{"id":12424,"date":"2024-10-11T21:52:36","date_gmt":"2024-10-11T19:52:36","guid":{"rendered":"https:\/\/valintermed.com\/?p=12424"},"modified":"2024-10-11T21:52:36","modified_gmt":"2024-10-11T19:52:36","slug":"bolezn-pompe","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/pump-disease\/","title":{"rendered":"Pompe disease"},"content":{"rendered":"
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Pompe disease (glycogen storage disease type II) is an inherited metabolic disorder caused by a deficiency of the enzyme acid glucosidase. This enzyme is responsible for breaking down glycogen, which accumulates in cells, primarily in the muscles, liver, and heart. First of all, the manifestations of the disease are associated with progressive muscle weakness and impaired functioning of the respiratory system. Progression of the disease can lead to serious dysfunction of organs and systems, which ultimately poses a threat to the life of patients. This disease has various forms of manifestation, which can vary significantly in different patients depending on the age of onset of symptoms and the severity of the course.<\/p>\n

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