Ɔhaw a nnipa pii wɔ nnansa yi ne srade te sɛ srade ne triglycerides a ɛkɔ soro wɔ mogya no mu. Nanso ɛtɔ mmere bi a srade dodow yi, titiriw triglyceride dodow, dɔɔso wɔ ɔkwan a ɛnteɛ so, na ɛboro nea ɛte daa so mpɛn mpempem pii. Ebia nea enti a ɛte saa ne awosu mu tebea bi a ɛntaa nsi koraa a efi awofo hɔ. Ɛnnɛ yɛreka tebea a ɛte saa biako ho asɛm, Familial Hyperchylomicronemia Syndrome, anaa FCS tiawa. Ɛwom sɛ eyi yɛ asɛmti a emu yɛ den kakra de, nanso momma yɛnte ase kɛkɛ.
Sɛ yɛbɛka no tiawa a, dɛn ne FCS?
FCS yɛ awosu mu tebea a ɛntaa nsi koraa a wunya fi w’awofo hɔ. Obi a ɔwɔ saa tebea yi ntumi nsɛe srade, titiriw triglycerides .
Fa no sɛ yɛn nipadua wɔ adwumayɛbea ketewaa bi a ɛpaapae srade. Wɔ aduruyɛ mu no, eyi yɛ enzyme bi a wɔfrɛ no lipoprotein lipase (LpL) . Wɔ obi a ɔwɔ FCS mu no, saa ‘adwumayɛbea’ yi ntumi nyɛ adwuma yiye, anaasɛ ɛnyɛ adwuma koraa.
Ne saa nti, srade a ɛwɔ aduan a yedi mu a wɔfrɛ no triglycerides no nipadua no ntumi nnye na mmom ɛboaboa ano wɔ mogya no mu sɛ srade nketenkete a wɔfrɛ no chylomicrons . Saa chylomicrons a ɛboaboa ano yi ne nea yɛfrɛ no chylomicronemia syndrome. Eyi ma triglyceride dodow a ɛkɔ soro wɔ mogya no mu wɔ ɔkwan a ɛnteɛ so.
Ɛntaa mma araa ma nnipa ɔpepem biako mu biako anaa baanu pɛ na ɛka wɔn wɔ wiase nyinaa. Mpɛn pii no, wohu ansa na wɔadi mfe 10, na wohu sɛ mmofra binom anya tebea no wɔ wɔn nkwa afe a edi kan no mu.
Dɛn nti na eyi rekɔ so?
Nea ɛde eyi ba titiriw ne awosu mu nsakrae. Nea enti a ɛte saa ne sintɔ bi a ɛwɔ awosu mu nkwaadɔm a ɛka ho ma wɔyɛ lipoprotein lipase (LpL) enzyme a yɛkaa ho asɛm no.
Nea ɛho hia ne sɛ, sɛ wubenya saa yare yi a, ɛsɛ sɛ wunya saa awosu mu abɔde a asɛe yi fi wo maame ne wo papa nyinaa hɔ. Sɛ wunya fi ɔwofo biako pɛ hɔ a, worennya yare no, na mmom wobɛyɛ saa awosu mu abɔde no ‘kurafo’ . Eyi kyerɛ sɛ wowɔ tumi a wode saa awosu mu abɔde yi bɛma wo mma.
Yɛn dwensɔtwaa na ɛyɛ LpL enzyme no. Ne dwumadi titiriw ne sɛ ɛbɛpaapae chylomicrons a ɛwɔ mogya no mu na aboa nipadua no ma wɔde srade a ɛwɔ mu no adi dwuma de anya ahoɔden. Enti sɛ LpL ntumi nyɛ adwuma yiye a, wontumi nsɛe srade no na triglyceride dodow a ɛwɔ mogya no mu no kɔ soro.
Dɛn ne eyi ho sɛnkyerɛnne?
FCS ho sɛnkyerɛnne no fi triglycerides a ɛdɔɔso wɔ mogya no mu. Triglyceride dodow a ɛwɔ obi a ɔwɔ apɔwmuden mu no nnu 150 mg/dL. Nanso, wɔ obi a ɔwɔ FCS fam no, saa dodow yi betumi aboro 1,000 mg/dL. Ɛho hia paa sɛ wuhu saa sɛnkyerɛnne ahorow yi.
| Nsɛnkyerɛnne a ɛkyerɛ | Nkyerɛkyerɛmu a ɛnyɛ den |
|---|---|
| Yafunu mu yaw | Eyi ne sɛnkyerɛnne a ɛtaa ba. Ɛyaw a ɛba bere ne bere mu yi betumi afi ase wɔ yafunu no atifi na akɔ akyi. Ɛtɔ da bi a, ebetumi ayɛ den paa. |
| Pancreatitis a ɛyɛ yare | Mpɛn pii no, eyi na ɛde yafunu mu yaw ba. Pancreas yɛ akwaa a ɛho hia wɔ yɛn yafunu mu. Eyi ne bere a ɛho ahonhon. Nsɛnkyerɛnne te sɛ akisikuru, fifiri, mmerɛwyɛ, ne aniwa ne honam ani yɛ kɔkɔɔ nso betumi de eyi aba. |
| Honam ani nsakrae | Nnipa binom nya tebea bi a wɔfrɛ no cutaneous xanthomas . Eyinom yɛ akuru nketenkete a ɛnyɛ yaw, ɛyɛ kɔkɔɔ-kɔla, a epue wɔ mmeae te sɛ asen, nkotodwe, ne nsateaa so. Srade na ɛyɛ eyinom. Sɛ wuhu eyinom a, ebetumi ayɛ sɛnkyerɛnne a ɛkyerɛ sɛ srade dɔɔso wɔ mogya mu. |
| Nsakrae a ɛba aniwa mu | Lipaemia retinalis yɛ tebea a ɛma mogya ntini a ɛwɔ aniwa no mu no te sɛ nufusu. Eyi fi srade a ɛdɔɔso. Nanso, ɛnsɛe anisoadehu. |
| Mmɛn ne akyi berɛmo a ɛtrɛw | Titiriw wɔ mmofra nkumaa mu no, mogya mu nkwammoaa fitaa bi (macrophages) bɔ mmɔden sɛ ɛbɛtwe srade a ɛboro so wɔ nipadua no mu. Saa nkwammoaa yi twetwe srade no na ɛde gu mmerɛbo ne akyi berɛmo mu, na ɛma saa akwaa no trɛw. |
| Adwene ne ntini mu yare ho sɛnkyerɛnne | Ayarefo binom betumi anya tebea horow te sɛ adwenemhaw, nkae a wontumi nkae, ne adwenemhaw. |
Ɔkwan bɛn so na wohu eyi na wɔsa?
Nhwehwɛmu a wɔyɛe
Esiane sɛ ɛyɛ tebea a ɛntaa nsi nti, ɛtɔ mmere bi a ebetumi agye bere kakra ansa na wɔahu yare no pɛpɛɛpɛ. Sɛ wo yafunu mu yɛ wo yaw bere nyinaa, wo dwensɔtwaa mu yare mpɛn pii, na triglyceride dɔɔso wɔ wo mogya mu a, ebia wo duruyɛfo besusuw sɛ FCS.
Wɔyɛ nhwehwɛmu ahorow a edidi so yi de si yare no so dua:
- Mmuadadi mu triglyceride mogya mu nhwehwɛmu: Wɔyɛ nhwehwɛmu yi bere a wɔadi mmuada nnɔnhwerew pii akyi. Sɛ triglyceride dodow boro 750 mg/dL a, ebetumi ayɛ FCS. Ɛtɔ mmere bi a, mogya a wɔde ma no betumi ayɛ te sɛ nufusu.
- Lipase dodow mu nhwehwɛmu: Wɔde aduru titiriw bi (heparin) ma de susuw LpL enzyme no dodow a nipadua no yɛ.
- Awosu mu nhwehwɛmu: Eyi nkutoo ne ɔkwan a wobɛfa so anya awerɛhyem 100%. Ebetumi ahwɛ sɛ ebia awofo baanu no nyinaa nyaa awosu mu abɔde a ɛtɔ sin no fii awo mu anaa.
- CT scan: Eyi boa ma wohu sɛ ebia akwaa te sɛ dwensɔtwaa ne mmerɛbo asɛe anaa.
Akwan a wɔfa so sa yare
Aduan a wodi ne ade titiriw a wɔde sa FCS, na aduru foforo bi nso wɔ hɔ a wɔapene so nnansa yi.
Ade a ɛho hia sen biara: Nnuru te sɛ statin ne fibrates a wɔtaa de ma ma srade srade no nyɛ adwuma mma FCS. Efisɛ sɛ saa nnuru no bɛyɛ adwuma a, ɛsɛ sɛ LpL enzyme a yɛadi kan aka ho asɛm no yɛ adwuma yiye wɔ nipadua no mu.
Aduan a wɔde ma FCS
Eyi ne adwuma sohwɛ fã a ɛho hia sen biara. Ɛho hia sɛ wo ne wo duruyɛfo ne aduannuru ho ɔbenfo yɛ adwuma na wobɛyɛ saa aduan ho nhyehyɛe yi.
- Tow srade ano: Ɛsɛ sɛ srade dodow a wodi da biara no nnu gram 20.
- Gyae nsa nom koraa: Nsã ma triglyceride dodow kɔ soro kɛse mpo.
- To asikre ne carbohydrates a ɛnyɛ den ano: Kwati nneɛma te sɛ anonne a asikre wom ne nnɔkɔnnɔkɔwade.
- Nneɛma a ɛsɛ sɛ wodi: Nnuadewa, nnuaba, atoko, aburow a wɔayam, ɛmo fitaa, nufusu a srade nnim, ne nam a srade nnim.
- Vitamin a wɔde ma: Ebia wo duruyɛfo bɛkamfo akyerɛ sɛ fa vitamin a ɛma srade te sɛ vitamin A, D, E, ne K a nipadua no hia na ama srade so atew no.
Aduru foforo
Olezarsen (Tryngolza) na ɔyɛ ɔkyerɛkyerɛfo.Ɛyɛ aduru foforo a wɔpenee so wɔ December 2024. Ɛyɛ aduru a wɔde twitwiw nipadua no ase pɛnkoro ɔsram biara. Sɛ yɛbɛka no tiawa a, ɛyɛ adwuma denam protein bi a wɔfrɛ no apoC-III a ɛboa ma srade boaboa ano wɔ mogya no mu a nipadua no yɛ so tew so. Wubetumi asua eyi ho ade pii afi wo duruyɛfo hɔ.
Nsɛnnennen a ɛwɔ FCS asetra mu
FCS yɛ nkwa nna nyinaa tebea a ebetumi anya nipadua ne adwene akwahosan nyinaa so nkɛntɛnso kɛse.
- Adidi mu nsɛnnennen: Adidi ne apontow ase a wobɛkɔ no betumi ayɛ den yiye.
- Adwene mu nsunsuanso: Ɛyaw a ɛtaa ba, daakye ho suro, dadwen, ne "amemene mu sum" betumi aba.
- Nkɛntɛnso a enya wɔ asetra ne adwuma mu asetra so: Ayaresabea a wɔtaa kɔ no betumi ama ayɛ den sɛ obekura ayɔnkofa ne adwuma mu.
Enti, ɛho hia yiye sɛ wo ne w’aduruyɛfo kuw no bɛyɛ adwuma yiye na sɛ ɛho hia a, wohwehwɛ mmoa fi adwenemyare ho ɔfotufo bi hɔ.
Nkrasɛm a Wɔde Kɔ Fie
- FCS yɛ awosu mu tebea a ɛntaa nsi a ɛma nipadua no ntumi nsɛe srade (triglycerides).
- Eyi ma triglyceride dodow a ɛkɔ soro wɔ mogya no mu wɔ ɔkwan a ɛnteɛ so, na sɛnkyerɛnne atitiriw ne yafunu mu yaw a emu yɛ den ne dwensɔtwaa mu ɔfe (pancreatitis).
- Ayaresa no fã titiriw ne sɛ wobedi aduan a srade nnim a ɛyɛ katee yiye akyi . Ɛsɛ sɛ wɔkwati nsa koraa.
- Nnuru a wɔde sa srade daa no nyɛ adwuma mma tebea yi, nanso aduru foforo bi wɔ hɔ a wɔde aba.
- Saa tebea yi a wobɛhwɛ so no yɛ w’asetra nyinaa mu asɛnnennen, enti ɛho hia sɛ wunya wo duruyɛfo, aduannuru ho ɔbenfo, ne w’abusua mmoa.
- Sɛ wo anaa w’abusua mu obi wɔ saa sɛnkyerɛnne yi a, kɔ wo duruyɛfo nkyɛn ntɛm na hwehwɛ afotu.
💬 Comments (0)
Wɔnnya nyɛɛ nsɛm biara. Fa wo nsɛm no ka ho wɔ ha nea edi kan.
Fa wo nsɛm no ka ho