Trajectory of Hyper IgE complications
- 1Children's Hospital of Philadelphia
Abstract
ObjectiveOur objective was to define the natural history of hyper IgE syndrome. Well, there have been a number of large cross-sectional studies. It has been difficult to provide anticipatory care for the growing adult population.
MethodsThe USIDNET registry was used to identify patients with hyper IgE syndrome. We evaluated all patients regardless of genetic etiology. Complications were extracted as text from the problem list or ICD codes, and each complication was date stamped, which allowed us to estimate the age at which the complication arose.
ResultsMany of the complications previously identified were seen in this cohort. The most feared complications were uncommon, however, developmental delay and psychosis affected nearly a fifth of the cohort which has not been previously reported.
ConclusionsAs the number of adults with hyper IgE syndrome increases our awareness of late onset complications is important. Aspergillus and pneumatoceles were less common than seen in some other cohorts. However, the significant rate of psychosis will represent a significant challenge for clinicians.
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BibTeX
@misc{mao2026trajectory,
title = {{Trajectory of Hyper IgE complications}},
author = {A. Mao and R. Xiao and K. E. Sullivan and R. Fuleihan and C. Cunningham-Rundles and J. Puck and R. Marsh and R. Abraham and L. D. Notarangelo and V. Vichare and USIDNET Network members},
howpublished = {medRxiv},
year = {2026},
month = sep,
doi = {10.64898/2026.09.11.26362846},
url = {https://www.medrxiv.org/content/10.64898/2026.09.11.26362846v1},
note = {CC BY-NC-ND},
}
RIS
TY - UNPB
TI - Trajectory of Hyper IgE complications
AU - A. Mao
AU - R. Xiao
AU - K. E. Sullivan
AU - R. Fuleihan
AU - C. Cunningham-Rundles
AU - J. Puck
AU - R. Marsh
AU - R. Abraham
AU - L. D. Notarangelo
AU - V. Vichare
AU - USIDNET Network members
PY - 2026
DA - 2026/09/11
PB - medRxiv
DO - 10.64898/2026.09.11.26362846
UR - https://www.medrxiv.org/content/10.64898/2026.09.11.26362846v1
AB - ObjectiveOur objective was to define the natural history of hyper IgE syndrome. Well, there have been a number of large cross-sectional studies. It has been difficult to provide anticipatory care for the growing adult population. MethodsThe USIDNET registry was used to identify patients with hyper IgE syndrome. We evaluated all patients regardless of genetic etiology. Complications were extracted as text from the problem list or ICD codes, and each complication was date stamped, which allowed us to estimate the age at which the complication arose. ResultsMany of the complications previously identified were seen in this cohort. The most feared complications were uncommon, however, developmental delay and psychosis affected nearly a fifth of the cohort which has not been previously reported. ConclusionsAs the number of adults with hyper IgE syndrome increases our awareness of late onset complications is important. Aspergillus and pneumatoceles were less common than seen in some other cohorts. However, the significant rate of psychosis will represent a significant challenge for clinicians.
ER -