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Glycogen storage disease due to acid maltase deficiency, infantile onset

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Disease definition

Glycogen storage disease due to acid maltase deficiency, infantile onset is the most severe form of glycogen storage disease due to acid maltase deficiency, characterized by cardiomegaly with respiratory distress, muscle weakness and feeding difficulties. It is often fatal.

ORPHA:308552

Classification level: Subtype of disorder

Synonym(s):
  • Glycogenosis type II, infantile onset
  • Glycogen storage disease type II, infantile onset
  • GSD type II, infantile onset
  • Pompe disease, infantile onset
  • Glycogenosis type 2, infantile onset
  • Alpha-1,4-glucosidase acid deficiency, infantile onset
  • GSD due to acid maltase deficiency, infantile onset
  • GSD type 2, infantile onset
  • Glycogen storage disease type 2, infantile onset
  • Glycogenosis due to acid maltase deficiency, infantile onset

Prevalence: Unknown

Inheritance: Autosomal recessive

Age of onset: Antenatal, Infancy, Neonatal

ICD-10: E74.0

ICD-11: 5C51.3

OMIM: 232300

UMLS: C3888924

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands Ελληνικά
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Svenska (2022) - Socialstyrelsen
Guidelines
Emergency guidelines
Anesthesia guidelines
English (2016) - Orphananesthesia
Español (2016) - Orphananesthesia
Čeština (2016) - Orphananesthesia
Clinical practice guidelines
Deutsch (2021) - Fortschr Neurol Psychiatr Logo ERN
English (2024) - Eur J Neurol
English (2017) - Eur J Neurol Logo ERN
English (2024) - Orphanet J Rare Dis Logo ERN
English (2011) - Gene Med
Disease review articles
Review article
Español (2016.pdf) - Rev Neurol
Clinical genetics review
English (2025) - GeneReviews
Genetic testing
Guidance for genetic testing
Patient-Centered Outcome Measures (PCOMs)
Access questionnaires assessing quality of life in this disease (English)
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