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suck abstract from ncbi


10.1016/j.ccm.2016.04.016

http://scihub22266oqcxt.onion/10.1016/j.ccm.2016.04.016
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C4987538!4987538!27514600
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suck abstract from ncbi

pmid27514600      Clin+Chest+Med 2016 ; 37 (3): 557-67
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  • Hyper IgE Syndromes and the Lung #MMPMID27514600
  • Freeman AF; Olivier KN
  • Clin Chest Med 2016[Sep]; 37 (3): 557-67 PMID27514600show ga
  • Elevated serum IgE has many etiologies including parasitic infection, allergy and asthma, malignancy and immune dysregulation. The hyper IgE syndromes due to mutations in STAT3, DOCK8 and PGM3 are monogenic primary immunodeficiencies that are associated with high IgE, eczema and recurrent infections. All of these primary immunodeficiencies are associated with recurrent pneumonias leading to bronchiectasis; however each has unique features and genetic diagnosis is essential in guiding therapy, discussing family planning and defining prognosis. STAT3 mutated HIES is unique with respect to its multi-organ features and frequent pneumatocele formation. DOCK8 deficiency is characterized by severe cutaneous viral infections and early mortality. PGM3 deficiency is newly defined and is distinct in having neurologic abnormalities and cytopenias.
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