2024-10-042024-10-042023-01-051047-9511https://doi.org/10.1017/S1047951122004164https://hdl.handle.net/11452/45878Danon disease is a rare and fatal disease caused by a mutation in the lysosome-associated membrane protein 2 gene. Impaired intracellular autophagy causes lysosomal vacuoles to accumulate mainly in myocardial and skeletal muscle cells, leading to hypertrophic cardiomyopathy, skeletal myopathy, and varying degrees of intellectual disability. Two distinct childhood presentations of Danon disease are described in this report.enDanon diseaseHypertrophic cardiomyopathyPre-excitationHeart failureScience & technologyLife sciences & biomedicineCardiac & cardiovascular systemsPediatricsCardiovascular system & cardiologyA rare and fatal cause of hypertrophic cardiomyopathy: Danon diseaseArticle0009078162000011448145033810.1017/S1047951122004164