Lamellar ichthyosis

ʻO Lamellar ichthyosis (LI) kahi ʻano ʻili laha ʻole. ʻIke ʻia i ka wā hānau a hoʻomau i ke ola holoʻokoʻa.

ʻO LI kahi maʻi recessive autosomal. ʻO ke kumu o ia mea pono i ka makuahine a me ka makuakāne ke hāʻawi i hoʻokahi kope ʻē aʻe o ka ʻāpana maʻi i kā lākou keiki i mea e ulu ai ka maʻi i ka maʻi.

Hānau ʻia nā pēpē he nui me LI me kahi ʻōlinolino, ʻōlinolino, a hana ʻia i ka ʻili i kapa ʻia he membrane collodion. No kēia kumu, ʻike ʻia kēia mau pēpē he pēpē collodion. Kahe ka membrane i loko o nā pule he 2 o ke ola. ʻUlaʻula a wīwī ka ʻili ma lalo o ka membrane e like me ka ʻili o ka iʻa.

Me LI, ʻo ka ʻili o waho o ka ʻili i kapa ʻia ʻo epidermis ʻaʻole hiki ke pale i ke kino e like me ka hiki i ka epidermis olakino. A ʻo kahi hopena, loaʻa paha i kahi pēpē me LI nā pilikia olakino e hiki mai ana:

• Ka paʻakikī i ka hānai ʻana
• Ka nalowale o ka wai (make wai)
• Ka nalowale o ke kaulike o nā minelala i ke kino (electrolyte imbalance)
• Nā pilikia hanu
• ʻAʻole paʻa ka mahana o ke kino
• ʻO nā maʻi ʻili a i ʻole ke kino

Loaʻa paha i nā keiki ʻelemakule a me nā mākua me LI kēia mau ʻōuli:

• ʻO nā unahi nunui e uhi i ka hapa nui o ke kino
• Hoemi i ka hiki i ka hou, hoʻonāukiuki i ka wela
• Lilo ka lauoho
• ʻO ka manamana lima a me nā manamana wāwae ʻole
• Mānoanoa ka ʻili o nā poho lima a me nā kapuwaʻi

Pono nā pēpē collodion e noho mau i ka neonatal intensive care unit (NICU). Hoʻokomo ʻia lākou i loko o kahi incubator hoʻoweliweli kiʻekiʻe. Pono lākou i nā hānai keu. Pono e hoʻopili i nā mākū i ka ʻili. Ma hope o ka hoʻokahe ʻia ʻana o ka membrane collodion, hiki i nā pēpē ke hoʻi maʻamau i ka home.

Mālama ke ola a pau i ka ʻili e mālama i ka ʻili e hoʻoliʻiliʻi i ka mānoanoa o nā unahi. Nā ana e:

• Hoʻopili ka Moisturizers i ka ʻili
• Kapa ʻia nā lāʻau i retinoids e lawe ʻia e ka waha i nā hihia koʻikoʻi
• Kūlana kiʻekiʻe-wai
• E ʻauʻau ana e hoʻonā i nā unahi

Kūpono nā pēpē no ka maʻi i ka wā e hoʻokahe ai lākou i ka membrane collodion.

Hiki ke pilikia i ka maka ma hope o ke ola no ka mea ʻaʻole hiki i nā maka ke pani paʻa loa.

LI; Pēpē Collodion - lamellar ichthyosis; Ichthyosis hānau; ʻO Autchomal recessive congenital ichthyosis - ʻano lamchar ichthyosis

• ʻIchthyosis, loaʻa - nā wāwae

ʻO Martin KL. Nā maʻi o ka keratinization. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS. Tasker RC, Wilson KM, hoʻoponopono. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 677.

Patterson JW. Nā maʻi o ka oʻo ʻana o ka epidermal a me ka keratinization. I: Patterson JW, hoʻoponopono. ʻO Weedon's Skin Pathology. 5th lā. Piladelapia, PA: Elsevier; 2021: chap 10.

Richard G, Ringpfeil F. Ichthyoses, erythrokeratodermas, a me nā maʻi e pili pū ana. I: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. ʻEhā ed. Piladelapia, PA: Elsevier; 2018: chap 57.