KDIGO's First Pediatric Renal Syndrome Guideline (draft For Comments) Tso tawm,

Tsis ntev los no, Lub Tsev Kho Mob Raum Txhim Kho Ntiaj Teb Kev Ua Tau Zoo (KDIGO) tau tshaj tawm 2024 KDIGO Cov Lus Qhia Txog Kev Kho Mob rau Kev Ntsuas thiab Kev Kho Mob Nephrotic Syndrome hauv Cov Me Nyuam (Draft for Comments) raws li 2021 KDIGO Cov Lus Qhia rau Cov Kab Mob Glomerular. Nws tau tshaj tawm tias qhov no yog thawj zaug uas KDIGO tau tshaj tawm ib qho kev soj ntsuam kev coj ua rau kev kuaj mob thiab kev kho mob nephrotic syndrome hauv cov menyuam yaus. Tsis tas li ntawd, txhawm rau txhim kho qhov tseem ceeb ntawm cov lus qhia no rau kev kho mob, KDIGO cia siab tias yuav tau txais cov kev xav ntawm ntau tus kws tshaj lij hauv nephrology, pediatrics, thiab lwm lub tuam tsev los ntawm cov qauv ntawm cov lus qhia no.

Nyem rau Cistanche rau mob raum

Kab lus no tau muab tso ua ke raws li cov lus qhia no (duab rau cov lus pom, tshem tawm tom qab), thiab muab faib ua plaub ntu, xws li kev kuaj mob, kev kuaj mob, kev kho mob, thiab steroid-resistant nephrotic syndrome hauv cov menyuam yaus.

kuaj mob

Cov kev kuaj mob rau cov mob nephrotic hauv cov menyuam yaus muaj ob yam: ① Proteinuria nyob rau hauv cov kab mob nephrotic (txhais tias yog cov zis thaum sawv ntxov lossis 24 teev cov zis protein rau creatinine piv [UPCR] Ntau dua lossis sib npaug rau 2g / g lossis 200mg / mmol lossis {{3} } thiab saum toj no); ② Ua ke nrog qis albumin Cov neeg mob pom tias muaj edema thaum muaj ntshav tsis txaus (serum albumin<30 g/L [3 g/dL]) or when the albumin level is undetectable.

Qee cov npe tsim nyog hauv kab lus no muaj cov ntsiab lus hauv qab no:

Kev tshem tawm kom tiav: Peb qhov kev ntsuam xyuas sib law liag pom tias thaum sawv ntxov tso zis lossis 24hUPCR Tsawg dua lossis sib npaug li 200mg/g (0.2g/g) lossis proteinuria tsis zoo.

Partial remission: morning urine or 24hUPCR>200mg/g (0.2g/g) tab sis<2g/g, and serum albumin ≥30g/L (3g/dL).

Relapse: Kev rov tshwm sim ntawm nephrotic syndrome nyob rau hauv cov menyuam yaus feem ntau yog ntsuas los ntawm cov zis tso zis, yog li kev rov ua dua yog txhais tau tias yog cov zis kuaj zis ntau dua lossis sib npaug rau 3+ nyob rau 3 hnub sib law liag.

Hormone-rhiab heev nephrotic syndrome: ua kom tiav kev tshem tawm tom qab 4 lub lis piam ntawm cov tshuaj prednisone lossis prednisolone.

Tsis tshua muaj relapsing nephrotic syndrome:<2 relapses every 6 months within 6 months of onset, or <4 relapses every 12 months within the subsequent 12 months.

Kev mob ntshav qab zib ntau zaus: Ntau dua lossis sib npaug li 2 rov qab dua txhua 6 lub hlis hauv 6 lub hlis tom qab pib, lossis Ntau dua lossis sib npaug rau 4 rov ua dua txhua 12 lub hlis hauv 12 lub hlis tom ntej.

Steroid-resistant nephrotic syndrome: Ua kom tiav kev tso cai tsis ua tiav tom qab 4 lub lis piam ntawm txhua hnub prednisone lossis tus qauv-kuaj prednisolone.

Hormone-dependent nephrotic syndrome: ob qhov sib law liag thaum lub sij hawm kho prednisone los yog prednisolone (tag nrho cov koob tshuaj los yog txo koob tshuaj) los yog nyob rau hauv 15 hnub ntawm kev txiav tawm ntawm prednisone los yog prednisolone.

Lub sijhawm lees paub: lub sijhawm ntawm 4 mus rau 6 lub lis piam txij thaum pib ntawm kev kho prednisone lossis prednisolone, thaum lub sijhawm ntawd, rau cov neeg mob tsuas yog ib feem ntawm cov lus teb ntawm 4 lub lis piam, ntxiv qhov ncauj prednisone lossis prednisolone thiab / lossis cov lus teb yog txiav txim siab los ntawm kev kho mem tes nrog rau cov tshuaj methylprednisolone thiab renin-angiotensin-aldosterone system inhibitor (RASi). Cov neeg mob uas ua tiav cov lus teb ntawm lub lim tiam 6 tau txhais tias yog cov neeg teb lig. Cov neeg mob uas ua tiav cov lus teb ib nrab ntawm 4 lub lis piam tab sis tsis ua tiav cov lus teb ntawm 6 lub lis piam tau txhais tau tias muaj steroid-resistant nephrotic syndrome.

Nephrotic syndrome nrog calcineurin inhibitor (CNI) relapse, tsis kam, thiab ntau yam tshuaj tiv thaiv zoo ib yam li cov tshuaj hormone-dependent, relapse, thiab dependent nephrotic syndrome, uas yog, ob leeg pom tias tsis teb rau cov tshuaj thaum lub sijhawm lees paub. , tshuaj tiv thaiv lossis rov tshwm sim dua.

kev kwv yees

Kev kwv yees ntawm cov menyuam yaus uas muaj tus mob nephrotic yog qhov zoo tshaj plaws los ntawm tus neeg mob cov lus teb rau kev kho thawj zaug thiab qhov zaus ntawm kev rov ua dua tshiab hauv 1 xyoos ntawm kev kho mob. Yog li, lub raum biopsy feem ntau tsis tas yuav tsum tau mus ntsib thawj zaug, tab sis yuav tsum tau ua hauv cov menyuam yaus uas tiv taus kev kho mob lossis muaj chav kho mob atypical.

kho

1 kev kho thawj zaug

Cov kws tshaj lij cov lus qhia: Kev kho thawj zaug yog qhov ncauj corticosteroids rau 8 lub lis piam (kev tswj hwm txhua hnub rau thawj 4 lub lis piam thiab txhua hnub rau 4 lub lis piam tom ntej) lossis 12 lub lis piam (kev tswj hwm txhua hnub rau thawj 6 lub lis piam thiab txhua hnub rau 6 lub lis piam tom ntej. ) (1B ).

Cov ntsiab lus tseem ceeb: thawj koob tshuaj yuav tsum yog: qhov ncauj prednisone lossis prednisolone 60mg / ㎡ / d lossis 2mg / kg / d hauv thawj 4/6 lub lis piam, qhov siab tshaj plaws koob tshuaj yuav tsum tsis pub tshaj 60mg / d, thiab txhua hnub hauv 4 lub lim tiam tom ntej. / 6 lub lis piam Dosing 40mg / ㎡ lossis 1.5mg / kg, qhov siab tshaj plaws koob tshuaj tsis pub tshaj 50mg.

2 Tiv thaiv kev rov tshwm sim

Cov Lus Pom Zoo: Rau cov menyuam yaus uas nquag rov qab los thiab steroid-dependent nephrotic syndrome, peb pom zoo tias cov glucocorticoids niaj hnub tsis tau muab txhua hnub thaum lub sij hawm ua pa sab sauv thiab lwm yam kab mob kom txo tau txoj kev pheej hmoo ntawm rov qab los (1C).

Cov Ntsiab Lus Qhia: Rau cov menyuam yaus uas muaj ① nquag relapses thiab steroid-dependent nephrotic syndrome, thaum muaj kab mob ua pa sab saud, lossis ② twb tau noj cov tshuaj prednisolone qis txhua hnub thiab nrog cov kab mob rov ua dua tshiab los yog prednisone lossis prednisolone Rau cov menyuam yaus uas muaj tus kab mob. keeb kwm ntawm kev kis kab mob pheromonal, noj tshuaj txhua hnub qis (0.5 mg/kg) luv-kawm prednisone lossis prednisolone kev kho mob yuav raug txiav txim siab.

3 Kev kho mob rov qab

Cov tswv yim kws tshaj lij:

Rau cov menyuam yaus uas muaj kev cuam tshuam loj heev ntawm glucocorticoid cuam tshuam thiab nquag rov qab mob nephrotic syndrome, nrog rau txhua tus menyuam yaus uas muaj glucocorticoid-dependent nephrotic syndrome, peb pom zoo kom muab cov tshuaj uas txo cov koob tshuaj glucocorticoids ntau dua li tsis muaj kev kho mob lossis txuas ntxiv kho glucocorticoid ib leeg (1B) .

Cov ntsiab lus tswv yim:

① Kev kho thawj zaug rau kev rov tshwm sim yuav tsum suav nrog prednisolone 60 mg / ㎡ / d lossis 2 mg / kg / d (tsis tshaj 60 mg / d). Cov tshuaj tuaj yeem txiav tsis tau tsuas yog tom qab tus menyuam tau tso tseg ntau dua lossis sib npaug li 3 hnub.

② Tom qab cov neeg mob relapsed hormone-rhiab heev nephrotic syndrome ua tiav kev tshem tawm, txo qhov ncauj prednisone / prednisolone rau 40 mg / m lossis 1.5 mg / kg (siab tshaj 50 mg), noj txhua hnub rau ntau dua lossis sib npaug li 4 lub lis piam.

③ Rau cov menyuam yaus uas nquag rov qab mob nephrotic syndrome lossis hormone-dependent nephrotic syndrome yam tsis muaj glucocorticoid toxicity, tib lub glucocorticoid regimen tuaj yeem siv rau kev rov tshwm sim tom qab.

④ Qhov zoo tshaj plaws, cov neeg mob yuav tsum tau txais kev tso cai ntawm kev kho glucocorticoid ua ntej pib cov tshuaj uas txo cov koob tshuaj glucocorticoid (xws li qhov ncauj cyclophosphamide, levamisole, MMF, rituximab, lossis CNI). Tom qab pib txoj kev kho glucocorticoid tapering, nws raug pom zoo tias qhov kev kho mob ua ke yuav tsum siab dua lossis sib npaug li 2 lub lis piam.

Cistanche kho mob raum li cas?

Cistancheyog ib hom tshuaj suav tshuaj ntsuab siv rau ntau pua xyoo los kho ntau yam mob, suav nrograumkab mob. Nws yog muab los ntawm cov stems qhuav ntawm Cistanche deserticola, ib tsob nroj nyob rau hauv cov suab puam ntawm Tuam Tshoj thiab Mongolia. Lub ntsiab active Cheebtsam ntawmcistancheyogphenylethanoidglycosides, echinacoside cov tshuaj, thiabacteoside, uas tau pom tias muaj txiaj ntsig zoo rauraumnoj qab haus huv.

Kab mob raum, tseem hu ua kab mob raum, hais txog ib yam mob uas lub raum ua haujlwm tsis zoo. Qhov no tuaj yeem ua rau muaj cov khoom pov tseg thiab cov co toxins hauv lub cev, ua rau muaj ntau yam tsos mob thiab teeb meem. Cistanche tuaj yeem pab kho mob raum ase los ntawm ntau lub tswv yim.

Ua ntej, cistanche tau pom tias muaj cov nyhuv diuretic, txhais tau tias nws tuaj yeem ua rau cov zis ntau ntxiv thiab pab tshem tawm cov khoom pov tseg ntawm lub cev. Qhov no tuaj yeem pab txo lub nra ntawm lub raum thiab tiv thaiv kev tsim cov co toxins. Los ntawm kev txhawb nqa diuresis, cistanche kuj tseem tuaj yeem pab txo qis ntshav siab, ib qho teeb meem ntawm cov kab mob raum.

Ntxiv mus, cistanche tau pom tias muaj cov teebmeem antioxidant. Kev ntxhov siab oxidative, tshwm sim los ntawm qhov tsis sib xws ntawm kev tsim cov dawb radicals thiab lub cev tiv thaiv antioxidant, ua lub luag haujlwm tseem ceeb hauv kev mob raum. ies pab neutralize dawb radicals thiab txo Oxidative kev nyuaj siab, yog li tiv thaiv lub raum los ntawm kev puas tsuaj. Cov phenylethanoid glycosides pom hauv cistanche tau tshwj xeeb hauv kev tshem tawm cov dawb radicals thiab inhibiting lipid peroxidation.

Tsis tas li ntawd, cistanche tau pom tias muaj cov nyhuv anti-inflammatory. Kev mob yog lwm yam tseem ceeb hauv kev loj hlob thiab kev loj hlob ntawm cov kab mob raum. Cistanche's anti-inflammatory zog pab txo cov zus tau tej cov pro-inflammatory cytokines thiab inhibit qhov ua kom o yuav tsum tau txoj kev, yog li alleviating o nyob rau hauv lub raum.

Tsis tas li ntawd, cistanche tau pom tias muaj cov teebmeem immunomodulatory. Hauv cov kab mob hauv lub raum, lub cev tiv thaiv kab mob tuaj yeem ua rau tsis zoo, ua rau muaj kev mob ntau dhau thiab cov ntaub so ntswg puas. Cistanche pab tswj lub cev tiv thaiv kab mob los ntawm kev hloov kho thiab kev ua haujlwm ntawm lub cev tiv thaiv kab mob, xws li T hlwb thiab macrophages. Txoj cai tiv thaiv kab mob no pab txo qhov mob thiab tiv thaiv kev puas tsuaj ntxiv rau lub raum.

Ntxiv mus, cistanche tau pom los txhim kho lub raum kev ua haujlwm los ntawm kev txhawb nqa kev tsim kho ntawm lub raum hlab nrog cov hlwb. Lub raum tubular epithelial hlwb ua lub luag haujlwm tseem ceeb hauv kev pom thiab rov nqus cov khoom pov tseg thiab electrolytes. Hauv kab mob raum, cov hlwb no tuaj yeem raug puas tsuaj, ua rau lub raum ua haujlwm puas. Cistanche lub peev xwm los txhawb kev tsim kho ntawm cov hlwb no pab kho lub raum kom zoo thiab txhim kho lub raum tag nrho.

Ntxiv nrog rau cov kev cuam tshuam ncaj qha rau lub raum, cistanche tau pom tias muaj txiaj ntsig zoo rau lwm yam kabmob thiab lub cev hauv lub cev. Txoj hauv kev zoo rau kev noj qab haus huv no tseem ceeb tshwj xeeb hauv cov kab mob raum, vim tias tus mob feem ntau cuam tshuam rau ntau lub cev thiab lub cev. che tau pom tias muaj kev tiv thaiv rau lub siab, lub plawv, thiab cov hlab ntsha, uas feem ntau cuam tshuam los ntawm kab mob raum. Los ntawm kev txhawb nqa kev noj qab haus huv ntawm cov kabmob no, cistanche pab txhim kho lub raum tag nrho thiab tiv thaiv kev mob ntxiv.

Hauv kev xaus, cistanche yog cov tshuaj suav tshuaj ntsuab siv rau ntau pua xyoo los kho mob raum. Nws cov active Cheebtsam muaj diuretic, antioxidant, anti-inflammatory, immunomodulatory, thiab regenerative teebmeem, uas pab txhim kho lub raum ua haujlwm thiab tiv thaiv ob lub raum los ntawm kev puas tsuaj ntxiv. , cistanche muaj txiaj ntsig zoo rau lwm yam kabmob thiab lub cev, ua rau nws txoj hauv kev zoo rau kev kho mob raum.