So worenya akuru a ɛyɛ nwonwa wɔ wo honam ani? So eyi betumi ayɛ Muir-Torre Syndrome?

So woafi ase reyɛ akuru nketenkete wɔ wo honam ani mpofirim? Ebia wubesusuw sɛ eyinom yɛ ade a ɛfata. Nanso, ɛtɔ mmere bi a, honam ani nsakrae yi nso betumi ne emu haw ahorow abata ho. Ɛnnɛ yɛrebɛka tebea bi a ɛntaa nsi nanso ɛho hia yiye a ɛsɛ sɛ yɛma yɛn ani da hɔ ho asɛm. Wɔfrɛ no Muir-Torre Syndrome.

Dɛn ne Muir-Torre Yare no? Sɛ yɛbɛka no tiawa a...

Muir-Torre Syndrome yɛ awosu mu yare a ɛntaa nsi a ɛma asiane a ɛwɔ hɔ sɛ wubenya kokoram ahorow wɔ wo nkwa nna mu no yɛ kɛse. Nnipa a wɔwɔ saa yare yi taa nya honam ani akisikuru, a ɛyɛ kokoram anaasɛ ɛnyɛ hu (i.e., ɛnyɛ bɔne) wɔ wɔn honam ani. Wobetumi anya kokoram biako anaa nea ɛboro saa nso wɔ nipadua no mu, titiriw wɔ yafunu mu .

Susuw ho hwɛ, nkwammoaa nketenkete ɔpepem pii na ɛka bom yɛ yɛn nipadua no. Sɛ saa nkwammoaa yi mu paapae a, ɛtɔ mmere bi a nsakrae nketenkete (nsakrae) tumi ba awosu mu nkwaadɔm no mu. Ɛno na ɛde awosu mu nsakrae yi ba.

Enti so Muir-Torre Syndrome ne Lynch Syndrome yɛ ade koro?

Mpɛn pii no, yiw. Wobu Moore-Torre yare no sɛ Lynch yare no fã bi . Lynch syndrome nso yɛ tebea a ɛma kokoram ho asiane yɛ kɛse esiane awosu mu nsakrae pii nti. Ɛtɔ mmere bi a nnuruyɛfo nso frɛ no Hereditary Non-Polyposis Colorectal Cancer (HNPCC). Eyi kyerɛ sɛ kokoram ba wɔ akyi berɛmo mu a polyps nni mu. Enti, Moore-Torre yɛ Lynch yare no fã bi a ɛda honam ani su soronko adi.

Tebea yi abu so dɛn? So ɛsɛ sɛ eyi ho asɛm haw yɛn wɔ Sri Lanka?

Moore-Torre yare no yɛ tebea a ɛntaa nsi koraa ankasa . Bɛyɛ 200 pɛ na wɔabɔ amanneɛ wɔ wiase nyinaa. Nanso, eyi nkyerɛ sɛ ɛnsɛ sɛ yɛma yɛn ani da hɔ. Nnipa a wɔwɔ `HNPCC` no mu bɛyɛ 9.2% betumi ada saa Moore-Torre nneɛma yi adi. Ɛte sɛ nea ɛka mmarima nso kakra sen mmea (kyerɛ sɛ, bɛyɛ mmea 2 wɔ mmarima 3 biara mu). Ɛwom sɛ wonni akontaabu pɔtee biara a ɛkyerɛ nnipa dodow a wɔwɔ Sri Lanka de, nanso ɛho hia sɛ wohu tebea horow a ɛtete saa.

Dɛn ne Moore-Torre yare no ho sɛnkyerɛnne? Yɛyɛ dɛn hu no?

Eyi fi akuru anaa nsakrae a ɛba honam ani ne kokoram a ɛwɔ nipadua no mu ho sɛnkyerɛnne titiriw . Ɛtɔ mmere bi a, honam ani haw betumi aba ansa na kokoram a ɛwɔ nipadua no mu aba, bere koro no ara mu, anaa bere a obi anya kokoram akyi. Nanso, honam ani nsakrae taa yɛ sɛnkyerɛnne a edi kan a wohu . Ebia kokoram afoforo renda sɛnkyerɛnne biara adi wɔ ne mfiase.

Nsakrae bɛn na wotumi hu wɔ honam ani?

Ebia wobehu nea edidi so yi wɔ obi a ɔwɔ Moore-Torre yare no honam ani:

• Sebaceous adenomas: Eyinom ne honam ani haw a ɛtaa ba (80% - 99%). Eyinom yɛ akisikuru a ɛnyɛ kokoram (a ɛnyɛ papa). Wɔyɛ kɛse wɔ srade ntini a ɛyɛ ngo ntini a ɛwɔ yɛn honam ani no mu. Wɔtaa hu wɔ ti ne kɔn mu, na wɔ nnipa a wɔwɔ Moore-Torre yare no mu no, wɔtaa hu wɔn koko, yafunu, asen, ne akyi . Wɔte sɛ akuru nketenkete, ɛyɛ den, ɛyɛ kɔkɔɔ anaa honam ani kɔla .
• Sebaceous carcinoma: Eyi yɛ kokoram (a ɛyɛ hu) akisikuru a ɛba srade ntini no mu. Ebetumi ayɛ te sɛ adenoma a ɛwɔ srade mu. Nanso ɛtrɛw ntɛmntɛm, titiriw wɔ aniwa no ho . Ebia mogya bɛtɔ saa akisikuru yi anaasɛ ɛbɛma ade bi a ɛyɛ kurukuruwa asen.
• Keratoacanthoma: Eyi yɛ honam ani akisikuru foforo. Ebetumi ayɛ kɛse wɔ ti, kɔn, dua, ne abasa a ɛbɛn nhwi ntini no so. Ebetumi ayɛ kokoram anaasɛ ɛnyɛ kokoram . Ɛte sɛ akuru ketewaa bi a ɛyɛ kurukuruwa, ɛtɔ mmere bi a mogya ntini a wotumi hu wɔ soro, na keratin nhama bi wɔ mfinimfini . Enyin ntɛmntɛm yiye, ne kɛse bɛyɛ sɛntimita 3 wɔ adapɛn kakraa bi mu, na afei ɛso tew nkakrankakra wɔ asram anaa mfe pii mu. Ebia biako anaa nea ɛboro saa wɔ hɔ.
• Fordyce nsensanee: Esiane sɛ srade ntini no taa ka Moore-Torre yare no ho nti, nnuruyɛfo binom bu saa "Fordyce nsensanee" yi sɛ ɛyɛ sɛnkyerɛnne. Eyinom yɛ srade ntini a ɛkɔ soro a ayɛ kɛse kakra a epue wɔ mmeae a nhwi nni hɔ, te sɛ anofafa ho . Nhwehwɛmu ahorow ada no adi sɛ wɔtaa yɛ wɔ Moore-Torre yare no mu.

Kae sɛ, sɛ wuhu sɛ akisikuru foforo anaa nsakrae te sɛ eyi wɔ wo honam ani, titiriw sɛ ɛrenyin ntɛmntɛm, kɔla sesa, anaasɛ mogya retu a, akyinnye biara nni ho sɛ ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn.

Kokoram bɛn na ɛne Moore-Torre yare no wɔ abusuabɔ?

Kokoram ahorow ahorow betumi aba wɔ saa yare yi ho. Kokoram a ɛtaa ba ne ne sɛnkyerɛnne ne:

• Kokoram a ɛwɔ akyi berɛmo mu: Eyi ne kokoram a abu so sen biara . Ɛka nnipa nyinaa mu bɛyɛ fã. Ɛnam kokoram nkwammoaa a enyin wɔ akyi berɛmo anaa akyi berɛmo mu. Nea ɛne eyi bɔ abira no, wɔ Moore-Torre fam no, kokoram yi betumi anya mfe 15-20 ntɛm sen sɛnea ɛtaa ba, mpɛn pii no bɛyɛ mfe 50. Ebetumi nso atrɛw ntɛmntɛm . Nsɛnkyerɛnne no bi ne yafunu mu yaw, ɔfe, nsakrae a ɛba dwensɔtwaa mu, ne mogya a ɛba nsu mu .
• Yafunu mu kokoram: Eyi nso yɛ kokoram a wɔtaa hu wɔ Moore-Torre ho. Nsɛnkyerɛnne no betumi ayɛ yafunu mu yaw, yafunu a ɛyɛ den, mogya a ɛwɔ nsu mu, ɛyɛ den sɛ ɔbɛyam aduan, akisikuru, ne aduan ho akɔnnɔ a ɛyera .
• Kokoram a ɛwɔ nsu mu:Wɔsan frɛ no `(Urothelial carcinoma)` anaa `(nsakrae mu kokoram)`. Eyi ka nsu a wɔde gu nipadua no mu no. Ebetumi ama obi ate yaw bere a ɔretotɔ ne mogya wɔ nsu mu .
• Endometrial cancer: Eyi yɛ kokoram a ɛba wɔ endometrium, awotwaa no mu ntini no mu . Ebetumi ama yafunu ase ayɛ no yaw, akyi berɛmo ayɛ no yaw, na nsu a ɛyɛ soronko afi awotwaa mu anaasɛ mogya atu .

Bio nso, ebia kokoram ahorow afoforo pii ne saa yare yi wɔ abusuabɔ, a nea ɛka ho ne:

• Kokoram a ɛwɔ awotwaa mu
• Prostate kokoram
• Kokoram a ɛwɔ akisikuru mu
• Asaabo mu kokoram
• Kokoram a ɛba dwensɔtwaa nketenkete mu
• mmerɛbo mu kokoram
• Ahurututu mu kokoram
• Mogya mu kokoram
• Amemene mu kokoram ne ahorow afoforo wɔ hɔ.

Ebia ɛbɛyɛ te sɛ nea saa nsɛm a wɔahyehyɛ yi yɛ hu. Nanso nea ɛho hia ne sɛ ɛnyɛ kokoram ahorow yi nyinaa na ebenya obiara mu. Na, sɛ wohu ntɛm a, wobetumi ayɛ wɔn aduru .

Dɛn nti na Moore-Torre yare yi ba? Dɛn na ɛde ba?

Sɛ yɛbɛka no tiawa a, Moore-Torrell yare no fi nsakrae a ɛba w’awosu mu nkwaadɔm no biako mu . Nsakrae a ɛba yɛn DNA mu no yɛ nsakrae a ɛba nnidiso nnidiso mu. Wɔyɛ saa DNA yi ho mfonini bere a yɛn nkwammoaa mu paapae na ɛyɛ nkwammoaa foforo no. Ɛtɔ mmere bi a, mfomso betumi asi. Sɛ nkwammoaa a ɛte saa a ɛnteɛ no kɔ so mu paapae a, ebetumi de nyarewa ahorow aba, a kokoram ka ho.

Awosu mu nsakrae bɛn na ɛka eyi?

Nneɛma abien titiriw na ɛwɔ hɔ:

• Type 1 Moore-Torre syndrome (Type 1 MTS): Eyi fi awosu mu nkwaadɔm a ɛma wodi mfomso wɔ wo DNA nhyehyɛe mu no biako mu nsakrae . Sɛ saa awosu mu nkwaadɔm yi ntumi nsiesie mfomso ahorow no a, nkwammoaa a ɛnteɛ boaboa ano wɔ ntini ahorow no mu. Wɔ nsɛm 90% mu no, awosu mu abɔde a wɔfrɛ no MSH2 no nya nkɛntɛnso. Nanso, ebia awosu mu nkwaadɔm afoforo pii te sɛ MLH1, MSH6, ne PMS2 ka ho.
• Type 2 Moore-Torrell Syndrome (MTS): Eyi fi nsakrae a ɛba MUTYH awosu mu abɔde mu. Saa awosu mu abɔde yi na ɛsiesie oxidative damage a ɛwɔ yɛn DNA so. Nnipa a wɔwɔ MTS no mu bɛyɛ nkyem abiɛsa mu biako wɔ saa ɔkwan yi. Oxidation ma nsakrae ba DNA molecule no mu, na ebetumi ama nkwammoaa nyin a wontumi nni so (i.e., kokoram). Sɛ awosu mu abɔde a wɔayɛ no foforo no ntumi nsiesie ɔsɛe yi a, ɔsɛe no kɔ so tra hɔ.

So eyi yɛ biribi a efi awo ntoatoaso ahorow mu?

Ɛda adi kɛse sɛ, yiw, efi awosu mu . Nanso, ɛtɔ mmere bi a obi tumi nya awosu mu nkwaadɔm mu nsakrae foforo a abusua no mu biara nni bi da. Ayarefo bɛyɛ 60% betumi ahu abusua abakɔsɛm a ɛfa Moore-Torre syndrome ho. Nanso, esiane sɛ ɛnyɛ obiara a ɔwɔ saa awosu mu nsakrae yi na onya yare no ho sɛnkyerɛnne nti, ɛnyɛ bere nyinaa na abusua mu abusuabɔ yi da adi.

Ebia nnipa binom benya saa tebea yi a wonni sɛnkyerɛnne biara, na ebia wonnim mpo. Afei nso, ɛtɔ da bi a sɛ nipadua no mu tumi a ɛko tia nyarewa no yɛ mmerɛw a, saa `MTS a ahintaw` yi betumi ayɛ adwuma. Nnipa binom anya saa tebea yi bere a wɔanya `akwaa a ɛyɛ den a wɔde si ananmu` na wɔanom nnuru a ɛma nipadua no tumi ko tia nyarewa no akyi.

Ɔkwan bɛn so na wɔde eyi kɔ awo ntoatoaso mu?

• Type 1 MTS yɛ ``autosomal dominant'' tebea. Eyi kyerɛ sɛ ɛsɛ sɛ wunya awosu mu abɔde a wɔayɛ no foforo no biako pɛ na ama woanya tebea no. Sɛ w’awofoɔ mu baako wɔ saa gene mutation yi a, wowɔ hokwan 50% sɛ wo nso wobɛnya bi. Nanso, ebia ɔkwan a wofa so nya sɛnkyerɛnne ahorow no bɛyɛ soronko wɔ ɔwofo a wunyaa awosu mu abɔde no fi ne hɔ no ho.
• Type 2 MTS yɛ ``autosomal recessive`` tebea. Eyi kyerɛ sɛ, sɛ wubenya yare no a, ɛsɛ sɛ w’awofo baanu nyinaa nya awosu mu nkwaadɔm mu nsakrae koro. Eyi ho yɛ na koraa. Nanso, ebia awofo a wɔwɔ saa ``autosomal recessive`` gene yi biako pɛ no rennya sɛnkyerɛnne biara, enti ebia wonnim sɛ wɔwɔ bi.

Ɔkwan bɛn so na nnuruyɛfo hu Muir-Torre Syndrome?

Sɛ wowɔ honam ani akisikuru te sɛ nea yɛaka ho asɛm wɔ atifi hɔ no, titiriw wɔ wo dua so, anaasɛ apue wɔ wo mmofraase a, ebia oduruyɛfo bi besusuw sɛ Moore-Torrell yare no. Obebisa wo nso sɛnkyerɛnne afoforo a ebetumi akyerɛ sɛ obi anya kokoram wɔ wo mu, ne sɛ ebia obi wɔ w’abusua mu a ɔwɔ kokoram ho abakɔsɛm anaa.

Sɔhwɛ ahorow bɛn na wɔyɛ ma eyi?

Ebia wo duruyɛfo bɛyɛ nhwehwɛmu ahorow pii a edi kan ansa na wakamfo akyerɛ sɛ wɔnyɛ awosu mu nhwehwɛmu. Nhwɛsoɔ:

• Immunohistochemistry (IHC): Wɔfa akuru ketewaa bi a ɛwɔ wo honam ani, de aduru titiriw bi gu so, na wɔde afiri a wɔde hwɛ nneɛma nketenkete hwehwɛ mu hwɛ sɛ ebia awosu mu nsakrae pɔtee bi wɔ nhwɛsode no mu anaa.

Ɛdenam nea efi nhwehwɛmu ahorow yi mu ba ne nsɛm afoforo so no, ebia wo duruyɛfo bɛkamfo akyerɛ sɛ wɔnyɛ awosu mu nhwehwɛmu mfa nhwɛ sɛ nsakrae bi aba awosu mu nkwaadɔm a ɛbata Moore-Torre yare no ho no mu anaa. Afei wɔbɛfa wo mogya no bi na wɔahwehwɛ nkwaadɔm mu nsakrae wɔ awosu mu nkwaadɔm a ɛsiesie a ɛne ne ho nhyia MSH2, MLH1, MSH6, PMS2, anaa base excision repair gene MUTYH no mu.

Kokoram ho nhwehwɛmu bɛn na ɛsɛ sɛ obi a ɔwɔ Moore-Torre syndrome yɛ no daa?

Ɛho hia yiye sɛ nnipa a wɔwɔ saa yare yi yɛ kokoram ho nhwehwɛmu daa , efisɛ sɛ wohu ntɛm a, ɛma hokwan a ɛwɔ hɔ sɛ wɔbɛsa wɔn yare no yɛ kɛse. Ebia sɔhwɛ ahorow a nnuruyɛfo bɛkamfo akyerɛ no bi ne:

• Colonoscopy: Nhwehwɛmu a wɔyɛ wɔ dwensɔtwaa kɛse no mu.
• Upper endoscopy (EGD): Wɔhwehwɛ aduan mu, yafunu, ne dwensɔtwaa ketewa no fã a edi kan no mu.
• Prostate nhwehwɛmu ( ma mmarima) .
• Mammogram a wɔde hwɛ:Nufu mu kokoram ho nhwehwɛmu (ma mmea) .
• Pelvic nhwehwɛmu ( ma mmea) .
• Pap smear: Wɔhwehwɛ awotwaa mu kokoram (ma mmea) .
• Urine cytology: Wɔ kokoram ahorow a ɛwɔ nsu a ɛkɔ nipadua mu no ho.
• Mmɛn dwumadi ho nhwehwɛmu
• Mogya dodow a wɔde ma a edi mũ (CBC) .
• Nipadua mu sɔhwɛ
• Skin nhwehwɛmu
• Koko so X-ray a wɔyɛ

Wo duruyɛfo no besi mpɛn dodow a ɛsɛ sɛ woyɛ saa nhwehwɛmu ahorow yi ho gyinae.

Dɛn ne ayaresa ahorow a wɔde sa Moore-Torre yare no?

Ade titiriw a wɔde wɔn adwene si so wɔ Moore-Torre yare no ho dwuma a wodi mu ne sɛ wobehu na wɔayi kokoram afi hɔ sɛ ɛba a . Ɛho behia sɛ woyɛ kokoram ho nhwehwɛmu daa na woyɛ ntini biara a ɛyɛ hu ho biopsies. Sɛ wo duruyɛfo hu kokoram a, wɔbɛsa no sɛnea ne su ne ne fã te. Mpɛn pii no oprehyɛn a wɔyɛ de yi kokoram no ne ayaresa a edi kan .

Ebia kokoram ayaresa afoforo bi ne:

• Nnuru a wɔde sa yare
• Mframa a wɔde sa yare
• Hormone ayaresa
• Nnuru a ɛko tia nyarewa
• Ayaresa a wɔde wɔn ani asi so
• Nnompe mu ntini a wɔde si ananmu

Nea ɛka ho no, ebia retinoid a wɔnom a wɔfrɛ no isotretinoin bɛboa ma wɔasiw honam ani akuru foforo ano. Sɛ wo dwensɔtwaa mu nhwehwɛmu no hu sɛ wo dwensɔtwaa mu polyps pii (a asiane kɛse wom sɛ wubenya kokoram) a, ebia wo duruyɛfo bɛkamfo akyerɛ sɛ wɔnyɛ colectomy a wɔde siw ano, a nea ɛka ho ne sɛ wobeyi wo dwensɔtwaa no fã bi anaa ne nyinaa afi hɔ.

Dɛn ne nkɔmhyɛ a ɛbɛba obi a ɔwɔ Moore-Torre yare no so?

Wo nkɔmhyɛ gyina kokoram dodow a wubenya ne baabi a ɛtrɛw kodu so. Akontaabu kyerɛ sɛ nnipa a wɔwɔ Moore-Torre yare no mu bɛyɛ fã nya kokoram bɛboro biako. Bɛboro fã nya kokoram a ɛtrɛw (kokoram a atrɛw akɔ nipadua no afã afoforo na ɛyɛ den sɛ wɔbɛsa).

Mpɛn pii no, kokoram a ɛwɔ Moore-Torre syndrome no pue bɛyɛ mfe 50. Saa kokoram ahorow yi betumi anyin na asɛe ntɛmntɛm sen nnipa dodow no ara. Enti, ɛho hia sɛ wohu ntɛm . Afei nso, hokwan kɛse wɔ hɔ sɛ kokoram bɛsan aba bere a wɔayi afi hɔ mpo awie no. Enti, ɛho hia sɛ wɔkɔhwɛ wɔn daa bere a wɔasa wɔn yare awie no.

So ɔkwan bi wɔ hɔ a wɔbɛfa so asiw eyi ano?

Mpɛn pii no, ɛnte saa. Eyi te saa efisɛ ɛyɛ tebea a wɔde wo wo. Nanso, ɛnyɛ obiara na obenya yare no ho sɛnkyerɛnne. Ebia wɔn a wonya yare no ho sɛnkyerɛnne no mpo, yare no bɛka wɔn kɛse anaa kakraa bi asen wɔn awofo. Nyansahufo nnya nhuu nea enti a eyi ba. Nanso wɔahu sɛ sɛ nipadua no mu tumi a ɛko tia nyarewa no yɛ mmerɛw a, tebea no betumi asɛe kɛse .

Nim a wunim sɛ wowɔ saa awosu mu nsakrae yi betumi aboa wo ma woasisi gyinae ahorow bi de asiw ano. Sɛ nhwɛso no, awosu mu nhwehwɛmu ne awosu mu afotu betumi aboa wo ma woasiw tebea no ankɔ wo mma so. Afei nso, sɛ wuhu eyi a, ebetumi aboa wo ma woahu kokoram ntɛm na woafi ase sa yare, na asiw nneɛma bɔne a ebefi mu aba no ano.

Ɛwom sɛ Moore-Torre yare no ho yɛ na de, nanso ɛnyɛ mmerɛw sɛ wobegyina yare a wɔahu a ɛte sɛɛ ano. Ebetumi ayɛ te sɛ nea ɛyɛ den sɛ yɛbɛko atia biribi a ɛhyɛ yɛn DNA mu. Nanso, nimdeɛ yɛ tumi . Sɛ wohu wɔ bere a ɛsɛ mu na wosa wo yare a, wo ne wo duruyɛfo betumi ahyia saa asɛnnennen yi.

Awiei koraa no, nkrasɛm a wɔde kɔ fie

Okay, enti momma yɛnbɔ nneɛma bi a ɛsɛ sɛ wokae fi nea yɛaka ho asɛm no mu no mua:

• Muir-Torre Syndrome yɛ awosu mu tebea a ɛma asiane a ɛwɔ hɔ sɛ obi benya honam ani akisikuru ne kokoram a ɛwɔ nipadua no mu (titiriw wɔ aduan mu ntini mu) no yɛ kɛse.
• Sɛ wuhu sɛ akisikuru foforo a ɛyɛ soronko wɔ wo honam ani, titiriw wɔ wo dua so a, hwehwɛ aduruyɛfo afotu ntɛm ara. Ebetumi ayɛ hwee, nanso eye sɛ wobɛma wɔahwɛ mu.
• Eyi yɛ Lynch yare no fã bi. Sɛ obi wɔ w’abusua mu wɔ kokoram ho abakɔsɛm a, ka ho asɛm nso kyerɛ wo duruyɛfo.
• Sɛ wobehu no ntɛm na wɔayɛ nhwehwɛmu daa no ho hia yiye. Eyi betumi aboa ma wɔahu kokoram ntɛm na wɔasa no yiye.
• Awosu mu nhwehwɛmu ne afotu betumi aboa ma wɔahu sɛ ebia tebea yi wɔ hɔ ne sɛ ebia ɛka abusua no mufo anaa.
• Nsuro, na mmom hwɛ yiye. Ɛnyɛ wo nko ara, na nnuruyɛfo wɔ hɔ sɛ wɔbɛboa wo.

Mewɔ anidaso sɛ saa nsɛm yi ho bɛba wo mfaso. Kɔ so nya apɔwmuden!

` Muir-Torre Syndrome, Lynch Syndrome, Honam ani Kokoram, Awosu mu Nyarewa, Yafunu mu Kokoram, Kokoram Nhwehwɛmu, Awosu mu Nsakrae