Clinical updates in inborn errors of immunity: a focus on the noninfectious clinical manifestations.
Curr Opin Pediatr
; 36(2): 228-236, 2024 04 01.
Article
em En
| MEDLINE
| ID: mdl-38299990
ABSTRACT
PURPOSE OF REVIEW In the last 5 years, several new inborn errors of immunity (IEI) have been described, especially in the areas of immune dysregulation and autoinflammation. As a result, the clinical presentation of IEIs has broadened. We review the heterogeneous presentation of IEIs and detail several of the recently described IEIs with a focus on the noninfectious manifestations commonly seen. RECENT FINDINGS:
IEIs may present with early onset and/or multiple autoimmune manifestations, increased risk for malignancy, lymphoproliferation, severe atopy, autoinflammation and/or hyperinflammation. Because of this, patients can present to a wide array of providers ranging from primary care to various pediatric subspecialists. The International Union of Immunological Societies (IUIS) expert committee has created a phenotypic classification of IEIs in order to help clinicians narrow their evaluation based on the laboratory and clinical findings.SUMMARY:
Both primary care pediatricians and pediatric subspecialists need to be aware of the common clinical features associated with IEI and recognize when to refer to allergy-immunology for further evaluation. Early diagnosis can lead to earlier treatment initiation and improve clinical outcomes for our patients.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Cognição
/
Pediatras
Tipo de estudo:
Screening_studies
Limite:
Child
/
Humans
Idioma:
En
Revista:
Curr Opin Pediatr
Assunto da revista:
PEDIATRIA
Ano de publicação:
2024
Tipo de documento:
Article