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Mesenteric venous thrombosis in a child with type 2 protein S deficiency
Takahiro Hayakawa1, Akira Morimoto, Yasuyuki Nozaki
1Department of Pediatrics, Jichi Medical University School of Medicine, Shimotsuke, Tochigi, Japan.
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.
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