Transient antiphospholipid syndrome in an infant with segmental small bowel infarction

J Pediatr Surg. 2004 Jan;39(1):124-7. doi: 10.1016/j.jpedsurg.2003.09.026.

Abstract

The clinical picture of venous or arterial thrombosis in the presence of circulating antiphospholipid antibodies is referred to as the antiphospholipid syndrome. A 5-month-old baby girl who was quite healthy so far was referred to our clinic with irritability, vomiting, and abdominal distension for 30 hours. Surgical exploration exposed a gangrenous ileal segment about 15 cm long. The postoperative period was unremarkable. Investigation to identify the risk factors for mesenteric thrombosis found anticardiolipin antibodies (isotype Ig G) and decreased protein C level. Protein S and antithrombin III were within normal levels. Hb electrophoresis results showed no HbS, and neither Factor V Leiden nor prothrombin 20210 mutations were detected. Eight months postoperatively, anticardiolipin antibodies were found within normal levels. Lupus anticoagulant, ds DNA, and ss DNA were negative. Direct coombs test and protein C, C3, and C4 were also within normal levels. She had no thrombotic episode in the 24 months postoperatively, although no anticoagulant medication was administered. To the authors' knowledge this case is the first report of segmental intestinal infarction in transient antiphospholipid syndrome in the pediatric population.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Anticardiolipin / blood
  • Antiphospholipid Syndrome / complications*
  • Antiphospholipid Syndrome / diagnosis
  • Female
  • Humans
  • Ileum / blood supply*
  • Infant
  • Infarction / etiology*
  • Protein C / analysis

Substances

  • Antibodies, Anticardiolipin
  • Protein C