Cardiac thrombus in Omenn syndrome.

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

Pediatric cardiology, cilt.26, sa.5, ss.694-7, 2005 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 26 Sayı: 5
  • Basım Tarihi: 2005
  • Doi Numarası: 10.1007/s00246-005-0868-9
  • Dergi Adı: Pediatric cardiology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.694-7
  • Bursa Uludağ Üniversitesi Adresli: Evet

Özet

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.