Immunoglobulin deficiencies: the B-lymphocyte side of DiGeorge Syndrome.
DiGeorge syndrome is associated with a T-lymphocyte immunodeficiency. The prevalence of hypogammaglobulinemia has not been reported. We found that 3% of patients with DiGeorge syndrome were receiving immunoglobulin replacement therapy and 6% of patients over the age of 3 years had hypogammaglobulinemia. We conclude that DiGeorge syndrome is associated with significant humoral immune deficiency.
Division of Allergy Immunology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Kiran
K
Patel
Javeed
J
Akhter
Lisa
L
Kobrynski
M A
MA
Benjamin Gathmann
Benjamin
B
Gathman
Onika
O
Davis
Kathleen E
KE
Sullivan
D000293:Adolescent; D000328:Adult; D001402:B-Lymphocytes / Q000166:cytology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004062:DiGeorge Syndrome / Q000175:diagnosis* / Q000235:genetics; D005060:Europe; D006801:Humans; D017098:IgA Deficiency / Q000097:blood / Q000175:diagnosis*; D007070:Immunoglobulin A / Q000378:metabolism; D007074:Immunoglobulin G / Q000378:metabolism; D007075:Immunoglobulin M / Q000378:metabolism; D007136:Immunoglobulins / Q000378:metabolism; D007223:Infant; D007231:Infant, Newborn; D008875:Middle Aged; D015995:Prevalence; D012042:Registries; D013601:T-Lymphocytes / Q000166:cytology; D014481:United States
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007136:Immunoglobulins