7/22/2019 Informationsbroschre fr rzte, Sphingotec

1/8

Grustlrcgsrfsflj cfob`dm crl`oot

spmfonjtcstprj-OY

7/22/2019 Informationsbroschre fr rzte, Sphingotec

2/8

Fo Icutsdmh`oi cotwfdlcht ecic dmtc Br`u fa H`ubc fmrcs Hcgcos Grustlrcgs, emrhfdm

wcrico ruoi >3.;;; Ocucrlr`oluonco if`nojstfzfcrt*. Ctw` cfo Rfcrtch `hhcr gctrjbbcoco

Br`uco fst gcf If`nojscstchhuon eoncr `hs 33 E`mrc uoi ecic zcmotc Br`u sjn`r eoncr `hs

93 E`mrc. Grustlrcgs fst i`aft ifc mubfnstc @rt icr Lrcgscrlr`oluon gcf Br`uco. Ec brm-

zcftfncr i`s Cotstcmco vjo Grustlrcgs crl`oot wfri, icstj mmcr sfoi ifc Dm`odco, fmo

crbjhnrcfdm zu gcm`oicho. Cotsdmcficoi fst gcr `udm, Wfsfljb`ltjrco br ifc Grustlrcgs-

cotwfdlhuon zu lcooco. B`ltjrco wfc z. G. @htcr, Cfotrftt icr crstco Wcnch gzw. icr Acoj-

p`usc, Ifdmtc ics Grustncwcgcs, gcr `udm ncoctfsdmc Rcroicruonco (Aut`tfjoco

sjnco`ootcr Grustlrcgsncoc) sfoi ofdmt gccfobhussg`r. @oicrc B`ltjrco, z. G. icr

Gjiy-A`ss-Foicx (GAF), wcrico iurdm Hcgcosncwjmomcftco wfc lrpcrhfdmc Gcttfnuon,

Cssncwjmomcftco uoi @hljmjhljosua gcstfaat. Aftmfhbc vjo capfrfsdmco Ajichhco

looco sjhdmc B`ltjrco zus`aaconcb`sst uoi foifvfiuchhc Wfsflco `om`oi gcl`ootcr

I`tco uoi Fobjra`tfjoco crrcdmoct wcrico. Aft [rjocurjtcosfo wuric ectzt cfo B`ltjr

ncbuoico, icr strlcr `hs `hhc gfsmcr gcl`ootco B`ltjrco aft ica Wfsflj, `o Grustlrcgs zu

crlr`olco, ljrrchfcrt.

Gcstcmt gcf cfocr Br`u cfo

crmmtcs Wfsflj, `o Grustlrcgs

zu crlr`olco, sj wfri fmr fo

ecica B`hh cfoc conc Rjrsjrnc,

z. G. iurdm Y`stuotcrsudmuonco

jicr rcnchafnc A`aaj-

nr`pmfc, capbjmhco.

*Lrcgs fo Icutsdmh`oi 7;;6/7;0;. 6. @usn`gc. Wjgcrt Ljdm-Fostftut (Mrsn) uoi ifc Ncschhsdm`bt

icr cpficafjhjnfsdmco Lrcgsrcnfstcr fo Icutsdmh`oi c.R. (Mrsn). Gcrhfo, 7;04

Wfsfljb`ltjrco br

Grustlrcgs sdmochhcrlcooco

7/22/2019 Informationsbroschre fr rzte, Sphingotec

3/8

[rjocurjtcosfo 0-00> (prj-OY) mfoncnco fst cfo

st`gfhcs Br`nacot ics Ocurjtcosfo-Rjrhubcr-Ajhc-

lhs uoi wfri fo nhcfdmca Rcrmhtofs wfc i`s Ocu-

rjtcosfo ncgfhict. Cs stchht i`aft cfoco `udm fo

icr H`gjrrjutfoc cfosctzg`rco Turrjn`ta`rlcr br

Ocurjtcosfo i`r.

Ocurjtcosfo fst cfo [cptfi `us 04 @afojsurco

uoi wfri fa ^cscothfdmco fo ico O-Uchhco ics

Iooi`ras ncgfhict. Tcfoc Brcfsctzuon fo ifc Ufrlu-

h`tfjo iurdm ico Iooi`ra wfri iurdm tfcrfsdmc

Bcttc uoi Uudlcr a`ncghfdm gccfobhusst, cs wfrlt

u. `. `hs Tttfnuonsmjrajo. Icr OY0-Wczcptjr ics

Ocurjtcosfos wfri vjo a`hfnoco Grustlrcgszchhco

gcrcxprfafcrt. Ifc OY0-Wczcptjr-@ltfvfcruon iurdm

Ocurjtcosfo foiuzfcrt cfo uoljotrjhhfcrtcs, fov`sfvcsUchhw`dmstua iurdm Rcrmfoicruon icr @pjptjsc

vjo Grustlrcgszchhco. Ocurjtcosfo stfauhfcrt i`s

Grustlrcgszchhw`dmstua uoi wfri, u. `. br Grust-

lrcgszchhco, ifrclt `otf-`pjptjtfsdm wfrlcoi

gcsdmrfcgco. I` Ocurjtcosfo fo vfvj uoi fo vftrj

fost`gfh fst, fst scfoc ifrcltc Gcstfaauon br ifc

H`gjrrjutfoc ofdmt nccfnoct.

[rjocurjtcosfo 0-00> uoi Ocurjtcosfo

st`gfh Ocurjtcosfo(07=-09;)

fost`gfh

Ocurjacifo O

(070-073)

D-tcraCoic

Tfno`hpcptfi

(74 @3)

Lrpcrcfncocs

Mjrajo

Ocurjtcosfo

gcwfrlt

uoljotrjhhfcrtcs

Uchhw`dmstua

[rjocurjtcosfo (0-00>)

7/22/2019 Informationsbroschre fr rzte, Sphingotec

4/8

Crncgofssc `us icr A`ha Ifct @oi D`odcr Ttuifc (AID)*fa E`mr 7;07 a`dmco

ico Uus`aacom`on zwfsdmco icr prj-OY-Ljozcotr`tfjo fa Ghut uoi ica Grust-

lrcgsrfsflj icuthfdm. Fo ifcscr prjspcltfvco Ljmjrtcostuifc wurico ruoi 7.;;;

Br`uco `us icr wcfghfdmco Ojra`hgcvhlcruon cfogczjnco, icoco zwfsdmco

0660 uoi 0669 Ghutph`sa`prjgco cotojaaco wurico. Ifcsc wurico `osdmhfc-

coi fo cfocr [rjgcog`ol nch`ncrt uoi 7;07 aft ica spmfonjtcst prj-OY ifc

ecwcfhfnco prj-OY-Ljozcotr`tfjoco crafttcht. Ifcsc ^crtc wurico aft ico gcr

ruoi 02 E`mrc vcrbjhntco uoi ncs`aachtco Fobjra`tfjoco zua Ncsuoimcfts-/

Crlr`oluonsst`tus icr fo ifc Ttuifc cfogczjncoco Br`uco vcrnhfdmco. 074 icr

Br`uco m`ttco fo icr Gcjg`dmtuonszcft Grustlrcgs cotwfdlcht. Mfcrgcf zcfntc sfdm,

i`ss crmmtc prj-OY-Ljozcotr`tfjoco aft cfoca lo`pp ircfb`dm crmmtco Wfsflj,

fa H`ubc icr bjhncoico 0;03 E`mrc `o Grustlrcgs zu crlr`olco, cfomcrncmco.

Ifc Crncgofssc icr AID-Ttuifc

ljootco fo cfocr wcftcrco Ttuifc,

icr A`ha [rcvcotfjo [rjecdtTtuifc (A[[, Rcrbbcothfdmuon fo

Rjrgcrcftuon), gcsttfnt uoi

mfosfdmthfdm icr Wfsflj`uss`nc sjn`r

ojdm gcrtrjbbco wcrico. @us

ico I`tco ifcscr Ttuifc crn`g sfdm

cfo d`. ocuob`dmcs Wfsflj zur Grust-

lrcgscotwfdlhuon gcf Rjrhfcnco

mjmcr prj-OY-Ljozcotr`tfjoco.

[rjocurjtcosfo5

icr Foifl`tjr brGrustlrcgsrfsflj

03 E`mrc

AID-Ttuifc A[[-Ttuifc

0; E`mrc 3 E`mrc 3 E`mrc

93

9;

43

4;

73

7;

03

0;

3

;

Grustlrcgscrlr`oluonco prj 0.;;; Br`uco

ofcirfncs prj-OY mjmcs prj-OY

Puchhco5 AID- uoi A[[-Ttuifc

* Ach`oicr, J, ct `h., [h`sa` [rjocurjtcosfo `oi Fodficodc jb If`gctcs, D`rifjv`sduh`r

Ifsc`sc, Grc`st D`odcr, `oi Ajrt`hfty. E@A@ (Ejuro`h jb tmc @acrfd`o Acifd`h @ssjdf`tfjo).

0;. Jdtjgcr 7;07, Gi. 4;=, 09, T. 0926->3.

7/22/2019 Informationsbroschre fr rzte, Sphingotec

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fo scfocr @uss`nclr`bt. Ifc crafttchtc prj-OY-

Ljozcotr`tfjo crh`ugt cfoc Cfostubuon ncncogcr

ica ojra`hco Grustlrcgsrfsflj cfocr Br`u.

**z. G. @htcr, Cfoo`mac ghutirudlscolcoicr Afttch,

Mjrajocrs`tztmcr`pfc, jr`hc Ljotr`zcptfv`,

Gfhiuonsofvc`u, @htcr gcf Aco`rdmc, @htcr gcf

0. Tdmw`oncrsdm`bt, @oz`mh icr Lfoicr, Acojp`usco-

Tt`tus, systjhfsdmcr Ghutirudl, GAF, If`gctcs achhftus,

W`udmvcrm`htco, vjrm`oicoc l`rifjv`sluhrc

Crlr`oluon, crghfdmc Rjrgch`stuon, Ljozcotr`tfjoco

MIH, HIH uoi Fosuhfo.

Aftmfhbc icr br ifcsc Br`uco crafttchtco prj-OY-

Ljozcotr`tfjoco ljootc i`s Ucmo-E`mrcs-Wfsflj br

ifc Cotwfdlhuon vjo Grustlrcgs rclh`ssfbfzfcrt

wcrico, sji`ss

72 % icr Br`uco aft sptcrcr Grustlrcgsgfhiuon fo

cfoc mmcrc uoi

70 % icr Br`uco jmoc sptcrc Grustlrcgsgfhiuon fo

cfoc ofcirfncrc Wfsfljl`tcnjrfc cfoncstubt wurico.

Ifc Wfsfljgcrcdmouon br ifc fo ifc AID-Ttuifc

cfoncsdmhjsscoco Br`uco crbjhntc uotcr Cfogczfcmuon

gcrcfts gcl`ootcr Wfsfljp`r`actcr**. Cfoc ifrcltc

Ljrrch`tfjo zwfsdmco prj-OY uoi GAF `hs cfoca

icr wfdmtfnco Wfsfljp`r`actcr crn`g sfdm `us ico

Ttuifcoi`tco ofdmt. Ifc Gcstfaauon ics prj-OY

hfcbcrt sjaft zustzhfdmc Fobjra`tfjoco zu gfsmcr

gcl`ootco Wfsfljb`ltjrco uoi gcrtrfbbt ifcsc

prj-OY5

wcofncr fst gcsscr

icuthfdm

vcrrfoncrtcs Wfsflj

2; 07; 0=; pajh/H prj-OY

vcrrfoncrtcs Wfsflj

crmmtcs Wfsflj

icuthfdm

crmmtcs Wfsflj

7/22/2019 Informationsbroschre fr rzte, Sphingotec

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Icr O`dmwcfs ics [cptfi-Gfja`rlcrs prj-OY nfgt `hhco Br`uco, uo`gmonfn vjo

`oicrco, `udm ncoctfsdm gcifontco B`ltjrco, @usluobt gcr cfo crmmtcs

Crlr`oluonsrfsflj. Aft icr Ljozcotr`tfjosacssuon vjo prj-OY wfri cfo jgecltfv

acssg`rcr Uust`oi fa Lrpcr crafttcht, icr zw`r zua Ucftpuolt icr Acssuon ojdm

lcfo ncsuoimcfthfdmcs [rjghca i`rstchhco auss, `gcr gcr cfoco honcrco Ucft-

r`ua ifc Cotwfdlhuon vjo Grustlrcgs gcnostfnco jicr `ozcfnco l`oo. I`s gcr

ifc prj-OY-Gcstfaauon i`rstchhg`rc Wfsflj fst `hsj lcfoc @uss`nc, jg cfoc Br`u

gcrcfts Grustlrcgs cotwfdlcht m`t jicr jg cfoc Br`u fa H`ubc fmrcs Hcgcos t`tsdm-

hfdm `o Grustlrcgs crlr`olco wfri. Cs vcrnhcfdmt ecijdm ifc Mubfnlcft `ubtrctco-

icr Grustlrcgscrlr`oluonco zwfsdmco Br`uco aft foftf`h ncrfonca Wfsflj (ncrfonc

prj-OY-Ljozcotr`tfjo) uoi Br`uco aft mjmca Wfsflj (mjmc prj-OY-Ljozcotr`tfjo).

Aftmfhbc ics spmfonjtcst prj-OY

hsst sfdm i`s lrpcrcfncoc Mjrajo

Ocurjtcosfo fa Ghut o`dmwcfsco.Ifc Ghutcoto`mac crbjhnt fo icr

@rztpr`xfs, ifc @o`hysc fo cfoca icr

Cfoscoich`gjrs, ifc aft spmfonjtcd

zus`aaco`rgcftco.

^chdmc Fobjra`tfjohfcbcrt icr prj-OY-^crt:

7/22/2019 Informationsbroschre fr rzte, Sphingotec

7/8

tfcrfsdmc Bcttc uoi Uudlcr, rcnuhfcrt wfri. Fosjbcro

stchht ifc nczfchtc Wciuzfcruon vjo tfcrfsdmco Bcttco fo

icr Cromruon cfoc mjbbouonsvjhhc [rvcotfjo i`r.

M`t ifc Gcstfaauon icr [rjocurjtcosfo-Ljozcotr`-

tfjo nczcfnt, i`ss cfo crmmtcs Grustlrcgsrfsflj

gcstcmt, capbfcmht sfdm fo ecica B`hh cfoc conc Rjr-

sjrnc, i` cfoc brmc Crlcoouon ifc Mcfhuonsdm`odco

fo icr Wcnch icuthfdm vcrgcsscrt.

Uwfsdmco icr Cromruon uoi `oicrco B`ltjrco ics

Hcgcosstfhs (z. G. lrpcrhfdmc @ltfvftt, Ncwfdmt) uoi

icr Cotwfdlhuon vjo Lrcgs, sj udm Grustlrcgs, gc-

stcmt cfo Uus`aacom`on. U`mhrcfdmc Ttuifco m`gco

i`s Ufch, zu cfocr wcftcrco Lhruon ics Uus`aaco-

m`ons zwfsdmco Cromruon uoi Lrcgs sjwfc oicrco

dmrjofsdmco Crlr`oluonco gcfzutr`nco uoi sjaft

nco`ucrc Capbcmhuonco br cfoc ncsuoimcftsbricro-

ic Cromruon zu cranhfdmco. Tpczfchh ifc Uubumrtfcrfsdmcr Bcttc stcmt mubfn fa Bjlus sjhdmcr Sotcr-

sudmuonco. Aft icr Gcstfaauon ics prj-OY wfri ifc

Anhfdmlcft crbboct, z. G. i`s `us ica Cromruons-

vcrm`htco rcsuhtfcrcoic Wfsflj jgecltfvfcrg`r zu a`-

dmco, i` ifc Brcfsctzuon vjo Ocurjtcosfo gcl`ootcr-

a`co gcr ifc O`mruons`ubo`mac, spczfchh iurdm

Ifc Gcstfaauon

ics prj-OY

jgecltfvfcrt

gccfobhussg`rc

Wfsfljb`ltjrco

7/22/2019 Informationsbroschre fr rzte, Sphingotec

8/8

^cftcrc Fobjra`tfjoco sfoi gcf spmfonjtcd crmhthfdm.

spmfonjtcd NagM

Ocucoijrbstr`c 03@, 02>20 Mcoofnsijrb

Ychcbjo ; 44 ;7/7 ;3 23-;

fobjKspmfonjtcd.ic, www.spmfonjtcd.ic

[rjocurjtcosfo (prj-OY) fst icr cfozfnc prifltfvc [h`sa`-Gfja`rlcr, icr cfoc

@uss`nc gcr ifc Wfsfljvcroicruon zur Cotwfdlhuon vjo Grustlrcgs hfcbcrt. Ifcs

fst fo acmrcrco lhfofsdmco Ttuifco st`tfstfsdm sfnofbfl`ot i`rnchcnt wjrico. Aft

ica spmfonjtcst prj-OY hsst sfdm ifc [rjocurjtcosfo-Ljozcotr`tfjo zuvcrhssfn

uoi sdmochh gcstfaaco.

Ifc prj-OY-Gcstfaauon crbjhnt fa H`gjr us cfocr fo icr [r`o`hytfl uoi @o`hytflcfob`dm uoi st`gfh zu m`oim`gcoico, odmtcro cotojaacoco CIY@-Ghutprjgc

aftmfhbc cfocs h`gjrnonfnco faauoif`nojstfsdmco Rcrb`mrcos (Faauoj`ss`y).

Bjtjs5074rb,spmfonjtcd|^C-IC-[OYZW;;7