Macroenzymes as a reason for aminotransferases flare in cystic fibrosis patients on CFTR modulators therapy – Report of three cases
Marek Woynarowski,
Ewa Sapiejka,
Maria Jóźwiak,
Justyna Milczewska,
Katarzyna Zybert,
Adam Krusiński,
Jan Siwiec,
Aldona Wierzbicka-Rucińska
Affiliations
Marek Woynarowski
Collegium Medicum, Jan Kochanowski University, Al. IX Wieków Kielc 19A, 25-516, Kielce, Poland; Cystic Fibrosis Centre, Paediatric Hospital, Dziekanów Leśny, Marii Konopnickiej 65, 05-092, Łomianki, Poland; Corresponding author. Collegium Medicum, Jan Kochanowski University, Al. IX Wieków Kielc 19A, 25-516 Kielce, Poland.
Ewa Sapiejka
Department of Paediatrics and Cystic Fibrosis, Paediatric Hospital Polanki, Polanki 119, 80-308, Gdańsk, Poland
Maria Jóźwiak
Department of Paediatrics and Cystic Fibrosis, Paediatric Hospital Polanki, Polanki 119, 80-308, Gdańsk, Poland
Justyna Milczewska
Cystic Fibrosis Centre, Paediatric Hospital, Dziekanów Leśny, Marii Konopnickiej 65, 05-092, Łomianki, Poland; Cystic Fibrosis Department, Institute of Mother and Child, Kasprzaka 17a, 01-211, Warsaw, Poland
Katarzyna Zybert
Cystic Fibrosis Centre, Paediatric Hospital, Dziekanów Leśny, Marii Konopnickiej 65, 05-092, Łomianki, Poland; Cystic Fibrosis Department, Institute of Mother and Child, Kasprzaka 17a, 01-211, Warsaw, Poland
Adam Krusiński
Department of Pulmonology, Oncology and Allergology, Medical University of Lublin, Jaczewskiego 8, 20-954, Lublin, Poland
Jan Siwiec
Department of Pulmonology, Oncology and Allergology, Medical University of Lublin, Jaczewskiego 8, 20-954, Lublin, Poland
Aldona Wierzbicka-Rucińska
Department of Biochemistry, Radioimmunology and Experimental Medicine, Children's Health Memorial Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland
It has been shown that macro-ALT/macro-AST cause false increase of ALT/AST activity in standard laboratory testing.This short communication presents a group of cystic fibrosis subjects who developed aminotranferases flare a few months after initiation of CFTR modulators therapy. Patients did not present any clinical signs or symptoms of liver failure and differential examination did not show any underlying liver disease. All patients tested positive for macro-ALT and macro-AST. Despite increased and fluctuating ALT/AST activity in standard tests patients restarted CFTR modulators therapy with good clinical effect and do not present any other than hypertransaminasemia signs or symptoms of progressing liver disease.Our data shows that ALT/AST flare during CFTR modulators therapy may be related to macro-ALT/macro-AST, thus patients with high ALT/AST during CFTR modulators therapy should be tested for macro-ALT/macro-AST.
