Abstract: The data of 4 children with hyperimmunoglobulin E syndrome (HIES) from Jan. 2010 to Jul. 2019, including clinical manifestations, laboratory test results, treatments and prognoses, was retrospectively analyzed. The age of the patients at onset of the disease was younger than 6 months. All the patients presented with multiple infections. The most common infections were skin and respiratory tract. One case had severe mitral incompetence with myocardial infarction. One case had renal abscess. Three cases had liver abscess and were alleviated after antibiotic treatment and abscess drainage. One case was treated with continuous trimethoprim-sulfamethoxazole and had a remission. HIES should be suspected when a child suffering from repeated infection of undetermined origin and screening for HIES-related genes should be performed as soon as possible. The targeted anti-infection therapy can be given as early as possible after diagnosed.

Key words: high IgE syndrome, infection, cardiac abnormalities