OBJECTIVE: Serum alanine transaminase (ALT) and aspartate transaminase (AST) levels are commonly used as biochemical indicators of hepatocellular injury but can also detect occult muscle disease. High concomitant serum creatine phosphokinase (CPK) levels can point to muscle as the source of high transaminase levels. Nevertheless, clinicians may be reluctant to attribute high transaminase levels to muscle. METHODS: Study patients were boys with a genetic or biopsy-confirmed dystrophinopathy whose concomitant serum CPK, ALT, and AST levels were measured. RESULTS: We report 82 enzyme data sets from 46 patients with Duchenne muscular dystrophy (DMD) and 9 with Becker muscular dystrophy. Our results show a linear relationship between serum CPK and serum ALT and AST and a logarithmic relationship between serum enzyme levels and age for boys with DMD. We developed a mathematical model to predict serum ALT and AST levels when the serum CPK level and age are known. For 6 boys, initial failure to consider muscle as a cause of high transaminase levels led to delay of diagnosis and extensive testing for hepatic dysfunction. A second group of 4 boys with known DMD were investigated for liver disease after high transaminase levels were detected. Serum transaminase levels were highest in ambulant boys with DMD, whose levels reached 1220 U/L (ALT) (22.6 times higher than upper-limit normal levels) and 801 U/L (AST) (12.3 times higher than upper-limit normal levels). CONCLUSIONS: By recognizing muscle as a potential source of serum transaminase levels, clinicians can avoid unnecessary and invasive procedures, expedite clinical diagnosis, and avoid unnecessary cessation of concomitant drug therapy.
OBJECTIVE: Serum alanine transaminase (ALT) and aspartate transaminase (AST) levels are commonly used as biochemical indicators of hepatocellular injury but can also detect occult muscle disease. High concomitant serum creatine phosphokinase (CPK) levels can point to muscle as the source of high transaminase levels. Nevertheless, clinicians may be reluctant to attribute high transaminase levels to muscle. METHODS: Study patients were boys with a genetic or biopsy-confirmed dystrophinopathy whose concomitant serum CPK, ALT, and AST levels were measured. RESULTS: We report 82 enzyme data sets from 46 patients with Duchenne muscular dystrophy (DMD) and 9 with Becker muscular dystrophy. Our results show a linear relationship between serum CPK and serum ALT and AST and a logarithmic relationship between serum enzyme levels and age for boys with DMD. We developed a mathematical model to predict serum ALT and AST levels when the serum CPK level and age are known. For 6 boys, initial failure to consider muscle as a cause of high transaminase levels led to delay of diagnosis and extensive testing for hepatic dysfunction. A second group of 4 boys with known DMD were investigated for liver disease after high transaminase levels were detected. Serum transaminase levels were highest in ambulant boys with DMD, whose levels reached 1220 U/L (ALT) (22.6 times higher than upper-limit normal levels) and 801 U/L (AST) (12.3 times higher than upper-limit normal levels). CONCLUSIONS: By recognizing muscle as a potential source of serum transaminase levels, clinicians can avoid unnecessary and invasive procedures, expedite clinical diagnosis, and avoid unnecessary cessation of concomitant drug therapy.
Authors: Matthew S Alexander; Juan Carlos Casar; Norio Motohashi; Natássia M Vieira; Iris Eisenberg; Jamie L Marshall; Molly J Gasperini; Angela Lek; Jennifer A Myers; Elicia A Estrella; Peter B Kang; Frederic Shapiro; Fedik Rahimov; Genri Kawahara; Jeffrey J Widrick; Louis M Kunkel Journal: J Clin Invest Date: 2014-05-01 Impact factor: 14.808
Authors: Rachel J Church; Gerd A Kullak-Ublick; Jiri Aubrecht; Herbert L Bonkovsky; Naga Chalasani; Robert J Fontana; Jens C Goepfert; Frances Hackman; Nicholas M P King; Simon Kirby; Patrick Kirby; John Marcinak; Sif Ormarsdottir; Shelli J Schomaker; Ina Schuppe-Koistinen; Francis Wolenski; Nadir Arber; Michael Merz; John-Michael Sauer; Raul J Andrade; Florian van Bömmel; Thierry Poynard; Paul B Watkins Journal: Hepatology Date: 2018-06-27 Impact factor: 17.425
Authors: Marc-Olivier Deguise; Chantal Pileggi; Yves De Repentigny; Ariane Beauvais; Alexandra Tierney; Lucia Chehade; Jean Michaud; Maica Llavero-Hurtado; Douglas Lamont; Abdelmadjid Atrih; Thomas M Wishart; Thomas H Gillingwater; Bernard L Schneider; Mary-Ellen Harper; Simon H Parson; Rashmi Kothary Journal: Cell Mol Gastroenterol Hepatol Date: 2021-02-02
Authors: Peter M Burch; Oksana Pogoryelova; Richard Goldstein; Donald Bennett; Michela Guglieri; Volker Straub; Kate Bushby; Hanns Lochmüller; Carl Morris Journal: J Neuromuscul Dis Date: 2015-09-02