Abstract
Increased aminotransferases do not necessarily mean hepatotoxicity of CFTR modulator therapy
- 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
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
macroAST, macroALT, hepatotoxicity, drug induced liver injury, CFTR modulators
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