Profile of the patients who present to immunology outpatient clinics because of frequent infections

Abstract

Aim: We aimed to determine the rate of primary immune deficiency (PID) among children presenting to our immunology outpatient clinic with a history of frequent infections and with warning signs of primary immune deficiency. Material and Methods:The files of 232 children aged between 1 and 18 years with warning signs of primary immune deficiency who were referred to our pediatric immunology outpatient clinic with a complaint of frequent infections were selected and evaluated retrospectively. Results: Thirty-six percent of the subjects were female (n=84) and 64% were male (n=148). PID was found in 72.4% (n=164). The most common diagnosis was selective IgA deficiency (26.3%, n=61). The most common diseases other than primary immune deficiency included reactive airway disease and/or atopy (34.4%, n=22), adenoid vegetation (12.3%, n=8), chronic disease (6.3%, n=4) and periodic fever, aphtous stomatitis and adenopathy (4.6%, n=3). The majortiy of the subjects (90.5%, n=210) presented with a complaint of recurrent upper respiratory tract infection. PID was found in all subjects who had bronchiectasis. The rates of the diagnoses of variable immune deficiency and Bruton agammaglubulinemia (XLA) were found to be significantly higher in the subjects who had lower respiratory tract infection, who were hospitalized because of infection and who had a history of severe infection compared to the subjects who did not have these properties (p<0.05 and p<0.01, respectively). Growth and developmental failure was found with a significantly higher rate in the patients who had a diagnosis of severe combined immune deficiency or hyper IgM compared to the other subjects (p<0.01). No difference was found in the rates of PID between the age groups, but the diagnosis of XLA increased as the age of presentation increased and this was considered an indicator which showed that patients with XLA were being diagnosed in a late period. Conclusions: It was found that the rate of diagnosis was considerably high (72.4%), when the subjects who had frequent infections were selected by the warning signs of PID. (Türk Ped Arş 2014; 49: 210-6)

Current Approach to Primary Immunodeficiency Diseases

“…Selective IgA deficiency and transient hypogammaglobulinemia in infants are among the leading disorders in our country. [11][12][13][14][15] Initially, quantitative immunoglobulin isotype values for age, screening tests, specific antibody levels after natural immunity (spontaneous) and immunization (vaccination), and IgG subgroup (IgG1-G2-G3-G4) levels are examined. In vitro functional studies that can evaluate Bcell immunophenotyping and antibody production can be performed in advanced centers.…”

Assessment of weight and height of patients with primary immunodeficiency disorders and group of children with recurrent respiratory tract infections

“…In Adirmaz et al study 11.2% of children with PID were retarded in their height and physical development and in the study on Egyptian children interrupted physical development and growth were detected among 28% of patients diagnosed with PID [21,22]. In our study higher percentage of malnourished children in Ig + therapy group can be explained by complexity of diseases' syndromes, often exceeding over immune system, which may also limit patients' development in a significant way (Nijmegen breakage syndrome, ataxia-telangiectasia).…”

Assessment of weight and height of patients with primary immunodeficiency disorders and group of children with recurrent respiratory tract infections.

“…In Adimaz et al study 11.2% of children with PID were retarded in their height and physical development and in the study on Egyptian children interrupted physical development and growth were detected among 28% of patients diagnosed with PID [27,28]. In our study higher percentage of malnourished children in Ig+therapy group can be explained by complexity of diseases' syndromes, often exceeding over immune system, which may also limit patients' development in a signi cant way (Nijmegen breakage syndrome, ataxia-telangiectasia).…”