Reversed papilledema in an MPS VI patient with galsulfase (Naglazyme®) therapy
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چکیده
منابع مشابه
Natural history and galsulfase treatment in mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome)--10-year follow-up of patients who previously participated in an MPS VI Survey Study.
Mucopolysaccharidosis VI (MPS VI) is a clinically heterogeneous and progressive disorder with multiorgan manifestations caused by deficient N-acetylgalactosamine-4-sulfatase activity. A cross-sectional Survey Study in individuals (n = 121) affected with MPS VI was conducted between 2001 and 2002 to establish demographics, urinary glycosaminoglycan (GAG) levels, and clinical progression of disea...
متن کاملpapilledema: an unusual finding in a patient with herpes encephalitis
herpes simplex virus accounts for 10 to 25% of sporadic viral encephalitis throughout the world among people of different age with two peaks, one at 5 to 30 and the other at > 50 years of age. pathologic process includes focal brain tissue inflammation and necrosis (predominantly temporal lobe). therefore local neurological signs and symptoms will ensue. although csf pressure rising due to infl...
متن کاملLong-term galsulfase enzyme replacement therapy in Taiwanese mucopolysaccharidosis VI patients: A case series
BACKGROUND Information regarding the long-term outcome of enzyme replacement therapy (ERT) with recombinant human N-acetylgalactosamine 4-sulfatase (rhASB, galsulfase, Naglazyme®, BioMarin Pharmaceutical Inc.) for Taiwanese patients with mucopolysaccharidosis (MPS) VI is limited. METHODS Nine Taiwanese patients with MPS VI (4 males and 5 females; age range, 1.4 to 21.1 years) treated with wee...
متن کاملDiagnostic difficulties in patients with attenuated form of MPS VI
Background Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome) is an autosomal recessive lysosomal storage disorder determined by mutations in the arylsulfatase B gene located in chromosome 5. Pathogenic mutations of this gene result in reduced or absent activity of enzyme arylsulfatase B (N-acetylgalactosamine 4-sulfatase, ARSB). Incomplete degradation and cellular accumulation of glyco...
متن کاملDRUG PROFILE Galsulfase: enzyme-replacement therapy for mucopolysaccharidosis Type VI (Maroteaux–Lamy syndrome)
10.1586/14750708.3.1.9 © 200 Since enzyme-replacement therapy has been successfully introduced for patients with Gaucher disease, Fabry disease and mucopolysaccharidosis Type I, the principle of this treatment has also been taken into consideration for individuals who are affected by mucopolysaccharidosis Type VI (Maroteaux–Lamy disease), a rare lysosomal storage disorder with multiple organ an...
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ژورنال
عنوان ژورنال: International Ophthalmology
سال: 2008
ISSN: 0165-5701,1573-2630
DOI: 10.1007/s10792-008-9213-7