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Published on: April 21, 2017
Transient antiphospholipid syndrome in an infant with segmental small bowel infarction
B Haluk Güvenç1, Nazan Sarper, Ayşe Tuzlaci
1Department of Pediatric Surgery, Kocaeli University School of Medicine, Kocaeli, Turkey.
Insights
This case study details a rare instance of transient antiphospholipid syndrome causing intestinal infarction in an infant. The child recovered fully, highlighting the potential for temporary antiphospholipid antibodies in pediatric mesenteric thrombosis.
Area of Science:
- Pediatric Gastroenterology
- Hematology
- Immunology
Background:
- Antiphospholipid syndrome (APS) is characterized by thrombosis with antiphospholipid antibodies.
- Mesenteric thrombosis is a rare but serious condition in children.
Observation:
- A 5-month-old infant presented with acute abdominal symptoms suggestive of bowel obstruction.
- Surgical intervention revealed a 15 cm segment of gangrenous ileum.
Findings:
- Post-operative investigations identified transient anticardiolipin antibodies (IgG) and a decreased protein C level.
- Other thrombophilia markers, including Factor V Leiden and prothrombin gene mutations, were negative.
- Anticardiolipin antibodies normalized within 8 months, with no recurrence of thrombosis over 24 months.
Implications:
- This case represents the first reported instance of segmental intestinal infarction due to transient antiphospholipid syndrome in a pediatric patient.
- It underscores the importance of considering and investigating antiphospholipid antibodies in pediatric mesenteric thrombosis, even if transient.
- The findings suggest that some pediatric cases of APS-related thrombosis may resolve spontaneously without long-term anticoagulation.
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.
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