Improvement of cardiomyopathy after high-fat diet in two siblings with glycogen storage disease type iii

JIMD Reports

Volumn 17, Issue , 2014, Pages 91-95

  Brambilla, Alessandra ^a   Mannarino, Savina ^b   Pretese, Roberta ^a   Gasperini, Serena ^a   Galimberti, Cinzia ^a   Parini, Rossella ^a  

^a SAN GERARDO HOSPITAL   (Italy)

^b FONDAZIONE IRCCS POLICLINICO SAN MATTEO   (Italy)

Author keywords

Amylo 1,6 glucosidase; Cardiac hypertrophy; Gsd iii; High protein diet; Ketogenic diet; Low carbohydrate diet

Indexed keywords

EID: 84937250916     PISSN: 21928304     EISSN: 21928312     Source Type: Book Series    
DOI: 10.1007/8904_2014_343     Document Type: Chapter

References (6)

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2. Dagli AI, Zori RT, McCune H, Ivsic T, Maisenbacher MK, Weinstein DA (2009) Reversal of glycogen storage disease type IIIa-related cardiomyopathy with modification of diet. J Inherit Metab Dis Suppl 32(Suppl 1):S103–106
3. Fuehrlein BS, Rutemberg MS, Silver JN et al (2004) Differential metabolic effects of saturated versus polyunsaturated fats in ketogenic diets. J Clin Endocrinol Metab 89:1641–1645
4. Kishnani PS, Austin SL, Arn P et al (2010) Glycogen storage disease type III diagnosis and management guidelines. Genet Med 12:446–463
5. Sentner CP, Calistan K, Vletter WB, Smit GPA (2012) Heart failure due to severe hypertrophic cardiomyopathy reversed by low calorie, high protein dietary adjustments in a Glycogen Storage Disease type IIIa patient. JIMD Rep 5:13–16
6. Valayannopoulos V, Bajolle F, Arnoux JB et al (2011) Successful treatment of severe cardiomyopathy in glycogen storage disease type III with D,L-3-hydroxybutyrate, ketogenic and high-protein diet. Pediatr Res 70(6):638–641