Mesangial Sclerosis hauv Ib Tus Neeg Mob Nrog Hom 1 Ntshav Qab Zib Tom Qab Simultaneous Pancreas-mob raum Hloov
Mar 14, 2022
Yog xav paub ntxiv: Ali.ma@wecistanche.com
Mesangial sclerosis nyob rau hauv cov neeg mob uas muaj ntshav qab zib hom 1 tom qab hloov pauv hloov pauv hauv lub raum mus rau hauv lub raum txawm tias muaj kev saib xyuas ntawm normoglycemia: daim ntawv tshaj tawm
Boonphiphop Boonpheng et al
Abstract
Keeb kwm:Ib txhijpancreas-hloov raumyog suav tias yog kev kho mob rau hom 1 mob ntshav qab zib mellitus nyuaj los ntawm cov kab mob raum kawg. Peb tshaj tawm ntawm no nyob rau hauv ib rooj plaub ntawm mesangial sclerosis nyob rau hauv tus neeg mob uas tau ua tiav kev hloov pauv hauv lub raum-pancreas txawm tias tswj tau cov piam thaj kom zoo thiab ua haujlwm zoo ntawm pancreatic allograft.
Case presentation:Ib tug 76-xyoo-laus tus txiv neej mob ntshav qab zib hom 1 uas tau txais ib txhijpancreas-hloov raum19 xyoo ua ntej tau nthuav tawm nrog nephrotic ntau yam proteinuria txawm hais tias creatinine nyob ntawm nws theem pib (ib txwm). Hemoglobin A1c thiab qabzib yoo mov tau tswj tau zoo yam tsis tas siv cov tshuaj insulin lossis tshuaj tiv thaiv qhov ncauj. Serum lipase thiab amylase nyob rau hauv qhov kev siv ntau yam thiab tsis muaj pov thawj ntawm cov tshuaj tiv thaiv tshwj xeeb pub dawb. Lub raum allograft biopsy tau ua los ntsuas cov proteinuria thiab pom tias diffuse capillary voj thickening thiab diffuse nruab nrab mus rau loj mesangial sclerosis zoo li ntshav qab zib nephropathy.
Cov lus xaus:Cov ntaub ntawv no qhia txog ib rooj plaub ntawm mesangial sclerosis zoo li mob ntshav qab zib nephropathy nyob rau hauv tus neeg mob uas tswj cov piam thaj zoo tom qab ib txhij.pancreas - raum hloov pauvnrog zoo heev pancreatic allograft muaj nuj nqi.
Ntsiab lus:Kev hloov hauv lub raum-pancreas, Mob ntshav qab zib nephropathy, Mesangial sclerosis, Case report
Nyem rau cistanche extract hmoov rau lub raum ua haujlwm
Keeb kwm
Pancreas allotransplantation yog suav tias yog ib qho kev kho mob kho mob rau cov neeg mob ntshav qab zib hom 1, thiab tib lub sijhawm txiav txiav -hloov raum(SPK) yog ib qho kev xaiv rau cov neeg mob mob raum thiab mob ntshav qab zib hom 1. Cov ntaub ntawv txwv tsis pub tshaj tawm tias kev hloov pauv cov txiav ua tiav tuaj yeem tiv thaiv lossis thim rov qab mob ntshav qab zib nephropathy [1-3] los ntawm kev rov tsim cov piam thaj homeostasis thiab kev saib xyuas ntawm normoglycemia. Hauv tsab ntawv tshaj tawm no, peb nthuav tawm cov ntaub ntawv ntawm tus neeg mob uas muaj mesangial sclerosis zoo li mob ntshav qab zib nephropathy txawm hais tias kev saib xyuas mus sij hawm ntev ntawm normoglycemia tom qab ua tiav.pancreas-mob raum hloov pauv.
Case nthuav qhia
Tus neeg mob yog {{{0}} xyoo-laus tus txiv neej mob ntshav qab zib hom 1 uas tau hloov pauv SPK 19 xyoo dhau los. Tus neeg pub dawb yog ib tug poj niam muaj hnub nyoog 48- xyoo uas tsis muaj keeb kwm mob ntshav qab zib thiab qhov ua rau tuag yog mob taub hau. Koj pub. creatinine kawg yog 44.2 micromol / L (0.5 mg / dL) thiab urinalysis tsis pom proteinuria. Tus neeg tau txais kev mob ntshav qab zib hom 1 txij li hnub nyoog 7 xyoo nyuaj los ntawm qhov mob me me. Kab mob raum tau suav tias yog theem nrab rau ntshav qab zib nephropathy (tsis muaj lub raum biopsy ua tiav) uas tau maj mam mus rau theem kawg ntawm lub raum kab mob (ESKD) xav tau kev lim ntshav peritoneal ib xyoos ua ntej hloov pauv. Nws tau txais induction nrog basiliximab thiab tau txais kev tswj hwm ntawm tus qauv triple immunosuppression nrog tacrolimus, mycophenolate acid, thiab prednisone. Nws txoj kev hloov pauv tau zoo heev yam tsis muaj kev tsis lees paub lossis teeb meem loj.
Ntawm qhov chaw kho mob niaj hnub mus ntsib 19 xyoo tom qab nws hloov pauv, urinalysis pom 2 ntxiv rau cov proteinuria tsis muaj hlwb lossis casts. Cov zis protein-creatinine piv piv tau los ntawm 3 mus rau 4.3 g / hnub ntawm ntau qhov kev ntsuas rov ua dua, sib haum nrog 24-h tso zis, uas pom 3.5 g / hnub ntawm proteinuria. Nws lub hauv paus antihypertensive regimen suav nrog amlodipine thiab metoprolol. Losar tan tau pib tom qab kuaj pom muaj proteinuria. Serum creatinine tseem nyob hauv nws theem pib (88.4–106.1 micromol / L, 1–1.2 mg / dL, eGFR 58–68 mL / min / 1.73m2). Kev kuaj lub cev yog qhov tseem ceeb rau cov kab pedal edema tab sis lwm yam tsis zoo. Kev tshuaj xyuas ntawm cov txiaj ntsig hauv chav kuaj yav dhau los tau pom tias muaj txiaj ntsig zoo ntawm dipstick proteinuria thawj zaug tau sau tseg 2 xyoo dhau los (17 xyoo tom qab hloov pauv). Kev yoo mov qib qabzib yog 5–5.2 mmol / L (90–94 mg / dL) thiab hemoglobin A1c tau tswj tau zoo, txij li 5.2–5.8 feem pua tsis siv cov tshuaj insulin lossis lwm yam tshuaj txo cov piam thaj. Lipase thiab amylase nyob rau hauv kev siv ntau yam ntawm ob qhov kev kuaj xyuas (amylase 88 thiab 91 U / L; Lipase 35 thiab 18 U / L). Nws lub tsev ntshav siab tswj tau zoo. Lub cev qhov hnyav yog 20.5 kg / m2. Ib qho tshuaj tiv thaiv antigen tau pom tias tsis muaj tus neeg pub dawb tshwj xeeb los tiv thaiv lub cev. Immunosuppression yog tacrolimus nrog qib 6.5-10 ng / mL, mycophenolic acid 360 mg ob zaug ib hnub thiab prednisone 5 mg / hnub.
Vim yog qhov pib tshiab nephrotic ntau yam proteinuria, lub raum allograft biopsy tau ua (Fig. 1). Lub biopsy muaj 30 glomeruli uas 8 yog thoob ntiaj teb sclerotic. Los ntawm lub teeb microscopy, glomeruli nthuav tawm diffuse capillary voj thickening thiab diffuse nruab nrab mus rau loj mesangial sclerosis. Yam tsawg kawg yog 4 glomeruli nthuav tawm segmental sclerosis, feem ntau hauv cov chaw perihilar. Tsis muaj glomerulitis, peritubular capillaritis, mesangial hypercellularity, lossis amyloid hom deposits. Tsis muaj necrotizing lesions lossis crescents. Tsis muaj capillary voj ob contours. Nruab nrab tubular atrophy thiab interstitial fibrosis (30-40 feem pua) tau tshwm sim thiab raug cuam tshuam los ntawm kev mob ntev. Tsis muaj tubulitis lossis qhov mob tseem ceeb hauv lub cortex uas tsis muaj qhov caws pliav. Muaj mob hnyav arteriosclerosis thiab intimal hyaline arteriolosclerosis. Muaj focal (~ 20 feem pua) immunofluorescence C4d staining ntawm peritubular capillaries. Glomerular capillary phab ntsa thiab tubular hauv qab daim nyias nyias tau nthuav tawm pseudo linear staining rau IgG thiab albumin. Cov cheeb tsam ntawm segmental sclerosis nthuav tawm smudgy IgM, C1q, thiab C3 staining. Tsis muaj lwm yam tseem ceeb glomerular lossis tubulointerstitial immunofluorescence staining. Electron microscopy qhia tau hais tias mesangial sclerosis loj heev thiab feem ntau cov txheej txheem ntawm ko taw tsis zoo. Qee cov glomerular capillary loops nthuav tawm tsawg kawg segmental thaum ntxov hauv qab daus daim nyias nyias ob lub contouring. Glomerular hauv qab daus daim nyias nyias nyias tsis tuaj yeem ntsuas raws li cov kev tshawb fawb electron microscopy tau ua tiav ntawm cov ntaub so ntswg paraffin rov ua dua. Peritubular capillaries pom ib txwm hauv qab daim nyias nyias. Tsis muaj cov kab mob tiv thaiv kab mob (xws li fibrils lossis microtubules) nyob hauv ib qho chaw. Zuag qhia tag nrho kev tshuaj ntsuam xyuas pom zoo rau mesangial sclerosis zoo li mob ntshav qab zib nephropathy nrog rau theem nrab perihilar focal segmental sclerosis. Muab qhov ntxov tshaj ob zaug contour tsim ntawm electron microscopic kev tshawb fawb ib feem ntawm kev hloov pauv tsis tu ncua ntawm glomerulopathy tsis tuaj yeem raug cais tawm. Tsis muaj lub raum biopsy yav dhau los tau ua lossis muaj rau kev sib piv.
Tom qab kev kuaj ntshav biopsy, peb pib tus neeg mob ntawm losartan nrog cov proteinuria txo qis hauv thaj tsam ntawm 2 mus rau 3 g / hnub thiab creatinine ntawm lub hauv paus (1.2mg / dl lossis eGFR 68ml / min / 1.73 m2). Peb kuj tau txhim kho kev tswj ntshav siab nrog rau kev tiv thaiv kev tiv thaiv xws li kev soj ntsuam lipid vaj huam sib luag thiab ceev ceev cov piam thaj uas tseem nyob li qub.
Kev sib tham thiab xaus
Peb tshaj tawm ib rooj plaub ntawm mesangial sclerosis zoo li mob ntshav qab zib nephropathy nyob rau hauv hom 1 ntshav qab zib cov neeg mob uas tau txais kev vam meej SPK yam tsis muaj kev soj ntsuam lossis biochemical pov thawj ntawm kev ua tsis tiav raws li pom los ntawm cov hemoglobin A1c zoo heev, cov ntshav zoo li amylase / lipase, thiab tsis tas yuav muaj cov tshuaj insulin lossis tshuaj noj hauv qhov ncauj hypoglycemic. .
Mob ntshav qab zib nephropathy tuaj yeem tshwm sim tom qabhloov raumhauv cov neeg mob ntshav qab zib tab sis tsuas yog tsis tshua muaj ua rau graft poob [4]. Kev tswj glycemic hnyav tuaj yeem tiv thaiv lossis ncua kev rov tshwm sim ntawm kev hloov pauv ntawm cov ntshav qab zib nephropathy. Kev sim tshuaj ntsuam xyuas randomized [5] muab piv rau kev tswj cov piam thaj hauv cov ntshav hauv hom 1 mob ntshav qab zib mellitushloov raumib leeg pom qhov nce ntawm glomerular hauv qab daus daim nyias nyias dav, mesangial matrix ntim, thiab arterial hyalinosis nyob rau hauv ob pawg ntawm 5 xyoo tab sis cov txheej txheem pab pawg neeg muaj ib tug ob-fold ntau dua nyob rau hauv mesangial matrix ntim feem ntawm lub raum biopsy piv rau cov intensive qabzib pab pawg neeg. Qhov tseem ceeb, txawm tias nyob hauv pawg tswj hwm hnyav, hemoglobin A1c tseem nyob siab dua qhov qub (5.1-7.3 feem pua); Yog li ntawd, txawm tias cov kab mob pathologic ntawm ntshav qab zib nephropathy tseem yuav txhim kho yog tias ua tiav normoglycemia tseem tsis tau teb.
Pancreas transplantation is considered a curative treatment of type 1 diabetes, and diabetic nephropathy lesions can possibly regress or at least be prevented after a successful SPK. This was illustrated by a study by Fioretto et al. [1] that showed signifcant regression of mesangial fraction volume on kidney biopsy at 10 years but not at 5 years in eight type 1 diabetic patients with biopsy-confirmed diabetic nephropathy undergoing pancreas transplantation alone. In contrast, Kim et al. [6] reported a case of diabetic nephropathy progression in type 1 diabetic patients after successful pancreas transplantation alone (PTA). The patient had normal kidney function (eGFR 123.3 mL/min/1.73m2 by CKD-EPI) and only moderately increased albuminuria (174.4 mg/g urine albumin creatine ratio) prior to transplant. After pancreas transplantation, hemoglobin A1c was normalized to less than 5%. Prior to pancreas transplantation hemoglobin, A1c was 11.1%. However, proteinuria continued to increase beginning 2 months post-transplant. A percutaneous kidney biopsy performed at 52 months after transplantation showed basement membrane thickening (>500 nm los ntawm electron microscopy) thiab Kimmelstiel-Wilson nodules raug mob ntshav qab zib nephropathy; tsis muaj pov thawj ntawm calcineurin inhibitor toxicity lossis lwm yam kev hloov pauv.
Cistanche yog qhov zoo raupancreas - raum hloov pauv
Qhov kev tshawb pom, qhov no, tsa cov lus nug txog lub hauv paus txheej txheem ntawm kev mob ntshav qab zib nephropathy thiab seb puas muaj lwm yam uas tsis yog hyperglycemia tuaj yeem ua lub luag haujlwm hauv nws cov kab mob. Muaj cov ntaub ntawv qhia txog biopsy-confirmed ntshav qab zib nephropathy thiab ntshav qab zib retinopathy hauv cov neeg mob uas tsis muaj ntshav qab zib txawm tias muaj cov piam thaj ib txwm [7, 8].
Nodular glomerulosclerosis tsis muaj ntshav qab zib (tseem hu ua idiopathic nodular glomerulosclerosis), tus cwj pwm los ntawm mesangial matrix expansion thiab nodularity zoo ib yam li cov pom hauv classic ntshav qab zib nephropathy, tau piav qhia [9]. Cov kev hloov pauv pathologic no tau cuam tshuam nrog kev haus luam yeeb thiab kev kub siab ntev ntev [9]. Nws tau pom zoo tias kev haus luam yeeb lossis kev kub siab ntev ntev tuaj yeem ua rau muaj cov khoom lag luam glycation siab, oxidative kev nyuaj siab, txawv txav ntawm lub raum hemodynamics, thiab angiogenesis, ua rau muaj kev hloov pauv hauv lub raum zoo ib yam li ntshav qab zib [10]. Kev tshuaj xyuas cov ntaub ntawv ntawm idiopathic nodular glomerulosclerosis los ntawm Lopez-Revuelta li al. [11] kuj pom muaj kev koom tes ntawm qhov chaw pathologic nrog rog rog thiab metabolic syndrome. Ua ke, qhov no qhia tau hais tias cov tshuaj insulin tsis kam txawm tias tsis muaj ntshav qab zib ncaj ncees tuaj yeem ua lub luag haujlwm tseem ceeb hauv kev tsim cov kab mob ntshav qab zib nephropathy thiab idiopathic nodular sclerosis. Peb tus neeg mob tsis haus luam yeeb thiab nws cov ntshav siab ntawm cov tshuaj tiv thaiv hypertensive thiab BMI yog qhov qub; Yog li, cov xwm txheej no tsis piav qhia txog kev tshawb pom pathological.
Interestingly, lub biopsy pom tau hais tias focal C4d positivity thiab nta suspicious rau heev thaum ntxov mob glomerupathies. Thaum cov kev tshawb pom no nce qhov muaj peev xwm ntawm qee qhov kev cuam tshuam ntawm cov tshuaj tiv thaiv kab mob ntev ntev-kev kho kom haum xeeb tsis lees paub, qib ntawm mesangial sclerosis tau nce siab dua li qhov feem ntau pom nrog cov kev hloov pauv ntawm cov tshuaj tiv thaiv me me. Tsis tas li ntawd, tsis muaj ntau txheej txheej ntawm peritubular capillary hauv qab daim nyias nyias tau tshwm sim thiab tsis muaj cov kab mob microvascular. Txawm li cas los xij, peb tsis tuaj yeem tshem tawm qhov ua tau tias qhov kev tsis lees txais cov tshuaj tiv thaiv kab mob ntev ntev yog lub luag haujlwm rau cov kev hloov pauv ntawm biopsy. Tsis tas li ntawd, muaj qhov tsis muaj cov tshuaj tiv thaiv tshwj xeeb lossis keeb kwm ntawm ib qho kev tsis lees paub ua ntej uas yuav pab txhawb kev tiv thaiv kab mob tsis tu ncua. Tsis tas li ntawd, kev loj hlob ntawm allograft dysfunction feem ntau pom nyob rau hauv transplant glomerupathies, uas tsis tau pom nyob rau hauv tus neeg mob no.
Qhov tseem ceeb ntawm arteriolar hyalinosis tau tshwm sim. Qhov kev tshawb pom no tuaj yeem pom nrog ntshav qab zib nephropathy, hnub nyoog, mob ntshav siab, nrog rau kev kho mob calcineurin inhibitor. Cov kab mob calcineurin inhibitor toxicity kuj tuaj yeem yog qhov tseem ceeb hauv kev hloov pauv hauv qhov kev kuaj ntshav no suav nrog cov segmental glomerulosclerosis; Txawm li cas los xij, mesangial sclerosis tsis yog feem ntau suav tias yog ib qho tseem ceeb pom hauv qhov chaw no.
Lwm yam kev kuaj mob sib txawv rau mesangial sclerosis muaj xws li kab mob teeb pom kev zoo, fibrillary glomerulopathy, thiab lwm yam kab mob esoteric deposition (xws li, fibronectin glomerupathies). Cov kev tshawb fawb immunofluorescence yog qhov tsis zoo uas txiav txim siab tawm cov kab mob hauv lub teeb pom kev, fibrillary glomerupathies, lossis lwm yam kev tiv thaiv kab mob sib kis. Cov kev tshawb fawb electron microscopic tsis tau qhia lwm cov deposits los yog fibrils. Congo liab stain tsis tau ua; Txawm li cas los xij, tsis muaj lwm yam kev tsis txaus ntseeg rau amyloidosis. Tsis tas li ntawd, cov ntshav cov protein electrophoresis tsis zoo rau monoclonal gammopathy. Nco ntsoov, cov kev tshawb fawb electron microscopic me ntsis txwv vim paraffin reprocessing artifacts.
Ib qho kev txwv hauv kev kuaj xyuas cov piam thaj tsis txawv txav hauv kev kho mob niaj hnub yog qhov kev hloov pauv ntawm cov piam thaj hauv ib hnub yuav tsis raug kuaj pom los ntawm kev siv hemoglobin A1c. Yog li ntawd, nws muaj peev xwm hais tias kev hloov pauv hyperglycemia tuaj yeem tshwm sim tsis pom hauv peb tus neeg mob, uas ua rau muaj kev hloov pauv hauv lub raum. Ib daim ntawv tshaj tawm tau piav qhia txog tus neeg mob lub raum biopsy tshawb pom tau sib xws nrog cov ntshav qab zib nephropathy nrog cov piam thaj tsis txaus siab, tab sis tsis muaj ntshav qab zib, tab sis kev soj ntsuam cov piam thaj tas li tom qab tau qhia tias hyperglycemia ua tiav cov qauv rau ntshav qab zib [12].
Peb tshaj tawm cov xwm txheej txawv ntawm mesangial sclerosis zoo li mob ntshav qab zib nephropathy hauv tus neeg mob uas muaj ntshav qab zib hom 1 uas tau ua tiav.pancreas-hloov raumNrog rau kev ua haujlwm zoo ntawm pancreatic allograft, kev saib xyuas mus sij hawm ntev ntawm normoglycemia raws li pom los ntawm hemoglobin A1c thiab insulin-ywj siab.
Cistanche yog qhov zoo raupancreas - raum hloov pauv
Cov ntawv luv
SPK: Simultaneous pancreas-hloov raum; PTA: Kev hloov pauv pancreas ib leeg; ESKD: Kab mob raum kawg; eGFR: kwv yees glomerular pom tus nqi; GBM: Glomerular hauv qab daus membrane; BMI: Lub cev-mas index
Kev lees paub
Tsis siv tau.
Cov neeg sau ntawv pab txhawb
BB tau sau cov ntaub ntawv kho mob thiab sau cov ntawv sau. JZ txhais cov kev tshawb pom ntawm lub raum biopsy. GL, MP, JY tau koom tes nrog kev kho mob ntawm tus neeg mob. BB, JZ, GL, GD, JY tau tshuaj xyuas cov qauv thiab ua qhov kev hloov kho tseem ceeb rau cov ntsiab lus kev txawj ntse. AP txhawb kev sau cov ntaub ntawv kho mob. Txhua tus kws sau ntawv tau nyeem thiab pom zoo cov ntawv sau kawg.
Nyiaj txiag
Tsis muaj.
Muaj cov ntaub ntawv thiab cov ntaub ntawv
Tag nrho cov ntaub ntawv tsim los yog tshuaj xyuas thaum lub sijhawm kawm no suav nrog hauv tsab xov xwm no.
Cov ntaub ntawv
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12. Nishihama K, Nakai T, Kanai K, Sugiyama T, Kosato H, Oka N, et al. Ib rooj plaub ntawm "pob ntseg" ntshav qab zib nephropathy kuaj pom los ntawm kev sib xyaw ua ke ntawm lub raum biopsy tshawb pom thiab kev soj ntsuam cov piam thaj tas li. CEN Case Rep. 2015;4(1:101–5
