ArticleSurgical case reports2025
Case Report: Perioperative Management of a Patient with Glycogen Storage Disease Type IXd.
Article in Surgical case reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
introductionGlycogen storage disease type IX (GSD type IX) is caused by a deficiency in phosphorylase b kinase (PHK) and is classified into hepatic (IXa-c) and muscular (IXd) subtypes. GSD type IXd leads to exercise intolerance, rhabdomyolysis, and myoglobinuria owing to impaired glycogen breakdown. It is a rare and mild metabolic disorder, with only 19 reported cases of CASE PRESENTATION: A 61-year-old male presented with a right inguinal hernia requiring surgical repair. He had experienced muscle weakness since the age of 53, which progressed to severe neck muscle atrophy by the age of 58. Genetic testing confirmed a
conclusionsWe successfully managed a patient with GSD type IXd perioperatively, without complications. Although this disease can cause rhabdomyolysis, the symptoms are often mild and may remain undiagnosed. Therefore, in patients with muscle weakness or elevated creatine kinase levels, careful surgical planning and perioperative monitoring are essential.
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