Cardiac thrombus in Omenn syndrome.

Kilic S. Ş., Cil E., Meral A., Villa A.

Pediatric cardiology, vol.26, no.5, pp.694-7, 2005 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 26 Issue: 5
  • Publication Date: 2005
  • Doi Number: 10.1007/s00246-005-0868-9
  • Journal Name: Pediatric cardiology
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.694-7
  • Bursa Uludag University Affiliated: Yes

Abstract

Omenn syndrome is characterized by a generalized erythematous skin rash, lymph node enlargement, hepatosplenomegaly, Increased serum IgE levels, eosinophilia, and evidence of severe combined immune deficiency. Patients develop fungal, bacterial, and viral infections. We present the case of a 3-month-old girl with Omenn syndrome who developed right ventricular thrombosis. Echocardiographic study revealed a round structure that filled the apex and corpus of the the right ventricle. We investigated this patient for hypercoagulation and made a diagnosis of ventricular thrombosis, which is an uncommon finding in Omenn syndrome.