Impaired specific antibody response and increased B-cell population in transient hypogammaglobulinemia of infancy.
ABSTRACT Transient hypogammaglobulinemia of infancy (THI) is a heterogeneous disorder with poorly understood pathophysiology.
To better characterize THI and improve understanding of its pathophysiology.
Twenty-four children with hypogammaglobulinemia defined by an IgG level less than 2 SDs below the mean on 2 occasions, who did not have other immunologic diagnoses, were followed and retrospectively reviewed.
The average z-score for IgG level at presentation was -2.4 (mean age, 12 months; median age, 8 months), with a mean level of 254 mg/dL. Thirteen of 24 patients had IgA levels less than 2 SDs below the mean, 5 had IgM levels less than 2 SDs below the mean, and 7 of 23 had elevated IgE levels. Eighteen were followed up until their IgG levels normalized (mean age, 27 months; median age, 23 months), with 12 of 18 normalizing by 24 months and the remainder by 59 months. There was a significant association between presenting IgG z-score and duration of disease (P = .05). Five of the 18 patients had absolute CD19+ B-cell counts greater than the 95% percentile for age (P < .001), and the mean percentage and absolute CD19+ B-cell count across all patients were greater than those of the age-matched controls (P = .02). Most patients had nonprotective titers to Haemophilus influenzae type b vaccine, and one third had nonprotective titers to tetanus vaccine. Twenty patients carried at least one atopic diagnosis, and 13 of those had recurrent wheezing.
THI is associated with a number of immunologic abnormalities beyond just hypogammaglobulinemia. These abnormalities include impaired specific antibody responses and increased proportions of CD19+ B cells and may be suggestive of particular immunologic mechanisms that result in hypogammaglobulinemia.
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ABSTRACT: Antibodies are an essential component of the adaptative immune response and hold long-term memory of the immunological experiences throughout life. Antibody defects represent approximately half of the well-known primary immunodeficiencies requiring immunoglobulin replacement therapy. In this article, the authors review the current indications and therapeutic protocols in the Latin American environment. Immunoglobulin replacement therapy has been a safe procedure that induces dramatic positive changes in the clinical outcome of patients who carry antibody defects.Allergologia et Immunopathologia 01/2013; 42(3). DOI:10.1016/j.aller.2012.09.006 · 1.58 Impact Factor
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ABSTRACT: Introduction Antibody production defects may predispose children to inflammatory pathologies and therefore we hypothesized that this group of immune deficiencies may be associated with food allergy. Objective of the study To better characterize the interrelated pathomechanisms of food allergy coexisting with hypogammaglobulinemia in children and to define the relationship between clinical manifestation of antibody production defects and food allergy. Material and methods Twenty-three children aged from 8 to 88 months regularly followed-up in the pediatric pneumonology, allergology and immunology clinic due to hypogammaglobulinemia concerning one or more major immunoglobulin isotypes were retrospectively reviewed in terms of incidence and manifestation of concomitant food allergy. Information regarding the patient's history of allergic diseases and laboratory data concerning serum levels of immunoglobulins, including total IgE, were obtained from chart review. Results Clinical symptoms of food allergy were identified in 17 of 23 (74%) children studied. The mean age of onset of clinical symptoms was 2.7 months. Eczema was the most frequent manifestation present in 16 children, diarrheas and abdominal cramps were noted equally in 3 children, gastroesophageal reflux disease was diagnosed in 2 children as well as vomiting was observed in 2 children. Atopy was revealed in 8 of 17 children (47%) with food allergy. Conclusions Food allergy is a common health problem coexisting with antibody production defects in infants and young children. Clinical symptoms correlate better with low immunoglobulin levels than with serum IgE, that is not a suitable diagnostic criterion for allergic disease in patients with hypogammaglobulinemia.Pediatria polska 10/2012; 87(5):444–448. DOI:10.1016/j.pepo.2012.07.004
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ABSTRACT: Transient hypogammaglobulinemia of infancy (THI) is characterized by recurrent infections and one or more reduced serum immunoglobulin levels. Typically, THI patients recover spontaneously, mostly within 30-40 months of age, but sometimes recovery may be delayed until 5-6 years of age. The use of intravenous immunoglobulin (IVIg) as an alternative to antibiotic prophylaxis remains contraversial also in symptomatic THI patients. In fact, some authors believe that IVIg therapy may cause a delay in the maturation of the humoral immune system because of the interference from passively transfered antibodies. The aim of this study was to investigate the effect of IVIg replacement on recovery from immunodeficiency in THI patients and determine new parameters in order to include these patients in IVIg therapy groups. In this retrospective study, 43 patients (65%) received IVIg replacement therapy while 23 patients (34.8%) showed spontaneous normalization without IVIg. The percentages of patients who had more than six times the number of febrile infections in a year decreased from 91% to 21% in the group receiving IVIg treatment. At admission, before being recruited to IVIg therapy, serum immunoglobulin G (IgG) levels and anti-hemophilus B (Hib) antibody titers were found to be significantly low in cases who were selected for IVIg replacement. The percentages of patients who did not have protective levels of anti-Hib, anti-rubella or anti-rubeola-IgG were also significantly high in IVIg cases. There was no statistically significant difference in the age at which IgG levels normalized between the IVIg and the non-IVIg group. Patients in the IVIg group and non-IVIg group reached normal IgG levels at the age of 42.9±22.0 and 40.7±19.8 months, respectively. In conclusion, IVIg infusions do not cause a delay in the maturation of the immune system in THI patients. Besides the well-established criteria, very low and non-protective specific antibody responses against previously applied vaccines are important factors to consider when selecting patients for IVIg therapy.Pediatric reports 07/2013; 5(3):e14. DOI:10.4081/pr.2013.e14