Primary Immune Deficiency Disorder
Conditions
Brief summary
* Background/Rationale: Epidemiological data about Primary Immunodeficiency Disorders (PIDD) in Kuwait is needed to better understand peculiarities and to compare them with other regions and ethnicity. * Study hypothesis: PIDD is relatively common in Kuwait compared to populations from different geographic areas. The distribution of PIDD in Kuwait is different from other geographic areas with more severe forms being more frequent. * Brief inclusion and exclusion criteria of study participants: PIDD patients presented at different clinics/hospital in Kuwait. Patients with secondary immunodeficiencies (drug induced, virus induced, and immunodeficiency associated with metabolic disorders... ect), will be excluded * Estimated sample size of the study: All patients who were registered in KNPIDR since 2004 will be included in the study along with the new patients who will be recruited during the study period. * Primary objectives: * Determine the prevalence and frequency of different PIDD in Kuwait * Identify clinical presentation patterns for PIDD in Kuwait * Identify natural history of PIDD in Kuwait * Help to asses epidemiology of PIDD in Kuwait * Determine particularities about PIDD affecting the population in Kuwait * Determine the health impact of PIDD in Kuwait * Development of strategies to improve the care and the quality of life of patients with PIDD
Interventions
None listed
Sponsors
Kuwait Society for Allergy and Clinical Immunology
Study design
Observational model
COHORT
Time perspective
OTHER
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
Inclusion criteria
* PIDD patients presented at different clinics/hospital in Kuwait *
Exclusion criteria
Patients with secondary immunodeficiencies (drug induced, virus induced, and immunodeficiency associated with metabolic disorders... ect)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Number of Participants With Different Epidemiological Features | 15 years |
| Number of Overall and New Cases of PID Per 100,000 Kuwaitis | 15 years |
Countries
Kuwait
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| PID Arab Patients All patients diagnosed with PID | 314 |
| Total | 314 |
Baseline characteristics
| Characteristic | PID Arab Patients |
|---|---|
| Age, Categorical <=18 years | 295 Participants |
| Age, Categorical >=65 years | 0 Participants |
| Age, Categorical Between 18 and 65 years | 19 Participants |
| Race/Ethnicity, Customized Arab patients | 314 participants |
| Sex: Female, Male Female | 149 Participants |
| Sex: Female, Male Male | 165 Participants |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | 81 / 314 |
| other Total, other adverse events | 0 / 0 |
| serious Total, serious adverse events | 0 / 0 |
Outcome results
Primary
Number of Overall and New Cases of PID Per 100,000 Kuwaitis
Time frame: 15 years
| Arm | Measure | Group | Value (NUMBER) |
|---|---|---|---|
| PID Patients | Number of Overall and New Cases of PID Per 100,000 Kuwaitis | Overall Cases | 20 Cases per 100,000 Kuwaiti |
| PID Patients | Number of Overall and New Cases of PID Per 100,000 Kuwaitis | New Cases | 25 Cases per 100,000 Kuwaiti |
Primary
Number of Participants With Different Epidemiological Features
Time frame: 15 years
Population: PID patients
| Arm | Measure | Group | Value (NUMBER) |
|---|---|---|---|
| PID Patients | Number of Participants With Different Epidemiological Features | Kuwaiti patients | 287 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | immunodeficiencies affecting cellular and humoral | 100 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | combined immunodeficiencies with associated syndro | 68 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | predominantly antibody deficiencies | 56 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | diseases of immune dysregulation | 47 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | Phagocytic defects | 20 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | autoinflammatory disorders | 1 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | Complement deficiencies | 22 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | Parental consanguinity | 245 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | Family history of PID | 157 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | Genetic testing | 217 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | IVIG treatment | 175 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | Stem cell transplant | 72 patients |
| PID Patients | Number of Participants With Different Epidemiological Features | Deaths | 81 patients |