[Common variable immunodeficiency in children].
Common variable immunodeficiency (CVID) is one of the more frequent primary immunodeficiencies (PID), after IgA deficiency, and affects a heterogeneous group of patients of various ages and with autosomal recessive inheritance. Our objective is to present the group of children diagnosed with CVID treated in our Hospital Infantil Vall d'Hebron and comment on the diagnostic problems that can arise. Sixteen boys and girls were diagnosed between the ages of 7 months and 15 years. The diagnosis is based on low immunoglobulins and a clinical picture of infection. Differential diagnosis in the paediatric age must consider mainly other PIDs: transient hypogammaglobulinaemia of infancy, X chromosome-linked agammaglobulinaemia (XLA), X chromosome-linked hyper IgM syndrome (X-HIM), IgG subclass deficiency and IgA deficiency (IgAD). Other processes that evolve with recurrent respiratory infections, such as cystic fibrosis, must also be discarded.


CONCLUSIONS
These patients present a high incidence of respiratory infections and bronchiectasias. We also observe associated allergic and autoimmune processes. Early diagnosis is indispensable to initiate suitable treatment and avoid the consequences of both respiratory and digestive infections.
Llobet
M P
MP
Hospital Vall d'Hebron. Unidades de Inmunodeficiencias e Inmunología. Barcelona. Spain.
Bertrán
J M
JM
Español
T
T
D000293:Adolescent; D000361:Agammaglobulinemia / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000175:diagnosis* / Q000453:epidemiology; D003550:Cystic Fibrosis / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D017098:IgA Deficiency / Q000175:diagnosis; D017099:IgG Deficiency / Q000175:diagnosis; D007223:Infant; D008297:Male; D012141:Respiratory Tract Infections / Q000175:diagnosis; D013030:Spain