Kev rov ua dua ntawm IgA nephropathy Tom qab hloov raum

Nyem qhov no kom paub ntau ntxiv txog Yuav Ua Li Cas Cistanche Kho IgA nephropathy

1 Pathogenesis ntawm IgAN rov tshwm sim tom qab hloov lub raum
Nyob rau theem no, pathogenesis ntawm IgAN tsis tau qhia meej meej, thiab tam sim no ntseeg tias qhov tshwm sim ntawm IgAN yog kho los ntawm ntau yam xws li caj ces, ib puag ncig, thiab kev tiv thaiv kab mob [4]. Kev lees paub ntau dua yog cov kab mob ntawm ntau yam hits: (1) ntau dhau ntawm galactose-deficient IgA1 (Gd-IgA1) yog thawj theem ntawm IgAN; (2) autoantibodies tiv thaiv glycans los yog glycopeptides yog li ntawd (3) Anti-glycan antibody paub thiab tsim ib tug tiv thaiv complex nrog Gd-IgA1 thiab deposits nyob rau hauv lub mesangial cheeb tsam; (4) Activates mesangial hlwb, induces lub secretion ntawm cytokines, chemokines thiab extracellular matrix proteins, thiab ces ua rauraum puas [5-6].

Kev tshawb fawb ntawm cov cim molecular hauv cov kab mob ntawm ntau yam hits tuaj yeem pab kwv yees qhov rov tshwm sim thiab kev loj hlob ntawm IgAN tom qab.r raum hloovthiab muabmuaj peev xwm kho lub hom phiaj. Berthelot et al. [7] pom tias qib siab dua ntawm cov ntshav hauv Gd-IgA1 thiab IgA-IgG cov tshuaj tiv thaiv kab mob thiab cov qib qis ntawm circulating IgA-sCD89 (myeloid cell receptor for IgA) complexes ua ntej kev hloov pauv tau kwv yees ntawm lub raum hloov pauv. rov tshwm sim tom qab IgAN. Txawm li cas los xij, Berthoux et al. [8] pom tias IgG autoantibodies rau cov ntshav Gd-IgA1 tom qab hloov pauv tau cuam tshuam nrog kev pheej hmoo siab ntawm IgAN rov ua dua piv nrog cov neeg noj qab haus huv.
Hauv kaum xyoo dhau los, genome-wide Association kev tshawb fawb (GWAS) tau ua rau muaj txiaj ntsig zoo rau txoj kev tshawb fawb ntawm cov caj ces ntawm IgAN, thiab pom ze li ntawm 20 qhov kev pheej hmoo ntawm kev pheej hmoo siab ntawm IgAN, tab sis kev qhia ntxiv ntawm cov molecules cuam tshuam tseem xav tau cov txheej txheem [9 ]. Ib tug loj GWAS ntawm 20 612 IgAN cov neeg mob ntawm European thiab East Asian poj koob yawm txwv, ntxiv rau kev txheeb xyuas qhov loj histocompatibility complex (MHC), cov khoom ua kom tiav H (cov khoom ntxiv H, CFH), cov qog necrosis factor ligand tsev neeg 13 (cov qog necrosis. factor ligand super tsev neeg 13, TNFSF13), HORMAD2 thiab DEFA loci, ntau yam tshiab IgAN loci raug txheeb xyuas [xws li ITGAM-ITGAX, VAV3, CARD9, human leukocyte antigen
(tib neeg leukocyte antigen, HLA)-DQB1, DEFA] nyob rau hauv plab hnyuv mucosa, nrog rau feem ntau loci txuam nrog inflammatory plob tsis so tswj plab hnyuv kab mob, txij nkawm ntawm plab hnyuv epithelial barrier, thiab cov lus teb rau mucosal pathogens [10]. Dysregulation ntawm cov txheej txheem ntxiv kuj tau txuam nrog IgAN relapse. Kev tshawb fawb los ntawm Zhang Chunyun et al. [11] pom tias feem ntau cov neeg mob uas rov muaj IgAN tom qab hloov lub raum tau nrog kev tso tawm ntawm cov khoom ntxiv C3, thaum tso tawm ntawm C1q thiab C4 tsis tshua muaj. Cov kev tshawb fawb tseem tau lees paub tias txoj hauv kev mannose-binding lectin (MBL) yog koom nrog cov txheej txheem rov qab los ntawm IgAN, thiab cuam tshuam nrog kev nce proteinuria thiab lub raum puas tsuaj, thiab qhov kev cia siab ntawm cov neeg mob C4d deposition tsis zoo [12]. Lub deposition ntawm C1q ntawm lub mesangium kuj muaj ib tug siab kwv yees tus nqi rau rov tshwm sim ntawm IgAN tom qab hloov lub raum [13].

Cov kev tshawb fawb tsis ntev los no tau pom tias ob peb lwm yam tsis yog biomarkers tau kwv yees qhovKev pheej hmoo ntawm IgAN rov tshwm sim tom qab hloov lub raum, tab sis lawv qhov chaw kho mob yuav tsum tau ua pov thawj ntxiv rau hauv pawg neeg ywj pheej loj dua. Piv txwv li Garnier et al [14] pom tias cov ntshav IgA qib tau cuam tshuam nrog kev rov ua dua ntawm IgAN tom qab hloov lub raum thiab tuaj yeem siv los ua tus cim rau kev rov tshwm sim ntawm IgAN. Wu et al [15] tau piav qhia txog lub luag haujlwm ntawm qog necrosis factor (tNF) hauv kev txhim kho txoj kev tshawb fawb tshiab. tau piav qhia tias qog necrosis factor (TNF) B cell activating factor (B cell activating factor) thiab proliferation factor (BAFF) hauv superfamily. BAFF) thiab kev loj hlob-inducing ligand (APRIL) nyob rau hauv cov qog necrosis factor (TNF) superfamily. ligand (APRIL) tuaj yeem koom nrog hauv glomerular o los ntawm kev txo cov ntshav ntshav IgA thiab IgA deposition hauv Martín-Penagos li al. thiab al [16-17] pom muaj kev sib raug zoo dua ntawm APRIL thiab IgAN piv rau BAFF. Kev nce siab ntawm APRIL tom qab hloov pauv lub raum tuaj yeem nce siab thaum ntxov thiab txhawb nqa ntawm APRIL tom qab hloov lub raum tuaj yeem ua rau muaj kev tsim tawm ntau dhau ntawm Gd-IgA1 thiab ua kom rov muaj dua ntawm IgAN, tab sis txij li txawm li cas los xij, vim yog qhov sib txawv ntawm IgAN, qhov xaus tsis yog. meej. Kev tshawb fawb soj ntsuam ntawm 69 tus neeg mob nrog Cov Ntaub Ntawv los ntawm kev tshawb fawb soj ntsuam ntawm 69 cov neeg mob nrog IgAN ua ntej thiab tom qab hloov pauv tau pom tias cov qib siab ua ntej hloov ntshav APRIL tau cuam tshuam nrog kev hloov pauv siab. Kev tshawb fawb soj ntsuam ntawm 69 IgAN cov neeg mob ua ntej thiab tom qab hloov pauv tau pom tias cov ntshav ntshav siab APRIL ua ntej hloov pauv tau cuam tshuam nrog IgAN rov tshwm sim tom qab hloov pauv [18]. Tsis tas li ntawd, Lee thiab al [19] tau siv cov tshuaj proteomics los txheeb xyuas cov serine protease inhibitors (SER) hauv cov zis. Tsis tas li ntawd, Lee et al. [19] siv cov proteomics los txiav txim seb muaj cov serine protease inhibitor (SERPIN) A1, transthyretin, apolipoprotein (APRIL), thiab serotonin hauv cov zis. (SERPIN), apolipoprotein (APO) A4, thiab retinol-binding protein (RTP) hauv cov zis. Retinol binding protein (RBP)4 tuaj yeem siv los saib xyuas IgAN tom qab hloov lub raum. Lawv tuaj yeem siv los ua biomarkers los saib xyuas qhov rov tshwm sim ntawm IgAN tom qab hloov lub raum.
Txoj kev tshawb no ntawm pathogenesis ntawm IgAN tuaj yeem txheeb xyuas cov neeg mob zoo dua ntawm kev pheej hmoo ntawm kev rov tshwm sim dua thiab pab txhim kho qhov rhiab heev ntawm kev kuaj mob. Cov neeg mob uas muaj kev pheej hmoo ntawm rov tshwm sim dua, uas tuaj yeem pab txhim kho qhov rhiab heev thiab qhov tshwj xeeb ntawm kev kuaj mob thiab Kev tshawb fawb ntawm pathogenesis ntawm IgAN tuaj yeem txheeb xyuas cov neeg mob uas muaj kev pheej hmoo rov tshwm sim dua, pab txhim kho qhov rhiab heev thiab tshwj xeeb ntawm kev kuaj mob, thiab muaj kev cuam tshuam rau kev kho mob. txhim kho txoj kev npaj kho mob thiab kev kwv yees.

2 Kev kuaj mob ntawm IgAN rov tshwm sim tom qab hloov lub raum

IgAN rov tshwm sim tuaj yeem txhais tau tias yog kev kho mob rov tshwm sim thiab histopathological rov tshwm sim. Clinical recurrence yog heev heterogeneous thiab tej zaum yuav tsis pom pom tseeb cov tsos mob. Nws yog tus cwj pwm los ntawm asymptomatic microscopic hematuria, proteinuria thiab nce zuj zus hauv kev ua haujlwm ntawm lub raum hloov pauv. Cov yam ntxwv tseem ceeb yog asymptomatic microscopic hematuria, proteinuria thiab kev poob qis hauv kev ua haujlwm ntawm lub raum. Cov yam ntxwv tseem ceeb yog asymptomatic microscopic hematuria, proteinuria thiab kev poob qis hauv kev ua haujlwm ntawm lub raum. Histopathologic recurrence yog tus cwj pwm los ntawm thylakoid
Histopathologic recurrence yog tus cwj pwm los ntawm thylakoid IgA deposition nrog los yog tsis muaj thylakoid hyperplasia thiab, nyob rau hauv ob peb kis, crescentic nta [20]. Histopathologic recurrence feem ntau precedes kev kho mob rov tshwm sim, raws li qhia los ntawm Ortiz li al [21 [21] pom tias histologic recurrence tshwm sim nyob rau hauv kwv yees li 52 feem pua ​​​​ntawm cov neeg mob nyob rau hauv 2 xyoos tom qab hloov lub raum Txoj kev tshawb no los ntawm Ortiz et al [21] qhia tias histological rov tshwm sim tsis muaj proteinuria. lossis hematuria tshwm sim hauv kwv yees li 52 feem pua ​​​​ntawm cov neeg mob hauv 2 xyoos tom qab hloov lub raum. Yog li ntawd, cov cuab yeej kuaj mob tseem ceeb rau kev rov ua dua ntawm IgAN tom qab hloov lub raum tso siab rau ntawm lub raum hloov pauv. Cov cuab yeej tshuaj ntsuam xyuas tseem ceeb rau kev hloov pauv IgAN rov ua dua tshiab (biopsy), suav nrog kho qhov muag, immunofluorescence, thiab electron microscopy, kom sib txawv IgAN rov tshwm sim los ntawm IgAN rov tshwm sim. Qhov no tso cai rau kev sib txawv ntawm IgAN rov tshwm sim los ntawm kev tsis lees paub ntev, tshuaj lom neeg, kev kis kab mob, thiab lwm yam. Cov kab mob thiab lwm yam ua rau muaj kab mob glomerular. Cov txheej txheem kuaj mob suav nrog.
(1) ua ntej hloov lub raum biopsy kom paub meej tias qhov kev kuaj mob ntawm IgAN yog thawj kab mob; (2) post-transplantation renal biopsy los txiav txim siab tib yam pathological ntawm rov tshwm sim; thiab (3) xoom-point puncture biopsy ntawm lub raum hloov pauv kom tsis suav cov neeg pub dawb IgAN (kev kawm ib nrab) [4]. Txawm li cas los xij, nws nyuaj rau muaj 3 lub ntsiab lus saum toj no hauv kev kho mob tiag tiag, thiab ntau tus neeg mob twb nyob rau theem ESRD thaum kuaj pom tus kab mob, muaj teeb meem hauv kev txheeb xyuas tus kab mob thawj zaug, lossis tsis kam ua lub raum biopsy vim muaj cov tsos mob me. thaum lub sijhawm rov tshwm sim dua, nrog rau qhov sib txawv ntawm qhov kev qhia txog biopsy, cov txheej txheem kuaj mob, thiab lub sijhawm rov qab los, thiab cov ntaub ntawv uas twb muaj lawm qhia txog qhov rov tshwm sim ntawm IgAN ntawm 4.5 feem pua ​​​​rau 70.5 feem pua ​​[22-23].
Tsis tas li ntawd, qhov rov tshwm sim ntawm IgAN yog nyob ntawm lub sijhawm. Kev tshuaj ntsuam xyuas rov qab ntawm cov neeg tau txais kev hloov lub raum hauv Australia thiab New Zealand Dialysis thiab Transplant Registry (ANZDATA) pom tias ntawm 2,501 cov neeg tau txais lub raum hloov nrog IgAN uas tau lees paub tias yog thawj tus kab mob, rov tshwm sim ntawm tus kab mob ntawm 5, 10, thiab 15 xyoo tom qab ua haujlwm yog ntau dua li lwm lub tebchaws.
Tus kab mob rov tshwm sim yog 5.1 feem pua ​​, 10.1 feem pua ​​, thiab 15.0 feem pua ​​​​ntawm 10 thiab 15 xyoo tom qab phais, raws li, thiab tus nqi rov muaj dua nrog lub sijhawm hloov pauv [24].
Cov pathological tshwm sim ntawm IgAN rov tshwm sim tom qab lub raum hloov pauv ntau dua li cov neeg ntawm IgAN hauv cov pej xeem vim yog lub xeev tiv thaiv kab mob mus ntev thiab kev sib koom ua ke [25]. endocapillary ypercellularity, E), thiab Segmental glomerulosclerosis / adhesion (S) thiab glomerular sclerosis (S). adhesion (S) thiab tubular atrophy / interstitial fibrosis (TIA). atrophy/interstitial fibrosis (T), uas yog ib qho tseem ceeb rau tus qauv ntawm kev kuaj mob ntawm IgAN [26
Nws yog ib qho tseem ceeb rau kev ntsuas tus qauv ntawm IgAN [26]. Nws tau pom tias rov tshwm sim IgAN nrog crescent tsim Qhov tshwm sim ntawm kev hloov lub raum tsis ua haujlwm nyob rau hauv recurrent IgAN nrog crescent tsim tau pom tias yog 88.8 feem pua, qhia tias neonatal qhov tshwm sim ntawm kev hloov lub raum tsis ua haujlwm nyob rau hauv recurrent IgAN nrog crescent tsim tau pom tias yog 88.8 feem pua. , tawm tswv yim tias kev tsim cov menyuam yaus muaj qhov tseem ceeb hauv qhov kev tshwm sim ntawm IgAN rov tshwm sim [27]. Yog li ntawd, Xyoo 2017, cellular thiab/lossis fibrocellular crescents (cellular/ fibrocellular crescents (C) kuj tau suav nrog rau qhov kev ntsuas ntsuas hauv 2017, thiab IgAN Oxford pathological typing tau hloov kho rau MEST-C [28]. Qhov tshwj xeeb IgAN Oxford pathology pathological typing yog qhia nyob rau hauv Table 1 [28].