Childhood dermatomyositis associated with intracranial tumor and liver cysts (CROSBI ID 129087)
Prilog u časopisu | izvorni znanstveni rad | međunarodna recenzija
Podaci o odgovornosti
Barišić, Nina ; Jakić-Razumović, Jasminka ; Harjaček, Miroslav ; Fanin Marina ; Lochmüller, Hanns ; Lehman, Ivan ; Angelini, Corrado
engleski
Childhood dermatomyositis associated with intracranial tumor and liver cysts
We present a girl with dermatomyositis, liver cysts and choroid plexus papilloma who was treated and followed for 7 years. Muscle histology revealed an inflammatory muscle disease and similar changes were detected in a brain tumor that was surgically removed at onset. Western blot analysis of the muscle revealed severely reduced calpain-3 protein. She was treated with pulse methylprednisolone treatment (800mg i.v. for 4 days) followed by oral prednisone treatment (16 mg on alternate day) for 14 months, which improved muscle strength. Moreover, the cystic liver formations disappeared during steroid treatment. This is an unusual association of muscular disorder, steroid responsive liver cysts, intracranial tumor and secondary calpain-3 deficiency. We speculate that this association is not coincidental, but mediated by an autoimmune attack against an antigen that is shared among the target tissues
dermatomyositis ; choroid plexus papilloma ; liver cysts ; calpain-3 deficiency ; child
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Podaci o izdanju
11 (2)
2007.
76-80
objavljeno
1090-3798
1532-2130
10.1016/j.ejpn.2006.11.004
Povezanost rada
Kliničke medicinske znanosti