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Hyper IgE Syndrome, emphasis on radiological findings: A case of pulmonary complications in a pediatric patient


Journal of Pediatrics & Neonatal Care
Jennifer Richardson Maturana,1 Nathalia Jacome Perez,2 Luz Ángela Moreno Gómez,3 Rubén Danilo Montoya Cárdenas,3 Lina Maria Castaño Jaramillo,4 Carlos Enrique Camargo Crespo5

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Abstract

Background: STAT3 hyper IgE Syndrome (STAT3-HIES), is a rare immunodeficiency that affects multiple organ systems. A clinical triad characterizes it and includes recurrent skin abscesses, frequent respiratory tract infections, and elevated levels of immunoglobulin E in the blood.


Case report: A 9-year-old female patient with STAT3-HIES was brought to the emergency department for cold abscesses. During her assessment, the patient was diagnosed with a large pneumatocele and cystic lung lesions detected by a CT scan. The patient underwent a successful surgical intervention, without complications.

Conclusions: This case underscores the importance of performing a complete and prompt evaluation along with multidisciplinary management in STAT3-HIES patients to prevent potentially life-threatening complications.

Keywords

hyper-IgE syndrome, job syndrome, signal transducer and activator of transcription 3 (STAT3)

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