ArticleCureus2025
Anaesthetic Management of Advanced Late-Onset Pompe Disease: Challenges in a Major Abdominal Surgery.
Article in Cureus, 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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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Authors and funding
3 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pompe disease is a rare autosomal recessive lysosomal storage disorder caused by a deficiency of acid alpha-glucosidase, leading to glycogen accumulation in various tissues. The late-onset form predominantly affects skeletal and respiratory muscles, with minimal cardiac involvement. Due to respiratory muscle weakness and potential pulmonary complications, this disease presents significant challenges in perioperative management. We report the case of a patient with late-onset Pompe disease with moderate restrictive disease, dependent on nocturnal bilevel positive airway pressure (BiPAP), who underwent elective major abdominal surgery under combined general and epidural anaesthesia. This case emphasises the importance of multidisciplinary perioperative management, which includes postoperative respiratory support strategies and the choice of regional analgesia. Despite the rarity of such cases, especially in major non-cardiac surgery, this report contributes valuable insights into safe anaesthetic approaches for this complex patient population.
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