He maha ngā wā ka pāngia tō tamaiti e te kirika? Tērā pea nā te mate pūnaha-mumura (SAID)?

Āe, he maha ngā wā ka pāngia tō tamaiti e te kirika i ēnei rā? Ina whakaaro koe kua pai ake koe, ka puta anō? I ētahi wā, ehara pea i te huaketo, i te huakita rānei te take o tēnei. I tēnei rā ka kōrero tātou mō tētahi āhuatanga uaua, engari he mea nui kia mōhiotia. Ko te ingoa o tēnei ko ngā SAID, arā, ko ngā mate pūnaha-whakapūmau-ā-roto . I mua, i huaina tēnei ko ngā `(Periodic Fever Syndromes)`, ko ngā `(Recurrent Fever Syndromes)` rānei.

He aha ngā SAID? Kia mārama tātou.

He whakarāpopototanga noa iho, ko ngā SAID he rōpū mate e puta mai ana i te hapa, i te raruraru whakahaere rānei i roto i te pūnaha ārai mate ā-roto o tō tātou tinana . Koinei te take ka pāngia te tamaiti e te kirika maha ahakoa kāore he mate (pēnei i te huaketo, i te huakita rānei).

Ko te mea nui, kaua ēnei āhuatanga e kiia nei ko ngā SAID e whakapoauautia ki ngā `(Ngā Mate Pūnaha Ārai Mate)` (hei tauira, `(Rheumatoid Arthritis)` `(Lupus)` rānei kua rongo pea koe. I roto i ngā mate `(Autoimmune)`, nā te hapa o te `(pūnaha ārai mate urutau)` o tō tātou tinana, ka whakaekea e taua pūnaha ōna ake pūtau hauora. Engari i roto i ngā SAID, he rerekē te āhua o te mate.

He tino onge ake ngā SAID i ngā mate pūnaha ārai mate. He maha ngā wā ka puta mai i te tuku iho, ā, ka puta mai i te whakarerekētanga ira .

I te nuinga o te wā, engari kāore i te katoa, ka tīmata ngā tohumate SAID i te wā e tamariki ana te tamaiti, ahakoa he pēpi, he tamaiti nohinohi rānei . Tērā pea ka pāngia tō tamaiti e te kirika me ētahi atu tohumate me tēnei mate. I waenganui i ēnei wā, tērā pea kāore he tohumate o tō tamaiti. Ahakoa kāore he rongoā mō ngā SAID, he maha ngā maimoatanga hei āwhina i te whakahaere i ngā tohumate o tō tamaiti.

He aha ngā momo matua o ngā SAID?

E 60 pea ngā momo SAID kua kitea e ngā kairangahau, ā, kei te kitea tonutia ētahi atu. Anei ētahi o ngā momo SAID e tino kitea ana:

• Te kirika whānau o te Moana-nui-a-Kiwa (FMF): Koinei te mate kirika tuku iho, e tino kitea ana i roto i ngā ira. Ka pupuhi mamae pea te puku, te uma, me ngā hononga i roto i ngā tamariki.
• Te kirika ā-wā, te aphthous-stomatitis, te pharyngitis, te adenitis (PFAPA): Ka tīmata tēnei i roto i ngā tamariki nohinohi i mua i te 4 tau te pakeke. I ētahi wā, ka pai ake te āhua i muri i te 10 tau te pakeke.
• Te mate pūmau e pā ana ki te pūtau mate pukupuku (TRAPS): Ka pā pea tēnei ki te tamarikitanga, ki te taiohitanga, ki te pakeketanga rānei.
• Te ngoikore o te Mevalonate kinase (MKD): I mua ko te "Hyper-IgD syndrome" te ingoa, ā, ka tīmata tēnei i mua i te kotahi tau te pakeke o te tamaiti.
• Ngā mate mumura-aunoa e pā ana ki te NLRP3:I mua ko te ingoa o tēnei ko `(Cryopyrin-associated periodic syndromes - CAPS)`. E toru atu ngā momo āhua o tēnei.
• Te mate a Still e tīmata ana i te pakeke (AOSD): E ai ki te ingoa, he momo mate tamarikitanga tēnei e tīmata ana i te pakeke, arā, "Systemic Juvenile Idiopathic Arthritis (JIA)".
• Te mate granulomatous e pā ana ki te NOD-2 (Blau syndrome): Ka tīmata tēnei i mua i te 4 tau te pakeke. Ko te kiri, ngā kanohi, me ngā hononga o te tamaiti te nuinga e pā ana.

He aha ngā tohu o ngā SAID?

Ko ngā tohu o ngā SAID ka tīmata i te tamarikitanga. Ko te tohu matua, ko te tohu tino kitea, ko te kirika auau . I ngā wā kirika, he maha ngā wā ka ora te tangata, ā, kāore he tohu. Heoi anō, tera pea ētahi atu tohu e motuhake ana ki ia momo SAID. Kia tirohia tātou he aha ēnei:

• FMF: Ka pupuhi, ka mamae nui hoki te puku, te uma, me ngā hononga o te tamaiti i tēnei. Tērā pea ka puta he ponana ki ngā waewae o raro, ki ngā pona rānei.
• PFAPA: Ka pā mai pea he mamae o te korokoro, he mate waha, me te pupuhi o ngā kōpū lymph i te kaki . Hei tauira, ki te whakaatu tāu tamaiti i ngā tohu pēnei i te tonsillitis auau me ngā mate waha, tera pea he PFAPA.
• NGĀ MAHANGA: Ka pā mai te makariri me te mamae o ngā uaua i te tinana/tinana me ngā ringa . Ka pā mai hoki he ponana whero mamae e tīmata ana i ngā ringa me ngā waewae ka horapa ki te tinana.
• MKD: Ka puta pea ngā tohu pēnei i te makariri, te ānini, te mamae o te puku, te kore hiahia kai, me ngā tohu rite ki te rewharewha .
• Ngā mate NLRP3: Ka pā mai pea ngā ponana kiri, ngā ānini, te ngoikore, te mamae o ngā hononga, me te kanohi māwhero/te mate pūkahukahu .
• AOSD: He āhuatanga tēnei e tohuhia ana e te ponana kiri, te mamae hononga, me te mamae uaua . Tērā pea ka pāngia ētahi tāngata e te mamae o te korokoro, te mamae o te puku, te ngenge, me te koretake o te hauora.
• Te mate Blau: Ka pā mai pea he whara kiri ki ngā ringa, ki ngā waewae, ki te puku rānei o tō pēpi . Ka pā mai hoki he mamae hononga me te mamae kanohi .

He aha ngā pūtake o ngā SAID?

He maha ngā SAID he mate ira . Arā, he whakarerekētanga motuhake (rerekētanga) i roto i tētahi ira motuhake te take o ia mate. Whakaarohia he huinga tohutohu (ira) kei roto i tō tātou tinana e whakaatu ana me pēhea te mahi i ia mahi, nō reira, ki te hē tētahi reta i roto i taua pukapuka, ka hē pea te toenga o te mahi.

Anei ngā momo matua o ngā SAID me ngā ira e pā ana ki a rātou:

• FMF:Ko te ira `MEFV` te mea e hoatu ana i ngā tohutohu mō te hanga i te pūmua `(pyrin)`.
• PFAPA: Kāore anō kia whakatauhia te tino take o tēnei.
• NGĀ MAHANGA: Ko te ira `TNFRSF1A`. Mā tēnei ka ārahi te hanga o tētahi pūmua e kiia nei ko te `(tumor necrosis factor receptor - TNFR)`.
• MDK: Ko te ira e kiia nei ko `MVK`. Mā tēnei ka tohutohu i te hanga o te pūmua e kiia nei ko `(mevalonic kinase)`.
• Ngā mate NLRP3: Ko te ira `NLRP3`. Mā tēnei ka ārahi te hanga o tētahi pūmua e kiia nei ko `(cryopyrin)`.
• AOSD: Kāore anō kia kitea te tino take mō tēnei.
• Te mate Blau: Ko te ira `NOD2`. Mā tēnei ka ārahi te hanga o tētahi pūmua e kiia nei ko `(NOD2)`.

He mea nui kia mārama, mēnā he take ira te take o ēnei, ehara i te he o ngā mātua. He tukanga koiora tino uaua ēnei.

Ngā raruraru i puta mai i ngā SAID

He mea tino nui kia whiwhi maimoatanga tika mō ēnei āhuatanga. Nā te mea, ki te haere tonu te mumura i roto i te tinana, ka arahi pea ki tētahi āhuatanga e kiia nei ko te `(Amyloidosis). Ko te `(Amyloidosis)` he putunga pūmua i roto i ngā whatukuhu . Ka taea e tēnei te whakararu tuturu i ngā whatukuhu . Nō reira, ki te puta ngā tohu, he mea nui kia rapu tohutohu hauora tonu.

Me pēhea te tautuhi i ngā SAID?

Ki te pono, he uaua pea te tautuhi i ngā SAID nā te mea he rite tonu ngā tohu ki ērā o ētahi atu mate kino, pērā i te lupus, te lymphoma rānei. Nō reira, he mea nui kia kite i tētahi tākuta he tohunga motuhake ki ngā mate mumura (he tākuta rumatiki) hei tautuhi tika i te mate o tāu tamaiti.

Ka whakamahia e te tākuta rumatiki o tāu tamaiti ētahi mea hei tātaritanga i ngā SAID. Ka pātai ia mō ngā tohu o tāu tamaiti, mehemea rānei kua pāngia tētahi o tō whānau e ēnei āhuatanga (hītori whānau koiora). Tērā pea ka whakapae te tākuta kei te pāngia tāu tamaiti e te Periodic Fever Syndrome mēnā:

• Mena he maha ōu kirika
• Mena he hītori o te kirika auau pēnei i tētahi o te whānau
• Mena kei roto koe i tētahi rōpū tāngata e pāngia ana e ēnei momo kirika

He aha ngā whakamātautau e whakamahia ana?

Tērā pea ka tūtohu tō tākuta i ētahi whakamātautau hei whakaū i te mate. Kei roto i ēnei ko:

• Ngā whakamātautau taiwhanga: Mā ngā whakamātautau pēnei i te `(C-reactive protein - CRP)` me te `(Complete Blood Count - CBC)` ka taea te tirotiro mēnā he mumura kei roto i te tinana. He `(pai)` ēnei whakamātautau ina pāngia te kirika, ā, ka hoki anō ki ngā uara noa ina mutu te kirika.
• Whakamātautau mimi:Ka taea te whakahaere i tētahi whakamātautau pūmua mimi hei tirotiro mēnā he nui ngā taumata pūmua i roto i te mimi. I roto i te MKD, ka whakaatuhia e te whakamātautau mimi mō ngā waikawa waro he nui ngā taumata o te waikawa mevalonic.
• Whakamātautau ira: Ka taea e tēnei te tirotiro mō ngā rerekētanga ira. Heoi, ko ētahi tamariki he SAID kāore pea i kitea te rerekētanga ira. Nō reira, ahakoa he "kino" ngā hua o te whakamātautau, kāore e taea te whakakore i te tūponotanga o te SAID.

Me pēhea te rongoā mō ngā SAID?

He rerekē te maimoatanga mō ngā SAID i runga i te momo me te kaha o te mate . Ahakoa kāore e taea te whakaora katoa i ēnei mate, ka taea te whakahaere pai i ngā tohu o te tamaiti mā te whakamahi i ngā rongoā . Hei tauira, ki te pāngia tō tamaiti e ēnei makariri i ētahi wā noa iho i te tau, ka taea e ngā rongoā whakamamae mamae me ngā rongoā anti-inflammatory kore-steroidal (NSAID) te whakahaere i ngā tohu. Heoi, ki te kaha ake te mate, ka tūtohu pea te tākuta i ētahi atu kōwhiringa maimoatanga.

Anei ētahi maimoatanga pēnei:

• FMF: Ka taea te rongoā i tēnei mā te rongoā e kīia nei ko `(Colchicine)` hei whakaiti i te mumura. Mēnā kāore e taea e koe te tango i te `(Colchicine)`, ka taea e koe te whakamātau i tētahi momo rongoā `(Biologic)` e kīia nei ko `(Canakinumab)`.
• PFAPA: Ka taea te whakapoto i ngā putanga mai o te PFAPA mā te hoatu i ngā steroids (he maha ngā wā ko te prednisone). Kua taea e ētahi tamariki te whakahaere i ō rātou tohu mā te whakamahi i te cimetidine, he rongoā e whakamahia ana hei rongoā i ngā mate puku.
• TRAPS: He tino whai hua te rongoā `(Canakinumab)` mō ngā TRAPS. Waihoki, ka taea e ngā rongoā ārai-mumura pēnei i te `(Glucocorticoids)` te āwhina ki te whakamamae i ngā tohu.
• MKD: He kōwhiringa pai te `(Canakinumab)` mō te rongoā MKD. Tērā pea he āwhina te whakamahi i ngā `(NSAID)` me ngā `(Steroids)` i te wā e kirika ana.
• Ngā mate NLRP3: Ko ētahi atu rongoā "Immunomodulators" pērā i te "Canakinumab" me te "Rilonacept" me te "Anakinra" e whakamahia angitu ana hei rongoā i ngā mate NLRP3.
• AOSD: He maha ngā momo rongoā ārai-mumura e whakamahia ana mō te AOSD. Kei roto i ēnei ko ngā `(Steroids)`, `(Disease-Modifying Anti-Rheumatic Drugs - DMARDs)`, me `(Biologics)`.
• Te mate Blau: I runga i ngā tohu o te tamaiti, ka taea te whakamātau i ngā rongoā ārai mate, ngā aukati i te tumor necrosis factor (TNF), me/ranei ngā rongoā kanohi o waho.

Ka pai ake te āhua o ngā SAID i a ia anō?

Ka roa pea ētahi SAID mō te wā katoa . Ko ētahi ka roa pea mō ētahi tau, ā, ka pai ake i te kaumātuatanga. Ahakoa he roa tonu ētahi āhuatanga mō te wā katoa, ka heke pea te auau o te kirika i roto i te wā, ā, ka iti haere ngā tohu . Ka taea e tō tākuta te hoatu ki a koe ngā taipitopito motuhake mō te āhua motuhake o tō tamaiti.

He uaua te tiaki i tētahi tamaiti me te SAID. Tērā pea kua pāngia koe e te SAID, engari ka taea e koe te whakamahi i ō wheako ake hei āwhina i tāu tamaiti ki te tiaki me te āwhina i a ia ki te aro atu ki tēnei mate mō tōna oranga katoa.

Ko te mea nui ko te rapu maimoatanga mai i ngā tohunga e mōhio ana ki ngā āhuatanga uaua o ngā SAID. Ka taea e rātou te āwhina i te whakahaere i ngā tohu o tāu tamaiti, me te āwhina i a ia kia pai rawa atu tōna tamarikitanga.

Ngā mea tino nui hei maumahara (Karere hei kawe ki te kāinga)

Āe, me whakarāpopoto tātou i ētahi o ngā mea hei maumahara māu mai i ngā mea kua kōrerotia e tātou.

• Mena he maha ngā wā ka pāngia tō tamaiti e te kirika, kāore he whakamārama , kōrero ki te tākuta mōna. He tohu pea tēnei nō te SAID.
• He raruraru ngā SAID nō te pūnaha ārai mate ā-roto , ehara i te mate pipiri noa.
• He rerekē ēnei i ngā mate "Autoimmune" .
• I te nuinga o te wā, ka puta ēnei i ngā āhuatanga ira .
• He mea nui kia toro atu ki tētahi tohunga mate rumatiki hei whakatau tika i te mate.
• Ka taea e te maimoatanga te whakahaere pai i ngā tohumate .
• He mea nui te maimoatanga wawe hei ārai i te kino o te whatukuhu.

Mena he pātai anō āu mō tēnei, kaua e wehi ki te pātai atu ki tō tākuta whānau, ki tō tākuta tamariki rānei. Ka taea e rātou te āwhina atu i a koe.

` Ngā SAID, te kirika tuku iho, te kirika pinepine, te kirika i roto i ngā tamariki, te pūnaha ārai mate, ngā mate ira, te tākuta rumatiki