The effect of tailoring of cornstarch intake on stature in children with glycogen storage disease type III
-
Hanaa El-Karaksy
,
Ghada Anwar
Abstract
Aim: To determine the individual fasting tolerance for patients with glycogen storage disease type III (GSD III) and to assess their linear growth velocity after tailoring of dose intervals of oral uncooked cornstarch.
Patients and methods: A prospective cohort study included 32 patients with GSD III aged 6 months–11.5 years (median: 3.3 years). The fasting tolerance of each patient was determined as the time interval between starch administration until the drop in blood glucose level was below 60 mg/dL.
Results: Some 27 patients (84.4%) developed hypoglycemia. The intervals between oral cornstarch administration were tailored for each child according to his/her individual fasting tolerance. After a 6-month follow up there was a significant reduction in seizure attacks (p<0.01) and liver size (p<0.01), but there was no statistically significant difference in liver transaminase and serum lactate levels. There was a significant improvement in height (p<0.01) and linear growth velocity (p<0.05) of these patients after at least a 12-month follow up.
Conclusion: Adjusting the intervals between the cornstarch doses for each patient with GSD III, according to individual fasting tolerance test was very beneficial and resulted in improvement of the linear growth velocity and reduction in the frequency of hypoglycemic seizures as well as the size of the liver. Individual scheduling of cornstarch doses prevents complications in those who develop hypoglycemia at short intervals; it also allows some relaxation in schedule for those who can tolerate longer fasting hours to improve their appetite and prolong their uninterrupted sleep hours.
Acknowledgments
This study was partially funded by the Graduate Studies and Research Office, School of Medicine, Cairo University.
References
1. Hendriksz CJ, Gissen P. Glycogen storage disease. Paediatr Child Health 2011;21:84–9.10.1016/j.paed.2010.09.013Search in Google Scholar
2. Heller S, Worona L, Consuelo A. Nutritional therapy for glycogen storage diseases. J Pediatr Gastroenterol 2008;47:S15–21.10.1097/MPG.0b013e3181818ea5Search in Google Scholar
3. Goldberg R, Slonim AE. Nutrition therapy for hepatic glycogen storage diseases. J Am Diet Assoc 1993;93:1423–30.10.1016/0002-8223(93)92246-TSearch in Google Scholar
4. Parker PH, Ballew M, Greene HL. Nutritional management of glycogen storage disease. Annu Rev Nutr 1993;13:83–109.10.1146/annurev.nu.13.070193.000503Search in Google Scholar
5. Lee R. Glycogen storage diseases. Curr Paediatr 1997;7:108–13.10.1016/S0957-5839(97)80191-1Search in Google Scholar
6. Dagli A, Sentner CP, Weinstein DA. Glycogen storage disease type III. In: Pagon RA, Adam MP, Ardinger HH, Bird TD, Dolan CR, et al., editors. GeneReviews® [Internet]. Seattle, WA: University of Washington, Seattle, 1993–2014. Accessed 7 July 2014 [updated 6 September 2012].Search in Google Scholar
7. Triomphe TJ. Glycogen storage disease: a basic understanding and guide to nursing care. J Pediatr Nurs 1997;12:238–49.10.1016/S0882-5963(97)80007-3Search in Google Scholar
8. Bodamer OA, Feillet F, Lane RE. Utilization of cornstarch in glycogen storage disease type Ia. Eur J Gastroenterol Hepatol 2002;14:1251–6.10.1097/00042737-200211000-00014Search in Google Scholar PubMed
9. Wolfsdorf JI, Weinstein DA. Glycogen storage diseases. Rev Endocr Metab Disord 2003;4:95–102.10.1023/A:1021831621210Search in Google Scholar
10. Okumiya T, Keulemans JL, Kroos MA, Van der Beek NM, Boer MA, et al. A new diagnostic assay for glycogen storage disease type II in mixed leukocytes. Mol Genet Metab 2006;88:22–8.10.1016/j.ymgme.2005.10.016Search in Google Scholar PubMed
11. Weinstein DA, Wolfsdorf JI. Effect of continuous glucose therapy with uncooked cornstarch on the long-term clinical course of type 1a glycogen storage disease. Eur J Pediatr 2002;161:35–9.10.1007/s00431-002-1000-2Search in Google Scholar PubMed
12. Harris JC. Psychosocial care of the child and family. In: Fernandes J, Saudubray JM, Berghe G van den, editors. Inborn metabolic diseases, 3rd ed. Berlin: Heidelberg, 2000:63–74.Search in Google Scholar
13. Coleman RA, Winter HS, Wolf B, Chen YT. Glycogen debranching enzyme deficiency: long-term study of serum enzyme activities and clinical features. J Inherit Metab Dis 1992;15:869–81.10.1007/BF01800225Search in Google Scholar PubMed
14. Seigel J, Weinstein DA, Hillman R, Colbert B, Matthews B, et al. Glycogen storage disease type IIIa presenting as non-ketotic hypoglycemia: use of a newly approved commercially available mutation analysis to non-invasively confirm the diagnosis. J Pediatr Endocrinol Metab 2008;21:587–90.10.1515/jpem-2008-210613Search in Google Scholar
15. Gremse DA, Bucurales JC, Balistreri WF. Efficacy of corn starch therapy in type III glycogen storage disease. Am J Clin Nutr 1990;52:671–4.10.1093/ajcn/52.4.671Search in Google Scholar PubMed
16. Lucchiari S, Santoro D, Pagliarani S, Comi GP. Clinical, biochemical and genetic features of glycogen debranching enzyme deficiency. Acta Myol 2007;26:72–4.Search in Google Scholar
17. Chen YT. Glycogen storage diseases. In: Scriver CR, Beaudet AL, Sly WS, Valle D, editors. The metabolic and molecular bases of inherited disease. New York: McGraw-Hill, 2001:1521–51.Search in Google Scholar
18. Demo E, Frush D, Gottfried M, Koepke J, Boney A, et al. Glycogen storage disease type III-hepatocellular carcinoma a long-term complication? J Hepatol 2007;46:492–8.10.1016/j.jhep.2006.09.022Search in Google Scholar PubMed PubMed Central
19. Bernier AV, Sentner CP, Correia CE, Theriaque DW, Shuster JJ, et al. Hyperlipidemia in glycogen storage disease type III: effect of age and metabolic control. J Inherit Metab Dis 2008;31:729–32.10.1007/s10545-008-0919-5Search in Google Scholar PubMed PubMed Central
20. Rake J, Visser G, Labrune P, Leonard JV, Ullrich K, et al. Guidelines for management of glycogen storage disease type I. European study on glycogen storage disease type I (ESGSD I). Eur J Pediatr 2002;161:112–9.10.1007/BF02680007Search in Google Scholar
21. Smith D, Pernet A, Hallett WA, Bingham E, Marsden PK, et al. Lactate: a preferred fuel for human brain metabolism in vivo. J Cereb Blood Flow Metab 2003;6:658–64.10.1097/01.WCB.0000063991.19746.11Search in Google Scholar PubMed
22. Hagen T, Korson MS, Wolfsdorf JI. Urinary lactate excretion to monitor the efficacy of treatment of type I glycogen storage disease. Mol Genet Metab 2000;70:189–95.10.1006/mgme.2000.3013Search in Google Scholar PubMed
©2015 by De Gruyter
Articles in the same Issue
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- Selected papers from the 3rd International Multidisciplinary Postgraduate Course, April 24–27, 2014
- Childhood craniopharyngioma – current status and recent perspectives in diagnostics and treatment
- The importance of interdisciplinary communication with patients about complex, chronic illnesses: our experiences as parents of a child with a craniopharyngioma
- Adamantinomatous craniopharyngioma: pathology, molecular genetics and mouse models
- Endocrine aspects and sequel in patients with craniopharyngioma
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- Eating behavior, weight problems and eating disorders in 101 long-term survivors of childhood-onset craniopharyngioma
- Carbohydrate-lipid profile and use of metformin with micronized fenofibrate in reducing metabolic consequences of craniopharyngioma treatment in children: single institution experience
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- Review article
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- Images in pediatric endocrinology
- Intestinal invagination in diabetic ketoacidosis: case report
- Original articles
- Managing incidental findings and disclosure of results in a paediatric research cohort – the LIFE Child Study cohort
- The heterogeneity of hyperinsulinaemic hypoglycaemia in 19 patients with Beckwith-Wiedemann syndrome due to KvDMR1 hypomethylation
- Gender differences and age-related changes in body fat mass in Tibetan children and teenagers: an analysis by the bioelectrical impedance method
- A study of genes involved in adipocyte differentiation
- Initiation of growth hormone therapy in idiopathic short stature: do gender differences exist?
- The influence of puberty on vitamin D status in obese children and the possible relation between vitamin D deficiency and insulin resistance
- Growth failure associated with early neglect: pilot comparison of neglected US children and international adoptees
- Effect of growth hormone deficiency on brain MRI findings among children with growth restrictions
- Variability of lipid and lipoprotein concentrations during puberty in Brazilian boys
- Skin advanced glycation endproducts are elevated at onset of type 1 diabetes in youth
- Can optical coherence tomography predict early retinal microvascular pathology in type 1 diabetic adolescents without minimal diabetic retinopathy? A single-centre study
- Decreased levels of homoarginine and asymmetric dimethylarginine in children with type 1 diabetes: associations with cardiovascular risk factors but no effect by atorvastin
- Influence of catch-up growth on abdominal fat distribution in very low birth weight children – cohort study
- Thyrotoxic, hypokalemic periodic paralysis (THPP) in adolescents
- Pulmonary function in children with type 1 diabetes mellitus
- Identification of deletions in children with congenital hypothyroidism and thyroid dysgenesis with the use of multiplex ligation-dependent probe amplification
- Effect of l-thyroxine supplementation on infants with transient hypothyroxinemia of prematurity at 18 months of corrected age: randomized clinical trial
- Vitamin D-containing supplements for children between 1–3 years of age: Are they essential for bone health?
- Anti-Mullerian hormone levels in American girls by age and race/ethnicity
- The effect of tailoring of cornstarch intake on stature in children with glycogen storage disease type III
- Thyroid disease in Chinese girls with Turner syndrome
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- Pure gonadal dysgenesis (Swyer syndrome) due to microdeletion in the SRY gene: a case report
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- A case of pediatric ectopic thyroid in lateral lymph nodes
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Articles in the same Issue
- Frontmatter
- Highlight: Childhood Craniopharyngioma
- Selected papers from the 3rd International Multidisciplinary Postgraduate Course, April 24–27, 2014
- Childhood craniopharyngioma – current status and recent perspectives in diagnostics and treatment
- The importance of interdisciplinary communication with patients about complex, chronic illnesses: our experiences as parents of a child with a craniopharyngioma
- Adamantinomatous craniopharyngioma: pathology, molecular genetics and mouse models
- Endocrine aspects and sequel in patients with craniopharyngioma
- Surgery for pediatric craniopharyngiomas: is less more?
- Eating behavior, weight problems and eating disorders in 101 long-term survivors of childhood-onset craniopharyngioma
- Carbohydrate-lipid profile and use of metformin with micronized fenofibrate in reducing metabolic consequences of craniopharyngioma treatment in children: single institution experience
- Effects of T3 treatment on brown adipose tissue and energy expenditure in a patient with craniopharyngioma and hypothalamic obesity
- Hypothalamic obesity after treatment for craniopharyngioma: the importance of the home environment
- Review article
- Association study of TAC3 and TACR3 gene polymorphisms with idiopathic precocious puberty in Chinese girls
- Images in pediatric endocrinology
- Intestinal invagination in diabetic ketoacidosis: case report
- Original articles
- Managing incidental findings and disclosure of results in a paediatric research cohort – the LIFE Child Study cohort
- The heterogeneity of hyperinsulinaemic hypoglycaemia in 19 patients with Beckwith-Wiedemann syndrome due to KvDMR1 hypomethylation
- Gender differences and age-related changes in body fat mass in Tibetan children and teenagers: an analysis by the bioelectrical impedance method
- A study of genes involved in adipocyte differentiation
- Initiation of growth hormone therapy in idiopathic short stature: do gender differences exist?
- The influence of puberty on vitamin D status in obese children and the possible relation between vitamin D deficiency and insulin resistance
- Growth failure associated with early neglect: pilot comparison of neglected US children and international adoptees
- Effect of growth hormone deficiency on brain MRI findings among children with growth restrictions
- Variability of lipid and lipoprotein concentrations during puberty in Brazilian boys
- Skin advanced glycation endproducts are elevated at onset of type 1 diabetes in youth
- Can optical coherence tomography predict early retinal microvascular pathology in type 1 diabetic adolescents without minimal diabetic retinopathy? A single-centre study
- Decreased levels of homoarginine and asymmetric dimethylarginine in children with type 1 diabetes: associations with cardiovascular risk factors but no effect by atorvastin
- Influence of catch-up growth on abdominal fat distribution in very low birth weight children – cohort study
- Thyrotoxic, hypokalemic periodic paralysis (THPP) in adolescents
- Pulmonary function in children with type 1 diabetes mellitus
- Identification of deletions in children with congenital hypothyroidism and thyroid dysgenesis with the use of multiplex ligation-dependent probe amplification
- Effect of l-thyroxine supplementation on infants with transient hypothyroxinemia of prematurity at 18 months of corrected age: randomized clinical trial
- Vitamin D-containing supplements for children between 1–3 years of age: Are they essential for bone health?
- Anti-Mullerian hormone levels in American girls by age and race/ethnicity
- The effect of tailoring of cornstarch intake on stature in children with glycogen storage disease type III
- Thyroid disease in Chinese girls with Turner syndrome
- Patient reports
- Pure gonadal dysgenesis (Swyer syndrome) due to microdeletion in the SRY gene: a case report
- Hypophosphatemic rickets caused by a novel splice donor site mutation and activation of two cryptic splice donor sites in the PHEX gene
- Novel heterozygous IGF1R mutation in two brothers with developing impaired glucose tolerance
- A case of pediatric ectopic thyroid in lateral lymph nodes
- Hyperostosis-hyperphosphatemia syndrome (HHS): report of two cases with a recurrent mutation and review of the literature
- Needle detachment from the Sure-T® infusion set in two young children with diabetes mellitus (DM) treated with continuous subcutaneous insulin infusion (CSII) and unexplained hyperglycaemia
- Familial dysalbuminemic hyperthyroxinemia in a 4-year-old girl with hyperactivity, palpitations and advanced dental age: how gold standard assays may be misleading
