Immunoglobulin A Deficiency
Serum IgA <7 mg/dL and a normal serum IgG and IgM in patients >4 years of age
- Exact pattern of inheritance remains unclear; however, the following associations may occur:
- 22q11 deletion syndrome
- 18q syndrome
- Partial deletions in the long or short arm and ring forms of chromosome 18
- Also associated with HLA-A1, HLA-A2, B8, DR3, DQ2 (8.1), and Dw3
- Also associated with non–MHC-associated genes involved in autoimmunity including IFIH1 on chromosome 2q24, CLEC16A on chromosome 16
- Failure of B lymphocyte differentiation into plasma cells producing IgA
- Block may be due to
- Defect in T-helper cells
- Antigen-presenting cells
- B cells
- Lack of effects from various cytokines, including IL-21, IL-4, IL-6, IL-7, or IL-10
Commonly Associated Conditions
Increased association with the following:
- Sinopulmonary infections
- GI infections (especially Giardia lamblia)
- Inflammatory bowel disease (Crohn disease and ulcerative colitis)
- Celiac disease
- Nodular lymphoid hyperplasia
- Autoimmune illnesses
- Systemic lupus erythematosus
- Immune endocrinopathies (e.g., Graves disease, type 1 diabetes)
- Autoimmune hematologic conditions
- Chronic active hepatitis
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Cabana, Michael D., editor. "Immunoglobulin a Deficiency." Select 5-Minute Pediatrics Topics, 7th ed., Wolters Kluwer Health, 2015. Medicine Central, im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency.
Immunoglobulin A Deficiency. In: Cabana MDM, ed. Select 5-Minute Pediatrics Topics. Wolters Kluwer Health; 2015. https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency. Accessed June 4, 2023.
Immunoglobulin A Deficiency. (2015). In Cabana, M. D. (Ed.), Select 5-Minute Pediatrics Topics (7th ed.). Wolters Kluwer Health. https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency
Immunoglobulin a Deficiency [Internet]. In: Cabana MDM, editors. Select 5-Minute Pediatrics Topics. Wolters Kluwer Health; 2015. [cited 2023 June 04]. Available from: https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Immunoglobulin A Deficiency ID - 14162 ED - Cabana,Michael D, BT - Select 5-Minute Pediatrics Topics UR - https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency PB - Wolters Kluwer Health ET - 7 DB - Medicine Central DP - Unbound Medicine ER -