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Case report

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  2. Case report
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A 16-year-old Caucasian boy presented with a 32-h history of priapism; it was painless but had become uncomfortable. He was mildly asthmatic but otherwise well. Partial detumescence was initially achieved with cavernosal aspiration and ephedrine injection. His priapism continued and he had a saphenocorporal shunt/circumcision 48 h after admission. The priapism resolved over the next few days but he continued to have low-grade pyrexia. He developed symptoms and signs of a pulmonary embolism although his V:Q scan was within normal limits. His symptoms improved with heparinization. A thrombophilia screen revealed he was heterozygous for factor V Leiden, as was his mother. He was commenced on life-long warfarin. He has had no erectile function since the priapism.

Comment

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  2. Case report
  3. Comment
  4. References

Thrombophilia refers to familial or acquired disorders of the haemostatic system that result in an increased risk of venous thrombosis. Resistance to activated protein C (APC) is the most common inherited risk factor for venous thrombosis. Most cases of APC resistance are caused by a point mutation in the factor V gene, referred to as the factor V Leiden mutation; the mutation carrier rate is 4.4% [1]. While this may not fully account for this boy’s priapism, considering thrombophilia as an aetiological factor has important implications for treatment. It may be that early anticoagulants are needed. Thrombophilia screening may increase our knowledge about so-called idiopathic priapism, which is usually considered responsible for one-third of all cases [2]. To the authors’ knowledge, no link between priapism and resistance to APC has previously been described. No association between priapism and other well established thrombophilic defects such as anti-thrombin, protein C or S deficiency, has been reported.

References

  1. Top of page
  2. Case report
  3. Comment
  4. References