Key result
Immunological abnormalities, including CD4 positive cells gathered among necrotic muscle fibers, were present in the specimens of four childhood-onset nemaline myopathy patients.
Why the study?
Are immunological abnormalities present in the muscle specimens of childhood-onset nemaline myopathy patients without autoimmune diseases?
Case Report (n=4)
Are immunological abnormalities present in the muscle specimens of childhood-onset nemaline myopathy patients without autoimmune diseases?
Immunological abnormalities, including the presence of CD4 positive cells, can be found in muscle specimens of childhood-onset nemaline myopathy patients without autoimmune diseases.
May suggest immune involvement in childhood-onset nemaline myopathy; hypothesis-generating and should not yet change practice.
We examined whether immunological abnormalities can be found in the specimens of four childhood-onset nemaline myopathy (NM) patients without autoimmune diseases. Pathological examination revealed that nemaline rods were found in all specimens. The immunohistochemical results showed that CD4 positive cells and some other cells were gathered among the necrotic muscle fibers. We conclude that immunological abnormalities are present in the specimens of certain childhood-onset NM patients without autoimmune diseases. Further evaluation of the immunological changes is warranted in childhood-onset NM patients.
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Jiang et al. (2012) conducted a case report in Childhood-onset nemaline myopathy (n=4). Immunological abnormalities was evaluated on Presence of immunological abnormalities in muscle specimens. Immunological abnormalities, including CD4 positive cells gathered among necrotic muscle fibers, were present in the specimens of four childhood-onset nemaline myopathy patients.
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