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:

Overview

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include:^[1]
- Cardiology recommendations
- [Strategy 2]
- [Strategy 3]

Usually there is no restriction on exercise.
Exercise should be restricted if:
- There is significant ventricular hypertrophy with ventricular outflow tract obstruction
- There is heart rhythm abnormalities

Routine 12 lead ECG should be performed every year to screen for ventricular hypertrophy.
Additional electrophysiological monitoring is indicated if:
- There is presence if clinical symptoms such as palpitations
- ECG abnormality develops
- Individuals develop moderate to severe ventricular hypertrophy on serial echo

Periodic echocardiogram should be done to measure wall thickness, ventricular mass, systolic function (shortening fraction and ejection fraction), and diastolic function.^[2]

It is recommended to perform serial echocardiogram beginning at the time of diagnosis.

It is recommended to perform serial echocardiogram beginning at the age of 5 years.

↑ 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.
↑ 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.