Peb lub cev yeej ib txwm ua haujlwm tom qab qhov xwm txheej, puas yog? Qee zaum, thaum cov txheej txheem ua haujlwm nyuaj no raug cuam tshuam, nws tuaj yeem ua rau muaj kev txhawj xeeb txog kev noj qab haus huv loj heev. Ib qho mob hnyav heev yog Hyperammonemia , uas tshwm sim thaum cov khoom lom hu ua ammonia sib sau ua ke hauv koj cov ntshav. Vim tias qhov mob no tuaj yeem ua rau tuag taus yog tias tsis tswj hwm sai, nws yog ib qho tseem ceeb kom paub txog nws.
Hyperammonemia yog dab tsi?
Hais yooj yim xwb, hyperammonemia yog qhov nce siab ntawm cov ammonia (NH3) hauv koj cov ntshav. Ammonia yog ib yam khoom tsim tawm hauv koj txoj hnyuv thaum koj lub cev zom cov protein uas koj noj. Feem ntau, koj lub siab ua cov ammonia no los ntawm ib qho kev sib law liag hu ua Urea Cycle . Lub voj voog no hloov cov ammonia mus ua urea, ib yam khoom tsis muaj teeb meem ntau uas raug tso tawm ntawm koj lub cev hauv koj cov zis.
Txawm li cas los xij, yog tias muaj kev cuam tshuam hauv lub voj voog urea no lossis yog tias koj lub siab tsis tuaj yeem ua cov ammonia tau zoo, nws pib sib sau ua ke hauv koj cov ntshav. Cov ammonia ntau hauv cov ntshav yog qhov lom heev rau koj lub paj hlwb hauv nruab nrab (CNS) - koj lub hlwb thiab tus txha caj qaum. Qhov no yog vim li cas hyperammonemia yog qhov xwm txheej ceev hauv kev kho mob uas xav tau kev kho mob tam sim ntawd thiab tuaj yeem ua rau tuag taus.
Leej twg muaj kev pheej hmoo tshaj plaws?
Hyperammonemia tuaj yeem cuam tshuam rau txhua tus neeg, txij li cov menyuam mos thiab cov menyuam yaus mus txog rau cov neeg laus.
- Ntawm cov neeg laus, kwv yees li 90% ntawm cov neeg mob muaj feem cuam tshuam nrog Cirrhosis ntawm lub siab .
- Rau cov menyuam mos, nws tuaj yeem tshwm sim los ntawm kev puas tsuaj ntawm lub voj voog urea txij thaum yug los , uas yog cov mob caj ces uas tau txais los ntawm niam txiv.
- Rau cov menyuam yaus, nws kuj tseem yuav tshwm sim vim yog lub siab ua haujlwm tsis zoo lossis lwm yam kab mob metabolic uas tau txais los ntawm noob caj noob ces.
Txawm hais tias qhov tseeb zaus tsis yooj yim los txheeb xyuas vim nws muaj ntau yam ua rau, kev tshawb fawb qhia tias ua ib qho mob tseem ceeb, Urea Cycle Disorders (UCDs) cuam tshuam txog li 1 ntawm 250,000 tus menyuam yug hauv Tebchaws Meskas, thiab txog li 1 ntawm 440,000 tus menyuam yug thoob ntiaj teb.
Dab tsi ua rau muaj hyperammonemia?
Muaj ntau yam ua rau, uas yuav txawv raws li hnub nyoog. Txawm li cas los xij, feem ntau, ob pawg tseem ceeb yog kev ua haujlwm tsis zoo ntawm daim siab thiab Urea Cycle Disorders (UCDs) .
Cov Teeb Meem Cuam Tshuam Nrog Lub Siab
Koj lub siab yog lub cev tseem ceeb uas ua rau koj hloov ammonia mus ua urea. Yog tias koj lub siab puas lawm lossis muaj mob, nws yuav tsis muaj peev xwm ua cov ammonia tau zoo, ua rau nws sib sau ua ke hauv koj cov ntshav.
Cov mob uas ua rau hyperammonemia muaj xws li:
- Kab mob siab : Thaum koj lub siab puas lawm , nws lub peev xwm ua cov ammonia yuav raug cuam tshuam. Txawm hais tias feem ntau tshwm sim rau cov kab mob hnyav, txawm tias cov neeg uas muaj mob siab ntev tuaj yeem muaj ammonia nce siab sai sai vim muaj tej yam xws li kev los ntshav hauv plab lossis electrolyte tsis sib npaug (piv txwv li, cuam tshuam nrog cov ntsev ). Kab mob siab tseem yog qhov ua rau muaj ammonia ntau ntxiv rau cov menyuam yaus thiab cov neeg laus.
- Kab mob hlwb hauv siab: Qhov no tshwm sim thaum lub siab puas ua rau ammonia sib sau ua ke thiab mus txog lub hlwb, ua rau muaj cov tsos mob xws li tsis meej pem, tsis paub qhov twg , thiab txawm tias tsis nco qab. Qhov no tuaj yeem ua rau tuag taus yog tias tsis kho.
- Kab mob cirrhosis ntawm daim siab:Kab mob cirrhosis cuam tshuam nrog kev hloov cov nqaij ntshiv ntawm lub siab noj qab haus huv nrog cov nqaij caws pliav, ua rau lub siab ua haujlwm tsis zoo thiab ua rau muaj hyperammonemia.
- Kev ua tsis tau haujlwm ntawm daim siab sai sai: Qhov no yog kev poob haujlwm ntawm daim siab tam sim ntawd. Piv txwv li, kev raug mob ntawm daim siab los ntawm tshuaj, xws li kev noj acetaminophen ntau dhau, suav txog 50% ntawm cov neeg mob ua tsis tau haujlwm ntawm daim siab sai sai hauv Tebchaws Meskas. Hauv cov menyuam yaus, 64% ntawm cov neeg mob hyperammonemia muaj feem cuam tshuam nrog kev ua tsis tau haujlwm ntawm daim siab sai sai.
- Kev ntshav ntws mus rau koj lub siab tsis txaus: Yog tias lub siab tsis tau txais ntshav txaus, nws tsis tuaj yeem lim thiab ua cov ammonia tau zoo, ua rau muaj kev sib sau ua ke hauv lub cev.
- Reye's syndrome: Qhov no yog ib qho mob tsis tshua muaj, mob hnyav uas ua rau lub siab thiab lub hlwb o. Nws feem ntau ua rau cov ammonia ntau thiab cov ntshav qab zib tsawg. Nws feem ntau pom muaj nyob rau hauv cov menyuam yaus thiab cov hluas uas rov zoo los ntawm kev kis kab mob xws li mob qaib ntxhw lossis mob khaub thuas, tshwj xeeb tshaj yog thaum siv aspirin . Vim yog qhov kev pheej hmoo no, tsis txhob muab aspirin rau cov menyuam yaus lossis cov hluas tshwj tsis yog tus kws kho mob hais kom ua.
Cov Kev Mob Hlwb Urea (UCDs)
Raws li tau hais lawm, lub voj voog urea yog cov txheej txheem ntawm kev hloov cov tshuaj lom ammonia mus rau hauv urea. Cov txheej txheem no muaj ntau kauj ruam, txhua kauj ruam xav tau ib qho enzyme tshwj xeeb, suav nrog:
- N-acetyl-glutamate synthase (NAGS)
- Carbamoyl phosphate synthetase (CPS)
- Ornithine transcarbamylase (OTC)
- Argininosuccinate synthetase (AS)
- Argininosuccinic acid lyase (ASL)
- Arginase (ARG1)
Yog tias muaj cov enzymes no tsis txaus, lub voj voog yuav tsis ua haujlwm, thiab ammonia yuav sib sau ua ke. Cov mob caj ces no, hu ua UCDs, tuaj yeem ua rau muaj hyperammonemia sai thiab ntev.
Cov kab mob UCDs muaj txij thaum yug los. Cov menyuam mos uas muaj qhov tsis txaus enzyme tag nrho feem ntau yuav muaj cov kab mob hyperammonemia sai sai hauv 24 txog 72 teev tom qab yug los. Yog tias qhov tsis txaus yog ib feem lossis tsis hnyav heev, cov tsos mob yuav tshwm sim tom qab hauv lub neej vim muaj mob lossis kev ntxhov siab. Cov kab mob UCDs yog lub luag haujlwm rau 23% ntawm cov menyuam yaus uas muaj mob hnyav heev.
Lwm Yam Ua Rau
Ntxiv rau cov yam uas tau hais los saum toj no, muaj ntau lwm yam ua rau muaj:
- Kev ua tsis tau haujlwm ntawm lub raum (raum): Thaum koj lub raum ua tsis tau haujlwm, lawv yuav tsis tuaj yeem lim cov urea tawm ntawm koj lub cev tau zoo. Qhov no ua rau ammonia sib sau ua ke hauv koj cov ntshav.
- Qee yam kab mob ntshav: Cov mob xws li ntau yam mob myeloma thiab mob leukemia kuj tuaj yeem ua rau muaj hyperammonemia.
- Tej yam kab mob: Cov kab mob uas tshwm sim los ntawm cov kab mob uas tsim cov urease—xws li Proteus mirabilis , Escherichia coli (E. coli) , thiab Klebsiella —tuaj yeem ua rau muaj hyperammonemia hnyav. Qhov no feem ntau tshwm sim rau cov menyuam yaus uas yug los muaj qhov tsis zoo ntawm txoj hlab zis lossis rau cov neeg laus uas raug kev tso zis vim yog UTIs.
Cov tsos mob ntawm hyperammonemia yog dab tsi?
Cov tsos mob yuav txawv ntau nyob ntawm koj lub hnub nyoog thiab qhov hnyav ntawm koj tus mob.
Cov tsos mob me me uas pom ntau rau menyuam yaus thiab cov neeg laus
- Xeev siab thiab ntuav
- Mob plab
- Kev chim siab
- Mob taub hau
- Teeb meem nrog kev sib npaug, kev sib koom tes, lossis kev hais lus (ataxia)
- Kev hloov pauv tus cwj pwm
Cov tsos mob me me ntxiv rau cov menyuam yaus
- Kev ua tsis tiav ntawm kev vam meej
- Cov leeg tsis muaj zog (hypotonia)
- Kev qeeb ntawm kev loj hlob ntawm lub paj hlwb
Cov tsos mob hnyav rau cov menyuam yaus thiab cov neeg laus
Cov no yog cov cim ceeb toom loj heev. Yog tias koj muaj ib qho ntawm cov no, nrhiav kev kho mob tam sim ntawd.
- Kev tsis meej pem thiab kev tsis paub qhov chaw
- Kev hloov pauv ntawm lub siab
- Kev tsaug zog ntau dhau
- Kev hloov pauv hauv kev paub
- Qaug dab peg
- Ua pa ceev heev (ua pa ceev heev)
- Coma
Ceeb Toom: Yog tias koj lossis ib tus neeg koj hlub muaj cov tsos mob no, thov hu rau 911 tam sim ntawd lossis mus rau chav kho mob ceev hauv tsev kho mob ze tshaj plaws. Cov ammonia ntau hauv cov ntshav yog qhov txaus ntshai rau lub neej thiab xav tau kev kho mob sai.
Cov tsos mob ntawm cov menyuam mos
Hauv cov menyuam mos, hyperammonemia feem ntau tshwm sim ntawm 24 thiab 72 teev tom qab yug los:
- Kev chim siab thiab quaj ntau dhau
- Ntuav
- Kev qaug zog
- Qaug dab peg
- Qw nrov thaum ua pa
- Kev ua pa ntau dhau
Tseem Ceeb: Yog tias koj tus menyuam muaj cov tsos mob no thaum koj tseem nyob hauv tsev kho mob, qhia rau koj tus kws kho mob tam sim ntawd. Yog tias koj nyob hauv tsev, hu rau 911 lossis mus rau chav kho mob ceev ze tshaj plaws tam sim ntawd. Tsis txhob ncua sijhawm nrhiav kev kho mob.
Pab neeg kho mob Nirogi Lanka kuaj mob li cas?
Cov kws kho mob feem ntau xav tias muaj hyperammonemia los ntawm kev kuaj xyuas koj cov ntshav ammonia.
- Rau cov menyuam mos txog ib hlis: Cov qib siab dua 80 micromol / L yog qhov txhawj xeeb.
- Rau cov menyuam loj dua: Cov theem siab dua 55 micromol / L yog qhov txhawj xeeb.
- Rau cov neeg laus: Cov theem siab dua 30 micromol / L yog qhov txhawj xeeb.
Micromol/L yog lub ntsuas tus qauv siv los ntsuas qhov ntau ntawm ammonia hauv koj cov ntshav.
Kev kuaj mob
Cov cuab yeej kuaj mob tseem ceeb yog kev kuaj ntshav los ntsuas cov ammonia.
Yog tias pom tseeb tias muaj cov ntshav ntau heev, koj tus kws kho mob yuav hais kom kuaj ntxiv kom paub qhov ua rau muaj qhov teeb meem no, xws li kev kuaj lub siab ua haujlwm , kev kuaj lub raum ua haujlwm , lossis kev kuaj zis .
Vim tias cov tshuaj ammonemia ntau dhau tuaj yeem ua rau lub paj hlwb puas tsuaj thiab ua rau muaj kev phom sij rau txoj sia, koj tus kws kho mob kuj tseem yuav pom zoo kom kuaj duab xws li CT (computed tomography) scan lossis MRI (magnetic resonance imaging) los kuaj xyuas cov teeb meem ntawm lub paj hlwb.
Cov kev kho mob rau hyperammonemia yog dab tsi?
Kev kho mob nyob ntawm seb qhov ua rau muaj teeb meem yog dab tsi—seb nws yog mob hlwb hepatic encephalopathy hauv ib tus neeg laus uas muaj kab mob siab, lossis mob urea cycle disorder (UCD) hauv ib tus menyuam mos.
Thaum muaj cov tshuaj ammonia ntau dhau, lub hom phiaj yog kom txo cov ammonia hauv cov ntshav sai sai thiab tswj cov teeb meem xws li cerebral edema (lub hlwb o) thiab intracranial hypertension.
- Rau cov menyuam mos, cov kws kho mob yuav tsum tsis txhob noj cov protein (vim tias kev zom cov protein ua rau muaj ammonia) thiab muab cov calories los ntawm cov kua glucose. Kev ntxuav ntshav yuav siv tau los ntxuav cov ntshav siv lub tshuab tshwj xeeb thiab lub lim (ua haujlwm zoo li lub raum cuav).
- Rau cov neeg uas muaj ib feem ntawm UCD enzyme tsis txaus los ntawm kev ntxhov siab, kev noj cov protein raug txwv thaum glucose muab kev txhawb nqa calorie. Hemodialysis yog tseg rau cov xwm txheej uas kev kho mob thawj zaug tsis ua rau cov ammonia qis dua hauv ob peb teev.
- Yuav kho tus kab mob hepatic encephalopathy, qhov tseem ceeb yog txo cov ammonia tsim tawm hauv cov hnyuv. Cov kev kho mob thawj zaug suav nrog cov tshuaj noj uas muaj lactulose lossis lactitol , uas pab tiv thaiv lub cev kom tsis txhob nqus ammonia.
Rau cov neeg uas muaj teeb meem ntawm lub voj voog urea ntev, cov kws kho mob yuav sau cov tshuaj noj tshwj xeeb kom tiv thaiv kev rov tshwm sim, suav nrog:
- L-carnitine
- L-ornithine-L-aspartate
- Arginine
Puas tiv thaiv tau qhov mob no?
Vim tias muaj ntau yam ua rau muaj cov tshuaj ammonemia ntau dhau, nws yeej tsis tuaj yeem tiv thaiv tau tas li. Txawm li cas los xij, koj tuaj yeem ua cov kauj ruam los tiv thaiv koj lub siab thiab kev noj qab haus huv tag nrho:
- Tsis txhob haus los yog txwv tsis pub haus cawv.
- Txo kev noj cov rog trans thiab cov kua txiv pob kws uas muaj fructose ntau.
- Ceev faj nrog cov tshuaj uas yuav tau los ntawm kws kho mob thiab cov tshuaj uas yuav tsis tas yuav muaj ntawv yuav tshuaj kom tsis txhob mob siab.
- Ua kom ib ce muaj zog tas li.
- Txwv kev noj nqaij liab.
Yuav kom pab tiv thaiv tau Reye's syndrome rau cov menyuam yaus thiab cov hluas—ib yam mob uas ua rau muaj hyperammonemia —tsis txhob muab aspirin rau cov tsos mob ntawm tus kab mob (xws li kub taub hau lossis mob qhua pias) tsuas yog tus kws kho mob hais kom ua xwb.
Cov kab mob Urea Cycle Disorders (UCDs) tsis tshua muaj, tab sis muaj kev kuaj noob caj noob ces los txiav txim siab seb koj puas muaj cov noob caj noob ces uas ua rau muaj cov mob no. Thov nrog koj tus kws kho mob tham seb qhov kev kuaj mob no puas tsim nyog rau koj.
Qhov kev kwv yees rau tus mob no yog dab tsi?
Qhov kev kwv yees rau hyperammonemia - lossis koj qhov kev cia siab rau kev rov zoo - nyob ntawm ntau yam tseem ceeb:
- Qhov hnyav ntawm koj qhov ammonia nce siab.
- Lub sijhawm uas koj cov ammonia tseem siab.
- Qhov ua rau muaj hyperammonemia yog qhov ua rau muaj mob hnyav.
Feem ntau, qhov kev kwv yees rau hyperammonemia tuaj yeem tiv thaiv tau. Yog tias kev kho mob qeeb lossis tsis muab, qhov o ntawm lub hlwb uas tsis rov qab zoo tuaj yeem ua rau muaj kev tuag ntau. Hmoov tsis zoo, rau cov menyuam mos uas muaj hyperammonemia hnyav, txawm tias kev kho mob sai thiab tam sim ntawd tsis lav qhov tshwm sim zoo.
Hauv Tebchaws Meskas, cov neeg mob uas muaj UCDs uas muaj cov tshuaj ammonemia ntau thaum lawv tseem hluas muaj feem yuav muaj sia nyob tau 11 xyoo li ntawm 35%. Rau cov neeg uas muaj cov tshuaj ammonemia ntau thaum lawv tseem hluas, qhov feem yuav muaj sia nyob no nce mus txog li ntawm 87%.
Rau cov tib neeg uas muaj mob hlwb hepatic encephalopathy hnyav vim yog hyperammonemia, cov feem pua ntawm kev ciaj sia nyob ntawm ib xyoos thiab peb xyoos tau tshaj tawm tias muaj 42% thiab 23%, raws li.
Cov teeb meem uas yuav tshwm sim los ntawm hyperammonemia yog dab tsi?
Yog tias qhov mob no tsis kho sai, cov ammonia ntau dhau tuaj yeem ua rau lub paj hlwb puas tsuaj, ua rau muaj teeb meem loj rau lub neej, suav nrog:
- Cerebral edema (lub hlwb o)
- Mob ntshav siab hauv lub hlwb (ntshav siab ntxiv hauv lub taub hau)
- Kev hernia ntawm lub hlwb - feem ntau yog qhov tshwm sim ntawm lub hlwb o thiab muaj kev ntxhov siab ntau ntxiv.
- Coma
- Kev tuag
Yog tias koj muaj cov tshuaj ammonemia me me uas ua rau koj lub hlwb tsis ua haujlwm zoo, tej zaum koj yuav muaj feem yuav muaj teeb meem kev txawj ntse mus sij hawm ntev thiab muaj teeb meem kev coj cwj pwm lossis kev puas siab puas ntsws mus tas li.
Thaum twg koj yuav tsum mus ntsib kws kho mob?
Yog tias koj lossis ib tus neeg koj hlub muaj cov tsos mob ntawm hyperammonemia, hu rau 911 lossis mus rau chav kho mob ceev ze tshaj plaws tam sim ntawd. Tsis txhob ncua sijhawm nrhiav kev kho mob.
Yog tias koj muaj kab mob siab lossis tsis muaj cov enzymes urea cycle txaus, nws yog ib qho tseem ceeb kom koj mus ntsib koj tus kws kho mob tas li kom tswj tau koj tus mob thiab saib xyuas koj cov ammonia kom zoo.
Hyperammonemia yog ib qho mob hnyav uas muaj ntau yam ua rau. Yog tias koj, koj tus menyuam, lossis tus menyuam mos muaj cov tsos mob, nrhiav kev pab kho mob tam sim ntawd. Yog tias koj muaj kab mob siab lossis keeb kwm tsev neeg ntawm UCDs, tham txog koj tus kheej cov yam ntxwv txaus ntshai nrog koj tus kws kho mob. Nirogi Lanka nyob ntawm no los txhawb koj hauv koj txoj kev noj qab haus huv.
Cov lus kawg uas yuav tsum nco ntsoov
Hauv kev xaus lus, ntawm no yog cov ntsiab lus tseem ceeb tshaj plaws uas yuav tsum nco ntsoov:
- Hyperammonemia tshwm sim thaum cov ntshav ammonia nce mus txog qib txaus ntshai, uas yog tshuaj lom heev rau koj lub hlwb thiab lub paj hlwb.
- Nws muaj ntau yam ua rau muaj, nrog rau kev ua haujlwm tsis zoo ntawm daim siab thiab Urea Cycle Disorders (UCDs) yog cov neeg pab txhawb tseem ceeb.
- Cov tsos mob sib txawv raws li hnub nyoog thiab qhov hnyav, xws li cov teeb meem me me xws li xeev siab, ntuav, thiab mob taub hau mus rau cov tsos mob hnyav xws li tsis meej pem, qaug dab peg, thiab tsis nco qab. Ib txwm ceev faj txog cov tsos mob ntawm cov menyuam mos.
- Yog tias muaj cov tsos mob hnyav, nrhiav kev kho mob tam sim ntawd. Qhov no tuaj yeem ua rau tuag taus.
- Kev kho mob yog tsim los rau qhov ua rau muaj teeb meem. Kev kho sai yog tsom mus rau kev txo cov ammonia thiab tswj cov teeb meem.
- Txawm hais tias qee yam ua rau peb tsis tuaj yeem tiv thaiv tau los xij, kev ua neej kom noj qab haus huv thiab saib xyuas koj lub siab kom zoo yuav pab txo qee yam kev pheej hmoo.
- Yog tias koj muaj kab mob siab lossis muaj keeb kwm tsev neeg ntawm UCDs, kev kuaj mob tsis tu ncua yog qhov tseem ceeb.
Nco ntsoov, koj paub koj txoj kev noj qab haus huv zoo tshaj plaws. Yog tias koj pom muaj kev hloov pauv lossis cov tsos mob txawv txawv, tsis txhob yig mus ntsib kws kho mob. Peb nyob ntawm no los pab.
Hyperammonemia, ammonia, siab, urea voj voog, lub hlwb, cov tsos mob, kev kho mob
