Ngaba umntwana wakho uyadinwa emva kokudlala okwethutyana? Okanye uhlala ebonakala egula, engafuni kutya, okanye elele? Ngamanye amaxesha sicinga ukuba ezi zizinto eziqhelekileyo, kodwa emva koku kunokubakho imeko engaqhelekanga kakhulu, kodwa enzulu kakhulu yemfuza . Namhlanje sithetha ngesinye sezifo ezinjalo, iLong-Chain Fatty Acid Oxidation Disorders, okanye ii-LC-FAODs .
Ngamafutshane, zithini ezi LC-FAODs?
Eli ligama elintsonkothileyo, kodwa masiligcine lilula. Umzimba wethu ufana nesithuthi. Udinga amandla, okanye ipetroli, ukuze usebenze. Ukutya esikutyayo kumafutha emizimbeni yethu. Inkqubo apho oku kutya kuguqulwa kube ngamandla ibizwa ngokuba yimetabolism .
Ukutya esikutyayo, okufana neoyile yomnquma , intlanzi , iiavokhado, kunye nenyama, kuqulethe into ebizwa ngokuba yi-'fatty acids'. Ezi zingumthombo olungileyo wamandla emzimbeni. Kukho iintlobo ezahlukeneyo zezi fatty acids. Phakathi kwazo, uhlobo olubizwa ngokuba yi-'long-chain fatty acids' lufuna i-enzyme ekhethekileyo ukuze luziguqule zibe ngamandla.
Abantwana abazelwe benesimo semfuza esibizwa ngokuba yi-LC-FAODs abanayo loo enzyme ithile emizimbeni yabo. Ngoko ke imizimba yabo ayinakuguqula ezi asidi zamafutha ezinde zibe ngamandla.
Kuza kwenzeka ntoni ke ngoko?
1. Amalungu abalulekileyo njengentliziyo, ingqondo , isibindi kunye nemisipha awafumani amandla afunekayo.
2. Ii-fatty acids ezingenakuguqulwa zibe ngamandla ziyaqokelelana kwezo zitho zize ziqale ukuzonakalisa.
Oku kunokukhokelela kwiingxaki ezinkulu ezifana nesifo sentliziyo , ukungasebenzi kakuhle kwesibindi , kunye namanqanaba aphantsi kakhulu eswekile egazini . Ke ngoko, kubaluleke kakhulu ukufumanisa esi sifo kwangethuba kunye nokulawula ukutya ngendlela efanelekileyo .
Zithini iimpawu zesi sifo?
Iimpawu zingahluka kumntu nomntu. Kuxhomekeke kuhlobo lwe-enzyme engekhoyo kunye nobunzima besifo. Abanye abantu basenokungabonakalisi zimpawu de babe sebatsha okanye babe badala. Kodwa kwiimeko ezinzima, iimpawu ziqala ukubonakala kwiinyanga ezimbalwa zokuqala zobomi.
Iimpawu zokuqala zesifo semisipha yentliziyo kwiintsana ezisandul’ ukuzalwa yi-"cardiomyopathy". Oku kuquka ubunzima bokuphefumla, ubunzima bokutya, kunye nokulala kakhulu. Iintsana kunye nabantwana abancinci banokubonisa iimpawu zokuqala zeengxaki zesibindi, ezifana nokutyheli kwamehlo nolusu (i-jaundice), kunye namanqanaba aphantsi eswekile egazini (i-hypoglycemia).
Makhe sijonge iimeko eziphambili ezinokubakho kule meko kwaye zeziphi iimpawu ezinxulumene nazo.
| Imeko | Iimpawu eziqhelekileyo |
|---|---|
| Iimpawu ngokubanzi |
|
| Iswekile ephantsi egazini (iHypoglycemia) | |
| Ukwanda kwamanqanaba e-ammonia egazini (i-Hyperammonemia) | |
| I-Cardiomyopathy |
Into ebalulekileyo kukuba kwabanye abantwana, ezi mpawu zibonakala kuphela xa begula, belambile, okanye bexinezelekile.
Yintoni ebangela esi sifo?
Esi sisifo esibangelwa yimfuza ngokupheleleyo. Oko kukuthi, sifunyanwa ngabazali ukuya ebantwaneni. Ngokufutshane nje, unobangela kukuguquka kwejini okufundisa ukuveliswa kwe-enzyme eqhekeza ii-fatty acids esithethe ngazo.
Ukuze umntwana abe nesi sifo, kufuneka afumane ikopi yejini enesiphene kubazali bobabini . Ukuba bayizuza kumzali omnye kuphela, umntwana "ungumthwali". Abayi kuba nazimpawu, kodwa banokudlulisela ijini ebantwaneni babo.
Esi asisosifo sisulelayo. Asinakudluliselwa komnye umntu siye komnye nangayiphi na indlela.
Indlela yokuxilonga esi sifo?
Kumazwe amaninzi, lonke usana olusandul’ ukuzalwa luyahlolwa ezi zifo zofuzo. Oku kwenziwa ngesampulu yegazi elincinci elithathwe esithendeni kwiintsuku ezi-1-2 emva kokuzalwa. Ukuba olu vavanyo lubonisa ukuba isifo sikhona, kwenziwa olunye uvavanyo ukuqinisekisa ukuba sikhona.
Iimvavanyo eziphambili ezenziweyo koku zezi:
- Uvavanyo lwegazi nomchamo: Olu vavanyo lujonga amanqanaba aphezulu ngokungaqhelekanga e-fatty acids kunye nezinye izinto egazini nasemchameni.
- Uvavanyo lwemfuza: Le yindlela ekuphela kwayo yokufumanisa ngokuqinisekileyo ukuba une-LC-FAODS kwaye, ukuba kunjalo, loluphi uhlobo.
Ukuba umntwana wakho uneempawu esizikhankanyileyo ngaphambili, kubaluleke kakhulu ukubonana nogqirha wabantwana ngokukhawuleza ukuze kuxoxwe ngale nto.
Iphathwa njani?
Nangona esi sifo singenakunyangwa ngokupheleleyo, ngokulawulwa ngokufanelekileyo , iingxaki ezinkulu zinokuphetshwa kwaye ubomi obuqhelekileyo bunokuphila. Unyango oluphambili luquka utshintsho kwindlela yokutya, izongezo zesondlo ezikhethekileyo, kunye notshintsho kwindlela yokuphila.
1. Ukutya
Le yeyona nto ibalulekileyo. Olu hlobo lokutya lufanele lulungiswe phantsi kwengcebiso yengcali yezokutya.
- Nciphisa ukutya okune-fatty acids eninzi: Ukutya okufana nenyama, iioyile zemifuno, amandongomane kunye nentlanzi kufuneka kuncitshiswe.
- Yitya ukutya okuninzi okune-carbohydrate: Ukuze ufumane amandla, kufuneka ufake ukutya okune-carbohydrates ezifana nerayisi, iitapile, kunye neebhatata ekutyeni kwakho.
- Yitya ukutya okuncinci rhoqo: Kubalulekile ukutya ngamaxesha aqhelekileyo emini ukuze kugcinwe amanqanaba eswekile egazini ezinzile.
- Kuphephe ukuzila ukutya: Akulunganga ukulamba ngaphezu kweeyure ezisi-8.
Ukuba iswekile yegazi lomntwana wakho yehla ngequbuliso kakhulu, kunokufuneka aye esibhedlele aze anikwe isisombululo seswekile (IV) kwisisombululo setyuwa esinikwa ngemithambo kwiYunithi yoNyango oluNgxamisekileyo (ETU).
2. Izongezo zesondlo
Ngenxa yokuba aba baguli bengenakusebenzisa ii-fatty acids ezinde, banikwa olunye uhlobo lwamafutha umzimba onokuluguqula lube ngamandla. Ezi zibizwa ngokuba yi -medium-chain triglycerides (MCTs) . Ezi zifumaneka njengeoyile ye-MCT. Ezi MCT zongezwa nakwezinye iifomyula zeentsana. Ugqirha wakho uza kukunika ulwazi oluthe kratya malunga noku.
3. Amayeza
Iyeza elibizwa ngokuba yiTriheptanoin (Dojolvi) livunyiwe kwi-LC-FAODs. Lifumaneka kuphela ngomyalelo kagqirha wakho .
4. Utshintsho kwindlela yokuphila
Kubalulekile ukunqanda izinto ezibangela ukuba iimpawu zibe mandundu.
- Kuphephe ukuzilolonga ngokugqithisileyo: Nciphisa imisebenzi ebangela uxinzelelo olukhulu emzimbeni.
- Lawula uxinzelelo: Izinto ezifana neyoga kunye nokucamngca zinokuba luncedo.
- Zikhusele kwizifo: Fumana zonke izitofu ezicetyiswa ngugqirha wakho ngexesha. Funa unyango ngokukhawuleza nokuba umkhuhlane okanye umkhuhlane omncinci.
Ngaba ukuphila nesi sifo kuyingxaki?
Ewe, ewe. Le yimeko yobomi bonke ekufuneka ilawulwe. Kufuneka uqaphele ukutya kwakho. Kufuneka wazi ukuba wenze ntoni xa kukho imeko engxamisekileyo. Oku kunokuba yingozi engqondweni yomntwana nakubazali.
Ngoko ke,
- Funda kakuhle ngesi sifo: ulwazi lulona gunya likhulu.
- Funa inkxaso yengqondo: Musa ukuthandabuza ukufuna uncedo kusapho, kubahlobo, okanye kumcebisi wezempilo yengqondo.
- Hlala unxibelelana nogqirha wakho: Mbuze ukuba unemibuzo okanye izinto ezikuxhalabisayo.
Nangona esi sifo singaqhelekanga, ngokuphathwa kakuhle nokunyanyekelwa, umntwana unokuba nobomi obuhle. Eyona nto ibalulekileyo kukufumanisa isifo kwangethuba nokulandela imiyalelo kagqirha.
Umyalezo Wokuya Ekhaya
- I-LC-FAODs yimeko enzima yemfuza eyenzeka ebantwaneni nakubantu abadala, kwaye ibangelwa kukungakwazi komzimba ukuguqula iintlobo ezithile zamafutha zibe ngamandla.
- Iimpawu ezinje ngokudinwa kakhulu, ubuthathaka bemisipha, iingxaki zentliziyo nesibindi, kunye neswekile ephantsi egazini zinokubakho.
- Esi asisosifo esosulelayo. Sisifo esidluliselwa ebantwaneni ngabazali.
- Izinto eziphambili zonyango kukutya okukhethekileyo, izongezo zesondlo ezifana neoyile ye-MCT, kunye nokuphepha izinto eziyandisa iimpawu.
- Ukuba umntwana wakho unenye yezi mpawu, bonana nogqirha wakho ngoko nangoko ukuze akunike icebiso. Ukuxilongwa kwangoko kubaluleke kakhulu.
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