Ectodermal dysplasias

Ectodermal dysplasias ndi gulu lazikhalidwe zomwe zimakula bwino khungu, tsitsi, misomali, mano, kapena thukuta la thukuta.

Pali mitundu yambiri yama ectodermal dysplasias. Mtundu uliwonse wa dysplasia umayambitsidwa ndi masinthidwe ena amtundu wina. Dysplasia amatanthauza kukula kosafunikira kwamaselo kapena minofu. Mtundu wofala kwambiri wa ectodermal dysplasia nthawi zambiri umakhudza amuna. Matenda ena amakhudza amuna ndi akazi mofanana.

Anthu omwe ali ndi ectodermal dysplasia sangachite thukuta kapena thukuta locheperako chifukwa chakuchepa kwa tiziwalo totuluka thukuta.

Kwa ana omwe ali ndi matendawa, matupi awo atha kukhala ndi vuto kuwongolera malungo. Ngakhale matenda ofatsa amatha kutulutsa malungo akulu kwambiri, chifukwa khungu silimatha kutuluka thukuta ndikuwongolera kutentha bwino.

Akuluakulu okhudzidwa sangathe kupirira malo otentha ndipo amafunikira zinthu, monga zowongolera mpweya, kuti azitha kutentha thupi.

Kutengera ndi majini omwe amakhudzidwa, zizindikilo zina zimatha kuphatikiza:

• Misomali yachilendo
• Mano osadziwika kapena osowa, kapena ochepera kuchuluka kwa mano
• Mlomo wonyezimira
• Kuchepetsa khungu (pigment)
• Chipumi chachikulu
• Mlatho wotsika wapansi
• Woonda, tsitsi lochepa
• Kulephera kuphunzira
• Kusamva bwino
• Maso olakwika ndikuchepa kwa misozi
• Kufooka kwa chitetezo cha mthupi

Mayeso omwe angachitike ndi awa:

• Chidutswa cha nembanemba mucous
• Chikopa cha khungu
• Kuyesedwa kwa majini (kupezeka pamitundu ina ya matendawa)
• Kujambula kwa mano kapena mafupa kumatha kuchitika

Palibe mankhwala enieni a vutoli. M'malo mwake, zizindikiro zimathandizidwa pakufunika.

Zinthu zomwe mungachite zingaphatikizepo:

• Valani tsitsi ndi mano ovekera kuti musinthe mawonekedwe.
• Gwiritsani ntchito misozi yokumba kuti musawume m'maso.
• Gwiritsani ntchito mankhwala amphuno amchere kuchotsa zinyalala ndikupewa matenda.
• Tengani malo osambiramo madzi ozizira kapena mugwiritsire ntchito madzi opopera madzi kuti thupi lizizizira (madzi amatuluka pakhungu m'malo mwa thukuta lomwe limatuluka pakhungu.)

Izi zitha kukupatsirani zambiri pa ectodermal dysplasias:

• Ectodermal Dysplasia Society - edsociety.co.uk
• National Foundation ya Ectodermal Dysplasias - www.nfed.org
• NIH Genetic and Rare Diseases Information Center - rarediseases.info.nih.gov/diseases/6317/ectodermal-dysplasia

Ngati muli ndi mitundu yosiyanasiyana ya ectodermal dysplasia izi sizingachepetse moyo wanu. Komabe, mungafunikire kulabadira kusintha kwa kutentha ndi zovuta zina zomwe zimakhudzana ndi vutoli.

Ngati sanalandire chithandizo, mavuto azaumoyo atha kuphatikizidwa:

• Kuwonongeka kwa ubongo komwe kumachitika chifukwa cha kutentha kwa thupi
• Kugwidwa kumene kumachitika chifukwa cha kutentha thupi kwambiri (khunyu)

Itanani kuti mudzakumane ndi wokuthandizani ngati mwana wanu akuwonetsa zizindikiro za matendawa.

Ngati muli ndi mbiri yabanja ya ectodermal dysplasia ndipo mukukonzekera kukhala ndi ana, upangiri wamtunduwu umalimbikitsidwa. Nthawi zambiri, ndizotheka kuzindikira ectodermal dysplasia mwana akadali m'mimba.

Anhidrotic ectodermal dysplasia; Matenda a Christ-Siemens-Touraine; Anondontia; Incontinentia pigmenti

• Magawo akhungu

Abidi NY, Martin KL. Ectodermal dysplasias. Mu: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 668.

Narendran V. Khungu la akhanda. Mu: Martin RJ, Fanaroff AA, Walsh MC, olemba., Eds. Fanaroff ndi Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020: chap 94.