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Extrahepatic portal hypertension due to familial protein S deficiency

Ira Shah1, Sushmita Bhatnagar

  • 1Department of Pediatric Hepatobiliary Clinic, B J Wadia Hospital for Children, Parel, Mumbai 400 012, India. irashah@pediatriconcall.com

Indian Pediatrics
|January 31, 2009
PubMed

Insights

Hereditary Protein S deficiency can cause portal vein thrombosis (PVT) in children, leading to portal hypertension. This study identified familial Protein S deficiency as the primary cause in an 8-year-old girl with portal cavernoma.

Area of Science:

  • Pediatric Gastroenterology
  • Hematology
  • Vascular Medicine

Background:

  • Portal vein thrombosis (PVT) is a significant cause of portal hypertension in pediatric populations.
  • Idiopathic PVT in children is often linked to Protein C deficiency or antiphospholipid antibodies.

Observation:

  • A case of an 8-year-old Indian girl presenting with portal cavernoma is described.
  • The patient was diagnosed with hereditary Protein S deficiency.

Findings:

  • Hereditary Protein S deficiency was identified as the underlying cause of portal vein thrombosis in this child.
  • Familial screening revealed asymptomatic Protein S deficiency in two siblings, supporting a genetic etiology.

Implications:

  • This case highlights hereditary Protein S deficiency as a critical, often overlooked, cause of pediatric PVT.
  • Early diagnosis and genetic screening for Protein S deficiency can prevent severe complications of portal hypertension in children.

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