Increased aminotransferases do not necessarily mean hepatotoxicity of CFTR modulator therapy

Marek Woynarowski; Ewa Sapiejka; Maria Jóźwiak; Aldona Wierzbicka-Rucińska

doi:10.5114/ceh.2023.132264

eISSN: 2449-8238
ISSN: 2392-1099

Clinical and Experimental Hepatology

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4/2023
vol. 9

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abstract:

Short report

Increased aminotransferases do not necessarily mean hepatotoxicity of CFTR modulator therapy

Marek Woynarowski

^{1, 2}

,

Ewa Sapiejka

³

,

Maria Jóźwiak

³

,

Aldona Wierzbicka-Rucińska

⁴

Collegium Medicum, Jan Kochanowski University, Kielce, Poland
Cystic Fibrosis Centre, Paediatric Hospital, Dziekanów Leśny, Łomianki, Poland
Department of Paediatrics and Cystic Fibrosis, Paediatric Hospital Polanki, Gdańsk, Poland
Department of Biochemistry, Radioimmunology and Experimental Medicine, Children’s Health Memorial Institute, Warsaw, Poland

Clin Exp HEPATOL 2023; 9, 4: 405-409

DOI: https://doi.org/10.5114/ceh.2023.132264

Online publish date: 2023/11/28

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Aim of the study:
The presence of macroenzymes may mimic treatment related hepatotoxicity.

Material and methods:
We present a female subject who developed high alanine aminotransferase (ALT)/aspartate aminotransferase (AST) activity during cystic fibrosis transmembrane regulator (CFTR) modulator therapy.

Results:
The differential work-up did not show any underlying liver disease. CFTR modulators were stopped with subsequent normalization and immediate rise of ALT/AST after modulators were restarted, which was interpreted as the presentation of CFTR modulator hepatotoxicity. Before permanent CFTR modulators’ discontinuation the patient’s blood was tested for the presence of macroALT/macroAST and the result was positive. The patient is continuing a CFTR modulator treatment that is being supervised using standard laboratory tests and a test detecting the presence of macroenzymes. At three subsequent measurements the tests showed the presence of macroenzymes.

Conclusions:
Our patient shows that increased ALT/AST during CFTR modulator therapy may be related to the induction of macroenzymes and not necessarily to hepatotoxicity. Patients with high ALT/AST activity should be considered for testing for the presence of macroenzymes.

keywords:

Increased aminotransferases do not necessarily mean hepatotoxicity of CFTR modulator therapy

Marek Woynarowski 1, 2 , Ewa Sapiejka 3 , Maria Jóźwiak 3 , Aldona Wierzbicka-Rucińska 4

macroAST, macroALT, hepatotoxicity, drug induced liver injury, CFTR modulators

Marek Woynarowski

^{1, 2}

,

Ewa Sapiejka

³

,

Maria Jóźwiak

³

,

Aldona Wierzbicka-Rucińska

⁴