Lamellar ichthyosis

Lamellar ichthyosis (LI) ndikosowa khungu. Amawonekera pakubadwa ndikupitilira moyo wake wonse.

LI ndimatenda obwereza otsogola. Izi zikutanthauza kuti mayi ndi bambo ayenera kupatsira mwana wawo mtundu umodzi wamtunduwu wa matendawa kuti mwana adziwe matendawa.

Ana ambiri omwe ali ndi LI amabadwa ndi khungu loyera, lonyezimira, lopaka phulusa lotchedwa collodion membrane. Pachifukwa ichi, makanda awa amadziwika kuti ana amakoloni. Kakhungu kamatuluka mkati mwa masabata awiri oyamba amoyo. Khungu lomwe lili pansi pa nembanemba ndi lofiira komanso lofiira ngati nkhope ya nsomba.

Ndi LI, khungu lakunja lotchedwa epidermis silingateteze thupi monga momwe epidermis yathanzi imathandizira. Zotsatira zake, mwana yemwe ali ndi LI atha kukhala ndi mavuto awa:

• Kuvuta kudyetsa
• Kutaya madzi (madzi m'thupi)
• Kuchepa kwa mchere m'thupi (kusalingana kwa electrolyte)
• Mavuto opumira
• Kutentha kwa thupi kosakhazikika
• Khungu kapena matenda opatsirana thupi

Ana okalamba ndi akulu omwe ali ndi LI atha kukhala ndi izi:

• Masikelo akulu kwambiri omwe amaphimba thupi lonse
• Kuchepetsa kutuluka thukuta, ndikupangitsa chidwi kutentha
• Kutaya tsitsi
• Chala chachilendo ndi zikhadabo
• Khungu la kanjedza ndi zidendene limakhuthala

Makanda a Collodion nthawi zambiri amafunika kukhala mchipinda cha ana osamalidwa mwakhama (NICU). Iwo anayikidwa mu mkulu-chinyezi chofungatira. Adzafunika zowonjezera zowonjezera. Zodzitetezera ziyenera kupakidwa pakhungu. Kamodzi kakapangidwe katulutsidwe, makanda amatha kupita kwawo.

Kusamalira khungu kwa moyo wonse kumaphatikizapo kusunga khungu lonyowa kuti lichepetse makulidwe ake. Njira ndi monga:

• Zodzola zopaka mafuta pakhungu
• Mankhwala otchedwa retinoids omwe amatengedwa pakamwa pakavuta
• Malo otentha kwambiri
• Kusamba kumasula masikelo

Makanda ali pachiwopsezo chotenga kachilombo akamakhetsa nthanda.

Mavuto amaso amatha kubwera pambuyo pake chifukwa maso sangathe kutseka kwathunthu.

LI; Mwana wakhanda - lamellar ichthyosis; Ichthyosis kobadwa nako; Autosomal recessive congenital ichthyosis - mtundu wa lamellar ichthyosis

• Ichthyosis, anapeza - miyendo

Martin KL. Zovuta za keratinization. Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS. Wogwira ntchito RC, Wilson KM, eds. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 677.

Patterson JW. Zovuta zakukula kwa khungu ndi keratinization. Mu: Patterson JW, mkonzi. Matenda a Khungu la Weedon. 5th ed. Philadelphia, PA: Elsevier; 2021: mutu 10.

Richard G, Ringpfeil F. Ichthyoses, erythrokeratodermas, ndi zovuta zina. Mu: Bolognia JL, Schaffer JV, Cerroni L, olemba. Matenda Opatsirana. Wolemba 4. Philadelphia, PA: Elsevier; 2018: mutu 57.