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Table 4 Morphologic criteria

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

    Number of affected fibers
HE Internalized nuclei + < 10%
++ 10–30%
+++ > 30%
Fibrotic areas + < 5%
++ 5–30%
+++ > 30%
Vacuoles + < 5%
++ 5–30%
+++ > 30%
Ring Fibers + < 5%
++ 5–10%
+++ > 10%
Necrosis + < 2%
++ 2–5%
+++ > 5%
Regeneration + < 2%
++ 2–5%
+++ > 5%
PAS Augmented intensity + < 5%
++ 5–30%
+++ > 30%
Positive vacuoles + <  5%
++ 5–30%
+++ > 30%
IHC SQSTM1/p62 + <  5%
++ 5–30%
+++ > 30%
EM Intermyofibrillar glycogen accumulation + < 5%
++ 5–30%
+++ > 30%
Autophagosomes + < 5%
++ 5–30%
+++ > 30%
Autophagic structures + < 5%
++ 5–30%
+++ > 30%
  1. For abbreviations see Table 3