Acetaminophen-induced hepatotoxicity in a glutathione synthetase-deficient patient

We report a patient with glutathione synthetase (GS) deficiency who developed acetaminophen-induced hepatotoxicity after a two-day treatment with regular doses of acetaminophen. A nine-month-old female was referred because of intractable metabolic acidosis. She was given acetaminophen at ther...

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Main Authors: Ayşegül Tokatli, H Serap Kalkanoğlu-Sivri, Aysel Yüce, Turgay Coşkun
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 2007-01-01
Series:The Turkish Journal of Pediatrics
Online Access:https://turkjpediatr.org/article/view/2512
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author Ayşegül Tokatli
H Serap Kalkanoğlu-Sivri
Aysel Yüce
Turgay Coşkun
author_facet Ayşegül Tokatli
H Serap Kalkanoğlu-Sivri
Aysel Yüce
Turgay Coşkun
author_sort Ayşegül Tokatli
collection DOAJ
description We report a patient with glutathione synthetase (GS) deficiency who developed acetaminophen-induced hepatotoxicity after a two-day treatment with regular doses of acetaminophen. A nine-month-old female was referred because of intractable metabolic acidosis. She was given acetaminophen at therapeutic doses over a 48-hour period. She was hospitalized because of confusion and metabolic acidosis. Liver function tests were abnormal with normal bilirubin levels. The urine gas chromatography-mass spectrometry (GC/MS) showed massive excretion of 5-oxoproline. She improved and liver function tests normalized in the next six days, but compensated metabolic acidosis and massive 5-oxoprolinuria persisted. The analysis of GS in erythrocytes revealed 5% of normal enzyme activity, and the patient had 491G > A mutation on both alleles in the GS gene. In this report it can be assumed that patients, even if heterozygous for a mutation of the GS gene, are at risk for acetaminophen toxicity.
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issn 0041-4301
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language English
publishDate 2007-01-01
publisher Hacettepe University Institute of Child Health
record_format Article
series The Turkish Journal of Pediatrics
spelling doaj-art-d014e7b8a7f144f3a45de8a29d66239c2025-08-20T03:16:23ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212007-01-01491Acetaminophen-induced hepatotoxicity in a glutathione synthetase-deficient patientAyşegül Tokatli0H Serap Kalkanoğlu-SivriAysel YüceTurgay CoşkunUnit of Nutrition and Metabolism, Department of Pediatrics, Hacettepe University, Faculty of Medicine, Ankara, Turkey. We report a patient with glutathione synthetase (GS) deficiency who developed acetaminophen-induced hepatotoxicity after a two-day treatment with regular doses of acetaminophen. A nine-month-old female was referred because of intractable metabolic acidosis. She was given acetaminophen at therapeutic doses over a 48-hour period. She was hospitalized because of confusion and metabolic acidosis. Liver function tests were abnormal with normal bilirubin levels. The urine gas chromatography-mass spectrometry (GC/MS) showed massive excretion of 5-oxoproline. She improved and liver function tests normalized in the next six days, but compensated metabolic acidosis and massive 5-oxoprolinuria persisted. The analysis of GS in erythrocytes revealed 5% of normal enzyme activity, and the patient had 491G > A mutation on both alleles in the GS gene. In this report it can be assumed that patients, even if heterozygous for a mutation of the GS gene, are at risk for acetaminophen toxicity. https://turkjpediatr.org/article/view/2512
spellingShingle Ayşegül Tokatli
H Serap Kalkanoğlu-Sivri
Aysel Yüce
Turgay Coşkun
Acetaminophen-induced hepatotoxicity in a glutathione synthetase-deficient patient
The Turkish Journal of Pediatrics
title Acetaminophen-induced hepatotoxicity in a glutathione synthetase-deficient patient
title_full Acetaminophen-induced hepatotoxicity in a glutathione synthetase-deficient patient
title_fullStr Acetaminophen-induced hepatotoxicity in a glutathione synthetase-deficient patient
title_full_unstemmed Acetaminophen-induced hepatotoxicity in a glutathione synthetase-deficient patient
title_short Acetaminophen-induced hepatotoxicity in a glutathione synthetase-deficient patient
title_sort acetaminophen induced hepatotoxicity in a glutathione synthetase deficient patient
url https://turkjpediatr.org/article/view/2512
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AT turgaycoskun acetaminopheninducedhepatotoxicityinaglutathionesynthetasedeficientpatient