ʻO ka maʻi maʻi mākua

ʻO ka maʻi maʻi mau (ASD) kahi maʻi maʻamau i loaʻa i nā fiva kiʻekiʻe, nā ʻūlū, a me nā ʻeha pū. Hiki iā ia ke alakaʻi i ka maʻi lōlō wā lōʻihi (mau).

ʻO ka maʻi maʻi mākia kahi ʻano nui o nā ʻōpio idiopathic ʻōpio (JIA), i nā keiki. Hiki i nā mākua ke loaʻa i ke ʻano like, ʻoiai ʻoi aku ka liʻiliʻi o ka maʻamau. Kāhea ʻia ia he maʻi makua ke hoʻomaka (AOSD).

Ma mua o 1 o 100,000 mau kānaka e hoʻomohala ASD i kēlā me kēia makahiki. Hoʻopili pinepine ia i nā wahine ma mua o nā kāne.

ʻO ke kumu o ka maʻi maʻi Maopopo ʻole. ʻAʻole ʻike ʻia nā kumu pilikia no ka maʻi.

ʻAneʻane loaʻa nā kānaka a pau i ka maʻi i ke kuni, ka ʻeha pū, ka ʻāʻī ʻeha, a me ka hū.

• ʻO ka ʻeha pū, ka mehana, a me ka pehu ka mea maʻamau. ʻO ka pinepine, pili kekahi mau hono i ka manawa like. ʻO ka pinepine, ʻo ka poʻe me ke ʻano loaʻa ka ʻāʻī o ke kakahiaka i nā hono i kū mau no kekahi mau hola.
• Hele koke ke kuni i hoʻokahi manawa i kēlā me kēia lā, ʻo ka mea maʻamau i ka ʻauinalā a i ke ahiahi.
• ʻO ka wīwī o ka ʻili he salmon-a pink ʻāpana a hele mai a hele pū me ke kuni.

ʻO nā hōʻailona hou aʻe:

• ʻEha o ka ʻōpū a me ka pehu
• ʻEha ke lawe i kahi hanu hohonu (pleurisy)
• Eha ka 'ā'ī
• Pehu nā lymph nodes (kelena)
• Kaumaha emi

Pehu paha ka ʻōpala a me ke akepaʻa paha. Lū a me ka puʻuwai puʻuwai e kū pū kekahi.

Hiki ke ʻike wale ʻia ʻo AOSD ma hope o ka hōʻole ʻia o nā maʻi ʻē aʻe (e like me nā maʻi a me nā maʻi ʻaʻai. Pono paha ʻoe i nā hoʻokolohua olakino he nui ma mua o ka hoʻoholo hope loa ʻia.

Hōʻike kahi hoʻokolohua kino i ke kuni, ka pehu, a me ka maʻi kōpaʻa. E hoʻohana ka mea mālama ola i ka stethoscope e hoʻolohe ai i nā loli i ke kani o kou puʻuwai a i ʻole nā ​​māmā.

Hiki ke kōkua i nā hoʻokolohua koko aʻe i ka ʻike ʻana i nā maʻi maʻi mau.

• Helu piha ke koko (CBC), hiki ke hōʻike i kahi helu kiʻekiʻe o nā hunaola keʻokeʻo (granulosit) a hoʻemi ʻia ka helu o nā hunaola ʻulaʻula.
• ʻO ka protein C-reactive (CRP), kahi ana o ka mumuna, e ʻoi aku ka kiʻekiʻe ma mua o ka maʻamau.
• ESR (sedimentation rate), kahi ana o ka pehu, e ʻoi aku ma mua o ka maʻamau.
• E kiʻekiʻe loa ka pae Ferritin.
• E kiʻekiʻe ka pae fibrinogen.
• E hōʻike nā hoʻokolohua hana ate i nā kiʻekiʻe kiʻekiʻe o AST a me ALT.
• ʻO Rheumatoid factor a me ANA hōʻike maikaʻi ʻole.
• E maikaʻi ʻole nā ​​moʻomeheu koko a me nā noiʻi viral.

Pono ʻia nā hoʻokolohua ʻē aʻe e nānā no ka mumū o nā hono, ka umauma, ke ake, a me ka iwi.

• ʻO ke ultrasound ʻōpū
• CT scan o ka ʻōpū
• X-kukuna o nā hono, ka umauma, a me nā wahi ʻōpū (ʻōpū)

ʻO ka pahuhopu o ka mālama ʻana no ka mākua Akā ʻo ka maʻi ka mālama ʻana i nā ʻōuli o ka maʻi kō. ʻO Aspirin a me nā lāʻau anti-inflammatory nonsteroidal ʻē aʻe (NSAIDs), e like me ka ibuprofen, hoʻohana pinepine ʻia ma mua.

Hoʻohana ʻia ʻo Prednisone no nā hihia ʻoi loa.

Inā kaumaha ka maʻi a hoʻomau paha no ka manawa lōʻihi (lilo i maʻi mau), pono ʻia nā lāʻau e kāohi i ka ʻōnaehana pale. ʻO nā lāʻau e like me:

• Methotrexate
• Anakinra (interleukin-1 receptor agonist)
• Tocilizumab (interleukin 6 mea kāohi)
• Tumor nekrosis factor (TNF) antagonists e like me etanercept (Enbrel)

I nā poʻe he nui, hiki i nā hōʻailona ke hoʻi i mau manawa i nā makahiki e hiki mai ana.

Ke hoʻomau nei nā ʻōuli no ka manawa lōʻihi (mau) ma kahi o ka hapakolu o ka poʻe me ka maʻi maʻi mau.

ʻO kahi ʻano laha ʻole o ka maʻi, i kapa ʻia macrophage activation Syndrome, hiki ke kaumaha loa me nā maʻi fiva kiʻekiʻe, nā maʻi nui a me nā helu o ke kahena haʻahaʻa. Pili ka iwi iwi a pono ka biopsy e hana i ke kuhi.

Hiki i nā pilikia ʻē aʻe ke:

• Pākuʻi i loko o kekahi ami
• Maʻi maʻi ate
• Pericarditis
• Hoʻonui effa
• Hoʻonui ākea

Kāhea i kāu mea hāʻawi inā loaʻa iā ʻoe nā ʻōuli o ka maʻi maʻi mau.

Inā ua hōʻoia ʻia ʻoe me kēia ʻano, pono ʻoe e kāhea i kāu mea hāʻawi inā loaʻa ʻoe i ka ʻeha a paʻakikī i ka hanu ʻana.

ʻAʻohe pale e ʻike ʻia.

Maʻi nō naʻe - makua; ʻO ka maʻi hoʻomaka o nā mākua; AOSD; ʻO Wissler-Fanconi Syndrome

Alonso ER, Marques AO. ʻO ka maʻi hoʻomaka mau nā mākua. I: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. ʻEpekema 7 Piladelapia, PA: Elsevier; 2019: chap 173.

Gerfaud-Valentin M, Maucort-Boulch D, Hot A, et al. ʻO ka maʻi hoʻomaka mua o nā mākua: nā hōʻike, nā lāʻau lapaʻau, nā hopena, a me nā mea wānana i nā maʻi he 57. Lapaʻau (Baltimore). 2014; 93 (2): 91-99. PMID: 24646465 www.ncbi.nlm.nih.gov/pubmed/24646465.

Kaneko Y, Kameda H, Ikeda K, et al. ʻO Tocilizumab i nā mea maʻi me ka maʻi o ka mākua hoʻomaka mua ʻole i ka mālama ʻana i ka glucocorticoid: kahi hoʻokolohua, pālua makapō, kahi hoʻokolohua i ka pae III hoʻokolokolo. ʻO Ann Rheum Dis. 2018; 77 (12): 1720-1729. PMID: 30279267 www.ncbi.nlm.nih.gov/pubmed/30279267.

ʻO ka National Organization for Rare Disorder pūnaewele. Nā maʻi kakaikahi.org. Ke hoʻomaka nei nā mākua no ka maʻi o Still. rarediseases.org/rare-diseases/adult-onset-stills-disease/. Loaʻa iā Malaki 30, 2019.

Ortiz-Sanjuán F, Blanco R, Riancho-Zarrabeitia L, et al. Ka hopena o ke anakinra i ka refactory mākua-hoʻomaka i ka maʻi o Still: multicenter hoʻopaʻa haʻawina o 41 nā mea maʻi a me ka moʻomeheu loiloi. Lapaʻau (Baltimore). 2015; 94 (39): e1554. PMID: 26426623 www.ncbi.nlm.nih.gov/pubmed/26426623.