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Table 2 Clinical features of patients

From: Deep morphological analysis of muscle biopsies from type III glycogenesis (GSDIII), debranching enzyme deficiency, revealed stereotyped vacuolar myopathy and autophagy impairment

Patients   n = 30  
Proportion of female (%)   n = 11 33%
Median current age (years)   n = 28 52
Median age at biopsy (years)   n = 27 38
Onset age Before 1 years old 7 23,3%
First decade 8 26,7%
Second decade 3 10,0%
Third decade 2 6,7%
Fourth decade 1 3,3%
Fifth decade 3 10,0%
After 50 years old 3 10,0%
Unknown 3 10,0%
Clinical features Asymptomatic 1 3,3%
Muscles disease (fatigability, exercise intolerance, muscle weakness) 21 70,0%
Cardiac involvement (cardiomegaly, rhythmic abnormalities) 10 33,3%
Hepatic involvement (hypoglycemia, hepatopathy) 18 60,0%
Respiratory involvement 3 10,0%
Biopsied muscle L. biceps brachii 10 33,3%
L. vastus (medialis or lateralis) 7 23,3%
L. Deltoid 5 16,7%
L. Palmaris 1 3,3%
Tibial anterior 1 3,3%
R. Rectus femoris 1 3,3%
Unknown 5 16,7%