Clinical significance of hyper-IgA in a paediatric laboratory series (original) (raw)

[Significance of determining concentrations of subclasses of IgG and IgA--our experience]

Srpski arhiv za celokupno lekarstvo, 2005

Selective IgG and IgA subclass (IgGsc and IgAsc) deficiencies may be either partial or complete, yet, combined subclass deficiencies are common. The serums of 65 patients with various clinical symptoms were investigated for quantitative and qualitative immunoglobulin levels. A high correlation (r=0.93) between the two methods used for measuring total IgG concentration (nephelometry) and IgGsc concentration (RID measurement) was demonstrated. Patients were divided into groups according to IgG and IgA class and subclass concentrations. The serums of 51 patients were investigated for IgGsc deficiencies. Three groups were set up and studied: a group with decreased IgG concentrations (5 patients), a group with elevated IgG concentrations (6 patients), and a group with normal IgG concentrations (40 patients). Within these groups, a total of 14 patients with decreased IgGsc levels and 14 patients with elevated IgGsc levels were discovered. Analyses of gender and age frequency within these ...

Diagnostic Significance of Reduced IgA in Children

Medical Archives, 2014

Th e fi nding of reduced value of immunoglobulin A (IgA) in children is frequent in daily medical practice. It is important to correctly interpret the fi ndings as adequate further diagnostic evaluation of the patient in order to make the determination on the signifi cance of such fi ndings. In children younger than 4 years always consider the transient impairment of immunoglobulins, maturation of child and his immune system can lead to an improvement in the clinical picture. In older children decreased IgA may lead to serious illnesses that need to be recognize and acknowledge through the appropriate diagnostic methods. Material and methods: Research was realized at the University Clinical Center Tuzla. Children with suspected defi cient immune response due to reduced values of IgA observed and , goes through further diagnostic evaluation at , as well as the Clinic of Radiology. In the period of year 2013, there were a total of 91 patients with reduced values of IgA, age up to 13 years, of which 55 boys and 36 girls. Results: Our study followed 91 patients, for the year 2013, through their medical charts and made evaluation of diagnostic and screening tests. Th e signifi cance of this paper is to draw attention to the importance of diagnostic approach to IgA defi cient pediatric patient and relevance of knowledge of individual diagnostic methods as well as to the proper interpretation of the results thereof.

Serum levels of IgA1 and IgA2 in children and in patients with IgA deficiency

Molecular Immunology, 1983

Serum levels of IgA, and IgAz were measured by solid phase radioimmunoassay in samples from 110 children between 3 months and 10 years of age. Both IgA, and IgAz were detectable in all samples, and both IgAi and IgAz increased with increasing age. The percent of total serum IgA that was IgA, did not change with age and was the same in samples from children (15.05 f 10.2%) as in samples from adults (15.86 + 7.98%). The proportion of serum IgA that was IgA, was much less variable within sibships than within the group at large (P < 0.005). In the 16 patients with IgA deficiency, the proportion of serum IgA that was IgA, or IgA, was highly variable. IgAt constituted more than 50% of the IgA in 5 patients and less than 7% of the IgA in an additional 5 patients. These findings suggest that regulation of serum concentrations of IgA, and IgA, is complex and influenced by genetic factors and probably other unidentified factors.

Follow-up and outcome of symptomatic partial or absolute IgA deficiency in children

European Journal of Pediatrics, 2018

Selective IgA deficiency is defined as absolute or partial when serum IgA level is < 7 mg/dl or 2 SD below normal for age, respectively. Few data are available on partial selective IgA deficiency, as probably most children with low serum IgA are seldom referred to a specialist clinic in common pediatric practice. The aim of our study was to better define the profile of both symptomatic forms and their clinical outcome in a pediatric immunology setting. Thus, clinical and immunological data from 103 symptomatic patients with selective IgA deficiency (53 absolute and 50 partial), 4-18 years of age, were collected at diagnosis and 80 patients (44 absolute and 36 partial) were monitored for a mean period of 5 years. Also, the prevalence of TNFRSF13B mutations has been assessed in 56 patients. The most common clinical features were infections (86/103; 83%), allergy (39/103; 38%), and autoimmunity (13/103; 13%). No significative differences were observed between absolute and partial selective IgA deficiency patients. However, a significative difference in the rate of IgA normalization between partial and absolute selective IgA deficiency patients (33 vs 9%, p = 0.01) was detected. Furthermore, a lower incidence of infections was associated to a normalization reversal compared to a final absolute or partial defect status (12 vs 53 and 64% respectively, p < 0.01).

IgA Deficiency: Correlation Between Clinical and Immunological Phenotypes

Journal of Clinical Immunology, 2009

Background IgA deficiency (IGAD) is the most common primary antibody deficiency. Although many affected individuals have no apparent symptom, selected patients suffer from recurrent mucosal infections, allergies, and autoimmune diseases. We aimed to investigate the clinical features in relation to immune function of Iranian patients with symptomatic IGAD. Methods Thirty-seven patients (21 male and 16 female), aged 4–32 years, were evaluated in this study. Patients were followed for a total of 131 patient years with a mean follow-up of 3.5 years per patient. Results The most prevalent presentations were recurrent infections occurring in 27 subjects, followed by allergy in eight cases and autoimmunity in two patients. However, during the follow-up period, 35 patients developed infections in respiratory and gastrointestinal tracts, necessitating medical care. Apart from infections, allergy was the most frequent complaint (31 cases); the major features were asthma, atopic dermatitis, and allergic rhinoconjunctivitis. Autoimmune diseases were documented in ten cases; thyroiditis was the most common. In 31 patients who received unconjugated pneumococcal polyvalent vaccine, antibody response against polysaccharide antigen was measured before and 28 days after vaccination. One fourth of vaccinated patients were hyporesponsive to vaccine; four of these patients developed bronchiectasis. The patients with IGAD were classified into two groups: group 1 (14 cases) consisted of patients with IGAD and other associated immune defects, such as immunoglobulin G (IgG) subclass deficiency and defective specific antibody production. Group 2 (23 cases) had isolated IGAD without other immunological abnormalities. There was a significantly increased number of lower respiratory tract infections in group 1 compared with group 2 (P = 0.006). Moreover, four patients of group 1 had bronchiectasis whereas none of the patients in group 2 developed this complication (P = 0.015). Conclusion Subclassification of IGAD regarding the existence of associated immune defects is useful in terms of morbidity and planning for medical care. IgA-deficient patients with concomitant immune defects such as defects in specific antibody production have higher rates of recurrent infections and bronchiectasis, which necessitates more effective monitoring.

Immunological investigations in individuals with selective IgA deficiency

Clinical and experimental immunology, 1986

Concentrations of IgG2, IgG4 and IgE were low in 16, 24 and 20% of 25 persons with selective IgA deficiency. Fifty-two per cent had IgD concentrations below 5 iu/ml. Trends for association between any of these parameters and the presence of clinical symptoms were not significant. All patients, except one, had normal amounts of Ig-bearing lymphocytes in the blood. IgG1 antibodies against casein were increased in titre and frequency, whereas IgG4 antibodies were normal. Similar results were found in other sera from persons with selective IgA deficiency.

Selective IgA Deficiency in Children in Israel

Journal of Clinical Immunology, 2010

IgA deficiency is the most common human primary immune-deficiency. We evaluated the clinical and immunological characteristics of selective IgA deficiency in children in Israel. The study group included 63 children diagnosed with IgA deficiency from 1987 to 2005. Mean follow-up time per child was 10.6 years. Average age at diagnosis was 10.5 years. In one child, the IgA deficiency was transient. Infectious diseases, mainly recurrent pneumonia and ear infection, were common and occurred in 25 patients (39.7%). Allergic diseases were documented in 20 (31.7%) of our patients. Thirteen children (20.6%) had autoimmune diseases. Malignancies were diagnosed in three children (4.8%), an association that has not been reported in previous series. IgA deficiency appears to be a risk factor for infections, allergic diseases, autoimmune conditions, and malignancy.

LIMITS AND POSSIBILITIES IN A PRIMARY SELECTIVE IgA DEFICIENCY TREATMENT – CASE PRESENTATION

Romanian Journal of Pediatrics, 2017

The primary deficit of IgA is the most common congenital immunodeficiency defined by the values of serum IgA below 0.07 g/l and normal values of IgG and IgM. Most cases are asymptomatic, only 10-15% is manifested by recurrent or persistent sinopulmonary infections, autoimmune diseases, allergies, malignancies. Some cases develop over the time a common variable immunodeficiency. The frequency of anti-IgA antibody justifies the cautiously use of transfusions of blood, plasma or intravenous immunoglobulin. The authors present a case of primary selective IgA deficiency, where the severity of infectious exacerbations appears to be due to the combination of a lack of IgG4, and discuss the possibilities and limits of therapy in light of recent data from the literature.