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AJR Am J Roentgenol. 2009 Dec;193(6):W478-81. doi: 10.2214/AJR.09.2623.

Coronary abnormalities in hyper-IgE recurrent infection syndrome: depiction at coronary MDCT angiography.

Author information

1
Department of Radiology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Clinical Research Center, Bethesda, MD 20892, USA. agharib@mail.nih.gov

Abstract

OBJECTIVE:

Hyper-IgE recurrent infection syndrome (HIES or Job's syndrome) is a rare disorder affecting the immune system and connective tissues. The purpose of this study is to describe the coronary abnormalities in genetically confirmed HIES patients as depicted by coronary MDCT angiography (MDCTA).

CONCLUSION:

Coronary MDCTA has provided an opportunity for noninvasive evaluation of the coronary arteries in patients with HIES. These coronary abnormalities vary from tortuosity to ectatic dilation and focal aneurysms of the coronary arteries. Such an evaluation has potential value in identifying new aspects of this disease and thereby providing better understanding of the pathophysiology of the disorder.

PMID:
19933621
PMCID:
PMC4103904
DOI:
10.2214/AJR.09.2623
[Indexed for MEDLINE]
Free PMC Article
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