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- W3209947822 abstract "Glycogen storage disease type Ia (GSDIa) is caused by defective glucose-6-phosphatase, a key enzyme in carbohydrate metabolism. Affected individuals cannot release glucose during fasting and accumulate excess glycogen and fat in the liver and kidney, putting them at risk of severe hypoglycaemia and secondary metabolic perturbations. Good glycaemic/metabolic control through strict dietary treatment and regular doses of uncooked cornstarch (UCCS) is essential for preventing hypoglycaemia and long-term complications. Dietary treatment has improved the prognosis for patients with GSDIa; however, the disease itself, its management and monitoring have significant physical, psychological and psychosocial burden on individuals and parents/caregivers. Hypoglycaemia risk persists if a single dose of UCCS is delayed/missed or in cases of gastrointestinal intolerance. UCCS therapy is imprecise, does not treat the cause of disease, may trigger secondary metabolic manifestations and may not prevent long-term complications. We review the importance of and challenges associated with achieving good glycaemic/metabolic control in individuals with GSDIa and how this should be balanced with age-specific psychosocial development towards independence, management of anxiety and preservation of quality of life (QoL). The unmet need for treatment strategies that address the cause of disease, restore glucose homeostasis, reduce the risk of hypoglycaemia/secondary metabolic perturbations and improve QoL is also discussed." @default.
- W3209947822 created "2021-11-08" @default.
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- W3209947822 date "2021-10-27" @default.
- W3209947822 modified "2023-10-05" @default.
- W3209947822 title "Glycogen Storage Disease Type Ia: Current Management Options, Burden and Unmet Needs" @default.
- W3209947822 cites W119379044 @default.
- W3209947822 cites W1489706399 @default.
- W3209947822 cites W1632592649 @default.
- W3209947822 cites W1800997121 @default.
- W3209947822 cites W1849163519 @default.
- W3209947822 cites W1976674774 @default.
- W3209947822 cites W1978674018 @default.
- W3209947822 cites W1982384048 @default.
- W3209947822 cites W1987139858 @default.
- W3209947822 cites W1987284859 @default.
- W3209947822 cites W1987864507 @default.
- W3209947822 cites W1988280203 @default.
- W3209947822 cites W1989491629 @default.
- W3209947822 cites W1989692826 @default.
- W3209947822 cites W1992605127 @default.
- W3209947822 cites W1994166452 @default.
- W3209947822 cites W1998259061 @default.
- W3209947822 cites W2000485864 @default.
- W3209947822 cites W2001149680 @default.
- W3209947822 cites W2002315967 @default.
- W3209947822 cites W2004223416 @default.
- W3209947822 cites W2009559888 @default.
- W3209947822 cites W2018964373 @default.
- W3209947822 cites W2025211084 @default.
- W3209947822 cites W2026201080 @default.
- W3209947822 cites W2028582215 @default.
- W3209947822 cites W2028707865 @default.
- W3209947822 cites W203418577 @default.
- W3209947822 cites W2040001895 @default.
- W3209947822 cites W2041073972 @default.
- W3209947822 cites W2046898785 @default.
- W3209947822 cites W2051584795 @default.
- W3209947822 cites W2053552573 @default.
- W3209947822 cites W2059694214 @default.
- W3209947822 cites W2081983895 @default.
- W3209947822 cites W2085941325 @default.
- W3209947822 cites W2091788799 @default.
- W3209947822 cites W2102768596 @default.
- W3209947822 cites W2103833102 @default.
- W3209947822 cites W2105845113 @default.
- W3209947822 cites W2127191858 @default.
- W3209947822 cites W2128059507 @default.
- W3209947822 cites W2137566647 @default.
- W3209947822 cites W2158705794 @default.
- W3209947822 cites W2162222437 @default.
- W3209947822 cites W2171605209 @default.
- W3209947822 cites W2216136197 @default.
- W3209947822 cites W2333956343 @default.
- W3209947822 cites W2409381002 @default.
- W3209947822 cites W2475078613 @default.
- W3209947822 cites W2530635436 @default.
- W3209947822 cites W2582135799 @default.
- W3209947822 cites W2606704283 @default.
- W3209947822 cites W2746695956 @default.
- W3209947822 cites W2789916950 @default.
- W3209947822 cites W2793487362 @default.
- W3209947822 cites W2804130304 @default.
- W3209947822 cites W2891941476 @default.
- W3209947822 cites W2919342633 @default.
- W3209947822 cites W2929153566 @default.
- W3209947822 cites W2930675045 @default.
- W3209947822 cites W2952479552 @default.
- W3209947822 cites W2968757418 @default.
- W3209947822 cites W2971542946 @default.
- W3209947822 cites W2979103136 @default.
- W3209947822 cites W3010110336 @default.
- W3209947822 cites W3017300624 @default.
- W3209947822 cites W3094223882 @default.
- W3209947822 cites W3119472901 @default.
- W3209947822 cites W3138774123 @default.
- W3209947822 cites W3142262712 @default.
- W3209947822 cites W3156499005 @default.
- W3209947822 cites W3165613694 @default.
- W3209947822 cites W3197337319 @default.
- W3209947822 cites W3197587423 @default.
- W3209947822 cites W3207708271 @default.
- W3209947822 cites W4243549407 @default.
- W3209947822 doi "https://doi.org/10.3390/nu13113828" @default.