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Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024.

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Figure 2. . Skeletal muscle histology of two children with Salih myopathy taken at age four years (A and D) and 14 years (B and C).

Figure 2.

Skeletal muscle histology of two children with Salih myopathy taken at age four years (A and D) and 14 years (B and C).

A and D. The early biopsy shows (A) increased fiber size variability, abundant centrally located nuclei (CNLs) but no endomyseal fibrosis or necrosis (Van Gieson's stain; original magnification x250). (D) Type 1 fibers (dark) predominate and type 2 fibers (pale, arrows) are scanty (myofibrillar ATPase [4.3]; original magnification x250).

B and C. At age 14 years there is also a remarkable number of CNLs (B), associated with endomyseal fibrosis and few necrotic fibers (arrow) (GT; original magnification x250). (C) Oxidative staining reveals the minicore-like lesions (arrowheads; original magnification x250).

From: Salih Myopathy

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