2007
DOI: 10.1016/s1081-1206(10)60887-3
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Hyperimmunoglobulinemia E syndrome associated with coronary artery aneurysms: deficiency of central memory CD4+ T cells and expansion of effector memory CD4+ T cells

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Cited by 24 publications
(24 citation statements)
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“…Coronary artery abnormalities (notably aneurysms) have been previously reported in patients with AD-HIES, including a recently studied group of 26 STAT3-deficient subjects. 9,10,12,13 Cerebral vascular events (stroke, retinal occlusion, and internal carotid aneurysm) were first reported in 3 patients with AD-HIES, 1 and 5 other cases with lacunar infarction were subsequently identified. 8 In the present cohort (in which all the patients had genetically confirmed STAT3 deficiency), it is noteworthy that all anatomic areas appear to be potentially affected, with a trend toward more damage in small-and medium-diameter vessels.…”
Section: Chandesris Et Al Vascular Abnormalities In Human Stat3 Deficmentioning
confidence: 99%
“…Coronary artery abnormalities (notably aneurysms) have been previously reported in patients with AD-HIES, including a recently studied group of 26 STAT3-deficient subjects. 9,10,12,13 Cerebral vascular events (stroke, retinal occlusion, and internal carotid aneurysm) were first reported in 3 patients with AD-HIES, 1 and 5 other cases with lacunar infarction were subsequently identified. 8 In the present cohort (in which all the patients had genetically confirmed STAT3 deficiency), it is noteworthy that all anatomic areas appear to be potentially affected, with a trend toward more damage in small-and medium-diameter vessels.…”
Section: Chandesris Et Al Vascular Abnormalities In Human Stat3 Deficmentioning
confidence: 99%
“…Vascular abnormalities are common in HIES (21–23). One man suffered a myocardial infarction due to a large left anterior descending coronary artery aneurysm (22).…”
Section: Clinical Manifestations (Table 1)mentioning
confidence: 99%
“…Leukocytosis and acute phase reactants, such as C-reactive protein during infection, may not be as high as expected. Lymphocyte phenotyping shows decreased memory T cells, and antibody responses are variable (3, 23, 27). …”
Section: Clinical Manifestations (Table 1)mentioning
confidence: 99%
“…Minimal trauma fractures occur as well as osteoporosis, although these two findings appear to be independent of each other. Vascular abnormalities, including intracranial aneurysms and coronary artery ectasia, tortuosity, and aneurysms are common [15,16]. Asymptomatic cerebral T2-weighted hyperintensities, increased prevalence of lacunar infarcts, and increased Arnold Chiari 1 malformations are seen on the brain MRIs of many patients [17][18][19].…”
Section: Autosomal Dominant and Sporadic Stat3 Deficiencymentioning
confidence: 99%