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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