Kev soj ntsuam ntawm lub raum ua haujlwm hauv cov neeg muaj sia nyob tom qab Fontan Palliation

Hu rau: Audrey Hu Whatsapp / hp: 0086 13880143964 Email:audrey.hu@wecistanche.com

Sheena Sharma, MD, * Rebecca L Ruebner, MD, * Susan L Furth, MD, * Kathryn M Dodds, CRNP, † Jack Rychik, MD, † thiab David J Goldberg, MD†

*Division of Nephrology and † Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pa, USA

Abstract

Lub hom phiaj.Kev ua haujlwm Fontan yog ib txoj kev palliative rau congenital single ventricle heart disease. Lub raum ua haujlwm ntev hauv pawg neeg no tsis paub zoo. Peb lub hom phiaj yog txhawm rau ntsuas lub raum ua haujlwm hauv cov neeg muaj sia nyob ntev tom qab Fontan palliation, thiab peb xav tias pawg neeg no yuav muaj ntau dua ntawmmob ntevraum kab mob(CKD) piv rau cov tswj.Tsim.Peb tau ua qhov kev tshawb fawb rov qab los ntawm 68 yam kev soj ntsuam los ntawm Single Ventricle Survivorship Program ntawm Children's Hospital of Philadelphia thaum Lub Xya Hli 2010 thiab Kaum Ob Hlis 2014 piv rau 70 tus menyuam noj qab haus huv zoo ib yam li hnub nyoog thiab poj niam txiv neej. Cov txiaj ntsig tseem ceeb yog CKD, txhais tau tias kwv yees glomerular filtration rate (eGFR)<90 ml/min/1.73="" m2="" using="" creatinine="" and="" cystatin="" c–based="" estimating="" equations.="" secondary="" outcomes="" included="" proteinuria="" and="" elevated="" intact="" parathyroid="" hormone.="">Cov txiaj ntsig.Cov pab pawg Fontan suav nrog 68 cov kev kawm uas muaj hnub nyoog nruab nrab ntawm 13 xyoo (IQR 9.0, 17.3) uas yog nruab nrab 11.1 xyoo (IQR 6.5, 15.7) post-Fontan palliation. Cov pawg neeg no tau muab piv rau 70 tus neeg noj qab haus huv (hnub nyoog nruab nrab 15.5 xyoo (IQR 12.5, 18.3).<18 years="" of="" age="" (full="" ckid="" equation),="" and="" 128.5="" vs.="" 129.7="" ml/min/1.73="" m2="" (p="" 5="" .56)="" in="" fontan="" vs.="" healthy="" subjects="" ="" 18="" years="" of="" age="" (ckd-epi="" creatinine="" and="" cystatin="" formula);="" 10%="" of="" fontan="" subjects="" had="" an=""><90 ml/min/1.73="" m2="" .="" median="" intact="" parathyroid="" hormone="" level="" was="" higher="" at="" 59.4="" pg/ml="" (iqr="" 43.0,="" 83.1)="" in="" the="" fontan="" group="" compared="" to="" 23.4="" pg/ml="" (iqr="" 16.7,="" 30.0)="" in="" controls="" (p="" ="" .001).="" proteinuria="" was="" present="" in="" 10%="" of="" the="" fontan="" group="" compared="" to="" 4.7%="" in="" controls="" (p="" 5="" .27).="">Xaus.Kaum feem pua ​​​​ntawm cov neeg muaj sia nyob ntev tom qab Fontan palliation muaj eGFR<90 ml/min/1.73="" m2,="" and="" higher="" median="" parathyroid="" hormone="" levels="" compared="" to="" controls.="" taken="" together,="" these="" measures="" may="" indicate="" early="">raumkab mob. Cov kev tshawb fawb yav tom ntej yuav tsom mus rau qhov kev soj ntsuam ntev ntawm lub raum kev ua haujlwm thiab kev soj ntsuam ntawm cov xwm txheej txaus ntshai rau CKD post-Fontan palliation.

Cov lus tseem ceeb. NtevLub raumKab mob; Ib leeg mob plawv; Fontan Palliation

Cistanche tuaj yeem kho mob raum --Nyem qhov no kom tau txais cov ntaub ntawv khoom

Taw qhia

Ib hom kab mob ventricle ntawm congenital mob plawv cuam tshuam txog li 1 ntawm 2000 tus menyuam yug los nyob.1 Kev ua haujlwm Fontan yog ib txoj kev palliative rau cov menyuam uas yug los nrog ib leeg ventricles. Nws yog qhov thib peb hauv cov txheej txheem phais mob uas muaj qhov sib txuas cavopulmonary uas tso cai rau cov ntshav rov qab mus rau lub ntsws los ntawm cov hlab ntsha hauv lub cev hauv kev sim tshem tawm hypoxemia thiab ventricular overload. Nws yog feem ntau ua tom qab muaj hnub nyoog 2 xyoos thaum pulmonary vascular resistance nyob rau hauv nadir, thiaj li yuav optimize lub peev xwm rau passive pulmonary ntshav txaus. Vim muaj kev txhim kho hauv kev kuaj mob ua ntej yug menyuam thiab kev nce qib hauv cov txheej txheem phais, ntau tus menyuam yaus muaj sia nyob mus rau cov neeg laus nrog qhov tshwj xeeb ntawm lub plawv plawv. Kev muaj sia nyob ntev yog kwv yees li 75-80 feem pua ​​​​hauv 20 xyoo tom qab txheej txheem.2,3

Kev txawv txav hauv cov hlab ntsha feem ntau tshwm sim nyob rau lub sijhawm hauv cov neeg mob tom qab Fontan palliation vim qhov tsis muaj lub ventricle subpulmonary. Muab qhov tsis muaj "lub twj tso kua mis" thiab lub ventricular thrust los txhawb cov ntshav los ntawm pulmonary vasculature, cov neeg mob uas muaj Fontan lub cev muaj zog yuav tsum tau nce siab hauv nruab nrab venous siab thiab txo cov ntshav siab.4,5 Sij hawm dhau los, cov yam ntxwv tseem ceeb ntawm Fontan physiology tuaj yeem ua tau. rau ib tus tswv tsev ntawm cov txiaj ntsig thib ob suav nrog daim siab fibrosis / cirrhosis, cov pob txha thiab cov leeg tsis zoo, kev loj hlob tsis zoo, cov protein-poob enteropathy, thiab cov kab mob ntsws yas.6-8 Cov txiaj ntsig ntawm Fontan physiology muaj peev xwm ua rau muaj kev cuam tshuam loj rau lub neej ntev thiab lub neej ntev.

Lub raum tau txais kwv yees li ntawm 20-25 feem pua ​​​​ntawm tag nrho cov kev mob plawv, thiab yog li ntawd, cov circulatory abnormalities nws thiaj li cuam tshuam rau lub raum system.9 Kev txo qis hauv lub raum tso zis ua rau lub raum tsis zoo, siv tshuaj nephrotoxic, thiab mob cyanosis uas ua rau cyanotic nephropathy nyob hauv. ob peb yam uas yuav ua rau lub raum raug mob.9–11 Txawm li cas los xij, kev tshawb fawb tsom mus rau lub raum ua haujlwm ntev nyob rau hauv pawg neeg no muaj tsawg.

Qhov Kev Pabcuam Tib Neeg Ventricle Survivorship (SVSP) ntawm Children's Hospital of Philadelphia yog ib txoj haujlwm tshwj xeeb uas cov neeg mob tom qab Fontan palliation raug xa mus rau ntau qhov kev qhuab qhia.12 Qhov kev zov me nyuam tsom mus rau kev ntsuam xyuas tag nrho ntawm qhov kawg ntawm lub cev muaj feem cuam tshuam nrog cov hlab ntsha. deficities manifest tom qab Fontan palliation. Peb tau ua ib qho kev tshawb fawb rov qab los soj ntsuam qhov muaj feem ntau thiab qib ntawm CKD hauv ib pawg ntawm cov neeg mob me thiab cov hluas Fontan uas raug xa mus rau SVSP piv rau kev tswj hwm kev noj qab haus huv. Peb kuj tau soj ntsuam rau cov proteinuria thiab hyperparathyroidism uas tej zaum yuav yog cov cim ntxiv ntawm lub raum raug mob. Thaum kawg, peb tau kawm txog qhov tshwm sim ntawm pre-, peri-, thiab postoperative yam tseem ceeb los txiav txim qhov muaj feem cuam tshuam rau kev loj hlob ntawm CKD.

cistanche cov txiaj ntsig kev noj qab haus huv: kho mob raum kab mob

Cov txheej txheem

Kawm Tsim

Peb tau ua qhov kev tshawb fawb rov qab los ntawm txhua tus neeg mob uas tau mob Fontan palliation thiab mus koom SVSP ntawm Children's Hospital of Philadelphia thaum Lub Xya Hli 1, 2010, thiab Kaum Ob Hlis 5, 2014. Tsis muaj cov txheej txheem cais tawm. Cov kev tswj xyuas tau raug xaiv los ntawm hauv Tsev Kho Mob Menyuam Yaus ntawm Philadelphia cov kev coj ua menyuam yaus thiab muaj hnub nyoog zoo sib xws thiab poj niam txiv neej. Txoj kev tshawb no tau pom zoo los ntawm lub koom haum saib xyuas ntawm lub Tsev Kho Mob Me Nyuam ntawm Philadelphia (CHOP IRB 14-011593). Thawj qhov kawg yog qhov tshwm sim ntawm CKD, txhais tau tias kwv yees glomerular filtration rate (eGFR)<90 ml/min/1.73="" m2,="" using="" age-appropriate="" creatinine="" and="" cystatin="" c–based="" estimating="" equations.="" for="" subjects,="" less="" than="" 18="" years="" of="" age,="" the="" traditional="" schwartz,="" bedside="" ckid,="" ckid="" cystatin="" c,="" and="" full="" ckid="" formulas="" were="" used.13="" for="" patients="" greater="" than="" or="" equal="" to="" 18="" years="" of="" age,="" the="" ckd-epidemiology="" (epi)="" creatinine,="" ckd-epi="" cystatin,="" and="" ckd-epi="" creatinine="" and="" cystatin="" formulas="" were="" used.14="" creatinine="" was="" measured="" by="" enzymatic="" assay.="" cystatin="" c="" was="" measured="" by="" immunonephelometric="" assay="" (associated="" regional="" and="" university="" pathologists,="" salt="" lake="" city,="" ut,="" the="" usa="" for="" the="" fontan="" cohort,="" and="" university="" of="" pennsylvania="" translational="" core="" lab,="" philadelphia,="" pa,="" the="" usa="" for="" the="" control="" cohort).="" all="" other="" laboratory="" studies="" were="" performed="" in="" the="" clinical="" laboratories="" of="" the="" children's="" hospital="" of="" philadelphia.="" secondary="" outcomes="" included="" proteinuria="" and="" elevated="" parathyroid="" hormone="" levels.="" proteinuria="" was="" defined="" as="" urine="" protein="" to="" urine="" creatinine="" ratio="">=0.2 or >= 11 proteinurias ntawm cov zis tso zis.

Peb muab piv rau qhov muaj feem cuam tshuam nrog rau kev ua haujlwm ua ntej (hnub nyoog gestational ntawm kev kuaj mob, hnub nyoog ntawm Fontan palliation, hom kab mob hauv lub plawv, paub cov kab mob urological), peri-operative (xav tau extracorporeal membrane oxygenation thiab / lossis hemodialysis), thiab postoperative (siv. ntawm angiotensin-hloov enzyme inhibitors [ACE inhibitors], angiotensin receptor blockers [ARBs], diuretics, aspirin) hauv cov neeg uas muaj eGFR<90 ml/min/="" 1.73="" m2="" to="" those="" with="" egfr="" ="" 90="" ml/min/1.73="" m2="" within="" the="" fontan="" cohort="" using="" the="" full="" ckid="" or="" ckd-epi="" creatinine="" and="" cystatin="">

Analytic Approach

Kev txheeb xyuas txheeb cais tau ua tiav siv STATA / IC 13.1 (Stata Corporation, College Station, TX, USA). Cov kev hloov pauv tsis tu ncua tau tshaj tawm raws li qhov nruab nrab nrog qhov sib txawv ntawm qhov sib txawv (IQR, 25th-75th feem pua) thiab piv nrog Wilcoxon rank-sum test. Categorical variables tau tshaj tawm raws li feem pua ​​​​thiab piv nrog kev ntsuas chi-square. AP tus nqi<.05 was="" the="" threshold="" for="" statistical="">

Cistanche kho mob raum

Cov txiaj ntsig

Tag nrho ntawm 68 tus neeg mob Fontan thiab 70 tswj cov neeg mob tau suav nrog hauv txoj kev tshawb no. Cov yam ntxwv ntawm cov pej xeem hauv paus ntawm cov pej xeem txoj kev tshawb fawb thiab kev tswj hwm pawg muaj nyob rau hauv Table 1. Lub hnub nyoog nruab nrab ntawm Fontan cov kev kawm yog 13 xyoo (IQR 9.0, 17.3). Lub hnub nyoog nruab nrab ntawm kev tswj hwm tau zoo ib yam li pawg Fontan ntawm 15.5 xyoo (IQR 12.5, 18.3). Hauv pawg Fontan, 42 (61 feem pua) yog txiv neej thiab 58 (85 feem pua) yog dawb piv rau pawg tswj hwm uas 39 (56 feem pua) yog txiv neej thiab 42 (60 feem pua) yog dawb. Kaum xya (32 feem pua) Fontan cov ntsiab lus piv rau tsis muaj kev tswj hwm muaj qhov siab qis dua 3 feem pua ​​​​ntawm poj niam txiv neej thiab hnub nyoog. Plaub (6 feem pua) Fontan cov ntsiab lus tsis hnyav (lub cev qhov hnyav<5th percentile="" for="" subjects="" less="" than="" 19="" years="" of="" age="" or=""><18.5 kg/m2="" in="" those="" greater="" than="" or="" equal="" to="" 20="" years="" of="" age)="" compared="" to="" 1="" subject="" (1%)="" within="" the="" control="" group.="" the="" median="" age="" at="" the="" time="" of="" fontan="" surgery="" was="" 25="" months="" (iqr="" 20.8,="" 38.8),="" and="" the="" most="" common="" congenital="" heart="" anomaly="" was="" hypoplastic="" left="" heart="" syndrome="" in="" 38="" subjects="" (56%).="" within="" the="" fontan="" cohort,="" 63="" (93%)="" were="" on="" aspirin,="" 42="" (61%)="" were="" on="" one="" or="" more="" ace="" inhibitors="" and/or="" arbs,="" and="" 22="" (32%)="" were="" on="" one="" or="" more="" diuretic(s)="" at="" the="" time="" of="" study="" visit.="" patients="" were="" on="" aspirin="" for="" surgical="" indications="" and="" not="" for="" any="" other="">

Cov txiaj ntsig ntawm qhov ntsuas qhov ntsuas rau ob qho tib si Fontan thiab cov neeg koom nrog kev noj qab haus huv tau pom nyob rau hauv Table 2. Cov pab pawg Fontan muaj cov ntshav hemoglobin ntau dua me ntsis nrog qhov nruab nrab ntawm 14.8 g / dL (IQR 14.0, 15.5) piv. rau pawg tswj hwm ntawm 14.0 g/dL (IQR 13.2, 15.1). Cov pab pawg Fontan muaj cov ntshav phosphorus ntau dua me ntsis nrog qhov nruab nrab ntawm 4.7 mg/dL (IQR 4.0, 5.{18}}) piv rau 4.3 mg/dL (IQR 4.{22}} , 4.8) nyob rau hauv pawg tswj. Qhov nruab nrab cov ntshav qab zib hauv cov tshuaj hormones parathyroid tau ntau dua ob zaug siab dua hauv Fontan pawg ntawm 59.4 pg / mL (IQR 43.0, 83.1), piv rau kev noj qab haus huv ntawm 23.4 pg / mL (IQR 16.7, 3{ {36}}.0) (P .001). Ob tus neeg koom nrog CKD theem 2 muaj qhov nce siab tsis zoo ntawm cov tshuaj parathyroid raws li tau hais los ntawm National Kidney FoundationLub raumKab mobOutcomes Quality Initiative guidelines with normal serum 25-OH vitamin D levels. Within the Fontan group, 10.2% (5/50 participants) had proteinuria compared to 4.6% (3/64 participants) within the control group (P5 .27). Of the five participants within the Fontan cohort with proteinuria, three were on one or more ACE inhibitors and/or ARBs. All five subjects had eGFR >= 90 mL/min/1.73 m2.

Ntawm cov ntsiab lus Fontan, qhov nruab nrab cov ntshav creatinine concentration yog {{0}}}.6 mg / dL (IQR 0.5, 0.7) thiab nruab nrab serum cystatin C concentration siab dua. ntawm 0.7 mg/L (IQR {{10}}.6, 0.8). Ntawm cov kev tswj hwm, qhov nruab nrab ntawm cov ntshav creatinine concentration yog 0.7 mg/dL (IQR 0.6, {{20}}}.8) thiab nruab nrab ntshav cystatin C concentration kuj yog 0.7. mg/L (IQR 0.6, 0.7). Cov txiaj ntsig ntawm eGFR suav nrog ntau yam creatinine thiab cystatin C-raws li kev kwv yees sib npaug rau ob qho tib si Fontan thiab pawg tswj hwm tau sau tseg hauv Table 3. Nyob rau hauv cov ntaub ntawv menyuam yaus, muaj qhov sib txawv mus rau eGFR siab dua ntawm Fontan cohort piv rau kev tswj kev siv creatinine-based kwv yees. Kev sib npaug, nrog rau kev txheeb xyuas ntau dua qhov nruab nrab eGFR siv tus qauv CKiD ntawm txaj. Kwv yees GFR zoo ib yam thaum siv CKiD cystatin ib leeg sib npaug. Ntawm cov neeg laus, eGFR zoo ib yam rau Fon tan cov ntsiab lus thiab kev tswj hwm siv creatinine-based sib npaug. Muaj ib qho qauv mus rau qis dua eGFR siv CKD-EPI cystatin ib leeg sib npaug, txawm hais tias qhov no tsis ncav cuag qhov tseem ceeb.

Table 4 qhia txog xya Fontan cov ntsiab lus nrog eGFR<90 ml/min/1.73="" m2="" compared="" to="" those="" with="" egfr="" ="" 90="" ml/min/1.73="" m2="" .="" these="" seven="" subjects="" were="" slightly="" older="" at="" the="" time="" of="" fontan="" surgery="" with="" a="" median="" age="" of="" 38="" months="" compared="" to="" 25="" months="" in="" those="" with="" egfr="" ="" 90="" ml/min/="" 1.73="" m2="" (p="" 5="" .59).="" the="" seven="" participants="" had="" a="" median="" time="" from="" surgery="" to="" follow-up="" of="" 10.8="" years="" which="" was="" slightly="" earlier="" compared="" to="" a="" median="" of="" 13="" years="" in="" those="" with="" egfr="">= 90 mL/ min/1.73 m2. Tsis muaj cov ntsiab lus hauv Fontan pawg muaj teeb meem ua ntej urology lossis xav tau hemodialysis lossis extracorporeal membrane oxygenation hauv lub sijhawm tom qab phais. Hauv cov neeg uas muaj eGFR<90 ml/min/1.73="" m2="" ,="" 100%="" were="" on="" aspirin="" compared="" to="" 92%="" on="" aspirin="" within="" the="" group="" whose="" egfr="" was="" ="" 90="" ml/min/1.73="" m2="" (p="" 5="" .43),="" 100%="" were="" on="" an="" ace="" inhibitor(s)="" or="" arb(s)="" compared="" to="" 57%="" on="" an="" ace="" inhibitor(s)="" or="" arb(s)="" (p="" 5="" .03),="" and="" 57%="" were="" on="" diuretic(s)="" compared="" to="" 30%="" on="" diuretic(s)="" (p="" 5="">

Cistanche txiaj ntsig: txhim kho kev ua haujlwm ntawm kev sib deev

Kev sib tham

In this retrospective cohort study of pediatric and young adult Fontan recipients, we found that approximately 90% had an eGFR >90 mL / min / 1.73 m2 siv cov hnub nyoog tsim nyog creatinine thiab cystatin C-raws li kev kwv yees sib npaug. Txawm hais tias txoj kev tshawb fawb no tau pom tias lub raum ua haujlwm zoo ib yam li kev tswj hwm hnub nyoog zoo sib xws thoob plaws hauv pawg neeg, tseem muaj cov neeg mob tseem ceeb hauv Fontan pawg nrog rau lub raum tsis ua haujlwm. Peb kuj pom tias cov kev kawm hauv Fontan pab pawg muaj qhov nruab nrab ntawm cov tshuaj parathyroid cov tshuaj hormones ntau dua ob npaug ntawm cov kev tswj hwm, thiab 10 feem pua ​​​​ntawm cov neeg Fontan muaj proteinuria. Cov no yog lwm cov cim uas yuav qhia tau tias muaj kev pheej hmoo siab ntawm CKD hauv cov neeg no. Rau peb txoj kev paub, qhov no yog qhov loj tshaj plaws ntawm ib lub chaw kawm ntawm lub raum ua haujlwm nyob rau hauv cov neeg muaj sia nyob ntev tom qab Fontan palliation nrog kev sib piv rau pawg tswj hwm.

Cov kev tshawb fawb ua ntej tau ntsuas lub raum ua haujlwm tom qab Fontan. Anne et al. piav txog 21 yam uas tau ua dhau los ntawm Fontan palliation.15 Cov neeg koom nrog no zoo ib yam li peb pawg neeg nyob rau hnub nyoog thiab ntev txij li Fontan palliation. Siv cov qauv Schwartz, lawv pom tias txhua tus neeg mob muaj eGFR ib txwm muaj thiab tias 43 feem pua ​​​​ntawm cov neeg mob Fontan muaj microalbuminuria. Tsuas yog ua ntej thiab tom qab-Fontan pulmonary vascular kuj tau pom tias muaj txiaj ntsig zoo nrog kev txhim kho ntawm microalbuminuria. Kev siv ACE inhibitors yog kev tiv thaiv, thiab cov neeg koom nrog uas tau txais cov koob tshuaj lisinopril sib npaug ntau dua 0.4 mg / kg / hnub tsis muaj proteinuria. Esch et al tau tshuaj xyuas 211 yam kev kawm rau kev txhim kho mob raum mob hauv thawj peb hnub tom qab kev phais Fontan.11 Tsawg kawg ib qho creatinine tau txais ntawm qhov nruab nrab ntawm 373 hnub (IQR 9 hnub rau 3.8 xyoo) rau 94 feem pua ​​​​ntawm cov neeg mob no. Qhov nruab nrab creatinine tshem tawm ntawm kev soj ntsuam yog 111 mL / min / 1.73 m2 tab sis 8 feem pua ​​(16 tus neeg mob) muaj eGFR ntawm<80 ml/min/1.73="" m2="" .="" of="" these="" 16="" patients,="" 25%="" had="" pre-fontan="" renal="" dysfunction.="" acute="" kidney="" injury="" was="" not="" associated="" with="" the="" development="" of="" long-term="" kidney="">

Hauv txoj kev tshawb no, peb pom tias txawm hais tias qhov nruab nrab ntawm cov ntshav creatinine concentration qis me ntsis ntawm Fontan cov ntsiab lus piv rau kev tswj hwm, lawv muaj qhov nruab nrab cystatin C ntau dua. Kuj tseem muaj feem ntau ntawm Fontan cov ntsiab lus nrog qis qhov siab thiab BMI. Qhov no yuav qhia tau tias cov neeg mob Fon tan muaj cov leeg nqaij tsis zoo lossis kev noj zaub mov zoo, thiab creatinine yuav tsis yog qhov qhia tau tseeb ntawm lub raum ua haujlwm hauv cov pejxeem. Qhov no tau hais txog ntau yam kev tshawb fawb.16–18 Hauv kev tshawb fawb los ntawm Ylinen li al, eGFR ntawm 52 cov neeg mob hauv lub raum tau txiav txim siab los ntawm 51Cr-EDTA clearance thiab piv rau cystatin C thiab creatinine.19 Ib qho kev sib raug zoo tau pom ntawm Qhov sib txuam ntawm cystatin C thiab eGFR ntsuas siv 51Cr-EDTA tshem tawm. Tsis tas li ntawd, nyob rau hauv cov neeg mob uas txo qis lub raum ua haujlwm, kev sib raug zoo ntawm eGFR thiab reciprocal cystatin C (r 5 0.90) muaj zog dua (P 5 .08) ntau dua li ntawm GFR thiab reciprocal creatinine (r {{12). }}.75 ib.). Yog li, suav GFR siv creatinine nkaus xwb raws li kev kwv yees sib npaug tuaj yeem muab qhov kev kwv yees siab ntawm lub raum ua haujlwm hauv pawg no. Cystatin C yog ywj siab ntawm cov leeg nqaij thiab tej zaum yuav yog li muab qhov kev kwv yees ntau dua ntawm lub raum kev ua haujlwm hauv cov neeg no, tshwj xeeb tshaj yog cov neeg mob menyuam yaus thiab cov neeg uas muaj cov nqaij ntshiv loj. Tanner staging tau ua nyob rau hauv kwv yees li ib nrab ntawm Fontan pawg thiab yog tag nrho raws li kev cia siab pubertal txoj kev loj hlob. Yog li ntawd, kev ncua pubertal tsis tshua muaj feem cuam tshuam rau kev txo cov leeg nqaij.

Txoj kev tshawb no muaj ntau yam kev txwv. Ua ntej, creatinine thiab cystatin C qhov tseem ceeb tau txais ntawm ib zaug. Yog li ntawd, peb tsis tuaj yeem tawm tswv yim txog kev pheej hmoo ntawm CKD lub sijhawm nyob rau hauv pawg neeg no. Kev ntsuam xyuas ntev ntawm GFR ntau xyoo yuav muaj txiaj ntsig los ntsuas kev hloov pauv hauv lub raum ua haujlwm raws li lub sijhawm nrog Fontan physiology nce. Tsis tas li ntawd, cov ntsiab lus Fontan suav nrog hauv txoj kev tshawb no tau cuv npe hauv SVSP ntawm Children's Hospital of Philadelphia. Cov neeg mob no raug xa mus rau thoob tebchaws raws li kev txiav txim siab ntawm lawv tus kws kho plawv thawj zaug rau kev saib xyuas ntxiv hauv chaw kho mob ntau yam. Yog li ntawd, muaj peev xwm xaiv qhov tsis ncaj ncees vim tias cov neeg mob no tuaj yeem ua rau muaj kev nyuaj lossis mob, txawm hais tias txhua tus tau pom hauv qhov chaw kho mob sab nraud thaum lub sijhawm ntsuas. Raws li qhov tshwm sim, peb cov txiaj ntsig yuav tsis tuaj yeem ua tau dav dav rau txhua tus neeg mob uas tau mob Fontan palliation. Tsis tas li ntawd, raws li cov neeg mob no tau raug xa mus rau CHOP, muaj cov ntaub ntawv tsis txaus hauv lawv cov chav kawm ua haujlwm uas tseem ceeb heev uas yuav tsum nco ntsoov. Hauv txoj kev tshawb no, cov zis kuaj tau tsis yog tag nrho cov qauv thawj zaug thaum sawv ntxov, thiab yog li ntawd, muaj peev xwm ua rau cov proteinuria orthostatic hauv cov neeg laus tuaj yeem ua rau muaj txiaj ntsig. Txawm hais tias proteinuria raug soj ntsuam sib txawv hauv Fontan thiab tswj pawg, ntau cov kev tshawb fawb tau pom tias muaj kev sib raug zoo ntawm cov zis dipsticks thiab cov zis protein rau cov zis creatinine ratios.20,21 Thaum kawg, peb tau soj ntsuam qhov muaj feem cuam tshuam rau kev txhim kho CKD tom qab Fontan, tab sis muab tus qauv me me ntawm Fontan cov ntsiab lus nrog eGFR qis, peb tsis muaj peev xwm ua tau cov lus xaus tseem ceeb. Cov xwm txheej txaus ntshai rau kev txhim kho CKD yuav tsum tau kawm ntxiv hauv cov kev tshawb fawb loj ntawm Fontan cov ntsiab lus.

Hauv cov ntsiab lus, peb pom tias kwv yees li 90 feem pua ​​​​ntawm cov neeg muaj sia nyob ntev tom qab Fontan palliation muaj lub raum ua haujlwm. Raws li kev muaj sia nyob ntev tom qab Fontan txuas ntxiv txhim kho, CKD tuaj yeem yog lwm qhov tseem ceeb uas cuam tshuam rau cov pej xeem no. Txawm hais tias peb tsis pom qhov sib txawv tseem ceeb hauv eGFR siv creatinine thiab cystatin C-raws li qhov sib npaug ntawm Fontan cov ntsiab lus piv rau ib pawg ntawm kev tswj hwm kev noj qab haus huv, qhov nce ntxiv ntawm proteinuria thiab hyperparathyroidism tuaj yeem yog lwm qhov ntsuas ntawm lub raum tsis ua haujlwm hauv Fontan cov pejxeem. Cystatin C tuaj yeem muab qhov ntsuas qhov tseeb ntawm GFR ntau dua li cov ntshav creatinine hauv cov neeg no tau muab qhov kev pheej hmoo ntawm cov leeg nqaij qis. Cov kev tshawb fawb yav tom ntej yuav tsom mus rau qhov kev ntsuam xyuas ntev ntawm GFR lub sijhawm thiab kev soj ntsuam ntawm cov kev pheej hmoo rau CKD ntawm cov neeg mob tom qab Fontan.

Sau Kev Pab Txhawb

SS yog lub luag haujlwm rau kev txheeb xyuas cov ntaub ntawv, sau cov kab lus, thiab txheeb cais. RLR yog lub luag haujlwm rau kev txheeb xyuas kev txheeb cais, kev txhais cov ntaub ntawv, kev hloov kho tseem ceeb ntawm tsab xov xwm, thiab kev pom zoo ntawm tsab xov xwm. SLF yog lub luag haujlwm rau kev kho qhov tseem ceeb ntawm tsab xov xwm thiab kev pom zoo ntawm tsab xov xwm. KMD yog lub luag haujlwm rau kev sau cov ntaub ntawv, kho qhov tseem ceeb, thiab kev pom zoo ntawm tsab xov xwm. JR yog lub luag haujlwm rau kev tsim cov kev tshawb fawb, kev tshuaj xyuas cov ntaub ntawv, kev kho qhov tseem ceeb, thiab kev pom zoo ntawm tsab xov xwm. DJG yog lub luag haujlwm rau kev tsim cov kev tshawb fawb, kev tshuaj xyuas cov ntaub ntawv, kev kho qhov tseem ceeb, thiab kev pom zoo ntawm tsab xov xwm.

Kev lees paub

Dr. Rychik thiab kev tshawb nrhiav kev tshawb fawb ntsig txog qhov project Single Ventricle Survivorship Program yog txhawb los ntawm Robert thiab Dolores Harrington Endowed Chair hauv Pediatric Cardiology.

Sau Sau:

Sheena Sharma, MD, Division of Nephrology, Children's Hospital of Philadelphia, 34th Street thiab Civic Center Boulevard, Philadelphia, PA 19104, USA.

Cistanche kho mob raum txhawm rau txhim kho kev sib deev

Cov ntaub ntawv

1 Khairy P, Fernandes SM, Mayer JE Jr, et al. Kev muaj sia nyob ntev, hom kev tuag, thiab kev kwv yees ntawm kev tuag hauv cov neeg mob nrog Fontan phais. Kev ncig. 2008; 117(1:85–92.
2 d'Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton JR. Redefining expectations of the long-term survival after the Fontan txheej txheem: nees nkaum-tsib xyoo ntawm kev soj ntsuam los ntawm tag nrho cov pej xeem ntawm Australia thiab New Zealand. Kev ncig. Xyoo 2014; 130: S32.
3 Gewillig M, Brown SC, Eyskens B, et al. Fontan ncig: leej twg tswj lub plawv tso zis? Interact Cardiovasc Thorac Surg. 2010; 10:428–433.
4 de Leval MR, Deanfield JE. Plaub xyoo ntawm Fontan palliation. Nat Rev Cardiol. 2010; 7(9):520–527.
5 Rychik J. Plaub caug xyoo ntawm kev ua haujlwm Fontan: Lub tswv yim ua tsis tiav. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2010; 13(1): 96–100.
6 Avitabile CM, Goldberg DJ, Zemel BS, et al. Kev tsis txaus ntawm cov pob txha pob txha thiab cov qauv hauv cov menyuam yaus thiab cov neeg laus hluas tom qab Fontan palliation. Pob txha. 2015; 77:12–16.
7 Avitabile CM, Leonard MB, Brodsky JL, et al. Kev siv tau zoo ntawm cov tshuaj insulin zoo li kev loj hlob 1 raws li lub cim ntawm lub plawv tsis ua haujlwm hauv cov menyuam yaus thiab cov hluas tom qab Fontan palliation txheej txheem. Yog J Cardiol. 2015; 115(6):816–820.
8 Goldberg DJ, Shaaddy RE, Ravishankar C, Rychik J. Fontan ua tsis tiav: etiology, kuaj mob, thiab kev tswj xyuas. Kws muaj txuj Rev Cardiovasc Ther. 2011; 9(6):785–793 : kuv.
9 Dimopoulos K, Diller GP, Koltsida E, et al. Prevalence, kwv yees, thiab prognostic tus nqi ntawm lub raum dysfunction nyob rau hauv cov neeg laus uas congenital mob plawv. Kev ncig. 2008; 117(18): 2320–2328.
10 Gupte PA, Vaideeswar P, Kandalkar BM. Cyanotic nephropathy - ib qho kev soj ntsuam morphometric. Congenit Heart Dis. 2013; 9(4): 280–285.
11 Esch JJ, Salvin JM, Thiagarajan RR, del Nido PJ, Rajagopal SK. Mob raum raug mob tom qab ua tiav Fontan: cov yam tseem ceeb thiab cov txiaj ntsig. J Thorac Cardiovasc Surg. 2015; 150(1):190–197.
12 Goldberg DJ, Dodds K, Rychik J. Cov ntsiab lus tshiab: kev txhim kho txoj kev muaj sia nyob rau cov neeg mob uas muaj lub plawv tsis ua haujlwm. Cardiol Young. 2011; 21(suppl 2): ​​77–79.
13 Zhu Y, Ye X, Zhu B, et al. Kev sib piv ntawm 2012 Tshiab CKD-EPI (ChronicLub raumKab mobEpidemiology Collaboration) sib npaug thiab lwm yam plaub qhov sib npaug pom zoo. PLoS Ib. 2014; 9(1):e84688.
14 Inker LA, Schmid CH, Tighiouart H, et al. Kwv yees glomerular filtration rate los ntawm creatinine thiab cystatin C. N Eng J Med. 2012; 367(1:20–29).
15 Anne P, Du W, Mattoo TK, Zilberman MV. nephropathy nyob rau hauv cov neeg mob tom qab Fontan palliation. Ib J Cardiol. 2009; 132(2): 244–247.
16 Bokencamp A, Domenetzki M, Zinck R, Schumann G, Byrd D, Brodehl J. Cystatin C- tus cim tshiab ntawm glomerular pom tus nqi hauv cov menyuam tsis muaj hnub nyoog thiab qhov siab. Pediatrics. 1998; 101(5): 875–881.
17 Schwartz GJ, Schneider MF, Maier PS, et al. Txhim kho qhov sib npaug kwv yees GFR hauv cov menyuam yaus uas muaj mob ntevraumkab mobsiv kev txiav txim siab immunonephelometric ntawm cystatin C. Raum Int. 2012; 82(4): 445–453.

18 Lin KY, Furth SL, Schwartz GJ, Shady RE, Ruebner RL. Kev ntsuam xyuas lub raum ua haujlwm hauv cov menyuam yaus thiab cov tub ntxhais hluas tau txais kev hloov lub plawv thaum lub sijhawm ua haujlwm catheterization. Pediatr Hloov. 2014; 18(7): 757–763.

19 Ylinen EA, Ala-Houhala M, Harmoinen APT, Knip M. Cystatin C ua tus cim rau glomerular pom tus nqi hauv cov neeg mob menyuam yaus. Pediatr Nephrol. 1999; 13(6): 506–509.
20 Puglia, MJ, Wallace JF, Lott JA, et al. Albuminuria thiab proteinuria hauv cov neeg mob pw hauv tsev kho mob raws li ntsuas los ntawm ntau thiab dipstick txoj kev. J Clin Lab Annal. 2001; 15(5): 295–300.
21 Haysom L, Williams R, Hodson E, et al. Kev kuaj pom tseeb ntawm cov zis dipsticks txhawm rau kuaj pom albuminuria hauv cov menyuam hauv paus txawm thiab tsis yog hauv paus txawm nyob rau hauv zej zog. Pediatr Nephrol. 2009; 24(2:323–331).
22 Mori M, Aguirre AJ, Txwj Laug RW, et al. Tshaj li lub siab tawg: circulatory dysfunction nyob rau hauv lub ua tsis tau tejyam Fontan. Pediatr Cardiol. 2014; 35(4): 569–579.
23 Sammour F, Haw M, Paisey J, et al. Lub raum ua haujlwm ntawm cov neeg mob uas tsis ua haujlwm ntawm Fontan Circuit Court tau ua tiav tag nrho cov kev phais kho cavopulmonary. Pediatr Cardiol. 2009; 30(3): 282–288.
24 Perloff JK, Latta H, Barsotti P. Pathogenesis ntawm glomerular abnormality nyob rau hauv cyanotic congenital mob plawv. Yog J Cardiol. 2000; 86(11): 1198–1204.
25 Tutorial O, Denecke A, Bode-Boger SM, et al. Symmetrical dimethylarginine outperforms CKD-EPI thiab MDRD-derived eGFR rau kev ntsuam xyuas ntawm lub raum ua haujlwm nyob rau hauv cov neeg mob uas muaj hnub nyoog congenital mob plawv. Ntshav Qab Zib Res. 2011; 34(1):41–45.
26 Fadrowski JJ, Neu AM, Schwartz GJ, Furth SL. Pediatric GFR kwv yees qhov sib npaug siv rau cov tub ntxhais hluas hauv cov pej xeem. Clin J Am Soc Nephrol. 2011; 6(6): 1427–1435.
27 Awad H, El-Safty I, Abdel-Gawad M, El-Said S. Glomerular thiab tubular dysfunction nyob rau hauv cov me nyuam uas muaj kab mob hauv lub plawv cyanotic: cuam tshuam ntawm kev phais palliative. Am J Med Sci. 2003; 325(3): 110–114.
28 National Raum Foundation. K / DOQI cov txheej txheem kev kho mob rau cov pob txha metabolism thiab kab mob hauv cov menyuam yaus uas muaj mob ntevraumkab mob. Am J Raum Dis. 2005; 46(4) S12–S17.