http://www.cnr.it/ontology/cnr/individuo/prodotto/ID27836
Cyclosporine a in ullrich congenital muscular dystrophy: long-term results. (Articolo in rivista)
- Type
- Label
- Cyclosporine a in ullrich congenital muscular dystrophy: long-term results. (Articolo in rivista) (literal)
- Anno
- 2011-01-01T00:00:00+01:00 (literal)
- Alternative label
Merlini L, Sabatelli P, Armaroli A, Gnudi S, Angelin A, Grumati P, Michelini ME, Franchella A, Gualandi F, Bertini E, Maraldi NM, Ferlini A, Bonaldo P, Bernardi P. (2011)
Cyclosporine a in ullrich congenital muscular dystrophy: long-term results.
in Oxidative medicine and cellular longevity (Online)
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- Merlini L, Sabatelli P, Armaroli A, Gnudi S, Angelin A, Grumati P, Michelini ME, Franchella A, Gualandi F, Bertini E, Maraldi NM, Ferlini A, Bonaldo P, Bernardi P. (literal)
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- ISI Web of Science (WOS) (literal)
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- Section of Medical Genetics, Department of Experimental and Diagnostic Medicine, University of Ferrara, 44121 Ferrara, Italy; Laboratorio Di Biologia Cellulare Muscoloscheletrica, Istituto Ortopedico Rizzoli, 40136 Bologna, Italy; Istituto di Genetica Molecolare, CNR, Istituto Ortopedico Rizzoli, 40136 Bologna, Italy; Medicina Generale, Istituto Ortopedico Rizzoli, 40136 Bologna, Italy; Department of Biomedical Sciences, University of Padova, 35121 Padova, Italy; Department of Histology, Microbiology and Medical Biotechnology, University of Padova, 35121 Padova, Italy; Pediatric Surgery Department, SantAnna Hospital, Corso Giovecca 203, 44121 Ferrara, Italy; Unit for Neuromuscular Disorders, Department of Neurosciences and Laboratory of Molecular Medicine; Bambino Gesù Childrens Research Hospital, 00165 Roma, Italy (literal)
- Titolo
- Cyclosporine a in ullrich congenital muscular dystrophy: long-term results. (literal)
- Abstract
- Six individuals with Ullrich congenital muscular dystrophy (UCMD) and mutations in the genes-encoding collagen VI, aging 5-9, received 3-5 mg/kg of cyclosporine A (CsA) daily for 1 to 3.2 years. The primary outcome measure was the muscle strength evaluated with a myometer and expressed as megalimbs. The megalimbs score showed significant improvement (P = 0.01) in 5 of the 6 patients. Motor function did not change. Respiratory function deteriorated in all. CsA treatment corrected mitochondrial dysfunction, increased muscle regeneration, and decreased the number of apoptotic nuclei. Results from this study demonstrate that long-term treatment with CsA ameliorates performance in the limbs, but not in the respiratory muscles of UCMD patients, and that it is well tolerated. These results suggest considering a trial of CsA or nonimmunosuppressive cyclosporins, that retains the PTP-desensitizing properties of CsA, as early as possible in UCMD patients when diaphragm is less compromised (literal)
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