Table 4.
The comparison of primary immunodeficiencies prevalence, frequencies, consanguinity, and availability of genetic results with various countries.
| Country | Malaysia | Singapore (42) | Korea (43) | Taiwan (44) | French (45)** | Brazil (46) | Kuwait (5) | USA (47) |
|---|---|---|---|---|---|---|---|---|
| Time of reporting | 1979–2020 | 1990–2000 | 2001–2005 | 1985–2004 | 2005–2009 | 1978–2011 | 2004–2018 | 1992–2020 |
| Types | Systematic review | Cohort report | National registry | National registry | National registry | Cohort report | National registry | National registry |
| Number of cases | 119 | 39 | 152 | 37 | 3,083 | 1,008 | 314 | 5,484 |
| Incidence | – | 2.65 per 100,000 live births | – | 2.17 per 100,000 livebirths | – | – | 24.96 per 100,000 | – |
| Prevalence | 0.37 cases per 100,000 population | – | 11.25 per million children | 1 in 46,000 live births | 4.4 per 100,000 inhabitants | – | *20.27 per 100,000 population | – |
| Consanguinity | 2.5% | Not reported | Not reported | 0% | 15% | Not reported | 78% | Not reported |
| Genetic mutations known | 21.7% | Not reported | Not reported | 16% | 40% | Not reported | 69% | 46% |
| Immunodeficiencies affecting cellular and humoral immunity | 30.3% | 10.3% | 13.2% | 11% | 17.2% | 6.7% | 31.8% | 9.6% |
| *T cell deficiencies 4.6% | *T cell deficiencies 19% | |||||||
| Combined immunodeficiencies with associated syndromic features | 17.6% | 12.8% | – | – | 14% | 8.3% | 21.7% | 20% |
| Predominantly antibody deficiencies | 20.2% | 41% | 53.3% | 46% | 42.8% | 60.8% | 17.8% | 49.2% |
| Diseases of immune dysregulation | 10.9% | – | – | – | 6.6% | 5.3% | 15% | 4.2% |
| Congenital defects of phagocyte number or function | 16.8% | 15.4% | 28.9% | 24% | 18.6% | 8.7% | 6.4% | 12% |
| Complement deficiencies | – | – | – | – | 0.5% | 2.9% | 7% | 0.5% |
| Autoinflammatory Syndromes | 0.8% | – | – | – | – | 1.3% | 0.3% | – |
| Defects of innate immunity | 3.4% | – | – | – | 0.2% | 5.9% | – | – |
| Others | – | 17.9% | – | – | – | – | – | 2.2% |