Glycogen storage disease type III natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Natural History, Complications, and Prognosis
Natural History
- If left untreated, patients with glycogen storage disease type 3 grow slowly and puberty is delayed.[1]
- The myopathy of glycogen storage disease type 3 typically develop in the third or fourth decades of life. It manifests as muscle weakness which is slow and progressive and usually involves large proximal muscle fo the shoulder and hips.
- Accumulation of limit dextrin in cardiac muscles causes cardiomyopathy with echocardiographic findings similar to idiopathic hypertrophic cardiomyopathy.
- Hepatomegaly improves as the age progresses.
Complications
- Common complications of glycogen storage disease type III include:[2][3][4][5][6]
- Left ventricular hypertrophy
- Cardiomyopathy
- Hyperlipidemia
- Hypercholesterolemia
- Hypertriglyceridemia
Prognosis
- Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
- Depending on the extent of the [tumor/disease progression/etc.] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
- The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
- [Subtype of disease/malignancy] is associated with the most favorable prognosis.
- The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
References
- ↑ Wolfsdorf JI, Weinstein DA (2003). "Glycogen storage diseases". Rev Endocr Metab Disord. 4 (1): 95–102. PMID 12618563.
- ↑ Lee P, Burch M, Leonard JV (1995). "Plasma creatine kinase and cardiomyopathy in glycogen storage disease type III". J Inherit Metab Dis. 18 (6): 751–2. PMID 8750616.
- ↑ Labrune P, Huguet P, Odievre M (1991). "Cardiomyopathy in glycogen-storage disease type III: clinical and echographic study of 18 patients". Pediatr Cardiol. 12 (3): 161–3. doi:10.1007/BF02238523. PMID 1876514.
- ↑ Coleman RA, Winter HS, Wolf B, Gilchrist JM, Chen YT (1992). "Glycogen storage disease type III (glycogen debranching enzyme deficiency): correlation of biochemical defects with myopathy and cardiomyopathy". Ann Intern Med. 116 (11): 896–900. PMID 1580445.
- ↑ Talente GM, Coleman RA, Alter C, Baker L, Brown BI, Cannon RA; et al. (1994). "Glycogen storage disease in adults". Ann Intern Med. 120 (3): 218–26. PMID 8273986.
- ↑ Smit GP, Fernandes J, Leonard JV, Matthews EE, Moses SW, Odievre M; et al. (1990). "The long-term outcome of patients with glycogen storage diseases". J Inherit Metab Dis. 13 (4): 411–8. PMID 2122109.