So wo ba kumaa no brɛ bere a wadi agoru kakra awie no? Anaasɛ ɛte sɛ nea ɔyare bere nyinaa, ɔmpɛ sɛ odidi, anaasɛ ɔda? Ɛtɔ mmere bi a yesusuw sɛ eyinom yɛ nneɛma a ɛfata, nanso eyi akyi betumi ayɛ awosu mu tebea bi a ɛntaa nsi koraa, nanso emu yɛ den yiye. Ɛnnɛ yɛreka tebea a ɛte saa biako ho asɛm, Long-Chain Fatty Acid Oxidation Disorders, anaa LC-FAODs .
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Sɛ yɛbɛka no tiawa a, dɛn ne saa LC-FAOD ahorow yi?
Eyi yɛ din a ɛyɛ den kakra, nanso momma yɛmma ɛnyɛ mmerɛw. Yɛn nipadua te sɛ kar. Ehia ahoɔden anaa pɛtro na ama ayɛ adwuma. Aduan a yedi no yɛ pɛtro ma yɛn nipadua. Wɔfrɛ ɔkwan a wɔfa so dan saa aduan yi ma ɛbɛyɛ ahoɔden no sɛ nipadua mu nneɛma a ɛsakra .
Nnuan a yedi, te sɛ ngodua ngo , mpataa , avocado, ne nam, wɔ biribi a wɔfrɛ no ‘fatty acids’. Eyinom yɛ ahoɔden fibea pa ma nipadua no. Saa srade ahorow yi ahorow pii wɔ hɔ. Wɔn mu no, nea wɔfrɛ no ‘srade a ɛwɔ nkɔnsɔnkɔnsɔn tenten’ no hia enzyme titiriw bi na ama wɔadan ahoɔden.
Mmofra a wɔwo wɔn a wɔwɔ awosu mu tebea bi a wɔfrɛ no LC-FAODs no nni saa enzyme pɔtee no wɔ wɔn nipadua mu. Enti wɔn nipadua ntumi nsakra srade a ɛwɔ nkɔnsɔnkɔnsɔn tenten yi nyɛ ahoɔden.
Dɛn na ɛba saa bere no?
1. Akwaa a ɛho hia te sɛ koma , amemene, mmerɛbo , ne ntini nnya ahoɔden a ɛho hia.
2. Srade a wontumi nsakra no ahoɔden boaboa ano wɔ saa akwaa ahorow no mu na efi ase sɛe.
Eyi betumi de nsɛnnennen a emu yɛ den te sɛ komayare , mmerɛbo a entumi nyɛ adwuma yiye , ne asikre a ɛba mogya mu kɛse aba . Enti, ɛho hia yiye sɛ wuhu yare yi ntɛm na wokura aduan a wodi so yiye .
Dɛn ne yare yi ho sɛnkyerɛnne?
Nsɛnkyerɛnne no betumi ayɛ soronko wɔ onipa biara ho. Egyina enzyme ko a enni hɔ ne sɛnea yare no mu yɛ den so. Ebia nnipa binom renkyerɛ yare no ho sɛnkyerɛnne biara kosi sɛ wɔbɛyɛ mmerante ne mmabaa anaa wɔanyinyin. Nanso wɔ tebea horow a emu yɛ den mu no, sɛnkyerɛnne ahorow no fi ase da adi wɔ nkwa nna mu asram kakraa a edi kan no mu.
Nsɛnkyerɛnne a edi kan a ɛkyerɛ sɛ koma ntini mu yare wɔ mmofra a wɔawo wɔn foforo mu ne cardiomyopathy. Nea ɛka eyi ho ne ahome a ɛyɛ den, aduan a ɛyɛ den, ne nna a ɛtra so. Nkokoaa ne mmofra nkumaa betumi ada mmerɛbo mu haw ho sɛnkyerɛnne adi ntɛm, te sɛ aniwa ne honam ani a ɛyɛ kɔkɔɔ (jaundice), ne asikre a ɛba mogya mu (hypoglycemia).
Momma yɛnhwɛ tebea atitiriw a ebetumi aba wɔ eyi mu ne sɛnkyerɛnne ahorow a ɛbata ho.
| Gyinabea | Nsɛnkyerɛnne a ɛtaa ba |
|---|---|
| Su ahorow a ɛwɔ hɔ nyinaa |
|
| Asikre a ɛwɔ mogya mu a ɛba fam (Hypoglycemia) . | |
| Ammonia dodow a ɛkɔ soro wɔ mogya mu (Hyperammonemia) . | |
| Koma ne ntini mu yare |
Nea ɛho hia ne sɛ, wɔ mmofra binom fam no, bere a wɔyare, ɔkɔm de wɔn, anaa adwennwen nkutoo na saa sɛnkyerɛnne ahorow yi da adi.
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Eyi yɛ awosu mu yare koraa . Ɛne sɛ, wonya fi awofo hɔ ma mmofra. Sɛ yɛbɛka no tiawa a, nea ɛde ba ne awosu mu nkwaadɔm mu nsakrae a ɛkyerɛ sɛnea wɔyɛ enzyme a ɛpaapae srade a yɛkaa ho asɛm no.
Sɛ abofra benya yare no a, ɛsɛ sɛ wonya awosu mu abɔde a asɛe no bi fi awofo baanu no nyinaa hɔ . Sɛ wonya fi ɔwofo biako pɛ hɔ a, abofra no yɛ ɔsoafo''. Wɔrennya yare no ho sɛnkyerɛnne, nanso wobetumi de awosu mu abɔde no akɔma wɔn mma.
Eyi nyɛ yare a wɔde kɔ nnipa mu. Wontumi mfa mfi onipa biako mu nkɔ foforo mu ɔkwan biara so.
Ɔkwan bɛn so na wobetumi ahu yare no?
Wɔ aman pii mu no, wɔhwehwɛ akokoaa biara a wɔawo no foforo mu hwɛ sɛ ɔwɔ awosu mu nyarewa yi anaa. Wɔde mogya ketewaa bi a wogye fi asen no mu wɔ nnafua 1-2 akyi na ɛyɛ eyi. Sɛ nhwehwɛmu yi ma yehu yare no a, wɔyɛ nhwehwɛmu foforo de si yare no so dua.
Sɔhwɛ atitiriw a wɔyɛ ma eyi ne:
- Mogya ne nsu mu nhwehwɛmu: Eyinom hwɛ sɛ srade ne nneɛma afoforo a ɛwɔ mogya ne nsu mu no dɔɔso wɔ ɔkwan a ɛnteɛ so anaa.
- Awosu mu nhwehwɛmu: Eyi nkutoo ne ɔkwan a wobɛfa so ahu pefee sɛ ebia wowɔ LC-FAODS, na sɛ ɛte saa a, nea ɛyɛ.
Sɛ wo ba no wɔ sɛnkyerɛnne ahorow a yɛadi kan aka ho asɛm no a, ɛho hia yiye sɛ wobɛkɔ mmofra oduruyɛfo nkyɛn ntɛm ara akɔbɔ eyi ho nkɔmmɔ.
Ɔkwan bɛn so na wɔsa?
Ɛwom sɛ wontumi nsa yare yi koraa de, nanso sɛ wodi ho dwuma yiye a , wobetumi akwati nsɛnnennen a emu yɛ den na wɔatra ase sɛnea ɛsɛ. Ayaresa atitiriw no bi ne nsakrae a wɔyɛ wɔ aduan mu, aduannuru titiriw a wɔde ma, ne asetra mu nsakrae.
1. Aduan a wodi
Eyi ne ade a ɛho hia sen biara. Ɛsɛ sɛ wɔyɛ saa aduan yi wɔ aduan ho ɔbenfo afotu ase.
- To nnuan a srade a ɛwɔ nkɔnsɔnkɔnsɔn tenten mu pii wom ano: Ɛsɛ sɛ wɔma nnuan te sɛ nam, afifide mu ngo bi, nnuaba, ne mpataa ano hye.
- Di nnuan a carbohydrate wom pii: Sɛ wubenya ahoɔden a, ɛsɛ sɛ wode nnuan a carbohydrates pii wom te sɛ aburow, ɛmo, ne ɛmo a ɛyɛ dɛ ka w’aduan ho.
- Di nnuan nketenkete a wotaa di: Ɛho hia sɛ wudi mmere daa wɔ da mũ no nyinaa mu na ama asikre dodow a ɛwɔ wo mogya mu no akɔ so ayɛ nea ɛyɛ den.
- Kwati mmuadadi: Ɛnyɛ papa sɛ ɔkɔm de wo bɛboro nnɔnhwerew 8.
Sɛ wo ba mogya mu asikre so tew mpofirim dodo a, ebia ebehia sɛ ɔkɔ ayaresabea na wɔma no asikre aduru (IV) a ɛwɔ nkyene aduru a wɔde fa ntini mu wɔ Emergency Treatment Unit (ETU) mu.
2. Nnuannuru a wɔde ma
Esiane sɛ saa ayarefo yi ntumi mfa srade a ɛwɔ nkɔnsɔnkɔnsɔn tenten nni dwuma nti, wɔma wɔn srade foforo a ɛnyɛ den sɛ nipadua no betumi adan no ahoɔden. Wɔfrɛ eyinom sɛ medium-chain triglycerides (MCTs) . Eyinom wɔ hɔ sɛ MCT ngo. Wɔde saa MCT ahorow yi nso ka nkokoaa aduan bi ho. Wo duruyɛfo bɛma wo eyi ho nsɛm pii.
3. Nnuruyɛ
Wɔapene aduru bi a wɔfrɛ no Triheptanoin (Dojolvi) so sɛ wɔmfa nyɛ LC-FAODs. Sɛ wo duruyɛfo akyerɛw wo nkutoo na wubetumi anya bi .
4. Asetra mu nsakrae
Ɛho hia sɛ wokwati nneɛma a ɛkanyan yare no a ɛma yare no ho sɛnkyerɛnne no mu yɛ den.
- Kwati apɔw-mu-teɛteɛ a ɛtra so: Tow dwumadi ahorow a ɛma nipadua no te nka sɛ wabrɛ dodo no ano hye.
- Di adwennwen ho dwuma: Nneɛma te sɛ yoga ne nsusuwii betumi aboa.
- Bɔ wo ho ban fi yare ho: Fa nnuru a wɔde bɔ wo ho ban a wo duruyɛfo kamfo kyerɛ nyinaa wɔ bere ano. Hwehwɛ ayaresa ntɛm ara wɔ atiridii anaa atiridii ketewaa bi mpo ho.
So yare yi a wobɛtra ase no yɛ asɛnnennen?
Nokwarem no, yiw. Eyi yɛ nkwa nna nyinaa tebea a ɛsɛ sɛ wodi ho dwuma. Ɛsɛ sɛ wohwɛ yiye wɔ w’aduan ho. Ɛsɛ sɛ wuhu nea ɛsɛ sɛ woyɛ wɔ tebea a egye ntɛmpɛ mu. Eyi betumi ahaw abofra no ne awofo no nyinaa wɔ adwene mu.
Enti,
- Sua yare no ho ade yiye: nimdeɛ ne tumi a ɛsen biara.
- Hwehwɛ adwene mu mmoa: ntwentwɛn wo nan ase sɛ wobɛhwehwɛ mmoa afi abusua, nnamfo, anaa adwenemyare ho ɔfotufo hɔ.
- Kɔ so ne wo duruyɛfo nni nkitaho: Bisa no sɛ wowɔ nsɛmmisa anaa nsɛm bi a ɛhaw wo.
Ɛwom sɛ saa yare yi ho yɛ na de, nanso sɛ wɔhwɛ abofra no so yiye na wɔhwɛ no yiye a, obetumi anya asetra pa. Nea ɛho hia sen biara ne sɛ wubehu yare no ntɛm na woadi oduruyɛfo no akwankyerɛ akyi.
Nkrasɛm a Wɔde Kɔ Fie
- LC-FAODs yɛ awosu mu tebea a emu yɛ den a wonya fi awo mu a ɛmma nipadua no ntumi nsakra srade ahorow bi ma ɛnyɛ ahoɔden.
- Nsɛnkyerɛnne te sɛ ɔbrɛ a ɛtra so, ntini a ɛyɛ mmerɛw, koma ne mmerɛbo mu haw, ne asikre a ɛba mogya mu betumi aba.
- Eyi nyɛ yare a wɔde kɔ nnipa mu. Ɛyɛ biribi a wonya fi awofo hɔ ma mmofra.
- Nneɛma atitiriw a ɛwɔ ayaresa mu ne aduan titiriw, aduannuru a wɔde ma te sɛ MCT ngo, ne nneɛma a ɛma yare no ho sɛnkyerɛnne no mu yɛ den a wɔkwati.
- Sɛ wo ba no wɔ saa sɛnkyerɛnne yi bi a, kɔ wo duruyɛfo nkyɛn ntɛm ara kɔhwehwɛ afotu. Sɛ wohu yare no ntɛm a, ɛho hia yiye.
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