ʻO Antiphospholipid Syndrome - APS

ʻO Antiphospholipid Syndrome (APS) kahi maʻi autoimmune e pili ana i nā kahe koko pinepine (thromboses).Ke loaʻa ʻoe i kēia ʻano, hana ka ʻōnaehana pale o kou kino i nā protein āpau e hoʻouka i nā huna koko a me ka uhi o nā kīʻaha koko. Hiki i ke kū ʻana o kēia mau mea ʻino ke kumu o nā pilikia me ke kahe o ke koko a alakaʻi i nā clots weliweli i nā kīʻaha koko ma loko o ke kino.

ʻAʻole ʻike ʻia ke kumu kūpono o APS. Hoʻololi kekahi mau ʻano ʻē aʻe a me nā kumu ʻē aʻe (e like me ka maʻi) i kumu e hoʻomohala i ka pilikia.

ʻIke pinepine ia i ka poʻe me nā maʻi autoimmune ʻē aʻe, e like me ka systemus lupus erythematosus (SLE). ʻOi aku ka maʻamau o nā wahine ma mua o nā kāne, ʻike pinepine ʻia ia i nā wahine i loaʻa ka mōʻaukala hana hewa hou ʻana.

Hāpai kekahi poʻe i nā mea ʻino i haʻi ʻia ma luna, akā ʻaʻohe APS. Hiki paha i kekahi mau mea hoʻonāukiuki ke loaʻa i kēia poʻe i ke koko koko, e like me:

• Puhipaka
• Hoʻomaha hoʻomaha lōʻihi
• Hapai keiki
• ʻO ka Hormone therapy a i ʻole nā ​​kaohi kaohi hānau
• Kanesa
• ʻO ka maʻi kīwī

ʻAʻohe paha ou mau ʻōuli, ʻoiai loaʻa iā ʻoe nā lāʻau ʻino. Nā ʻōuli e kū aʻe paha:

• Hoʻopili ke koko i nā wāwae, nā lima a me nā akemāmā. Hiki i nā clots i loko o nā uaua a i ʻole nā ​​ʻaʻa.
• Hānau hewa hou a hānau hou paha.
• Rash, i kekahi poʻe.

I nā hihia maʻamau, ulu koke nā ʻōpala i nā aʻa he nui ma kahi o nā lā. Kāhea ʻia kēia ʻo catastrophic anti-phospholipid syndrome (CAPS). Hiki iā ia ke alakaʻi i ka hahau a me nā clots i ka puʻupaʻa, ke ake, a me nā mea ʻē aʻe i loko o ke kino, a me ka gangrene i nā lālā.

Hiki ke hana i nā hoʻāʻo no ka antusagulant lupus a me nā antipospolipid antodies i ka wā:

• Kū mai kahi maʻi koko i manaʻo ʻole ʻia, e like me ka poʻe ʻōpio a i ʻole nā ​​mea me nā kumu pilikia ʻē aʻe ʻole no ka hoʻopili ʻana o ke koko.
• He moʻolelo ko ka wahine no ka lilo hou ʻana o ka hāpai.

ʻO nā hoʻokolohua anticoagulant lupus he mau hōʻuluʻulu koko. ʻO ka antiphospholipid antibodies (aPL) hoʻonāukiuki i ka hoʻāʻo ʻana ma ka hale hana.

Hiki i nā ʻano o nā hoʻāo ʻana i ka hoʻopili ʻana ke komo pū me:

• Hoʻonohonoho ʻia ka manawa thromboplastin hapa (aPTT)
• ʻO Russell viper wā make
• Hōʻike hoʻōla hoʻōla thromboplastin

E hana pū ʻia nā hoʻāʻo ʻana no nā mea ʻino antiphospholipid (aPL). Pākuʻi ʻia lākou:

• Nā hoʻāʻo antibody Anticardiolipin
• Nā Antibodies i ka beta-2-glypoprotein I (Beta2-GPI)

E hōʻoia kāu mea mālama ola i ka antiphospholipid antibody syndrome (APS) inā he hōʻoia maikaʻi kāu no ka aPL a i ʻole ka lupus anticoagulant, a hoʻokahi a ʻoi paha o nā hanana aʻe:

• Koko koko
• Nā hana hewa hou ʻole

Pono e hōʻoia i nā hōʻoia maikaʻi ma hope o 12 mau pule. Inā he hōʻoia maikaʻi kāu me ka ʻole o nā hiʻohiʻona ʻē aʻe o ka maʻi, ʻaʻole e loaʻa iā ʻoe ka hōʻailona o ka APS.

Hoʻonohonoho ʻia ka lapaʻau ʻana no ka APS i ka pale ʻana i nā pilikia mai nā pehu koko hou a i ʻole nā ​​clots o kēia wā e nui ana. Pono ʻoe e lawe i kahi ʻano o ka lāʻau lapaʻau koko. Inā loaʻa iā ʻoe kekahi maʻi autoimmune, e like me lupus, pono ʻoe e mālama i kēlā ʻano ma lalo o ka kaohi.

E pili ka mālama pono ʻana i ka nui o kou ʻano a me nā kūpikipiki e hana nei.

ANTIPHOSPHOLIPID ANTIBODY Syndrome (APS)

Ma ka laulā, pono ʻoe i ka lāʻau lapaʻau me ka mea lahilahi o ke koko no ka manawa lōʻihi inā he APS kāu. Heparin paha ka mālama mua ʻana. Hāʻawi ʻia kēia mau lāʻau i ke kō.

I ka hapanui o nā hihia, warfarin (Coumadin), i hāʻawi ʻia e ka waha, a laila hoʻomaka. Pono pono e nānā pinepine i ka pae o anticoagulation. Hana pinepine ʻia kēia i ka hoʻohana ʻana i ka hōʻike INR.

Inā loaʻa iā ʻoe ka APS a hāpai, pono ʻoe e ukali pono ʻia e kahi mea hoʻolako i mākaukau i kēia ʻano. ʻAʻole ʻoe e lawe i ka warfarin i ka wā hāpai, akā hāʻawi ʻia nā kiʻi heparin iā ʻoe.

Inā loaʻa iā SLE a me APS, e paipai pū kāu mea lawelawe iā ʻoe e lawe i ka hydroxychloroquine.

I kēia manawa, ʻaʻole pono e koi ʻia nā ʻano lāʻau ʻokiʻoki koko ʻē aʻe.

CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME (CAPS)

Hoʻomaʻamaʻa no ka CAPS e pili ana i ka hui pū ʻana o anticoagulation therapy, nā kaha kiʻekiʻe o nā corticosteroids, a me nā pānaʻi plasma i maikaʻi i ka hapanui o ka poʻe. I kekahi manawa hoʻohana ʻia ʻo IVIG, rituximab a i ʻole eculizumab no nā hihia koʻikoʻi.

KA HOʻOLOHI ANA NO KA LUPUS ANTICOAGULANT A i ʻole APL

ʻAʻole ʻoe e pono i ka lāʻau lapaʻau inā ʻaʻohe ou ʻōuli, pohō hāpai, a inā ʻaʻole ʻoe i loaʻa i ke koko.

E hana i nā kaʻina hana aʻe e kōkua ai i ka pale ʻana i nā kahe koko.

• Hōʻalo i ka nui o nā lāʻau hoʻomake hānau a i ʻole nā ​​mālama ʻana i ka hormone no ka menopause (wahine).
• Mai puhi a hoʻohana paha i nā huahana paka ʻē aʻe.
• E ala aʻe a neʻe i kahi o nā mokulele mokulele a i ʻole nā ​​manawa ʻē aʻe inā ʻoe e noho a moe i lalo no nā kau lōʻihi.
• E hoʻoneʻe i kou kuʻekuʻe wāwae i luna ke hiki ʻole ke neʻe.

E kuhikuhi ʻia ʻoe i nā lāʻau lapaʻau hōʻemi koko (e like me heparin a me warfarin) i mea e pale aku ai i ke koko koko:

• Ma hope o ke kaha ʻana
• Ma hope o ka haki iwi
• Me ka maʻi ʻaʻai ikaika
• Ke pono ʻoe e noho a moe i lalo no nā manawa lōʻihi, e like me ka wā o kahi haukapila e noho ana a i ʻole ke ola ʻana i ka home

Pono paha ʻoe e lawe i nā mea lahilahi o ke koko no 3 a 4 mau pule ma hope o ke kahakaha ʻana e hoʻohaʻahaʻa ai i kou makaʻi o ke koko koko.

Me ka ʻole o ka lapaʻau, e loaʻa hou i nā poʻe me APS i ke kāpili hou ʻana. ʻO ka hapanui o ka manawa, maikaʻi ka hopena me ka lāʻau kūpono, i hoʻopili ʻia me ka anticoagulation therapy lōʻihi. Loaʻa paha i kekahi poʻe ka huhū koko paʻakikī e kaohi ʻia me ke ʻano o nā ʻano lapaʻau. Hiki i kēia ke alakaʻi iā CAPS, hiki ke hoʻoweliweli i ke ola.

Kāhea i kāu mea hāʻawi inā ʻike ʻoe i nā ʻōuli o ke koko, e like me:

• ʻO ka pehu a ʻulaʻula paha i ka wāwae
• Pokole o ka hanu
• ʻEha, puʻuhonua, a me ke kala ʻulaʻula i ka lima a me ka wāwae

E kamaʻilio pū me kāu mea hāʻawi inā loaʻa hou iā ʻoe ka lilo o ka hāpai ʻana (hānau hānau ʻole).

Nā Anticardiolipin nā mea pale; Hughes maʻi maʻi

• Hoʻoweliweli ka lupus erythematosus ʻōnaehana i ka maka
• Huʻi koko

Amigo M-C, Khamashta MA. Antiphospholipid Syndrome: pathogenesis, ʻike, a me ka hoʻokele. I: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. ʻEpekema 7 Piladelapia, PA: Elsevier; 2019: chap 148.

Cervera R, Rodríguez-Pintó I, Colafrancesco S, et al. Hōʻike ka 14th International Congress ma ka Antiphospholipid Antibodies Task Force e pili ana i ka maʻi maʻi catiphopholipid catastrophic. ʻO Autoimmun Rev. 2014; 13 (7): 699-707. PMID: 24657970 www.ncbi.nlm.nih.gov/pubmed/24657970.

ʻO Dufrost V, Risse J, Wahl D, Zuily S. Hoʻohana i nā anticoagulants waha i ka antiphospholipid syndrome: he hopena maikaʻi a palekana paha kēia i ka warfarin? He hōʻike ʻōnaehana o nā puke: ka pane i ka ʻōlelo. ʻO Curr Rheumatol Rep. 2017; 19 (8): 52. PMID: 28741234 www.ncbi.nlm.nih.gov/pubmed/28741234.

ʻO Erkan D, Salmon JE, Lockshin MD. Pākuʻi anti-phospholipid. I: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, nā hua. ʻO Kelley lāua ʻo Firestein's Bookbook of Rheumatology. 10 o ka lā. Piladelapia, PA: Elsevier; 2017: chap 82.

Pūnaewele National Heart, Lung, a me Blood Institute. ʻO Antiphospholipid antibody syndrome. www.nhlbi.nih.gov/health-topics/antiphospholipid-antibody-syndrome. Loaʻa iā Iune 5, 2019.