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Titolo:
PROGRESSION OF X-LINKED ADRENOLEUKODYSTROPHY UNDER INTERFERON-BETA THERAPY
Autore:
KORENKE GC; CHRISTEN HJ; KRUSE B; HUNNEMAN DH; HANEFELD F;
Indirizzi:
UNIV GOTTINGEN,DEPT PAEDIAT & NEUROPAEDIAT,ROBERT KOCH STR 40 D-37075GOTTINGEN GERMANY
Titolo Testata:
Journal of inherited metabolic disease
fascicolo: 1, volume: 20, anno: 1997,
pagine: 59 - 66
SICI:
0141-8955(1997)20:1<59:POXAUI>2.0.ZU;2-8
Fonte:
ISI
Lingua:
ENG
Soggetto:
IMMUNOSUPPRESSION; FAILURE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
15
Recensione:
Indirizzi per estratti:
Citazione:
G.C. Korenke et al., "PROGRESSION OF X-LINKED ADRENOLEUKODYSTROPHY UNDER INTERFERON-BETA THERAPY", Journal of inherited metabolic disease, 20(1), 1997, pp. 59-66

Abstract

The cerebral phenotype of X-linked adrenoleukodystrophy (ALD) is a rapidly progressive neurodegenerative disorder characterized by a cerebral inflammatory response and elevated very long-chain fatty acids (VLCFA). Interferon-beta (INFB) is known to suppress the synthesis of tumour necrosis factor alpha and interferon-gamma, which have been reported to be elevated in the margin of the areas of demyelination in ALD brains. We report on treatment with interferon-beta in 8 patients with cerebral ALD, who additionally received glycerol trioleate/glycerol trierucate. INFB-1a (Rebif, Serono, Switzerland) was given subcutaneouslyonce a week, 3 million units for the first 3 months and 6 million units for the next 9 months. All patients showed an unimpeded progressionof neurological symptoms during INFB therapy. Therapy was stopped within 6 months in 4 patients because of the fast neurological deterioration with loss of walking. In all patients the MRI demonstrated a progression of demyelination with a qualitatively unchanged gadolinium enhancement. Further studies are needed to elucidate the pathomechanism ofdemyelination in ALD in order to find an effective therapy for cerebral ALD patients.

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Documento generato il 01/10/20 alle ore 04:40:48