Ehlers-Danlos syndrome associated with multiple pulmonary artery stenoses and tortuous systemic arteries

Ehlers-Danlos syndrome associated with multiple pulmonary artery stenoses and tortuous systemic arteries

A young girl with Ehlers-Danlos syndrome had evidence of multiple severe peripheral pulmonary artery stenoses and of tortuosity of the aorta and its major thoracic divisions, including the coronary arteries. It appears likely that the basic defect of Ehlers-Danlos syndrome may involve many arteries...

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Veröffentlicht in:The Journal of pediatrics 1969-12, Vol.75 (6), p.1031-1036
Hauptverfasser: Less, Martin H., Menashe, Victor D., Sunderland, Cecille O., Morgan, Clarence L., Dawson, Peter J.
Format: Artikel
Sprache:eng
Schlagworte:
Angiocardiography
Aorta - abnormalities
Aortography
Arteries - abnormalities
Child, Preschool
Coronary Vessel Anomalies
Ehlers-Danlos Syndrome - complications
Elastic Tissue - pathology
Female
Humans
Infant
Pulmonary Artery - diagnostic imaging
Pulmonary Artery - pathology
Skin - pathology
Skin Manifestations
Vascular Diseases - etiology
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Zusammenfassung:A young girl with Ehlers-Danlos syndrome had evidence of multiple severe peripheral pulmonary artery stenoses and of tortuosity of the aorta and its major thoracic divisions, including the coronary arteries. It appears likely that the basic defect of Ehlers-Danlos syndrome may involve many arteries and may cause important vasular obstruction.
ISSN:0022-3476
1097-6833
DOI:10.1016/S0022-3476(69)80342-2