Background: IGA DEFICIENCY is a common type of primary immunoDEFICIENCY syndrom. Clinical manifestation of this disease is chronic and recurrent sinopulmonary infection and diarrea. This report describes a case of IGA DEFICIENCY with chronic and recurrent paranasal synositis, pneumonia and diarrea.    

Background/objectives: SIGAD is the most frequent of the primary antibody deficiencies. Patients with IGAD can be either symptomatic or asymptomatic. Symptomatic patients suffer from a wide range of manifestations including allergy, malignancy, and autoimmunity. The prevalence of allergic diseases is assumed to be increased in IGAD patients. In this study, we aimed to evaluate the frequency of allergic disorders in IGAD patients as well as a comparison between these patients and IGA deficient patients without allergy. Methods: The present cohort study included 166 IGAD patients who were diagnosed at the Research Center for immunodeficiencies in children’ s medical Center. To compare clinical data and laboratory records, all IGAD patients were classified into two groups as follows: patients with allergic diseases and patients without allergic diseases. Results: Among 166 patients with IGA DEFICIENCY, allergy was seen in 33 patients (19. 8%). In this study, respiratory tract infections were the most common clinical presentation in all patients (47. 6%). Among the infectious manifestations, pneumonia and sinusitis were significantly higher in patients with allergy compared with patients without allergy (respectively 48. 5% vs 26. 3%; p = 0. 013, 48. 5% vs 20. 3%; p = 0. 001). Based on the laboratory data, the number of platelet and B cells (CD20+) were significantly higher in patients with allergy in comparison to patients without allergy (respectively, p = 0. 025, p = 0. 44). Conclusions: The relation between IGAD disease and allergy could lead to severe clinical complications. Thus, these allergy disorders should be considered as an important feature for suitable management and enhancing the life quality in patients with IGAD.

Background: Selective IGA DEFICIENCY (SIGAD), is known as the most common antibody DEFICIENCY. This study, investIGAted the respiratory disorders among these patients. Method: In this retrospective descriptive study, the data of patients with SIGAD in “, Iranian Primary ImmunoDEFICIENCY Registry”,were extracted. Then, demographic characteristics, frequency and type of respiratory disorders, in addition to immunologic findings were gathered and analyzed by SPSS software. Results: One hundred and twenty three patients with SIGAD (78 males/45 females), with the mean age of 18±,9. 6 years old were investIGAted. Respiratory complications (recurrent cold, chronic cough and pneumonia), were the first presentation in 45% of the patients, and respiratory system involvement was detected among 66 patients (54. 1%). The prevalence of pneumonia, otitis media and sinusitis, were 33. 3%, 12. 5% and 20. 7%,respectively,which were significantly reduced after the diagnosis (P<0. 05). Bronchiectasis had also been found in five patients (4. 1%). Conclusion: Respiratory problems were shown to be common presenting disorders in SIGAD. Early diagnosis and appropriate therapy could be crucial for better protection, and avoidance of severe respiratory complications in these patients.

Background/objectives: Among primary immunoDEFICIENCY (PID), selective immunoglobulin A DEFICIENCY (SIGAD) is the most prevalent type. SIGAD patients can be either asymptomatic or symptomatic. Symptomatic patients suffer from a wide range of manifestations including infections, allergy, autoimmunity, and malignancy. SIGAD patients are more susceptible to some autoimmune diseases, while the exact mechanisms behind this association are not found yet. Therefore, this study was conducted in order to evaluate the possible association between autoimmune disease and specific clinical records or immunological data in SIGAD patients. Methods: The present cohort included 166 SIGAD patients who were diagnosed at the Research Centre for Immunodeficiencies at the children’ s medical Centre. A comprehensive history, demographic information, clinical manifestations, laboratory data were obtained from all patients to assess the autoimmune complications. Results: Autoimmunity was seen in 16 patients (9. 6%, 10 males and 6 females). The most common autoimmunity types were juvenile idiopathic arthritis, vitiligo and alopecia (18. 8%). 9 patients (6. 5%) had a PID family history. Significant data that were higher in patients with autoimmunity were the mean age at the study time (p=0. 019), rheumatoid problem (p=0. 043), liver problem (p=0. 031), IgG level (p=0. 006) and IgE level (p=0. 004). Conclusion: The association between SIGA DEFICIENCY and autoimmunity could lead to severe clinical complications. So, it is better for immunologists to aware of these problems.

Background and Objectives: Anaphylactic reaction is one of the most important transfusion anaphylactic reactions in medicine. During transfusion if Individuals with IGA DEFICIENCY take blood which containing IGA, they would suffer from severe allergic reaction. Since there is a little known about this in different ethnic groups in Iran we have decided to evaluate this issue in Sistan & Baluchestan province in Iran.Methods: During one year, blood samples were collected from 3837 volunteer blood donors. Then the serum samples were tested for IGA titer by nephlometry and SRID techniques.Results: According to our results only, one donor (0.026%) was found to have IGA less than 5mg/dl and 34(0.9%) cases had IGA: 5-30 mg/dl while 3798(99%) had IGA more than 30mg/dl.Conclusions: With regards to very low prevalence of IGA DEFICIENCY in blood donors in Southeast part of Iran, it seems that anaphylactic reactions most likely be due to other factors in the individuals that receive blood products.