Clinical and immunological features in a cohort of patients with partial DiGeorge syndrome followed at a single center.
Journal
Blood
ISSN: 1528-0020
Titre abrégé: Blood
Pays: United States
ID NLM: 7603509
Informations de publication
Date de publication:
13 06 2019
13 06 2019
Historique:
received:
08
11
2018
accepted:
03
04
2019
pubmed:
25
4
2019
medline:
10
1
2020
entrez:
25
4
2019
Statut:
ppublish
Résumé
DiGeorge syndrome (DGS) is a primary immunodeficiency characterized by various degrees of T-cell deficiency. In partial DGS (pDGS), other risk factors could predispose to recurrent infections, autoimmunity, and allergy. The aim of this study was to assess the effect of different factors in the development of infections, autoimmunity, and/or allergy in patients with pDGS. We studied 467 pDGS patients in follow-up at Great Ormond Street Hospital. Using a multivariate approach, we observed that palatal anomalies represent a risk factor for the development of recurrent otitis media with effusion. Gastroesophageal reflux/dysphagia and asthma/rhinitis represent a risk factor for the development of recurrent upper respiratory tract infections. Allergy and autoimmunity were associated with persistently low immunoglobulin M levels and lymphopenia, respectively. Patients with autoimmunity showed lower levels of CD3
Identifiants
pubmed: 31015189
pii: S0006-4971(20)42489-9
doi: 10.1182/blood.2018885244
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
2586-2596Informations de copyright
© 2019 by The American Society of Hematology.