Hyperammonemic Coma in a Post-partum Patient with Undiagnosed Urea Cycle Defect

Overview

Journal Indian J Crit Care Med

Specialty Critical Care

Date 2013 Aug 29

PMID 23983417

Authors

Sananta Kumar Dash

Munish Chauhan

Vishakh Varma

Rakesh Sharma

Sudha Kansal

Rajesh Chawla

Affiliations

Soon will be listed here.

Abstract

Urea cycle disorders (UCD) are common during neonatal period, and it is rarely reported in adults. We are reporting a patient presenting with post-partum neuropsychiatric symptoms rapidly progressing to coma. Markedly raised serum ammonia level on presentation with an initial normal magnetic resonance imaging (MRI) of brain and normal liver function tests led to the suspicion of UCD, which was confirmed on the basis of urine orotic acid and elevated serum amino acid levels. We had to resort to hemodialysis to correct the hyperammonemic coma, which was unresponsive to conventional anti-ammonia measures. She exhibited remarkable improvement with a progressive decline in serum ammonia with repeated hemodialysis and made a full recovery. Timely diagnosis and early institution of hemodialysis in the setting of a poor neurological status maybe considered a suitable treatment option.

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