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Page 8 of 12 Politei et al. Rare Dis Orphan Drugs J 2024;3:10 https://dx.doi.org/10.20517/rdodj.2023.46
Figure 4. Flow chart for decision making in patients with Fabry disease and gastrointestinal manifestations. *Late-onset Fabry patients
do not show GL-3 accumulation in the gastrointestinal system. In these patients, GI symptoms must be attributable to gastric
comorbidities.
No specific dietary interventions on FD patients have been reported. Useful non-drug management
approaches are dietary modifications such as opting for low-fat meals in cases of pancreatic dysfunction or
incorporating pancreatic enzyme supplements. Additionally, consuming several smaller meals throughout
the day, as opposed to a single large “main” meal, has shown promise in alleviating upper gastrointestinal
(GI) symptoms .
[5]
Considering that Fermentable Oligosaccharides, Disaccharides, Monosaccharides, and Polyols (FODMAPs)
may play a role in GI manifestations and dysbiosis in FD patients, a low-FODMAP diet was explored as a
[53]
complementary alternative treatment in individuals with FD . Patients were assessed for GI symptoms
using IBS severity score and GSRS questionnaires. For symptomatic patients, the low-FODMAP diet
involved an initial phase of eliminating fermentable saccharides, followed by a gradual reintroduction of
