Koj tus me nyuam puas nkees tom qab ua si ib pliag? Los yog nws puas zoo li mob tas li, tsis xav noj mov, lossis tsaug zog? Qee zaum peb xav tias cov no yog tej yam ib txwm muaj, tab sis tom qab qhov no yuav muaj ib qho mob caj ces uas tsis tshua muaj, tab sis loj heev. Niaj hnub no peb tab tom tham txog ib qho mob zoo li no, Long-Chain Fatty Acid Oxidation Disorders, lossis LC-FAODs .
Hais yooj yim xwb, cov LC-FAODs no yog dab tsi?
Qhov no yog lub npe nyuaj me ntsis, tab sis cia peb ua kom yooj yim. Peb lub cev zoo li lub tsheb. Nws xav tau lub zog, lossis roj, kom ua haujlwm. Cov khoom noj uas peb noj yog roj rau peb lub cev. Cov txheej txheem uas cov khoom noj no hloov mus ua lub zog hu ua metabolism .
Cov khoom noj uas peb noj, xws li roj txiv roj , ntses , txiv avocados, thiab nqaij, muaj cov khoom hu ua 'fatty acids'. Cov no yog cov khoom noj zoo rau lub cev. Muaj ntau hom fatty acids no. Ntawm lawv, hom hu ua 'long-chain fatty acids' xav tau ib qho enzyme tshwj xeeb los hloov lawv mus ua lub zog.
Cov menyuam yaus uas yug los muaj ib yam mob caj ces hu ua LC-FAODs tsis muaj cov enzyme tshwj xeeb ntawd hauv lawv lub cev. Yog li ntawd, lawv lub cev tsis tuaj yeem hloov cov roj fatty acids no mus ua zog.
Tom qab ntawd muaj dab tsi tshwm sim?
1. Cov kab mob tseem ceeb xws li lub plawv , lub hlwb, lub siab , thiab cov leeg nqaij tsis tau txais lub zog tsim nyog.
2. Cov roj fatty acid uas tsis tuaj yeem hloov mus ua zog tau sib sau ua ke hauv cov kab mob ntawd thiab pib ua rau lawv puas tsuaj.
Qhov no tuaj yeem ua rau muaj teeb meem loj xws li kab mob plawv , daim siab tsis ua haujlwm , thiab cov ntshav qab zib qis heev. Yog li ntawd, nws yog ib qho tseem ceeb heev uas yuav tsum kuaj mob no thaum ntxov thiab tswj kev noj zaub mov kom zoo .
Cov tsos mob ntawm tus kab mob no yog dab tsi?
Cov tsos mob yuav txawv ntawm ib tus neeg mus rau ib tus neeg. Nws nyob ntawm hom enzyme uas ploj lawm thiab qhov hnyav ntawm tus kab mob. Ib txhia neeg yuav tsis pom cov tsos mob txog thaum lawv tseem hluas lossis laus. Tab sis thaum muaj mob hnyav, cov tsos mob yuav pib tshwm sim hauv ob peb lub hlis thawj zaug ntawm lub neej.
Cov cim qhia thawj zaug ntawm tus kab mob plawv hauv cov menyuam mos yog "cardiomyopathy." Qhov no suav nrog ua pa nyuaj, noj zaub mov nyuaj, thiab tsaug zog ntau dhau. Cov menyuam mos thiab cov menyuam yaus me yuav pom cov cim qhia thaum ntxov ntawm teeb meem siab, xws li daj ntawm lub qhov muag thiab daim tawv nqaij (jaundice), thiab cov ntshav qab zib qis (hypoglycemia).
Cia peb los saib cov mob tseem ceeb uas tuaj yeem tshwm sim hauv qhov no thiab cov tsos mob cuam tshuam nrog lawv yog dab tsi.
| Xwm txheej | Cov tsos mob feem ntau |
|---|---|
| Cov yam ntxwv dav dav |
|
| Ntshav Qab Zib Qis (Hypoglycemia) | |
| Cov ammonia ntau ntxiv hauv cov ntshav (Hyperammonemia) | |
| Kab mob plawv |
Qhov tseem ceeb yog tias rau qee tus menyuam yaus, cov tsos mob no tsuas yog tshwm sim thaum lawv mob, tshaib plab, lossis ntxhov siab.
Dab tsi ua rau muaj tus kab mob no?
Qhov no yog ib yam kab mob uas muaj los ntawm noob caj noob ces . Yog li ntawd, nws yog los ntawm niam txiv mus rau menyuam. Hais yooj yim xwb, qhov ua rau yog kev hloov pauv hauv cov noob caj noob ces uas qhia kom tsim cov enzyme uas rhuav cov roj fatty acids uas peb tau tham txog.
Yog ib tug me nyuam muaj tus kab mob no, lawv yuav tsum tau txais ib daim qauv ntawm cov noob caj noob ces uas tsis zoo los ntawm ob niam txiv . Yog tias lawv tau txais los ntawm ib tug niam txiv xwb, tus me nyuam yog tus "neeg nqa kab mob." Lawv yuav tsis muaj tsos mob, tab sis lawv tuaj yeem xa cov noob caj noob ces mus rau lawv cov me nyuam.
Qhov no tsis yog ib yam kab mob sib kis tau. Nws tsis tuaj yeem kis tau ntawm ib tus neeg mus rau lwm tus li cas los xij.
Yuav kuaj mob li cas?
Hauv ntau lub teb chaws, txhua tus menyuam mos uas yug los yuav raug kuaj seb puas muaj cov kab mob caj ces no. Qhov no yog ua los ntawm kev coj cov ntshav me me los ntawm pob taws hauv 1-2 hnub tom qab yug los. Yog tias qhov kev kuaj no qhia txog tus kab mob, yuav muaj kev kuaj ntxiv kom paub meej tias muaj tus kab mob.
Cov kev ntsuam xyuas tseem ceeb uas tau ua rau qhov no yog:
- Kev kuaj ntshav thiab zis: Cov no kuaj seb puas muaj cov roj fatty acids thiab lwm yam tshuaj ntau dhau hauv cov ntshav thiab zis.
- Kev kuaj noob caj noob ces: Qhov no yog tib txoj kev los txiav txim siab seb koj puas muaj LC-FAODS thiab, yog tias muaj, hom twg.
Yog tias koj tus menyuam muaj cov tsos mob uas peb tau tham ua ntej, nws yog ib qho tseem ceeb heev uas yuav tsum mus ntsib kws kho mob menyuam yaus tam sim ntawd los tham txog qhov no.
Yuav kho li cas?
Txawm hais tias tus kab mob no tsis tuaj yeem kho tau tag nrho los xij, yog tias tswj tau zoo , cov teeb meem loj tuaj yeem zam tau thiab ua neej nyob li qub. Cov kev kho mob tseem ceeb suav nrog kev hloov pauv zaub mov, cov tshuaj noj tshwj xeeb, thiab kev hloov pauv lub neej.
1. Kev Noj Haus
Qhov no yog qhov tseem ceeb tshaj plaws. Cov zaub mov no yuav tsum tau npaj raws li cov lus qhia ntawm tus kws noj haus.
- Txwv cov khoom noj uas muaj cov roj fatty acids ntau: Cov khoom noj xws li nqaij, qee cov roj zaub, txiv ntoo qhuav, thiab ntses yuav tsum txwv.
- Noj cov khoom noj uas muaj carbohydrate ntau ntxiv: Yuav kom tau txais lub zog, koj yuav tsum suav nrog cov khoom noj uas muaj carbohydrate ntau xws li mov, qos yaj ywm, thiab qos liab qab zib rau hauv koj cov zaub mov noj.
- Noj cov pluas noj me me, tsis tu ncua: Nws yog ib qho tseem ceeb uas yuav tsum noj zaub mov tsis tu ncua thoob plaws hnub kom cov ntshav qab zib ruaj khov.
- Tsis txhob yoo mov: Nws tsis zoo kom tshaib plab ntau tshaj 8 teev.
Yog tias koj tus menyuam cov ntshav qab zib poob qis dhau, lawv yuav tsum mus rau tsev kho mob thiab muab cov kua qab zib (IV) hauv cov kua ntsev uas muab rau hauv cov hlab ntsha hauv Chav Kho Mob Thaum Muaj Xwm Ceev (ETU).
2. Cov tshuaj noj qab haus huv
Vim tias cov neeg mob no siv tsis tau cov roj fatty acids uas muaj cov saw ntev, lawv thiaj li tau txais lwm hom roj uas lub cev hloov tau yooj yim mus ua zog. Cov no hu ua medium-chain triglycerides (MCTs) . Cov no muaj ua roj MCT. Cov MCTs no kuj tseem ntxiv rau qee cov mis mos rau menyuam yaus. Koj tus kws kho mob yuav muab cov ntaub ntawv ntxiv rau koj txog qhov no.
3. Tshuaj Kho Mob
Ib hom tshuaj hu ua Triheptanoin (Dojolvi) tau txais kev pom zoo rau LC-FAODs. Nws tsuas yog muaj nrog koj tus kws kho mob daim ntawv qhia xwb .
4. Kev hloov pauv txoj kev ua neej
Nws yog ib qho tseem ceeb kom tsis txhob ua rau cov tsos mob hnyav dua.
- Tsis txhob ua si nawv ntau dhau: Txwv cov dej num uas ua rau lub cev nkees dhau.
- Tswj kev ntxhov siab: Tej yam xws li yoga thiab kev xav yuav pab tau.
- Tiv thaiv koj tus kheej kom tsis txhob muaj mob: Mus txhaj tshuaj tiv thaiv kab mob raws sijhawm. Nrhiav kev kho mob sai sai txawm tias yog mob khaub thuas me me lossis kub taub hau los xij.
Puas yog kev nyob nrog tus kab mob no yog ib qho kev cov nyom?
Tau kawg, yog lawm. Qhov no yog ib qho mob uas yuav tsum tau tswj hwm mus ib txhis. Koj yuav tsum ceev faj txog koj cov zaub mov noj. Koj yuav tsum paub tias yuav ua li cas thaum muaj xwm txheej ceev. Qhov no tuaj yeem ua rau lub siab ntxhov siab rau tus menyuam thiab niam txiv.
Yog li ntawd,
- Kawm txog tus kab mob kom zoo: kev paub yog lub hwj chim loj tshaj plaws.
- Nrhiav kev pab txhawb nqa kev puas siab puas ntsws: Tsis txhob yig nrhiav kev pab los ntawm tsev neeg, phooj ywg, lossis tus kws pab tswv yim kev noj qab haus huv ntawm lub hlwb.
- Sib txuas lus nrog koj tus kws kho mob: Nug nws yog tias koj muaj lus nug lossis kev txhawj xeeb.
Txawm hais tias tus kab mob no tsis tshua muaj, yog tias tswj thiab saib xyuas zoo, tus menyuam tuaj yeem muaj lub neej zoo. Qhov tseem ceeb tshaj plaws yog kuaj mob thaum ntxov thiab ua raws li tus kws kho mob cov lus qhia.
Cov Lus Qhia Txog Kev Coj Mus Tsev
- LC-FAODs yog ib qho mob hnyav uas tau txais los ntawm noob caj noob ces uas tiv thaiv lub cev los ntawm kev hloov qee hom rog mus ua lub zog.
- Tej zaum yuav muaj cov tsos mob xws li qaug zog heev, cov leeg nqaij tsis muaj zog, teeb meem plawv thiab siab, thiab cov ntshav qab zib tsawg.
- Qhov no tsis yog ib yam kab mob sib kis tau. Nws yog ib yam uas tau txais los ntawm niam txiv mus rau cov menyuam.
- Cov khoom tseem ceeb ntawm kev kho mob yog kev noj zaub mov tshwj xeeb, cov tshuaj ntxiv xws li roj MCT, thiab zam cov khoom uas ua rau cov tsos mob hnyav dua.
- Yog koj tus menyuam muaj ib qho ntawm cov tsos mob no, mus ntsib koj tus kws kho mob tam sim ntawd kom tau txais kev qhia. Kev kuaj mob thaum ntxov yog qhov tseem ceeb heev.
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Tsis muaj lus teb tau muab tso tawm. Ntxiv koj cov lus tawm ntawm no thawj zaug.
Ntxiv koj cov lus