HyperImmunoglobulin E Syndrome: Clinical and Immunologic Evaluation within One Family 41
نویسندگان
چکیده
منابع مشابه
Hyperimmunoglobulin E syndrome.
Hyperimmunoglobulin E (HIE) syndrome is a primary immunodeficiency disorder characterized by recurrent bacterial infections in presence of very high serum Ig E levels. We are reporting a nine-year-old child with HIE syndrome and reviewing literature on this disease.
متن کاملThe hyperimmunoglobulin E syndrome.
Hyperimmunoglobulin E syndrome is a primary immunodeficiency disease characterized by markedly high titers of serum immunoglobulin E (IgE), chronic eczema, recurrent staphylococcal infections, pneumatoceles, reduced neutrophil chemotaxis, and variable impaired T cell function. There are no clinical tools for diagnosis and definitive laboratory investigation. Variability of presentation makes it...
متن کاملHyperimmunoglobulin E syndrome: Genetics, immunopathogenesis, clinical findings, and treatment modalities
The hyperimmunoglobulin E syndromes (HIESs) are very rare immunodeficiency syndromes with multisystem involvement, including immune system, skeleton, connective tissue, and dentition. HIES are characterized by the classic triad of high serum levels of immunoglobulin E (IgE), recurrent staphylococcal cold skin abscess, and recurrent pneumonia with pneumatocele formation. Most cases of HIES are s...
متن کاملhyperimmunoglobulin e syndrome: genetics, immunopathogenesis, clinical findings, and treatment modalities
the hyperimmunoglobulin e syndromes (hiess) are very rare immunodeficiency syndromes with multisystem involvement, including immune system, skeleton, connective tissue, and dentition. hies are characterized by the classic triad of high serum levels of immunoglobulin e (ige), recurrent staphylococcal cold skin abscess, and recurrent pneumonia with pneumatocele formation. most cases of hies are s...
متن کاملHyperimmunoglobulin E syndrome associated with nephrotic syndrome.
A 21-year-old man was admitted to Kure National Hospital with nephrotic syndrome in September 1996. He had suffered from an intractable pruritic skin rash and recurrent subcutaneous abscesses caused by the hyperimmunoglobulin E syndrome since the age of 18 months. Renal biopsy gave a diagnosis of membranoproliferative glomerulonephritis. Steroid therapy decreased urinary protein loss and hypopr...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1998
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-199804001-00062