Glycogen storage disease type III medical therapy: Difference between revisions
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==Overview== | ==Overview== | ||
The medical therapy of glycogen storage disease type 3 is directed towards management of hypoglycemia. However [[hypoglycemic]] episodes are less marked in glycogen storage disease type 3 than [[glycogen storage disease type 1]].A high protein diet is recommended, especially for children with growth failure and myopathy. | The medical therapy of glycogen storage disease type 3 is directed towards management of hypoglycemia. However [[hypoglycemic]] episodes are less marked in glycogen storage disease type 3 than [[glycogen storage disease type 1]]. A high protein diet is recommended, especially for children with growth failure and myopathy. A [[Metabolism|metabolic]] [[dietician]] should be consulted once a case of GSD type 3 is diagnosed. Preferred treatment is use of [[cornstarch]]. Careful monitoring of [[blood glucose]] is needed during [[illness]], if diet or schedule is changed, start of [[exercise]] routine, and randomly to detect asymptomatic [[hypoglycemia]]. | ||
==Medical Therapy== | ==Medical Therapy== | ||
*The primary concern in infants and young children with GSD type 3 is [[hypoglycemia]]. | *The primary concern in infants and young children with GSD type 3 is [[hypoglycemia]]. | ||
*However [[hypoglycemic]] episodes are less marked in glycogen storage disease type 3 than [[glycogen storage disease type 1]].<ref name="pmid3292244">{{cite journal| author=Fernandes J, Leonard JV, Moses SW, Odièvre M, di Rocco M, Schaub J et al.| title=Glycogen storage disease: recommendations for treatment. | journal=Eur J Pediatr | year= 1988 | volume= 147 | issue= 3 | pages= 226-8 | pmid=3292244 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3292244 }} </ref> | *However [[hypoglycemic]] episodes are less marked in glycogen storage disease type 3 than [[glycogen storage disease type 1]].<ref name="pmid3292244">{{cite journal| author=Fernandes J, Leonard JV, Moses SW, Odièvre M, di Rocco M, Schaub J et al.| title=Glycogen storage disease: recommendations for treatment. | journal=Eur J Pediatr | year= 1988 | volume= 147 | issue= 3 | pages= 226-8 | pmid=3292244 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3292244 }} </ref> | ||
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* '''3 Adults''' | * '''3 Adults''' | ||
** '''Glycogen | ** '''3.1 Glycogen storage disease type 3a''' | ||
**:* Increase [[Protein-arginine deiminase|protein]] in diet | **:* Increase [[Protein-arginine deiminase|protein]] in diet | ||
** '''Glycogen | ** '''3.2 Glycogen storage disease type 3b''' | ||
**:* No specific treatment is needed | **:* No specific treatment is needed | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anmol Pitliya, M.B.B.S. M.D.[2]
Overview
The medical therapy of glycogen storage disease type 3 is directed towards management of hypoglycemia. However hypoglycemic episodes are less marked in glycogen storage disease type 3 than glycogen storage disease type 1. A high protein diet is recommended, especially for children with growth failure and myopathy. A metabolic dietician should be consulted once a case of GSD type 3 is diagnosed. Preferred treatment is use of cornstarch. Careful monitoring of blood glucose is needed during illness, if diet or schedule is changed, start of exercise routine, and randomly to detect asymptomatic hypoglycemia.
Medical Therapy
- The primary concern in infants and young children with GSD type 3 is hypoglycemia.
- However hypoglycemic episodes are less marked in glycogen storage disease type 3 than glycogen storage disease type 1.[1]
- A high protein diet is recommended, especially for children with growth failure and myopathy due to reasons including:[2]
- A low fat diet is recommended due to reasons including:
- High protein and high carbohydrates necessitate reduction of fats.
- Young age group individuals may be inefficient in oxidizing long chain fatty acids.
- Moderate amount of medium chain triglycerides (MCT) are preferred as they are ketogenic and may provide additional energy source for brain.
- It is not needed to avoid sucrose (fructose and glucose) and lactose (galactose and glucose).
- A metabolic dietician should be consulted once a case of GSD type 3 is diagnosed.
- Distribution of calories:[3]
Nutritional therapy
- 1 Infants or young child
- 1.1 Asymptomatic or mild hypoglycemia
- Preferred treatment (1): Cornstarch (CS) 1 g/kg body weight PO q4h
- NOTE (1): Digestive enzymes such as amylase are not fully functional during infancy. So, CS may not be well tolerated leading to gas, diarrhea, and bloating. Addition of pancrelipase with CS may help with digestion and reduces side effects.
- NOTE (2): Pancrealipase consists of three enzymes including lipase, protease, and amylase.
- NOTE (3): Typically, CS requirement of glycogen storage disease type 3 is less than glycogen storage disease type 1. So, starting CS at a lower dose and increasing the dose as per requirement is recommended.
- NOTE (4): During the initiation of treatment, blood glucose should be monitored every hour to establish the adequacy of CS dosage. As the dosage of CS is fixed, the frequency of blood glucose monitoring may be decreased thereafter.
- NOTE (5): Careful monitoring of blood glucose is needed during:
- Illness
- Diet or schedule is changed
- During start of exercise routine
- Randomly to detect asymptomatic hypoglycemia
- 1.2 Severe hypoglycemia
- Preferred treatment (1): Cornstarch (CS) 1.6 g/kg body weight PO q4h
- Preferred treatment (2): Overnight gastric feedings (OGFs)
- 1.1 Asymptomatic or mild hypoglycemia
- 2 Older child
- 2.1 Asymptomatic or mild hypoglycemia
- Preferred treatment (1): Cornstarch (CS) 1 g/kg body weight PO q4h
- 2.2 Severe hypoglycemia
- Preferred treatment (1): Cornstarch (CS) 1.7–2.5 g/kg body weight PO q6h
- 2.1 Asymptomatic or mild hypoglycemia
- 3 Adults
- 3.1 Glycogen storage disease type 3a
- Increase protein in diet
- 3.2 Glycogen storage disease type 3b
- No specific treatment is needed
- 3.1 Glycogen storage disease type 3a
References
- ↑ Fernandes J, Leonard JV, Moses SW, Odièvre M, di Rocco M, Schaub J; et al. (1988). "Glycogen storage disease: recommendations for treatment". Eur J Pediatr. 147 (3): 226–8. PMID 3292244.
- ↑ Slonim AE, Coleman RA, Moses WS (1984). "Myopathy and growth failure in debrancher enzyme deficiency: improvement with high-protein nocturnal enteral therapy". J Pediatr. 105 (6): 906–11. PMID 6438290.
- ↑ Goldberg T, Slonim AE (1993). "Nutrition therapy for hepatic glycogen storage diseases". J Am Diet Assoc. 93 (12): 1423–30. PMID 8245377.