Maʻi maʻi granulomatous

ʻO ka maʻi granulomatous maʻi (CGD) kahi maʻi i hoʻoilina ʻia kahi e holo pono ʻole ai kekahi mau ʻōnaehana pale. ʻO kēia ke alakaʻi i nā maʻi hou a koʻikoʻi.

I ka CGD, hiki ʻole i nā hunaola ʻōnaehana i kapa ʻia nā phagocytes ke pepehi i kekahi ʻano o nā koʻohune a me nā fungi. ʻO kēia maʻi e alakaʻi i nā maʻi lōʻihi (mau) a hoʻomau hou (recurrent). ʻIke pinepine ʻia ke ʻano i ka wā kamaliʻi. ʻIke ʻia paha nā ʻano Milder i nā makahiki ʻōpio, a i ʻole i ka wā makua.

Hoʻopili nā mea pilikia i kahi moʻolelo ʻohana o nā maʻi hou a mau paha.

Ma kahi o ka hapalua o nā hihia CGD i hoʻoili ʻia i waena o nā ʻohana ma ke ʻano he recessive e pili ana i ka moekolohe. ʻO ke kumu o ka loaʻa ʻana o kēia maʻi i nā keikikāne ma mua o nā kaikamahine. Lawe ʻia ka ʻāpana hemahema ma ka X chromosome. Loaʻa i nā keikikāne he 1 X chromosome a me 1 Y chromosome. Inā loaʻa i kahi keikikāne kahi chromosome X me ka gen defective, hiki iā ia ke hoʻoilina i kēia ʻano. Loaʻa i nā kaikamahine 2 chromosome X. Inā he 1 X chromosome ko kekahi kaikamahine me ka ʻāpana hemahema, loaʻa i kahi X chromosome ʻē aʻe kahi ʻohana hana e pani no ia. Pono i kahi kaikamahine ke hoʻoilina i ka X hapa hemahema mai kēlā me kēia makua i mea e loaʻa ai ka maʻi.

Hiki i ka CGD ke hana i nā ʻano maʻi ʻili he paʻakikī e mālama, me:

• Pālū a ʻeha paha ma ka maka (impetigo)
• Eczema
• ʻO nā ulu i piha me ka pus (abscesses)
• Pahu piha i ka ʻili i loko o ka ʻili (paila)

Hiki iā CGD ke kumu:

• Pohā mau
• Pehu nā lymph node i ka ʻāʻī
• ʻO nā maʻi lolo, e like me ka pulumonia a me ka abscess

E nānā ka mea mālama ola a loaʻa paha:

• Huʻi ka ake
• Huʻi ʻāpala
• Nā pehu lymph pehu

Aia paha he hōʻailona o ka maʻi o ka iwi, kahi e hopena ai i nā iwi he nui.

ʻO nā hoʻāʻo e hana ʻia ana paha:

• ʻImi iwi
• Pākuʻi x-ray
• Helu piha koko (CBC)
• ʻO nā hoʻokolohua cytometry kahe e kōkua i ka hōʻoia ʻana i ka maʻi
• ʻO ka hoʻāʻo genetic e hōʻoia i ka ʻike
• Hōʻike o ka hana keʻokeʻo koko keʻokeʻo
• ʻO ka biopsy kope

Hoʻohana ʻia nā lāʻau antibiotic e mālama i ka maʻi, a hoʻohana ʻia paha e pale aku i nā maʻi. Hiki i kekahi lāʻau i kapa ʻia interferon-gamma ke kōkua i ka hōʻemi ʻana i ka nui o nā maʻi ʻino. Pono paha ke ʻoki e mālama i kekahi mau abscesses.

ʻO ka mea hoʻōla wale no ka CGD kahi iwi iwi a i ʻole ke transplant cell stem.

Hiki i nā lāʻau antibiotic lōʻihi ke kōkua i ka hōʻemi ʻana i nā maʻi, akā hiki i ka make mua ke kū mai nā maʻi maʻi māmā hou.

Hiki iā CGD ke hana i kēia mau pilikia:

• Poino a me nā maʻi
• Nā maʻi maʻi ma ka ihu
• ʻO ka pulumonia e hoʻi mau nei a paʻakikī e hoʻōla
• Poino o ka puʻu
• Poino ʻili
• Pehu nā lymph node e noho pehu, hana pinepine, a i ʻole e hana i nā abscesses e pono ai ke ʻoki e hoʻokahe iā lākou

Inā loaʻa iā ʻoe a ʻo kāu keiki paha kēia ʻano maʻi a kuhi ʻoe i ka maʻi ponia a i ʻole ka maʻi ʻē aʻe, e kāhea koke aku i kāu mea hāʻawi.

E haʻi i kāu mea hāʻawi inā ʻaʻohe pane ka maʻi, ka ʻili, a i ʻole nā ​​maʻi ʻē aʻe i ka lāʻau.

Paipai ʻia ka ʻōlelo aʻoaʻo genetic inā ʻoe e hoʻolālā i nā keiki a he moʻolelo ʻohana kāu no kēia maʻi. ʻO ka holomua i ka kānana genetika a me ka hoʻonui ʻana i ka hoʻohana ʻana i ka chorionic villus sampling (kahi hōʻike e hiki ke hana ʻia i ka manawa o ka wahine ma kahi o 10 a 12th wiki o ka hāpai ʻana) ua hiki ke ʻike ʻia ma kahi o ka CGD hiki. Eia nō naʻe, ʻaʻole hoʻolaha a ʻae ʻia kēia mau hana.

CGD; ʻO ka granulomatosis make o ka wā kamaliʻi; Maʻi granulomatous maʻi o ka wā kamaliʻi; Holomua septic granulomatosis; Ka hemahema Phagositte - maʻi maʻi granulomatous mau

ʻO Glogauer M. Nā maʻi o ka hana phagocyte. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 25th hua. Piladelapia, PA: Elsevier Saunders; 2016: chap 169.

ʻO Holland SM, nā hemahema ʻo Uzel G. Phagocyte. I: Rich RR, Fleisher TA, Shearer WT, Schroeder JR. HW, Frew AJ, Weyand CM, hoʻoponopono. ʻO Immunology Haumāna: Nā Kumu a me nā Haʻawina. 5th lā. Piladelapia, PA: Elsevier; 2019: chap 22.