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10.1186/s13023-016-0473-4 http://scihub22266oqcxt.onion/10.1186/s13023-016-0473-4 C4928260!4928260!27356758     free     free     free Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 263.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Orphanet+J+Rare+Dis 2016 ; 11 (ä): ä Nephropedia Template TP {{tp|p=27356758|t=2016. Multicenter Female Fabry Study (MFFS) - clinical survey on current treatment of females with Fabry disease |pdf=|usr=}}{{27356758}} gab.com Text Multicenter Female Fabry Study (MFFS) - clinical survey on current treatment of females with Fabry disease JO: Orphanet J Rare Dis http://www.kidney.de/pget.php?&tw=1&pmid=27356758 #FOAvip Background: The aim of the present study was to assess manifestations of and applied treatment concepts for females with Fabry disease (FD) according to the current European Fabry Guidelines. Methods: Between 10/2008 and 12/2014, data from the most recent visit of 261 adult female FD patients from six German Fabry centers were retrospectively analyzed. Clinical presentation and laboratory data, including plasma lyso-Gb3 levels were assessed. Results: Fifty-five percent of females were on enzyme replacement therapy (ERT), according to recent European FD guidelines. Thirty-three percent of females were untreated although criteria for ERT initiation were fulfilled. In general, the presence of left ventricular hypertrophy (LVH) seemed to impact more on ERT initiation than impaired renal function. In ERT-naïve females RAAS blockers were more often prescribed if LVH was present rather than albuminuria. Affected females with missense mutations showed a similar disease burden compared to females with nonsense mutations. Elevated plasma lyso-Gb3 levels in ERT-naïve females seem to be a marker of disease burden, since patients showed comparable incidences of organ manifestations even if they were ~8 years younger than females with normal lyso-Gb3 levels. Conclusion: The treatment of the majority of females with FD in Germany is in line with the current European FD guidelines. However, a relevant number of females remain untreated despite organ involvement, necessitating a careful reevaluation of these females. Electronic supplementary material: The online version of this article (doi:10.1186/s13023-016-0473-4) contains supplementary material, which is available to authorized users. Twit Text FOAVip Multicenter Female Fabry Study (MFFS) - clinical survey on current treatment of females with Fabry disease ????Orphanet J Rare Dis http://www.kidney.de/pget.php?&tw=1&pmid=27356758 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=27356758 #FOAvip English Wikipedia <ref>{{Cite pmid|27356758}}</ref> Multicenter Female Fabry Study (MFFS) - clinical survey on current treatment of females with Fabry disease #MMPMID27356758 Lenders M; Hennermann JB; Kurschat C; Rolfs A; Canaan-Kühl S; Sommer C; Üçeyler N; Kampmann C; Karabul N; Giese AK; Duning T; Stypmann J; Krämer J; Weidemann F; Brand SM; Wanner C; Brand E Orphanet J Rare Dis 2016[]; 11 (ä): ä PMID27356758show ga Background: The aim of the present study was to assess manifestations of and applied treatment concepts for females with Fabry disease (FD) according to the current European Fabry Guidelines. Methods: Between 10/2008 and 12/2014, data from the most recent visit of 261 adult female FD patients from six German Fabry centers were retrospectively analyzed. Clinical presentation and laboratory data, including plasma lyso-Gb3 levels were assessed. Results: Fifty-five percent of females were on enzyme replacement therapy (ERT), according to recent European FD guidelines. Thirty-three percent of females were untreated although criteria for ERT initiation were fulfilled. In general, the presence of left ventricular hypertrophy (LVH) seemed to impact more on ERT initiation than impaired renal function. In ERT-naïve females RAAS blockers were more often prescribed if LVH was present rather than albuminuria. Affected females with missense mutations showed a similar disease burden compared to females with nonsense mutations. Elevated plasma lyso-Gb3 levels in ERT-naïve females seem to be a marker of disease burden, since patients showed comparable incidences of organ manifestations even if they were ~8 years younger than females with normal lyso-Gb3 levels. Conclusion: The treatment of the majority of females with FD in Germany is in line with the current European FD guidelines. However, a relevant number of females remain untreated despite organ involvement, necessitating a careful reevaluation of these females. Electronic supplementary material: The online version of this article (doi:10.1186/s13023-016-0473-4) contains supplementary material, which is available to authorized users. äMulticenter Female Fabry Study (MFFS) - clinical survey on current tre(epmc).pdf DeepDyve Pubget Overpricing