Loaʻa pinepine anei i kāu pēpē nā kuni i wehewehe ʻole ʻia? Hiki paha kēia ma muli o nā SAID (Systemic Autoinflammatory Diseases)!

Ke loaʻa mau nei ke kuni i kāu keiki liʻiliʻi? I kekahi manawa loaʻa iā lākou ke kuni hoʻokahi a ʻelua paha i ka mahina, akā ʻōlelo nā kauka ʻaʻohe maʻi, ʻo ia hoʻi, ʻaʻohe virus a bacteria paha i loko o ke kino. I kēlā mau manawa, ma ke ʻano he makuahine a makua paha, he mea kūpono loa ʻoe e manaʻo i ka makaʻu a me ka hopohopo. "No ke aha kēia e hana nei i kaʻu keiki?" Ua noʻonoʻo paha ʻoe i hoʻokahi tausani manawa. Malia paha ʻo ke kumu o kēia he mea āu i lohe ʻole ai ma mua. I kēia lā, e kamaʻilio mākou e pili ana i nā SAID (Systemic Autoinflammatory Diseases), he mea paʻakikī ke hoʻomaopopo a hiki ke hoʻoulu i nā kuni pinepine. I ka wā ma mua, ua kapa ʻia kēia mau mea ʻo `(Periodic Fever Syndromes)` a i ʻole `(Recurrent Fever Syndromes)`.

He aha kēia mau SAID (Nā maʻi Autoinflammatory Systemic)?

I ka ʻōlelo maʻalahi, ʻo nā SAID he hui o nā maʻi e kū mai ana ma muli o kahi hana hewa a i ʻole ka pilikia kaohi i loko o ka ʻōnaehana pale kūlohelohe o ko mākou kino. ʻO ka mea e hana ʻia, me ke kumu ʻole o waho, ʻo ia hoʻi, he virus a bacteria paha, hoʻokumu wale ke kino i ka mumū. ʻO ia ke kumu e hiki mai ai kēia kuni pinepine.

I kēia manawa, ke noʻonoʻo nei paha ʻoe, "ʻAuē, ʻo ia kekahi o kēlā mau maʻi Autoimmune?" ʻAʻole, he ʻokoʻa iki kēia mau mea ʻelua. I loko o nā maʻi Autoimmune, no ka laʻana, i nā maʻi e like me ka Rheumatoid Arthritis a i ʻole Lupus, hoʻouka hewa ka ʻōnaehana pale kino o ko mākou kino i kāna mau hunaola olakino ponoʻī. Akā i loko o 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 ka manawa, he mau maʻi hoʻoilina kēia, ʻo ia hoʻi, ua hoʻokumu ʻia e kahi mutation genetic a i ʻole variant.

Hoʻomaka pinepine kēia mau SAID (akā ʻaʻole i nā manawa a pau) i ka wā he pēpē a he kamaliʻi paha kāu keiki . E loaʻa i ke keiki nā hanana a i ʻole nā ​​hoʻouka ʻana o ka maʻi i kekahi mau manawa. I loko o kēia mau hanana, hiki ke ʻike ʻia ke kuni a me nā hōʻailona ʻē aʻe. Eia nō naʻe, ma waena o kēia mau "hoʻouka ʻana," ʻaʻohe hōʻailona o ke keiki. ʻAʻohe lāʻau lapaʻau no nā SAID, akā aia pinepine nā lāʻau lapaʻau kūpono e kāohi i nā hōʻailona.

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 aku. Eia kekahi o nā ʻano maʻamau:

• ʻO ke kuni Mediterranean Familial (FMF): ʻO kēia ka maʻamau o nā maʻi kuni i hoʻoholo ʻia e ka genetics, recurrent. Hiki iā ia ke hoʻoulu i ka pehu ʻeha i loko o ka ʻōpū o ke keiki, ka umauma, a me nā hono.
• ʻO ke kuni kau, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Hoʻomaka pinepine kēia i nā keiki ʻōpio, ma mua o ka makahiki 4. Eia nō naʻe, i kekahi mau keiki, e nalowale paha kēia ʻano ma hope o ka makahiki 10.
• ʻO ka Tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS): Hiki ke hana kēia mai ka wā kamaliʻi a hiki i ka wā ʻōpio, a i kekahi manawa a hiki i ka wā makua.
• ʻO ka hemahema o ka kinase Mevalonate (MKD): Ma mua i ʻike ʻia ʻo Hyper-IgD syndrome, 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 Still i hoʻomaka i ka pākeke (AOSD): E like me ka inoa e hōʻike nei, hoʻomaka kēia maʻi i ka wā makua. ʻO ia ke ʻano makua o kahi maʻi i kapa ʻia ʻo 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 manawa a i ʻole episodic . E like me ka mea i ʻōlelo ʻia ma mua, hiki i ke keiki ke maikaʻi i nā wā i ʻole ai ke kuni. Eia nō naʻe, aia kekahi mau hōʻailona ʻē aʻe e pili pono ana i kēlā me kēia ʻano SAID:

• I loko o FMF: Hiki i ka ʻōpū, ka umauma, a me nā hono o ke keiki ke lilo i mea ʻeha loa a pehu. I kekahi manawa, aia paha kahi ʻulaʻula ʻili ma nā wāwae haʻahaʻa a i ʻole nā ​​kuʻekuʻe wāwae.
• I loko o PFAPA: ʻEha ka ʻāʻī, nā ʻeha waha, nā ʻōpuʻu lymph pehu ma ka ʻāʻī. ʻO kēia nā hōʻailona nui.
• I loko o nā TRAPS: Hiki i ke kino ke anu a me ke kuni. Hiki i ke keiki ke loaʻa ka ʻeha o nā ʻiʻo ma ke kino a me nā lima. Hiki ke ʻike ʻia kahi ʻulaʻula ʻeha, e laha paha mai nā lima a i nā wāwae a laila i ke kino.
• I ka MKD: Hiki iā ʻoe ke ʻike i nā hōʻailona like me ka maʻi flū e like me ke anu, ka ʻeha poʻo, ka ʻeha ʻōpū, ka nalowale o ka makemake e ʻai, a me nā hōʻailona ʻē aʻe.
• I nā maʻi NLRP3: hiki ke loaʻa nā ʻulaʻula o ka ʻili, nā poʻo hina, ka malaise, ka ʻeha o nā hono, a me ka mumū o nā maka (conjunctivitis).
• I loko o AOSD: nā ʻōhū ʻili, ʻeha hui, ʻeha ʻiʻo. Hiki i kekahi poʻe ke ʻeha ka ʻāʻī, ʻeha ʻōpū, luhi, a me ka manaʻo nāwaliwali.
• I ka maʻi Blau: Hiki i ka pēpē ke loaʻa nā ʻeha ʻili ma nā lima, nā wāwae, a i ʻole ka ʻōpū. Hiki iā lākou ke ʻike i ka ʻeha o nā hono a me ka ʻeha o nā maka.

E noʻonoʻo, ua loaʻa i ke kaikamahine liʻiliʻi a Nalini kahi kuni kiʻekiʻe no ʻekolu a ʻehā mau lā i kēlā me kēia mahina no nā mahina i hala iho nei. Me kēlā, ke ulu nei hoʻi ʻo ia i nā ʻeha liʻiliʻi ma kona waha a me nā puʻupuʻu ma kona ʻāʻī. I kona hōʻike ʻana i ke kauka, ua ʻōlelo ʻo ia he PFAPA paha.

He aha nā kumu o nā SAID?

ʻO ka hapa nui o nā SAID he mau maʻi hoʻoilina . ʻO ke ʻano kēia, ua hoʻokumu ʻia kēlā me kēia o kēia mau syndromes e kahi ʻano like ʻole o kā mākou mau genes. Eia nā ʻano maʻamau o nā SAID a me nā genes e hoʻopilikia iā lākou:

• FMF: ʻO ke gene i kapa ʻia ʻo `(MEFV)`. Kuhikuhi kēia gene i ka hana ʻana o ka protein `(pyrin)`.
• PFAPA: ʻAʻole i ʻike ʻia ke kumu kikoʻī e hoʻopilikia nei i kēia.
• Nā Pahele: ʻO ka gene i kapa ʻia ʻo `(TNFRSF1A)`. Maanei mai nā kuhikuhi no ka hana ʻana i ka protein `(tumor necrosis factor receptor - TNFR)`.
• MKD: ʻO ke gene `(MVK)`. Kuhikuhi kēia i ka hana ʻana o kahi protein i kapa ʻia ʻo `(mevalonic kinase)`.
• Nā maʻi NLRP3: ʻO ka gene i kapa ʻia ʻo `(NLRP3)`. Hāʻawi kēia i nā kuhikuhi no ka hana ʻana i kahi protein i kapa ʻia ʻo `(cryopyrin)`.
• AOSD: ʻAʻole maopopo ke kumu kikoʻī o kēia.
• ʻO ka maʻi Blau: ʻO ka gene `(NOD2)`. Kuhikuhi kēia i ka hana ʻana o ka protein `(NOD2)`.

ʻIke ʻia paha he mea paʻakikī iki nā kikoʻī o kēia mau genes iā ʻoe. I ka ʻōlelo maʻalahi, hoʻopilikia kēia mau genes i ka hana ʻana o kekahi mau protein e kāohi ana i ka mumū i loko o ko mākou kino. ʻO ia ke kumu e hoʻokumu ai ke kino i nā kūlana mumū me ke kumu ʻole.

He aha ka mea e hiki ke hana inā ʻaʻole mālama pono ʻia nā SAID?

He mea nui loa ka loaʻa ʻana o ka lāʻau kūpono no kēia mau SAID. No ka mea, inā hoʻomau ka mumū i loko o ke kino, hiki ke loaʻa kahi maʻi i kapa ʻia ʻo `(amyloidosis)`. ʻO ia hoʻi, ua waiho ʻia kekahi ʻano protein i loko o ko kākou mau puʻupaʻa, hiki ke hōʻeha mau i nā puʻupaʻa . No laila, inā he mau hōʻailona, ​​he mea nui loa ia e ʻimi i ka ʻōlelo aʻo lapaʻau me ka nānā ʻole ʻana iā lākou.

Pehea e ʻike ai nā kauka i nā SAID?

Hiki ke paʻakikī 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 , ʻoi aku ka maikaʻi o ka ʻike ʻana i kahi rheumatologist, he kauka i loaʻa ka hoʻomaʻamaʻa kūikawā i nā maʻi mumū , e ʻike pololei a mālama i ke kūlana o kāu keiki.

E nānā ke kauka rheumatologist o kāu keiki i kekahi mau mea 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 moʻolelo o ke anu pinepine kekahi o kou ʻohana. Hiki i ke kauka ke kānalua i kēia kūlana inā:

• Inā loaʻa i ke keiki ke kuni pinepine .
• Inā he moʻolelo o ke kuni pinepine kekahi o ka ʻohana .
• Inā no kahi hui lāhui ke keiki, ʻoi aku ka nui o ka loaʻa ʻana o ia mau kuni pinepine.

He aha nā hoʻokolohua i hoʻohana ʻia?

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

• Nā hoʻāʻo keʻena hoʻokolohua: Hiki i nā hoʻāʻo koko 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 iā ʻoe ke kuni a hoʻi i ka maʻamau ke emi ke kuni.
• Hoʻāʻo mimi: Nānā ʻia ka mimi no nā pae kiʻekiʻe o ka protein. I nā hihia o MKD, nānā ʻia hoʻi ka mimi no nā pae kiʻekiʻe o kahi waikawa organik i kapa ʻia ʻo "mevalonic acid".
• Hoʻāʻo ʻana i ka hoʻoilina: Hiki i ka hoʻāʻo ʻana i ka hoʻoilina ke nānā i nā ʻano hoʻoilina i ʻōlelo ʻia ma luna. Eia nō naʻe, ʻaʻole paha i loaʻa i kekahi mau keiki me nā SAID nā ʻano hoʻoilina, no laila he maikaʻi ʻole paha nā hopena hoʻāʻo.

He aha nā lāʻau lapaʻau no nā SAID?

ʻO ka mālama ʻana no nā SAID e pili ana i ke ʻano a me ke koʻikoʻi o ke kūlana . ʻAʻohe lāʻau lapaʻau no kēia mau kūlana, akā hiki i nā lāʻau lapaʻau ke kōkua i ka kaohi ʻana i nā hōʻailona o kāu keiki . Inā loaʻa i kāu keiki kekahi mau hoʻouka kaua i ka makahiki, hiki iā ʻoe ke hoʻokele i nā hōʻailona me nā lāʻau ʻeha a me nā lāʻau anti-inflammatory nonsteroidal (NSAID). Eia nō naʻe, inā ʻoi aku ka koʻikoʻi o ke kūlana, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau ʻē aʻe:

• FMF: Hiki ke mālama ʻia kēia me kahi lāʻau i kapa ʻia ʻo `(Colchicine)` e hōʻemi i ka mumū. Inā ʻaʻole hiki ke hāʻawi ʻia ʻo `(Colchicine)` i ke keiki, hiki iā ʻoe ke hoʻāʻo i kahi ʻano lāʻau `(biologic)` i kapa ʻia ʻo `(Canakinumab)`.
• PFAPA: Hiki i nā steroid e like me ka prednisone ke hoʻopōkole i ka lōʻihi o kahi "hoʻouka" PFAPA. ʻO Cimetidine, kahi lāʻau lapaʻau i hāʻawi ʻia i kekahi mau keiki no nā ʻōpū ʻōpū, hiki ke kōkua i ka hōʻoluʻolu ʻana i nā hōʻailona.
• TRAPS: He kūpono loa ka lāʻau `(Canakinumab)` no nā TRAPS. Eia kekahi, hiki ke hoʻomalu ʻia nā hōʻailona me nā lāʻau anti-inflammatory e like me `(glucocorticoids)` i kauoha ʻia e ke kauka.
• MKD: He lāʻau lapaʻau kūpono nō hoʻi ʻo `(Canakinumab)` no MKD. Hiki i nā `(NSAID)` a i ʻole `(steroids)` ke kōkua i ka wā o kahi "hoʻouka".
• Nā maʻi NLRP3: Ua hoʻohana pono ʻia nā Immunomodulators e like me Canakinumab, Rilonacept, a me Anakinra e mālama i nā maʻi e pili ana i ka NLRP3.
• AOSD: Hoʻohana ʻia nā lāʻau lapaʻau anti-inflammatory like ʻole e like me nā steroids, nā lāʻau anti-rheumatic hoʻololi maʻi (DMARDs), a me nā biologics e mālama ai iā AOSD.
• 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.

ʻO ka heluhelu ʻana e pili ana i kēia mau lāʻau lapaʻau e hoʻomāʻewaʻewa iki paha iā ʻoe. Akā e hoʻomanaʻo, ua hāʻawi ʻia kēia mau lāʻau lapaʻau a pau ma lalo o ka nānā pono ʻana a ke kauka, ma ke ʻano kūpono loa i ke keiki. No laila, he mea nui e hahai pono i ka lāʻau lapaʻau e like me ka ʻōlelo a ke kauka.

Ke nalowale nei ke kūlana o SAIDs?

He mau maʻi ola kekahi mau SAID. Hiki i kekahi ke mau no kekahi mau makahiki a nalowale me ka ʻelemakule . He mau maʻi ola kekahi, akā i ka hala ʻana o ka manawa, e emi paha ke alapine o nā hoʻouka ʻana a e emi paha ka koʻikoʻi o nā hōʻailona. E wehewehe kāu kauka i nā kikoʻī o ke kūlana o kāu keiki iā ʻoe.

Hiki ke paʻakikī ka mālama ʻana i kahi keiki me SAID. Inā ua loaʻa iā ʻoe iho ka SAID, hiki iā ʻoe ke hoʻohana i kou ʻike e kōkua i ka mālama ʻana i kāu keiki. He mea nui nō hoʻi kou hoʻomaopopo ʻana i ke kōkua ʻana i kāu keiki e ola me kēia maʻi no ke ola holoʻokoʻa.

ʻO ka mea nui loa, ʻo ia ka ʻimi ʻana i ka lāʻau lapaʻau mai nā kauka akamai i ʻike e pili ana i nā SAID. Hiki iā lākou ke kōkua i ka mālama 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 kumu nui e pono ai ʻoe e hoʻomanaʻo mai ka mea a mākou i kamaʻilio ai:

• Inā loaʻa i kāu keiki nā kuni pinepine me ka wehewehe ʻole ʻia , he hōʻailona paha ia o ka SAID.
• ʻAʻole i hoʻokumu ʻia nā SAID e kahi maʻi , akā, he pilikia ia me ka ʻōnaehana pale ponoʻī o ke kino.
• He mau maʻi laha ʻole kēia i hoʻokumu pinepine ʻia e nā kumu hoʻoilina .
• Hiki ke ʻokoʻa nā hōʻailona ma muli o ke ʻano o SAID, akā ʻo ke kuni pinepine ka hōʻailona nui .
• He mea nui e ʻike i kahi rheumatologist no ka maʻi kūpono a me ka mālama ʻana.
• ʻOiai ʻaʻole hiki i ka lāʻau lapaʻau ke ho'ōla piha i ka maʻi, hiki ke kaohi maikaʻi ʻia nā hōʻailona .
• Mai hoʻowahāwahā i nā hōʻailona, ​​​​​​no ka mea, hiki iā lākou ke hōʻino i nā puʻupaʻa inā ʻaʻole mālama ʻia.

Inā he mau nīnau hou aku kāu e pili ana i kēia, mai kānalua e kamaʻilio me kāu kauka ʻohana a i ʻole he kauka rheumatologist. E hiki iā lākou ke kōkua hou aku iā ʻoe.

Nā SAID, nā maʻi kuni manawa, kuni hou, nā maʻi autoinflammatory, kuni, kuni i nā keiki, nā maʻi genetic