Common variable immunodeficiency in children
- Mary Beth Hogan, MD
Mary Beth Hogan, MD
- Vice Chair of Pediatric Research
- Professor of Pediatrics
- University of Nevada, Las Vegas School of Medicine
- Nevin W Wilson, MD
Nevin W Wilson, MD
- Professor and Chairman of Pediatrics
- University of Nevada, Las Vegas School of Medicine
Common variable immunodeficiency (CVID) is a heterogeneous disorder involving immune dysfunction of B and T cells and dendritic cells. The characteristic immunologic defect is the inability of B cells to differentiate into plasma cells capable of secreting all immunoglobulin types. While CVID is often regarded as a disease of adults, it does affect children, and diagnosis in young children can be challenging.
Clinical features of CVID and issues in the diagnosis and management of this disorder that are specific to pediatric patients (less than 18 years of age) will be reviewed here. Additional reviews of CVID, which emphasize adult manifestations and management, are found separately. (See "Clinical manifestations, epidemiology, and diagnosis of common variable immunodeficiency in adults" and "Pathogenesis of common variable immunodeficiency" and "Treatment and prognosis of common variable immunodeficiency".)
DEFINITION AND CLASSIFICATION
Definition — CVID is defined by the following :
●Age-specific reduction in serum concentrations of immunoglobulin G, in combination with low levels of immunoglobulin A and/or immunoglobulin M
●Presence of B cells
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- DEFINITION AND CLASSIFICATION
- CLINICAL MANIFESTATIONS
- Recurrent infections
- Pulmonary manifestations
- Allergic diseases
- Gastrointestinal problems
- Autoimmune disease
- - Evans syndrome
- Indications for referral
- Immunoglobulin levels
- Antibody function studies
- Other testing at the time of diagnosis
- - B cell studies
- - T cell studies
- - Genetic studies
- - Endocrine studies
- Diagnostic uncertainties
- - Periodic reassessment
- DIFFERENTIAL DIAGNOSIS
- Primary hypogammaglobulinemia
- - Agammaglobulinemia
- - Transient hypogammaglobulinemia of infancy
- Specific antibody deficiency
- IgG subclass deficiency
- Hyperimmunoglobulin M syndromes
- X-linked lymphoproliferative disease
- Combined immunodeficiency disorders
- Immune globulin
- Evaluation of illnesses
- Monitoring pulmonary status
- Autoimmune cytopenias
- Management of allergic disease
- Psychosocial and educational issues
- General supportive care
- SOCIETY GUIDELINE LINKS