Isifo seWiskott-Aldrich

Umxholo

• Iimpawu zeWiskott-Aldrich Syndrome
• Unyango lweWiskott-Aldrich Syndrome

I-Wiskott-Aldrich syndrome sisifo semfuza, esibeka esichengeni amajoni omzimba abandakanya ii-lymphocyte ze-T kunye ne-B, kunye neeseli zegazi ezinceda ukulawula ukopha, iiplatelets.

Iimpawu zeWiskott-Aldrich Syndrome

Iimpawu ze-wiskott-Aldrich syndrome zinokuba:

Utyekelo lokopha:

• Inani elincitshisiweyo kunye nobungakanani beeplatelets egazini;
• Ukopha okukhawulezileyo okuphawulwa ngamachaphaza abomvu-luhlaza okwesibhakabhaka ubukhulu bentloko yepini, ebizwa ngokuba yi "petechiae", okanye zinokuba nkulu kwaye zifane nemivumbo;
• Izitulo ezinegazi (ngakumbi ebuntwaneni), iintsini ezophayo kunye neempumlo ezinde zeempumlo.

Izifo ezenzeka rhoqo ezibangelwa zizo zonke iintlobo zeentsholongwane ezinje:

• Otitis media, sinusitis, inyumoniya;
• I-Meningitis, inyumoniya ebangelwa yi-Pneumocystis jiroveci;
• Usulelo lwegciwane lesifo esibangelwa yi-molluscum contagiosum.

Onokhwekhwe:

• Izifo ezenzeka rhoqo kwesikhumba;
• Amabala amnyama kulusu.

Ukubonakaliswa okuzenzekelayo:

• IVasculitis;
• I-hemolytic anemia;
• Idiopathic thrombocytopenic purpura.

Ukuchongwa kwesi sifo kunokwenziwa ngugqirha wabantwana emva kokubona iimpawu kunye novavanyo oluthile. Ukuvavanya ubungakanani beeplatelets yenye yeendlela zokufumanisa isifo, njengoko zimbalwa izifo ezinolu phawu.

Unyango lweWiskott-Aldrich Syndrome

Olona nyango lufanelekileyo lweWiskott-Aldrich syndrome kukufakwa komongo wethambo. Ezinye iindlela zonyango kukususa udakada, njengoko eli lungu litshabalalisa inani elincinci leeplatelets ezinabantu abanesi sifo, ukusetyenziswa kwehemoglobin kunye nokusetyenziswa kweyeza lokubulala iintsholongwane.

Ubomi obulindelweyo kubantu abanale syndrome buphantsi, abo basindayo emva kweminyaka elishumi badla ngokuvela amathumba afana ne-lymphoma kunye neleukemia.