Yintoni i-SCID (i-Severe Combined Immunodeficiency Syndrome)

Umxholo

• Izizathu ezinokubangela
• Ziintoni iimpawu
• Yintoni isifo
• Unyango lwenziwa njani

I-Severe Combined Immunodeficiency Syndrome (i-SCID) ibandakanya uluhlu lwezifo ezikhoyo ukusukela ekuzalweni, ezibonakaliswa lutshintsho kwinkqubo yamajoni omzimba, apho izilwa-buhlungu zikumanqanaba asezantsi kwaye ii-lymphocyte ziphantsi okanye azikho, zisenza umzimba ungakwazi ukuzikhusela kwizifo, ukubeka umntwana emngciphekweni, kwaye kungakhokelela nasekufeni.

Ezona mpawu zixhaphakileyo zesi sifo zibangelwa zizifo ezosulelayo kwaye unyango olusinyanga esi sifo lubandakanya ukufakelwa umongo wethambo.

Izizathu ezinokubangela

I-SCID isetyenziselwa ukuhlelwa kweseti yezifo ezinokubangelwa ziziphene zemfuza ezinxulunyaniswe ne-X chromosome kunye nokusilela kwe-enzyme ye-ADA.

Ziintoni iimpawu

Iimpawu ze-SCID zihlala zibonakala kunyaka wokuqala wobomi kwaye zinokubandakanya izifo ezosulelayo ezingaphenduliyo kunyango olunje nge-pneumonia, meningitis okanye sepsis, ekunzima ukuzinyanga kwaye zingaphenduli ngokusetyenziswa kwamayeza, kunye nosulelo lolusu, usulelo lomngundo emlonyeni nakwindawo ye-diaper, isifo sorhudo kunye nokusuleleka kwesibindi.

Yintoni isifo

Ukuxilongwa kwenziwa xa umntwana esiba nezifo eziphindaphindayo, ezingasonjululwanga ngonyango. Njengokuba esi sifo sililifa, ukuba naliphi na ilungu losapho linesi sifo, ugqirha uya kuba nakho ukusiqonda esi sifo kwangoko xa umntwana sele ezelwe, esenza uvavanyo lwegazi ukuvavanya amanqanaba ee-antibodies kunye neeseli ze-T .

Unyango lwenziwa njani

Olona nyango lusebenzayo lwe-SCID kukukhutshelwa kweeseli zomongo wethambo ukusuka kumnikeli ophilileyo nohambelanayo, othi kwiimeko ezininzi usinyange esi sifo.

Kude kube kufunyanwe umnikeli ofanelekileyo, unyango luqulathe ukusombulula usulelo kunye nokuthintela usulelo olutsha ngokuhlukanisa umntwana ukuthintela ukunxibelelana nabanye abanokuba sisiseko sokwasulelwa zizifo.

Umntwana unokuphinda alungiswe ukulungiswa kwe-immunodeficiency correction ngokutshintshwa kwe-immunoglobulin, okufuneka ukuba ilawulwe kuphela kubantwana abangaphezu kweenyanga ezi-3 kunye / okanye abaye bafumana izifo.

Kwimeko yabantwana abane-SCID ebangelwa kukusilela kwe-enzyme ye-ADA, ugqirha unokucebisa nge-enzyme yonyango yokutshintsha, kunye nokusetyenziswa kwe-ADA esebenzayo ngeveki, enikezela ukumiliselwa kwakhona kwamajoni omzimba malunga neenyanga ezi-2 ukuya kwezi-4 emva kokuqala konyango .

Ukongeza, kubalulekile ukukhankanya ukuba izitofu ezineentsholongwane eziphilayo okanye ezifunyanisiweyo akufuneki zinikwe aba bantwana, de ugqirha ayalele ngenye indlela.