Ectodermal dysplasias

Ectodermal dysplasias yog ib pawg ntawm kev mob uas muaj kev loj hlob tsis zoo ntawm daim tawv nqaij, plaub hau, rau tes, hniav, lossis qog ua haujlwm.

Muaj ntau ntau yam ectodermal dysplasias. Txhua yam mob ntawm cov dysplasia yog tshwm sim los ntawm kev hloov pauv hauv qee cov noob. Dysplasia txhais tau tias yog kev loj hlob ntawm cov hlwb lossis cov ntaub so ntswg. Qhov feem ntau ua rau ectodermal dysplasia feem ntau cuam tshuam rau cov txiv neej. Lwm hom kab mob cuam tshuam rau tus txiv neej thiab poj niam sib npaug.

Cov neeg uas mob ectodermal dysplasia yuav tsis tawm hws lossis hws tsawg dua qhov qub vim tias tsis muaj cov qog ua hws.

Hauv cov menyuam yaus uas muaj tus kabmob, lawv lub cev yuav muaj teeb meem tswj kev ua npaws. Txawm hais tias mob mentsis tuaj yeem tsim kev kub taub hau heev, vim tias daim tawv nqaij yuav tsis tawm hws thiab tswj tsis tau qhov kub thiab txias.

Cov neeg laus uas muaj kev cuam tshuam tsis tuaj yeem tiv taus qhov chaw sov thiab xav tau kev ntsuas, xws li cua txias, ua kom lub cev tsis kub tsis txias.

Nyob ntawm seb cov noob twg cuam tshuam, lwm cov tsos mob tuaj yeem suav nrog:

• Txawv ntsia hlau
• Txawv lossis ploj ntawm cov hniav, lossis tsawg dua tus naj npawb ntawm cov hniav
• Cem di ncauj
• Txo cov tawv nqaij xim (xim)
• Cov hauv pliaj loj
• Qes qhov ntswg qis
• Nyias, cov plaub hau sib zog
• Kev kawm tsis taus
• Lub rooj sib hais tsis zoo
• Lub zeem muag tsis zoo nrog kev tsim kua muag poob
• Lub cev tsis muaj zog tiv thaiv kab mob

Kev ntsuam xyuas uas yuav ua tau muaj xws li:

• Txau cov nqaij mos ntawm lub cev mos
• Biopsy ntawm daim tawv nqaij
• Kev tshuaj ntsuam caj ces (muaj rau qee yam kev tsis txaus siab no)
• Kev xoo hluav taws xob ntawm cov hniav lossis pob txha yuav ua tau

Tsis muaj kev kho tshwj xeeb rau yam kev mob no. Hloov chaw, cov tsos mob tau kho raws li xav tau.

Tej yam koj tuaj yeem ua muaj xws li:

• Hnav cov wig thiab cov hniav cuav txhawm rau txhim kho qhov ua kom pom kev zoo.
• Siv cov kua muag cuav tiv thaiv kom tsis txhob qhuav ntawm lub qhov muag.
• Siv cov tshuaj ua ntsev saline rau kev tshem tawm cov khib nyiab thiab tiv thaiv kev kis tus kab mob.
• Siv dej txias los da dej lossis siv tshuaj txau dej kom lub cev kub cev kom zoo li qub (dej tso tawm ntawm daim tawv nqaij los hloov lub txias ua haujlwm ntawm hws yaj tawm ntawm daim tawv.)

Cov peev txheej no tuaj yeem muab cov ntaub ntawv qhia ntxiv ntawm ectodermal dysplasias:

• Ectodermal Dysplasia Society - edsociety.co.uk
• Teb Chaws Tuam Tsev rau Ectodermal Dysplasias - www.nfed.org
• NIH Chaw Qhia Txog Kab Mob thiab Tsis Muaj Kab Mob - rarediseases.info.nih.gov/diseases/6317/ectodermal-dysplasia

Yog tias koj muaj qhov txawv txav ntawm ectodermal dysplasia qhov no yuav tsis ua rau koj lub neej luv luv. Txawm li cas los xij, koj yuav tsum them sai sai rau kev hloov pauv kub thiab lwm yam teeb meem cuam tshuam nrog tus mob no.

Yog tsis kho, kev mob los ntawm tus mob no yuav suav nrog:

• Lub paj hlwb raug mob los ntawm kev ua haujlwm kub hauv lub cev
• Qaug dab peg tshwm sim los ntawm kub taub hau (febrile qaug dab peg)

Hu teem sijhawm nrog koj tus kws khomob yog koj tus menyuam muaj tsos mob ntawm qhov tsis zoo no.

Yog tias koj muaj tsev neeg keeb kwm ntawm mob txha caj qaum thiab koj tab tom npaj yuav muaj menyuam, kev tawm tswv yim caj ces tau pom zoo. Feem ntau, nws muaj peev xwm txheeb xyuas tau ectodermal dysplasia thaum tus menyuam tseem nyob hauv niam plab.

Tshuaj Mob Ntshav Quav Hnav Qog Khoom; Tswv Yexus-Siemens-Touraine syndrome; Anondontia; Kev tsis txaus ntseeg pigmenti

• Tawv khaubncaws sab nraud povtseg

Abidi NY, Martin KL. Ectodermal dysplasias. 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 668.

Narendran V. Cov tawv nqaij ntawm cov leeg (neonate). Hauv: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff thiab Martin's Neonatal-Perinatal TshuajCov. 11th ed. Philadelphia, PA: Elsevier; 2020: cha 94.