Nā dysplasias Ectodermal

ʻO ka Ectodermal dysplasias kahi hui o nā ʻano i ulu ʻole ka ulu o ka ʻili, ka lauoho, nā kui, nā niho, a me nā ʻaukā.

Nui a hewahewa nā ʻano like ʻole o nā ectodermal dysplasias. Hoʻokumu ʻia kēlā me kēia ʻano o ka dysplasia e nā hoʻololi kikoʻī i kekahi mau ʻāpana. ʻO Dysplasia ke kumu o ka hoʻomohala ʻole ʻana o nā hunaola a i ʻole nāʻaʻaʻa. ʻO ke ʻano maʻamau o ka ectodermal dysplasia ka mea maʻa mau i nā kāne. Hoʻopili like nā ʻano maʻi ʻē aʻe i nā kāne a me nā wahine.

ʻO ka poʻe me ka ectodermal dysplasia ʻaʻole paha e ka hou a i ʻole ka hou ma mua o ka maʻamau no ka nele o ka momona.

I nā keiki me ka maʻi, pilikia paha ko lākou kino i ke kaohi ʻana i nā fiva. ʻOiai kahi maʻi ʻino hiki ke hana i kahi wela nui loa, no ka mea ʻaʻole hiki i ka ʻili ke hou a kaohi pono i ka mahana.

ʻAʻole hiki i nā mākua i hoʻopilikia ke hoʻomanawanui i kahi ʻano mehana a pono i nā ana, e like me ka ea ʻana, e mālama ai i ka mahana o ke kino.

Kaukaʻi ʻia i nā ʻāpana i hoʻopili ʻia, hiki i nā ʻōuli ʻē aʻe ke hoʻopili pū.

• Nā kui kuʻi ʻole
• Nā niho maʻamau a nalowale paha, a ʻoi aku paha ma mua o ka helu maʻamau o nā niho
• Lip lehelehe
• Hoʻoemi i ke kala o ka ʻili (pigment)
• Lae nui
• Alahaka ihu haʻahaʻa
• Lahilahi, lauoho lauoho
• Nā pilikia kīnā
• Lohe maikaʻi ʻole
• ʻIke maikaʻi ʻole me ka hoʻemi ʻana i ka hana waimaka
• ʻŌnaehana pale nawaliwali

ʻO nā hoʻāʻo e hana ʻia ana paha:

• ʻO Biopsy o nā membrane mucous
• ʻO Biopsy o ka ʻili
• Ke hoʻāʻo genetic (loaʻa no kekahi ʻano o kēia maʻi)
• Hana ʻia nā kukuna X o nā niho a iwi paha

ʻAʻohe mālama kūikawā no kēia maʻi. Akā, mālama ʻia nā ʻōuli e pono ai.

Hiki i nā mea hiki iā ʻoe ke hoʻopili i:

• Lawe i ka wig a me nā niho pilikino e hoʻomaikaʻi i ka helehelena.
• E hoʻohana i nā waimaka hana e pale i ka maloʻo ʻana o nā maka.
• E hoʻohana i ka saline ihu ʻehu e hemo i nā ʻōpala a pale i ka maʻi.
• E lawe i ka wai ʻauʻau anuanu a hoʻohana i nā wai wai e mālama i ka mahana o ke kino maʻamau (hoʻololi ka wai mai ka ʻili i ka hana hōʻoluʻolu o ka hou i mahu mai ka ʻili.)

Hiki i kēia mau kumuwaiwai ke hāʻawi i ka ʻike hou aku e pili ana i ka ectodermal dysplasias:

• ʻO ka Ectodermal Dysplasia Society - edsociety.co.uk
• National Foundation no Ectodermal Dysplasias - www.nfed.org
• NIH Genetic and Rare Diseases Information Center - rarediseases.info.nih.gov/diseases/6317/ectodermal-dysplasia

Inā loaʻa iā ʻoe kahi ʻano like ʻole o ka ectodermal dysplasia ʻaʻole kēia e hoʻopōkole i kou ola. Eia nō naʻe, pono paha ʻoe e nānā i nā loli o ka mahana a me nā pilikia ʻē aʻe e pili ana i kēia ʻano.

Inā mālama ʻole ʻia, hoʻopili pū ʻia nā pilikia olakino mai kēia ʻano:

• Hoʻopilikia ka lolo i ka hoʻonui ʻia o ka mahana o ke kino
• Ka hopu ʻia ʻana e ka fiva kiʻekiʻe (febrile seizures)

Kāhea i kahi manawa me kāu kahu mālama ola inā hōʻike kāu keiki i nā ʻōuli o kēia maʻi.

Inā loaʻa iā ʻoe kahi moʻolelo ʻohana o ectodermal dysplasia a ke hoʻolālā nei ʻoe i nā keiki, paipai ʻia ka ʻōlelo aʻoaʻo ʻiʻo. I nā manawa he nui, hiki ke ʻike pono i ka ectodermal dysplasia ʻoiai e noho ana ka pēpē i loko o ka ʻōpū.

Anhidrotic ectodermal dysplasia; ʻO Christ-Siemens-Touraine maʻi; Anondontia; Pahu hoʻohenehene

• Nā ʻili o ka ʻili

Abidi NY, Martin KL. Nā dysplasias Ectodermal. I: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 668.

ʻO Narendran V. Ka ʻili o ka neonate. I: Martin RJ, Fanaroff AA, Walsh MC, hua. ʻO Fanaroff lāua ʻo Martin's Neonatal-Perinatal Medicine. 11th hua. Piladelapia, PA: Elsevier; 2020: chap 94.