Definition

1. The Study Of Frequency Of Primary ImmunoDeficiency Disorders In Iran And Constructing A Database For Registering The Patients

2. Definition A group of inherited disorders, characterized by recurrent and/or unusual infections in different organs of the body. 1. Antibody deficiencies 2. Cellular deficiencies 3. Phagocytic disorders 4. Complement deficiencies

3. Antibody deficiencies include: • Common variable immunodeficiency (CVID) • X-linked agammaglobulinemia (XLA) • Selective IgA deficiency (SIgAd) • Selective IgG subclass deficiency (SIgGsd) • Hyper IgM syndrome (HIgM) • Transient hypogammaglobulinemia of Infancy (THI) • Functional antibody deficiency

4. Cellular deficiencies include: • Combined immunodeficiency (CID) • Severe combined immunodeficiency (SCID) • Ataxia-Telangiectasia syndrome (AT) • Wiskott-Aldrich syndrome (WAS) • DiGeorge syndrome • Chronic mucocutaneous candidiasis (CMCC)

5. Phagocytic disorders include: • Chronic granulomatous disease (CGD) • Leukocyte adhesion defect (LAD) • Chediak-Higashi syndrome (CHS) • Swhachman syndrome (Swh.S) • Hyper IgE syndrome (Job syndrome) Complement deficiencies

6. Characteristics of infections • Increasing susceptibility to infections • Increasing severity of infection • Increasing duration of infections • Unusual infection • Infection with opportunistic agents • Continuous illness • Dependence to antibiotics

7. The 10 Warning Signs Of Primary Immunodeficiency Eight or more new ear infections within 1 year. Recurrent, deep skin or organ abscesses. Two or more serious sinus infections within 1 year. Persistent thrush in mouth or elsewhere on skin, after age 1. Two or more months on antibiotics with little effect. Need for intravenous antibiotics to clear infections. Two or more pneumonias within 1 year. Two or more deep-seated infections. Failure of an infant to gain weight or grow normally. A family history of Primary Immunodeficiency.

8. Laboratory Tests in Immunodeficiency

9. Design of the Iranian Primary Immunodeficiency Registry (IPIDR)

10. Iranian Primary ImmunoDeficiency Registry (IPIDR) was established in August 1999. The clinical files of the patients with PID were reviewed from 1980 till now.

11. Main goals • To determine the frequency of primary immunodeficiency disorders in Iran. • Constructing a database for registering the patients with primary immunodeficiency disorders.

12. Other purposes • To enhance the knowledge about Primary Immunodeficiency Disorders (PID) among general practitioners and pediatricians. • To emphasize the importance of early diagnosis and treatment of PID. • To promote research about primary immunodeficiencies in our country.

13. Contributing centers 1. Department of Clinical Pediatric Immunology, Children's Medical Center Hospital. Tehran University of Medical Sciences Dr. Aghamohammadi A. Dr. Farhoudi A. Dr. Moin M. Dr. Pourpak Z. Dr. Movahedi M. Dr. Gharagozlou M. Dr. Mir Saeid Ghazi B. Dr. Atarod L. Dr. Rezaei N.

14. Contributing centers 2. Department of Infectious disease, Daneshvari Hospital. Beheshti University of Medical Sciences, Tehran Dr. Mansouri D. 3. Department of Immunology and Allergy, Al-rasoul Hospital.Iran University of Medical Sciences, Tehran Dr. Arshi S. Dr. JavaherTrash N.

15. Contributing centers 4. Department of Clinical Pediatric Immunology, Al- Zahara Hospital. Isfahan University of Medical Sciences, Isfahan Dr. Akbari H. Dr. SherkatR. 5. Department of Immunology and Allergy, Nemazi Hospital,Shiraz University of Medical Sciences, Shiraz Dr. Amin R. Dr. Alborzi A. Dr. Karimi A. Dr. Kashef S.

16. Contributing centers 6. Department of Immunology and Allergy, Mashhad University of Medical Sciences, Mashhad Dr. Farid Hosayni M.R. Dr. Hashemzadeh A. 7. Department of Clinical Pediatric Immunology, Babol University of Medical Sciences, Babol Dr. Mohammadzadeh I. 8. Department of Immunology and Allergy, Zahedan University of Medical Sciences, Zahedan Dr. Khazaei H.

17. Project outline Preliminary entering to database Diagnosis confirmed Final revision Complete Preliminary questionnaire Final questionnaire Checking Final entering to database Incomplete Contributing centers Return to be revised

18. General findings No. of pts: We have analyzed the records of 440 patients with the diagnosis of primary immunodeficiency, derived from IPIDR during a period of 20 years (1980-2000). Sex: Among all of our patients, 277 patients were male and 163 patients were female. Male to female ratio was 1.7/1

19. General findings Patients’ status: • Alive: 282 pts. • Dead: 65 pts. • Non-available: 93 pts. Age: • Mean study age: 11.0 yrs • Range: 2 months - 42 yrs Around two thirds of the patients were in pediatrics age group (63.42%).

20. The number and percentage of registered PID patients from different centers of Iran

21. Registered primary immunodeficient patients according to the system involved, between 1981-2001 n=440

22. Primary antibody deficiencies in Iran n = 202 Number

23. Primary T-cell disorders in Iran n = 107 Number

24. Primary phagocytic disorders in Iran n = 128 Number

25. Total number of PID registered in IPIDR

26. All patients with recurrent infections should be screened for immunodeficiency. Those patients with a confirmed PID should be registered as a immunodeficient patient.

27. These definite PID cases should be closely followed for development of infections and complications. Their infections should be properly treated to prevent their further morbidity and mortality.

28. So, early diagnosis of PID in suspicious patients should be considered to reduce the mortality and morbidity of these disorders.

29. In order to diagnose PID patients earlier, the general knowledge of physicians should be increased. Such registries will increase the physicians’ knowledge about such disorders.