Infections led reported causes of death in inborn immune disorders
A pooled review identified infections, transplant-related deaths and noninfectious lung complications as leading causes of death. Its mortality estimate lacks a stated follow-up period.
Based on the published abstract. The full paper may contain additional methods, results and limitations.
The 30-second takeaway
The review pooled mortality reports for people with inborn errors of immunity and found different patterns across disorders. Its overall estimate describes the published cases gathered for the analysis. Without a stated observation period or sufficiently described patient mix, the figure cannot be used as an individual's expected risk of death.
This abstract-based account cannot establish a common follow-up interval or how differences in disease severity and treatment settings were handled; those details are not provided.
What researchers found
Pooled reported mortality
Proportion of published inborn immune-disorder cases reported to have died; no comparator for this estimate, and the period over which deaths were counted is not reported.
Counts and proportions differ
Reported death counts and mortality proportions answered different questions. Severe combined immunodeficiency had the largest reported death count, while familial hemophagocytic lymphohistiocytosis had the highest reported mortality proportion.
Interpreting the global comparison
The authors also compared pooled mortality with a global average. The abstract does not establish matching by age, observation period or clinical setting, limiting what that contrast says about excess risk.
The original publication
Mortality rate and causes of death in inborn errors of immunity: A systematic review and meta-analysis.
Fekrvand S, Esfahani ZH, Yarahmadi M et al.
Mutat Res Rev Mutat Res · 2025
- PubMed ID
- 41016093
- Record checked
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