So wuhuu awo agyiraehyɛde kɛse bi a ɛyɛ kɔkɔɔ tuntum a ne kɔla te sɛ bobesa wɔ wo ba no nipadua so bere a wɔwoo no no? Anaasɛ, bere a wo ba no nyin no, ɛte sɛ nea nan biako ware na ɛyɛ den sen biako no? Ebia wubehu nso sɛ ntini bi wɔ hɔ a ɛkyinkyim. Sɛ wo ba no wɔ saa sɛnkyerɛnne ahorow yi mu biako anaa nea ɛboro saa a, ɛho hia sɛ wuhu tebea a ɛntaa nsi a yɛreka ho asɛm nnɛ a wɔfrɛ no Klippel-Trenaunay Syndrome (KTS) no. Ɛwom sɛ ebia ɛbɛyɛ te sɛ nea ɛyɛ den de, nanso momma yɛmma ɛnyɛ mmerɛw.
Sɛ yɛbɛka no tiawa a, dɛn ne Klippel-Trenaunay Syndrome (KTS)?
KTS yɛ awo mu tebea a ɛntaa nsi koraa a ɛba bere a wɔawo no. Ɛka nnipa bɛyɛ ɔpepem biako biara mu biako pɛ wɔ wiase nyinaa. Enti ɛnyɛ nea abu so kɛse. Ɔhaw atitiriw abiɛsa na wobetumi ahu wɔ obi a ɔwɔ saa tebea yi mu.
1. Awo agyiraehyɛde kɔkɔɔ a ɛwɔ honam ani: Wɔsan frɛ eyi ``Port-wine stain'' efisɛ ɛte sɛ bobesa ketewaa bi kɔla.
2. Varicose veins: Eyi ne tebea a yɛfrɛ no ``Varicose veins''.
3. Basa anaa nan biako a enyin kɛse sen foforo no: Nan biako titiriw betumi anyin atenten anaasɛ ayɛ den asen foforo no.
Wɔ sɛnkyerɛnne atitiriw abiɛsa yi akyi no, nnipa binom nso betumi anya ɔhaw ahorow wɔ ntini a ɛboa ma nsu kari pɛ wɔ yɛn nipadua mu no ho.
Ɛtɔ da bi a nnuruyɛfo frɛ eyi ``CLVM.'' Wei kyerɛ sɛ saa tebea yi ka yɛn nipadua no afã horow pii.
- C (Capillaries): Eyinom ne mogya ntini a ɛyɛ fɛ yiye a ɛka yɛn ntini ne yɛn ntini ahorow bom. Saa awo agyiraehyɛde kɔkɔɔ no fi nsakrae a ɛba eyinom mu.
- L (Lymphatic system): Ɛno kyerɛ sɛ lymphatic system. Yɛn nipadua mu tumi a ɛko tia nyarewa no fã bi.
- V (Ntini): Ɛno kyerɛ ntini. Mogya ntini a ɛde mogya kɔ koma no mu.
- M (Malformation): Eyi kyerɛ sɛ ɔfã bi nnyaa nkɔso sɛnea ɛsɛ.
Fransefo nnuruyɛfo baanu na wodii kan huu saa tebea yi wɔ 1900. Ɛno nti na wɔde wɔn din too saa yare yi so no.
Dɛn ne obi a ɔwɔ KTS ho sɛnkyerɛnne?
Momma yɛnhwɛ sɛnea tebea yi ka obi a ɔwɔ KTS mogya ntini, ntini a ɛyɛ mmerɛw, nnompe, ne ntini a ɛma mogya tu no yiye. Sɛ wopɛ sɛ wote saa sɛnkyerɛnne ahorow yi ase yiye a, hwɛ pon a ɛwɔ ase hɔ no.
| Nsɛnkyerɛnne a ɛkyerɛ | Nkyerɛkyerɛmu a ɛnyɛ den |
|---|---|
| Port-nsã a wɔde yɛ nkekae | Eyi betumi ayɛ KTS ho sɛnkyerɛnne a edi kan. Ɛde mogya ntini nketenkete (capillaries) a ɛwɔ honam ani no a ɛho ahonhon na ɛde ba. Ne kɔla betumi ayɛ soronko fi pink a ɛyɛ hann so kosi bobesa kɔkɔɔ a ɛyɛ tumm so. Bere a worenyin no, saa beae yi betumi ayɛ tuntum anaasɛ ayɛ hann. Ɛtɔ da bi a, akisikuru nketenkete pue wɔ hɔ, na ebetumi apae na mogya atu. |
| Ntini a ɛyɛ mmerɛw | Ɛkame ayɛ sɛ saa tebea yi betumi aka obiara a ɔwɔ KTS. Sɛ ɔhaw ahorow yi ba ntini a ɛwɔ honam ani no mu a, ntini a ɛwɔ nan no mu, titiriw wɔ asen ne asen mu no kɔla yɛ bruu. Ɛtɔ da bi a, ebetumi aka ntini a ɛkɔ nipadua no mu tɔnn no nso. Sɛ eyi ba a, asiane wɔ hɔ sɛ mogya bɛtɔ (Deep Vein Thrombosis - DVT). |
| Basa anaa nan bi nyin a ɛnteɛ | Mpɛn pii no, abasa anaa nan biako pɛ na enyin kɛse sen biako. Eyi betumi afi ase wɔ nkokoaabere mu. Ɛtaa ka nan biako. Ebetumi aba sɛ nan biako tenten anaasɛ ɛyɛ den sen foforo no. Eyi betumi ama ayɛ den sɛ wobɛnantew na woatu. |
| Ntini a ɛma mogya tu ho haw ahorow | Ebia nnipa binom wɔ ntini a ɛma mogya tu a ɛboro so anaasɛ ɛnyɛ nea ɛfata. Sɛ eyinom ntumi nyɛ adwuma yiye a, ebetumi ama ntini mu nsu atu, ama anan ho ahon, anaasɛ ɛde akisikuru, akisikuru, anaa dwensɔtwaa mu haw aba. |
| Yeaw | Ntini no betumi ayɛ wo yaw anaasɛ ayɛ no yaw. Ɔhaw ahorow a ɛfa mogya a ɛkɔ nipadua no ho no betumi ama nan no hohoro na ayɛ yaw. Afei nso, nan a ayɛ kɛse no betumi ate nka sɛ emu yɛ duru anaasɛ ɛyɛ yaw. |
Dɛn nti na tebea yi ba ankasa?
Mpɛn pii no, nsakrae a ɛba yɛn awosu mu nkwaadɔm biako mu na ɛde KTS ba. Sɛ yɛbɛka no pɛpɛɛpɛ a, ɛnam nkwaadɔm mu nsakrae bi a ɛba awosu mu nkwaadɔm a wɔfrɛ no PIK3CA mu.
Nea ɛho hia ne sɛ eyi nyɛ biribi a wonya fi ɛna hɔ kɔ abofra hɔ. Saa awosu mu nsakrae yi ba bere ne bere mu, a ntease biara nni mu. Enti, sɛ́ awofo no, biribiara nni hɔ a enti ɛsɛ sɛ wudi awerɛhow anaa wode eyi ho asodi to wo ho so.
Ɛtɔ mmere bi a nnipa a wonni nsakrae biara wɔ PIK3CA awosu mu abɔde mu nso wɔ KTS, enti nhwehwɛmufo gye di sɛ ebia awosu mu nsakrae afoforo na ɛde ba.
Nsɛnnennen afoforo bɛn na ebetumi aba esiane KTS nti?
Sɛ wɔanhwɛ KTS so yiye a, nsɛnnennen bi betumi aba, na ɛho hia sɛ wohu.
- Mogya a ɛtwetwe: Mogya a ɛtwetwe (DVT) betumi ayɛ wɔ nan no ntini a emu dɔ no mu. Asiane kɛse ne sɛ, sɛ mogya a ɛtɔ yi mu paapae na ɛkɔhyɛ ntini bi mu wɔ ahurututu no mu a, ebetumi ayɛ tebea a ɛde nkwa to asiane mu (Pulmonary Embolism).
- Honam ani yare: Mmoawa a ɛba honam ani (cellulitis) betumi aba.
- Ɔhonhon: Ɔhaw ahorow a ɛba nipadua no mu no betumi ama nsu aboaboa ano wɔ nipadua no mu na ama obi ho ahonhon (Lymphedema).
- Mogya a ɛtɔ wɔ nipadua no mu: Mogya betumi afi yafunu, dwensɔtwaa, akisikuru, anaa mmea mu no, awo nhyehyɛe no mu.
Ɛntaa mma sɛ nnipa binom a wɔwɔ KTS nso betumi anya awo mu dɛmdi a ɛfa nsateaa anaa nansoaa ho.
- Nsateaa foforo a wobenya (Polydactyly) .
- Syndactyly (nsateaa a wɔaka abom) .
Ɔkwan bɛn so na oduruyɛfo hu KTS pɛpɛɛpɛ?
Oduruyɛfo bedi kan ahu tebea no denam akokoaa no nipadua a ɔbɛhwɛ so. Sɛ anyɛ yiye koraa no sɛnkyerɛnne atitiriw abiɛsa a yɛadi kan aka ho asɛm no mu abien (awo agyiraehyɛde, akɔre ntini, ne akwaa a ayɛ kɛse) wɔ hɔ a, wosusuw sɛ ebia ɛyɛ KTS. Esiane sɛ wɔtaa hu saa nsɛnkyerɛnne yi bere a wɔawo no nti, mmere bi wɔ hɔ a wobetumi ahu ansa na akokoaa no afi ayaresabea akɔ fie.
Oduruyɛfo no betumi ahyɛ sɛ wɔnyɛ nhwehwɛmu ahorow de si tebea yi so dua na wahu nkɛntɛnso a enya wɔ nipadua no so ankasa.
| Sɔhwɛ | Dɛn na wuhu wɔ eyi mu? |
|---|---|
| CT scan anaa MRI scan | Hwɛ sɛnea nipadua no ntini a ɛyɛ mmerɛw ne nnompe te, ne baabi a enyin wɔ ɔkwan a ɛnteɛ so. |
| MRA (Magnetic Resonance Angiogram) a Wɔde Di Dwuma. | Eyi yɛ MRI scan soronko bi. Etumi hu sɛnea mogya ntini ne ntini ahorow te no yiye. |
| Doppler Ultrasound a Wɔde Yɛ Nneɛma | Hwɛ ɔkwan a mogya fa ntini ne ntini ahorow no mu, na hwɛ sɛ ebia biribi asiw anaasɛ ɛsan kɔ baabiara anaa. |
Dɛn ne ayaresa ahorow a wɔde sa KTS?
Aduru biara nni hɔ a wɔde sa KTS. Nanso, ayaresa pii wɔ hɔ a ebetumi aboa nkurɔfo ma wɔadi wɔn yare no ho sɛnkyerɛnne ne wɔn nsɛnnennen ho dwuma na wɔatra ase sɛnea ɛsɛ. Ayaresa no gyina onipa no yare no ho sɛnkyerɛnne so.
| Ɔkwan a wɔfa so sa yare | Dɛn na ɛto so? |
|---|---|
| Nnuru a ɛma mogya yɛ mmerɛw (Anticoagulants) . | Saa aduru yi boa ma asiane a ɛwɔ hɔ sɛ mogya bɛtɔ no so tew. |
| Aduru a wɔfrɛ no Sirolimus | Saa aduru yi ma mogya ntini a ɛnyɛ ne kwan so nyin brɛoo anaasɛ egyae, na ɛnam so ma sɛnkyerɛnne ne nsɛnnennen so tew. |
| Nhyɛso Stockings a Wɔde Yɛ Nneɛma | Eyinom boa ma nan ho ahonhon ne ɛyaw so tew, na ɛtew asiane a ɛwɔ hɔ sɛ mogya bɛtɔ no so. Wɔboa ma mogya fi nan no mu kɔ koma no mu. |
| Mpaboa a wɔma so | Sɛ wo nan tenten nyɛ pɛ a, sɛ wode ɔfã a ɛkorɔn ka mpaboa biako ho a, ebetumi ama nantew ayɛ mmerɛw. Eyi nso boa ma asiane a ɛwɔ hɔ sɛ obi benya scoliosis no so tew. |
| Laser ayaresa | Wɔde saa ayaresa yi di dwuma de ma ``port-wine stain'' awo agyiraehyɛde a ɛwɔ honam ani no kɔla yɛ hare. |
| Sclerotherapy a wɔde sa yare | Wɔde nsu titiriw bi gu ntini a ɔhaw wom no mu, na ɛma ɛyɛ adwuma na ɛkata so. Eyi yɛ aduru a etu mpɔn a wɔde sa ntini a ɛyɛ mmerɛw. |
| Sɛɛgyiri | Wobetumi ayɛ oprehyɛn de asiesie ntini mu haw atitiriw, ama nan tenten ayɛ pɛ, anaasɛ wɔbɛtew basa/nan a ayɛ kɛse no kɛse so denam ntini a ɛboro so a wobeyi afi hɔ no so. |
Nneɛma bɛn na ɛsɛ sɛ yɛhwɛ yiye wɔ ho titiriw bere a wo ne KTS te no?
Sɛ wo ne KTS te a, ɛho hia yiye sɛ wubenya wo nipadua ho ntease pa na woahwɛ nneɛma bi so.
- Hwɛ wo honam ani yiye: Sɛ woma wo honam ani tew na wopirapira a, ebetumi atew asiane a ɛwɔ hɔ sɛ wubenya honam ani yare no so.
- Kɔ so yɛ nnam sɛnea wubetumi: Ntra gyinabea koro no ara mu nkyɛ dodo. Apɔw-mu-teɛteɛ ne nantew a wobɛyɛ daa no betumi atew asiane a ɛwɔ hɔ sɛ mogya bɛtɔ no so.
- Hyɛ ntade a wɔde mia wo ho sɛnea wo duruyɛfo kyerɛ no: Sɛ wo duruyɛfo akyerɛw ama wo a, hyɛ sɔks anaa ntade titiriw bi bere nyinaa a ɛtew ɔfe so.
- Kwati akwan bi a wɔfa so siw awo ano: Wo ne wo duruyɛfo nkasa mfa nneɛma te sɛ awo ano aduru a estrogen hormone wom a wobɛkwati ho, efisɛ ɛma asiane a ɛwɔ hɔ sɛ mogya bɛtɔ no yɛ kɛse.
- Wɔ nyinsɛn mu ne ansa na wɔreyɛ oprehyɛn: Wɔ saa mmere yi mu no, asiane a ɛwɔ hɔ sɛ mogya bɛtɔ wo no yɛ kɛse, enti ebia ebehia sɛ wofa nnuru a ɛma mogya tu na woasiw ano. Wo ne wo duruyɛfo nka eyi ho asɛm na nya afotu.
Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn? Mmere bɛn na ɛsɛ sɛ yɛkɔ ETU?
Ɛho hia sɛ obi a ɔwɔ KTS kɔhwɛ no daa wɔ n’asetra nyinaa mu. Eyi ne bere a wotumi hwɛ tebea no so, wotumi hu sɛ ayaresa no reyɛ adwuma, na wɔayɛ nsakrae biara a ɛho hia.
Nanso, ɛtɔ da bi a, ebia ɛho behia sɛ wokɔ ayaresabea wɔ tebea a egye ntɛmpɛ mu. Hwɛ saa sɛnkyerɛnne ahorow yi yiye.
Eyinom ne tebea horow a ɛsɛ sɛ wokɔ ETU (Emergency Treatment Unit) ntɛm ara:
* Sɛ wuhu sɛnkyerɛnne ahorow a ɛkyerɛ sɛ mogya atu: Sɛ wo nan biako yɛ yaw mpofirim, ɛho ahonhon, ɛyɛ kɔkɔɔ, na sɛ woka wo a, ɛyɛ hyew a, ebetumi ayɛ ntini a emu dɔ a ɛma mogya tu (DVT).
* Sɛ wowɔ sɛnkyerɛnne ahorow a ɛkyerɛ sɛ mogya atɔ wo ahurututu mu: Wo koko mu yaw mpofirim, ɛyɛ den sɛ wobɛhome, wobɛhome ntɛmntɛm, anaasɛ wobɛfefe mogya a, ebetumi ayɛ ahurututu mu akisikuru. Eyi yɛ asɛm a egye ntɛmpɛ.
* Sɛ mogya tu kɛse a: Sɛ mogya tu fi nipadua no mu baabiara a wontumi nni so a.
Sɛ wuhu saa nsɛnkyerɛnne yi bi a, nsɛe bere na kɔ ayaresabea a ɛbɛn wo no Emergency Department (ETU) ntɛm ara.
KTS yɛ tebea a ɛyɛ den, enti ebia wowɔ nsɛmmisa pii. Bere biara a wobɛkɔ wo duruyɛfo nkyɛn no, tie biribiara a ɛwɔ w’adwenem. Nsusuw sɛ, "Eyi yɛ ɔhaw bi kɛkɛ a enhia sɛ midi ho dwuma." Ɛyɛ papa sɛ wobɛka wo ho asɛm pefee na woadi nokware akyerɛ wo duruyɛfo.
Nkrasɛm a Wɔde Kɔ Fie
- Klippel-Trenaunay Syndrome (KTS) yɛ awo mu dɛmdi a ɛntaa nsi a ɛnyɛ awofo no mfomso biara na ɛde ba.
- Nsɛnkyerɛnne atitiriw abiɛsa ne: awo agyiraehyɛde kɔkɔɔ tuntum (port-wine stain), ntini a ɛyɛ mmerɛw, ne nsa anaa nan biako a ɛsõ sen foforo.
- Ɛwom sɛ wontumi nsa tebea yi koraa de, nanso ayaresa pii wɔ hɔ a ebetumi aboa wo ma woadi wo yare no ho sɛnkyerɛnne ahorow ho dwuma na woabɔ bra pa ara.
- Nsɛnnennen a ɛho hia sen biara a ɛsɛ sɛ yɛhwɛ yiye ne mogya a ɛtɔ (DVT, Pulmonary Embolism) ne mogya a ɛtɔ dodo. Hwɛ kɔkɔbɔ nsɛnkyerɛnne a ɛwɔ eyinom mu bere nyinaa.
- Ɛho hia yiye sɛ wo ne wo duruyɛfo di nkitaho wɔ w’asetra nyinaa mu na wokɔ nhwehwɛmu daa.
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