Abdollahzade S, Aghamohammadi A, Soheili H, Salehi Sadaghiani M, Abolhassani H, Rezaei N,
Volume 68, Issue 10 (1-2011)
Abstract
Background: Common Variable Immunodeficiency (CVID)
is a primary immunodeficiency disease, characterized by hypogammaglobulinemia
and heterogeneous clinical manifestations. This study was performed to evaluate
the clinical and immunological features of pediatric patients with CVID.
Methods: We reviewed the records of 69 children diagnosed
under age of 16 years with CVID
(35 males and 34
females).
Results: By the year 2008, 15 patients (21%)
had died. The total follow-up period was 333
patient-years. The mean diagnostic time between onset and diagnosis in our
patient group was 4.40 years. The overall
rate of consanguineous marriages was 58%.
10 patients had a positive family history of
immunodeficiency. At the time of diagnosis, the mean levels of serum
immunoglobulin G (IgG),
IgM,
and IgA levels
were 286.86, 39.92, and 18.39
mg/dl, respectively which were below the normal levels for age. All of the
patients presented with infectious diseases at the time of onset, the most
common of which were pneumonia, diarrhea and sinusitis. Acute and recurrent
infections were also found in almost all of the patients, particularly
involving respiratory and gastrointestinal systems. The most common infections
during follow-up period were pneumonia (31.9%),
acute diarrhea (18.8%), acute sinusitis (18.8%),
and otitis media (14.5%). Post-diagnosis
survival was estimated to be 79% during the first
five years. The survival rate was not shown to be influenced by delayed
diagnosis, serum levels of IgG
and B-lymphocyte count at the time of diagnosis.
Conclusions: Any child with a history of recurrent infections, decreased levels of serum
immunoglobulin isotypes and consanguineous parents should be considered as a CVID
patient.
