Mesenteric venous thrombosis in a child with type 2 protein S deficiency

J Pediatr Hematol Oncol. 2011 Mar;33(2):141-3. doi: 10.1097/MPH.0b013e3181fce4d4.

Abstract

A 5-year-old girl presented with abdominal pain and bloody stools 2 weeks after suffering from influenza A infection. Enhanced computed tomographic scan showed widespread splanchnic venous thrombosis and small intestine necrosis. She recovered after the necrotic bowel was resected. The patient continues to receive anticoagulant therapy. Thrombophilia screening after the complete resolution consistently showed mildly decreased protein S (PS) activity with normal PS antigen levels. Sequence analysis detected a heterozygous K196E mutation in the PROS1 gene. Type 2 PS deficiency was diagnosed. This is the first report of mesenteric vein thrombosis in a child with a type 2 PS deficiency.

Publication types

  • Case Reports

MeSH terms

  • Antiviral Agents / therapeutic use
  • Blood Proteins / genetics
  • Child, Preschool
  • Female
  • Humans
  • Influenza A virus
  • Influenza, Human / complications
  • Influenza, Human / drug therapy
  • Intestines / blood supply
  • Intestines / surgery
  • Ischemia / genetics
  • Ischemia / surgery
  • Mesenteric Vascular Occlusion / genetics*
  • Mesenteric Vascular Occlusion / surgery
  • Mutation
  • Oseltamivir / therapeutic use
  • Protein S
  • Protein S Deficiency / complications*
  • Protein S Deficiency / genetics
  • Venous Thrombosis / genetics*
  • Venous Thrombosis / surgery

Substances

  • Antiviral Agents
  • Blood Proteins
  • PROS1 protein, human
  • Protein S
  • Oseltamivir