Hyper Ige Recurrent Infection Syndrome 5 Autosomal Recessive C5394550
A severe immune deficiency where the body cannot produce normal protective antibodies. Patients suffer repeated infections affecting skin, lungs, and other organs. Lifelong infection prevention and immune support are essential.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Pathological fractures due to bone fragility
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent severe bacterial infections
(immunologic)Severe eczema with markedly elevated IgE
(skin)Congenital cardiac anomalies (e.g., ventricular septal defect)
(cardiovascular)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Aggressive Infection Management
Prompt initiation of broad-spectrum antimicrobials for any suspected infection, given the severe immunodeficiency and high mortality risk in autosomal recessive Hyper-IgE syndrome.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement Therapy
IV or subcutaneous immunoglobulin supplementation to provide passive humoral immunity against common pathogens when endogenous antibody production is deficient.
Additional treatment options
Nutritional Support for Failure to Thrive
High-calorie enteral nutrition with micronutrient supplementation to address the severe growth failure and catabolic state associated with recurrent infections.
dietaryAntimicrobial Air Filtration and Environmental Control
HEPA filtration, UV sterilization, and careful air quality management in living spaces to reduce environmental pathogen exposure for these severely immunocompromised patients.
complementaryHematopoietic Stem Cell Transplant Evaluation
Comprehensive transplant candidacy assessment for definitive immune reconstitution, as this represents the only curative approach for severe autosomal recessive Hyper-IgE syndrome.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Hyper Ige Recurrent Infection Syndrome 5 Autosomal Recessive C5394550?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy