358
Rozpoznejte3402900263135267368508642744872998105511271253
varovné16482900263111230310440551679805
příznaky25082900263140219276378506625741850
(tzv.3413290026369141243354400
„red3862290026393173295435
flags“).4352290026363126245384482576645702
Myslete3405600263199310407464590663789
i1184560026357
na12965600263128247
hereditární1598560026312925533445659665372584592410521109
transthyretinovou276256002637315226939749456769580688610081080113712651400151016451773
amyloidózu.3408200263119314425482617674813948104911771234
Pacienti3401180016990162233274358444496537
s9121180016966
hereditární10131180016987170226307399440492571627713755
transthyretinovou18031180016952107184270336389475554610691743784870959103811271213
amyloidózou30511180016979208288329418459551640712800886
vyžadují39721180016979159230310402488530571
včasnou45781180016979150229295381470556
a3401350016979
přesnou4541350016992148229295381469555
diagnózu10441350016992134213305392480552638
z17171350016971
důvodu18231350016992178258346438524
rychlé23821350016956135206292334417
progrese28341350016992148233326381462528611
onemocnění2,11,1234801350016989175259388476547633717803844432413600995377130183207260313
Klinická340153001236387111167191239287339
manifestace7071530012385137192216246302344377429478533
hereditární12681530012355111144198259283316368401459483
amyloidózy17791530012352137185209268292353413455503
se2310153001234398
může24361530012385252215300123562577153001234398
velice27031530012348104128152200256
lišit298615300123244891115148
a31621530012352
rozpoznání3242153001233390133194253296352403459483
příznaků3753153001236138141530012333384715300123246712217422240701530012356
může41531530012385423815300123564294153001234398
být44201530012361109143
zásadní4590153001234395137189250306330
pro340165001236194152
včasné52016500123485681650012348616165001235294150206
stanovení849165001234376128184243291347402427
diagnózy.213031650012361851371982543133564044231726166007232
Oboustranný6783590012383145204259302335368420476532580
syndrom777371001234391146208241298383
karpálního5823830012348100133194246270326350406465
tunelu1075383001233388144200224279
Neúmyslná7204930012378133189274322365389445496
ztráta608505001234376109161194246
hmotnosti8825050012355141200233289348391424448
Renální18363590012359115171222246302326
abnormality21903590012352113168228261348400424448481529
(např.17423710099216510715618983710099271917371009922
albuminurie196137100994261110155223242286331357377421
nebo240437100994589138186
mírná261237100996887114160201
azotemie)210438000994276123150194262281326347
Kardiovaskulární17414930012363115148207231291339391433482537561613646704728
příznaky24964930012362255749300123332591493001232467122174222270
(např.18475040099216510715620035040099272022504009922
srdeční2066504009935611091532219504009939225750400994464
hypertro2343504009944831321762032292563022645504009943
e,268850400994467
ventrikulární196851400993983128154181200238283302344370416435
bloky,242651400994868115154192207
kardiomyopatie176552300993980107154173221289327375424465492511556
nebo234352300994489138185
arytmie)255052300994268107133202221265286
Early31233590012359111144168217
(časná)33673590012326339335900123483441359001235295150202228
autonomní36973590012352107140200255315400455480
dysfunkce34273710012361109152181237293341389445
(např.31073830099216510715632623830099273281383009922
erektilní332538300994572115153180199218263282
dysfunkce362938300994987122145190234273311356
nebo400738300994489138185
posturální326539200994996130157201228270289333352
hypotenze)364039200994483132179206250295329373394
Opacity32654930012383145196245269302350
ve36434930012348104
sklivci3774493001234391115139187236260
Gastrointestinální44453590012378130172205239296320376409465507540564620672696751775
potíže52483590012361121154178220276
(např.44463710099216510715646013710099274621371009922
chronický466537100993984110156201220258297335
průjem,5023371009949755098371009944514237100991964132154
zácpa531837100993476114163205
nebo462738000994489138185
průjem/zácpa)4835380009948754910380009944495538000991964132161196237276324366387
Pozitivní46174930012357117159183217241289344368
rodinná5013493001233391152176231287339
anamnéza47595050012352108159245300356398450
Progresivní765255001216499161226266324371401457519549
symetrická13392550012147104196256293332362412472528
senzitivně18922550012147106168219249286316372434494
-24112550012132
motorická24682550012193156193256296325376435491
polyneuropatie29842550012166129159216277337398438499565621658687747
a37562550012157
≥138372550012167132
z39942550012151
následujících40702550012161118165195255321382412441492521572633
příznaků2:47282550012166106135186248305364424515325600713851902550012133
Dodatečné3405650012177141206263299359409471530
projevy:8955650012166106167197257313370403
Rychlá13235650012166123173235264321
progrese16695650012166105167232272330377437
onemocnění21315650012163124184276340390451511573602
nebo27585650012162122187251
nedostatečná30345650012161121187250297334391427487537598655
odpověď37145650012163129195258315374453
na41925650012162119
předchozí43365650012165105163228279340404455484
terapie148465650012137961361912572863465192565007138
Kombinace3402000012171131224290319381437488547
periferní9122000012166126165195231291330392422
polyneuropatie13592000012165129158215277336398437499564621657687746
s21302000012148
autonomní22022000012157118156219280344436498527
dysfunkcí27542000012166123170206268329389439469
nebo32482000012161121187250
srdečním3523200001214787151210261322352444
postižením39922000012166129176214243294354416445538
mohou45552000012192155217280342
indikovat4922200001212991157186246306363420456
hereditární3402120012162121161219284314351408448509539
transthyretinovou904212001213776132193240278339396436493530560622685742805866
amyloidózu2179521200121571492062362993283944575095702365212007138
Převzato34057800994638657800992741157800994483117159185233
z666578009934
Conceição722578009957104148187231251289331378
I,112257800992244
et118857800994571
al.12815780099426183
J1387578009739
Peripher144857800974792120139188232277305
Nerv1775578009763107135174
Syst.197157800974483117145168
2016;21(1):5-9.216057800994489133178200245289310354375397442469513535
V8486260013375
případě9496260013372115148220282355420
podezření13976260013372142214280336380443511543
na19686260013368130
hereditární21256260013368134177241313346387449493560593
transthyretinovou2746626001334084144212264305373435479542583616684753816885953
amyloidózu37266260013362164226259329361434503560627
se43816260013351117
doporučuje45266260013372141214283327394450517550616
genetické8486390013372138206271312345400466529
testování,14056390013341106158199268331393461493526
které19566390013365106171215279
pomůže22626390013372142244311367433
při27226390013373116149
včasné28986390013363118180232300366
diagnostice32916390013372105167240307377429470502558623
a39426390013362
rychlému40316390013344106162230262328430497
skríningu45566390013352117161193261293361434501
rodiny.50856390013343111183216283346375
©1509797008656
2020158479700863978117156
Alnylam176079700864864103137154190250
Pharmaceuticals,202979700864079116139200236270309348371388422458475505524
Inc.25727970086195892111
All27027970086496582
rights28047970086234082121145174
reserved.29987970086236191129153186225268288
TTR02-Global-00050330579700864080122161200223278294336379415432455494533572611650
CZ-ALN-032-07/202521038080012456102129185225287314359403448474519563593637682726771
datum2963808001244890118163231
přípravy:3216808001244932658080012428329480800124196896138176215237
07/20253553808001244589119163208252297
Medison3407760011180130185207245298348
Pharma7147760011151101148178256303
s.1042776001113863
r.1130776001113047
o.,1202776001115378103
Plynární1330776001115274117167214244295317
10/1617,16727760011150100133183233283333358
17020557760011150100151
0022317760011150100
Praha2356776001115181128178225
7,2606776001115075
e-mail:2706776001115080156203225246271
of300277600111548030837760011148
ce.czech@medisonpharma.com3131776001114393118162200250294344416492542597619657711761816866912942102110671092113611891266
Reference:340683001214789117158188230275308349373
1.340695001183653
Adams409695001184277110168195
D,620695001184154
Tournev68469500118336298120157190222
IL,92169500118174966
Taylor99869500118335990107143165
MS,1178695001185892109
et1302695001183355
al.137369500118324965
Efficacy14546950011832517089118151180213
and1683695001183270106
safety180569500118276079112134167
of1987695001183655
vutrisiran205869500118316890112128155171193225262
for233569500118205577
patients242769500118377091107140177198225
with266869500118536991128
hereditary281269500118377092123160176198230252287
transthyretin-mediated311469500118224476114140162200231252283305321359388446479516532565586617654
amyloidosis3784695001183391122139174191227263290306333
with413269500118547092129
polyneuropathy:427669500118377390122159192228250283320353374411442459
a47516950011833
randomized479969500118225491128163222238267297334
clinical51496950011829466299115144177194
trial.3407060011822446093110125
Amyloid.4817060011842100131149184200237253
2022;749706001183672108144161
doi:92670600118367288105
10.1080/13506129.2022.2091985;104670600118367289125161197233262297334370406442477514550566602638674710727764800836872907944980996
2.2058706001183653
Conceição2126706001183669106135167183212245280
I,2422706001181734
González-Duarte2471706001184277114143176193226254283325361394416441473
A,2959706001184259
Obici303470600118427995124140
L,3190706001183249
et3254706001183355
al.332570600118324965
J34067060011824
Peripher344670600118366688104141178211233
Nerv369470600118447698133
Syst.384270600118346591113129
2016;21(1):5-9;3986706001183672108144161197233258294319336372401437454
3.4456706001183653
Hanna4524706001184476114151184
M.4723706001185875
Curr481370600118367092113
Heart4942706001184376109131155
Fail51137060011831607693
Rep.5221706001183770107120
2014;11(1):50-57;340717001183672108144161197233258294319336372408437473509526
4.882717001183653
Mohty950717001185893130151185
D,1150717001184254
Damy1220717001184173132163
T,1393717001183343
Cosnay145171700118366996133166197
P,1663717001183739
et1718717001183254
al.178871700118335065
Arch186971700118426491128
Cardiovasc201271700118366789124140176208240266295
Dis.232371700118415784100
2013;106(10):528-540;2438717001183672108144161197233269294330366391408444480516545581617653670
5.3124717001183653
Shin319271700118347288125
SC,333271700118347087
Robinson-Papp3435717001183772109125163189225262291327357394431
J.3881717001182542
Mt3938717001185880
Sinai403371700118345088120137
J41857170011825
Med.4225717001185890127143
2012;79(6):733-748;4383717001183672108144161197233258294319336372408444473509545581599
6.4997717001183653
Adams5066717001184277110168195
D,5277717001184154
Coelho5346717001183669102119156192
T,340728001183342
Obici39872800118427995124140
L,554728001183249
et619728001183254
al.68972800118335065
Neurology.770728001184376112134168185220257289301
2015;85(8):675-682;1087728001183672108144161197233258294319336372408444473509545581598
7.1701728001183653
Damy1769728001184274133163
T,1943728001183342
Judge200172800118246198135167
DP,218472800118427880
Kristen228072800118375975102124156193
AV,248872800118427685
et2589728001183254
al.265972800118335065
J27407280011824
Cardiovasc278072800118366789124140175207239266295
Transl308572800118334880117144161
Res.326272800118377097112
2015;8(2):117-127;3390728001183672108144161197222258283300336372408437473509545562
8.3967728001183653
Hawkins4036728001184376128160176213240
PN,429272800118367996
Ando4404728001184279116152
Y,4566728001183946
Dispenzeri462772800118425885122155192221252274290
A,4933728001184259
et5007728001183355
al.507872800118324965
Ann5158728001184380117
Med.5290728001185891128143
2015;47(8):625-638;340739001183672108144161197233258294319336372408444473509545581598
9.954739001183653
Ando1022739001184279116152
Y,1184739001183946
Coelho1246739001183668101118156191
T,1447739001183343
Berk150573900118387293125
JL,164673900118245674
et1735739001183355
al.180573900118335066
Orphanet1886739001184365102139172209242264
J21657390011825
Rare220573900118377193124
Dis.234473900118425885100
2013;8:31;2460739001183672108144161197214250286303
10.277873900118367289
Dharmarajan2883739001184178111133191224246278295327365
K,3263739001183754
Maurer3333739001185790127149179201
MS.3550739001185791108
J36747390011824
Am37147390011842100
Geriatr383073900118417496112145166188
Soc.403373900118347099115
2012;60(4):765-774;.4163739001183672108144161197233258294319336372408444473509545581598615
11.479473900118367289
Adams4898739001184278111169196
D,5109739001184254
Suhr5179739001183470107129
OB,532473900118428095
Hund5435739001184379116153
E,340750001183351
et406750001183355
al.47775000118324965
Curr55875000118356991113
Opin68675000118438096133
Neurol.834750001184477113135168186201
2016;29(suppl1051750001183672108144161197233258284321358395412
1):S14-S26;147875000118366178112148184213247283319336
12.183075000118367289
Obici193475000118438096125141
L,2090750001183249
Kuks2155750001183772103130
JC,230175000118246077
Buades2394750001183774106143176203
J,2612750001182542
et2669750001183355
al.274075000118325065
Curr282175000118366991113
Opin295075000118427995132
Neurol.3098750001184376112134168185200
2016;29(suppl3314750001183672108144161197233258284321358395412
1):S27-S35374175000118366179113149185214248284320
Určeno34076100118436592124161196
pouze552761001183772109138169
pro73676100118375993
zdravotnické84476100118296486118149184206243259288320351
pracovníky121076100118375991120154186223240271303