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Case Report
9 (
1
); 65-67

A Case report: Abernethy syndrome type-2.

Professor & Head of unit, Department of Paediatrics, Civil hospital, Ahmedabad.
Assiatant professor, Department of Paediatrics, Civil hospital, Ahmedabad.
First year resident, Department of Paediatrics, Civil hospital, Ahmedabad.

*Corresponding Author: Dr. Aditi Parekh Email: - aditi_parekh92@hotmail.com

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This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

Abstract

We are reporting a case of Abernethy syndrome type 2, a rare disorder of the splanchnic venous system. Abernethy syndrome consists of congenital porto-systemic shunts that are a result of persistence of embryonic vessels. A 6 year old child presented to us with generalised weakness involving all four limbs with bilateral ptosis followed by altered sensorium and flapping tremors. Investigations were suggestive of hyperammonemia and coagulopathy. CT angiography of abdomen was suggestive of Abernethy syndrome type 2.

Keywords

Abernethy syndrome
Hyperammonemia
Splanchnic venous system

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