نتایج جستجو برای: hyperimmunoglobulin e syndrome
تعداد نتایج: 1604051 فیلتر نتایج به سال:
A 13-year-old girl presented with multiple skin abscesses. She was diagnosed as having juvenile dermatomyositis (DM) at the age of 7 years. She had suffered from recurrent skin infections, atypical pruritic dermatitis and pneumonia since the age of 8 years. Bacteriologic and fungal cultures for skin abscesses and oral mucosa were positive S. aureus and C. albicans, respectively. Chemotactic def...
The hyper-IgE syndromes are rare, complex primary immunodeficiencies characterized by clinical manifestation diversity, by particular susceptibility to staphylococcal and mycotic infections as well as by a heterogeneous genetic origin. Two distinct entities--the classical hyper-IgE syndrome which is inherited in an autosomal dominant pattern and the autosomal recessive hyper-IgE syndrome--have ...
BACKGROUND Signal transducer and activator of transcription 3 (STAT3) deficiency is responsible for autosomal dominant hyperimmunoglobulin E syndrome, characterized by recurrent bacterial and fungal infections, connective tissue abnormalities, hyperimmunoglobulin E, and Th17 lymphopenia. Although vascular abnormalities have been reported in some patients, the prevalence, characteristics, and et...
A 19-year-old male presented to the emergency department with severe hypoxic respiratory distress. He had a history of hyperimmunoglobulin E syndrome (HIES) diagnosed at the age of 24 months. He had recurrent skin abscesses with Staphylococcus aureus and multiple respiratory infections with S. aureus and Pseudomonas aeruginosa. He had also developed bronchiectasis and required left lower lobe l...
Members of the serine protease, matrix metalloproteinase, and cysteinyl protease families have been found to be associated with lung in ammation and airway extracellular matrix [1]. The serine protease neutrophil elastase induces protease and tissue destruction in the lung [1]. Protease-antiprotease imbalance in humans has been described in chronic destructive lung diseases such as cystic fibro...
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