Koj Yuav Tiv Thaiv Kab Mob Polycystic raum Li Cas

Mar 20, 2023

Polycystic raum kab mob(PKD) yog ib hom kab mob caj ces uas cov hlwv tsim feem ntau hauv ob lub raum, ua rau lawv loj thiab poob haujlwm dhau sijhawm. Cov cysts tsis yog mob qog noj ntshav, cov hnab ntim sib npaug uas muaj cov kua dej. Cysts sib txawv ntawm qhov loj thiab tuaj yeem loj tuaj. Ntau cov hlwv lossis cov hlwv loj tuaj yeem ua rau lub raum puas. Cov kab mob polycystic raum kuj tuaj yeem ua rau cov hlwv hauv lub siab thiab lwm qhov ntawm lub cev. Tus kab mob no tuaj yeem ua rau muaj teeb meem loj, suav nrog ntshav siab thiab raum tsis ua haujlwm.

Cov kab mob polycystic raum yog ib qho mob hauv lub cev uas tuaj yeem ua rau ntau lub hlwv uas muaj cov kua dej hauv lub raum. Cov tsos mob no tuaj yeem ua rau muaj ntau yam tsos mob, suav nrog ntshav siab, mob nraub qaum lossis sab nraub qaum, hematuria, kev xav ntawm lub plab tag nrho, lub plab loj vim lub raum loj, mob taub hau, pob zeb raum, raum tsis ua haujlwm, thiab kab mob urinary. Yog tias koj muaj cov tsos mob no, nws yog ib qho tseem ceeb mus ntsib kws kho mob vim tias tsis kho tus kab mob polycystic raum tuaj yeem ua rau muaj teeb meem loj.

Cov noob txawv txav tuaj yeem ua rau mob polycystic raum, uas txhais tau hais tias feem ntau, tus kab mob no khiav hauv tsev neeg. Qee lub sij hawm, kev hloov pauv tshwm sim tsis tu ncua, yog li ntawd leej niam leej txiv tsis muaj daim qauv ntawm cov noob hloov pauv.

Ob yam tseem ceeb ntawm tus kab mob polycystic raum yog tshwm sim los ntawm kev sib txawv ntawm caj ces:

1. Autosomal dominant polycystic raum kab mob(ADPKD). Cov tsos mob thiab cov tsos mob ntawm ADPKD feem ntau tshwm sim thaum muaj hnub nyoog ntawm 30 - 40. Yav dhau los, hom no tau hu ua tus neeg laus mob polycystic raum kab mob, tab sis cov menyuam yaus kuj tuaj yeem tsim tus kab mob no. Nws tuaj yeem kis mus rau tus menyuam yog tias ib tus niam txiv muaj tus kabmob. Yog tias ib tus niam txiv muaj ADPKD, txhua tus menyuam muaj 50 feem pua ​​​​ntawm kev tsim tus kabmob. Hom no suav rau feem ntau ntawm cov kab mob polycystic raum.

2. Autosomal recessive polycystic raum kab mob(ARPKD). Hom no muaj tsawg dua li ADPKD. Cov tsos mob thiab cov tsos mob feem ntau tshwm sim sai tom qab yug me nyuam. Qee zaum, cov tsos mob tsis tshwm sim txog thaum me nyuam yaus lossis hluas. Ob leeg niam txiv yuav tsum muaj cov noob txawv txav thiaj li yuav tau txais tus kab mob. Yog tias ob leeg niam txiv nqa cov noob rau tus kabmob, txhua tus menyuam muaj 25 feem pua ​​​​ntawm kev tsim tus kabmob.


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Lub raum cystsyog cov koom haum uas muaj txij li kev kho mob tsis tseem ceeb mus rau kev txhim kho lub raum tsis ua haujlwm. Tej zaum lawv yuav raug cais tawm qhov kev tshawb pom, muaj cov caj ces, lossis yog ib feem ntawm cov kab mob uas muaj cov tsos mob ntxiv, xws li cerebral aneurysms, valvular heart disease, colonic diverticula, thiab extrarenal cysts.Autosomal dominant polycystic raum kab mob(ADPKD) yog cov kab mob cystic raum tshaj plaws hauv cov neeg laus, uas feem ntau tshwm sim raws li ntau cov hlwv thiab kev koom tes ntawm ob lub raum. ADPKD raug faib ua filariasis thiab 50 feem pua ​​​​ntawm cov neeg mob yuav ua rau lub raum tsis txaus thiab lub raum tsis ua haujlwm kawg. Hauv ob peb kis, ADPKD tuaj yeem yog ib leeg lossis raug cais raws li qhov chaw cais tag nrho hu ua atypical polycystic raum kab mob lossis unilateral raum cystectomy. Atypical polycystic raum kab mob tsis tuaj yeem paub qhov txawv ntawm ob sabpolycystic raum kab mob(PKD) los ntawm kev kos duab lossis histology. Nws yog suav hais tias yog benign, tsis paub tias muaj extrarenal manifestations, tsis mus rau lub raum tsis ua hauj lwm, thiab tsis paub genetic predisposition.

Atypical polycystic raum kab mob(APKD) yog ib qho tsis tshua muaj, benign kab mob uas tsis paub qhov txawv ntawm autosomal dominant polycystic raum kab mob (ADPKD) ntawm cov duab thiab histology tab sis tam sim no suav tias yog ib qho kab mob sib txawv kiag li. Nws tuaj yeem sib txawv ntawm ADPKD hauv ntau txoj hauv kev. APKD yog benign, non-progressive, tsis muaj extrarenal tshwm sim, thiab suav hais tias yog ib tug kab mob uas tsis yog-genetic kab mob, whereas ADPKD yog ib tug symmetrical, ob tog, monogenic kab mob. Unilateral ADPKD tau tshaj tawm hauv cov ntaub ntawv tab sis tsis tshua muaj neeg. Tsis zoo li ADPKD thiab lwm yam kab mob polycystic raum, cov hlwv extrarenal tsis pom nyob rau hauv APKD, uas tau piav qhia tias muaj ntau yam hlwv uas cuam tshuam rau tag nrho lub raum lossis cov cheeb tsam tshwj xeeb ntawm lub raum nrog parenchyma nyob nruab nrab ntawm.

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Lub pathogenesis tsis meej, tab sis muaj cov kev xav tias nws yuav tshwm sim los ntawm kev hloov pauv ntawm lub raum parenchyma. ADPKD tam sim no suav tias yog ciliopathy. Hauv cov kab mob no, kev hloov pauv lossis kev tshem tawm hauv cov noob uas hloov cov qauv thiab / lossis kev ua haujlwm ntawm thawj cilia (immobile) ntawm lub raum (thiab qee zaum cov kua tsib duct), tubular hlwb ua rau muaj cystic expansion ntawm cov qauv no. Cov kab mob raum cuam tshuam nrog ADPKD tam sim no raws li ob qho tib si cortical thiab medullary cysts nyob rau hauv qhov chaw feem ntau yog qhov yooj yim thiab feem ntau nce qhov loj thiab tus lej nyob rau lub sijhawm.

Tshwj xeeb, hauv ADPKD, kev hloov pauv hauv ob lub noob, PKD1 thiab PKD2 ua rau tshem tawm ntawm daim nyias nyias cov proteins polycystin -1 thiab polycystin -2, raws li. Cov proteins no feem ntau yog lub luag haujlwm rau cov dej-tswj cov dej num hauv thawj cilia ntawm lub raum, thiab lawv qhov tsis tuaj yeem yog qhov ua rau muaj cyst tsim, ntxiv rau lwm yam hauv zos lossis somatic. PKD1 thiab PKD2 kev hloov pauv ua rau 85 feem pua ​​​​thiab 15 feem pua ​​​​ntawm ADPKD, feem, thiab kwv yees li 5 feem pua ​​-15 feem pua ​​​​ntawm cov neeg mob yog tshwm sim los ntawm kev tshaj tawm de novo kev hloov pauv uas tsis muaj keeb kwm txheeb xyuas tsev neeg. Nws tau kwv yees tias cov xwm txheej nthuav tawm ntawm PKD tuaj yeem ua rau "atypical", xws li cov xwm txheej APKD tau nthuav tawm ntawm no. Qee qhov xwm txheej no yog vim kev hloov pauv hauv cov protein biosynthesis txoj hauv kev endoplasmic reticulum.

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Lwm qhov xwm txheej ntawm PKD, hauv cov neeg mob uas tsis muaj kev hloov pauv, xav tias yog vim muaj kev xav tsis zoo. Chimerism yog qhov tshwm sim ntawm 2 kab mob sib txawv ntawm cov cell hauv ib tus neeg vim yog kev hloov pauv ntawm somatic thaum lub sij hawm embryogenesis. Cov kev hloov pauv no yuav cuam tshuam nrog cov kab mob ntawm tes thiab / lossis cov hlwb somatic. Somatic chimerism yog xav tias yuav tsum muaj lub luag haujlwm rau cov qauv ntawm lub raum tsis zoo nyob rau hauv cov xwm txheej ntawm PKD, suav nrog cov qauv asymmetric, unilateral, thiab tsis sib npaug. Cov neeg mob nrog APKD tuaj yeem tshwm sim nrog cov tsos mob xws li hematuria lossis mob nraub qaum, tab sis cov neeg mob feem ntau yog asymptomatic thiab cysts pom tshwm sim. Feem ntau, cysts zoo li koom nrog lub raum ncej hauv zos, thaum kev koom tes ntawm tag nrho lub raum tsis tshua muaj.

APKD feem ntau tsuas yog muaj 1 lub raum xwb, tab sis qee qhov xwm txheej tau tshaj tawm nrog qee cov hlwv hauv lub raum contralateral, zoo li hauv peb rooj plaub. Qee qhov xwm txheej atypical tuaj yeem tsim cov qauv ob sab. Hauv daim ntawv no, peb tshaj tawm cov ntaub ntawv tsis tshua muaj ntawm APKD. Txawm hais tias txoj kev ntawm tus kab mob no yog benign los ntawm cov ntaub ntawv tam sim no, nws tseem yog ib qho kev kuaj mob tseem ceeb rau cov kws kho mob kom paub txog. Raws li tau hais los saum toj no, kev saib xyuas tsis tu ncua yuav muaj txiaj ntsig zoo rau cov neeg mob raws li cov xwm txheej ntawm atypical PKD nce mus rau ob sab PKD tau tshaj tawm.

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Yog tias koj muaj kab mob polycystic raum thiab tab tom xav txog kev muaj menyuam, tus kws pab tswv yim caj ces tuaj yeem pab koj ntsuas qhov kev pheej hmoo kis tus kabmob mus rau koj cov xeeb ntxwv. Ua kom koj ob lub raum noj qab nyob zoo raws li qhov ua tau tuaj yeem pab tiv thaiv qee qhov teeb meem ntawm tus kab mob no. Ib txoj hauv kev tseem ceeb tshaj plaws los tiv thaiv koj lub raum yog tswj koj cov ntshav siab.

Zuag qhia tag nrho, tus yuam sij rau kev ua kom ntshav siab ruaj khov yog noj koj cov tshuaj tsis tu ncua raws li koj tus kws kho mob qhia thiab tswj kev noj qab haus huv uas muaj ntsev tsawg thiab noj zaub mov uas muaj txiv hmab txiv ntoo, zaub, thiab cov nplej tag nrho. Tsis tas li ntawd, kev tswj hwm qhov hnyav kom zoo, txiav luam yeeb, ua haujlwm tsis tu ncua, thiab txwv kev haus cawv kuj tseem ceeb rau kev tswj ntshav siab, thiab tsawg kawg 30 feeb ntawm kev siv lub cev muaj zog hauv ib lub lis piam yog pom zoo. Yog tias koj muaj ntshav siab, nws raug nquahu kom koj ua raws li cov lus qhia saum toj no thiab ntsuas koj cov ntshav siab ntau zaus kom ntseeg tau tias koj muaj kev noj qab haus huv.



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