Humoral immune responses and CD27+ B cells in children with DiGeorge syndrome (22q11.2 deletion syndrome)

2006 ◽  
Vol 17 (5) ◽  
pp. 382-388 ◽  
Author(s):  
A. Finocchi ◽  
S. Di Cesare ◽  
M. L Romiti ◽  
C. Capponi ◽  
P. Rossi ◽  
...  
Keyword(s):  
B Cells ◽  
Immune Responses ◽  
Digeorge Syndrome ◽  
22Q11.2 Deletion Syndrome ◽  
Deletion Syndrome ◽  
22Q11.2 Deletion ◽  
Humoral Immune ◽  
Humoral Immune Responses

scholarly journals Clozapine-induced myocarditis in an adolescent male with DiGeorge Syndrome

2018 ◽  
Vol 8 (6) ◽  
pp. 313-316 ◽  
Author(s):  
Ann Marie Ruhe ◽  
Imran Qureshi ◽  
David Procaccini
Keyword(s):  
Digeorge Syndrome ◽  
Rare Complication ◽  
22Q11.2 Deletion Syndrome ◽  
Adolescent Male ◽  
Deletion Syndrome ◽  
22Q11.2 Deletion ◽  
Clozapine Therapy ◽  
Chromosomal Disorder ◽  
Congenital Heart Malformations ◽  
Pediatric Populations

Abstract DiGeorge Syndrome (22q11.2 deletion syndrome) is a chromosomal disorder associated with both congenital heart malformations and schizophrenia, which is often treatment-resistant and may warrant treatment with clozapine. Clozapine-induced myocarditis (CIM) is a rare complication of clozapine therapy, with a reported incidence ranging from 0.015% to 3%. Fulminant CIM has a nonspecific presentation in both adult and pediatric populations and a mortality rate approaching 50%. Few cases of pediatric CIM have been documented in the literature. This report highlights a case of CIM in an adolescent male with DiGeorge Syndrome whose clinical course was characterized by a subtle, nonspecific presentation and resolution with supportive care.

Chondroitin sulfate activates B cells in vitro, expands CD138+cells in vivo, and interferes with established humoral immune responses

2014 ◽  
Vol 96 (1) ◽  
pp. 65-72 ◽  
Author(s):  
Hilke Brühl ◽  
Josef Cihak ◽  
Nicole Goebel ◽  
Yvonne Talke ◽  
Kerstin Renner ◽  
...  
Keyword(s):  
B Cells ◽  
Immune Responses ◽  
Chondroitin Sulfate ◽  
Humoral Immune ◽  
Humoral Immune Responses

scholarly journals Unexpected combination: DiGeorge syndrome and myeloperoxidase deficiency

2020 ◽  
Vol 13 (2) ◽  
pp. e232741
Author(s):  
Simona Abraitytė ◽  
Elisabeth Kotsi ◽  
Lisa Anne Devlin ◽  
John David Moore Edgar
Keyword(s):  
T Cell ◽  
Digeorge Syndrome ◽  
Fungal Infections ◽  
Nicotinamide Adenine Dinucleotide Phosphate ◽  
22Q11.2 Deletion Syndrome ◽  
Deletion Syndrome ◽  
22Q11.2 Deletion ◽  
Cell Counts ◽  
Myeloperoxidase Deficiency ◽  
Rare Diagnosis

We report a case of a 3-year-old boy who presented with recurrent bacterial and fungal infections and a known diagnosis of partial DiGeorge (22q11.2 deletion) syndrome. The nature and severity of his infections were more than normally expected in partial DiGeorge syndrome with normal T-cell counts and T-cell proliferative response to phytohaemagglutinin. This prompted further investigation of the immune system. An abnormal neutrophil respiratory oxidative burst, but normal protein expression of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase system, led to the identification of myeloperoxidase deficiency. DiGeorge syndrome has a heterogeneous clinical phenotype and may not be an isolated diagnosis. It raises awareness of the possibility of two rare diseases occurring in a single patient and emphasises that even when a rare diagnosis is confirmed, if the clinical features remain atypical or unresponsive, then further investigation for additional cofactors is warranted.

scholarly journals Guidance of B Cells by the Orphan G Protein-Coupled Receptor EBI2 Shapes Humoral Immune Responses

Immunity ◽  
2009 ◽  
Vol 31 (2) ◽  
pp. 259-269 ◽  
Author(s):  
Dominique Gatto ◽  
Didrik Paus ◽  
Antony Basten ◽  
Charles R. Mackay ◽  
Robert Brink
Keyword(s):  
B Cells ◽  
Immune Responses ◽  
G Protein ◽  
G Protein Coupled Receptor ◽  
Humoral Immune ◽  
Humoral Immune Responses ◽  
G Protein Coupled
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