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Cryptococcal meningitis in a child with hyperimmunoglobulin E syndrome
B Z Garty1, B Wolach, S Ashkenazi
1Kipper Institute of Pediatric Allergy and Immunology, Department of Pediatrics, Petah Tiqva, Israel.
Abstract:
A 13-year-old boy with hyperimmunoglobulin E (hyper-IgE) syndrome presented with headache, blurred vision, photophobia and bilateral papilledema due to cryptococcal meningitis. Treatment with amphotericin B, and S-fluorocytosine for several weeks and repeated lumbar punctures did not reduce the intracranial pressure, and a myeloperitoneal shunt was performed. The child was maintained on fluconazole for an additional six months. Patients with hyper-IgE syndrome are at increased risk of opportunistic fungal infections such as cryptococcal meningitis.
Insights
Patients with hyperimmunoglobulin E syndrome face increased risks of fungal infections like cryptococcal meningitis. This case highlights challenges in managing increased intracranial pressure in such infections.
Area of Science:
- Infectious Diseases
- Immunology
- Neurology
Background:
- Hyperimmunoglobulin E (hyper-IgE) syndrome is a primary immunodeficiency associated with recurrent sinopulmonary infections and eczema.
- Individuals with hyper-IgE syndrome have a compromised immune system, increasing susceptibility to opportunistic infections.
- Cryptococcal meningitis is a serious fungal infection of the central nervous system.
Observation:
- A 13-year-old male with hyper-IgE syndrome presented with symptoms of increased intracranial pressure, including headache, blurred vision, and papilledema.
- Cerebrospinal fluid analysis confirmed cryptococcal meningitis.
Findings:
- Standard treatment with amphotericin B and flucytosine, along with repeated lumbar punctures, failed to adequately reduce intracranial pressure.
- A myeloperitoneal shunt was surgically placed to manage refractory intracranial hypertension.
- The patient required six months of fluconazole maintenance therapy for cryptococcal meningitis.
Implications:
- This case underscores the significant risk of severe opportunistic fungal infections, such as cryptococcal meningitis, in individuals with hyper-IgE syndrome.
- Management of intracranial pressure in cryptococcal meningitis can be challenging and may necessitate invasive procedures like shunting.
- Prolonged antifungal therapy is crucial for successful treatment and prevention of relapse in immunocompromised patients.