| Literature DB >> 27693026 |
Yael Gernez1, Angela Tsuang1, Tukisa D Smith1, Khurram Shahjehan2, Yiqun Hui1, Paul J Maglione1, Charlotte Cunningham-Rundles3.
Abstract
Recurrent pneumonia with cavitation leading to pneumatoceles, secondary fungal infections, and hemoptysis are major causes of mortality and morbidity in patients with hyper-IgE syndrome. Prevention and aggressive treatment of pneumonia in these patients are essential to prevent further lung damage, but treatment may be delayed because the classic signs/symptoms of infection such as fever, chills, or rigors may be lacking. Early imaging to identify infection is essential for diagnosis and treatment. The mainstay of therapy is continuous, full-dose daily trimethoprim-sulfamethoxazole and commonly fungal coverage. Because hyper-IgE syndrome is a progressive disease, patients' condition may worsen despite compliance with prophylactic therapy.Entities:
Keywords: Aspergilloma; Eczema; Immunodeficiency; STAT3; hyper-IgE syndrome
Mesh:
Substances:
Year: 2016 PMID: 27693026 PMCID: PMC5322743 DOI: 10.1016/j.jaip.2016.08.003
Source DB: PubMed Journal: J Allergy Clin Immunol Pract