Serum levels of immunoglobulins G, A and M were determined by nephelometry in 414 infants and children aged 1 month to 14 years. The children were admitted to "Aghia Sophia" Teaching Hospital for Children for surgical corrections of minor anatomical abnormalities, but they were otherwise healthy. Statistical analysis was performed through multiple regression after logarithmic transformation of the immunoglobulin values. Immunoglobulin G (IgG) levels increased significantly by 18% per year until the age of 5 years and by 2% per year thereafter. Among children less than 5 years old, IgG levels tended to increase significantly with the number of doses of either DTP or Sabin vaccine. Immunoglobulin A (IgA) levels increased significantly by 27% until the age of 7 years and by 4% thereafter. Among children less than 7 years old, history of breast feeding was significantly associated with higher levels of IgA. Vaccination with either DTP or Sabin was associated with elevation of IgA levels among younger and, to a lesser extent, older children, but the elevations did not reach statistical significance. Immunoglobulin M (IgM) levels increased rapidly during the first 12 months of life and very slowly thereafter and they were significantly higher among girls. Among children less than one year old, there was evidence that vaccination with either DTP or Sabin was associated with elevated IgM levels, although the differences were not statistically significant possibly because of small numbers of infants in the study sample.(ABSTRACT TRUNCATED AT 250 WORDS)

Although many children develop frequent infections, only a few have an underlying immune disorder. Children with dysfunction of the immune system develop frequent infections and/or recurrent, persistent, severe, and rare infections. The aim of this review is to provide to the clinician a valuable tool for recognizing any 'discords' of the 'immune-system symphonic orchestra'. By following a reverse route, it will be possible to brighten up the dark and winding road of immunodeficiencies and identify the exact point of immune dysfunction. This is fundamental and crucial to perceive etiologic management and subsequently achieve the best for these young patients and their families.

Chronic Granulomatous Disease (CGD) is an uncommon primary immunodeficiency caused by the absence or dysfunction of one of NADPH oxidase subunits, with heterogeneous genetic aetiologies. The aim of this study was the CGD patient registry in Greece, the identification of the responsible genotype and the potential correlation with the patient's clinical phenotype. Medical charts of 24 CGD patients, investigated by NBT test or DHR for NADPH oxidase activity, Western blot analysis for NADPH oxidase component expression and DNA sequencing (pyro- and cycle sequencing) for mutation analysis, were reviewed. All patients, but one, were classified into the different types of CGD. Sixteen patients from 14 unrelated families had X-linked CGD (66.7 %), four had mutations in the NCF1 gene (19 %), and three, from two unrelated families, had mutations in NCF2 (9.5 %) [Corrected]. Fifteen mutations were detected in the CYBB gene, including nonsense (53.8 %), splice site (30.8 %) and missense mutations (7.7 %), and deletions (7.7 %). Two novel mutations were identified; one in CYBB and one in NCF1. Carrier detection for X-CGD revealed that the de novo mutation rate was about 7 %. Prenatal diagnosis identified one affected male in three male fetuses tested. In both the X-linked and the autosomal recessive (AR-CGD) group, the gastrointestinal and respiratory manifestations were more common, followed by lympadenopathy in X-CGD and skin infections in the AR-CGD group. The patients with a mutation in CYBB had a wider variability of clinical manifestations and earlier diagnosis (4.6 years) compared to the AR-CGD group (12.9 years). The incidence of CGD in Greece is estimated at 0.90 (95 % CI 0.89-0.91) per 100,000 live births for the last decade.

The hypothesis whether exposure to certain infections protects from atopy remains equivocal. To further investigate this, we compared serologic markers of infection and allergic sensitization prevalence in Roma children, who live under unfavorable hygienic conditions that facilitate the spread of infections, and non-Roma children who live in the same area. Analyses included 98 Roma and 118 non-Roma children. Serum IgG antibodies for 13 foodborne- airborne- and bloodborne infectious agents were determined, and a cumulative index of exposure was calculated by adding one point for each positive infection. Specific serum IgE to certain common food- and aero-allergens was also tested. and positivity to any of them was defined as indication of atopy. Roma children were found significantly more seropositive for T. gondii, Hepatitis A, H. pylori, HSV-1, CMV, and Hepatitis B (p < 0.0001). Non-Roma children were found more seropositive for RSV and M. pneumonia (p < 0.0001). Regarding the overall prevalence of atopy or the specific IgE responses to the allergens tested, no statistically significant differences were found between Roma and non-Roma children. A positive association of the cumulative index of exposure to infections with atopy was found in the non-Roma children (OR: 1.38, 95% CI: 1.08-1.75, p = 0.01) and in the total population (OR: 1.42, 95% CI: 1.11-1.83, p = 0.01). Regarding the specific infectious agents tested, a statistically significant positive association of atopy with seropositivity was found for M. pneumoniae in the non-Roma children (OR: 3.93, 95% CI: 1.39) as well as in the total population studied (OR: 2.83, 95% CI: 1.32-6.07, p = 0.01). Despite the higher burden of exposure to the battery of the infectious agents tested among Roma children, no protective effect for allergic disease development was evident. On the contrary, a positive association of exposure to infections with evidence of atopy was found, especially evident in the non-Roma children.

Serum levels of immunoglobulin E (IgE) were determined by enzyme immunoassay in 414 Greek infants and children of both sexes, 1 month to 14 years old. The children were admitted to the "Aghia Sophia" Teaching Hospital for Children (Athens, Greece) for surgical corrections of minor anatomic abnormalities, but they were otherwise healthy. Statistical analysis was performed through multiple regression after logarithmic transformation of the immunoglobulin values. IgE levels increased significantly by about 80% per year up to the age of 5 years, without noticeable impact of age on these levels thereafter. History of allergic disease showed a significant positive association with serum levels of IgE in both younger (less than 5 years) and older children. History of frequent infections was positively associated with IgE levels, although the relation was statistically significant only in the older age group, IgE levels in Greek children appear to be higher than the corresponding levels of children living in northern Europe, but much lower than those of children in Southeast Asia, a fact that may reflect different exposure level during childhood to infections known to influence IgE levels.