Hyperammonemic Encephalopathy in An Adenocarcinoma Patient Managed with Carglumic Acid

Lazier, J.; Lupichuk, S.M.; Sosova, I.; Khan, A.A.

doi:10.3747/co.21.2076

Open AccessCase Report

Hyperammonemic Encephalopathy in An Adenocarcinoma Patient Managed with Carglumic Acid

by

J. Lazier

^1,*,

S.M. Lupichuk

²,

I. Sosova

³ and

A.A. Khan

^1,4

¹

Department of Medical Genetics, Alberta Children’s Hospital, University of Calgary, Calgary, AB, Canada

²

Department of Oncology, Tom Baker Cancer Centre, University of Calgary, Calgary, AB, Canada

³

Biochemical Genetics Laboratory, Alberta Children’s Hospital, University of Calgary, Calgary, AB, Canada

⁴

Department of Pediatrics, Alberta Children’s Hospital, University of Calgary, Calgary, AB, Canada

^*

Author to whom correspondence should be addressed.

Curr. Oncol. 2014, 21(5), 736-739; https://doi.org/10.3747/co.21.2076

Submission received: 9 July 2014 / Revised: 4 August 2014 / Accepted: 7 September 2014 / Published: 1 October 2014

Download

Abstract

Hyperammonemic encephalopathy (HE) is a rare complication of malignancy and chemotherapy. Although the cause of HE is unclear, a functional arginine deficiency secondary to increased catabolism has been suggested as a possible mechanism. Either that deficiency or an undetermined metabolite could lead to inhibition of N-acetylglutamate synthase (NAGS), a urea cycle enzyme, resulting in hyperammonemia. We present a case of chemotherapy-induced HE in a patient with no underlying primary urea cycle disorder. The patient had a successful trial of carglumic acid (a synthetic analog of the product of NAGS), which suggests that, at least in some cases, HE can be treated by overcoming proximal inhibition of the urea cycle. Further, our case is the first in the literature to exclude genetic defects and disorders of the proximal urea cycle, suggesting that hyperammonemia in these patients is probably secondary to chemotherapy.

Keywords: carglumic acid; idiopathic hyperammonemic encephalopathy; hyperammonemia; carbamoyl phosphate synthase; chemotherapy; drug toxicity; N-acetylglutamate

Share and Cite

MDPI and ACS Style

Lazier, J.; Lupichuk, S.M.; Sosova, I.; Khan, A.A. Hyperammonemic Encephalopathy in An Adenocarcinoma Patient Managed with Carglumic Acid. Curr. Oncol. 2014, 21, 736-739. https://doi.org/10.3747/co.21.2076

AMA Style

Lazier J, Lupichuk SM, Sosova I, Khan AA. Hyperammonemic Encephalopathy in An Adenocarcinoma Patient Managed with Carglumic Acid. Current Oncology. 2014; 21(5):736-739. https://doi.org/10.3747/co.21.2076

Chicago/Turabian Style

Lazier, J., S.M. Lupichuk, I. Sosova, and A.A. Khan. 2014. "Hyperammonemic Encephalopathy in An Adenocarcinoma Patient Managed with Carglumic Acid" Current Oncology 21, no. 5: 736-739. https://doi.org/10.3747/co.21.2076

APA Style

Lazier, J., Lupichuk, S. M., Sosova, I., & Khan, A. A. (2014). Hyperammonemic Encephalopathy in An Adenocarcinoma Patient Managed with Carglumic Acid. Current Oncology, 21(5), 736-739. https://doi.org/10.3747/co.21.2076

Article Menu

Hyperammonemic Encephalopathy in An Adenocarcinoma Patient Managed with Carglumic Acid

Abstract

Share and Cite

Article Metrics

Article Access Statistics

Further Information

Guidelines

MDPI Initiatives

Follow MDPI