A monthly publication of the Brazilian Society of Cardiology and considered the main channel for the promotion of Brazilian cardiovascular research.
ISSN 0066-782X
eISSN 1678-4170
Fabio Mastrocola, William Santos de Oliveira
, Adalberto Atsushi Porto, Roberto Moreno Mendonça, Nestor Rodrigues de Oliveira Neto
Fabio Mastrocola, William Santos de Oliveira
, Adalberto Atsushi Porto, Roberto Moreno Mendonça, Nestor Rodrigues de Oliveira Neto
An 18-year-old male patient presented to our ambulatory services due to a 3-month course of progressive dyspnea and non-productive cough. Symptoms were present even at rest, and he exhibited severe limitation at physical activity. He had a diagnosis of glycogen storage disease (GSD) type IV attained by liver biopsy since three years of age, when he developed hepatomegaly and mild hepatic dysfunction. At the time, histopathological examination showed grade 2 fibrosis along with numerous intracytoplasmic PAS-positive deposits that were resistant […]
Keywords: Cardiomyopathy, Hypertrophic; Fibrosis; Glycogen Storage Disease Type IV; Heart failure; Magnetic Resonance Spectroscopy/methods; PRKAG2 Syndrome; Prognosis