757
KAZUISTIKY512322001004696138190209248289309356404
Naše414033001005186115151
první430733001003762100139154
zkušenost447733001003165104132169206245275302
s4795330010028
kaplacizumabem483933001003469108123158188204235273332367405441501
u5356330010037
TTP540933001003677119
|5584220010018
467564222001004184116
/35838040010732
Vnitř3660804001074592111139166
Lék.3845804001074184122140
2025;71(7):462-4674005804001074487128172185226267286329354368411457499525571617658
/47088040010732
VNITŘNÍ48058040010746106127172221280301
LÉKAŘSTVÍ5128804001074084130188235279325377399
www.casopisvnitrnilekarstvi.cz42580400107651332012142542953293774274464795215705896176436927117307748128548809159449871007102310631101
Willebrandovým42562001077493111128171220246287334383430469512585
faktorem.103562001072365102132177204246318334
Nedílnou1393620010758101150168188235282329
součástí174762001073381128168208240269287
léčby205862001071962102151189
pacientů227262001074990130147190238263310
s2607620010733
iTTP266562001071962108153
je284362001071962
další42576001074989107138156
jejich605760010719628097137184
sledování8137600107335194143191230271318336
a1173760010740
monitorace1237760010774122169187214260286327366408
ADAMTS131669760010753110159229270312349386
každé207976001073879116163206
32309760010744
měsíce2377760010774117149167206247
během264876001075094141184257
prvních42590001075076119167185224271
dvou72090001074989135183
let9269000107206390
k1039900010738
predikci110190001075076118167185222260278
relapsu14039000107266887126175208254
onemocnění168190001074795138211259299346390437454
(1–2,215990001072463103142156
6).23399000107446578
V2441900010745
případě25109000107507694143183232275
relapsu280990001072668425104001071959107140186
onemocnění631104001074795138211259299346390437454
či1105104001074057
refrakterity118210400107266891118159196226267293311339380
na1582104001074888
imunosupresi1689104001071991138185233265311360386428461478
rituximabem218710400107264572119157176248288337381454
lze266010400107195597
užít2776104001074884102129
některá425118001074789125154195219259
další698118001074887104134151
imunosupresiva863118001071889135180227258303350376417448465504543
jako142011800107195793139
cyklosporin1572118001074080117136182213260307333350396
A,1982118001075167
azathioprin,2063118001074075116141187204250298323340386400
mykofenolát24771180010772109146191213254300347365403428
mofetil,4251320010775123147191218237255270
cyklofosfamid,720132001074183122142191214262296320362436455504519
vinkristin1264132001074261109147175194226255274322
či1611132001074159
splenektomii.1695132001073483103147195239277308355429448466481
Toxicita2202132001074285123142182200228270
těchto2497132001072871112160186233
léků2755132001072064102150
však425146001074173114151
limituje597146001071937109127154201219262
jejich8791460010720638198138185
užití1084146001074884102129147
v12521460010741
praxi.1313146001075076117155174189
Novými15221460010758105145187261279
nadějnými1821146001074888137180197244282356373
léky221514600107196299141
k23771460010738
léčbě2436146001071962102150194
relapsu265114600107266987127175208254
či425160001074058
refrakterity50216000107266891118159196226267293311338380
jsou90116000107205199146
další1066160001074989107139156
generace1241160001074992139183208249289330
anti1590160001074188114132
CD2017411600010752108148190
protilátek1950160001075076122149167184224250292329
–22981600010745
ofatumumab2362160001074871113139186259305378418466
či2847160001074158
obinutuzumab,425174001074897115161208235282317365437477525539
ale978174001074159102
také1094174001072868105147
inhibitor1256174001071865112130178196223268294
proteazomu1565174001074975121147189228264310383429
–20091740010745
bortezomib2068174001075098124153195231277350367415
či2498174001074058
anti2570174001074188114131
CD3827161740010751108147190
monoklonální4251880010774122170218256276324372412430478495
protilátka945188001075076123150168186226252290332
daratumumab1301188001075090115156183230304351424464513
(10–13).1839188001072463102150188225245258
U21211880010757
naší2203188001074888121139
pacientky2366188001075091131149192240266304346
jsme2737188001071951124168
zvažovali304762001073679120156202241282300318
bortezomib338562001075098124154196232278351369418
v3823620010741
dávce388462001074989128167208
1,341136200107435397
mg/m24224620010773122154224444762006326
podávané449462001075098147187226266314357
2×487162001074497
týdně498962001072769117164207
po521762001074997
dobu533562001074996145193
dvou304776001074887132178
týdnů323976001072767114159204
s.345776001073348
c.351876001074054
při35867600107487391
refrakteritě36907600107266789115154190219259284301327367
TTP407176001074186130
na421476001074886
imunosupresi431476001071889134180226257302349374415447463
rituximabem.47917600107264369115152170240279326369440454
Naštěstí525976001075695126153193224251268
jsme304790001071951123167
jej32369000107196280
nemuseli333890001074892164211243286305322
použít,368290001075098146181200227242
neboť394690001074992141189216
naše418490001074988121164
pacientka437090001075090130148191238264301342
dosáhla473490001074997129170218236275
po503190001075098
25152900010743
měsících5218900010773117149166205223262309
remise,3047104001072769142159191235248
která3320104001073868110135176
trvá352110400107275397137
dodnes.3683104001074996146193236269285
Příčina399310400107457088127144192231
akutní4249104001074178125153200218
ataky4492104001074167107145187
TTP4703104001074288134
u48621040010747
naší4934104001074888120138
pacientky5096104001075091131148191239265302344
je5465104001071962
zatím3047118001073678104122195
idiopatická,326711800107196785133182223249267307344386401
původně3693118001074997137184234281324
zvažovaný4042118001073680120157203243284331369
vliv443511800107416078118
psychiatrické4578118001075083123162209227267293319338377414457
medikace50591180010774118167185222264304346
po5429118001075099
důkladném3047132001074995132152192240287330403
prostudování3468132001075076122155183230278326365406453470
SPC3956132001074285136
nebyl4110132001074891140178197
shledán.432513200107348199142191231278293
Sepsáním3187146001074286136170212260279352
kazuistiky3566146001073880118166185218247266304347
jsem393914600107205397171
chtěla4136146001074189116158178218
poděkovat4381146001074999149193231279319361388
všem4796146001074074119192
zdravotníkům,5015146001073685111153193241268317335375423497513
kteří3047160001073869111138157
se3226160001073478
na3326160001074889
péči3437160001075095136155
o36141600010748
pacientku3684160001075091132151194243269308356
podíleli,4062160001075099149168188232252270285
a43701600010741
chtěla4433160001074088115157176217
jsem467216000107205296170
vyzdvihnout4864160001074184123172213232280328377425453
důležitost5339160001074997115159304717400107365684131164193
multidisciplinárního32671740010774121140168187237255288329348397417435483524551600618668717
týmu,4010174001072871146194209
a42461740010740
to4313174001072875
nejen4414174001074893111155204
v46441740010741
léčbě4711174001071964105154199
TTP,4937174001074189135140
ale5104174001074060104
i52341740010719
dalších5279174001074990109142160200249
prolínajících3047188001075077124143162210250269288328347387435
se3501188001073478
diagnóz3599188001074967108157205254289
napříč3908188001074889138165184224
obory.4151188001074898148175218230
PROHLÁŠENÍ48521800974387144199239292335376430452
AUTORŮ:954218009749101143200245299316
Prohlášení128621700984573119166187229263305352373
o1674217009845
původnosti:173521700984693134179227274320354383404423
Publikace217421800954082127144161195233269308
byla24982180095447997133
zpracována264721800953378102139176218254291334371
s3034218009529
využitím307921800953775119152169195212277
uvedené337221800954380119163203246286
literatury3674218009517335997121158183226249288
a3978218009536
nebyla403021800954383127163181217
publikována426321800954487132150166200243279316359396
ani46752180095367996
zaslána478721800953270100117153197233
k5036218009534
recenznímu50862180095236299137180213257274340382
řízení4852300095244174112156172
do67523000954387
jiného7792300095173477117160203
média.1000230009565105150167203218
Střet12352290098426998141170
zájmů:142122900983679100169215234
Žádný.167323000954380125168202213
Financování:190422900984161108149195232277318359406427446
Ne.236723000955191104
Poděkování:248822900984590137180221266307348395416435
N/A.294123000955180122139
Registrace309722900984688135156190219248290326368
v3481229009841
databázích:353922900984687116158205247284306342389408
N/A.396423000955180122139
Projednání412022900984573119141183231278319365387
etickou45232290098417192128170214261
komisí:480022900984186155176210231250
N/A.506823000955180122139
LITERATURA426253001234978134189250318368438498567
1.42527000853648
Lánská49127000833165103129159193
M,70127000835468
Radocha78627000833568107145178216248
J.105227000832436
Kaplacizumab110627000833568107123155188203232270328360399
v1523270008331
léčbě15722700083154982121156
získané17462700083284470100133171206
trombotické19692700083224381139178217239254286316350
trombocytopenické23372700083214380138178216249283307344384419458473505535569
purpury.4252800083397798138176197231241
Acta6822800083407296129
Med.82628000835590129142
2022;4.98428000833468101136148183195
2.42529000853651
Lánská49029000833165103129159192
M.69529000835468
Léčba7762900083316598137170
získané959290008328436999132170205
trombotické11772900083224380138178216238253285315349
trombocytopenické15392900083224380138177216249282307344383419457472503533567
purpury21192900083397798138176197231
–2363290008335
kazuistika.24112900083296392130145171194210239273286
Farmakoterapeutické271029000833262831411734253000083306789123144177216252289312327359389423
Revue.86430000833570102141176188
2022;7(6).106730000833468101136148181198231249260
3.42531000853651
Scully49231000833265103118133165
M,67331000835468
Cataland7573100083407294127142175213252
SR,10253100083326781
Peyvandi112231000833569100135168206245260
F,139831000833339
et145331000833456
al.15253100083324760
Caplacizumab160131000834073112128160192207236275333365404
Treatment20213100083335084116138196231269290
for23273100083195678
Acquired242131000834172110148163184218258
Thrombotic269531000833272931301884253200083397799115147
Thrombocytopenic5873200083337293130188228266299333357394434470508523555
Purpura.11573200083357293133171193226239
N1411320008345
Engl147132000833371110125
J1611320008324
Med.165132000835489129142
2019;380(4):335346.180832000833468100130143178213248268300318330365398431464499535549
4.42533000853651
Peyvandi49133000833569100135168206245260
F,76533000833339
Scully81833000833265102118133164
M,99733000835468
Kremer10793300083355791149184205
Hovinga129833000834583115131168207240
JA,15523300083246478
et164433000833457
al.17153300083314760
Caplacizumab178933000834073112128160192207236275332365404
for22073300083185678
Acquired229933000834172110148163184218257
Thrombotic25713300083327193130188227266288303335
Thrombocytopenic4253400083337293130188228266299333357394434470508523555
Purpura.9953400083357293133171193226239
N1249340008345
Engl130934000833371110125
J1449340008324
Med.148934000835489129142
2016;374(6):511522.164634000833469100131144179211241261294312324359390418448481516528
5.42535000853651
Elverdi4973500083334881116137176191
T,70935000833342
Eskazan7723500083336191125155189227
AE.10203500083417588
Caplacizumab112935000834074113129162195210239278337369409
as155935000833259
an163935000833270
emerging173035000833493128150188204242282
treatment20323500083224479111133192227266288
option234035000833878100116154193
for25543500083195779
acquired265335000833265103141157178213253
thrombotic4253600083215980117175213252273288320
thrombocytopenic7583600083215980117174213252284317341378417452490505536
purpura.13073600083387697136174195228241
Drug156036000834567105143
Des171636000834580106
Devel183536000834580112146161
Ther200936000833271105126
[Internet].2148360008318347293126147185220242259271
2019;13:12511258.24313600083346899129142171201212242271306336364393428461475
Available42537000834171104120135167206222257
from:6973700083194178136148
doi:10.2147/DDDT.S134470.8613700083387691104133165177213245276308328375421467499508542573603638675707740753
6.42538000853652
Zheng49538000833876111149188
XL,7013800083387083
Vesely8033800083366895130146178
SK,9993800083326782
Cataland10993800083407394127143175213252
SR,13693800083336882
Coppo146938000834077117156195
P,168338000833539
et174138000833456
al.18153800083324760
ISTH18943800083154781127
guidelines20393800083387691130165180195233268295
for23533800083185677
the24493800083215994
diagnosis25623800083385385124162200227242268
of284838000833857
thrombotic4253900083226081118176216254276292324
thrombocytopenic7643900083226081118176216254287321345382422458496511543
purpura.13223900083397798138176197230243
J1581390008324
Thromb16203900083337293130189228
Haemost.186339000834477112169208235258271
2020;18(10):24862495.214939000833469101136148179210229260292310322357389425460494526559593605
7.42540000853645
Cablivi.48540000834073112128143175191204
SPC.70440000833368108122
8.42541000853652
Knoebl49441000833574112148187203
P,71441000833540
Cataland7714100083407395127143175213252
S,104041000833345
Peyvandi110241000833569100135168206245261
F,138041000833238
et143541000833456
al.15084100083324760
Efficacy1585410008333516987119152184217
and181941000833270109
safety19454100083265978113135169
of213141000833756
openlabel220541000833776112150165198237272288
caplacizumab251041000833164103119151184199228266324356395
in42542000831552
patients4904200083397294109143181203230
with7334200083516689127
exacerbations8734200083346598130163185224257279294332370396
of128242000833857
acquired135242000833164101139154175209248
thrombotic16134200083226081118176215254276291323
thrombocytopenic19494200083226081118176215254286320345381421456494509541
purpura25034200083397798138176197230
in274642000831452
the28114200083226095
HERCULES42543000834477113153197229263296
study.7374300083264987126158168
J920430008324
Thromb9604300083327293130188227
Haemost.120343000834476111169207234257271
2020;18(2):479484.148943000833468101135148178209228262280292326358392427463500512
9.42544000853649
Peyvandi48844000833569100135168206245260
F,76244000833238
Scully81444000833264102118133164
M,99244000835468
Kremer10734400083355892150185206
Hovinga129344000834482114130168207239
JA,15464400083246478
et163744000833457
al.17074400083324760
Caplacizumab178144000834073112128160192207236274332365404
reduces21984400083215595132164198225
the24374400083215994
frequency25454400083184074114152186225257290
of284944000833756
major42545000835789104141163
thromboembolic6004500083225980117175213252287344383421437452483
events,10964500083336599137158185198
exacerbations13074500083336497128162183222254276291328366392
and171245000833169107
death183245000833872104125163
in200745000831552
patients20724500083387192107141179200227
with23124500083506588125
acquired245045000833163100138152173207246
thrombotic270845000832260801171754254600083397799115147
thrombocytopenic5874600083226081118176216254287321345382422458496511543
purpura.11454600083397798138176197230243
J1404460008324
Thromb14434600083337293130189228
Haemost.168646000834477112169208235258271
2017;15(7):14481452.197246000833469100130140170200217252271283313344381417449480513546559
10.30472700085367085
Wali314827000835789104119
JA,32832700083246478
Quigley337727000834785100139155189221
BM,361427000833590104
Schaefer373327000833365103136170190224245
B.399427000833549
Successful405927000833269102132166193220239278293
Use436827000834470105
of448927000833756
Bortezomib45612700083357496120154183221279294333
in491027000831553
an497827000833270
Adolescent506427000834179118134168195227261299321
with54012700083506688126
Refractory30472800083357089112145177201238259293
TTP.335628000833269106111
Case34832800083407299134
Rep363228000833671110
Hematol.375728000834479137170191228244257
2023;2023:8173903.402928000833569101136148183217250284296331362392426459495528541
11.30472900085366678
Lee314629000833166101
CJN,326729000834067112125
Yates34132900083356586120147
S,358029000833346
Rambally364629000833568126165198214229261
S,392729000833345
Sarode39932900083326586124163198
R,421129000833650
et428129000833457
al.43582900083324760
Bortezomib44382900083367496121154184221280295334
in479229000831553
relapsed/refractory48652900083215571103142169204243270289323342365398430454491513547
immune543229000831572304730000835795133168
thrombotic32333000083226081118177216255277292324
thrombocytopenic35753000083226081118177216255287321346383422458496511543
purpura:41363000083397798138176197230243
a4397300008332
singlecentre44473000083264179118133168201234273294316350
retrospective4815300008321557798136162201237270294309341376
cohort520930000833168107145167191
and541930000833169108
systematic30473100083265986109143200233255270302
literature3364310008315305286107140162200221255
review.36343100083215587103138189200
Br384931000833556
J3920310008324
Haematol396031000834476111169201223260276
[Internet].4251310008318347294127149187222245262274
2023:???454031000833468101136147172199225
Available478031000834172105120135167207223257
from:50523100083194178136149
https://doi.52163100083375983122149162188209245283298311
org/10.1111/bjh.19035.30473200083375997123156188200231259286314336376391429443473503539572605617
12.30473300085356681
Yin31413300083355087
J,324033000832435
Tian32873300083324879116
H,341533000834456
Kong348333000833471108146
D,364133000834455
et370833000833355
al.37753300083314658
Bortezomib,38453300083357293117149178214271286324335
a4192330008331
promising42353300083385995152167192206243281
alternative45283300083314668100121159190211225257290
for48303300083185576
patients49183300083387091106139176197224
with51543300083506486123
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thrombotic34443400083215980118176215254276291323
thrombocytopenic37813400083215980118176215254287320345382422457495510542
purpura43373400083387698137176197230
after45803400083325176110131
rituximab4724340008321365997128144201234273
treatment.50103400083224377109131189224262284297
Br532134000833556
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Haematol542834000834477304735000833492124146183199
[Internet].3261350008319347294128149188223245262274
2022.355135000833468101135148
Available371435000834172105120135167206222257
from:39863500083194178136149
https://doi.org/10.1111/bjh.18430.41503500083385983123150162189209245283299312350371409436469500513543571599626648688704742755786817853886920933
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Weisinger314836000835791106132147185224258280
J,344536000832537
Fadlallah349936000833362101117149164179211249
J,376636000832436
Joly38203600083246378110
B,394836000833548
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purpura36073700083387698137176197230
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rituximab4001370008321365997128144201234273
failure4291370008318516782119140175
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intolerance.45583700083155374111127162183216254286320332
Br490737000833557
J4981370008324
Haematol.502237000834476111169201223260276289
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press.30473800083396094121148161
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X,330739000833852
AlHousni337339000834157101140178204242257
Z,364539000833751
Cataland37103900083407395128143175213252
SR,39773900083336882
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al.41453900083314760
2025422039000833468101136
focused43703900083195689127153188228
update461239000833877116149170204
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ISTH51623900083154781127
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management325540000835790128160199234292327365386
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purpura.46264000083397798138176197230244
ISTH48854000083154781127
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press.34794100083396195121148162
15.30474200085366883
Cuker314342000834076107141162
A,331842000834155
Cataland33864200083407395127143175213252
SR,36514200083336882
Coppo374642000834077116156195
P,395442000833539
et400742000833356
al.40764200083324760
Redefining414942000833570110145162179217232270309
outcomes44714200083377698129166224259286
in477042000831553
immune483642000831472130167205240
TTP:509042000833269106119
an522242000833169
international5305420008314527410712916719930474300083223775113146161
working322343000835189110141157195234
group34724300083386097135174
consensus366143000833269107133168206233270296
report.39734300083205494132154179192
Blood.41804300083365189128168181
2021;137(14):18551861.437643000833468101133141171201233250281312330342371402436469500531566597606
KNIŽNÍ50855100286124271329430579637
NOVINKA120155100286148316448503653777930
Vděčnost14596100013870129188237300362412455
Srdce1459624001385390145190240
modlitby17306240013884140197221245284340393
David14596390012560107155178229
Steindl-Rast1716639001254881128151201253275311368415455491
Slavný1459654001074261101141190228
rakousko-americký1705654001072768106154202235272320349390463507534553593631674
benediktinský2397654001075095143187237256293324343391424462504
mnich29196540010774122140181229
David3166654001075898138158207
Steindl-Rast3390654001074268111130178227246274321362395424
ve3832654001074184
své3934654001073375118
kontemplativní4070654001073886134161204277327346387414433474523541
knize4629654001073888107143186
zkoumá4833654001073675122170244284
vztah5135654001074180111152200
mezi53536540010774118155174
modlitbou14596670010773120170188206233282330377
a18526670010741
vděčností,1908667001074188131171219266299327345360
která2284667001073868109135176
plyne2476667001074968107156199
z26916670010736
lidské274266700107193785118154197
schopnosti2954667001073473121168217265312345374392
milovat.3362667001077291109156195236263278
Objevuje36566670010760109128171211258277320
krásu,3992667001073765105138184199
která4207667001073868109135176
se4399667001073376
ukrývá4491667001074784112154197238
v47456670010740
každodenním4801667001073879115163210259302350397415487
životě.530466700107365494141168209223
Věří,1459680001074688114133148
že1629680001073679
pokud1730680001074999137185235
oslavujeme1986680001074882101141181229248292366410
věci2418680001074084125143
proto,2583680001075077124151198212
že2817680001073679
prostě2918680001074976123157186228
jsou,3168680001071952100149164
žijeme335468000107365574118191235
život361168000107365697144171
je3804680001071963
v38896800010740
jeho3951680001071963112160
plnosti.4133680001074969117165199228247262
Překvapení4417680001074571114152196237287332380398
je4837680001071963
podle4922680001074999149168212
něj5155680001074892111
cestou5288680001074082116145192240
k14596930010738
tomu,1514693001072874149196212
abychom1742693001074191130170218266341
si2099693001073452
uvědomovali2168693001074889132182230304353393435454472
hodnotu2657693001074897147195243271319
života.299369300107365596143171212228
Strach323869300107426895137177225
znamená3480693001073785126200244292333
podmínku3830693001074998149222241289327375
odvahy.4222693001074898139180229267279
Klíčem451869300107456684124166240
k47756930010738
životu483069300107365596143171219
je5066693001071963
rovnováha5146693001072673114163211251293341382
mezi14597060010774118155174
dáváním1650706001074989129170218237310
a19777060010740
přijímáním,203470600107507695114132206246294313386402
přičemž245270600107507796136178251288
dávat2757706001074989129170197
můžeme29717060010774121157200274318
tím,3305706001072847120136
že3457706001073779
se3553706001073478
něčeho3647706001074992133175224272
vzdáme,3936706001074079127168241285300
že4252706001073779
vzdáme4348706001074179127168241286
díky4650706001074968107150
nebo4817706001074892141191
že5024706001073679
odpustíme.5120706001074898147195228257276349393408
Radost1459719001074587136184218247
není1724719001074892141159
výsledkem1901719001074184118137181231269312385
toho,2304719001072875123172187
že2509719001073679
se2606719001073377
nám2701719001074989163
daří,2882719001074990116135150
nýbrž3050719001074987137164203
naší3271719001074889122140
vděčnosti3429719001074189133174222271304333352
–37997190010745
pokud3862719001075099137186235
jsme4115719001071952125170
vděční4303719001074089133174222240
za4561719001073679
to,465871900107277489
co4765719001074086
máme,48697190010774115188233247
nacházíme5134719001074889130178218256275348393
hlubokou1459732001074867115165214253300349
a18347320010741
trvalou190173200107285599141160208257
radost.2184732001072769118167200230246
I24567320010720
smysl25027320010734107146180199
života272773200107375697145172214
podle2967732001075099150169213
autora3206732001074189117164191233
objevujeme3465732001074899118162203251270314388433
skrze3924732001073472100138182
vděčnost.4132732001074190134175223271305334351
Autor,45097320010753100128175202214
který4749732001073969112139182
věří,4957732001074184110129145
že5128732001073780
součástí5234732001073482131171213246275294
kontemplace1459745001073885134161203277327346387427470
je1947745001071963
aktivní2027745001074179110129170219238
účast2282745001074888130163192
na2492745001074889
životě259974500107365596144171213
v28307450010741
lásce288974500107195993133176
a30827450010741
vděčnosti,3141745001074189133174222271304333352367
ve3526745001074184
své3628745001073375118
knize3764745001073888107143186
propojuje3968745001075077124174223242289308352
duchovní4338745001074996137185233274323341
myšlenky46977450010774112146165209257295338
s50537450010733
každodenní5104745001073881118166214264308356404423
zkušeností,1459758001073675123156200248296330359378393
kterou1872758001073869111138185233
může21257580010773121157200
získat234575800107365588126168195
každý2560758001073880117166206
z27857580010737
nás.2841758001074989123139
1761459777001074481121
str.,160077700107336289101117
brož.1736777001075077124160177
Cena:1459790001075194142183198
3691676790001074488131
Kč1822790001074584