# Comprehensive Multidisciplinary Management of Hyper-IgE Syndrome in an 11-Year-Old Female: A Pediatric Case Report

**Authors:** Ahmed Elashmawy, Mohammadali Chokr, Saima Sharif, Lauren Ferrantino

PMC · DOI: 10.7759/cureus.65377 · Cureus · 2024-07-25

## TL;DR

This case report details the treatment of an 11-year-old girl with a rare immune disorder causing frequent infections and bone issues.

## Contribution

The paper presents a comprehensive management approach for HIES in a pediatric patient with pneumonia and osteopenia.

## Key findings

- The patient required a rigorous course of antibiotics and antifungals for infection control.
- Imaging and pathogen cultures were essential for diagnosing and managing the patient's condition.
- Effective multidisciplinary care improved the patient's outcomes and reduced infection risks.

## Abstract

Hyper-IgE syndrome (HIES) or Job syndrome is a rare immunodeficiency characterized by elevated levels of IgE and recurrent infections, eczema, and connective tissue abnormalities. Patients with HIES are prone to recurrent pyogenic and opportunistic infections due to impaired immune responses. Here, we present the case of an 11-year-old female diagnosed with HIES, who was admitted to the hospital with bacterial pneumonia and leg pain associated with a history of osteopenia. The patient’s clinical course included fever, cough, throat pain, and leg pain. Management involved a rigorous course of antibiotics, antifungals, and cultures of pertinent pathogens, along with imaging of the lower extremity. This case underscores the importance of appropriate management strategies for patients with HIES and their comorbidities to mitigate the risk of infections and improve patient outcomes.

## Linked entities

- **Diseases:** Hyper-IgE syndrome (MONDO:0018037), Job syndrome (MONDO:0007818), bacterial pneumonia (MONDO:0004652)

## Full-text entities

- **Genes:** IGHE (immunoglobulin heavy constant epsilon) [NCBI Gene 3497] {aka IgE}
- **Diseases:** bacterial pneumonia (MESH:D018410), osteopenia (MESH:D001851), HIES (MESH:D007589), fever (MESH:D005334), immunodeficiency (MESH:D007153), cough (MESH:D003371), connective tissue abnormalities (MESH:D003240), opportunistic infections (MESH:D009894), eczema (MESH:D004485), leg pain (MESH:D010146), infections (MESH:D007239)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11346673/full.md

## References

10 references — full list in the complete paper: https://tomesphere.com/paper/PMC11346673/full.md

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Source: https://tomesphere.com/paper/PMC11346673