H. H. KESARWALA*, R. V. S. K. PRASADt, R. SZEP, E. OLDMAN, S. LANE & P. S. PAPAGEORGIOU The Department ofPediatrics, CMDHJ, Rutgers Medical School, Piscataway, New Jersey, * Division ofAllergy Immunology, Children's Hospital Medical Center, Elland and Bethesda Avenue, Cincinnati, Ohio 45229 and t Division ofAllergy, Department ofPediatrics, Children's Hospital ofMichigan, 3901 Beaubien Boulevar...

Hyper IgE Syndrome (HIES) is an immunodeficiency disorder characterized by increased serum levels of IgE, eczema, and recurrent cutaneous and pulmonary infections. In this report, we present, to our knowledge, the first documented case of renal cell carcinoma (RCC) found in a patient with HIES. The patient received infectious disease clearance prior to obtaining a partial nephrectomy which reve...

Article type: Review Article Hyper IgE Syndrome (HIES) is a rare primary immunodeficiency disease. Most of HIES cases are sporadic. Autosomal dominant HIES is caused by mutation in signal transducer and activator of transcription-3 (STAT-3). A number of mosaicism HIES has been reported that is associated with intermediate phenotype. Autosomal recessive HIES is due to mutation in Dock-8 or cytok...

Understanding how class switch recombination (CSR) is regulated to produce immunoglobulin E (IgE) has become fundamental because of the dramatic increase in the prevalence of IgE-mediated hypersensitivity reactions. CSR requires the induction of the enzyme AICDA in B cells. Mutations in AICDA have been linked to Hyper-IgM syndrome (HIGM2), which shows absence of switching to IgE as well as to I...

Background The measurement of total serum IgE is made in some cases to investigate if there is the occurrence of allergic processes. However, does not identify specific antibodies. When it is high may indicate an allergic process, or the ability to initiate some. However, increased IgE levels may also indicate other problems such as parasitic diseases, IgE myeloma, hyper-IgE syndrome and some s...

The status of suppressor T cells (Ts) was assessed in seven children with the hyper IgE syndrome (recurrent staphylococcal infections, eczematous skin rash, and elevated serum IgE) to determine whether a deficiency in Ts is associated with increased IgE synthesis. When circulating T cells and their subsets were enumerated with the aid of monoclonal antibodies that identify T cells (T3), helper/...