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Möhrenschlager, M. ; Ollert, M.* ; Ring, J.

A study on serum IgE and clinical symptomatology of atopy in patients suffering from the lysosomal storage disorder Fabry disease.

J. Eur. Acad. Dermatol. Venereol. 22, 692-695 (2008)
DOI PMC
Open Access Green möglich sobald Postprint bei der ZB eingereicht worden ist.
Background Lysosomal storage disorders may impair intracellular lysosomal processing of antigen with consequences for antibody production [e.g. immunoglobulin E (IgE)] and atopic disease status. Aims Serum concentration of total IgE as well as clinical symptoms of atopic disorders as an indirect consequence of lysosomal impairment of antigen processing were studied in male and female Fabry disease (FD) patients with and without replacement of the missing lysosomal enzyme, alpha-galactosidase A (alpha-Gal A). Methods Observational study in 31 adult FD patients with measurements of total serum IgE concentration. Questionnaire-derived data were obtained for atopic eczema (AE) skin lesions, allergic rhinoconjunctivitis (RCA) and allergic asthma (AA) at present or in the past. Results Among 12 FD males under enzyme replacement therapy (ERT), 2 showed total IgE concentrations above 100 kU/L. Clinical symptoms for AA were found in 2, RCA and AE in 1, respectively. Among 10 FD females under ERT, 4 showed total IgE concentrations above 100 kU/L. Clinical symptoms for AA were found in 4, RCA in 2 and AE in 2. Among 9 females without ERT, 2 showed total IgE concentrations above 100 kU/L. Clinical symptoms for AA were found in 2, RCA in 2 and AE in none. Conclusions FD patients may demonstrate an increased total serum IgE concentration and may show symptoms of atopic disorders (AA, RCA, AE) in a prevalence rate comparable to international experience for individuals without FD. There was no difference between patients with and without ERT. Lack of detection of AE in females without ERT is suggested to be caused by the small sample size. FD patients without any alpha-Gal A activity prior to initiation of ERT should be in the focus of future studies.
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Publikationstyp Artikel: Journalartikel
Dokumenttyp Wissenschaftlicher Artikel
Schlagwörter alpha-galactosidase A deficiency; allergic asthma; allergic rhinoconjunctivitis; atopic eczema; enzyme replacement therapy; Fabry disease; immunoglobulin E; intracellular antigen processing; lysosomal storage disorder; lysosome
Sprache englisch
Veröffentlichungsjahr 2008
HGF-Berichtsjahr 0
ISSN (print) / ISBN 0926-9959
e-ISSN 1468-3083
Zeitschrift Journal of the European Academy of Dermatology and Venereology
Quellenangaben Band: 22, Heft: 6, Seiten: 692-695 Artikelnummer: , Supplement: ,
Verlag Wiley
Verlagsort Oxford
Begutachtungsstatus Peer reviewed
Institut(e) Institute of Lung Health and Immunity (LHI)
Institute of Epidemiology (EPI)
POF Topic(s) 30202 - Environmental Health
PSP-Element(e) G-521200-001
FE 73991
PubMed ID 18384565
DOI 10.1111/j.1468-3083.2008.02638.x
Erfassungsdatum 2008-01-01
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