Pregled bibliografske jedinice broj: 283021
Childhood dermatomyositis associated with intracranial tumor and liver cysts
Childhood dermatomyositis associated with intracranial tumor and liver cysts // European journal of paediatric neurology, 11 (2007), 2; 76-80 doi:10.1016/j.ejpn.2006.11.004 (međunarodna recenzija, članak, znanstveni)
CROSBI ID: 283021 Za ispravke kontaktirajte CROSBI podršku putem web obrasca
Naslov
Childhood dermatomyositis associated with intracranial tumor and liver cysts
Autori
Barišić, Nina ; Jakić-Razumović, Jasminka ; Harjaček, Miroslav ; Fanin Marina ; Lochmüller, Hanns ; Lehman, Ivan ; Angelini, Corrado
Izvornik
European journal of paediatric neurology (1090-3798) 11
(2007), 2;
76-80
Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni
Ključne riječi
dermatomyositis ; choroid plexus papilloma ; liver cysts ; calpain-3 deficiency ; child
Sažetak
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
Izvorni jezik
Engleski
Znanstvena područja
Kliničke medicinske znanosti
POVEZANOST RADA
Projekti:
0214213
Ustanove:
Klinički bolnički centar Zagreb,
Klinika za dječje bolesti
Profili:
Miroslav Harjaček
(autor)
Nina Barišić
(autor)
Ivan Lehman
(autor)
Jasminka Jakić-Razumović
(autor)
Citiraj ovu publikaciju:
Časopis indeksira:
- Current Contents Connect (CCC)
- Web of Science Core Collection (WoSCC)
- Science Citation Index Expanded (SCI-EXP)
- SCI-EXP, SSCI i/ili A&HCI
- Scopus
- MEDLINE