Ngaba umntwana wakho uhlala enomkhuhlane ngaphandle kwesizathu? Masifunde ngezi SAIDs (Systemic Autoinflammatory Diseases)!

Ngaba umntwana wakho omncinci ngamanye amaxesha ufumana umkhuhlane ngaphandle komkhuhlane okanye esinye isifo? Ngaba umkhuhlane uyafika, uphele emva kweentsuku ezimbalwa, uze ubuye emva kweentsuku ezimbalwa? Abanye abazali bajongana namava anjalo. Ngoko ke namhlanje siza kuthetha ngesizathu esixhaphakileyo nesinzima ukuyifumana somkhuhlane. Oku kubizwa ngokuba yi-SAIDs (Systemic Autoinflammatory Diseases) . Ngaphambili, oku kwakubizwa ngokuba yi-`(Periodic Fever Syndromes)` okanye `(Recurrent Fever Syndromes)`.

Zithini ezi SAIDs? Masiziqonde ngokulula!

Ngamafutshane, ii-SAID liqela lezifo ezibangelwa kukungasebenzi kakuhle okanye ingxaki yokulawula inkqubo yomzimba yethu yokuzikhusela . Kwimeko enjalo, ngaphandle kosulelo lwangaphandle (njengentsholongwane okanye iibhaktheriya), umzimba wethu udala ukudumba nje. Kungathi inkqubo yokuzikhusela yomzimba wethu iya ngokugqithisileyo.

Cinga nje, sineqela lamajoni ngaphakathi emizimbeni yethu (oko kukuthi, inkqubo yomzimba yokuzikhusela). Umsebenzi wabo kukulwa neentsholongwane ezibangela izifo xa zingena. Kodwa emzimbeni womntu one-SAIDs, la majoni ngamanye amaxesha ayaphazamiseka aze abangele iingxaki emzimbeni.

Ngoku usenokuba uzibuza ukuba oku kufana `(izifo ezizikhuselayo)` (oko kukuthi, izifo ezizikhuselayo). Umzekelo, `(i-rheumatoid arthritis)` okanye `(i-lupus)` ziyafana. Hayi, ezi zimbini zahlukile kancinci. Kwizifo ezizikhuselayo, okwenzekayo kukuba inkqubo yomzimba yethu yokuzikhusela efunyenweyo okanye eguquguqukayo ihlasela ngempazamo iiseli zayo eziphilileyo. Kodwa kwi-SAIDs, ingxaki ikunkqubo yomzimba yokuzikhusela engaphakathi.

Ii-SAID zinqabile kakhulu kunezifo ezizikhuselayo emzimbeni. Uninzi lwazo lufuzo , oko kuthetha ukuba zibangelwa kukuguquka kwezakhi zofuzo okanye uhlobo oluthile.

Ii-SAID zihlala ziqala ebuntwaneni okanye ebuntwaneni . Kodwa oko akusoloko kunjalo. Umntwana wakho usenokuba neziqendu okanye uhlaselo lomkhuhlane kunye nezinye iimpawu. Umntwana wakho usenokuba engenazo kwaphela iimpawu phakathi kohlaselo. Akukho nyango lwe-SAID. Nangona kunjalo, kukho unyango olunokunceda ukulawula iimpawu kwiimeko ezininzi.

Ziziphi iintlobo eziphambili ze-SAIDs?

Abaphandi ngoku bafumanise malunga neentlobo ezingama-60 ze-SAID, kwaye ezinye ziyafunyanwa. Makhe sijonge ezinye zezona zixhaphakileyo.

• I-Familial Mediterranean Fever (FMF): Le yeyona ixhaphakileyo kwizifo ezibangelwa lufuzo, eziphindaphindayo. Inokubangela ukudumba okubuhlungu kwesisu, isifuba kunye namalungu omntwana .
• Umkhuhlane odla ngokuvela rhoqo, i-aphthous-stomatitis, i-pharyngitis, i-adenitis (PFAPA): Oku kudla ngokuqala kubantwana abancinci ngaphambi kweminyaka emi-4 ubudala. Le meko inokuphela ngokuzenzekelayo emva kweminyaka eli-10 ubudala.
• I-Tumor necrosis factor receptor-associated periodic syndrome (i-TRAPS): Oku kunokwenzeka ebuntwaneni, ebusheni, kwaye ngamanye amaxesha nasebudaleni.
• Ukungabikho kwe-Mevalonate kinase (MKD): Ngaphambili yayibizwa ngokuba yi-hyper-IgD syndrome, oku kudla ngokuqala kwiintsana ezingaphantsi konyaka omnye ubudala.
• Izifo ezibangelwa kukudumba okubangelwa yi-NLRP3: Oku ngaphambili kwakubizwa ngokuba yi-cryopyrin-associated periodic syndromes (CAPS). Kukho ezinye iintlobo ezintathu ngaphakathi koku.
• Isifo sika-Adult-onset Still's (AOSD): Olu luhlobo lwe-systemic juvenile idiopathic arthritis (JIA) oluqala kubantu abadala.
• Isifo se-granulomatous esinxulumene ne-NOD-2 (i-Blau syndrome): Oku kudla ngokuqala ngaphambi kokuba kudlule iminyaka emi-4. Sichaphazela kakhulu ulusu lomntwana, amehlo kunye namalungu .

Zithini iimpawu ze-SAIDs? Sizibona njani ?

Iimpawu ze-SAID zihlala ziqala ebuntwaneni. Uphawu oluqhelekileyo nolubonakalayo yimfiva eyenzeka rhoqo okanye eyenzeka rhoqo . Abantu bahlala bengenazo iimpawu phakathi kokuhlaselwa. Nangona kunjalo, kunokubakho ezinye iimpawu ezithile kuhlobo ngalunye lwe-SAID.

Makhe sijonge iimpawu ezithile zeentlobo ezahlukeneyo ze-SAID ekuthethwe ngazo apha ngasentla:

• I-FMF: Oku kunokubangela iintlungu eziqatha esiswini somntwana, esifubeni, nasemalungeni, kunye nokudumba . Ngamanye amaxesha, irhashalala ingavela emilenzeni esezantsi okanye emaqatheni. Khawuthelekelele, umntwana ngequbuliso uba nesisu esibuhlungu kunye nomkhuhlane. Xa abazali bexhalabile baze bamse esibhedlele, banokucinga ukuba yi-appendicitis. Kodwa ke iyaphela ize ibuye emva kweentsuku ezimbalwa.
• I-PFAPA: Oku kunokubangela iimpawu ezifana nomphimbo obuhlungu, izilonda emlonyeni, kunye nokudumba kwama-lymph nodes entanyeni .
• IMIGXA: Oku kunokubangela ukubanda, iintlungu zemisipha esifubeni nasezingalweni , kunye nokudumba okubomvu okubuhlungu okuqala ezingalweni nasemilenzeni kusasazeka emzimbeni wonke.
• MKD: Nakule ntoUsenokuziva ungaphilanga kakuhle ngenxa yokubanda, intloko ebuhlungu, isisu esibuhlungu, ukungabi namdla wokutya, kunye neempawu ezifana nomkhuhlane.
• Izifo ze-NLRP3: Ezi zinokubangela iimpawu ezifana nokurhawuzelela kwesikhumba, iintloko ezibuhlungu, ukungondleki kakuhle, iintlungu zamalungu, kunye namehlo abomvu (conjunctivitis) .
• I-AOSD: Le yimeko edla ngokubangela ukurhawuzelela kwesikhumba, iintlungu zamalungu, kunye neentlungu zemisipha . Abanye abantu banokuba nomphimbo obuhlungu, iintlungu zesisu, ukudinwa kakhulu, kunye neentlungu zomzimba.
• I-Blau syndrome: Oku kunokubangela izilonda zesikhumba ezandleni, ezinyaweni, okanye emzimbeni womntwana wakho . Kunokubangela neentlungu zamalungu kunye nentlungu yamehlo .

Kutheni ezi SAID zenzeka? Zithini izizathu?

Uninzi lwee-SAID zizifo zemfuza . Oko kukuthi, isifo ngasinye sibangelwa kukwahluka (utshintsho) kwimfuza ethile.

Makhe sijonge iijini ezichaphazela iintlobo ezininzi eziphambili ze-SAID:

• I-FMF: Oku kubangelwa yijini ebizwa ngokuba yi-`(MEFV gene)`. Le jini iyalela indlela yokwenza iproteni ebizwa ngokuba yi-`(pyrin)`.
• I-PFAPA: Isizathu esichanekileyo soku asikafunyanwa .
• IMIGXA: Oku kubangelwa yijini ebizwa ngokuba yi-`(TNFRSF1A gene)`. Yile nto inika imiyalelo yokwenza iproteni ebizwa ngokuba yi-`(tumor necrosis factor receptor - TNFR)`.
• I-MKD: Isizathu soku yijini ebizwa ngokuba yi-`(MVK gene)`. Oku kufundisa ukuveliswa kweproteni ebizwa ngokuba yi-`(mevalonic kinase)`.
• Izifo ze-NLRP3: Oku kubangelwa yijini ebizwa ngokuba yi-`(NLRP3 gene)`. Oku kunika imiyalelo yokwenza iproteni ebizwa ngokuba yi-`(cryopyrin)`.
• I-AOSD: Isizathu esichanekileyo soku asikafunyanwa .
• I-Blau syndrome: Oku kubangelwa yijini ebizwa ngokuba yi-`(NOD2 gene)`. Oku kufundisa ukuveliswa kweproteni ebizwa ngokuba yi-`(NOD2)`.

Ngaba ezi SAIDs zingabangela ezinye iingxaki?

Ewe, oku kunokubangela iingxaki ukuba akunyangwa ngokufanelekileyo . Ukuba ukudumba kuyaqhubeka emzimbeni, kunokukhokelela kwimeko ebizwa ngokuba yi-"amyloidosis." Oko kukuthi, uhlobo oluthile lweproteni lufakwa kwizintso zethu. Oku kunokubangela umonakalo ongunaphakade kwizintso . Yiyo loo nto kubalulekile ukufumanisa isifo kwangethuba kwaye ufumane unyango.

Ugqirha uyixilonga njani le meko ye-SAIDs?

Enyanisweni, ukufumanisa ezi zifo ezibangela ukudumba komzimba kunokuba nzima kancinci , njengoko iimpawu zazo zinokufana nezinye iimeko ezinzima ezifana ne-lupus okanye umhlaza ofana ne-lymphoma. Ke ngoko, kubalulekile ukubona ugqirha oqeqeshwe ngokukodwa kwizifo ezibangela ukudumba, ingcali yamathambo, ukuze axilonge ngokufanelekileyo aze alawule imeko yomntwana wakho.

Ingcali yezifo zamathambo yomntwana wakho iza kusebenzisa izinto ezininzi ukuxilonga ii-SAID. Uza kukubuza ngeempawu zomntwana wakho kunye nokuba kukho nabani na kusapho lwakho onazo ezi meko (imbali yosapho lwebhayoloji). Ugqirha unokurhanela ukuba umntwana wakho unokuba ne-periodic fever syndrome ukuba:

• Ukuba umntwana usoloko enomkhuhlane .
• Ukuba kukho umntu kusapho lwakhe okhe wanefiva efana nale .
• Ukuba umntwana ungoweqela labantu abanengxaki yokuba nomkhuhlane ofana nalo .

Zeziphi iimvavanyo ezenziwayo koku?

Ukuqinisekisa ukuxilongwa, ingcali yezifo zamathambo yomntwana wakho inokucebisa iimvavanyo ezininzi. Ezi zingabandakanya:

• Uvavanyo lwelabhoratri: Uvavanyo olufana ne-"C-reactive protein (CRP)`` kunye ne-"complete blood count (CBC)`` lunokusetyenziswa ukujonga ukudumba emzimbeni. Olu vavanyo luba neziphumo ezilungileyo ngexesha "lokuhlaselwa" komkhuhlane kwaye lubuyela kwimeko yesiqhelo xa "ukuhlaselwa" kuphelile.
• Uvavanyo lomchamo: Uvavanyo lweproteni yomchamo lungenziwa ukujonga ukuba kukho iproteni egqithisileyo kumchamo. Kwi-MKD, uvavanyo lomchamo olujonga ii-organic acids (ii-asidi eziqulethe i-carbon atom enye) lunokubonisa amanqanaba aphezulu e-mevalonic acid.
• Uvavanyo lwemfuza: Oku kunokujonga iinguqulelo zemfuza. Nangona kunjalo, abanye abantwana abane-SAIDs basenokungafumani iinguqulelo zemfuza, nto leyo enokubangela iziphumo zovavanyo ezingezizo.

Ziziphi iindlela zonyango zezi SAIDs?

Unyango lwe-SAIDs luyahluka ngokuxhomekeke kuhlobo kunye nobunzima bemeko . Akukho nyango lwezi meko. Nangona kunjalo , iimpawu zomntwana wakho zihlala zilawulwa ngamayeza . Ukuba umntwana wakho uhlaselwa kambalwa ngonyaka, unokukwazi ukumnika amayeza okudambisa iintlungu afana namayeza angengo-steroidal anti-inflammatory (NSAIDs) ukunceda ukunciphisa iimpawu zakhe. Nangona kunjalo, ukuba imeko inzima ngakumbi, ugqirha wakho unokucebisa ezinye iindlela zonyango.

Nazi ezinye zazo:

• I-FMF: Iyeza elibizwa ngokuba yi-`(colchicine)` lingasetyenziswa ukulawula oku. Oku kunciphisa ukudumba. Ukuba umntwana akanakukwazi ukuthatha i-colchicine, uhlobo lweyeza `(biologic)` olubizwa ngokuba yi-`(canakinumab)` lunokusetyenziswa.
• I-PFAPA: Ii-steroids (ezidla ngokuba yi-prednisone) zinokunikwa ukunciphisa ixesha "lohlaselo" lwe-PFAPA. Kwabanye abantwana, i-cimetidine, iyeza elisetyenziselwa ukunyanga izilonda zesisu, inokunceda nokulawula iimpawu.
• IMIGXA: Iyeza elibizwa ngokuba yi-"canakinumab" lidla ngokuba luncedo koku. Kwakhona, iimpawu zinokuthomalaliswa ngamayeza okulwa nokuvuvukala amiselwe ngugqirha, anjenge-"glucocorticoids".
• I-MKD:I-Canakinumab ikwalunyango olusebenzayo lwe-MKD. Ukunika ii-NSAID okanye ii-steroids ngexesha "lohlaselo" nako kunokuba luncedo.
• Izifo ze-NLRP3: Ii-immunomodulators ezifana ne-canakinumab, i-rilonacept, kunye ne-anakinra zisetyenziswa ngempumelelo koku.
• I-AOSD: Iindidi ezahlukeneyo zamayeza okulwa nokuvuvukala zisetyenziselwa i-AOSD. Imizekelo ibandakanya ii-steroids, amayeza okulwa nokuvuvukala aguqula izifo (ii-DMARD), kunye ne-biologics.
• I-Blau syndrome: Ngokuxhomekeke kwiimpawu zomntwana wakho, angazama amayeza okunciphisa umzimba, amayeza okuthintela isifo se-tumor necrosis factor (TNF), kunye/okanye amayeza asetyenziswa emehlo.

Ingaba ezi meko ze-SAIDs zihlala zihleli? Okanye ziza kuphela?

Ezinye izifo ezibangelwa kukudumba komzimba zihlala ixesha elide . Ezinye zihlala iminyaka embalwa kwaye ziphele xa ukhula. Ezinye izifo zihlala ixesha elide, kodwa ngokuhamba kwexesha, ubuninzi kunye nobukhali bokuhlaselwa bunokwehla. Ugqirha womntwana wakho unokukunika ulwazi oluthile malunga nemeko yomntwana wakho.

Ukukhulisa umntwana onesifo esibangela ukudumba komzimba kunokuba nzima. Ukuba une-SAID, ungasebenzisa amava akho obuqu ukuxhasa ngcono ukhathalelo lomntwana wakho. Ungasebenzisa namava akho ukunceda umntwana wakho aqhelane nale meko yobomi bakhe bonke.

Eyona nto ibalulekileyo kukufuna unyango kwiingcali ezinamava ezinolwazi nge-SAIDs. Oogqirha abanjalo banokunceda ukulawula iimpawu zomntwana wakho kwaye babancede babe nobuntwana obungcono kangangoko.

Umyalezo Wokugqibela Wokuya Ekhaya

Ngoko ke, ndiyathemba ukuba unalo ulwazi malunga ne-SAIDs esithethe ngazo namhlanje. Khumbula, ukuba umntwana wakho unefiva engapheliyo, engachazwanga, ngokuqinisekileyo thetha nogqirha ngayo . Musa ukuyityeshela nje, ucinga ukuba iqhelekile.

• Ii-SAID liqela lezifo ezidla ngokubangela umkhuhlane kwaye zibangelwa yingxaki kwinkqubo yomzimba yokuzikhusela.
• Ezi zihlandlo zihlala zibangelwa lufuzo kwaye ziqala ebuntwaneni.
• Nangona kungekho nyango luhlala luhleli loku, kukho unyango olunokulawula iimpawu.
• Kubalulekile ukufuna ukuxilongwa ngokufanelekileyo kunye nonyango kugqirha okhethekileyo (Rheumatologist).
• Ukuba unyango luqalwe kwangethuba, iingxaki ezifana ne-amyloidosis zinokuthintelwa kwaye umntwana angaphila ubomi obuqhelekileyo.

Ukuba unemibuzo engaphezulu malunga noku, ungathandabuzi ukubuza ugqirha wosapho lwakho okanye ugqirha wabantwana.

Ii -SAID, Izifo Ezibangelwa Kukuvuvukala Kwemizimba, Umkhuhlane Oqhelekileyo, Izifo Zemfuza, Umkhuhlane Ebantwaneni, I-Immune System, I-Rheumatologist