Yini i-SCID (Severe Combined Immunodeficiency Syndrome)

-Delile
I-Severe Combined Immunodeficiency Syndrome (i-SCID) ifaka phakathi iqoqo lezifo ezikhona kusukela wazalwa, ezibonakala ngokushintsha kwesimiso somzimba sokuzivikela ezifweni, lapho amasosha omzimba esemazingeni aphansi nama-lymphocyte aphansi noma engekho, okwenza umzimba ungakwazi ukuvikela ezifweni, ukubeka ingane engcupheni, futhi kungaholela nasekufeni.
Izimpawu ezivame kakhulu zalesi sifo zibangelwa izifo ezithathelwanayo kanti ukwelashwa okwelapha lesi sifo kuqukethe ukufakelwa umnkantsha wethambo.

Izimbangela ezingaba khona
I-SCID isetshenziselwa ukuhlukanisa iqoqo lezifo ezingabangelwa ukukhubazeka kwezakhi zofuzo okuxhunyaniswe ne-X chromosome kanye nokushoda kwe-enzyme ye-ADA.
Ziyini izimpawu
Izimpawu ze-SCID zivame ukuvela ngonyaka wokuqala wokuphila futhi zingafaka izifo ezithathelwanayo ezingaphenduli ekwelashweni okufana nenyumoniya, i-meningitis noma i-sepsis, okunzima ukuselapha futhi ngokuvamile akuphenduli ekusetshenzisweni kwemithi, nokutheleleka kwesikhumba, izifo zokukhunta emlonyeni nasendaweni enabukeni, isifo sohudo kanye nokutheleleka kwesibindi.
Yini ukuxilongwa
Ukuxilongwa kwenziwa lapho ingane ihlushwa izifo eziphindaphindayo, ezingaxazululwa ngokwelashwa. Njengoba lesi sifo siyifa, uma noma yiliphi ilungu lomndeni linalesi sifo, udokotela uzokwazi ukuxilonga lesi sifo ngokushesha nje lapho umntwana ezalwa, okuqukethe ukwenza ukuhlolwa kwegazi ukuhlola amazinga ama-antibodies nama-T cell .
Yelashwa kanjani
Ukwelashwa okuphumelela kakhulu kwe-SCID ukufakelwa kwamangqamuzana emithambo yomnkantsha kusuka kumnikeli onempilo futhi ohambisanayo, okuvame ukwelapha lesi sifo.
Kuze kutholakale umnikeli ohambisanayo, ukwelashwa kuqukethe ukuxazulula ukutheleleka nokuvikela ukutheleleka okusha ngokuhlukanisa ingane ukugwema ukuxhumana nabanye abangaba ngumthombo wezifo ezithathelwanayo.
Ingane nayo ingalungiswa ngokulungiswa kwe-immunodeficiency ngokusebenzisa i-immunoglobulin esikhundleni, okumele inikezwe kuphela izingane ezineminyaka engaphezu kwezinyanga ezintathu kanye / noma esivele zithole izifo.
Endabeni yezingane ezine-SCID ezibangelwa ukuntuleka kwe-enzyme ye-ADA, udokotela angancoma ukwelashwa esikhundleni se-enzyme, ngokusetshenziswa kwamasonto onke kwe-ADA esebenzayo, enikeza ukwakhiwa kabusha kwesistimu yomzimba cishe ezinyangeni ezi-2-4 ngemuva kokuqala kokwelashwa .
Ngaphezu kwalokho, kubalulekile ukusho ukuthi imigomo enamagciwane aphilayo noma ancishisiwe akufanele inikezwe lezi zingane, kuze kube yilapho udokotela eyalela okunye.