What does this research mean for the field?

In pediatric patients with inborn errors of immunity in middle-income settings, survival varies significantly by disease class, and hematopoietic stem cell transplantation (HSCT) substantially reduces mortality compared to non-transplant management. Novelty: ClaimNovelty.CONFIRMATORY. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

To evaluate long-term clinical characteristics, disease classification patterns, and survival determinants among pediatric patients with inborn errors of immunity.

August 30, 2026Journal of Clinical ImmunologyOpen Access

Three Decades of Outcomes in Pediatric Inborn Errors of Immunity in a Middle-Income Setting: Survival by IUIS Class and the Role of HSCT

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To evaluate long-term clinical characteristics, disease classification patterns, and survival determinants among pediatric patients with inborn errors of immunity.
Retrospective cohort study of N=123 pediatric patients diagnosed with inborn errors of immunity between 1991 and 2022 at a tertiary referral center in Thailand.
Patients were categorized according to the 2022 International Union of Immunological Societies classification, with survival evaluated using Kaplan–Meier and multivariable Cox proportional hazards models.
Overall mortality was 31.7% with infection as the primary cause of death, while predominantly antibody deficiencies accounted for 40.7% of diagnoses and demonstrated the highest survival rates.
Hematopoietic stem cell transplantation significantly reduced mortality compared to non-transplant therapy for both matched related donors (aHR 0.01, 95% CI 0.001–0.10) and haploidentical donors (aHR 0.10, 95% CI 0.03–0.33).
Male sex and combined immunodeficiency were independently associated with increased mortality risk.

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Implication

Retrospective cohort study shows stem cell transplantation reduces mortality in pediatric inborn errors of immunity, highlighting the critical value of early diagnosis and transplant access.

Cite This Study

Watcharaporn et al. (2026) studied this question.

synapsesocial.com/papers/6a93f0ce6c1a8fb52e79d4e4 https://doi.org/10.1007/s10875-026-02072-4

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Implication

Retrospective cohort study shows stem cell transplantation reduces mortality in pediatric inborn errors of immunity, highlighting the critical value of early diagnosis and transplant access.

Three Decades of Outcomes in Pediatric Inborn Errors of Immunity in a Middle-Income Setting: Survival by IUIS Class and the Role of HSCT

Key Points

Discussion

Implication

Cite This Study

Three Decades of Outcomes in Pediatric Inborn Errors of Immunity in a Middle-Income Setting: Survival by IUIS Class and the Role of HSCT

Key Points

Discussion

Implication

Cite This Study

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