Sɛ́ ɔwofo no, ebia wo ba ketewa no akwahosan ho asɛm haw wo. Ɛtɔ da bi a, ɛyɛ ade a ɛfata sɛ wobɛte ehu nka kakra bere a wuhu tebea horow a ɛntaa nsi a yɛntee ho asɛm mpo no. Wiɛ, ɛnnɛ yɛreka tebea bi a ɛntaa nsi a nnipa pii ntee ho asɛm, nanso ɛho hia sɛ wohu ho asɛm. Ne din de Homocystinuria.
Sɛ yɛbɛka no tiawa a, dɛn ne Homocystinuria (HCY)?
Susuw ho hwɛ, yenim sɛ protein wɔ nnuan a yedi, titiriw nam, mpataa, nufusu, ne nkesua mu . Nneɛma nketenkete a wɔfrɛ no amino acid na ɛyɛ protein kɛse yi . Te sɛ adansi nneɛma. Wɔ obi a ɔwɔ apɔwmuden nipadua mu no, saa amino acid ahorow yi mu paapae na wɔyam, na ɛbɛyɛ ahoɔden a yɛn nipadua hia.
Nanso, wɔ obi a ɔwɔ Homocystinuria (HCY) mu no, nipadua no ntumi nsɛe amino acid bi a wɔfrɛ no methionine yiye na ɛnyam . Wɔfrɛ eyi sɛ nipadua mu nneɛma a ɛsakra. Sɛ eyi ba a, saa methionine yi ne nnuru foforo a wɔfrɛ no homocysteine a efi mu ba no fi ase boaboa ano wɔ nipadua no mu, titiriw wɔ mogya no mu. Saa nneɛma a wɔaboaboa ano yi yɛ nea asiane wom. Sɛ wɔansa eyi a, ebetumi de akwahosan ho haw a emu yɛ den aba.
Dɛn nti na eyi rekɔ so? Dɛn nti na ɛte saa?
Enzyme titiriw bi a ɛwɔ yɛn nipadua mu boa ma wɔpaapae amino acid a wɔfrɛ no methionine yi mu. Fa saa enzyme yi sɛ ntwamutam. Saa nkankyee yi twitwa ade kɛse a wɔfrɛ no methionine no mu nketenkete.
Wɔ obi a ɔwɔ homocystinuria mu no, saa enzyme (scissors) yi nnyɛ wɔ nipadua no mu, anaasɛ sɛ wɔyɛ a, ɛnyɛ adwuma yiye.
Nea ɛho hia ne sɛ eyi yɛ yare a wonya fi awo mu . Ɛno kyerɛ sɛ wɔde fa awo ntoatoaso ahorow mu. Awosu mu nkwaadɔm abien na ɛkyerɛ wo sɛ yɛ saa enzyme yi. Ɛsɛ sɛ wonya biako fi wo maame hɔ na biako nso fi wo papa hɔ. Sɛ homocystinuria betumi aba a, ɛsɛ sɛ sintɔ bi wɔ awosu mu nkwaadɔm a wonya fi wo maame ne wo papa hɔ no nyinaa mu .
Nsɛnkyerɛnne bɛn na yebetumi ahu?
Nea ɛyɛ nwonwa no, sɛ wohwɛ akokoaa a wɔwoo no a ɔwɔ Homocystinuria a, nsonsonoe biara nni mu. Wɔyɛ nkokoaa a wɔte ase koraa. Nsɛnkyerɛnne no fi ase da adi wɔ nkwa mfe kakraa a edi kan no mu. Ɛnyɛ mmofra nyinaa na wohu saa sɛnkyerɛnne ahorow yi wɔ ɔkwan koro so. Ebia mmofra binom benya saa sɛnkyerɛnne ahorow yi pii, bere a afoforo nso renkyerɛ bi.
Hwɛ su ahorow a ɛwɔ pon a ɛwɔ ase ha no so yiye.
| Su ahorow a ɛwɔ hɔ no fã | Nsɛnkyerɛnne a wɔtaa hu |
|---|---|
| Nipadua a ɛte sɛ nea ɛte | Ɔwɔ honam ani ne ti nhwi a ɛyɛ mmerɛw yiye, ne koko yɛ soronko, ne nipadua tenten na ɛyɛ tratraa sen mmofra afoforo, ne nsateaa atenten a ɛyɛ tratraa. |
| Onyini | Mu duru a ɛkɔ soro brɛoo na enyin. |
| Aniwa a wɔde hwɛ ade | Anisoadehu a ɛyera kɛse (nhu a ɛbɛn), aniwa a ɛtwetwe, ne anifurae mpo. |
| Nsɛnkyerɛnne afoforo a anibere wom | Nnompe a ɛyɛ mmerɛw (ɛyɛ mmerɛw), mogya a ɛtɔ (a ɛma asiane a ɛwɔ hɔ sɛ obi benya akisikuru ne komayare no yɛ kɛse), ne akisikuru. |
| Nkɔso ne nneyɛe | Ɔkyɛ a wɔwea, nantew, ne kasa mu. Adesua mu dɛmdi ne adwene mu haw ahorow. Suban ne nkate mu tumidi ho haw ahorow. |
Ɔkwan bɛn so na nnuruyɛfo hu eyi?
Wɔ aman pii mu no, wɔde afiri a wɔde hwɛ mmofra a wɔawo wɔn foforo no di dwuma de hu tebea horow te sɛ Homocystinuria ntɛm. Saa mogya mu nhwehwɛmu yi ma yehu sɛ ebia abofra no wɔ asiane mu sɛ obenya yare no anaa.
Sɛ nea efi mu ba no nyɛ nea ɛfata a, oduruyɛfo no bɛkamfo akyerɛ sɛ wɔnyɛ mogya ne nsu mu nhwehwɛmu titiriw foforo de si yare no so dua. Wɔ Sri Lanka no, wɔtaa hyɛ sɛ wɔnyɛ nhwehwɛmu ahorow yi bere a yare no ho sɛnkyerɛnne ahorow ada adi akyi na bere a adwenem naayɛ bi aba akyi nkutoo.
Ɔkwan bɛn so na wɔsa? So ɛyɛ biribi a ɛsɛ sɛ yesuro?
Dabi, mma ɛnhaw wo. Nea ɛho hia sen biara ne sɛ wobefi ase sa yare no bere a wɔahu yare no ara pɛ.Oduruyɛfo a ɔhwɛ nipadua mu nneɛma so bɛboa wo wɔ eyi mu. Ayaresa nhyehyɛe no betumi ayɛ soronko wɔ abofra biara mu.
Akwan titiriw abien na ɛwɔ hɔ a wɔfa so sa yare:
1. Vitamin B6 ayaresa
Homocystinuria bi wɔ hɔ a ɛnyɛ den pii. Wobetumi asiw ano denam vitamin B6 a wɔde ma no dodow a ɛdɔɔso so. Wo duruyɛfo bɛsɔ wo ba no ahwɛ sɛ saa ayaresa yi fata wɔn anaa. Saa vitamin yi betumi aboa ma wɔasiw suban ne adwene mu haw ahorow ano wɔ mmofra binom mu, na ebetumi aboa nso ma asiane a ɛwɔ hɔ sɛ aniwa, nnompe, ne mogya a ɛtwetwe ho haw no so atew.
2. Aduan titiriw (Low-Methionine Diet) .
Sɛ abofra no annye vitamin B6 ayaresa no ho biribi a, ade a edi hɔ ne sɛ obefi ase adi aduan a methionine nnim . Saa aduan yi taa yɛ nkwa nna nyinaa. Ɛho hia sɛ wohwehwɛ mmoa fi aduan ho ɔbenfo a ne ho akokwaw wɔ amino acid mu yare mu.
| Nneɛma a ɛsɛ sɛ wususuw ho bere a wudi aduan a methionine kakraa bi nnim akyi no | |
|---|---|
| Nnuan a protein pii wom a ɛsɛ sɛ wɔkwati koraa |
|
| Nnuan afoforo a ɛsɛ sɛ wɔto ano hye anaasɛ wobegyae |
|
| Nneɛma a wubetumi de ahwɛyiye adi | Methionine kakraa bi na ɛwɔ nnuaba ne nhabannuru mu, enti wobetumi adi no dodow a wɔahyɛ da ayɛ , sɛnea wo duruyɛfo ne aduannuru ho ɔbenfo de afotu ma no. |
Nea ɛka ho no, oduruyɛfo no kamfo kyerɛ sɛ wɔmfa aduan titiriw bi mma abofra no sen nufusu. Nnuannuru afoforo a ɛho hia ma abofra no nyin nyinaa wɔ saa aduan yi mu, na woyi methionine no fi hɔ.
Afei nso, oduruyɛfo no betumi akyerɛw nnuru afoforo pii a wɔde ka aduan ho.
- Betaine ne folic acid: Eyinom ma homocysteine a epira a ɛboaboa ano wɔ mogya no mu no so tew.
- Vitamin B12 ne L-cysteine: Eyinom betumi ayɛ nea ɛho nhia esiane yare no nti. Wɔde B12 ma sɛ tuo na wɔde L-cysteine ma sɛ aduan a wɔde ka ho.
Sɛnea ɛbɛyɛ na wɔahwɛ ahu sɛ ayaresa no reyɛ adwuma no, ɛho behia sɛ wɔyɛ wo ba no mogya ne ne nsu mu nhwehwɛmu daa. Bere a wo ba nyin no, ebia ebehia sɛ woyɛ nsakrae nketenkete wɔ ayaresa nhyehyɛe no mu.
Enti, dɛn na ɛsɛ sɛ yesusuw wɔ daakye ho?
Asɛmpa no ne sɛ sɛ wofi ase sa yare no ntɛm na wɔtoa so yiye a, abofra no betumi anyin na wanyin sɛnea ɛsɛ . Ayaresa a ɛfata nso betumi atew asiane a ɛwɔ hɔ sɛ obi benya akisikuru, komayare, ne mogya a ɛbɛtɔ no so kɛse.
Ɛtɔ da bi a, aniwa mu haw betumi aba ɛmfa ho ayaresa no. Nanso wobetumi de oprehyɛn anaa akwan foforo adi ho dwuma. Ade a ɛho hia sen biara ne sɛ wo ne wo duruyɛfo bɛkɔ so adi nkitaho daa na woadi n’akwankyerɛ akyi pɛpɛɛpɛ.
Nkrasɛm a Wɔde Kɔ Fie
- Homocystinuria yɛ awosu mu yare a ɛntaa nsi a nipadua no ntumi nwe methionine, amino acid bi a ɛwɔ nnuan a yedi mu.
- Eyi yɛ tebea a awosu mu nkwaadɔm a asɛe a wonya fii ɛna ne agya no nyinaa hɔ na ɛde ba.
- Nsɛnkyerɛnne (ne tenten tiaa, aniwa mu haw, onyin a ɛkyɛ) da adi wɔ abofra no awo akyi bere tiaa bi.
- Wobetumi adi tebea yi ho dwuma yiye denam aduan titiriw a vitamin B6 anaa methionine nnim so.
- Sɛ wohu wo ba no ntɛm na wosa no yare wɔ ne nkwa nna nyinaa mu a, ebetumi aboa ma watra ase yiye na ɔte ase. Sɛ wowɔ eyi ho dadwen bi a, wo ne wo duruyɛfo nkasa pefee.
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