Primary immunodeficiency diseases in the newborn

The normal neonate’s immune system is anatomically completed but antigenically inexperienced and shows somewhat decreased role of a number of immunological pathways. Aside from anatomic characteristics (e.g., thin skin and mucosal barriers) of newborn, weakened pro-inflammatory and T-helper cell type 1 cytokine release and lessened cell-mediated immunity predispose the neonate more susceptible to all types of infections. Furthermore, many types of primary immunodeficiency diseases (PIDs) that present in neonatal period are potentially life threatening. However, most of the newborns stand this period without sickness due to complete innate immunity with other adaptive immune system mechanisms and transferred maternal immunoglobulin G. Besides unique immunity of the preterm and normal newborns; risk factors, clinical features, and laboratory evaluation of most common PIDs in newborn are told in this article. The range of PIDs is growing, and the diagnosis and management of these disorders continues to increase in complexity. The most common PID types of the newborn including antibody deficiencies, cellular/combined immunodeficiencies, phagocytic diseases, complement deficiencies, and innate immune system and other disorders are briefly mentioned here as well.

Yenidoðanda Primer Ýmmün Yetmezlik Hastalýklarý

Normal yenidoðanýn immün sistemi anatomik olarak eksik olmamakla beraber antijenik olarak deneyimsizdir ve bazý immünolojik yolaklarýn rolünde bir miktar azalma gösterir. Yenidoðanýn anatomik (ince cilt ve mukozal bariyerler gibi) özellikleri dýþýnda, zayýflamýþ proinflamatuvar ve yardýmcý T hücre tip 1 (Th1) sitokin salýnýmý ve azalmýþ hücre-aracýlý immünite yenidoðaný tüm enfeksiyon tiplerine daha hassas kýlar. Yine yenidoðan döneminde görülen birçok primer immün yetmezlik hastalýk (PÝYH) tipleri potansiyel olarak hayatý tehdit edicidir. Buna raðmen, yenidoðanlarýn çoðu bu dönemi doðal ve diðer adaptif immün sistem mekanizmalarý ve transferle anneden geçen immunoglobulin G ile bereber hastalýk geçirmeden atlatýr. Bu makalede pretem ve normal yenidoðanýn benzersiz baðýþýklýk sistemi yanýnda, en sýk rastlanan PÝYH’nýn risk faktörleri, klinik özellikleri ve laboratuvar deðerlendirilmesi anlatýlacaktýr. PÝYH’nýn kapsamý büyümekte ve bu hastalýklarýn teþhis ve bakýmýndaki karýþýklýk artmaya devam etmektedir. Antikor eksiklikleri, hücresel/kombine immün yetmezlikler, fagositik hastalýklar, komplement eksiklikleri ve innat (doðal) immün sistem ve diðer bozukluklarýný içeren yenidoðanýn en sýk görülen PÝYH tiplerinden burada özetle bahsedilmektedir. (NCI-2020-0211.R1)