Primary immunodeficiency in the neonate: Early diagnosis and management

Seminars in Fetal & Neonatal Medicine
Kelly Walkovich, James A Connelly

Abstract

Many primary immunodeficiencies (PIDs) manifest in the neonatal period but can be challenging to diagnose and manage optimally. In part, the difficulty stems from the natural immaturity of the neonatal immune system that may mask immune deficits and/or complicate interpretation of clinical findings and laboratory assays. The great diversity of PIDs--from innate immune system defects to those that impact the humoral and/or cellular components of the adaptive immune system--and the rapid rate at which new PIDs are being discovered makes it challenging for practitioners to stay current. Moreover, recent appreciation for immune deficiencies that lead to autoinflammation and autoimmunity have broadened the spectrum of neonatal PID, adding additional complexity to an already intricate field. This article serves to highlight the deficiencies in the neonatal immune system, while providing a review of the more common PIDs that present in the neonate and guidelines for diagnosis and supportive care.

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Citations

Dec 19, 2019·Acta Microbiologica Et Immunologica Hungarica·Elham RayzanNima Rezaei
Jan 15, 2020·Human Genetics·Alessandro BorghesiJacques Fellay
Jul 2, 2019·NeoReviews·Thomas F MichniackiElizabeth Secord
Jul 2, 2019·NeoReviews·Amy E O'Connell
Jan 13, 2017·Orphanet Journal of Rare Diseases·Ioanna A Rota, Fatima Dhalla
Jun 27, 2019·Biochemistry. Biokhimii︠a︡·N R KhasbiullinaN V Bovin
Feb 23, 2020·Frontiers in Immunology·Zeinab A El-Sayed, Nesrine Radwan
May 14, 2021·Frontiers in Immunology·Jingyan ZhangHuub F J Savelkoul

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