Primary Immunodeficiency disorders

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 deep-seated infections.
Two or more pneumonias within 1 year.
A family history of Primary Immunodeficiency.
Failure of an infant to gain weight or grow
normally.
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
n440
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.