikela esitholwe noma esizivumelanisa nezimo sihlasela ngephutha amangqamuzana aso anempilo. Kodwa kuma-SAID, inkinga isesimisweni somzimba sokuzivikela esingokwemvelo.

Ama-SAID awavamile kakhulu kunezifo ezizimele. Iningi lawo liyifa , okusho ukuthi abangelwa ukuguquka kwezakhi zofuzo noma uhlobo oluthile.

Ama-SAID avame ukuqala ebuntwaneni noma ebuntwaneni . Kodwa lokho akuvamile. Ingane yakho ingaba neziqephu noma ukuhlaselwa umkhuhlane nezinye izimpawu. Ingane yakho ingase ingabi nazimpawu nhlobo phakathi kokuhlaselwa. Ayikho ikhambi lama-SAID. Kodwa-ke, kunezindlela zokwelapha ezingasiza ukulawula izimpawu ezimweni eziningi.

Yiziphi izinhlobo eziyinhloko ze-SAIDs?

Abacwaningi sebethole izinhlobo ezingaba ngu-60 ze-SAID, futhi ezinye ziyatholakala. Ake sibheke ezinye zezinhlobo ezivame kakhulu.

• I-Familial Mediterranean Fever (FMF): Lesi yisifo esivame kakhulu esizuzwe ngofuzo, esiphindaphindayo. Singabangela ukuvuvukala okubuhlungu esiswini, esifubeni nasemalungeni engane .
• Umkhuhlane ovamile, i-aphthous-stomatitis, i-pharyngitis, i-adenitis (PFAPA): Lokhu kuvame ukuqala ezinganeni ezincane ngaphambi kweminyaka engu-4. Lesi simo singase siphele ngokuzenzekelayo ngemva kweminyaka engu-10.
• I-Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Lokhu kungenzeka ebuntwaneni, ebusheni, futhi ngezinye izikhathi ngisho nasebudaleni.
• Ukuntuleka kwe-Mevalonate kinase (MKD): Ngaphambilini kwakuthiwa yi-hyper-IgD syndrome, lokhu kuvame ukuqala ezinganeni ezingaphansi konyaka owodwa ubudala.
• Izifo zokuvuvukala okuhambisana ne-NLRP3: Lokhu ngaphambilini kwakubizwa ngokuthi ama-cryopyrin-associated periodic syndromes (CAPS). Kunezinhlobo ezintathu zalesi sifo.
• Isifo sika-Adult-onset Still (AOSD): Lolu uhlobo lokuqala kwesifo samathambo esihlasela abesilisa nabesifazane (i-JIA) esihlasela abantu abadala.
• Isifo se-granulomatous esihlobene ne-NOD-2 (i-Blau syndrome): Lokhu kuvame ukuqala ngaphambi kweminyaka engu-4. Kuthinta kakhulu isikhumba, amehlo, kanye namalunga engane.

Ziyini izimpawu ze-SAID? Sizibona kanjani ?

Izimpawu ze-SAID zivame ukuqala ebuntwaneni. Uphawu oluvame kakhulu noluvelele umkhuhlane oqhubekayo noma oqhubekayo . Abantu bavame ukuhlala bengenazo izimpawu phakathi nokuhlaselwa. Kodwa-ke, kungase kube nezinye izimpawu eziqondene nohlobo ngalunye lwe-SAID.

Ake sibheke izimpawu ezithile zezinhlobo eziningana ze-SAID okukhulunywe ngazo ngenhla:

• I-FMF: Lokhu kungabangela ubuhlungu obukhulu esiswini sengane, esifubeni, nasemalungeni, kanye nokuvuvukala . Ngezinye izikhathi, ukuqubuka kungavela emilenzeni engezansi noma emaqakaleni. Cabanga nje, ingane ivele ibe nobuhlungu besisu kanye nomkhuhlane ngokuzumayo. Lapho abazali bekhathazekile futhi beyiyisa esibhedlela, bangase bacabange ukuthi yi-appendicitis. Kodwa-ke iyaphela bese ibuya ngemva kwezinsuku ezimbalwa.
• I-PFAPA: Lokhu kungabangela izimpawu ezifana nomphimbo obuhlungu, izilonda emlonyeni, kanye nokuvuvukala kwama-lymph node entanyeni .
• IZINGCINGO: Lokhu kungabangela ukubanda, ubuhlungu bemisipha esifubeni nasezingalweni , kanye nokuqubuka okubomvu okubuhlungu okuqala ezingalweni nasemilenzeni bese kusakazeka emzimbeni wonke.
• MKD: Nakulokhu futhiUngase uzizwe ungaphilile kahle ngenxa yokubanda, ikhanda elibuhlungu, ubuhlungu besisu, ukuphelelwa yisifiso sokudla, kanye nezimpawu ezifana nomkhuhlane.
• Izifo ze-NLRP3: Lezi zingabangela izimpawu ezifana nokuqubuka kwesikhumba, ikhanda elibuhlungu, ubuthakathaka, ubuhlungu bamalunga, kanye namehlo abomvu (conjunctivitis) .
• I-AOSD: Lesi yisimo esivame ukubangela ukuqubuka kwesikhumba, ubuhlungu bamalunga, kanye nobuhlungu bemisipha . Abanye abantu bangase babe nomphimbo obuhlungu, ubuhlungu besisu, ukukhathala okukhulu, kanye nobuhlungu bomzimba.
• I-Blau syndrome: Lokhu kungabangela izilonda zesikhumba ezandleni, ezinyaweni, noma emzimbeni wengane yakho . Kungabangela nobuhlungu bamalunga kanye nobuhlungu bamehlo .

Kungani la ma-SAID esenzeka? Ziyini izimbangela?

Iningi lama-SAID liyizifo zofuzo . Okusho ukuthi, isifo ngasinye sibangelwa ukuhlukahluka (ukuguquguquka) kwesakhi sofuzo esithile.

Ake sibheke izakhi zofuzo ezithonya izinhlobo eziningana ezinkulu ze-SAID:

• I-FMF: Lokhu kubangelwa yi-gene ebizwa ngokuthi `(MEFV gene)`. Le gene iyala ukuthi yenziwa kanjani iphrotheni ebizwa ngokuthi `(pyrin)`.
• I-PFAPA: Isizathu sangempela salokhu asikatholakali okwamanje .
• IZINGCINGO: Lokhu kubangelwa yi-gene ebizwa ngokuthi `(TNFRSF1A gene)`. Yilokhu okunikeza imiyalelo yokwenza iphrotheni ebizwa ngokuthi `(tumor necrosis factor receptor - TNFR)`.
• I-MKD: Isizathu salokhu yi-gene ebizwa ngokuthi `(i-MVK gene)`. Lokhu kufundisa ukukhiqizwa kweprotheni ebizwa ngokuthi `(i-mevalonic kinase)`.
• Izifo ze-NLRP3: Lokhu kubangelwa yi-gene ebizwa ngokuthi `(NLRP3 gene)`. Lokhu kunikeza imiyalelo yokwenza iphrotheni ebizwa ngokuthi `(cryopyrin)`.
• I-AOSD: Isizathu esiqondile salokhu asikatholakali okwamanje .
• I-Blau syndrome: Lokhu kubangelwa yi-gene ebizwa ngokuthi `(i-NOD2 gene)`. Lokhu kufundisa ukukhiqizwa kweprotheni ebizwa ngokuthi `(NOD2)`.

Ingabe la ma-SAID angabangela ezinye izinkinga?

Yebo, lokhu kungabangela izinkinga uma kungelashwa kahle . Uma ukuvuvukala kuqhubeka emzimbeni, kungaholela esimweni esibizwa ngokuthi "i-amyloidosis." Okusho ukuthi, uhlobo oluthile lweprotheni lufakwa ezinso zethu. Lokhu kungabangela umonakalo ohlala njalo ezinso . Yingakho kubalulekile ukuxilonga lesi sifo kusenesikhathi bese uthola ukwelashwa.

Udokotela usithola kanjani lesi simo se-SAID?

Eqinisweni, ukuthola lezi zifo ezivuvukalayo kungaba nzima kancane , njengoba izimpawu zazo zingafana nezinye izimo ezimbi njenge-lupus noma umdlavuza njenge-lymphoma. Ngakho-ke, kubalulekile ukubona udokotela oqeqeshwe ngokukhethekile ezifweni ezivuvukalayo, isazi samathambo, ukuze ahlole kahle futhi aphathe isimo sengane yakho.

Udokotela wezifo zamathambo wengane yakho uzosebenzisa izici eziningana ukuxilonga ama-SAID. Uzokubuza ngezimpawu zengane yakho nokuthi ngabe kukhona yini emndenini wakho onalezi zimo (umlando womndeni wezinto eziphilayo). Udokotela angase asole ukuthi ingane yakho ingaba nesifo somkhuhlane esivamile uma:

• Uma ingane inomkhuhlane ovamile .
• Uma kukhona emndenini onomlando womkhuhlane ojwayelekile onje .
• Uma ingane ingeyeqembu labantu abavame ukuba nomkhuhlane onjalo njalo .

Yiziphi izivivinyo ezenziwayo ngalokhu?

Ukuqinisekisa ukuxilongwa, udokotela wezifo zamathambo wengane yakho angase ancome ukuhlolwa okuningana. Lokhu kungafaka:

• Ukuhlolwa kwelebhu: Ukuhlolwa okufana ne-"C-reactive protein (CRP)`` kanye ne-"complete blood count (CBC)`` kungasetshenziswa ukuhlola ukuvuvukala emzimbeni. Lokhu kuhlolwa kuba nomthelela omuhle ngesikhathi "sokuhlasela" komkhuhlane futhi kubuyela esimweni esijwayelekile lapho "ukuhlasela" sekuphelile.
• Ukuhlolwa komchamo: Ukuhlolwa kwephrotheni yomchamo kungenziwa ukuhlola iphrotheni eningi emchameni. Ku-MKD, ukuhlolwa komchamo okubheka ama-organic acid (ama-acid aqukethe i-carbon atom eyodwa) kungabonisa amazinga aphezulu e-mevalonic acid.
• Ukuhlolwa kofuzo: Lokhu kungahlola ukuhlukahluka kofuzo. Kodwa-ke, ezinye izingane ezine-SAIDs zingase zingatholakali ukuhlukahluka kofuzo, okungaholela emiphumeleni yokuhlolwa engemihle.

Yiziphi izindlela zokwelapha lezi zifo ze-SAID?

Ukwelashwa kwe-SAID kuyahlukahluka kuye ngohlobo kanye nobunzima besimo . Akukho ukwelashwa kwalezi zimo. Kodwa-ke , izimpawu zengane yakho ngokuvamile zingalawulwa ngemithi . Uma ingane yakho ihlaselwa kambalwa ngonyaka, ungase ukwazi ukuyinika imithi yobuhlungu njengemithi yokulwa nokuvuvukala engeyona i-steroidal (NSAIDs) ukusiza ukunciphisa izimpawu zayo. Kodwa-ke, uma isimo sibi kakhulu, udokotela wakho angaphakamisa ezinye izindlela zokwelapha.

Nazi ezinye zazo:

• I-FMF: Umuthi obizwa ngokuthi `(colchicine)` ungasetshenziswa ukulawula lokhu. Lokhu kunciphisa ukuvuvukala. Uma ingane ingakwazi ukuthatha i-colchicine, uhlobo lomuthi `(biologic)` olubizwa ngokuthi `(canakinumab)` lungazanywa.
• I-PFAPA: Ama-steroid (ngokuvamile i-prednisone) anganikezwa ukuze kufinyezwe isikhathi "sokuhlasela" kwe-PFAPA. Kwabanye abantwana, i-cimetidine, umuthi osetshenziselwa ukwelapha izilonda zesisu, ingasiza futhi ekulawuleni izimpawu.
• IZINGCINGO: Umuthi obizwa ngokuthi `(canakinumab)` uvame ukusebenza kahle kulokhu. Futhi, izimpawu zingadambisa ngemithi yokulwa nokuvuvukala enqunywe udokotela, njenge `(glucocorticoids)`.
• I-MKD:I-Canakinumab nayo iyindlela yokwelapha ephumelelayo ye-MKD. Ukunikeza ama-NSAID noma ama-steroid ngesikhathi "sokuhlaselwa" nakho kungaba usizo.
• Izifo ze-NLRP3: Ama-immunomodulators afana ne-canakinumab, i-rilonacept, kanye ne-anakinra asetshenziswa ngempumelelo kulokhu.
• I-AOSD: Izinhlobo ezahlukene zemithi yokulwa nokuvuvukala zisetshenziselwa i-AOSD. Izibonelo zifaka phakathi ama-steroid, imithi yokulwa namathambo eguqula izifo (ama-DMARD), kanye nemithi yezinto eziphilayo.
• I-Blau syndrome: Kuye ngezimpawu zengane yakho, ingase izame imithi yokucindezela amasosha omzimba, imithi yokuvimbela i-tumor necrosis factor (TNF), kanye/noma imithi yamehlo esetshenziswa endaweni yokunakekelwa kwamehlo.

Ingabe lezi zimo ze-SAID zihlala njalo? Noma zizophela?

Ezinye izimo zokuvuvukala okuzenzakalelayo zihlala isikhathi eside . Ezinye zingase zihlale iminyaka embalwa futhi ziphele njengoba ukhula. Ezinye izimo zihlala isikhathi eside, kodwa ngokuhamba kwesikhathi, ukuvama nobukhali bokuhlaselwa kungase kunciphe. Udokotela wengane yakho angakunikeza ulwazi oluthile mayelana nesimo sengane yakho.

Ukukhulisa ingane enesifo sokuvuvukala komzimba kungaba yinselele. Uma une-SAID, ungasebenzisa okuhlangenwe nakho kwakho ukuze usekele kangcono ukunakekelwa kwengane yakho. Ungasebenzisa futhi okuhlangenwe nakho kwakho ukusiza ingane yakho ijwayele lesi simo sempilo yonke.

Into ebaluleke kakhulu ukufuna ukwelashwa kochwepheshe abanolwazi ngama-SAID. Odokotela abanjalo bangasiza ekuphatheni izimpawu zengane yakho futhi bayisize ibe nobuntwana obuhle kakhulu.

Umyalezo Wokugcina Wokuya Nasekhaya

Ngakho-ke, ngithemba ukuthi unawo umbono ngama-SAID esikhulume ngawo namuhla. Khumbula, uma ingane yakho inemfiva evame ukungachazeki, khuluma nodokotela ngayo nakanjani . Ungamane uyishaye indiva, ucabanga ukuthi kuyinto evamile.

• Ama-SAID ayiqembu lezifo ezivame ukubangela umkhuhlane futhi ezibangelwa inkinga ngesimiso somzimba sokuzivikela.
• Lokhu kuvame ukuba yizakhi zofuzo futhi kuqala esemncane.
• Nakuba kungekho ikhambi elihlala njalo lalokhu, kunezindlela zokwelapha ezingalawula izimpawu.
• Kubalulekile ukufuna ukuxilongwa okufanele kanye nokwelashwa kudokotela ochwepheshe (i-Rheumatologist).
• Uma ukwelashwa kuqalwe kusenesikhathi, izinkinga ezifana ne-amyloidosis zingavinjelwa futhi ingane ingaphila impilo evamile.

Uma uneminye imibuzo mayelana nalokhu, ungangabazi ukubuza udokotela womndeni wakho noma udokotela wezingane.

Ama -SAID, Izifo Ezivuvukala Ngokwesimiso, Umkhuhlane Ovamile, Izifo Zofuzo, Umkhuhlane Ezinganeni, Isimiso Sokuzivikela Emzimbeni, Isazi Sezifo Zamathambo