Lamellar ichthyosis

Lamellar ichthyosis (LI) yog qee yam tsis tshua muaj tawv nqaij. Nws tshwm sim thaum yug los thiab txuas mus tas lub neej.

LI yog mob autosomal recessive kab mob. Qhov no txhais tau hais tias leej niam thiab leej txiv yuav tsum faib ob daim qauv sib txawv ntawm cov kab mob mus rau lawv cov menyuam kom tus menyuam muaj tus kabmob.

Coob tus me nyuam nrog LI yog yug los muaj qhov pom tseeb, ci, tawv txheej ntawm tawv nqaij hu ua collodion membrane. Vim li no, cov menyuam mosliab no lub npe hu ua cov menyuam yaus sib xyaws. Daim nyias nyias nyias li hauv thawj 2 lub lim tiam ntawm lub neej. Daim tawv nqaij hauv qab daim nyias nyias yog xim liab thiab txaij zoo li cov ntses.

Nrog LI, txheej txheej sab nrauv ntawm daim tawv nqaij hu ua epidermis tsis tuaj yeem tiv thaiv lub cev zoo li cov epidermis noj qab nyob zoo. Raws li qhov tshwm sim, tus menyuam mos nrog LI yuav muaj teeb meem kev noj qab haus huv:

• Nyuaj hauv kev pub mis
• Ua kua dej (lub cev qhuav dej)
• Kev poob ntawm cov zaub mov hauv lub cev (electrolyte tsis txaus)
• Cov teeb meem ua pa
• Lub cev kub uas tsis ruaj khov
• Cov tawv nqaij lossis lub cev muaj thoob plaws

Cov menyuam yaus me thiab cov neeg laus nrog LI yuav muaj cov tsos mob no:

• Cov dos loj uas npog yuav luag txhua lub cev
• Tsis tshua muaj peev xwm ua hws, ua rau rhiab heev rau tshav kub
• Cov plaub hau poob lawm
• Txawv ntiv tes thiab ntiv taw
• Daim tawv nqaij ntawm xib teg thiab cov qab xib taws tuab

Cov menyuam mos uas nyob hauv ib qho chaw zov menyuam feem ntau yuav tsum tau nyob hauv lub tsev tu menyuam menyuam yaus (NICU). Lawv muab tso rau hauv qhov chaw siab ua kom sov siab. Lawv yuav xav tau noj mov ntxiv. Hws noo yuav tsum tau siv rau daim tawv nqaij. Tom qab muab cov ntaub qhwv ua ke tas, cov menyuam mos feem ntau tuaj yeem mus tsev.

Lub neej tu ntawm daim tawv nqaij koom nrog ua kom cov tawv nqaij noo kom txo cov tuab ntawm cov nplai. Kev ntsuas suav nrog:

• Hws noo noo siv rau daim tawv nqaij
• Cov tshuaj hu ua retinoids uas tau noj ntawm qhov ncauj yog mob hnyav
• High-humidity ib puag ncig
• Da dej kom plam nplai

Cov menyuam mos yog cov muaj kev pheej hmoo kis tus mob thaum lawv tso lub tshuab lo rau.

Teeb meem qhov muag yuav tshwm sim tom qab lub neej vim lub qhov muag tsis pom kev kiag li.

LI; Collodion tus me nyuam - lamellar ichthyosis; Ichthyosis congenital; Autosomal recessive congenital ichthyosis - lamellar ichthyosis yam

• Ichthyosis, nrhiav - ceg

Martin KL. Kev tsis sib haum ntawm keratinization. Hauv: Kliegman RM, St. Geme JW, Blum NJ, Shah SS. Tasker RC, Wilson KM, eds. Nelson Phau Ntawv ntawm PediatricsCov. 21st ed. Philadelphia, PA: Elsevier; 2020: chap 677.

Patterson JW. Kev tsis sib haum ntawm cov mob epidermal maturation thiab keratinization. Hauv: Patterson JW, ed. Weedon's Cov Khoom Siv Tawv TawvCov. Thib 5 ed. Philadelphia, PA: Elsevier; 2021: chap 10.

Richard G, Ringpfeil F. Ichthyoses, erythrokeratodermas, thiab lwm yam kev cuam tshuam. Hauv: Bolognia JL, Schaffer JV, Cerroni L, eds. Lub cev ntaj ntsugCov. 4 tug ed. Philadelphia, PA: Elsevier; 2018: chap 57.