466
PŘEHLEDOVÉ42522001004388130181216258306358404446
ČLÁNKY88722001003973122176223272
Moderní425330010061100139174198237252
léčba6933300100155081120155
hereditárního8643300100377297132170186212249273311327366405
angioedému128533001003471110125164199238274334371
s1672330010029
deficitem17173300100377291109139154181216276
C1200933001003964
inhibitoru:20893300100145290105143158185224248287302
od240733001003876
kontroly250033001003371109135160199215251
atak27673300100346197131
k2914330010034
úplné29643300100377590128163
kontrole314333001003472110136161200216251
onemocnění341033001003876111171210241279315353368
39619122001003476119
|350220010018
VNITŘNÍ4268040010746106127172220280301
LÉKAŘSTVÍ748804001074185130188236280325378400
/11948040010731
Vnitř1270804001074693112140166
Lék.1456804001074083122140
2026;72(6):396-4001615804001074487129171186227271292334356371415457502532578623669
/23298040010732
www.casopisvnitrnilekarstvi.cz442680400107651332012142542953293774274464795215705896176436927117307748128548809159449871007102310631101
https://doi.org/10.36290/vnl.2026.07342544001003865941331631802022236484400100418310274944001001554791171398904400100287389124167204247293316355396414432470516552594611655686723
Moderní4259800275198324452571664791857
léčba1359980027560179281409531
hereditárního196798002751222413344525816477378599521079114512771403
angioedému3447980027511624437243856468281192911171245
s4251240027599
deficitem57112400275123242333401502568658775962
C1161012400275112245
inhibitoru:1932124002756018831638251157666679188310101079
od308812400275120249
kontroly341412400275121244371461554681746875
atak42515000275116206328455
k95715000275121
úplné112615000275122250317444563
kontrole176615000275121244371461555681747865
onemocnění270815000275121248367555680781910102811571223
Roman4251890013765129224282347
Hakl,7941890013776134194226255
Jan10711890013745103168
Baroš,12611890013766125167231278308
Jiří1591189001374575117147
Litzman1760189001375786127180275334398
Ústav4252040013371113150203254
klinické702204001335279103164189237288341
imunologie10672040013325116176237297323383445470525
a16152040013353
alergologie,1691204001335379134169230290316376438463518540
FN22542040013353123
u24012040013360
sv.2485204001334397114
Anny26222040013368128188238
v28832040013353
Brně,2959204001336096157212233
Lékařská32162040013352106157211246290341395
fakulta3634204001333286136197222258311
Masarykovy39692040013388141184237272327379438489545
univerzity45372040013360121146199252288336362398452
v50132040013352
Brně5089204001335995156211
Hereditární4252550012165117151202260284318368402458482
angioedém9342550012149105162186242294352404488
s14492550012140
deficitem15162550012156107138165211235269319404
C119472550012158107
inhibitoru2081255001212379136160217242276331365421
(HAE-C1-INH)2529255001212994156205237300349377403470537565
je3121255001212476
vzácné,3224255001214894145190247298319
potenciálně35702550012157114147198254300324373398454505
život4102255001214367116171205
ohrožující43342550012155112145201244300325349394418
onemocnění47792550012155112163248304351407458515539
charakterizované53452550012145101150425269001213382130167217251276319375423473529580
rekurentními1034269001213384132188222273329363419443527552
epizodami16142690012150109133177233291340424448
otoků2091269001215589144192248
zprostředkovaných23682690012143101135190231266299350408456510559608664711756812
bradykininem.3209269001215690140198246295321377401457508593615
V38532690012156
posledních39382690012157113154178229288344368414470
letech4436269001212475110160207263
došlo47282690012156112152177233
k49902690012147
zásadnímu5065269001214394134184242298322407462
posunu4252830012157114154209266321
v7712830012148
diagnostice844283001215680129186243299339374398444494
i13622830012124
terapii1410283001213384118167225249274
HAE-C1-INH,17082830012166127177209271320348375442508531
zejména2264283001214293118202254311360
díky2648283001215781131182
rozvoji2855283001213288131183238262287
cílené316628300121456995147203255
biologické3445283001215782138163219277301347394444
a39142830012148
molekulární39872830012183139164216264320345394427484508
léčby.4520283001212475121179226244
Nová47882830012166123171220
doporučení50332830012156113171228262318364414470494
WAO4252970012185143211
z6602970012142
roku726297001213389137193
20259432970012152103152204
zdůrazňují11712970012143101156190239284340396420444
individualizovaný1639297001212480138162211236294349398423447491546594644700747
přístup,2410297001215690115155190245303324
dostupnost27582970012156112152187243301358414454489
účinné32712970012155101125181237289
akutní3584297001214895152186242266
léčby3874297001212475122179227
pro4125297001215791146
všechny4295297001214889140187243299345
pacienty46642970012157107153177228285318369
a50572970012148
léčebnou5129297001212475122171229286342399
strategii425311001214074108158191241299323347
zaměřenou796311001214293177229262312369425481
na13013110012155104
dosažení14283110012157113153203247298355379
úplné18303110012155113138194246
kontroly20993110012147101158191225281306354
onemocnění.24763110012155112163248304351407458515539561
Tento425325001215095151185240
přehledový687325001215791141198223274332388437488
článek1198325001214468117174225273
shrnuje1494325001213996129186242266318
současné1834325001214096152198248288344395
možnosti22523250012183139182239295335370394
léčby2668325001212475122179227
HAE-C1-INH,29173250012165127177209271320348375442508531
včetně3470325001214893143177234285
terapie3777325001213384118167225249301
akutních4100325001214896152187243267313369
atak,4491325001214882132179203
krátkodobé4717325001214782131165212267325382440492
a52313250012148
dlouhodobé53023250012156801371934253390012155112170226284336
profylaxe.784339001215791146177228254303351401422
Diskutovány1229339001216791131179235270325373422478525
jsou1777339001212464121177
jak1977339001212473121
zavedené2121339001214394141191249301357409
terapeutické2553339001213383117167224276333367391437484534
přístupy3110339001215791115155190246304351
(koncentráty3484339001212876131187233283340373407456490541
C140483390012158107
inhibitoru,4178339001212379136160217242276331365422443
antagonista46443390012149105138188245301358382422457507
bradykininového5174339001215791140198246295321425353001215681137194243294351408
receptoru864353001213385132183241276332366423
a13183530012149
inhibitory1398353001212380137162220245280336370422
kalikreinu),1851353001214798123147196231283308365421448468
tak2350353001213384132
nové25133530012156113162212
terapeutické2756353001213484119169227280337372396443491541
možnosti,33283530012184141185242299340375400422
včetně3780353001214994145180237289
perorálních41003530012157110144201235285310367391438494
inhibitorů4625353001212481138162221246280336371428
kalikreinu,5083353001214899124148196232283308365422444
monoklonálních4253670012183140196252301327383439488513569593639695
protilátek1143367001215791146180204228277311361409
a15763670012148
antisense16473670012148104138162202253310350402
oligonukleotidů.2072367001215580104160217273329377403454511545569626682703
Klíčová425384001235885111155211261312
slova:758384001234268124174225248
hereditární10273840012156106139189246269302351383439462
angioedém,15113840012148103159182237288346396479500
C120333840012158106
inhibitor,2160384001212378134157213237270324357373
bradykinin,2554384001215790139195243291315371394449470
kalikrein,3045384001214796119142189223273296352373
profylaxe,3439384001215790144173224248296344393412
biologická3873384001215680135159214271294339385435
léčba.4329384001212374119176225246
Modern42540900150108175244307356424
treatment887409001504796160224272373436505552
of14774090015066114
hereditary16304090015066130179242311346393458508578
angioedema22474090015063131200235302364433497597662
due29484090015067135198
to31854090015046112
C133364090015061132
inhibitor35064090015033102170204273307355421470
deficiency:40154090015067130178214267302365433487557593
from4647409001504795163263
attack49484090015064111160225278346
control4254230015052119187234284351385
to8494230015046113
complete10004230015052119219288322386434496
disease15354230015067101157220284340403
control19764230015053119187234284351386
Hereditary4254430012164114146195251273306353385434
angioedema8764430012148102157179233283339389471518
due14114430012155109158
to1586443001213386
C116894430012157104
inhibitor1810443001212377131154209232264317349
deficiency21764430012156105134159203225275329373422
(HAE-C1-INH)2615443001212890150198228288335361386451515542
is3174443001212361
a32524430012147
rare,3316443001213380112160180
potentially35134430012156111143191245277299346369391437
life-threatening396744300121244675124156189243274323370402450504527581636
disorder4620443001215578116170202257307339
characterized4976443001214498145176224268302350382405447496552
by4254570012157103
recurrent547457001213383128183216249299354387
episodes9524570012150107131170225283333372
of1343457001215584
bradykinin-mediated1446457001215790138195243290315370393449481565616673696744777826883
angioedema.23484570012148103160183238289346396480528549
In2916457001212479
recent3014457001213382128177233265
years,3298457001214796144177217238
major35554570012184132155210243
advances38174570012148105153202257302351390
have42264570012156104150199
been44444570012157108159215
achieved4677457001214993149172222269319376
in5072457001212378
both51694570012157113146201
the5388457001213489139
diagnosis425471001215679127183238293332355394
and8374710012148104160
treatment1015471001213366115163196279329385417
of1450471001215584
HAE-C1-INH,15524710012165125174205266314341367433498519
particularly20894710012157106138174197242296320368400424471
due25784710012156111161
to2757471001213387
the2862471001213388138
development30184710012157107154203227282339423473529561
of3597471001215584
targeted3699471001213382114170220253303360
biological4077471001215780135159214271294339387410
and45054710012149104160
molecular46834710012184139163213258313336384417
therapies.5118471001213388138171219276299350389410
The4254850012150106157
updated6064850012155111169217249299356
20259864850012151101150201
WAO12114850012185142209
guidelines14444850012155110133190240264287343393432
emphasize19004850012151134191247295334357400450
an23744850012148103
individualized2501485001212379135158207231287342390413436479529586
approach,31114850012149105162195250299344399419
the3554485001213489139
availability3717485001214895143166190238294318341364397447
of4188485001215584
effective4296485001215080113163209244267315364
on-demand46844850012155111143202252336384439496
therapy5204485001213388139172220277323
for425499001212984117
all56349900121497295
patients,6794990012157106138161212267300340361
and10624990012148103160
a12434990012148
treatment1312499001213366116164196280330386418
strategy1751499001214074106155187237293341
aimed2113499001214972155205263
at2397499001214880
achieving2499499001214893148171221269293348404
complete2924499001214598182239263313347396
disease3341499001215780119169217256307
control.3669499001214599154187219274298319
This4255130012150106129167
review610513001213382129153203276
article904513001214880116138183206256
summarizes1178513001213993176259307339362405454494
current1689513001214599132165214269302
therapeutic2008513001213388138171219275326381414437481
options25075130012155112144167222277316
for2841513001212984116
HAE-C1-INH,29755130012165125174204266313340366431496518
including3510513001212478123146201257280335390
on-demand39185130012155110142201251334382437493
treatment4429513001213365115163195278328383415
of4862513001215584
acute4963513001214893147180229
attacks,5210513001214880115164209255297318
short-term425527001214095149182218246279328361444
prophylaxis,887527001215789144201257302326374421444483504
and14095270012148103160
long-term1586527001212479134190222255304337420
prophylaxis.2024527001215790144201257302326374421445484505
Both25475270012154109142197
established2761527001215090123172229252275314369419477
therapeutic3255527001213388139171220276327382415438482
approaches37555270012148105162194249297342397447486
(C14259527001212884132
inhibitor4409527001212378133156212236269323355
concentrates,4782527001214598154199248303335368416448497537558
bradykinin53575270012157901384255410012156104152176232255310
B27555410012154104
receptor879541001213383128177234267322354
antagonists,12545410012148103136185240296351374413447488509
and17835410012149104161
kallikrein1964541001214796119143166212246296320375
inhibitors)2359541001212479134157214238271325358398423
and28025410012148104160
novel29835410012155110157207231
treatment3234541001213366115163196279330385418
strategies,3672541001214074106155187236293316367406427
including4120541001212378123146201258281336392
oral4533541001215487136159
kallikrein4713541001214796119142165212246296319375
inhibitors,5108541001212379134157214237270324357398419
monoclonal4255550012183139194249295318373429477500
antibodies,9475550012148103135158215271328351401441462
and14305550012148104160
antisense16115550012149104136159198249304343394
oligonucleotides,2026555001215579102158213269323368391442498530553610660700721
are2768555001214881131
discussed.2920555001215679118163218258297347404425
Key4255720012358108158
words:6045720012374130166224266290
hereditary9155720012155106139189246269302351383434
angioedema,13705720012148103160183238289346396480528549
C119405720012158106
inhibitor,2067572001212479134157214238271325358374
bradykinin,2462572001215790138195243290315370393448469
kallikrein,2952572001214797120143166213247297320375396
prophylaxis,3369572001215790145202258303328376423447486507
biological3897572001215781136160215272295340388412
therapy.4330572001213388139171220277323339
Úvod4256190013874137198262
Hereditární5656360010758103131175227246275318345395415
angioedém1007636001074291142161211257309354429
s14646360010734
deficitem1526636001074995119140182201230274349
C11903636001075294
inhibitoru2024636001071969118137188208237285313363
(HAE-C1-INH)2414636001072583136179207263304328350408467491
představuje425650001075076119169202231273312361380424
vzácné867650001074180122163211255
geneticky1141650001074993141185213232272310353
podmíněné1513650001075099149222241289333381425
onemocnění1957650001074897141214263304352396444463
charakterizované2439650001074088128155196234265308334354390437425664001074182130174
rekurentními619664001072770108156182225274300349367441459
epizodami1098664001074493113149196246287361379
nebolestivých,1497664001074892142191211255289318336377421460508524
nesvědivých2041664001074892126168211260279320363403451
otoků2512664001074875122160208
kůže2740664001073886123166
a4256780010741
sliznic.48967800107335270107156174215231
V7436780010746
patogenezi812678001074991118165215259307351388408
onemocnění1242678001074897141215263304353397445463
hraje1728678001074875116135179
dominantní1930678001074997171190238278326353402420
roli237367800107277493112
bradykinin.2508678001074976118167208246267315333381397
Onemocnění4256920010760108152225272313361404451469
se911692001073376
projevuje1004692001074975122140184224272290333
rekurentními1353692001072769107154180223270296344362435453
otoky,1822692001074875121159201212
které2050692001073868110136179
mohou22456920010773121169216264
postihovat2525692001074998131159178225273313354380
kůži,3047622001073887124144159
gastrointestinální3233622001074990124153181228248296323366400430449497539558606625
trakt388562200107285597136167
i40796220010718
horní4124622001074998125175193
cesty4344622001074184118147190
dýchací.4561622001074990130179220261280296
Laryngeální4884622001074083110154203253298338358406425
ataka5336622001074168110149191
představuje3047636001075179123175209239282324374394439
nejzávažnější3514636001074995115153197238281319370415435469488
projev4030636001075179128148193235
onemocnění4293636001074999145221271314364410459479
a47996360010742
může48696360010776125163207
vést5104636001074186121151
k52836360010739
život5350636001073757100149177
ohrožující3047650001074897123170206254273291331350
obstrukci3417650001074898131160187235273313331
dýchacích3768650001074989129177217258277317365
cest4152650001074183117146
(1).43176500010724648295
Prevalence3187664001074569111151192210253300339381
HAE-C1-INH35836640010756107148174227266289309365420
se4019664001073376
odhaduje4111664001074796143182231277295338
přibližně446566400107497593141160178213261304
na4785664001074787
148876640010744
:49466640010716
504977664001074486
000–1005078664001074488133180218256301
0005395664001074387132
obyvatel.3047678001074898137181222250292311327
Onemocnění33906780010761109154227275316365408456475
je3881678001072063
spojeno3961678001073382131150194242290
s42686780010734
významnou4318678001074184123171212285333381429
morbiditou,47646780010774122149199218267286313360408423
sníženou5204678001073481100136179227275323
kvalitou3047692001073882123142161189235284
života335269200107365697144171213
a35866920010741
celoživotním3649692001074082101150185204245293320369387461
rizikem413169200107274683102140183256
fatálního440969200107236693134153201219269318
průběhu4748692001075077125174219267315
při508469200107507796
postižení5201692001075099133162180217260308326
MUDr.425740009562112164189200
Roman64374000954083150186229
Hakl,89074000955087121140153
Ph.D.10617400095408297150163
Ústav42575200955079105142178
klinické6207520095345370112129165199238
imunologie87675200951682124168211229272317334373
a1266752009537
alergologie,13207520095375493117160204221265310326366379
FN171675200953788
u1821752009543
sv.18817520095306879
Anny197775200954790133168
v2162752009536
Brně,221675200954064108148161
Lékařská239475200953675109147171201235273
fakulta26857520095215892136153178215
Masarykovy291875200956299129166190229263306342381
univerzity331675200954386103139178202237254280318
v3651752009536
Brně370575200954064108148
roman.hakl@fnusa.cz42576400952466132169212226270307341360418440485527557594609645680
Cit.43737400095466388103
zkr:44937400095336792108
Vnitř461874000954183100125149
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