Lamellar ichthyosis

Lamellar ichthyosis (LI) ndeye zvisingawanzo ganda mamiriro. Inoonekwa pakuzvarwa uye inoenderera muhupenyu hwese.

LI chirwere che autosomal recessive. Izvi zvinoreva kuti amai nababa vanofanirwa kupfuudza imwechete yekodhi rechirwere kumwana wavo kuitira kuti mwana akure chirwere.

Vazhinji vacheche vane LI vanozvarwa vaine yakajeka, inopenya, waxy layer yeganda inonzi collodion membrane. Nechikonzero ichi, vacheche ava vanozivikanwa sevana vacheche. Iyo membrane inodurura mukati memavhiki maviri ekutanga ehupenyu. Ganda riri pasi pechidziro rakatsvuka uye rine mitsara yakafanana nepamusoro pehove.

NeI, iyo yekunze nhete yeganda inonzi iyo Epidermis haigone kuchengetedza muviri sezvingaite epidermis ine hutano. Nekuda kweizvozvo, mwana ane LI anogona kuve nezvinetso zvinotevera zvehutano:

• Zvakaoma mukudyisa
• Kurasikirwa kwemvura (kupera mvura mumuviri)
• Kurasikirwa kwechiyero chemaminerari mumuviri (kusaenzana kwemagetsi)
• Matambudziko ekufema
• Tembiricha yemuviri isina kugadzikana
• Ganda kana hutachiona hwemuviri

Vana vakura nevakuru vane LI vanogona kuva nezviratidzo izvi:

• Zviyero zvihombe zvinovhara muviri wese
• Yakadzikira kugona kudikitira, zvichikonzera kushushikana pakupisa
• Bvudzi kurasikirwa
• Chigunwe chisina kujairika nezvigunwe
• Ganda remichindwe nematete rakakora

Collodion vacheche kazhinji vanofanirwa kugara muyonatal intensive care unit (NICU). Ivo vanoiswa mune yakakwira-hunyoro incubator. Ivo vanozoda imwe yekuwedzera feedings. Zvinodzora zvinoda kuiswa paganda. Mushure mekudonhwa kwemboro yembudzi, vacheche vanogona kazhinji kuenda kumba.

Kuchengetedza kwese kweganda kunosanganisira kuchengetedza ganda rakanyorova kudzikisira huremu hwezviyero. Matanho anosanganisira:

• Mafuta anodzora paganda
• Mishonga inonzi retinoids inotorwa nemuromo mune zvakakomba zviitiko
• Yakakwirira-mwando nharaunda
• Kugeza kusunungura zviyero

Vacheche vari panjodzi yekutapukirwa pavanodurura iyo collodion membrane.

Zvinetso zvemaziso zvinogona kuitika mushure mehupenyu nekuti maziso haakwanise kuvhara zvachose.

LI; Collodion mucheche - lamellar ichthyosis; Ichthyosis kuberekwa; Autosomal recessive congenital ichthyosis - lamellar ichthyosis mhando

• Ichthyosis, yakawanikwa - makumbo

Martin KL. Kukanganiswa kwekeratinization. Mune: Kliegman RM, St. Geme JW, Blum NJ, Shah SS. Tasker RC, Wilson KM, eds. Nelson Chinyorwa chePediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020: chap 677.

Patterson JW. Kukanganiswa kwepidermal maturity uye keratinization. Mune: Patterson JW, ed. Weedon's Ganda Pathology. 5 ed. Philadelphia, PA: Elsevier; 2021: chits.

Richard G, Ringpfeil F. Ichthyoses, erythrokeratodermas, uye zvimwe zvinetso zvakabatana. Mune: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2018: chits. 57.