Loaʻa pinepine ke kuni o kāu keiki? Hiki paha ma muli o nā SAID (Systemic Autoinflammatory Diseases)?

ʻAuē, loaʻa pinepine anei i kāu keiki liʻiliʻi ke kuni i kēia mau lā? Ke manaʻo ʻoe ua maikaʻi ʻoe, ke puka hou mai nei? I kekahi manawa ʻaʻole paha he maʻi maʻamau a bacteria paha ke kumu o kēia. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi paʻakikī iki, akā he mea nui loa ia e ʻike. Ua kapa ʻia kēia ʻo SAIDs, ʻo ia hoʻi ke kū nei no Systemic Autoinflammatory Diseases . I ka wā ma mua, ua kapa ʻia kēia ʻo `(Periodic Fever Syndromes)` a i ʻole `(Recurrent Fever Syndromes)`.

He aha nā SAID? E hoʻomaopopo maʻalahi kākou.

I ka ʻōlelo maʻalahi, ʻo nā SAID he hui o nā maʻi i hoʻokumu ʻia e kahi hana hewa a i ʻole ka pilikia hoʻoponopono i loko o ka ʻōnaehana pale kūlohelohe o ko mākou kino . ʻO ia ke kumu e loaʻa ai i kahi keiki ke kuni pinepine ʻoiai me ka ʻole o kahi maʻi (e like me ka maʻi virus a bacteria paha).

ʻO ka mea nui loa, ʻaʻole pono e huikau ʻia kēia mau kūlana i kapa ʻia ʻo SAID me nā `(Autoimmune Diseases)` (no ka laʻana `(Rheumatoid Arthritis)` a i ʻole `(Lupus)` āu i lohe ai. I nā maʻi `(Autoimmune)`, ma muli o kekahi hewa i loko o ka `(adaptive immune system)` o ko kākou kino, hoʻouka kēlā ʻōnaehana i kāna mau cell olakino ponoʻī. Akā i loko o nā SAID, hana ʻia kekahi mea ʻokoʻa.

ʻOi aku ka kakaikahi o nā SAID ma mua o nā maʻi autoimmune. He mau maʻi hoʻoilina pinepine lākou a hoʻokumu ʻia e kahi hoʻololi genetic .

ʻO ka maʻamau, akā ʻaʻole mau, hoʻomaka nā hōʻailona SAID i ka wā ʻōpiopio loa o ke keiki, ma ke ʻano he pēpē a he keiki liʻiliʻi paha . Loaʻa paha i kāu keiki nā "kau" o ke kuni a me nā hōʻailona ʻē aʻe me kēia maʻi. Ma waena o kēia mau wā, ʻaʻohe hōʻailona o kāu keiki. ʻOiai ʻaʻohe lāʻau lapaʻau no nā SAID, aia pinepine nā lāʻau lapaʻau e kōkua i ka kaohi ʻana i nā hōʻailona o kāu keiki.

He aha nā ʻano nui o nā SAID?

Ua ʻike nā kānaka noiʻi ma kahi o 60 mau ʻano SAID, a ke ʻike ʻia nei nā mea hou. Eia kekahi o nā ʻano SAID maʻamau:

• ʻO ka fiva Mediterranean Familial (FMF): ʻO kēia ka maʻamau o nā maʻi fiva i hoʻoilina ʻia ma ke ʻano genetic, recurrent fever syndromes. Hiki iā ia ke hoʻoulu i ka pehu ʻeha o ka ʻōpū, ka umauma, a me nā hono i nā keiki.
• Fiva kau, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Hoʻomaka pinepine kēia i nā keiki ʻōpio ma mua o 4 mau makahiki. I kekahi manawa, hiki i ke kūlana ke hoʻomaikaʻi iā ia iho ma hope o 10 mau makahiki.
• ʻO ka Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Hiki ke hana kēia i ka wā kamaliʻi, ʻōpio, a i ʻole ka wā makua.
• ʻO ka hemahema o ka kinase Mevalonate (MKD): Ua kapa ʻia ʻo "Hyper-IgD syndrome" ma mua, hoʻomaka kēia ma mua o ka piha ʻana o hoʻokahi makahiki o ke keiki.
• Nā maʻi autoinflammatory e pili ana iā NLRP3:Ua kapa ʻia kēia ma mua ʻo `(Cryopyrin-associated periodic syndromes - CAPS)`. Aia ʻekolu mau ʻano ʻē aʻe i loko o kēia.
• ʻO ka maʻi ʻo Adult-onset Still (AOSD): E like me ka inoa e hōʻike nei, he ʻano makua kēia o ke ʻano kamaliʻi "Systemic Juvenile Idiopathic Arthritis (JIA)".
• ʻO ka maʻi granulomatous e pili ana iā NOD-2 (Blau syndrome): Hoʻomaka pinepine kēia ma mua o ka piha ʻana o 4 makahiki. Hoʻopilikia nui ia i ka ʻili, nā maka, a me nā hono o ke keiki.

He aha nā hōʻailona o nā SAID?

Hoʻomaka pinepine nā hōʻailona o nā SAID i ka wā kamaliʻi. ʻO ka hōʻailona nui a maʻamau ka wela mau . I ka wā o ke kuni, hiki i nā kānaka ke olakino a ʻaʻohe hōʻailona. Eia nō naʻe, aia paha kekahi mau hōʻailona ʻē aʻe e pili pono ana i kēlā me kēia ʻano SAID. E nānā kākou i ke ʻano o lākou:

• FMF: Hiki i kēia ke hoʻoulu i ka pehu a me ka ʻeha nui i loko o ka ʻōpū o ke keiki, ka umauma, a me nā hono . Hiki paha ke loaʻa kahi ʻulaʻula ma nā wāwae haʻahaʻa a i ʻole nā ​​kuʻekuʻe wāwae.
• PFAPA: Hiki i kēia ke hana i ka ʻāʻī ʻeha, nā ʻeha waha, a me nā ʻōpū lymph pehu ma ka ʻāʻī . No ka laʻana, inā hōʻike kāu keiki i nā hōʻailona e like me ka tonsillitis pinepine a me nā ʻeha waha, he PFAPA paha ia.
• NĀ PAHU: Hiki i kēia ke hoʻoulu i nā ʻūlū a me ka ʻeha ʻiʻo ma ke kino/kino a me nā lima . Hiki iā ia ke hana i kahi ʻulaʻula ʻeha e hoʻomaka ana ma nā lima a me nā wāwae a laha i lalo o ke kino.
• MKD: Hiki i kēia ke hana i nā hōʻailona e like me ke anu, ka ʻeha poʻo, ka ʻeha ʻōpū, ka nalowale o ka makemake, a me nā hōʻailona like me ka maʻi flu .
• Nā maʻi NLRP3: Hiki i kēia kūlana ke hana i nā ʻulaʻula ʻili, nā poʻo ʻeha, ka malaise, ka ʻeha hui, a me ka maka ʻulaʻula/conjunctivitis .
• AOSD: He kūlana maʻamau kēia e hoʻoulu ai i nā ʻulaʻula ʻili, ʻeha hui, a me ka ʻeha ʻiʻo . Hiki i kekahi poʻe ke ʻeha ka ʻāʻī, ka ʻeha ʻōpū, ka luhi, a me ka malaise.
• ʻO ka maʻi Blau: Hiki i kēia ke hana i nā ʻeha ʻili ma nā lima, nā wāwae, a i ʻole ka ʻōpū o kāu pēpē . Hiki iā ia ke hana i ka ʻeha o nā hono a me ka ʻeha o nā maka .

He aha nā kumu o nā SAID?

He nui nā SAID he mau maʻi genetic . ʻO ia hoʻi, ʻo kahi mutation kikoʻī (variant) i loko o kahi gene kikoʻī ke kumu o kēlā me kēia syndrome. E noʻonoʻo he hoʻonohonoho o nā kuhikuhi (genes) ko ko mākou kino e haʻi mai iā mākou pehea e hana ai i kēlā me kēia hana, no laila inā hewa kekahi leka ma kēlā puke, hiki iā ia ke hana i ke koena o ka hana e hewa.

Eia nā ʻano nui o SAIDs a me nā genes e pili ana me lākou:

• FMF:ʻO ke gene `MEFV` ka mea e hāʻawi i nā ʻōlelo kuhikuhi no ka hana ʻana i ka protein `(pyrin)`.
• PFAPA: ʻAʻole i hoʻoholo ʻia ke kumu kikoʻī o kēia.
• Nā Pahele: ʻO ka gene `TNFRSF1A`. Kuhikuhi kēia i ka hana ʻana o kahi protein i kapa ʻia ʻo `(tumor necrosis factor receptor - TNFR)`.
• MDK: ʻO ka gene i kapa ʻia ʻo `MVK`. Kuhikuhi kēia i ka hana ʻana o kahi protein i kapa ʻia ʻo `(mevalonic kinase)`.
• Nā maʻi NLRP3: ʻO ka gene `NLRP3`. Kuhikuhi kēia i ka hana ʻana o kahi protein i kapa ʻia ʻo `(cryopyrin)`.
• AOSD: ʻAʻole i loaʻa ke kumu kikoʻī no kēia.
• ʻO ka maʻi Blau: ʻO ka gene `NOD2`. Kuhikuhi kēia i ka hana ʻana o kahi protein i kapa ʻia ʻo `(NOD2)`.

He mea nui e hoʻomaopopo he hewa ʻole ia o nā mākua ke kumu o kēia mau mea i nā kumu hoʻoilina. He mau kaʻina hana olaola paʻakikī loa kēia.

Nā pilikia i hoʻokumu ʻia e nā SAID

He mea nui loa ka loaʻa ʻana o ka lāʻau kūpono no kēia mau kūlana. No ka mea, inā hoʻomau ka mumū i loko o ke kino, hiki iā ia ke alakaʻi i kahi kūlana i kapa ʻia ʻo `(Amyloidosis). ʻO `(Amyloidosis)` kahi waihona protein i loko o nā puʻupaʻa . Hiki i kēia ke hōʻeha mau loa i nā puʻupaʻa . No laila, inā he mau hōʻailona, ​​​​he mea nui e ʻimi koke i ke kōkua lapaʻau.

Pehea e ʻike ʻia ai nā SAID?

I ka ʻoiaʻiʻo, hiki ke paʻakikī iki ka ʻike ʻana i nā SAID no ka mea hiki ke like nā hōʻailona me nā maʻi koʻikoʻi ʻē aʻe, e like me ka lupus a i ʻole ka lymphoma. No laila, he mea nui e ʻike i ke kauka i loaʻa ka hoʻomaʻamaʻa kūikawā i nā maʻi mumū (he rheumatologist) e ʻike pololei a mālama i ke kūlana o kāu keiki.

E hoʻohana ke kauka rheumatologist o kāu keiki i kekahi mau mea e ʻike ai i nā SAID. E nīnau ʻo ia e pili ana i nā hōʻailona o kāu keiki a inā paha ua loaʻa i kekahi o kou ʻohana kēia mau kūlana (mōʻaukala ʻohana biological). Manaʻo paha ke kauka he Periodic Fever Syndrome kāu keiki inā:

• Inā loaʻa iā ʻoe nā kuni pinepine
• Inā he moʻolelo o nā kuni manawa like me kēia kekahi o ka ʻohana
• Inā ʻoe no kahi hui o ka poʻe i maʻalahi i kēlā mau kuni

He aha nā hoʻokolohua i hoʻohana ʻia?

Hiki i kāu kauka ke paipai i kekahi mau hoʻokolohua e hōʻoia i ka hōʻailona. Hiki i kēia mau mea ke komo pū me:

• Nā hoʻāʻo lab: Hiki i nā hoʻāʻo e like me `(C-reactive protein - CRP)` a me `(Complete Blood Count - CBC)` ke nānā no ka mumū i loko o ke kino. He `(maikaʻi)` kēia mau hoʻāʻo ke loaʻa ke kuni, a hoʻi i nā waiwai maʻamau ke pau ke kuni.
• Hoʻāʻo mimi:Hiki ke hana ʻia kahi hoʻāʻo protein mimi e nānā i nā pae kiʻekiʻe o ka protein i loko o ka mimi. I ka MKD, e hōʻike kahi hoʻāʻo mimi no nā waikawa organik i nā pae kiʻekiʻe o ka waikawa mevalonic.
• Hoʻāʻo ʻana i ka hoʻoilina: Hiki i kēia ke nānā i nā ʻano hoʻoilina. Eia nō naʻe, ʻaʻole paha i loaʻa i kekahi mau keiki me nā SAID ke ʻano hoʻoilina. No laila, ʻoiai inā he "maikaʻi ʻole" nā hopena o ka hoʻāʻo, ʻaʻole hiki ke kāpae ʻia ka hiki ke loaʻa iā SAID.

Pehea e mālama ʻia ai nā SAID?

ʻOkoʻa ka mālama ʻana no nā SAID ma muli o ke ʻano a me ke koʻikoʻi o ka maʻi . ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia kēia mau maʻi, hiki ke hoʻomalu maikaʻi ʻia nā hōʻailona o ke keiki me ka hoʻohana ʻana i nā lāʻau lapaʻau . No ka laʻana, inā loaʻa i kāu keiki kēia mau anu i kekahi mau manawa i ka makahiki, hiki i nā lāʻau ʻeha a me nā lāʻau anti-inflammatory nonsteroidal (NSAID) ke hoʻomalu i nā hōʻailona. Eia nō naʻe, inā ʻoi aku ka koʻikoʻi o ka maʻi, hiki i ke kauka ke kuhikuhi i nā koho lapaʻau ʻē aʻe.

Eia kekahi mau lāʻau lapaʻau:

• FMF: Hiki ke mālama ʻia kēia me kahi lāʻau i kapa ʻia ʻo `(Colchicine)` e hōʻemi i ka mumū. Inā ʻaʻole hiki iā ʻoe ke lawe i ka `(Colchicine)`, hiki iā ʻoe ke hoʻāʻo i kahi ʻano lāʻau `(Biologic)` i kapa ʻia ʻo `(Canakinumab)`.
• PFAPA: Hiki ke hoʻopōkole ʻia ka piʻi ʻana o ka PFAPA ma ka hāʻawi ʻana i nā steroids (pinepine prednisone). Ua hiki i kekahi mau keiki ke kāohi i ko lākou mau hōʻailona ma ka hoʻohana ʻana i ka cimetidine, kahi lāʻau lapaʻau i hoʻohana ʻia e mālama i nā ʻōpū ʻōpū.
• TRAPS: He kūpono loa ka lāʻau lapaʻau `(Canakinumab)` no nā TRAPS. Eia kekahi, hiki i nā lāʻau lapaʻau anti-inflammatory i kuhikuhi ʻia e like me `(Glucocorticoids)` ke kōkua i ka hōʻoluʻolu ʻana i nā hōʻailona.
• MKD: He koho maikaʻi ʻo `(Canakinumab)` no ka mālama ʻana iā MKD. Hiki ke kōkua pū ka hoʻohana ʻana i nā `(NSAIDs)` a i ʻole `(Steroids)` i ka wā kuni.
• Nā maʻi NLRP3: Hoʻohana pono ʻia nā lāʻau ʻē aʻe "Immunomodulators" e like me "Canakinumab" a me "Rilonacept" a me "Anakinra" e mālama i nā maʻi NLRP3.
• AOSD: Hoʻohana ʻia nā ʻano lāʻau anti-inflammatory like ʻole no AOSD. ʻO kēia mau mea ʻo `(Steroids)`, `(Disease-Modifying Anti-Rheumatic Drugs - DMARDs)`, a me `(Biologics)`.
• ʻO ka maʻi Blau: Ma muli o nā hōʻailona o ke keiki, hiki ke hoʻāʻo ʻia nā immunosuppressants, nā mea hoʻopaʻa i ka tumor necrosis factor (TNF), a me/a i ʻole nā ​​​​lāʻau lapaʻau maka topical.

E hoʻomaikaʻi anei ke kūlana o nā SAIDs iā ia iho?

Hiki i kekahi mau SAID ke mau no ke ola holoʻokoʻa . Hiki i kekahi ke mau no kekahi mau makahiki a e hoʻomaikaʻi me ka ʻelemakule. ʻOiai kekahi mau maʻi e ola holoʻokoʻa, hiki ke emi ke alapine o ke kuni i ka hala ʻana o ka manawa a hiki ke emi iki ka koʻikoʻi o nā hōʻailona . Hiki i kāu kauka ke hāʻawi iā ʻoe i nā kikoʻī kikoʻī e pili ana i ke kūlana kikoʻī o kāu keiki.

Hiki ke paʻakikī ka mālama ʻana i kahi keiki me SAID. Malia paha ua SAID ʻoe iho, akā hiki iā ʻoe ke hoʻohana i kāu mau ʻike pilikino e kōkua i kāu keiki e mālama a kōkua iā lākou e kū i kēia kūlana no ke ola holoʻokoʻa.

ʻO ka mea nui loa, ʻo ia ka ʻimi ʻana i ka lāʻau lapaʻau mai nā loea i ʻike pono i nā ʻano paʻakikī o nā SAID. Hiki iā lākou ke kōkua i ka hoʻokele ʻana i nā hōʻailona o kāu keiki a kōkua iā lākou e loaʻa i ka wā kamaliʻi maikaʻi loa.

Nā mea nui e hoʻomanaʻo (Leka Lawe-Home)

ʻAe, e hōʻuluʻulu kākou i kekahi o nā mea e pono ai ʻoe e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai.

• Inā loaʻa i kāu keiki nā kuni pinepine a wehewehe ʻole ʻia , e kamaʻilio me ke kauka e pili ana i ia mea. He hōʻailona paha ia o SAID.
• He pilikia nā SAID i ka ʻōnaehana pale kūlohelohe , ʻaʻole ia he maʻi lele maʻamau.
• He ʻokoʻa kēia mai nā maʻi "Autoimmune" .
• ʻO ka hapa nui o ka manawa, ua hoʻokumu ʻia kēia mau mea e nā kumu genetic .
• He mea nui e ʻike i kahi rheumatologist no ka hoʻoholo pololei ʻana i ka maʻi.
• Hiki i ka lāʻau lapaʻau ke hoʻomalu maikaʻi i nā hōʻailona .
• He mea nui ka mālama mua ʻana e pale aku i ka hōʻino ʻana o nā puʻupaʻa.

Inā he mau nīnau hou aku kāu e pili ana i kēia, mai makaʻu e nīnau i kāu kauka ʻohana a kauka keiki paha. E hiki iā lākou ke kōkua hou aku iā ʻoe.

Nā SAID, kuni hoʻoilina, kuni pinepine, kuni i nā keiki, ʻōnaehana pale, nā maʻi genetic, kauka rheumatologist