[Clinical and therapeutic features in patients with common variable immunodeficiency from two third-level care
Raul Dorbeker-Azcona1, Marìa Dolores Mogica-Martìnez, Martìn Becerril-Ngeles
1Unidad de InvestigaciÛn en Inmunodeficiencias, Instituto Nacional de PediatrÌa, MÈxico D.F.
Background:
Common variable immunodeficiency (CVID) is one of the most common antibody deficiencies, is characterized by low serum immunoglobulins, defective antibody response and increased susceptibility to chronic and recurrent infections.
Objective:
we present the clinical findings of patients with CVID in two hospitals in Mexico City.
Methods:
We performed a retrospective study of patients who filled CVID criteria. We collected the following information, demographic data, age at onset, age at diagnosis, family history, infection, autoimmunity, lymphoproliferative disease, allergy, malignancy, immunoglobulin levels at diagnosis, route of administration, dosage and frequency of IVIG of each patient. Data were analyzed with descriptive statistics.
Results:
Amongst 26 patients who filled CVID criteria, 14 were men and 12 women. The mean diagnosis delay was 48 months (22-128), serum immunoglobulins at diagnosis in mg/dL were IgG 216 (114-316), IgM 21 (12-121), IgA 21 (6-26) and IgE 4.6 (1.8) IU/mL. 81% of patients suffered pneumonia. There was a decrease in the number of pneumonias before and after treatment with gammaglobulin (p = 0.028). 27% of the patients had autoimmune diseases, 35% allergies, 35% chronic diarrhea, 62% bronchiectasis, 73% chronic cough, 50% lymphadenopathy. One patient had lymphoproliferative disease and none developed malignancy.
Conclusions:
We found that the delay in the diagnosis and initiation of gammaglobulin replacement affects the occurrence of complications such as bronchiectasis.
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