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...

We present a case of hyperimmunoglobulin E (hyper-IgE) syndrome in a three year old boy. There are many pitfalls in diagnosing this disease in the very young population, mainly due to the ambiguity of some diagnostic criteria in this population. Recognizing this syndrome early in life can potentially be very beneficial to the patients involved and the medical system as a whole. Early diagnosis ...

Introduction The syndrome of hyper-IgE syndrome or Job syndrome or Burckley syndrome, is a rare primary immunodeficiency characterized by defective phagocytic manifesting recurrent infections, mainly Staphylococcal associated with eczema severe atopic, musculoskeletal disorders, pathological fractures, scoliosis and levels high IgE (> 2.000UI / ml). Among the immunological characteristics, we c...

Hyper-immunoglobulin E syndromes (HIES) including compound primary immunodeficiency and nonimmunological abnormalities are characterized by extremely high serum IgE levels, eosinophilia, eczema, susceptibility to infections, distinctive facial appearance, retention of deciduous teeth, cyst-forming pneumonias, and skeletal abnormalities. Itis reported that some cases of familial HIES are relativ...