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Primary immunodeficiency diseases
Published in Gabriel Virella, Medical Immunology, 2019
John W. Sleasman, Gabriel Virella
Transient hypogammaglobulinemia of infancy is a disorder that is an accentuation of the normal physiologic decline in passively acquired maternal IgG. Normally, passively acquired maternal IgG is catabolized in a newborn infant with a half-life of approximately 1 month, but the infant's production is delayed, failing to maintain adequate IgG levels. As a result, IgG nadirs occur between 3 and 6 months of age. This process is more pronounced in preterm infants whose IgG levels are low at birth, as the transport of IgG across the placenta is greatest during the third trimester of gestation. If the infant's IgG nadir drops below two standard deviations for age, the criteria for transient hypogammaglobulinemia of infancy is fulfilled. Most of these patients most often are evaluated if they have increased frequency and/or severity of infections. Differentiation from more severe forms of humoral immunodeficiency is based on normal Ig function since these infants have normal response to immunization and normal B-cell enumeration.
Immunology (primary Immunodeficiency Syndromes
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Stephan Strobel, Alison M. Jones
International criteria for diagnosis of CVID include: Patient over the age of 2 years (to distinguish from transient hypogammaglobulinemia of infancy).Hypogammaglobulinaemia – low levels of at least two of three major immunoglobulin isotypes.Poor specific antibody responses to vaccine antigens.Exclusion of other defined genetic defects, e.g.X-linked agammaglobulinaemia (BTK deficiency) or hyper-IgM syndrome (CD40 ligand deficiency).Lymphocyte subpopulations are frequently normal, but there may be low T-cells and/or low B-cells. There may also be abnormalities of memory B-cell populations.Causes of secondary hypogammaglobulinaemia must be excluded (e.g. drugs and protein-losing states).
The Prevalence of Selective and Partial Immunoglobulin A Deficiency in Patients with Autoimmune Polyendocrinopathy
Published in Immunological Investigations, 2022
Mahnaz Jamee, Mohammad Reza Alaei, Mehrnaz Mesdaghi, Shahab Noorian, Mehdi Moosavian, Elahe Dolatshahi, Habibeh Taghavi Kojidi, Zahra Chavoshzadeh, Mazdak Fallahi, Samaneh Parviz, Fatemeh Aghamahdi, Gholamreza Azizi
IgA deficiency may be detected in the setting of transient hypogammaglobulinemia of infancy, ataxia-telangiectasia (AT), IgG2 subclass deficiency, DiGeorge syndrome, and common variable immunodeficiency (CVID) (Hostoffer 2020). The latter should be particularly considered in those with autoimmune features as SIgAD patients with autoimmune features are more prone to develop CVID and also autoimmune disorders are reported as the presenting sign in patients with SIgAD or those who progress to CVID (Abolhassani et al. 2015; Lougaris et al. 2019). Evaluation of serum IgA could be helpful in early diagnosis of these conditions, preventing diagnostic delay and development of further complications.