Koj puas muaj ammonia ntau dhau hauv koj cov ntshav? Cia peb kawm txog qhov xwm txheej txaus ntshai ntawm hyperammonemia!

Hyperammonemia yog dab tsi?

Leej twg tuaj yeem txhim kho qhov no tau zoo tshaj plaws?

• Ntawm cov neeg laus, kwv yees li 90% ntawm cov neeg uas raug kev txom nyem los ntawm tus mob no yog cov neeg uas muaj cirrhosis ntawm lub siab .
• Cov menyuam mos uas yug los muaj qhov teeb meem no vim lawv yug los muaj qhov tsis zoo ntawm lub voj voog urea (congenital urea cycle disorder) . Cov no yog cov mob caj ces.
• Tsis tas li ntawd xwb, kev ua haujlwm tsis zoo ntawm daim siab thiab cov kab mob metabolic uas tau txais los ntawm noob caj ces tuaj yeem ua rau muaj hyperammonemia hauv cov menyuam yaus.

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Dab tsi yog cov ua rau muaj hyperammonemia?

Vim muaj teeb meem siab

• Kab mob siab : Thaum lub siab puas lawm , nws lub peev xwm los ua cov ammonia tsawg. Qhov no feem ntau tshwm sim hauv cov kab mob siab hnyav, tab sis txawm tias cov neeg uas muaj kab mob siab ruaj khov, qee zaum cov ammonia tuaj yeem nce siab sai sai, piv txwv li, yog tias muaj qee yam tshwm sim hauv lub plab lossis txoj hnyuv ( kev los ntshav hauv plab) lossis yog tias muaj kev tsis sib npaug ntawm cov electrolyte . Qhov no yog qhov ua rau muaj cov ammonia ntau hauv cov neeg laus thiab cov menyuam yaus.
• Kab mob hlwb hauv siab: Qhov no tshwm sim thaum lub siab puas lawm thiab tsis tuaj yeem ua cov ammonia kom zoo. Ammonia sib sau ua ke hauv cov ntshav thiab mus rau lub hlwb. Qhov no tuaj yeem ua rau tsis meej pem, tsis paub qhov chaw , thiab txawm tias tsis nco qab. Qee zaum nws tuaj yeem ua rau tuag taus.
• Kab mob siab cirrhosis: Kab mob siab cirrhosis yog qhov hloov cov nqaij ntawm daim siab noj qab haus huv nrog cov nqaij caws pliav. Qhov no tuaj yeem ua rau daim siab tsis ua haujlwm zoo thiab tuaj yeem ua rau muaj hyperammonemia. Kab mob siab cirrhosis yog ib qho kab mob siab ntev.
• Kev ua tsis tau zoo ntawm daim siab sai sai: Qhov no yog thaum lub siab tsis ua haujlwm tam sim ntawd. Nws tuaj yeem tshwm sim los ntawm ntau yam kab mob thiab tej yam mob. Piv txwv li, kev raug mob ntawm daim siab los ntawm tshuaj uas ua rau muaj qee yam tshuaj, xws li acetaminophen , suav txog 50% ntawm tag nrho cov neeg mob uas muaj kev ua tsis tau zoo ntawm daim siab sai sai hauv Tebchaws Meskas. Nws suav txog 64% ntawm cov menyuam yaus uas muaj cov kua qaub ntau dhau hauv cov ntshav.
• Cov ntshav ntws mus rau koj lub siab tsis zoo: Yog tias cov ntshav tsis ntws mus rau koj lub siab kom zoo, lub cev tsis tuaj yeem xa ammonia mus rau lub siab kom ua tiav. Txawm tias yog li ntawd los, ammonia tseem sib sau ua ke hauv cov ntshav.
• Reye's syndrome:Qhov no yog ib qho mob tsis tshua muaj. Nws cuam tshuam rau cov ntshav, lub hlwb, thiab daim siab. Feem ntau nws ua rau cov ntshav ammonia ntau ntxiv thiab cov ntshav qab zib qis. Feem ntau nws cuam tshuam rau cov menyuam yaus thiab cov hluas uas tab tom rov zoo los ntawm cov kab mob xws li mob qaib ntxhw lossis mob khaub thuas/mob ntsws thiab cov uas tau noj tshuaj aspirin rau cov tsos mob ntawd. Tsis paub meej tias yog vim li cas thiaj ua rau muaj tus mob Reye's syndrome. Txawm li cas los xij, vim muaj kev pheej hmoo no, yuav tsum tsis txhob muab tshuaj aspirin rau cov menyuam yaus thiab cov hluas tshwj tsis yog tus kws kho mob pom zoo.

Vim yog Urea Cycle Disorders (UCDs)

• N-acetyl-glutamate synthase (NAGS)
• Carbamoyl phosphate synthetase (CPS)
• Ornithine transcarbamylase (OTC)
• Argininosuccinate synthetase (AS)
• Argininosuccinic acid lyase (ASL)
• Arginase (ARG1)

Lwm yam laj thawj

• Kev ua tsis tau zoo ntawm lub raum ): Yog tias lub raum tsis tuaj yeem tso cov urea tawm kom raug vim yog lub raum tsis ua haujlwm, nws tuaj yeem ua rau ammonia sib sau ua ke hauv cov ntshav.
• Qee yam kab mob ntshav: Hyperammonemia kuj tseem tuaj yeem tshwm sim los ntawm cov kab mob ntshav xws li ntau yam myeloma thiab mob leukemia mob hnyav.
• Qee yam kab mob: cov kab mob uas tsim cov urease xws li Proteus mirabilis , Escherichia coli (E. coli) thiab KlebsiellaCov kab mob bacterial xws li streptococci, tshwj xeeb tshaj yog rau cov menyuam yaus uas muaj qhov tsis zoo ntawm txoj hlab zis txij thaum yug los thiab rau cov neeg laus uas muaj kev tso zis ntau dhau vim yog kab mob ntawm txoj hlab zis (UTIs), tuaj yeem ua rau muaj hyperammonemia hnyav.

Cov tsos mob ntawm hyperammonemia yog dab tsi?

Cov tsos mob me me uas feem ntau tshwm sim rau menyuam yaus thiab cov neeg laus

• Xeev siab thiab ntuav
• Mob plab
• Kev chim siab ntau zaus
• Mob taub hau (mob taub hau)
• Teeb meem nrog kev sib npaug, kev sib koom tes, thiab kev hais lus (ataxia)
• Kev hloov pauv tus cwj pwm

Cov tsos mob me me ntxiv uas tsuas yog cuam tshuam rau menyuam yaus xwb

• 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

• 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 sai (Hyperventilation)
• Coma

Ceeb Toom: Yog tias koj lossis ib tus neeg ze koj muaj cov tsos mob no, thov hu rau 911 tam sim ntawd, lossis mus rau chav kho mob ceev ntawm koj lub tsev kho mob ze tshaj plaws. Cov ntshav ammonia nce siab yog qhov txaus ntshai rau lub neej thiab xav tau kev kho mob tam sim ntawd!

Cov tsos mob ntawm cov menyuam mos tshiab

• Kev chim siab ntau zaus, quaj ntau zaus
• Ntuav
• Kev qaug zog
• Qaug dab peg
• Qw nrov thaum ua pa
• Ua pa sai (Hyperventilation)

Tseem Ceeb: Yog tias koj tus menyuam muaj cov tsos mob no, hu rau kws kho mob tam sim ntawd yog tias koj tseem nyob hauv tsev kho mob. Yog tias koj nyob hauv tsev, hu 911 tam sim ntawd, lossis coj lawv mus rau tsev kho mob ze tshaj plaws. Qhov no tsis yog ib yam dab tsi uas yuav tsum ncua sijhawm.

Cov kws kho mob kuaj mob li cas ?

• Rau cov menyuam mos uas muaj hnub nyoog txog ib hlis, yog tias cov ammonia hauv cov ntshav ntau dua 80 micromol/liter (80 micromol/L).
• Rau cov menyuam loj dua, yog tias nws ntau dua 55 micromol/liter (55 micromol/L).
• Hauv cov neeg laus, yog tias nws ntau dua 30 micromol/liter (30 micromol/L).

Yuav ua li cas thiaj kuaj tau qhov no?

Cov kev kho mob rau hyperammonemia yog dab tsi?

• Yog tias ib tug menyuam mos muaj hyperammonemia, cov kws kho mob yuav tsum tsis txhob muab protein rau lawv (vim tias ammonia raug tsim tawm thaum cov protein raug zom) thiab hloov chaw muab glucose rau lawv, uas yog cov kua qab zib, los muab calories. Lawv kuj tseem yuav siv cov txheej txheem hu ua hemodialysis . Qhov no suav nrog kev ntxuav cov ntshav ntawm tus menyuam thiab tshem tawm ammonia los ntawm lub tshuab dialysis thiab lub lim tshwj xeeb (zoo li lub raum cuav).
• Cov neeg uas muaj qhov tsis txaus ntawm cov enzyme urea cycle (UCD) uas muaj cov kua qaub ntau dhau vim yog kev ntxhov siab, xws li kev ntxhov siab ua rau muaj cov kua qaub ntau dhau, kuj tau txais cov protein withdrawal thiab glucose ua calories. Kev ntxuav ntshav tsuas yog ua yog tias lawv cov ammonia tsis txo qis hauv ob peb teev ntawm kev kho mob thawj zaug.
• Kev kho mob rau tus kab mob hepatic encephalopathy yog kom txo qhov tsim cov ammonia hauv cov hnyuv. Kev kho mob thawj kab yog cov tshuaj noj uas muaj lactulose thiab lactitol . Cov suab thaj no txo ​​qhov tsim thiab nqus cov ammonia hauv cov hnyuv.

• L-carnitine
• L-ornithine-L-aspartate
• Arginine

Puas muaj txoj hauv kev los zam qhov xwm txheej no?

• Tsis ua li cas yog tias koj tsis haus los yog tsis haus cawv.
• Tsis txhob noj cov khoom noj thiab dej haus uas muaj cov roj trans fat lossis cov kua txiv pob kws uas muaj fructose ntau.
• Yuav kom tsis txhob muaj kev puas tsuaj rau daim siab, ceev faj heev thaum siv cov tshuaj uas kws kho mob sau ntawv kom yuav thiab cov tshuaj uas yuav tsis tas yuav muaj ntawv qhia.
• Ua si nawv tsis tu ncua .
• Txwv kev noj nqaij liab.

Qhov kev kwv yees rau tus mob no yog dab tsi?

• Koj cov ammonia ntau npaum li cas.
• Cov tshuaj ammonia tau nce siab ntev npaum li cas.
• Dab tsi ua rau muaj hyperammonemia?

Cov teeb meem uas yuav tshwm sim los ntawm hyperammonemia yog dab tsi?

• Kev mob o ntawm lub hlwb
• Muaj kev ntxhov siab ntxiv nyob ib puag ncig lub hlwb (Intracranial hypertension)
• Kev mob hlwb hernia - Qhov no feem ntau yog tshwm sim los ntawm o ntawm lub hlwb thiab kev ntxhov siab ntxiv nyob ib puag ncig lub hlwb.
• Coma
• Kev tuag

Thaum twg peb yuav tsum mus ntsib kws kho mob?

Hyperammonemia yog ib qho mob hnyav uas tuaj yeem tshwm sim los ntawm ntau yam. Yog tias koj, koj tus menyuam, lossis koj tus menyuam muaj cov tsos mob no, nrhiav kev pab tam sim ntawd. Yog tias koj muaj kab mob siab, lossis yog tias ib tus neeg hauv koj tsev neeg muaj teeb meem urea cycle (UCDs), tham nrog koj tus kws kho mob txog koj qhov kev pheej hmoo ntawm kev muaj hyperammonemia. Lawv nyob ntawd los pab koj.

Thaum kawg, nco ntsoov qhov no.

• Hyperammonemia yog ib qho teeb meem loj heev uas muaj ammonia ntau heev hauv cov ntshav. Qhov no ua rau peb lub hlwb thiab lub paj hlwb puas tsuaj heev.
• Muaj ntau yam ua rau qhov no. Cov teeb meem siab thiab cov kab mob urea cycle (UCDs) yog cov ua rau tseem ceeb.
• Cov tsos mob yuav txawv nyob ntawm seb qhov mob hnyav npaum li cas thiab hnub nyoog li cas. Lawv tuaj yeem yog cov tsos mob me me xws li xeev siab, ntuav, thiab mob taub hau mus rau cov tsos mob hnyav dua xws li tsis meej pem, qaug dab peg, thiab tsis nco qab. Cov menyuam mos yuav tsum txhawj xeeb txog lawv cov tsos mob.
• Yog koj muaj cov tsos mob hnyav, koj yuav tsum nrhiav kev pab kho mob sai li sai tau. Qhov no tuaj yeem ua rau koj tuag taus.
• Kev kho mob nyob ntawm seb ua rau muaj li cas. Kev kho mob sai sai tuaj yeem txo cov ammonia thiab tswj tau cov teeb meem.
• Txawm hais tias qee yam ua rau peb tsis tuaj yeem tiv thaiv tau los xij, qee yam kev pheej hmoo tuaj yeem txo tau los ntawm kev ua neej noj qab haus huv thiab saib xyuas koj lub siab .
• Txawm koj muaj kab mob siab lossis ib tug neeg hauv koj tsev neeg muaj UCDs, nws yog ib qho tseem ceeb uas yuav tsum tau mus kuaj mob tsis tu ncua.