Unusual Cause of Coagulopathy in a Child

Abstract

A 13-year-old girl diagnosed with progressive familial intrahepatic cholestasis type 2 during infancy and underwent internal biliary diversion at the age of seven, developed worsening pruritus in the last few weeks. At that time, she was on ursodeoxycholic acid 500 mg twice daily. Cholestyramine was added which showed no effect. Therefore, she was started on rifampicin 150 mg twice daily. Her baseline coagulation profile was normal with mildly elevated transaminases. Physical examination revealed an active, well-grown girl with generalized icterus. She had scratch marks over both forearms. She has no other peripheral stigmata of chronic liver disease. Abdominal examination revealed a surgical scar from the previous internal biliary diversion surgery. There was no palpable hepatosplenomegaly. Two weeks into treatment with rifampicin, her pruritus improved dramatically. However, her coagulation profile was significantly deranged. Prothrombin time was 66 s (normal range: 10.5–12.7 s), activated partial thromboplastin time was 116 s (normal range: 25–37.7 s), and the international normalized ratio was 6.7 (normal range: 0.90–1.10). The detailed coagulation factor assay revealed a low level of vitamin K-dependent factors: factor II at 0.073 IU /mL (normal range: 0.61–1.04), factor VII at 0.085 IU/mL (normal range: 0.6–1.15), factor IX at 0.12 IU/mL; (normal range: 0.6–1.2), and factor X at 0.06 IU/mL (normal range: 0.5–1.17). Consent for publication was taken from the patient’s guardians.

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