Literature DB >> 20532791

[Lysosomal storage diseases].

B Manger1.   

Abstract

Lysosomal storage diseases are a heterogeneous group of disorders caused by lysosomal enzyme dysfunction. Individually they are very rare, but this group as a whole has a prevalence of more than 1:8,000 live births. While severe phenotypes are easily diagnosed this can be a real challenge with attenuated forms. Because musculoskeletal complaints are frequently the first reason for the patient to seek medical advice, the rheumatologist plays a key role for the early recognition of these diseases. Since several of these can be treated very effectively by enzyme replacement, a timely diagnosis and start of therapy are essential to avoid irreversible organ damage and poor quality of life. Therefore, each clinical rheumatologist should be aware of the cardinal symptoms of lysosomal storage diseases.

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Year:  2010        PMID: 20532791     DOI: 10.1007/s00393-010-0627-z

Source DB:  PubMed          Journal:  Z Rheumatol        ISSN: 0340-1855            Impact factor:   1.372


  23 in total

1.  Scheie syndrome (MPS-IS) presented as bilateral trigger thumb.

Authors:  Yoshito Matsui; Hidehiko Kawabata; Masahiro Nakayama; Keiichi Ozono; Hiroshi Kitoh; Kazuko Sukegawa; Hideki Tsuboi; Hideki Yoshikawa
Journal:  Pediatr Int       Date:  2003-02       Impact factor: 1.524

2.  Safety and efficacy of recombinant human alpha-galactosidase A replacement therapy in Fabry's disease.

Authors:  C M Eng; N Guffon; W R Wilcox; D P Germain; P Lee; S Waldek; L Caplan; G E Linthorst; R J Desnick
Journal:  N Engl J Med       Date:  2001-07-05       Impact factor: 91.245

3.  A follow-up study of MPS I patients treated with laronidase enzyme replacement therapy for 6 years.

Authors:  Monica Sifuentes; Robin Doroshow; Richard Hoft; Greg Mason; Irwin Walot; Michael Diament; Susan Okazaki; Kenneth Huff; Gerald F Cox; Stuart J Swiedler; Emil D Kakkis
Journal:  Mol Genet Metab       Date:  2006-09-29       Impact factor: 4.797

4.  American Rheumatism Association nomenclature and classification of arthritis and rheumatism (1983).

Authors:  J L Decker
Journal:  Arthritis Rheum       Date:  1983-08

5.  Enzyme replacement therapy in Fabry disease: a randomized controlled trial.

Authors:  R Schiffmann; J B Kopp; H A Austin; S Sabnis; D F Moore; T Weibel; J E Balow; R O Brady
Journal:  JAMA       Date:  2001-06-06       Impact factor: 56.272

Review 6.  Lysosomal storage diseases as differential diagnoses to rheumatic disorders.

Authors:  Hartmut Michels; Eugen Mengel
Journal:  Curr Opin Rheumatol       Date:  2008-01       Impact factor: 5.006

Review 7.  Rare monogenetic syndromes in rheumatology practice.

Authors:  K Manger; H Nüsslein; G Schett; B Manger
Journal:  Clin Rheumatol       Date:  2009-02-18       Impact factor: 2.980

Review 8.  [Gaucher disease, Fabry disease and mucopolysaccharidosis type I--how can the rheumatologist recognise these patients?].

Authors:  B Manger; E Menge; R Schaefer; C Haase; J Seidel; H Michels
Journal:  Z Rheumatol       Date:  2006-02       Impact factor: 1.372

9.  Fabry disease defined: baseline clinical manifestations of 366 patients in the Fabry Outcome Survey.

Authors:  A Mehta; R Ricci; U Widmer; F Dehout; A Garcia de Lorenzo; C Kampmann; A Linhart; G Sunder-Plassmann; M Ries; M Beck
Journal:  Eur J Clin Invest       Date:  2004-03       Impact factor: 4.686

10.  Fabry disease: baseline medical characteristics of a cohort of 1765 males and females in the Fabry Registry.

Authors:  C M Eng; J Fletcher; W R Wilcox; S Waldek; C R Scott; D O Sillence; F Breunig; J Charrow; D P Germain; K Nicholls; M Banikazemi
Journal:  J Inherit Metab Dis       Date:  2007-03-08       Impact factor: 4.750

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