I-Ectodermal dysplasias

I-Ectodermal dysplasias liqela leemeko apho kukho ukukhula okungaqhelekanga kolusu, iinwele, iinzipho, amazinyo, okanye amadlala okubila.

Zininzi iintlobo ezahlukeneyo ze-ectodermal dysplasias. Uhlobo ngalunye lwe-dysplasia lubangelwa lutshintsho oluthile kuhlobo oluthile. I-Dysplasia ithetha ukukhula okungaqhelekanga kweeseli okanye izicubu. Eyona ndlela ixhaphakileyo ye-ectodermal dysplasia ihlala ichaphazela amadoda. Ezinye iintlobo zesifo zichaphazela ngokulinganayo amadoda nabafazi.

Abantu abane-ectodermal dysplasia abanakho ukubila okanye ukubila ngaphantsi kwesiqhelo ngenxa yokunqongophala kwamadlala okubila.

Kubantwana abanesi sifo, imizimba yabo inokuba nengxaki yokulawula umkhuhlane. Nokuba isigulo esincinci sinokuvelisa umkhuhlane omkhulu kakhulu, kuba ulusu alunakubila kwaye lulawule ubushushu ngokufanelekileyo.

Abantu abadala abachaphazelekayo abakwazi ukuyinyamezela imeko eshushu kwaye bafuna amanyathelo, anjengomoya womoya, ukugcina iqondo lobushushu lomzimba eliqhelekileyo.

Kuxhomekeka ekubeni zeziphi iintlobo zofuzo ezichaphazelekayo, ezinye iimpawu zinokubandakanya:

• Izikhonkwane ezingaqhelekanga
• Amazinyo angaqhelekanga okanye alahlekileyo, okanye angaphantsi kwenani eliqhelekileyo lamazinyo
• Umlomo ococekileyo
• Ukunciphisa umbala wesikhumba (i-pigment)
• Ibunzi elikhulu
• Ibhulorho ngeempumlo Low
• Izinwele ezincinci, ezinqabileyo
• Ukukhubazeka ekufundeni
• Ukungeva kakuhle
• Ukungaboni kakuhle ngokuhla kweenyembezi
• Amandla omzimba abuthathaka

Uvavanyo olunokwenziwa lunokubandakanya:

• I-Biopsy yenwebu yangaphakathi
• I-Biopsy yolusu
• Ukuvavanywa kwemfuza (kuyafumaneka kwezinye iintlobo zle ngxaki)
• IX-reyi yamazinyo okanye yamathambo inokwenziwa

Akukho lunyango oluthile kwesi sifo. Endaweni yoko, iimpawu ziphathwa xa kufuneka njalo.

Izinto onokuzenza zingabandakanya:

• Nxiba iwigi kunye namazinyo emboleko ukuze uphucule inkangeleko.
• Sebenzisa iinyembezi ezingezizo ukwenza ukuthintela ukomiswa kwamehlo.
• Sebenzisa i-saline impumlo yokutshiza ukususa i-debris kunye nokuthintela usulelo.
• Thatha iibhafu zamanzi okupholisa okanye usebenzise amanzi okutshiza ukugcina iqondo lobushushu lomzimba eliqhelekileyo (amanzi aphuma eluswini athabathe indawo yomsebenzi wokupholisa ukubila okuphuma eluswini.)

Ezi zixhobo zinokubonelela ngolwazi oluthe kratya kwi-ectodermal dysplasias:

• Umbutho we-Ectodermal Dysplasia-edsociety.co.uk
• Isiseko seSizwe se-Ectodermal Dysplasias- www.nfed.org
• IZiko loLwazi lweZifo zeMvelo zeNiH kunye neRare-rarediseases.info.nih.gov/diseases/6317/ectodermal-dysplasia

Ukuba unokwahluka okuqhelekileyo kwe-ectodermal dysplasia oku akuyi kunciphisa ixesha lokuphila. Nangona kunjalo, kusenokufuneka uhoye utshintsho lobushushu kunye nezinye iingxaki ezinxulumene nale meko.

Ukuba ayinyangwa, iingxaki zempilo ezivela kule meko zinokubandakanya:

• Umonakalo wobuchopho obangelwa kukunyuka kweqondo lobushushu emzimbeni
• Ukuxhuzula okubangelwa ngumkhuhlane omkhulu (ukuxhuzula okungathethekiyo)

Biza ixesha lokubonana nomboneleli wakho wezempilo ukuba umntwana wakho ubonakalisa iimpawu zesi sifo.

Ukuba unembali yosapho ye-ectodermal dysplasia kwaye ucwangcisa ukuba nabantwana, ukucebisa ngemfuza kuyacetyiswa. Kwiimeko ezininzi, kunokwenzeka ukuba uchaze i-ectodermal dysplasia ngelixa umntwana esesibelekweni.

I-anhidrotic ectodermal dysplasia; Isifo seChrist-Siemens-Touraine; Anondontia; Incontinentia pigmenti

• Uluhlu lolusu

UAbhidi NY, uMartin KL. I-Ectodermal dysplasias. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 668.

UNarendran V. Ulusu lweentsana. Ku: UMartin RJ, uFanaroff AA, uWalsh MC, ii-eds. UFanaroff kunye noMartin's Neonatal-Perinatal Medicine. Ngomhla we-11. IPhiladelphia, PA: Elsevier; 2020: isahl. 94.