Loaʻa pinepine anei i kāu keiki ke kuni me ke kumu ʻole? E aʻo kākou e pili ana i kēia mau SAID (Systemic Autoinflammatory Diseases)!

Loaʻa i kāu keiki liʻiliʻi ke kuni i kekahi manawa me ka ʻole o ke anu a i ʻole kekahi maʻi ʻē aʻe? Hiki mai ke kuni, emi iho i loko o kekahi mau lā, a laila hoʻi hou mai i kekahi mau lā ma hope? Ke kū nei kekahi mau mākua i kēlā mau ʻike. No laila i kēia lā e kamaʻilio mākou e pili ana i kahi kumu pinepine a paʻakikī ke loaʻa o ke kuni. Ua kapa ʻia kēia ʻo SAIDs (Systemic Autoinflammatory Diseases) . I ka wā ma mua, ua kapa ʻia kēia ʻo `(Periodic Fever Syndromes)` a i ʻole `(Recurrent Fever Syndromes)`.

He aha kēia mau 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 . I kēia hihia, me ka ʻole o kekahi maʻi o waho (e like me ka maʻi virus a bacteria paha), hoʻokumu wale ko mākou kino i ka mumū. Me he mea lā e hoʻonui nui ana ka ʻōnaehana pale o ko mākou kino.

E noʻonoʻo e pili ana i ia mea, aia iā mākou kahi hui o nā koa i loko o ko mākou kino (ʻo ia ka ʻōnaehana pale). ʻO kā lākou hana ke kaua aku i nā germs e hoʻoulu ai i ka maʻi ke komo mai lākou. Akā i loko o ke kino o kekahi me SAIDs, hoʻonāukiuki wale kēia mau koa i kekahi manawa a hoʻoulu i nā pilikia i loko o ke kino.

I kēia manawa, ke noʻonoʻo nei paha ʻoe inā he mea like kēia me `(nā maʻi autoimmune)` (ʻo ia hoʻi, nā maʻi autoimmune). No ka laʻana, ua like like `(rheumatoid arthritis)` a i ʻole `(lupus)`. ʻAʻole, he ʻokoʻa iki kēia mau mea ʻelua. I nā maʻi autoimmune, ʻo ka mea e hana ʻia, ʻo ia ka hoʻouka hewa ʻana o ka ʻōnaehana pale i loaʻa a i ʻole adaptive o ko mākou kino i kāna mau cell olakino ponoʻī. Akā i nā SAID, aia ka pilikia me ka ʻōnaehana pale kūlohelohe.

ʻOi aku ka kakaikahi o nā SAID ma mua o nā maʻi autoimmune. ʻO ka hapa nui o lākou he hoʻoilina , ʻo ia hoʻi, ua hoʻokumu ʻia lākou e kahi mutation genetic a i ʻole variant.

Hoʻomaka pinepine nā SAID i ka wā kamaliʻi a i ʻole ka wā kamaliʻi . Akā, ʻaʻole ia ka hihia mau. Loaʻa paha i kāu keiki nā hanana a i ʻole nā ​​hoʻouka ʻana o ke kuni a me nā hōʻailona ʻē aʻe. ʻAʻohe hōʻailona o kāu keiki ma waena o nā hoʻouka ʻana. ʻAʻohe lāʻau lapaʻau no nā SAID. Eia nō naʻe, aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona i ka hapa nui o nā hihia.

He aha nā ʻano nui o nā SAID?

Ua ʻike ʻia e nā kānaka noiʻi ma kahi o 60 mau ʻano SAID, a ke ʻike ʻia nei nā mea hou aku. E nānā kākou i kekahi o nā ʻano maʻamau.

• ʻO ka Familial Mediterranean Fever (FMF): ʻO kēia ka maʻamau o nā maʻi kuni i hoʻoilina ʻia ma ke ʻano genetic, a me ka hoʻi hou ʻana. Hiki iā ia ke hoʻoulu i ka mumū ʻeha o ka ʻōpū o ke keiki, ka umauma, a me nā hono .
• Fiva kau, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Hoʻomaka pinepine kēia i nā keiki ʻōpio ma mua o 4 mau makahiki. Hiki ke emi koke kēia ʻano ma hope o 10 mau makahiki.
• ʻO ka Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Hiki ke hana kēia i ka wā kamaliʻi, ka wā ʻōpio, a i kekahi manawa ʻoiai ke kanaka makua.
• ʻO ka hemahema o ka kinase Mevalonate (MKD): Ua kapa ʻia ʻo hyper-IgD syndrome ma mua, hoʻomaka kēia i nā pēpē ma lalo o 1 makahiki.
• Nā maʻi autoinflammatory e pili ana iā NLRP3: Ua kapa ʻia kēia ma mua he cryopyrin-associated periodic syndromes (CAPS). Aia ʻekolu mau ʻano ʻē aʻe i loko o kēia.
• ʻO ka maʻi ʻo Adult-onset Still (AOSD): He ʻano makua kēia o ka 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? Pehea mākou e ʻike ai iā lākou?

Hoʻomaka pinepine nā hōʻailona o nā SAID i ka wā kamaliʻi. ʻO ka hōʻailona maʻamau a koʻikoʻi loa, ʻo ia ke kuni manawa a i ʻole episodic . ʻAʻohe hōʻailona o ka poʻe ma waena o nā hoʻouka ʻana. Eia nō naʻe, aia paha kekahi mau hōʻailona ʻē aʻe e pili ana i kēlā me kēia ʻano SAID.

E nānā kākou i nā hōʻailona kikoʻī o kekahi mau ʻano SAID i kūkākūkā ʻia ma luna:

• FMF: Hiki i kēia ke hōʻeha nui i ka ʻōpū o ke keiki, ka umauma, a me nā hono, me ka mumū . I kekahi manawa, hiki ke ulu kahi ʻulaʻula ma nā wāwae haʻahaʻa a i ʻole nā ​​kuʻekuʻe wāwae. E noʻonoʻo, loaʻa koke i kahi keiki ka ʻeha ʻōpū a me ke kuni. Ke hopohopo nā mākua a lawe iā lākou i ka haukapila, manaʻo paha lākou he appendicitis. Akā a laila emi iho a hoʻi mai ma hope o kekahi mau lā.
• PFAPA: Hiki i kēia ke hana i nā hōʻailona e like me ka ʻeha ʻāʻī, nā ʻeha waha, a me nā kīʻaha lymph pehu i ka ʻāʻī .
• NĀ PAHU: Hiki i kēia ke hoʻoulu i ke anu, ka ʻeha ʻiʻo ma ka umauma a me nā lima , a me kahi ʻulaʻula ʻeha e hoʻomaka ana ma nā lima a me nā wāwae a laha ma ke kino holoʻokoʻa.
• MKD: Ma kēia pū kekahiHiki paha iā ʻoe ke maʻi me ke anu, ka ʻeha poʻo, ka ʻeha ʻōpū, ka nalowale o ka makemake e ʻai, a me nā hōʻailona like me ka maʻi flū.
• Nā maʻi NLRP3: Hiki i kēia mau mea ke hana i nā hōʻailona e like me ka ʻili ʻili, ka ʻeha poʻo, ka malaise, ka ʻeha hui, a me nā maka ʻulaʻula (conjunctivitis) .
• AOSD: He ʻano 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 nui, a me nā ʻeha kino.
• ʻO ka maʻi Blau: Hiki i kēia ke hana i nā ʻeha ʻili ma nā lima, nā wāwae, a i ʻole ke kino o kāu pēpē . Hiki iā ia ke hana i ka ʻeha o nā hono a me ka ʻeha o nā maka .

No ke aha i loaʻa ai kēia mau SAID? He aha nā kumu?

ʻO ka hapa nui o nā SAID he mau maʻi hoʻoilina . ʻO ia hoʻi, ua hoʻokumu ʻia kēlā me kēia maʻi e kahi ʻano like ʻole (variant) i loko o kahi gene kikoʻī.

E nānā kākou i nā genes e hoʻohuli i kekahi mau ʻano nui o SAID:

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

Hiki i kēia mau SAID ke hoʻoulu i nā pilikia ʻē aʻe?

ʻAe, hiki i kēia ke hoʻopilikia inā ʻaʻole mālama pono ʻia . Inā hoʻomau ka mumū i loko o ke kino, hiki iā ia ke alakaʻi i kahi maʻi i kapa ʻia ʻo "amyloidosis." ʻO ia hoʻi, ua waiho ʻia kekahi ʻano protein i loko o ko kākou mau puʻupaʻa. Hiki i kēia ke hōʻeha mau loa i nā puʻupaʻa . ʻO ia ke kumu he mea nui e ʻike mua i ka maʻi a loaʻa ka lāʻau lapaʻau.

Pehea e ʻike ai ke kauka i kēia maʻi SAIDs?

ʻO kaʻoiaʻiʻo, hiki ke paʻakikī iki ka ʻike ʻana i kēia mau maʻi autoinflammatory, no ka mea hiki i kā lākou mau hōʻailona ke hoʻohālikelike i nā kūlana koʻikoʻi ʻē aʻe e like me ka lupus a i ʻole nā ​​maʻi ʻaʻai e like me 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 pono a mālama i ke kūlana o kāu keiki.

E hoʻohana ke kauka rheumatologist o kāu keiki i kekahi mau kumu e ʻike ai i nā SAID. E nīnau ʻo ia iā ʻoe e pili ana i nā hōʻailona o kāu keiki a inā paha he mau kūlana kēia i loko o kou ʻohana (mōʻaukala ʻohana biological). Manaʻo paha ke kauka he periodic fever syndrome paha kāu keiki inā:

• Inā loaʻa i ke keiki ke kuni pinepine .
• Inā he moʻolelo o nā kuni pinepine e like me kēia kekahi o ka ʻohana .
• Inā no kahi hui o ka poʻe i maʻalahi i nā kuni manawa e like me kēia ke keiki .

He aha nā hoʻokolohua e hana ʻia nei no kēia?

No ka hōʻoia ʻana i ka maʻi, hiki i ke kauka rheumatologist o kāu keiki ke ʻōlelo i kekahi mau hoʻokolohua. Hiki i kēia mau mea ke komo pū me:

• Nā hoʻāʻo lab: Hiki ke hoʻohana ʻia nā hoʻāʻo e like me ka "C-reactive protein (CRP)" a me ka "helu koko piha (CBC)" e nānā i ka mumū i loko o ke kino. He maikaʻi kēia mau hoʻāʻo i ka wā o ka "hoʻouka" ʻana o ke kuni a hoʻi i ka maʻamau ke pau ka "hoʻouka".
• Hoʻāʻo mimi: Hiki ke hana ʻia kahi hoʻāʻo protein mimi e nānā i ka nui o ka protein i loko o ka mimi. I ka MKD, hiki i kahi hoʻāʻo mimi e nānā ana i nā waikawa organik (nā waikawa i loaʻa hoʻokahi ʻātoma kalapona) ke hōʻike i nā pae kiʻekiʻe o ka waikawa mevalonic.
• Hoʻāʻo ʻana i ka genetics: Hiki i kēia ke nānā i nā ʻano genetic. Eia nō naʻe, ʻaʻole paha i loaʻa i kekahi mau keiki me nā SAID nā ʻano genetic, hiki ke hopena i nā hopena hoʻāʻo maikaʻi ʻole.

He aha nā lāʻau lapaʻau no kēia mau SAID?

ʻOkoʻa ka mālama ʻana no nā SAID ma muli o ke ʻano a me ke koʻikoʻi o ke ʻano . ʻAʻohe lāʻau lapaʻau no kēia mau ʻano. Eia nō naʻe , hiki ke hoʻomalu ʻia nā hōʻailona o kāu keiki me ka lāʻau lapaʻau . Inā loaʻa i kāu keiki kekahi mau hoʻouka kaua i ka makahiki, hiki paha iā ʻoe ke hāʻawi iā lākou i nā lāʻau hōʻeha e like me nā lāʻau anti-inflammatory nonsteroidal (NSAID) e kōkua i ka hōʻemi ʻana i kā lākou mau hōʻailona. Eia nō naʻe, inā ʻoi aku ka koʻikoʻi o ke ʻano, hiki i kāu kauka ke kuhikuhi i nā koho lapaʻau ʻē aʻe.

Eia kekahi o lākou:

• FMF: Hiki ke hoʻohana ʻia kahi lāʻau lapaʻau i kapa ʻia ʻo `(colchicine)` e kāohi i kēia. Hoʻemi kēia i ka mumū. Inā ʻaʻole hiki i ke keiki ke lawe i ka colchicine, hiki ke hoʻāʻo ʻia kahi ʻano lāʻau lapaʻau `(biologic)` i kapa ʻia ʻo `(canakinumab)`.
• PFAPA: Hiki ke hāʻawi ʻia nā steroids (ʻo ka prednisone maʻamau) e hoʻopōkole i ka lōʻihi o kahi "hoʻouka" PFAPA. I kekahi mau keiki, ʻo cimetidine, kahi lāʻau lapaʻau i hoʻohana ʻia e mālama i nā ʻōpū ʻōpū, hiki ke kōkua i ka kaohi ʻana i nā hōʻailona.
• NĀ PAHU: He lāʻau lapaʻau i kapa ʻia ʻo `(canakinumab)` ka mea e pono ai no kēia. Eia kekahi, hiki ke hoʻomaha ʻia nā hōʻailona me nā lāʻau anti-inflammatory i kuhikuhi ʻia e ke kauka, e like me `(glucocorticoids)`.
• MKD:He lāʻau lapaʻau kūpono nō hoʻi ʻo Canakinumab no MKD. Hiki ke kōkua ka hāʻawi ʻana i nā NSAID a i ʻole nā ​​steroid i ka wā o kahi "hoʻouka".
• Nā maʻi NLRP3: Hoʻohana pono ʻia nā immunomodulators e like me canakinumab, rilonacept, a me anakinra no kēia.
• AOSD: Hoʻohana ʻia nā ʻano lāʻau anti-inflammatory like ʻole no AOSD. ʻO nā laʻana e komo pū me nā steroids, nā lāʻau anti-rheumatic hoʻololi maʻi (DMARDs), a me nā biologics.
• ʻO ka maʻi Blau: Ma muli o nā hōʻailona o kāu keiki, hiki iā lākou ke ho'āʻo i nā immunosuppressants, nā mea hoʻopaʻa i ka tumor necrosis factor (TNF), a me / a i ʻole nā ​​​​lāʻau lapaʻau maka topical.

He mau loa anei kēia mau kūlana SAID? A i ʻole e nalo aku lākou?

ʻO kekahi mau maʻi autoinflammatory he mau maʻi no ke ola holoʻokoʻa . Hiki i kekahi ke mau no kekahi mau makahiki a nalowale me ka ʻelemakule. ʻO kekahi mau maʻi no ke ola holoʻokoʻa, akā i ka hala ʻana o ka manawa, hiki ke emi ka pinepine a me ke koʻikoʻi o nā hoʻouka ʻana. Hiki i ke kauka o kāu keiki ke hāʻawi iā ʻoe i ka ʻike kikoʻī e pili ana i ke kūlana o kāu keiki.

Hiki ke paʻakikī ka hānai ʻana i kahi keiki me ka maʻi autoinflammatory. Inā loaʻa iā ʻoe ka SAID, hiki iā ʻoe ke hoʻohana i kāu mau ʻike pilikino e kākoʻo maikaʻi i ka mālama ʻana i kāu keiki. Hiki iā ʻoe ke hoʻohana i kāu mau ʻike e kōkua i kāu keiki e hoʻomaʻamaʻa i kēia kūlana no ke ola holoʻokoʻa.

ʻO ka mea nui loa, ʻo ia ka ʻimi ʻana i ka lāʻau lapaʻau mai nā loea akamai i ʻike e pili ana i nā SAID. Hiki i kēlā mau kauka ke kōkua i ka mālama ʻana i nā hōʻailona o kāu keiki a kōkua iā lākou e loaʻa i ka wā kamaliʻi maikaʻi loa.

Leka Hoʻihoʻi Hope Loa

No laila, ke lana nei koʻu manaʻo ua loaʻa iā ʻoe kekahi manaʻo e pili ana i nā SAID a mākou i kamaʻilio ai i kēia lā. E hoʻomanaʻo, inā loaʻa pinepine nā kuni o kāu keiki me ka wehewehe ʻole ʻia, e kamaʻilio pono me ke kauka e pili ana i ia mea . Mai hoʻowahāwahā wale iā ia, me ka manaʻo he mea maʻamau ia.

• ʻO nā SAID kahi hui o nā maʻi e hoʻoulu pinepine ai i ke kuni a ua hoʻokumu ʻia e kahi pilikia me ka ʻōnaehana pale.
• He pinepine kēia mau mea he genetic a hoʻomaka i ka wā ʻōpiopio.
• ʻOiai ʻaʻohe lāʻau lapaʻau mau loa no kēia, aia nā lāʻau lapaʻau e hiki ke kaohi i nā hōʻailona.
• He mea nui e ʻimi i ka hōʻoia kūpono a me ka mālama ʻana mai ke kauka loea (Rheumatologist).
• Inā hoʻomaka koke ʻia ka lāʻau lapaʻau, hiki ke pale ʻia nā pilikia e like me ka amyloidosis a hiki i ke keiki ke ola i kahi ola maʻamau.

Inā he mau nīnau hou aku kāu e pili ana i kēia, mai kānalua e nīnau i kāu kauka ʻohana a kauka pediatric paha.

Nā SAID, Nā Maʻi ʻŌnaehana Autoinflammatory, Nā Fiva Pinepine, Nā Maʻi Hoʻoilina, Nā Fiva i nā Keiki, ʻŌnaehana Palekana, Kauka Lāʻau Lapaʻau