Nesrin GÜLEZ, Hasan AĞIN, Şebnem ÇALKAVUR, Yeşim ERGÜDENLER, Ferah GENEL, Mustafa BAK

AĞIR KOMBİNE İMMÜN YETMEZLİK: OLGU SUNUMU

Anahtar Kelimeler:

Ağır kombine immün yetmezlik, solunum yolu enfeksiyonu, mantar enfeksiyonu

A Case Of Severe Combined Immunodeficiency

Severe combined immunodeficiency (SCID) is a heterogeneous group of inherited disorders characterized byprofound abnormalities in T, B and natural killer cell development and function, resulting in failure of bothcellular and humoral immunity. The age of presentation is variable but occurs typically between 3 and 6 months.The major clinical manifestations include recurrent diarrhea, persistant oral candidiasis, pneumonia, recurrentotitis media, cutaneous fungal infections and sepsis. Opportunistic infection induced end organparticularly to the lungs and liver, is associated with greatly increased morbidity and mortality. Therefore SCIDhas been described as a pediatric emergency.In this article a 5-month-old female infant admitted with the complaint of recurrent respiratory infectionsand disseminated fungal infections and diagnosed as severe combined immunodeficiency (SCID) ispresented

Keywords:

Severe combined immunodeficiency, respiratory infection, fungal infection,

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