Ingabe ingane yakho ivame ukuba nomkhuhlane ongachazeki? Lokhu kungaba ngenxa ye-SAIDs (Systemic Autoinflammatory Diseases)!

Ingabe ingane yakho encane ithola umkhuhlane ngaso sonke isikhathi? Ngezinye izikhathi ithola umkhuhlane kanye noma kabili ngenyanga, kodwa odokotela bathi akukho ukutheleleka, okungukuthi, akukho gciwane noma amagciwane emzimbeni. Ngezikhathi ezinjalo, njengomama noma ubaba, unengqondo kakhulu ukuzizwa wesaba kakhulu futhi ukhathazekile. "Kungani lokhu kwenzeka enganeni yami?" Cishe uke wacabanga izikhathi eziyinkulungwane. Mhlawumbe isizathu salokhu yinto ongakaze uyizwe ngaphambili. Namuhla, sizokhuluma ngama -SAID (Izifo Ezizivuvukalayo Ezihambisana Nokuvuvukala Kwesistimu), okunzima ukuyiqonda futhi okungabangela umkhuhlane ovamile. Esikhathini esidlule, lawa ayebizwa ngokuthi `(Periodic Fever Syndromes)` noma `(Recurrent Fever Syndromes)`.

Ziyini lezi zifo ze-SAID (Systemic Autoinflammatory Diseases)?

Kalula nje, ama-SAID ayiqembu lezifo ezenzeka ngenxa yokungasebenzi kahle noma inkinga yokulawula ohlelweni lokuzivikela lomzimba lwemvelo . Okwenzekayo ukuthi, ngaphandle kwesizathu sangaphandle, okungukuthi, igciwane noma amagciwane, umzimba umane udale ukuvuvukala. Yingakho lo mkhuhlane ovamile uvela.

Manje ungase ucabange, "O, ingabe lokhu kungenye yalezo Zifo Ezivikela Umzimba?" Cha, lezi ezimbili zihlukile kancane. KuZifo Ezivikela Umzimba, isibonelo, ezifweni ezifana ne-Rheumatoid Arthritis noma i-Lupus, isimiso somzimba wethu sokuzivikela esizivumelanisa nezimo sihlasela ngephutha amangqamuzana aso aphilile. Kodwa kuma-SAID, inkinga isesimisweni somzimba sokuzivikela esingokwemvelo.

Ama-SAID awavamile kakhulu kunezifo ezizivikela ngokuzenzakalelayo. Esikhathini esiningi, lawa adluliselwa ngofuzo , okusho ukuthi abangelwa ukuguquka kwezakhi zofuzo noma uhlobo oluthile.

Lawa ma-SAID ngokuvamile (kodwa hhayi njalo) aqala lapho ingane yakho iseyingane noma ingane encane . Ingane izoba neziqephu noma ukuhlaselwa yilesi sifo ngezikhathi ezithile. Phakathi nalezi ziqephu, umkhuhlane nezinye izimpawu zingase zivele. Kodwa-ke, phakathi kwalokhu "kuhlaselwa," ingane ingase ingabi nazimpawu. Akukho ukwelashwa kwama-SAID, kodwa kuvame ukuba khona ukwelashwa okuphumelelayo ukulawula izimpawu.

Yiziphi izinhlobo eziyinhloko ze-SAIDs?

Abacwaningi bathole izinhlobo ezingaba ngu-60 ze-SAID, futhi ezinye ziyatholakala. Nazi ezinye zezinhlobo ezivame kakhulu:

• Umkhuhlane waseMedithera womndeni (i-FMF): Lona umkhuhlane ovame kakhulu ezifweni zofuzo, ophindaphindayo. Ungabangela ukuvuvukala okubuhlungu esiswini, esifubeni nasemalungeni engane.
• Umkhuhlane ovamile, i-aphthous-stomatitis, i-pharyngitis, i-adenitis (PFAPA): Lokhu kuvame ukuqala ezinganeni ezincane, ngokuvamile ngaphambi kweminyaka engu-4. Kodwa-ke, kwezinye izingane, lesi simo singase siphele ngemva kweminyaka engu-10.
• I-Tumor necrosis factor receptor-associated periodic fever syndrome (i-TRAPS): Lokhu kungenzeka kusukela ebuntwaneni kuya ebudaleni, futhi ngezinye izikhathi kuze kube sebudaleni.
• Ukuntuleka kwe-Mevalonate kinase (MKD): Ngaphambilini okwakwaziwa ngokuthi i-Hyper-IgD syndrome, lokhu kuvame ukuqala ngaphambi kokuba ingane ibe nonyaka 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's (AOSD): Njengoba igama lisho, lesi sifo siqala lapho umuntu emdala. Siwuhlobo lomuntu omdala lwesimo esibizwa ngokuthi i-systemic juvenile idiopathic arthritis (JIA).
• 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?

Izimpawu ze-SAID zivame ukuqala ebuntwaneni. Uphawu oluyinhloko noluvame kakhulu umkhuhlane oqhubekayo noma oqhubekayo . Njengoba kushiwo ngaphambili, ingane ingase ibe ngcono ngezikhathi lapho kungekho mkhuhlane. Kodwa-ke, kunezinye izimpawu eziqondene nohlobo ngalunye lwe-SAID:

• Ku-FMF: Isisu sengane, isifuba, kanye namalunga kungase kube buhlungu kakhulu futhi kuvuvuke. Ngezinye izikhathi, kungase kube nokuqubuka kwesikhumba emilenzeni engezansi noma emaqakaleni.
• Ku-PFAPA: Umphimbo obuhlungu, izilonda emlonyeni, ama-lymph node avuvukele entanyeni. Lezi yizimpawu eziyinhloko.
• Ku-TRAPS: Umzimba ungase ubande futhi ube nomkhuhlane. Ingane ingase ibe nobuhlungu bemisipha esithweni nasezingalweni. Kungase kuvele ukuqubuka okubomvu okubuhlungu, okungase kusakazeke kusukela ezingalweni kuya emilenzeni bese kudlulela emzimbeni.
• Ku-MKD: Ungase ube nezimpawu ezifana nomkhuhlane njengokugodola, ikhanda elibuhlungu, ubuhlungu besisu, ukulahlekelwa yisifiso sokudla, nezinye izimpawu.
• Ezifweni ze-NLRP3: ukuqubuka kwesikhumba, ikhanda elibuhlungu, ubuthakathaka, ubuhlungu bamalunga, kanye nokuvuvukala kwamehlo (conjunctivitis) kungase kwenzeke.
• Ku-AOSD: ukuqubuka kwesikhumba, ubuhlungu bamalunga, ubuhlungu bemisipha. Abanye abantu bangase babe nomphimbo obuhlungu, ubuhlungu besisu, ukukhathala, kanye nomuzwa wobuthakathaka.
• Uma une-Blau syndrome: Ingane ingase ibe nezilonda zesikhumba ezingalweni, emilenzeni, noma phakathi nokuhlinzwa. Ingase futhi ibhekane nobuhlungu bamalunga kanye nobuhlungu bamehlo.

Cabanga nje, indodakazi encane kaNalini ibilokhu inomkhuhlane ophakeme izinsuku ezintathu kuya kwezine njalo ngenyanga ezinyangeni ezimbalwa ezedlule. Ngaphezu kwalokho, ithola nezilonda ezincane emlonyeni wayo kanye namaqhubu entanyeni yayo. Lapho eyikhombisa udokotela, wathi kungaba yi-PFAPA.

Yiziphi izimbangela ze-SAID?

Iningi lama-SAID liyizifo zofuzo . Lokhu kusho ukuthi ngayinye yalezi zifo ibangelwa ukuhlukahluka kwezakhi zofuzo zethu. Nazi izinhlobo ezivame kakhulu zama-SAID kanye nezakhi zofuzo eziwathintayo:

• I-FMF: I-gene ebizwa ngokuthi `(MEFV)`. Le gene iyala ukukhiqizwa kweprotheni `(pyrin)`.
• I-PFAPA: I-gene eqondile ethinta lokhu ayikakatholakali.
• IZINGCINGO: I-gene ebizwa ngokuthi `(TNFRSF1A)`. Yilapho imiyalelo yokwenza iphrotheni `(i-tumor necrosis factor receptor - TNFR)` ivela khona.
• I-MKD: I-gene `(MVK)`. Lokhu kufundisa ukukhiqizwa kweprotheni ebizwa ngokuthi `(mevalonic kinase)`.
• Izifo ze-NLRP3: I-gene ebizwa ngokuthi `(NLRP3)`. Lokhu kunikeza imiyalelo yokwenza iphrotheni ebizwa ngokuthi `(cryopyrin)`.
• I-AOSD: Isizathu esiqondile salokhu asikaziwa.
• I-Blau syndrome: I-gene `(NOD2)`. Lokhu kufundisa ukukhiqizwa kweprotheni `(NOD2)`.

Imininingwane yalezi zakhi zofuzo ingase ibonakale iyinkimbinkimbi kuwe. Kalula nje, lezi zakhi zofuzo zithinta ukukhiqizwa kwamaprotheni athile alawula ukuvuvukala emizimbeni yethu. Yingakho umzimba udala izimo zokuvuvukala ngaphandle kwesizathu.

Yini engenzeka uma ama-SAID engelashwa kahle?

Kubaluleke kakhulu ukuthola ukwelashwa okufanele kwala ma-SAID. Ngoba uma ukuvuvukala emzimbeni kuqhubeka, kungenzeka isimo esibizwa ngokuthi `(amyloidosis)`. Lokhu kusho ukuthi uhlobo oluthile lweprotheni lufakwa ezinso zethu, olungabangela umonakalo ohlala njalo ezinso . Ngakho-ke, uma kunezimpawu, kubaluleke kakhulu ukufuna iseluleko sezokwelapha ngaphandle kokuzishaya indiva.

Odokotela bawaxilonga kanjani ama-SAID?

Ukuxilonga ama-SAID kungaba yinselele ngoba izimpawu zingafana nezezinye izimo ezingathi sína, njenge-lupus noma i-lymphoma. Ngakho-ke , kungcono ukubona udokotela wamathambo, udokotela oqeqeshwe ngokukhethekile ezifweni zokuvuvukala , ukuze axilonge futhi aphathe kahle isimo sengane yakho.

Udokotela wezifo zamathambo wengane yakho uzobheka izinto eziningana ukuze ahlole ama-SAID. Uzokubuza ngezimpawu zengane yakho nokuthi ngabe kukhona yini emndenini wakho onomlando womkhuhlane ovamile. Udokotela angase asole lesi simo uma:

• Uma ingane inomkhuhlane ovamile .
• Uma kukhona emndenini onomlando womkhuhlane ovamile .
• Uma ingane ingeyeqembu lesizwe, lokho kunamathuba amaningi okuba nomkhuhlane onjalo njalo.

Yiziphi izivivinyo ezisetshenziswayo?

Ukuqinisekisa ukuxilongwa, udokotela wezifo zamathambo wengane yakho angase ancome ukuhlolwa okuningana. Ezinye zalezi zifo zifaka:

• Ukuhlolwa kwelebhu: Ukuhlolwa kwegazi okufana ne-`(C-reactive protein - CRP)` kanye ne-`(Complete blood count - CBC)` kungahlola ukuvuvukala emzimbeni. Lokhu kuhlolwa kuba `(positive)` uma unomkhuhlane futhi kubuyela esimweni esijwayelekile lapho umkhuhlane uphela.
• Ukuhlolwa komchamo: Umchamo uhlolwa amazinga aphezulu eprotheni. Ezimweni ze-MKD, umchamo uhlolwa namazinga aphezulu e-organic acid ebizwa ngokuthi i-``mevalonic acid''.
• Ukuhlolwa kofuzo: Ukuhlolwa kofuzo kungahlola izinhlobo zofuzo ezishiwo ngenhla. Kodwa-ke, ezinye izingane ezine-SAIDs zingase zingatholakali izinhlobo zofuzo, ngakho-ke imiphumela yokuhlolwa ingaba mibi.

Yiziphi izindlela zokwelapha i-SAID?

Ukwelashwa kwe-SAID kuncike ohlotsheni kanye nobunzima besimo . Akukho ukwelashwa kwalezi zimo, kodwa imithi ingasiza ekulawuleni izimpawu zengane yakho . Uma ingane yakho ihlaselwa kambalwa ngonyaka, ngokuvamile ungakwazi ukulawula izimpawu ngemithi yobuhlungu kanye nemithi yokulwa nokuvuvukala engeyona i-steroidal (NSAIDs). Kodwa-ke, uma isimo sibi kakhulu, udokotela wakho angase ancome ezinye izindlela zokwelapha:

• I-FMF: Lokhu kungelashwa ngomuthi obizwa ngokuthi `(Colchicine)` ukunciphisa ukuvuvukala. Uma `(Colchicine)` ingenakunikezwa ingane, ungazama uhlobo lomuthi `(biologic)` obizwa ngokuthi `(Canakinumab)`.
• I-PFAPA: Ama-steroid afana ne-prednisone anganciphisa isikhathi "sokuhlasela" kwe-PFAPA. I-Cimetidine, umuthi onikezwa ezinye izingane wezilonda zesisu, ungasiza futhi ekunciphiseni izimpawu.
• IZINGCONGO: Umuthi `(Canakinumab)` usebenza kahle kakhulu kwi-TRAPS. Futhi, izimpawu zingalawulwa ngemithi yokulwa nokuvuvukala efana ne-`(glucocorticoids)` enqunywe udokotela.
• I-MKD: `(Canakinumab)` iyindlela yokwelapha ephumelelayo ye-MKD. `(NSAIDs)` noma `(steroids)` ingasiza ngesikhathi "sokuhlaselwa".
• Izifo ze-NLRP3: Ama-immunomodulators afana ne-Canakinumab, i-Rilonacept, ne-Anakinra asetshenziswe ngempumelelo ekwelapheni izifo ezihlobene ne-NLRP3.
• I-AOSD: Imithi ehlukahlukene yokulwa nokuvuvukala njenge-steroids, imithi yokulwa namathambo eguqula izifo (i-DMARD), kanye ne-biologics isetshenziselwa ukwelapha i-AOSD.
• I-Blau syndrome: Kuye ngezimpawu zengane, imithi yokucindezela amasosha omzimba, imithi yokuvimbela i-tumor necrosis factor (TNF), kanye/noma imithi yamehlo esetshenziswa endaweni yokwelashwa ingazanywa.

Ukufunda ngale mithi kungakwenza uzizwe wesaba kancane. Kodwa khumbula, yonke le mithi inikezwa ngaphansi kokuqondisa okuqinile kukadokotela, ngendlela efanele ingane. Ngakho-ke, kubalulekile ukulandela ukwelashwa njengoba nje udokotela esho.

Ingabe isimo se-SAID siyaphela?

Amanye ama-SAID ayizimo zokuphila konke . Amanye angase ahlale iminyaka embalwa futhi aphele njengoba ekhula . Amanye amagciwane ahlala impilo yonke, kodwa ngokuhamba kwesikhathi, ukuvama kokuhlaselwa kungancipha futhi izimpawu zingase zingabi zimbi kakhulu. Udokotela wakho uzokuchazela imininingwane yesimo sengane yakho.

Ukunakekela ingane ene-SAID kungaba yinselele. Uma uke waba ne-SAID, ungasebenzisa ulwazi lwakho ukusiza ekunakekeleni ingane yakho. Ukuqonda kwakho kubalulekile nasekusizeni ingane yakho iphile nalesi simo sokuphila kwayo yonke impilo.

Into ebaluleke kakhulu ukufuna ukwelashwa kodokotela abanolwazi abanolwazi ngama-SAID. Bangasiza ekuphatheni izimpawu zengane yakho futhi bayisize ibe nobuntwana obuhle kakhulu.

Izinto ezibaluleke kakhulu okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ake siphinde sixoxe ngamanye amaphuzu abalulekile okudingeka uwakhumbule kulokho esikhulume ngakho:

• Uma ingane yakho inemfiva evame ukungachazeki , kungaba uphawu lwe-SAID.
• Ama-SAID awabangelwa ukutheleleka , kodwa ayinkinga ngesistimu yomzimba yokuzivikela yomzimba.
• Lezi yizifo ezingavamile ezivame ukubangelwa yizimbangela zofuzo .
• Izimpawu zingahluka kuye ngohlobo lwe-SAID, kodwa umkhuhlane ovamile uphawu oluyinhloko .
• Kubalulekile ukubona udokotela wezifo zamalunga ukuze uthole ukuxilongwa nokwelashwa okufanele.
• Nakuba ukwelashwa kungeke kuselaphe ngokuphelele lesi sifo, izimpawu zingalawulwa kahle .
• Ungazinaki izimpawu, ngoba zingaholela ekulimaleni kwezinso uma zingelashwa.

Uma uneminye imibuzo mayelana nalokhu, ungangabazi ukukhuluma nodokotela womndeni wakho noma udokotela wamathambo. Bazokwazi ukukusiza kabanzi.

Ama -SAID, izifo zomkhuhlane eziphindaphindayo, umkhuhlane ophindaphindayo, izifo ezivuvukalayo, umkhuhlane, umkhuhlane ezinganeni, izifo zofuzo