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

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

 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 Antibody deficiencies include:

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

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

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

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 deep-seated infections. A family history of Primary Immunodeficiency. Two or more pneumonias within 1 year. Failure of an infant to gain weight or grow normally. The 10 Warning Signs Of Primary Immunodeficiency

Laboratory Tests in Immunodeficiency

Design of the Iranian Primary Immunodeficiency Registry (IPIDR)

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

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

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

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. 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. Contributing centers

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. Contributing centers 4. Department of Clinical Pediatric Immunology, Al- Zahara Hospital. Isfahan University of Medical Sciences, Isfahan Dr. Akbari H. Dr. SherkatR.

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

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

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

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

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

Registered primary immunodeficient patients according to the system involved, between n=440

Primary antibody deficiencies in Iran n = 202 Number

Primary T-cell disorders in Iran n = 107 Number

Primary phagocytic disorders in Iran n = 128 Number

Total number of PID registered in IPIDR

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

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.

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

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