Glycogen storage disease type I medical therapy: Difference between revisions
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***:'''Note (1):''' As hypoglycemia in GSD type 1 can be life-threatening and may cause seizures, permanent brain damage and even death, training of the parents (and/or child, when older) in inserting a nasogastric (NG) tube or that a G-tube be surgically placed so that there is always access to treat for hypoglycemia, especially during times of illness or refusal to eat is recommended. | ***:'''Note (1):''' As hypoglycemia in GSD type 1 can be life-threatening and may cause seizures, permanent brain damage and even death, training of the parents (and/or child, when older) in inserting a nasogastric (NG) tube or that a G-tube be surgically placed so that there is always access to treat for hypoglycemia, especially during times of illness or refusal to eat is recommended. | ||
***:'''Note (2):''' A G-tube may not be a good option in patients with GSD type 1b and neutropenia as it increases the risk of recurrent infections at the surgical site. Granulocyte colony-stimulating factor (G-CSF) (Neupogen) is should be administered before placing a G-tube if the child has neutropenia. | ***:'''Note (2):''' A G-tube may not be a good option in patients with GSD type 1b and neutropenia as it increases the risk of recurrent infections at the surgical site. Granulocyte colony-stimulating factor (G-CSF) (Neupogen) is should be administered before placing a G-tube if the child has neutropenia. | ||
***:'''Note (3):'''Blood glucose level should be maintained at more than 70 mg/dl or 4 mmol/l. | |||
***:'''Note (4):'''Feeding regimen are decided on a case by case basis. | |||
***:'''Note (5): The rate of the continuous tube feeding should be calculated to provide a glucose infusion rate of | |||
***:: In infancy: 8–10 mg glucose/kg/min | |||
***:: In older children: 4–8 mg glucose/kg/min | |||
***:'''Note (6): Infant should be immediately fed after discontinuing tube feedings in order to avoid a rapid decrease in blood glucose due to high circulating insulin levels. | |||
***:'''Note (7): It is advisable to use safety precautions such as bed-wetting devices (to detect formula spilling onto the bed), infusion pump alarms, safety adapters, connectors, and tape for tubing to detect pump failure and occluded or disconnected tubing. These events may lead to hypoglycemia, seizures, and even death. | |||
==References== | ==References== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Medical Therapy
The medical management of GSD is divided into nutritional therapy and medical management of systemic complications.[1]
- The primary concern in infants and young children with GSD type 1 is hypoglycemia.
- So, the first line treatment for GSD type 1 is the prevention of hypoglycemia.
- Small frequent feeds high in complex carbohydrates (preferably those high in fiber) are distributed evenly throughout 24 hours for the prevention of hypoglycemia.
- A metabolic dietician should be consulted once a case of GSD type 1 is diagnosed.
- Distribution of calories:
- Calories from carbohydrate: 60-70%
- Calories from protein: 10-15%
- Calories from fats: Remaining calories (<30% for children older than 2 years)
- Sucrose (fructose and glucose) and lactose (galactose and glucose) may be limited or avoided.
Nutritional Therapy
- 1. Infants
- 1.1 Formula and enteral feedings
- 1.1.1 Infant sleep <3-4 hours
- Preferred treatment (1): Soy-based formula, fed on demand q2h – q3h
- Preferred treatment (2): Sugar-free formula, fed on demand q2h – q3h
- Preferred treatment (3): A formula that is free of sucrose, fructose, and lactose; fed on demand q2h – q3h
- 1.1.2 Infant sleep >3-4 hours
- Preferred treatment (1): Overnight gastric feedings (OGFs)
- Preferred treatment (2): Wake up infant q3h - q4h; monitor blood glucose and offer feeding
- Note (1): As hypoglycemia in GSD type 1 can be life-threatening and may cause seizures, permanent brain damage and even death, training of the parents (and/or child, when older) in inserting a nasogastric (NG) tube or that a G-tube be surgically placed so that there is always access to treat for hypoglycemia, especially during times of illness or refusal to eat is recommended.
- Note (2): A G-tube may not be a good option in patients with GSD type 1b and neutropenia as it increases the risk of recurrent infections at the surgical site. Granulocyte colony-stimulating factor (G-CSF) (Neupogen) is should be administered before placing a G-tube if the child has neutropenia.
- Note (3):Blood glucose level should be maintained at more than 70 mg/dl or 4 mmol/l.
- Note (4):Feeding regimen are decided on a case by case basis.
- Note (5): The rate of the continuous tube feeding should be calculated to provide a glucose infusion rate of
- In infancy: 8–10 mg glucose/kg/min
- In older children: 4–8 mg glucose/kg/min
- Note (6): Infant should be immediately fed after discontinuing tube feedings in order to avoid a rapid decrease in blood glucose due to high circulating insulin levels.
- Note (7): It is advisable to use safety precautions such as bed-wetting devices (to detect formula spilling onto the bed), infusion pump alarms, safety adapters, connectors, and tape for tubing to detect pump failure and occluded or disconnected tubing. These events may lead to hypoglycemia, seizures, and even death.
- 1.1.1 Infant sleep <3-4 hours
- 1.1 Formula and enteral feedings
References
- ↑ 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.