| Sweet syndrome, cutis laxa, and fatal cardiac manifestations in a 2-year-old girl. | |
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MedLine Citation:
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PMID: 21720474 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A 2-year-old black girl was diagnosed with Sweet syndrome and cutis laxa, and she was given corticosteroid therapy. At that presentation, a cardiac evaluation revealed nothing unusual. Nine days later, she emergently presented with respiratory distress, and circulatory collapse rapidly developed. A 2-dimensional Doppler echocardiogram showed a dilated and poorly contractile left ventricle, severe aortic regurgitation, and a large aneurysm of the sinus of Valsalva. Despite resuscitative efforts, the child died. Inspection on autopsy revealed a markedly enlarged heart and 2 large aneurysms of the sinus of Valsalva. Histologic analysis disclosed acute necrosis in the cardiac apex and interventricular septum, and focal chronic inflammatory and granulation tissue in the aortic wall.Because cardiac lesions may remain clinically silent, we recommend that children with Sweet syndrome and cutis laxa undergo complete cardiac evaluation, including 2-dimensional Doppler echocardiography, by a pediatric cardiologist. Subsequent monitoring is also appropriate. Early recognition and aggressive therapeutic treatment could prevent fatal cardiac complications. |
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Authors:
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Malini Guhamajumdar; Brojendra Agarwala |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital Volume: 38 ISSN: 1526-6702 ISO Abbreviation: Tex Heart Inst J Publication Date: 2011 |
Date Detail:
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Created Date: 2011-07-01 Completed Date: 2011-10-31 Revised Date: 2012-01-23 |
Medline Journal Info:
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Nlm Unique ID: 8214622 Medline TA: Tex Heart Inst J Country: United States |
Other Details:
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Languages: eng Pagination: 285-7 Citation Subset: IM |
Affiliation:
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Section of Pediatric Cardiology, University of Chicago Comer Children's Hospital, Chicago, Illinois 60637-1470, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adrenal Cortex Hormones
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therapeutic use Aortic Aneurysm Aortic Valve Insufficiency / etiology Autopsy Child, Preschool Cutis Laxa / complications*, diagnosis, drug therapy Echocardiography, Doppler Fatal Outcome Female Heart Diseases / etiology*, pathology, ultrasonography Humans Sinus of Valsalva Sweet Syndrome / complications*, diagnosis, drug therapy Ventricular Dysfunction, Left / etiology |
| Chemical | |
Reg. No./Substance:
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0/Adrenal Cortex Hormones |
| Comments/Corrections | |
Comment In:
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Tex Heart Inst J. 2011;38(5):608-9
[PMID:
22163152
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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