So wo were ne w’ani fitaa reyɛ kɔkɔɔ? Momma yensua pii fa Dubin-Johnson Syndrome ho!

So woahyɛ no nsow pɛn sɛ wo honam ani kɔla anaa w’ani fitaa no te sɛ nea ɛyɛ kɔkɔɔ kakra? Anaasɛ woahyɛ no nsow pɛn sɛ wo nsu no yɛ kɔkɔɔ tuntum sen sɛnea wotaa yɛ no? Sɛ worehyia saa nneɛma yi a, ade biako a ebetumi de aba ne tebea bi a ɛntaa nsi nanso ɛnyɛ aniberesɛm kɛse a wɔfrɛ no Dubin-Johnson syndrome . Enti, momma yɛnka eyi ho asɛm nkɔ akyiri, tiawa koraa.

Dɛn ne Dubin-Johnson Yare no?

Sɛ yɛbɛka no tiawa a, Dubin-Johnson yare no yɛ awosu mu yare a ɛka yɛn mmerɛbo . Nea ɛba wɔ eyi mu ne sɛ ade kɔkɔɔ bi a wɔfrɛ no bilirubin a wɔyɛ wɔ yɛn nipadua mu no boaboa ano sen sɛ ebeyi afi nipadua no mu yiye. Seesei ebia woresusuw nea bilirubin yɛ ho. Bere bi akyi no, sɛ wɔn nkwa nna twam a, mogya mu nkwammoaa kɔkɔɔ a ɛwɔ yɛn nipadua mu no sɛe. Ɛno ne bere a wɔyɛ bilirubin yi. Mpɛn pii no, ɛsɛ sɛ woyi bilirubin yi fi yɛn mmerɛbo mu ka bile ho na woyi fi nipadua no mu sɛ nwura. Nanso, saa adeyɛ yi nsi yiye wɔ obi a ɔwɔ Dubin-Johnson yare no nipadua mu. Enti, bilirubin boaboa ano wɔ mmerɛbo ne mogya mu, na ɛde sɛnkyerɛnne te sɛ jaundice ba . Ɛno na honam ani hwɛbea ne aniwa fitaa dan kɔla.

Hena na tebea yi ka no kɛse?

Dubin-Johnson syndrome yɛ awosu mu tebea , enti ebetumi aka obiara, wɔ mfe biara mu. Sɛ wubenya tebea no a, ɛsɛ sɛ w’awofo baanu no nyinaa de awosu mu abɔde no ma wo. Wɔfrɛ eyi sɛ autosomal recessive agyapade. Sɛ nhwɛso no, sɛ wunya awosu mu abɔde no fi w’awofo no mu biako pɛ hɔ a, worenkyerɛ yare no ho sɛnkyerɛnne, na mmom wobɛyɛ awosu mu abɔde no kurafo . Eyi kyerɛ sɛ wubetumi de awosu mu abɔde no akɔma wo mma.

Tebea yi abu so dɛn?

Akontaabu pɔtee biara nni hɔ a ɛkyerɛ sɛnea eyi abu so, nanso ɛtaa ba nnipa a wɔte aman te sɛ Iran, Iraq, Morocco, ne Japan mu. Wobu akontaa sɛ ebia nnipa 1,300 biara mu biako wɔ saa aman no mu.

Ɔkwan bɛn so na Dubin-Johnson yare no ka me nipadua?

Dubin-Johnson yare no fi ade kɔkɔɔ a wɔfrɛ no bilirubin a ɛboaboa ano wɔ wo mmerɛbo mu no. Eyi na ɛma wo honam ani ne w’ani fitaa yɛ kɔkɔɔ. Ɛtɔ mmere bi a, sɛ wowɔ dan mu na hann kakraa bi na ɛwɔ mu a, ebia worenhu saa kɔkɔɔ yi. Nanso, wɔ baabi a hann wɔ hɔ yiye, te sɛ owia mu no, saa kɔkɔɔ yi da adi kɛse. Mpɛn pii no, ade a edi kan a wuhu wɔ saa kɔkɔɔ yi ho ne anim.. Afei ebetumi atrɛw akɔ nipadua no afã afoforo te sɛ koko ne yafunu. Sɛ wohwɛ ahwehwɛ mu a, sɛ wubue w’ano na wohwɛ wo tɛkrɛma ase a, wubetumi ahu sɛ ayɛ kɔkɔɔ wɔ hɔ nso.

Dɛn ne eyi ho sɛnkyerɛnne?

Nnipa dodow no ara a wohu sɛ wɔwɔ Dubin-Johnson yare no na wɔwɔ jaundice ho sɛnkyerɛnne titiriw, a efi bilirubin a ɛboaboa ano wɔ mmerɛbo mu no. Eyi kyerɛ sɛ:

• Wo honam ani yɛ kɔkɔɔ ne w’ani fitaa.
• Wo nsuo kɔla a ɛsakra (ɛdan kɔ kɔkɔɔ anaa bruu tuntum).

Ɛtɔ da bi a, sɛ nipadua no wɔ adwennwen bi mu, sɛ nhwɛso no, bere a wowɔ yare foforo, bere a wonyinsɛn, anaa sɛ wode awo ano aduru (aduru a wɔde siw nyinsɛn ano) redi dwuma a, kɔla kɔkɔɔ yi betumi ayɛ kɛse.

Eyinom akyi no, sɛnkyerɛnne nketenkete afoforo betumi aba, nanso mpɛn pii no ɛnyɛ den saa:

• Ɔbrɛ a wobɛte nka no yɛ `(Ɔbrɛ)`.
• Ɛyaw kakra a ɛba yafunu no ase (Abdominal Pain).

Ade a ɛho hia ne sɛ Dubin-Johnson yare no yɛ tebea a ɛnyɛ hu. Eyi kyerɛ sɛ ɛrensɛe w’asetra kɛse biara.

Sɛ woyɛ sɔhwɛ ahorow bi a, ebia wubehu nneɛma te sɛ eyi:

• Wo mmerɛbo no wɔ nneɛma bi a ɛboaboa ano, na ɛma ɛyɛ te sɛ tuntum (wɔhwɛ eyi denam ntini ketewaa bi a wogye fi mmerɛbo no mu so - wɔfrɛ no `Liver Biopsy` ).
• Ebia mmerɛbo no ayɛ kɛse kakra ( `Hepatomegaly` ).

Dɛn na ɛde Dubin-Johnson yare no ba?

Ade titiriw a ɛde Dubin-Johnson yare no ba ne nkwaadɔm mu nsakrae a ɛba `ABCC2` awosu mu abɔde mu. Saa `ABCC2` gene yi na ɛyɛ protein a ɛboa ma yɛn nkwammoaa yi nwura fi hɔ. Ne titiriw no, saa protein yi boa mmerɛbo mu nkwammoaa ma woyi bilirubin (ade kɔkɔɔ a aka wɔ mogya mu nkwammoaa kɔkɔɔ a ɛpaapae mu) na woyi fi mu sɛ bile (nsu a ɛboa ma wɔyam aduan).

Enti, sɛ wowɔ Dubin-Johnson syndrome a, wo nipadua no ntumi nyi saa ade fĩ a wɔfrɛ no bilirubin yi mfi wo nkwammoaa mu yiye. Ne saa nti, nneɛma te sɛ bilirubin boaboa ano wɔ wo nipadua mu (wɔfrɛ eyi hyperbilirubinemia ). Ɛno nti na sɛnkyerɛnne te sɛ jaundice da adi no.

Ɔkwan bɛn so na wohu sɛ obi anya Dubin-Johnson yare no?

Wo duruyɛfoɔ bɛdi kan ahwɛ wo, abisa wo yareɛ no ho nsɛnkyerɛnneɛ, na wahunu sɛ ebia obi wɔ w’abusua mu anya tebea a ɛte saa pɛn (abusua aduruyɛ ho abakɔsɛm).

Afei, wɔbɛhyɛ sɛ wɔnyɛ nhwehwɛmu pii bio de si so dua sɛ wɔahu yare no. Ebia eyinom bi ne:

• Mfoninitwa nhwehwɛmu (sɛ nhwɛso no , X-ray , ultrasound scan ) de hwɛ wo mmerɛbo.
• Mogya mu nhwehwɛmu (titiriw bilirubin nhwehwɛmu ) .baako).
• Nsu mu nhwehwɛmu .
• Ebia sɔhwɛ a wɔyɛ mmerɛbo no fã ketewaa bi ( `Liver Biopsy` ).

Sɛ bilirubin nhwehwɛmu no kyerɛ sɛ wo mogya mu bilirubin dodow dɔɔso dodo a, ɛkyerɛ sɛ wowɔ tebea bi a wɔfrɛ no hyperbilirubinemia (bilirubin a ɛdɔɔso wɔ mogya mu). Sɛ wo yare no ho sɛnkyerɛnne kɔ so a, wo duruyɛfo betumi ahyɛ sɛ wɔnyɛ awosu mu mogya mu nhwehwɛmu na ama wɔahu awosu mu nkwaadɔm a ɛde wo sɛnkyerɛnne ahorow no ba. Eyi nkutoo ne ɔkwan a wobɛfa so asi so dua sɛ wowɔ Dubin-Johnson yare no.

Dɛn ne ayaresa ahorow a wɔde sa eyi?

Asɛmpa no ne sɛ nnipa dodow no ara a wɔahu sɛ wɔwɔ Dubin-Johnson yare no nhia ayaresa biara. Eyi te saa efisɛ ɛnyɛ tebea a emu yɛ den. Nanso, sɛ ɛho hia sɛ wɔsa yare a, mpɛn pii no ɛyɛ tebea anaa adeyɛ foforo a ɛrema wo bilirubin dodow akɔ soro nti. Sɛ nhwɛso no, sɛ wowɔ atiridiinini a, ebia wo duruyɛfo bɛka akyerɛ wo sɛ nom nsu pii na gye w’ahome. Anaasɛ, sɛ aduru bi a worenom rema wo yare no ho sɛnkyerɛnne no asɛe kɛse a, ebia wo duruyɛfo betu wo fo sɛ gyae saa aduru no nom.

Dɛn na ebetumi ama Dubin-Johnson yare no ho sɛnkyerɛnne no asɛe kɛse?

Jaundice sɛnkyerɛnne a Dubin-Johnson yare no de ba no betumi atra hɔ nkwa nna nyinaa. Nanso, sɛ wo:

• Sɛ wonom nsa a.
• Sɛ worenom nnuru a wɔde siw awo ano a.
• Sɛ ɔyare mmoawa bi wɔ hɔ a.
• Sɛ woanyinsɛn a.

Sɛ wote nka sɛ wo yare no ho sɛnkyerɛnne reyɛ kɛse a, wo ne wo duruyɛfo nkasa mfa ayaresa a ebetumi aboa wo ma woanya ahotɔ ho.

So wobetumi asiw Dubin-Johnson yare no ano?

Esiane sɛ ɛyɛ awosu mu tebea nti, yɛrentumi nsiw Dubin-Johnson yare no kwan. Nanso, sɛ woreyɛ nhyehyɛe sɛ wobɛwo ba na wopɛ sɛ wuhu asiane a ɛwɔ hɔ sɛ wo ba no benya saa awosu mu yare yi a, wubetumi ne wo duruyɛfo akasa afa awosu mu nhwehwɛmu anaa awosu mu afotu ho .

Sɛ mewɔ Dubin-Johnson syndrome a, dɛn na ɛsɛ sɛ mehwɛ kwan?

Adwene a nnipa a wɔahu sɛ wɔwɔ Dubin-Johnson yare no wɔ no ye yiye. Asa biara nni hɔ, na mpɛn pii no ɛho nhia sɛ wɔde sa yare, na wodi sɛnkyerɛnne ahorow no ho dwuma. Nea ɛho hia sen biara no, tebea no nka obi nkwa nna.

Bere bɛn na ɛsɛ sɛ mekɔ me duruyɛfo nkyɛn?

Sɛ wo Dubin-Johnson syndrome ho sɛnkyerɛnne no mu yɛ den, titiriw sɛ wo jaundice no de sɛnkyerɛnne ahorow a ɛte sɛ atiridii ba a, kɔ wo duruyɛfo nkyɛn. Nhwɛsoɔ:

• Yafunu mu yaw .
• Huraeɛ .
• Ɔbrɛ .
• Akisikuru anaasɛ ɔfe .

Nsɛm bɛn na ɛsɛ sɛ mibisa me duruyɛfo?

Wubetumi abisa wo duruyɛfo nsɛm te sɛ eyinom:

• So me sɛnkyerɛnne ahorow no hwehwɛ sɛ wɔsa me yare?
• So me ba no benya saa tebea yi afi awo mu?
• Sɛ mete nka sɛ me ho nyɛ me den esiane me yare no ho sɛnkyerɛnne nti a, dɛn na ɛsɛ sɛ meyɛ?

Nsonsonoe bɛn na ɛda Dubin-Johnson yare ne Rotor yare no ntam?

Dubin-Johnson yare ne Rotor yare no nyinaa yɛ awosu mu tebea horow. Wɔn baanu nyinaa wɔ sɛnkyerɛnne a ɛte saa ara, te sɛ jaundice. Nanso, nsonsonoe titiriw a ɛda tebea abien no ntam ne nea ɛde ba.

Dubin-Johnson syndrome no fi nkwaadɔm mu nsakrae a ɛba `ABCC2` awosu mu abɔde mu. Nanso, Rotor syndrome fi nsakrae a ɛba `SLCO1B1` ne `SLCO1B3` awosu mu nkwaadɔm mu .

Ade foforo a ɛho hia sɛ yɛhyɛ no nsow ne sɛ Rotor Syndrome betumi aka ɔkwan a obi nipadua fa so di nnuru bi ho dwuma no kɛse. Enti, sɛ wo anaa wo ba wɔ Rotor Syndrome a, ɛho hia yiye sɛ wobɔ wo duruyɛfo amanneɛ ansa na woakyerɛw nnuru bi ama wo.

Bio nso, Rotor Syndrome mma mmerɛbo no kɔla nsakra. Nanso, ɛte sɛ nea nnipa a wɔwɔ Dubin-Johnson Syndrome mmerɛbo yɛ tuntum .

Ɛyɛ ade a ɛfata sɛ wobɛte dadwen nka bere a wo anaa wo ba no honam ani yɛ kɔkɔɔ no. Bere a jaundice abu so wɔ mmofra a wɔawo wɔn foforo mu no, jaundice a Dubin-Johnson syndrome de ba no yɛ tebea a ɛkɔ so tra hɔ, na ɛtra hɔ daa.

Nea etwa to no, nneɛma a ɛsɛ sɛ wokae (Take-Home Message) .

Enti, seesei wunim Dubin-Johnson yare no ho nsɛm pii. Mma ɛnhaw wo, ɛnyɛ tebea a emu yɛ den. Wo duruyɛfo bɛkyerɛkyerɛ ayaresa a ehia na ama w’ani ne wo honam ani yɛ kɔkɔɔ no so atew, anaasɛ sɛ wunya sɛnkyerɛnne afoforo a. Ade a ɛho hia sen biara ne sɛ wo ne wo duruyɛfo bɛkasa afa wo sɛnkyerɛnne ahorow ho daa na woahwɛ ahu sɛ wowɔ apɔwmuden. Wubetumi de tebea yi abɔ bra a ɛfata na anigye wom.

` Dubin-Johnson syndrome, atiridii kɔkɔɔ, mmerɛbo, bilirubin, awosu mu nyarewa, honam ani kɔkɔɔ, jaundice