Autosomal dominant polycystic raum kab mob (ADPKD) yog cov kab mob hauv lub raum uas tshwm sim ntau tshaj plaws. Txawm li cas los xij, cov lus ceeb toom ntawm qee zaus ntawm aortic dissection hauv PKD fa milial cov neeg mob tseem tsawg. Ntawm no, peb piav qhia txog cov mob aortic tsis tshua muaj tshwm sim hauv PKD tsev neeg cov neeg mob (piv txwv li, niam thiab tus ntxhais) thiab peb txoj kev kho mob tau zoo. Niam (84 xyoo) thiab tus ntxhais (53 xyoos) tau xa tuaj rau peb los kho hom A mob aortic dissection. Peb tau ua kev phais mob xwm txheej ceev thiab ua tiav kho cov neeg mob nrog cov khoom siv dag zog. Rau kev ntsuam xyuas dav dav thiab kev kho mob,ADPKDcov neeg mob thiab lawv tsev neeg yuav tsum tau kuaj xyuas cov kab mob aortic.

CISTANCHE SUPPLEMENT KIDNEY FUNCTION SUPPORT SUPPORT PHGS75% ECH 30% ACT 12%

Ntsiab lus:aortic kev txiav txim, ADPKD

Taw qhia Autosomal dominant polycystic raum kab mob (ADPKD) yog cov kab mob hauv lub raum feem ntau. ADPKD yog genetic heterogeneous, thiab kev hloov pauv ntawm cov kab mob raum polycystic (PKD) 1 thiab PKD 2 noob pab txhawb rau nws txoj kev loj hlob. Qhov zaus ntawm intracranial aneurysms hauv ADPKD cov neeg mob tau tshaj tawm 9% -12% 1) Txawm li cas los xij, muaj qee cov lus ceeb toom txog qee zaus ntawm aortic dissection hauv PKD tsev neeg cov neeg mob. Ntawm no, peb tshaj tawm cov mob aortic dissection hauv PKD tsev neeg cov neeg mob (piv txwv li, niam thiab tus ntxhais) thiab peb txoj kev kho mob tau zoo.

Case Reports Ib tug {{0}} tus poj niam muaj hnub nyoog tau tuaj rau lub chaw kho mob xwm txheej ceev kom muaj mob rov qab sai. Tus neeg mob muaj kev kho mob ntshav siab los ntawm tsib xyoos dhau los. Computed tomography (CT) pom muaj mob B aortic dissection, polycystic raum, thiab ntau yam kab mob siab. Kev kuaj ntshav ntawm kev lees paub tau qhia txog qib creatinine ntawm 0.95 mg / dl thiab kwv yees glomerular filtration rate (eGFR) ntawm 48.5 ml / min / 1.73 m2. Tus neeg mob tau txais kev xa mus rau peb lub chaw kho mob. Kaum rau hnub tom qab, tus neeg mob tau mob siab heev. A CT pom qhov mob A aortic dissection (Fig. 1A thiab 1B), thiab tus neeg mob tau txais kev phais xwm txheej ceev. Ib qho kev txiav tawm nkag tau kuaj pom los ntawm ascending aorta mus rau distal aorta. Tus neeg mob tau hloov pauv tag nrho aortic koov nrog 3-branched artificial graft (J-Graft, Nyiv Lifeline, Tokyo, Nyiv) thiab Frozen ntxhw pob tw (FROZENIX, Nyiv Lifeline). Nws tsis xav tau kev ua haujlwm hemodialysis tom qab. Tus neeg mob tau tso tawm ib hlis tom qab phais tas. Kev soj ntsuam histological pom tau tias muaj kev cuam tshuam ntawm sab hauv elastic lamina thiab tunica media thiab ntws ntawm cov ntshav. Muaj qhov siab ntawm atherosclerosis hauv tunica intima thiab xov xwm. Elastica van Gieson staining pom tsis muaj qhov nruab nrab cystic necrosis (Daim duab 2A thiab 2B).

Ib lub lim tiam tom qab tso tawm, tus niam tus neeg mob muaj hnub nyoog 84- xyoo tau raug xa los ntawm lwm lub tsev kho mob mus rau peb lub chaw rau hom A aortic dissection. CT pom hom A aortic dissection, polycystic ob lub raum, thiab ntau yam kab mob siab (Daim duab 1C thiab 1D). Ib tug dissection nkag tau pom nyob rau hauv lub ascending aorta. Kev kuaj ntshav ntawm kev nkag tau pom tias qib creatinine ntawm 3.82 mg / dl thiab eGFR ntawm 9.3 ml / min / 1.73 m2. Tus neeg mob tau kho mob ntshav siab los ntawm 50 xyoo nrog keeb kwm ntawm PKD thiab lub raum tsis ua haujlwm. Kev hloov pauv sai sai ntawm aortic tau ua tiav siv cov khoom siv ncaj nraim (J-Graft, Nyiv Lifeline). Txawm hais tias tus neeg mob xav tau kev kho hemodialysis nyob rau hauv lub tsev kho mob hnyav, kev ntaus tsis tsim nyog thaum tso tawm. Tus neeg mob tau tsiv mus rau lwm lub tsev kho mob kom rov kho dua ib hlis tom qab phais tas. Kev soj ntsuam histological pom cov kab mob aortic dis seem, rupture ntawm elastic fibers, thiab medial cystic necrosis (Fig. 2C thiab 2D). Tom qab ua haujlwm magnetic resonance imaging pom tsis muaj cerebral aneurysms hauv ob tus neeg mob. Nws tus tij laug tau tuag ntawm aortic dissection ob peb xyoos dhau los, tab sis nws cov keeb kwm kho mob tsis paub meej.