Korean J Med.  1999 Feb;56(2):209-214.

A Case of Osteogenesis Imperfecta associated with Aortic Regurgitation

Affiliations
  • 1Department of Internal Medicine, Sejong Hospital, Buchon, Korea.

Abstract

Osteogenesis imperfecta is one of the groups of hereditary disorders of connective tissue which includes the Ehlers-Danlos syndrome, the Marfan syndrome, pseudoxanthoma elasticum, and Hurler syndrome. While cardiovascular involvement is associated with each of these disorders, it is least common in osteogenesis imperfecta and is overshadowed by the bony, ocular, otologic, cutaneous, and dental manifestations that are characteristic of the disorder. In evaluating patients with osteogenesis imperfecta, careful attention should be paid to cardiovascular findings and if valvular lesions are noted, patients should be instructed regarding the need for antibiotic prophylaxis for dental and surgical procedures. We report a case of osteogenesis imperfecta associated with aortic regurgitation.

Keyword

osteogenesis imperfecta; aortic regurgitation

MeSH Terms

Antibiotic Prophylaxis
Aortic Valve Insufficiency*
Connective Tissue
Ehlers-Danlos Syndrome
Humans
Marfan Syndrome
Mucopolysaccharidosis I
Osteogenesis Imperfecta*
Osteogenesis*
Pseudoxanthoma Elasticum
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