Recurrent Thromboses Thiab Loj Vessel Compressions hauv Autosomal Dominant Polycystic raum Kab Mob
May 09, 2024
Abstract
Ib tug txiv neej 41- xyoo nrogautosomal dominant polycystic raum kab mob(ADPKD), uas muaj ntau yam kev tshwm sim tsis tau tshwm sim yav dhau los thiab tsis muaj ib qhopaub txog kev mob coagulopathic, nthuav tawm nrogCov tsos mob uas nws kim heev thrombusdistal mus rau qhov chaw compression ntawm sab laug ntau iliac leeg los ntawm ib qho tseem ceeb cyst nyob rau hauv sab laug inferior raum ncej. Qhov no tau tswj hwm nrog qhov tsis zoo vena cava lim ntxig, sab laug nephrectomy, thiab warfarinization. Tom qab ntawd, muaj qhov tsis zoo vena cava compression los ntawm txoj cai polycystic raum, ua rau xaiv txoj cai nephrectomy. Tom qab lub raum hloov pauv, nws muaj cov ntu ntxiv ntawm ib feem ntawm kev lim ntshav nkag mus rau stenosis thiab cov thromboses dav hauv sab laug thiab sab xis sab xis ntawm cov hlab ntsha ntawm cov ceg qis txawm tias tsis muaj extrinsic compression. Qhov no sawv cev rau thawj tsab ntawv ceeb toom ntawm cov nyhuv rov tshwm sim thiab cov xwm txheej thromboembolic hauv ADPKD, ob qho tib si ua ntej thiab tom qab nephrectomy thiab anticoagulation. Qhov muaj peev xwm nce thromboembolic txaus ntshai ntawm cov neeg mob nrog ADPKD lav kev tshawb nrhiav ntxiv.
Taw qhia
Autosomal dominant polycystic raum kab mob (ADPKD)yog qhov feem ntau tau txais txiaj ntsigmob raum, cuam tshuam rau 12 lab tus tib neeg thoob ntiaj teb [1]. Nyob ib ncig ntawm70% ntawm ADPKDCov neeg mob tau nyob ntawm kev kho lub raum, thiab ntau qhov kev mob hnyav thiab mob ntev, mob cyst, rupture thiab hemorrhage, thiabntshav siab [1]. ADPKDcov neeg mob muaj ib tugnce kev pheej hmoo ntawm kev loj hlobintracranial cerebral aneurysms thiabpolycystic daim siab[1, 2], whereas pov thawj ntawm lub raum cell carcinoma yog tsis sib haum xeeb [3–5]. Cov nyhuv loj thiab compression ntawm lwm yam kabmob tau raug tshaj tawm hauvADPKD, txawm tias disproportionately los ntawm daim siab cysts es lub raum cysts. Ob peb kis ntawm cov xwm txheej thromboembolic (TEs) raws li qhov tshwm sim ntawm lub nkoj loj compression tau tshaj tawm [6–10], txawm hais tias qhov tsis zoo vena cava (IVC) compression ntawm qhov tsis meej thiab tom qab hloov pauv TE txaus ntshai tshwm sim rau cov neeg mob ADPKD [11 , 12] ib. Peb tshaj tawm cov ntaub ntawv ntawm tus neeg mob ADPKD uas tau rov pom dua tias muaj cov hlab ntsha loj los ntawm lub raum lub raum thiab cov TEs rov tshwm sim dua, ob qho tib si ua ntej thiab tom qab nephrectomy thiab anticoagulation therapy.
YUAV UA LI CAS RAU CISTANCHE ua haujlwm rau cov neeg mob rau lub raum?
CAS REPORT
Ib tug txiv neej muaj hnub nyoog 41- xyoo ntawm hemodialysis raulub raum tsis ua haujlwm kawglos ntawm niamtau txais ADPKDnthuav tawm nrog 2-lub lim tiam keeb kwm ntawm sab laug calf mob thiab nce peripheral edema. Nws yog lwm yam asymptomatic uas tsis muaj qhov cuam tshuam. Nws keeb kwm kev kho mob yav dhau los yog qhov tseem ceeb rau ntau yam TEs, suav nrog ob sab uas tsis muaj kev cuam tshuam sab sauv sab sauv sab sauv sab sauv sab sauv (DVTs) thiab ntau ntu ntawm arteriovenous fistula thromboses xav tau thrombectomy thiab / lossis fistuloplasty. Nws kuj muaj ntshav siab thiab hypercholesterolemia. Kev tshuaj xyuas lub cev tau qhia txog qhov o ntawm lub hauv caug o thiab palpable tsis xwm yeem plab plab ob sab, qhov kawg ntawm qhov zoo ib yam nrog ADPKD. Nws qhov kev tshawb nrhiav hauv chav kuaj tsis zoo nrog cov platelet suav ntawm 256 × 109 / l, lub sijhawm prothrombin ib txwm, lub sijhawm ua haujlwm thromboplastin ib nrab, thiab cov tshuaj tiv thaiv cardiolipin tsis zoo thiab beta-2 glycoprotein 1 cov tshuaj tiv thaiv. Kev ntsuam xyuas thrombosis yav dhau los, tom qab qhov teeb meem pom ntawm txoj cai sab sauv DVT, yog ib txwm muaj rau kev ua haujlwm antithrombin III, protein C kev ua, protein S qib, Factors II thiab V, thiab cov tshuaj tiv thaiv cardiolipin.
Ultrasound (Tebchaws Asmeskas) thiab tomography suav tomography (CT) ntawm lub plab thiab lub plab pelvis qhia compression ntawm sab laug iliac vein los ntawm ib qho cyst ntawm sab laug polycystic raum (Daim duab 1), nrog rau qhov uas nws kim heev nonocclusive thrombus mus rau sab laug calf. Kuj tseem muaj ntau yam kab mob siab nyob rau hauv ob lub siab lobes, thiab cov duodenal ntu D1 thiab D2 tau hloov mus rau sab laug, tab sis tsis muaj pov thawj los qhia txog kev mob plab hnyuv.
Ib qho xwm txheej ceev sab laug nephrectomy tau ua tom qab IVC lim ntxig. Lub raum ntsuas 350 × 200 × 200 mm thiab hnyav 5530 g; tsis muaj malignancy. Tom qab CT angiogram, tau ua tiav rau kev txiav txim siab ntawm thrombectomy, qhov xwm txheej pom tias compression ntawm IVC los ntawm txoj cai polycystic raum, nrog ze-ua tiav effacement.
Tus neeg mob tau pib ntawm 6- hli warfarin therapy thiab IVC lim tau rov qab tau 6 lub lis piam tom qab tso. Ib lub catheter venogram ua ua ntej IVC lim tshem tawm tau qhia patent txoj leeg sab xis uas tsis muaj thrombus; qhov no tau lees paub rau tom ntej duplex US. Intracranial aneurysm tau raug txiav tawm.
Ntawm 3- hli tom qab, CT angiogram qhia persis tsev pheeb suab IVC compression los ntawm txoj cai polycystic raum, nrog ze-ua tiav effacement (Fig. 2). Tsis muaj thrombus distal rau qhov compression thiab tus neeg mob yog asymptomatic. May-Thurner syndrome tau txiav tawm. Raws li lub raum txoj cai tau pom tias ua rau IVC compression thiab txuas mus rau hauv lub plab mog, ib qho kev xaiv txoj cai nephrectomy tau ua los tiv thaiv ntxiv thromboses nyob rau hauv txoj cai-sab sib sib zog nqus venous systems thiab tsim kom muaj chaw rau kev hloov pauv tom ntej. Lub raum sab xis ntsuas 270 × 170 × 150 mm thiab hnyav 3098 g. Muaj kev hloov pauv cystic nrog fibrosis thiab tubular atrophy; tsis muaj malignancy.
Xya lub hlis tom qab txoj cai nephrectomy, tus neeg mob tau txais kev hloov lub raum los ntawm tus neeg pub rau niam txiv nyob. Nws raug tso tawm 3 hnub tom qab hloov pauv yam tsis muaj teeb meem tam sim ntawd, cov zis tso zis zoo, thiab ua haujlwm tam sim ntawd nrog creatinine ruaj khov [13]. Kaum xya hnub tom qab hloov pauv, tus neeg mob tau nthuav tawm nrog sab laug qis qis edema. Doppler US tau txheeb xyuas lwm ntu ntawm DVT txuas ntxiv los ntawm sab laug ntawm tus ncej puab sab laug femoral leeg mus rau tibioperoneal trunk leeg leeg, cov hlab ntsha sab nraud ntawm sab xis sab xis rau hauv caug cheeb tsam, thiab 50% stenosis ntawm nws sab caj npab sab xis arteriovenous fistul, xav tau fistul. Nws raug pom zoo rau lub neej warfarin.
Kev sib tham
Daim ntawv tshaj tawm no yog qhov tseem ceeb hauv kev nthuav tawm cov xwm txheej ntawm (i) cov nyhuv rov tshwm sim thiab compression ntawm cov hlab ntsha loj raws li cov teeb meem ntawm lub raum hlwv thiab (ii) rov tshwm sim TEs hauv tus neeg mob ADPKD, ob qho tib si nyob rau hauv lub xub ntiag thiab tsis muaj lub nkoj loj compression thiab txawm tias tsim nyog anticoagulation. .
Thaum lub raum loj muaj nyob rau hauv ADPKD, cov lus ceeb toom ntawm lub raum hlwv compressing ntawm cov hlab ntsha loj yog tsawg, nrog rau cov kws sau ntawv tsuas yog paub txog tsib qhov xwm txheej no [6- 10]. Qhov no yog ua raws li peb qhov kev paub dhau los hauv ib qho chaw, qhov twg feem ntau pretransplant nephrectomies tau qhia rau kev txiav txim siab ntawm qhov chaw allograft, uas yog ua raws li kev coj ua lwm qhov thiab cov kws tshaj lij pom zoo [14]. Txawm li cas los xij, kev tshawb fawb hluav taws xob tsis ntev los no tau txheeb xyuas txog li 21% ntawm ADPKD cov neeg mob nrog extrinsic IVC compression los ntawm lub raum hlwv, txawm hais tias ib qho kev cuam tshuam ntawm lub cev tseem yuav txiav txim siab [11].
Daim duab 1. (A) Coronal, (B) sagittal, thiab (C) axial views ntawm tus neeg mob lub CT scan ntawm nws lub plab thiab pelvis ntawm thawj zaug nthuav qhia; qhov no qhia tau hais tias loj polycystic ob lub raum, nrog rau sab laug raum mus rau hauv tus neeg mob lub pelvis; muaj compression ntawm sab laug iliac leeg los ntawm ib tug tseem ceeb cyst nyob rau hauv sab laug polycystic raum tiv thaiv tus neeg mob lub sacrum; nyob rau hauv (c), sab laug iliac hlab ntsha yog depicted nyob rau hauv xiav thiab cov iliac cov hlab ntsha yog qhia nyob rau hauv liab; hepatic cysts thiab duodenal effacement tsis pom nyob rau hauv cov dav hlau xaiv; R, yog; Kuv, qis dua; A, pem hauv ntej; P, qab.
Virchow's triad txiav txim siab tias kev raug mob endothelial, venous stasis, thiab hypercoagulability yog peb yam tseem ceeb uas ua rau thromboses. Cov ntaub ntawv xov xwm dhau los tau hais tias TEs rau hemostasis thib ob rau cov hlab ntsha loj [6-10]. Nyob rau hauv cov ntaub ntawv no, muaj ib tug zuj zus ntawm DVT uas twb muaj lawm thiab thromboses tshiab txawm nephrectomies (uas tshem tawm extrinsic hlab ntsha compression) thiab anticoagulation. Txoj cai polycystic raum compression ntawm IVC tuaj yeem ua rau qeeb ntawm cov hlab ntsha hauv lub plawv, tab sis tsis suav rau cov thrombose tom ntej. Ib qho kev cuam tshuam rau TEs hauv ADPKD cov neeg mob tau txais kev txhawb nqa los ntawm yav dhau los tau tshaj tawm pulmonary embolism thaum tsis muaj lub nkoj loj compression [15] nrog rau cov ntaub ntawv tseem ceeb ntawm TEs ntawm cov neeg mob hloov ADPKD [12]. Peb ib leeg-chaw kev paub dhau los 25 xyoo kuj tau txheeb xyuas cov neeg mob ADPKD ntau dua uas tsim cov xwm txheej thrombotic piv rau lwm pawg neeg mob (cov ntawv sau hauv kev npaj). Ntxiv cov yam ntxwv ntawm ADPKD cov neeg mob yuav tsum tau stratify lawv txoj kev pheej hmoo ntawm cov nyhuv loj thiab TEs. Cov ntaub ntawv tshaj tawm no ua rau cov lus nug ntawm seb cov neeg mob ADPKD puas yuav tsum tau nce mus rau lub neej anticoagulation tom qab thawj ntu ntawm TE thiab seb puas yuav tshem tawm lub raum loj polycystic yuav tsum tau txiav txim siab, tshwj xeeb tshaj yog muab cov txiaj ntsig muaj txiaj ntsig zoo thaum kho mob asymptomatic [6]. Cov ntu tsis tu ncua ntawm TEs hauv peb tus neeg mob tom qab nephrectomy thiab tsim nyog anticoagulation ntxiv rau kev koom tes ntawm ADPKD thiab TEs. Qhov kev sib koom ua tau no thiab nws cov hauv paus pathogenesis lav kev tshawb nrhiav ntxiv.
REFERENCES
1. Nobakht N, Hanna RM, Al-Baghdadi M, Ameen KM, Arman F, Nobakht E, et al. Kev nce qib hauv autosomal dominant polycystic raum kab mob: kev tshuaj xyuas kev kho mob. Raum Med 2020; 2:196–208.
2. Kim H, Park HC, Ryu H, Kim H, Kim HS, Oh KH, et al. Clinical correlates ntawm huab hwm coj nyhuv nyob rau hauv autosomal dominant polycystic raum kab mob. PLoS One 2015; 10:e0144526.
3. Orskov B, Sørensen VR, Feldt-Rasmussen B, Strandgaard S. Kev hloov pauv hauv qhov ua rau tuag thiab kev pheej hmoo ntawm mob qog noj ntshav hauv Danish cov neeg mob uas muaj autosomal dominant polycystic raum kab mob thiab kab mob raum kawg. Nephrol Dial Hloov 2012; 27: 1607.
4. Wetmore JB, Calvet JP, Yu AS, Lynch CF, Wang CJ, Kasiske BL, et al. Kab mob polycystic raum thiab mob qog noj ntshav tom qab hloov lub raum. J Am Soc Nephrol 2014; 25:2335. 5. Yu TM, Chuang YW, Yu MC, Chen CH, Yang CK, Huang ST, et al. Kev pheej hmoo ntawm mob qog noj ntshav hauv cov neeg mob uas muaj kab mob polycystic raum: apropensity-score matched tsom xam ntawm ib lub teb chaws, pej xeem-raws li kev tshawb fawb cohort. Lancet Oncol 2016; 17:1419.
