Glycogen storage disease type I secondary prevention: Difference between revisions
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===General medical care recommendations=== | |||
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*All patients should have a primary care provider ("medical home"). | |||
*Routine immunizations should be given as recommended by Centers for Disease Control and Prevention. | |||
*Avoid medications that can potentially cause hypoglycemia and check for potential drug interactions/side effects when a new medication is prescribed. | |||
*Patients should be encouraged to participate in age-appropriate physical activities, but contact or competitive sports should be avoided because of the risk of liver injury. | |||
*Good dental hygiene and frequent monitoring of dental health are advised | |||
*During intercurrent illnesses, early evaluation and treatment are encouraged. Patients who cannot maintain normal dietary intake/CS treatment or who have emesis should proceed to the nearest emergency department for evaluation and i.v. glucose treatment. | |||
*All patients/families should carry an emergency letter and an emergency kit at all times. | |||
*all patients should wear a medical alert identification. | |||
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===Gastrointestinal or Nutritional recommendations=== | ===Gastrointestinal or Nutritional recommendations=== | ||
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===Hepatic and hepatic transplantation recommendations=== | ===Hepatic and hepatic transplantation recommendations=== | ||
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Revision as of 16:19, 13 November 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Secondary Prevention
Effective measures for secondary prevention of GSD type 1 include:[1]
- Blood glucose (BG) monitoring
- Prevent overtreatment
- Growth tracking
- Gastrointestinal or Nutritional recommendations
- Hepatic and hepatic transplantation recommendations
- Nephrology recommendations
- Hematology recommendations
- Cardiovascular recommendations
Blood glucose (BG) monitoring
- Initial diet prescription is established on the basis of frequent BG monitoring. Afterwards, BG monitoring is done randomly to avoid asymptomatic hypoglycemia.
- Documentation of BG testing is done before each clinic visit to adjust diet, CS intake, and overnight gastric feedings (OGFs).
The following BG levels should be checked for 2–3 days before the clinic visit:
- Before meals
- Before cornstarch (CS) intake
- Before and after exercise
- If the cornstarch dose is changed, BG levels should be checked after 4 hours and then at hourly intervals to establish the duration of effectiveness. Effectiveness is measured by the duration of time for which the dose of CS will maintain the BG level >70 mg/dl.
Lactate meter
- The lactate meter is a portable device to measure lactate concentration.[2]
- Lactate concentrations are higher in patients with GSD type 1.
- The lactate meter may act as a good supplement to glucose monitoring, particularly during times of illness to help prevent acute deterioration, to avoid hospitalization, or to alert the caregivers about emergencies.
Continuous blood glucose monitoring system
- This is a method for monitoring and managing BG control in GSD patients.[3]
- This system may also help detect asymptomatic hypoglycemia.
Prevent overtreatment
- Parents should be educated to avoid overtreating patients.
- Overtreatment may result in complications including increased glycogen storage and over time may lead to hyperinsulinemia and insulin resistance.[4]
Growth tracking
- Growth should be tracked through parameters including:[1]
- Height
- Weight
- Weight/height ratio
- Body mass index
- Head circumference
- Changes in growth pattern is observed in poor metabolic control of GSD type 1.
Gastrointestinal or Nutritional recommendations
General medical care recommendations |
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Hepatic and hepatic transplantation recommendations |
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Nephrology recommendations |
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Hematology recommendations |
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Cardiovascular recommendations= |
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Adopted fromGenetics in medicine[5][6][7][7][7] |
References
- ↑ 1.0 1.1 Kishnani, Priya S.; Austin, Stephanie L.; Abdenur, Jose E.; Arn, Pamela; Bali, Deeksha S.; Boney, Anne; Chung, Wendy K.; Dagli, Aditi I.; Dale, David; Koeberl, Dwight; Somers, Michael J.; Burns Wechsler, Stephanie; Weinstein, David A.; Wolfsdorf, Joseph I.; Watson, Michael S. (2014). "Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics". Genetics in Medicine. doi:10.1038/gim.2014.128. ISSN 1098-3600.
- ↑ Saunders AC, Feldman HA, Correia CE, Weinstein DA (2005). "Clinical evaluation of a portable lactate meter in type I glycogen storage disease". J Inherit Metab Dis. 28 (5): 695–701. doi:10.1007/s10545-005-0090-1. PMID 16151900.
- ↑ White FJ, Jones SA (2011). "The use of continuous glucose monitoring in the practical management of glycogen storage disorders". J Inherit Metab Dis. 34 (3): 631–42. doi:10.1007/s10545-011-9335-3. PMID 21556835.
- ↑ Bhattacharya K (2011). "Dietary dilemmas in the management of glycogen storage disease type I." J Inherit Metab Dis. 34 (3): 621–9. doi:10.1007/s10545-011-9322-8. PMID 21491105.
- ↑ "Gastrointestinal/nutrition recommendations : Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics : Genetics in Medicine : Springer Nature".
- ↑ "Hepatic and hepatic transplant recommendations : Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics : Genetics in Medicine : Springer Nature".
- ↑ 7.0 7.1 7.2 "images.nature.com".