Aortic Dissection hauv Cov Neeg Mob Hauv Tsev Neeg Nrog Autosomal Dominant Polycystic raum Kab Mob Ⅱ

Kev sib tham

ADPKDyog feem ntau congenitalmob raum cysticnrog kwv yees li ntawm ib tug ntawm 1000 thiab ib tug ntawm 2500 tus neeg.1) Nws cov chav kawm yog characterized los ntawm txoj kev loj hlob thiab inexorable expansion ntawm ntau cysts tawg nyob rau hauv lub raum parenchyma. ADPKD cov neeg mob muaj 10% qhov tshwm sim ntawm intracranial aneurysms.2) Mitral valve prolapse tshwm sim txog li 26% ntawm PKD-1 cov neeg mob.3)Aortic dissectionyog ib qho teeb meem tsis tshua muaj tshwm sim ntawm ADPKD, thiab muaj ob peb tsab ntawv ceeb toom ntawm cov zaus ntawm aortic aneurysm thiab aortic dissection.ADPKDyog genetic heterogeneous, thiab PKD 1 thiab PKD 2 noobʼmutation pab txhawb rau nws txoj kev loj hlob. Kev hloov pauv hauv PKD cov noob uas encode polycystin, uas feem ntau tau qhia hauv cov leeg nqaij leeg, suav nrog rau lub raum, tau qhia tias yuav ua rau comorbid cysts thiab plawv plawv. Hauv kev tshawb nrhiav tus qauv nas, PKD 1 tau cuam tshuam rau kev tswj hwm lub nkoj phab ntsa ntawm cov qauv kev ntseeg siab.4) Qhov no yog ib daim ntawv qhia tsis tshua muaj ntawm aortic dissection hauv ADPKD cov neeg mob hauv tsev neeg tau kho tiav nrog cov khoom siv dag zog kom zoo tshaj plaws ntawm peb txoj kev paub. Congenital polycystic raum thiab ntau yamlub raum cystskuaj pom los ntawm CT ua tiav ADPKD cov qauv ntawm Ravine et al.5) Qhov kev tshawb pom nthuav dav yog tias peb tsev neeg zoo ib yam raug kev txom nyem los ntawm kev phais aortic. Yog vim li cas rau qhov sib txawv histological ntawm leej niam thiab tus ntxhais yog tsis meej. Txawm hais tias medial cystic necrosis tshwm sim hauv niamʼs aortic phab ntsa, nws yog undetected nyob rau hauv tus ntxhais. Cystic medial necrosis yuav tsis tas ua rau aortic dissection. Lwm yam tseem ceeb, suav nrog kev hloov pauv hauv extracellular matrix lossis cellular cuam tshuam los ntawmPKD noob mutation, tej zaum yuav ua rau aortic dissection.

Nyem qhov no kom tau txais Natural organic CISTANCHE EXTRACT nrog 30% ECHINACOSIDE thiab 12% ACTEOSIDE rau(IBD)

Leej niam thiab tus ntxhais raug kuaj mobADPKDua ntej qhov pib ntawmmob aortic dissection(AAD), hais txog qhov kev tshawb pom tseem ceeb uas ua rau lawv AAD yog ADPKD. Kev kub siab thiab lub raum tsis ua haujlwm cuam tshuam nrog ADPKD tau suav tias yog qhov muaj feem cuam tshuam rau aortic dissection tom qab arteriosclerosis.6) Kev tshuaj xyuas zoo tau piav qhia txog qhov muaj ntau zaus ntawm cov ntshav siab thiab cov hnub nyoog yau hauv cov neeg mob aortic dissection nrog ADPKD dua li cov pej xeem aortic dissection tag nrho. Qhov tshwm sim ntawm aortic dissection ua ntej thiab qhov ua tsis tiav ntawm cov tsos mob qhia txog kev phais mob aortic hauv cov neeg mob ADPKD qhia txog qhov tseem ceeb ntawm kev soj ntsuam kev soj ntsuam ze ntawm cov hnub nyoog hluas.6) Kev kho mob ntshav siab tuaj yeem tiv thaiv lub raum tsis ua haujlwm hauv ADPKD cov neeg mob.7)

Hais txog kev kho mob thaum muaj xwm txheej ceev ntawm mob aortic dissection, kev kho mob antihypertensive yog qhov tseem ceeb rau kev tiv thaiv kev ua tiav ntawm kev phais thiab lub cev tsis zoo. Kev ua haujlwm ntawm renin-angiotensin tau tshaj tawm hauv ADPKD. Antihypertensive therapy siv angiotensin-hloov-enzyme inhibitor yuav ua tau zoo rau cov neeg mob aortic dissection nrog ADPKD.8)

Hauv kev xaus, peb ua tiav kho mob hom A aortic dissection hauvADPKDtsev neeg cov neeg mob. Rau kev ntsuam xyuas thiab kev kho mob, ADPKD cov neeg mob thiab lawv tsev neeg yuav tsum tau kuaj xyuas cov kab mob aortic tsis tu ncua.

Kev lees paub

Peb ua tsaug rau Dr. Edward Barroga (http://orcid.org/ 0000-0002-8920-2607) rau kev tshuaj xyuas thiab kho cov ntawv sau.

Daim ntawv tshaj tawm

Txhua tus kws sau ntawv tsis muaj qhov tsis sib haum xeeb ntawm kev txaus siab.

Sau Kev Pab Txhawb

Kawm conception: YI, MO Cov ntaub ntawv sau: YI, MK, MA, KY, MO Kev Ntsuam Xyuas: YI Kev Tshawb Fawb: YI Sau: YI Kev Tau Txais Nyiaj Txiag: Tsis muaj ib qho kev tshuaj xyuas thiab kho dua: tag nrho cov neeg sau ntawv pom zoo kawg ntawm cov ntawv: tag nrho cov sau phau ntawv Kev lees paub rau txhua tus yam ntawm kev ua haujlwm: txhua tus sau

Cov ntaub ntawv

1) Cornec-Le Gall E, Alam A, Perrone RD. AutosomalCov kab mob polycystic raum tseem ceeb. Lancet 2019; 393: 2 ib.

2) Zhou Z, Xu Y, Delcourt C, et al. Puas yog kev tshuaj xyuas tsis tu ncua rau intracranial aneurysm tsim nyog rau cov neeg mob uas muaj autosomal dominant polycystic raum kab mob? Kev tshuaj xyuas qhov system thiab meta-analysis. Cerebrovasc Dis 2017; 44: {4}}. 3) Lumiaho A, Ikaheimo R, Miettinen R, et al. Mitral valve prolapse thiab mitral regurgitation muaj ntau nyob rau hauv cov neeg mob polycystic raum kab mob hom 1. Am J raum Dis 2001; 38: 9:ua. 4) Hassane S, Claij N, Lantinga-van Leeuwen IS, et al. Pathogenic sequence rau dissecting aneurysm tsim nyob rau hauv ib tug hypomorphic polycystic raum kab mob 1 nas qauv. Arterioscler Thromb Vasc Biol 2007; 27: 2177-83}. 5) Ravine D, Sheffield L, Danks DM, thiab al. Kev ntsuam xyuas ntawm ul trasonographic diagnostic cov qauv rau autosomal dominant polycystic raum kab mob 1. Lancet 1994; 343: 20 ib. 6) Silverio A, Prota C, Di Maio M, thiab al. Aortic dissection nyob rau hauv cov neeg mob uas autosomal dominant polycystic raum tsis yooj yim: ib tug series ntawm ob rooj plaub thiab kev tshuaj xyuas ntawm Phau Ntawv. Nephrology (Carlton) 2015; 20: 229-35 ib.

7) Schrier RW, Mcfann KK, Johnson AM. Kev tshawb fawb epidemiological ntawm lub raum ciaj sia nyob rau hauv autosomal dominant polycystic raum kab mob. Raum Int 2003; 63: 3 :ua.

8) Loghman-Adham M, Soto CE, Inagami T, et al. Kev nthuav qhia ntawm cov khoom ntawm renin-angiotensin system hauv autosomal recessive polycystic raum kab mob. J Histochem Cytochem 2005; 53:5 :ua.