Glycogen storage disease type III secondary prevention

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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]


Effective measures for the secondary prevention of glycogen storage disease type 3 include blood glucose monitoring, prevent overtreatment, general medical care recommendations, gastrointestinal/nutritional recommendations, cardiology recommendations, physical therapy, surgery/anesthesia recommendations, and gynecological/obstetrical recommendations.

Secondary Prevention

Blood glucose (BG) monitoring

  • The following blood glucose levels should be checked in general:
    • Before meals
    • Before cornstarch (CS) intake
    • Before bed
    • First thing in the morning
  • If the cornstarch dose is changed, blood glucose 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 blood glucose level 70-140 mg/dl.

Prevent overtreatment

Growth tracking

  • Growth should be tracked through parameters including:
  • Changes in growth pattern is observed in poor metabolic control of GSD type 3.

General medical care recommendations

Gastrointestinal/nutritional recommendations

Cardiology recommendations



  • Periodic echocardiogram should be done to measure wall thickness, ventricular mass, systolic function (shortening fraction and ejection fraction), and diastolic function.[2]
Glycogen storage disease type 3a
  • It is recommended to perform serial echocardiogram beginning at the time of diagnosis.
Glycogen storage disease type 3b
  • It is recommended to perform serial echocardiogram beginning at the age of 5 years.

Physical Therapy

  • Periodic assessment is recommended for the following:
    • Strength and endurance (both direct and functional)
    • Standardized gross and fine motor testing
  • If hepatomegaly is present, contact sports should be avoided.
  • If carpal tunnel syndrome is present due to deposition of glycogen in median nerve, wrist splint during sleep is recommended.
  • Orthoses
    • Custom-molded foot orthoses
    • Taller orthotic intervention with ankle-foot orthoses are recommended for adults with:
      • More severe malalignment
      • Instability
      • Distal weakness

Surgery and anesthesia recommendations

Gynecological and obstetrical recommendations


  1. Kishnani, Priya S; Austin, Stephanie L; Arn, Pamela; Bali, Deeksha S; Boney, Anne; Case, Laura E; Chung, Wendy K; Desai, Dev M; El-Gharbawy, Areeg; Haller, Ronald; Smit, G Peter A; Smith, Alastair D; Hobson-Webb, Lisa D; Wechsler, Stephanie Burns; Weinstein, David A; Watson, Michael S (2010). "Glycogen Storage Disease Type III diagnosis and management guidelines". Genetics in Medicine. 12 (7): 446–463. doi:10.1097/GIM.0b013e3181e655b6. ISSN 1098-3600.
  2. Lee PJ, Deanfield JE, Burch M, Baig K, McKenna WJ, Leonard JV (1997). "Comparison of the functional significance of left ventricular hypertrophy in hypertrophic cardiomyopathy and glycogenosis type III". Am J Cardiol. 79 (6): 834–8. PMID 9070576.
  3. Lee PJ, Patel A, Hindmarsh PC, Mowat AP, Leonard JV (1995). "The prevalence of polycystic ovaries in the hepatic glycogen storage diseases: its association with hyperinsulinism". Clin Endocrinol (Oxf). 42 (6): 601–6. PMID 7634500.
  4. Giannitrapani L, Soresi M, La Spada E, Cervello M, D'Alessandro N, Montalto G (2006). "Sex hormones and risk of liver tumor". Ann N Y Acad Sci. 1089: 228–36. doi:10.1196/annals.1386.044. PMID 17261770.
  5. Mairovitz V, Labrune P, Fernandez H, Audibert F, Frydman R (2002). "Contraception and pregnancy in women affected by glycogen storage diseases". Eur J Pediatr. 161 Suppl 1: S97–101. doi:10.1007/s00431-002-1013-x. PMID 12373581.

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