Tsis tshua muaj lub raum tsis ua haujlwm nrog kev mob ntshav siab, Qhov no tuaj yeem cawm tus menyuam txoj sia!

Bilateral renal agenesis (BRA) yog ib qho kev ua txhaum ntawm lub raum tsis zoo uas tshwm sim los ntawm kev ua tsis tiav ntawm ob lub raum kom loj hlob ib txwm thaum ntxov embryonic kev loj hlob. Txawm hais tias muaj kev txhim kho hauv kev kho tus menyuam hauv plab thiab menyuam mos, kev mob thiab kev tuag ntawm cov menyuam mos uas muaj BRA tseem nyob siab.

Nyem rau Cistanche rau mob raum

Nyob rau tib lub sijhawm, cov menyuam yaus uas muaj BRA tuaj yeem cuam tshuam los ntawm kev mob ntshav siab hnyav, thiab cov tshuaj vasopressors tuaj yeem tsim kev tawm tsam. Tsis ntev los no, PEDIATRICS tau tshaj tawm cov ntaub ntawv qhia txog cov menyuam yaus nrog BRA [1], qhia tias angiotensin II tuaj yeem dhau los ua ib qho kev xaiv tshiab rau kev kho mob ntawm refractory hypotension hauv cov menyuam mos uas muaj BRA.

Tom qab tus poj niam cev xeeb tub tau kuaj pom ua ntej nrog BRA hauv nws tus menyuam hauv plab, cov kws kho mob tau ua cov tshuaj amnioinfusions raws li Fetal Treatment of Intraamniotic Renal Insufficiency Trial Protocol (NCT 03101891).

Tus poj niam cev xeeb tub tau txais 11 amnioinfusions ntawm qhov kwv yees lub hnub nyoog gestational (EGA) ntawm 22 thiab 31 lub lis piam. Tus poj niam cev xeeb tub tau tsim fetal membrane rupture ntawm 31+1 lub lis piam thiab tau txais kev kho betamethasone. Tom qab ntawd ntawm 33+0 lub lis piam, tus poj niam cev xeeb tub tau ua tiav ib tug me nyuam tub los ntawm txoj kev phais.

Tus menyuam yuav tsum tau siv lub tshuab ua pa ntau zaus thiab kev kho nitric oxide vim yog pulmonary artery hypoplasia, pulmonary hypertension, thiab ua pa nyuaj siab. Hnub tom qab, tus kws kho mob tau pib siv Aqaudex (ib hom tshiab ntawm cov khoom siv ultrafiltration yooj yim) ntawm tus menyuam rau kev kho lub raum. Tus me nyuam thawj zaug cranial ultrasound pom qib III hemorrhage nyob rau sab laug ntawm lub ventricle, nrog rau ventricular o. Hnub 10 ntawm kev kho mob, tus me nyuam raug cais tawm ntawm lub raj oxygen thiab pom tias muaj kev sib cuam tshuam hypoxia, yuav tsum tau siv cov pa oxygen ntxiv raws sijhawm. Thaum kho, tus menyuam kuj tau txais kev kho mob dopamine thiab vasopressin tsis tu ncua thiab qee zaus txhaj tshuaj midodrine.

Thaum muaj hnub nyoog 3 lub hlis, tus me nyuam tau tsim muaj kev tsis sib haum xeeb txawm tias kho nrog midodrine thiab Aqaudex txhua hnub. Cov kws kho mob tau kho nws nrog phenobarbital, fosphenytoin, thiab levetiracetam, tab sis nws cov ntshav siab zuj zus ntxiv thiab nws tsim muaj kev qaug dab peg uas tau nce mus rau qhov xwm txheej mob vwm hauv 24 teev.

Cov kws kho mob tau tshuaj xyuas tus menyuam qhov ua rau kis tau, suav nrog kev tshuaj xyuas cov kua cerebrospinal thiab intracranial ultrasound, thiab tsis pom muaj pov thawj ntawm cerebral hemorrhage lossis hydrocephalus. Nws yog ib qho tsim nyog hais tias tus me nyuam yav dhau los qhov tsis sib haum xeeb hypotension tseem nyob, thiab tom qab ntsuas, cov ntshav siab systolic (DBP) nyob nruab nrab ntawm 40 thiab 50 mmHg, thiab qhov nruab nrab arterial siab (MAP) yog nyob nruab nrab ntawm 25 thiab 35 mmHg.

Tus menyuam tau txais cov kua dej resuscitation thiab tau rov pib dua ntawm hydrocortisone, nrog rau kev txhaj tshuaj ntawm dopamine, vasopressin, thiab epinephrine. Tus me nyuam lub hlwb cov ntshav oxygen saturation yog 30%-50% (yav dhau los 50%-70%), lub cev (flank) ntshav saturation yog 80%, thiab perfusion Performance index (PI) yog intermittently qis dua 1%.

Vim yog tus me nyuam lub refractory hypotension, tus kws kho mob pib txhaj tshuaj angiotensin II ntawm 1.25 ng / kg / min nyob rau hnub 5 ntawm kev kho mob thiab titrated nws mus rau 40 ng / kg / min hauv 4 hnub tom ntej. Maj mam txo qhov ntau npaum li cas hauv ob peb hnub thiab txiav kev siv. Nyob rau tib lub sijhawm, thaum siv angiotensin II, kev txhaj tshuaj tas li ntawm heparin yog ntxiv los tiv thaiv thrombosis.

Nws tau pom tias tus neeg mob pib xav tau hydrocortisone (2mg / kg) txhua 6 teev, qhov siab tshaj plaws txhaj tshuaj ntawm dopamine 13mcg/kg/min, vasopressin 0.7mU/kg/min, thiab epinephrine 0. 5 mcg/kg/min. Tom qab kev tswj hwm ntawm angiotensin II, txhua qhov kev tawm dag zog tau ua tiav tsis muaj qhov txo qis hauv MAP.

Tus menyuam tau raug kho nrog angiotensin II thiab nws thiaj li rov zoo thiab raug tso tawm.

BRA yog ib yam kab mob tsis tshua muaj nyob rau hauv kev loj hlob ntawm fetal uas ua rau mob hnyav rau fetus. Ultrasound qhia oligohydramnios feem ntau qhia txog BRA. Txawm hais tias qhov tseeb tsis paub, BRA cuam tshuam nrog kev hloov pauv hauv ntau lub raum kev loj hlob noob.

BRA tuaj yeem yog ib tus kab mob, lossis nws tuaj yeem yog kev hloov pauv hauv qee yam mob, xws li X-txuas qub txeeg qub teg, autosomal recessive qub txeeg qub teg, lossis autosomal dominant qub txeeg qub teg.

Thaum lub sij hawm embryonic lub sij hawm, lub raum primitive muaj xws li pronephros, mesonephros, thiab metanephros. Thaum lub sij hawm kev loj hlob, feem ntau ntawm pronephros thiab mesonephros degenerate ib tug tom qab lwm. Cov tubules mesonephric txuas nrog cov ducts pronephric los tsim Wolff's ducts, thiab cov ureteral buds loj hlob tawm, tom qab. Nws hlob nyob rau hauv lub raum thiab induces tsim metanephros.

Qhov tsis muaj lub raum feem ntau yog vim qhov ua tsis tiav ntawm cov hlab mesonephric kom loj hlob ureteric buds, yog li tsis ua rau cov metanephric primordia kom sib txawv rau hauv metanephros. Yog li ntawd, tus me nyuam tsis muaj zis los yog cov zis uas tsim tawm tsis tuaj yeem tawm mus rau hauv cov kab noj hniav amniotic, ua rau oligohydramnios lossis txawm tias tsis muaj kua dej amniotic, ua rau pulmonary hypoplasia, uas yog qhov ua rau cov menyuam mos tuag.

Fetuses nrog BRA feem ntau tuag hauv lub sijhawm luv luv tom qab yug menyuam. Rau tus me nyuam hauv plab kom xa tau zoo, amnioinfusion feem ntau yog muab tom qab kuaj mob ua ntej kom tsis txhob cuam tshuam cov kua dej amniotic. Intraamniotic infusion therapy siv rab koob txhaj tshuaj kom tsim nyog ntawm cov ntsev ib txwm rau hauv cov kab noj hniav amniotic los ntawm lub plab raws li kev qhia ntawm B-ultrasound los txhim kho cov mob ntawm oligohydramnios. Nws yog ib txoj hauv kev nyab xeeb, txuag nyiaj, thiab siv tau zoo.

Lwm txoj hauv kev kom rov ua kom cov kua amniotic sai sai muaj xws li:

Noj kua dej ntau

Koj tuaj yeem nce cov kua amniotic los ntawm kev haus dej ntau. Kev haus dej 2000ml hauv 2 teev tuaj yeem nce cov kua amniotic hauv lub sijhawm luv, tab sis cov kua amniotic yuav txo qis tom qab qee lub sijhawm thiab tsis tuaj yeem nyob ntev.

Infusion thiab oxygenation

Thaum qhov xwm txheej ntawm cov kua dej amniotic tsawg yog qhov hnyav, qhov kev txhaj tshuaj yuav tsum tau noj tam sim ntawd kom ceev cov kua amniotic, thiab cov pa oxygen yuav tsum tau ntxiv tam sim ntawd. Qhov no tuaj yeem ua rau cov kua dej amniotic nce ntxiv thiab tiv thaiv tus menyuam hauv plab kom tsis txhob muaj cov kua dej amniotic tsawg.

Ntxiv ntshav

Ntau tus poj niam cev xeeb tub raug kev txom nyem los ntawm cov kua dej amniotic tsis txaus vim cov ntshav tsis txaus hauv lawv tus kheej. Tsis txaus leej niam cov ntshav ntim ua rau cov ntshav ntim tsis txaus hauv tus menyuam hauv plab, uas cuam tshuam ncaj qha rau cov kua dej amniotic thiab ua rau cov kua dej amniotic tsis txaus.

Nws yog ib qho tsim nyog yuav tsum tau ua kom tiav qhov kev ntsuam xyuas ua ntej cov kev kho mob no tau ua tiav, thiab kev kho mob yuav tsum tau ua nyob rau hauv kev saib xyuas kom tsis txhob muaj qhov tshwm sim ntawm kev tsis haum tshuaj, kev xav los ntshav, yug ntxov ntxov, nchuav menyuam, kab mob, thiab lwm yam tsis zoo. cov tshuaj tiv thaiv.

Ua tib zoo saib xyuas cov teeb meem, kev kho tshiab yuav tsum tau tshawb nrhiav thiab tawg!

Tsis tas li ntawd, vim yog lub raum tsis zoo, lawv txoj haujlwm raug puas tsuaj, ua rau muaj teeb meem xws li qhov tsis txaus ntawm cov dej thiab ntsev metabolism, txo cov ntshav ntim, txo cov ntshav siab, thiab hypotension, uas yuav tsum tau kho sai.

Nyob rau hauv cov ntaub ntawv saum toj no, tus me nyuam nrog BRA uas muaj hypotension tau zoo kho tom qab siv angiotensin II. Rov qab mus rau lub hauv paus chiv keeb, thaum angiotensin-hloov enzyme ua rau angiotensin I, endogenous angiotensin II raug tso tawm, uas muaj vasoconstrictive nyhuv thiab tuaj yeem nce MAP. Txawm li cas los xij, nws yog metabolized hauv cov ntshav thiab cov kabmob loj los ntawm cov esterases uas tsis yog tshwj xeeb thiab muaj lub neej luv luv (<1 minute).

Hloov cov suab paj nruag thiab txo cov qib angiotensin II yog tsim nyog rau cov me nyuam mos nrog BRA. Feem ntau hais lus, cov neeg mob uas tsis muaj lub raum muaj qis renin qib thiab qis angiotensin I, uas tiv thaiv kev tso tawm ntawm angiotensin II. Tam sim no, ntau cov kev tshawb fawb tau pom tias angiotensin II muaj txiaj ntsig zoo hauv kev kho mob ntshav siab (xws li refractory hypotension) hauv cov neeg laus thiab menyuam yaus.

Previously, in a large study of adults with vasodilatory shock (symptoms including hypotension), nearly 70% of patients were able to achieve MAP >75 mm Hg nyob rau hauv 3 teev ntawm kev kho mob angiotensin II, piv nrog cov neeg mob placebo. Ntawm 23% ntawm cov neeg mob, tsawg dua cov xwm txheej tsis zoo tshwm sim hauv pawg kho mob. Tsis tas li ntawd, muaj ob qhov xwm txheej piav qhia txog kev siv angiotensin II hauv cov menyuam yaus.

Txawm li cas los xij, cov menyuam yaus hauv cov xwm txheej uas tau piav qhia hauv tsab xov xwm no yog cov menyuam mos, thiab tsis muaj kev tshawb fawb txog kev qhia ua ntej. Nws tsis paub meej thaum angiotensin II tau txais txiaj ntsig zoo rau cov neeg mob BRA thiab refractory hypotension. Kev tshawb fawb ntxiv yog xav tau ntawm kev sib koom ua ke ntawm cov tshuaj tshwj xeeb xws li tshuaj tua kab mob antidiuretic lossis hydrocortisone, lub sijhawm zoo los pib angiotensin II, cov txheej txheem ntawm kev saib xyuas cov teebmeem kev kho mob, thiab cov lus pom zoo rau kev txiav tshuaj.

Hauv kev xaus, angiotensin II tau txhim kho zoo hauv tus menyuam mos uas mob hnyav nrog BRA thiab refractory hypotension. Txhawm rau txiav txim siab txoj hauv kev zoo tshaj plaws, lub sijhawm, thiab kev ua tau zoo ntawm qhov kev kho no hauv cov menyuam mos, kev tshawb fawb ntxiv yog xav tau los tshawb nrhiav thiab txheeb xyuas nws. Txawm li cas los xij, nws tsim nyog hais tias kev kho mob zoo ntawm tus menyuam no muab kev nkag siab tshiab rau kev kho mob ntawm BRA nrog los ntawm refractory hypotension.

Cistanche kho mob raum li cas?

Cistanche yog cov tshuaj suav tshuaj ntsuab siv rau ntau pua xyoo los kho ntau yam mob, suav nrog kab mob raum. 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. Cov khoom tseem ceeb ntawm cistanche yog phenylethanoid glycosides, echinacoside, thiab acteoside, uas tau pom tias muaj txiaj ntsig zoo rau lub raum noj 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.