Hyperimmunoglobulin E syndrome (Job syndrome): chest CT findings

Santana, Pablo Rydz Pinheiro; Medeiros, Augusto Kreling; Barbisan, Cinthia Callegari; Gomes, Antônio Carlos Portugal; Marchiori, Edson

doi:10.1590/s1806-37562017000000175

Cited by 2 publications

(1 citation statement)

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“…Nonimmunological Characteristics include distinctive face characteristics that first show in early adolescence or earlier. 2,4 Reviewing documented lymphoma cases in people with Job's syndrome reveals a higher relative risk, particularly for class C Hodgkin and mature B cell lymphomas. 5 Proper skin care, infection prevention, and aggressive infection treatment are the cornerstones of HIES therapy.…”

Section: Discussionmentioning

confidence: 99%

Hyper Immunoglobulin E (IgE); an immune deficiency (Job Syndrome)

Ismail

Khurshid

2023

JSTMU

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Hyper-IgE Syndrome (HIES), also known as Job Syndrome; is a rare genetic disorder that affects the immune system and can lead to various health complications. The text mentions cornerstone of HIES therapy is proper skin care, infection prevention, and aggressive infection treatment. Treatments for HIES can include antibiotics that target S. aureus, typically used to reduce the incidence of pneumonia and the risk of lung damage. Skincare routines can involve bleach baths or chlorhexidine washes, oral anti-staphylococcal prophylactic antibiotics, and other treatments to avoid abscesses. Antifungal prophylaxis may be beneficial for HIES patients who have chronic Candida infections. Defects in antibody synthesis can vary, making it difficult to provide general recommendations for immune globulin replacement in HIES.

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Section: Discussionmentioning

confidence: 99%