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In contrast, substrate reduction therapy presents an efficient alternative with a rate-limiting strategy to control the accumulation of substrate of the deficient enzyme [9, 17]

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Patients should report severe, persistent abdominal pain radiating to the back (with or without vomiting), signs of gallbladder disease, persistent vomiting, or signs of dehydration promptly and seek immediate medical attention for symptoms of pancreatitis or severe allergic reactions

London: Elsevier Ltd