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Alpha-Mannosidosis - Europe PMC

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Last Updated: 28 July 2022

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Mortality in patients with alpha-mannosidosis: a review of patients' data and the literature.

Alpha-mannosidosis is a rare autosomal recessive lysosomal storage disorder characterized by reduced alpha-mannosidase production. This research was designed to investigate the age at death and the causes of death in patients with alpha-mannosidosis who did not receive disease-modifying therapy. Methods Clinicians and LSD patient groups from 33 countries were invited to complete a questionnaire between April and May 2021. Median age at death for patients reported by physicians/POs was 45 years; 53% were female. Median age at death for patients identified from the literature was 4. 3 years, two of whom were female. Conclusions The article found that pneumonia has been the primary cause of death in untreated patients with alpha-mannosidosis in recent decades, followed by cancer.

Source link: https://europepmc.org/article/MED/35871018

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions