Primer immun yetersizliklerin tanısında farkındalığı arttıracak klinik özelliklerin değerlendirilmesi

Abstract

Objective: Each step effective on the emergence of immune response creates a potential for primary immune deficiency disease (PID). From the year 1952 on when X-linked agammaglobulinemia was described firstly, more than 150 cases with PID have been defined. Although as a common finding most of the patients manifest predisposition to infections, controversies exist about the choice of laboratory tests, and candidate patients. Materials and Methods: The study group consisted of 100 children aged between 2008-2009 who applied to our hospital with complaints of recurrent or deep-seated infection and had at least one of the 10 PID warning signs reported by the Jeffrey Model Foundation Medical Advisory Board. Study forms contained questions regarding the patient’s age, gender, immunization status, diet, age at onset of complaints, presence of parental consanguinity and other demographic features. Results: Twenty-five of 100 patients were diagnosed with PID, while the remaining 75 were found to be normal. There was a significant difference between PID and nonPID groups in ≥ 2 out of 10 warning signs: >2 sinus infections, ≥2 pneumonias and requirement for IV antibiotic use for the recovery from infection (p<0.05). While consanguinity and formula feeding were more frequently seen in group of PID, while age distribution, gender and immunization history were similar between two groups (p>0.05). Conclusion: While 10 warning signs determined by the Jeffrey Model Foundation are important for screening of immunodeficiencies, they are not sufficient on their own. Gathering additional information on consanguinity, diet and age, a detailed physical examination and basic laboratory analyses will facilitate early diagnosis of PID.