[Pulmonary manifestations of pediatric hyper IgE syndrome]

Hyper IgE syndrome is a rare primary immune deficiency disorder characterized by pulmonary and cutaneous infection, eczema, and elevated serum IgE levels. In this article, 4 patients with hyper IgE syndrome and recurrent pulmonary infection were reported. Four girls with history of recurrent pulmonary infections were worked up and hyper IgE syndrome was diagnosed for them. All patients had recurrent pulmonary infections including pneumonia, bronchiectasis, pulmonary abscess, hydropneumothorax. Serum IgE levels were greater than 2000 Iu/ml in all cases. Microbial cultures showed staphylococcus aureas and pseudomans aeroginosa in two cases. All patients respond well to the wide spectrum intravenous antibiotics. Extra-pulmonary manifestations including purulent lymphadenitis, and skin and brain abcesses were observed. Hyper IgE syndrome causes recurrent pulmonary and extra-pulmonary infections which response fairly well to wide spectrum antibiotic therapy

Pulmonary manifestations of hyper IgE syndrome: case series and literature review

This study aimed to present four children suffering from recurrent pulmonary infections in the context of hyper lgE syndrome. All patients had recurrent pulmonary infections including pneumonia, bronchiectasis, pulmonary abscess and hydropneumothorax. Serum IgE level was greater than 2000 IU/ml in all cases. Microbial cultures showed Staphylococcus aureus and Pseudomonas aeruginosa in two cases. All responded well to the wide spectrum intravenous antibiotics. Extra-pulmonary manifestations included purulent lymphadenitis as well as skin and brain abscesses. Hyper IgE syndrome causes recurrent pulmonary and extra-pulmonary infections which respond fairly well to wide spectrum antibiotic therapy