So wowɔ yare bi a ɛyɛ nwonwa a wuntumi nte ase? Ebetumi ayɛ Acute Hepatic Porphyria!

So wote nka sɛ wo yafunu mu yɛ wo yaw a wuntumi nsusuw ho, ɛtɔ mmere bi a wo koma mu yɛ duru, na wo nsa ne wo ho yɛ wo yaw? Ebia nnuruyɛfo mpo ntumi nhu nea ɛyɛ. Saa sɛnkyerɛnne ahorow a ɛyɛ nwonwa a ɛte sɛ nea enni abusuabɔ yi mu biako a yɛbɛka ho asɛm nnɛ ne Acute Hepatic Porphyria (AHP). Mma ɛnhaw wo, eyi yɛ nea ɛyɛ den kakra, nanso momma yɛnte ase kɛkɛ.

Dɛn ankasa ne acute hepatic porphyria (AHP)?

Sɛ yɛbɛka no tiawa a, AHP yɛ awosu mu yare a ɛntaa nsi koraa . Efi ase wɔ wo mmerɛbo mu . Nanso ɛka wo ntini ahorow nso , na ɛde sɛnkyerɛnne ahorow ba wo nipadua nyinaa mu. Susuw ho hwɛ, biribi wɔ hɔ a wɔfrɛ no heme, a ɛyɛ hemoglobin a ɛwɔ yɛn mogya mu no fã. Yɛn nipadua hia enzyme ahorow na ama yɛatumi ayɛ saa heme yi. Obi a ɔwɔ AHP no nni enzyme ahorow a ehia na ama wɔayɛ heme yi bi, anaasɛ onni bi.

Sɛ eyi ba a, dɛn na ɛba? Nnuru a anka ɛsɛ sɛ wɔde yɛ heme no bi aka. Yɛfrɛ saa nkae yi porphyrin precursors . Sɛ eyinom dɔɔso a, ɛyɛ awuduru ma nipadua no. Wɔ AHP mu no, awuduru yi di kan boaboa ano wɔ mmerɛbo no mu. Afei, sɛ wɔkɔ mogya no mu na wɔne ntini ahorow no di nkitaho a, sɛnkyerɛnne ahorow no fi ase da adi. So wote ase?

So AHP ahorow ahorow wɔ hɔ?

Yiw, AHP ahorow atitiriw anan na ɛwɔ hɔ. Wɔakyekyɛ saa ahorow anan yi mu ayɛ no enzyme ahorow a madi kan aka ho asɛm no, a egyina enzyme a enni mu so. Wɔde ɔfã a wɔfrɛ no "hepatic" din too ɔkwan biara so efisɛ efi ase wɔ mmerɛbo mu, ne ɔfã a wɔfrɛ no "acute" efisɛ sɛnkyerɛnne ahorow no da adi mpofirim. Sɛnea wɔahyehyɛ no fi nea abu so sen biara so kosi nea ɛntaa nsi so no, ahorow no ni:

1. Acute Intermittent Porphyria (AIP): Eyi ne nea abu so sen biara. Nsakrae bi a ɛba HMBS awosu mu abɔde mu nkwaadɔm mu no ma enzyme hydroxymethylbilane synthase (HMBS) (a wɔsan frɛ no porphobilinogen deaminase (PBGD)) no ho yɛ na. Eyi betumi ayɛ awosu mu nsakrae a ɛba foforo, anaasɛ wobetumi anya afi awo mu sɛ autosomal dominant trait (i.e., yare no betumi aba sɛ ɔwofo biako pɛ na onya awosu mu nkwaadɔm no mpo a).

2. Variegate Porphyria (VP): Nsakrae bi a ɛba PPOX awosu mu abɔde mu no ma enzyme protoporphyrinogen oxidase (PPO anaa PPOX) no ho yɛ na. Eyi nso betumi afi awo mu sɛ autosomal dominant anaa autosomal recessive.

3. Hereditary Coproporphyria (HCP): Nsakrae a ɛba awosu mu nkwaadɔm `CPOX` mu no ma enzyme `coproporphyrinogen oxidase (CPOX) a enni hɔ.` Eyi nso betumi anya afi awo mu sɛ autosomal dominant anaa autosomal recessive.

4. ALAD-a Ɛho Nni Porphyria (ALAD-Nnipa Porphyria - ADP):Nsakrae a ɛba `ALAD` awosu mu abɔde mu no ma enzyme `delta-aminolevulinic acid dehydratase (ALAD) no ho yɛ na.` Ɛyɛ awo wɔ `autosomal recessive` kwan so (i.e., sɛ awofo baanu no nyinaa nya awosu mu abɔde no nkutoo a, yare no betumi aba).

Ɔkwan bɛn so na AHP ka me?

Eyi yɛ nwonwa, efisɛ AHP nka obiara pɛpɛɛpɛ. Nnipa binom wɔ awosu mu nkwaadɔm mu nsakrae a ɛma ɛba saa nanso wonnya sɛnkyerɛnne biara da . Ebia afoforo nso benya "ntua" a ɛkyerɛ sɛnkyerɛnne ahorow a ɛba pɛnkoro anaa mprenu pɛ wɔ wɔn nkwa nna mu. Nanso nnipa binom taa nya saa ntua yi, na afoforo nso nya bi bere tenten , a ɛkyerɛ sɛ wɔkɔ so nya sɛnkyerɛnne ahorow a ɛka wɔn da biara da asetra. Ɛtɔ da bi a, ntua betumi ayɛ den araa ma ebia ɛho behia sɛ wɔde wo kɔ ayaresabea dan a wɔhwɛ ntɛmpɛ so ntɛm ara. Ebia wobɛte ntini mu yaw a emu yɛ den, wo honam ani nsakrae, ne sɛnkyerɛnne ahorow a ɛfa ntini ahorow ho.

Hena na ɛda adi kɛse sɛ obenya eyi?

AHP betumi aka nnipa a wofi abusuakuw anaa kɔla biara mu. Wobetumi anya afi awofo biako anaa wɔn baanu nyinaa a wɔwɔ awosu mu nkwaadɔm mu nsakrae no hɔ. Nea ɛyɛ nwonwa no, nnipa pii nnya yare no ho sɛnkyerɛnne ahorow . Sɛ sɛnkyerɛnne ahorow bɛba a, awosu mu nkwaadɔm mu nsakrae no nkutoo nnɔɔso. Ɛsɛ sɛ `triggers` afoforo pii nso wɔ hɔ.

Dɛn ne nneɛma a ɛkanyan adwene yi?

• Nsakrae a ɛba nipadua mu nkwaadɔm mu (titiriw wɔ mmerantebere ne mmabaabere, nyinsɛn, ne brayɛ mu) .
• Nnuru bi
• Nsã a wɔnom
• Sigaretnom
• Adwennwen a emu yɛ den
• Aduan a wɔde di dwuma denneennen (titiriw calorie anohyeto) .

Ɛsɛ sɛ yɛhyɛ no nsow nso sɛ wɔn a wonya yare no ho sɛnkyerɛnne no mu 80% yɛ mmea a wɔadi mfe a wobetumi awo .

AHP abu so dɛn?

Ɛyɛ den sɛ yɛbɛka no pen, efisɛ mpɛn pii no, wonhu yare yi yiye. Wɔ wiase nyinaa no, wobu akontaa sɛ ebia nnipa 100,000 biara mu bɛyɛ 5 na wɔwɔ bi. Wɔ ahorow a yɛadi kan aka ho asɛm no mu no, `Acute Intermittent Porphyria (AIP)` ne ɔkwan a wohu wɔ nsɛm a wɔabɔ ho amanneɛ no mu 80% pɛ mu. `ALAD-Deficiency Porphyria (ADP)` ne nea ɛntaa nsi, na wɔabɔ amanneɛ sɛ nnipa 9 pɛ na wɔanya bi wɔ wiase nyinaa. Fa no sɛ, nnipa 10 biara a wɔwɔ awosu mu nkwaadɔm mu nsakrae a ɛne AHP wɔ abusuabɔ no mu biako pɛ na obenya yare no ho sɛnkyerɛnne.

Dɛn ne AHP ho sɛnkyerɛnne ahorow?

AHP ho sɛnkyerɛnne ahorow no yɛ nwonwa yiye. Ebetumi ayɛ den ama ayarefo ne nnuruyɛfo sɛ wɔbɛte ase, efisɛ sɛnkyerɛnne ahorow no betumi ada adi mpofirim, emu yɛ den, na ɛte sɛ nea ɛne wɔn ho wɔn ho nni abusuabɔ.

Nneɛma yi bi ni:

Ntini mu yaw

Saa ɛyaw yi betumi aba wɔ nipadua no mmeae ahorow:

• Yafunu mu yaw a emu yɛ den – Eyi ne sɛnkyerɛnne a edi kan a ɛba nnipa dodow no ara so.
• Koko mu yaw
• Akyi yaw
• Ɛyaw a ɛba nsa ne anan mu

Nnuan a wɔyam no mu haw ahorow

Esiane sɛ ntini ahorow no ka nti, ɔhaw ahorow nso betumi aba wɔ aduan a wɔyam no mu:

• Akisikuru ne ɔfe
• Nnuan a wɔmfa nni dwuma yiye
• Ayamtim
• Ayamtuo

Central nervous system ho sɛnkyerɛnne ahorow

Eyinom ne amemene ne adwene wɔ abusuabɔ tẽẽ:

• Brɛ
• Asoroben
• Hahaahayɔ
• Delirium a ɛma obi ho kyere no
• Hallucinations – nneɛma a wohu anaa wote a enni hɔ ankasa
• Gyinabea epilepticus/akisikuru (`Akisikuru`) .

Ntini a ɛwɔ akyi no ho sɛnkyerɛnne

Eyinom ne ntini ahorow a ɛwɔ nipadua no afã afoforo no wɔ abusuabɔ:

• Ntini mu mmerɛwyɛ
• Akwaa a ɛyɛ mmerɛw na ɛyɛ mmerɛw wɔ akwaa no mu
• Ɔbrɛ
• Nkate mu adehwere
• Ntini a ɛyɛ mmerɛw
• Ɔhome mu obubuafo – Eyi yɛ asiane kɛse na ebetumi ama ahome ayɛ den.

Autonomic nervous system ho sɛnkyerɛnne ahorow

Eyinom yɛ nneɛma a ɛba ara kwa, nneɛma a yenni so tumi:

• Koma a ɛbɔ denneennen
• Mogya mmoroso

Honam ani sɛnkyerɛnne (titiriw wɔ `Variegate Porphyria` ne `Hereditary Coproporphyria` mu) .

Nnipa a wɔwɔ saa ahorow abien yi betumi anya honam ani haw bere a wɔde wɔn ho hyɛ owia mu no :

• Owia hann a ɛma obi te nka
• Blistering a ɛyɛ akisikuru
• Wo honam ani kɔla a ɛsakra (Pigmentation) .
• Akuru a ɛwɔ hɔ

Ade a ɛho hia sen biara ne sɛ sɛ wowɔ porphyria a, ebia wo nsu no kɔla bɛsakra . Sɛ saa porphyrin a edi kan a mekaa ho asɛm no boaboa ano wɔ wo nipadua mu na woyi fi wo nsu mu a, wo nsu betumi adan kɔla kɔkɔɔ-akuturuku . Asɛmfua "porphyria" no fi Hela asɛmfua "porphura," a ɛkyerɛ "kɔkɔɔ." Porphyrin a ɛwɔ yɛn mogya mu nkwammoaa kɔkɔɔ mu no na ɛma mogya kɔla kɔkɔɔ.

Dɛn ne AHP ntua?

Wɔ nnipa pii a wɔwɔ AHP sɛnkyerɛnne fam no, eyinom ba wɔ "ntua" a ɛba bere ne bere mu. Nnipa binom ntaa nhu, na ebinom nso ntaa nhyia. Nnipa binom hyia wɔn kɛse, bere a afoforo nso nnya bi. Mpɛn pii no, saa ntua yi kɔ so nna kakraa bi, na afei ɛkɔ soro nkakrankakra na ɛso tew. Sɛ wowɔ sɛnkyerɛnne ahorow a emu yɛ den a ɛyɛ hu a, ɛsɛ sɛ wokɔ ayaresabea. Sɛnea mekae no, nneɛma te sɛ ɔhome mu yare a ɛma ɛyɛ den sɛ wɔbɛhome no yɛ tebea horow a ɛhwehwɛ sɛ wɔde wɔn kɔ ayaresabea ntɛm ara.

AHP ntua ho sɛnkyerɛnne a ɛtaa ba ne yafunu mu yaw a emu yɛ den a wontumi nkyerɛkyerɛ mu.. Nnipa a wɔwɔ AHP bɛboro 90% kɔ oduruyɛfo nkyɛn esiane yafunu mu yaw yi nti. Nanso, esiane sɛ nneɛma pii na ɛde yafunu mu yaw ba, na esiane sɛ X-ray anaa CT scan ntumi nhu nea ɛde ɛyaw yi ba (efisɛ ɛyɛ ntini mu yaw nti) nti, ɛyɛ den yiye sɛ wobehu. Esiane sɛ nneɛma te sɛ akisikuru, akisikuru, ne akisikuru ka yafunu mu yaw yi ho nti, ebia nnuruyɛfo besusuw sɛ ɛyɛ aduan mu yare. Sɛ wowɔ adwene ne nkate mu haw nso a, ebia wobesusuw nso sɛ ɛyɛ ntini mu yare. Sɛ sɛnkyerɛnne ahorow a ɛte sɛ nea enni abusuabɔ yi ka bom a, ɛho hia sɛ wohu oduruyɛfo a onim ho asɛm na ama wahu sɛ ebia ɛyɛ porphyria.

Nsɛnkyerɛnne a enni sabea bɛn na AHP betumi de aba?

Nnipa a wɔwɔ yare a emu nyɛ den (enzyme a wonni pii) ntaa nnya yare no ho sɛnkyerɛnne a enni sabea. Ebia wɔbɛtow ahyɛ wɔn so pɛnkoro anaa abien pɛ wɔ wɔn nkwa nna mu. Sɛ wohu nea ɛkanyan wɔn a, wobetumi akwati. Nanso, nnipa binom taa nya ntua, na yare no ho sɛnkyerɛnne bi taa ba araa ma wobetumi abu no sɛ ɛyɛ nea enni sabea. Nsɛnkyerɛnne a ɛtete saa no bi ne:

• Yeaw
• Ɔbrɛ
• Abofono
• Neuropathy ( akwaa a ɛyɛ mmerɛw, ɛyaw, anaa mmerɛwyɛ wɔ akwaa mu) .

Dɛn ne nsɛnnennen a ɛwɔ AHP mu?

Nsɛnkyerɛnne a ɛfa ntini a ɛwɔ mfinimfini no ho (te sɛ dadwen, nsusuwii hunu, ne akisikuru) taa ba bere a ntua bi aba nkutoo. Nanso, sɛnkyerɛnne ahorow a ɛfa ntini a ɛwɔ akyi no ho (te sɛ ntini a ɛyɛ mmerɛw ne obubuafo) betumi aba mpɛn pii, na ebetumi asɛe daa wɔ ntua a emu yɛ den akyi. Porphyrin a edi kan yi a ɛboaboa ano nso betumi asɛe akwaa ahorow bi bere tenten. Ebia eyinom bi ne:

• Mogya mmoroso a enni sabea
• mmerɛbo mu yare a enni sabea
• Asaabo mu yare a enni sabea
• mmerɛbo mu kokoram titiriw
• Adwenemhaw ne dadwen

Dɛn na ɛde AHP ba?

Nea ɛde AHP ba titiriw ne awosu mu nsakrae . Nanso sɛnea madi kan aka no, sɛnkyerɛnne ahorow no mpue ntɛm ara bere a awosu mu nsakrae no aba akyi. Mpɛn pii no, sɛnkyerɛnne ahorow no di kan da adi wɔ mmerantebere ne mmabaabere mu, ne nsakrae a ɛba nipadua mu nkwaadɔm mu. Nea ɛka ho no, nnuru bi, nnubɔne, nsa a wɔnom boro so, ahoɔdennuru a wɔde siw ano kɛse, ne nyarewa a ɛde adwennwen kɛse ba nipadua no so nso betumi akanyan no.

Nea nneɛma a ɛkanyan saa yi nyinaa yɛ pɛ ne sɛ ɛkanyan mmerɛbo no heme a ɛyɛ no. Nanso, wɔ obi a ɔwɔ AHP mmerɛbo mu no, nneɛma a efi heme a wɔyɛ mu ba (te sɛ porphyrin a edi kan) no ntumi nkɔ nipadua mu yiye, kyerɛ sɛ, wɔde di dwuma. Enti, saa porphyrin a edi kan a aka yi boaboa ano wɔ mmerɛbo no mu. Ɛyɛ bere a eyinom boaboa ano kodu baabi a efi ase ka ntini ahorow no na ɛde sɛnkyerɛnne ahorow ba.

Ɔkwan bɛn so na wohu sɛ ɔwɔ AHP? (Nyare a Wɔahu) .

Wɔn a wɔwɔ AHP no famSɛ́ wobehu yare no yiye no betumi ayɛ den, efisɛ ɛnyɛ tebea yi nkutoo na sɛnkyerɛnne ahorow no wɔ na ebia ɛte sɛ nea ɛne no nni abusuabɔ. Nanso, oduruyɛfo a onim AHP betumi asusuw sɛ AHP wɔ hɔ sɛ wowɔ yafunu mu yaw ka ntini a ɛwɔ mfinimfini ne akyi no ho sɛnkyerɛnne ahorow ho a. Wobu saa sɛnkyerɛnne abiɛsa yi a wɔaka abom sɛ ɛyɛ "classic triad" a ɛwɔ AHP mu.

Sɛ oduruyɛfo bi susuw sɛ AHP wɔ hɔ a, ebia wɔbɛyɛ nhwehwɛmu pii de asi so dua.

• Nsu mu nhwehwɛmu: Wobetumi ayɛ eyi ntɛmntɛm bere a ntua bi rekɔ so no. Ebia nsuo bɛyɛ nea ɛfata bere a worennya ntua, nanso bere a ntua bi rekɔ so no, nsu no bɛda porphyrin precursors (PBG ne ALA) dodow a ɛkɔ soro adi. Eyi nyɛ sɔhwɛ a wɔyɛ ma AHP nkutoo, nanso ɛyɛ nhwehwɛmu titiriw a ebetumi aboa nnuruyɛfo ma wɔakyerɛ wo ɔkwan pa so.
• Awosu mu nhwehwɛmu: Eyi ne ɔkwan a eye sen biara na ɛyɛ pintinn sen biara a wɔfa so hu AHP (`sika kɔkɔɔ gyinapɛn`) . Sɛ́ ebia wowɔ sɛnkyerɛnne ahorow anaasɛ wunni bi no, ebetumi asi so dua sɛ ebia wowɔ awosu mu nkwaadɔm mu nsakrae a ɛne AHP wɔ abusuabɔ. Ebetumi nso akyerɛ wo AHP ko a wowɔ ne sɛnea enzyme a enni mu no mu yɛ den no. Eyi hwehwɛ sɛ wɔfa mogya anaa nnompe. Ebia ɛho behia nso sɛ ayaresabea a ɛwɔ wo mpɔtam hɔ no de nhwɛsode no kɔma aduruyɛdan titiriw bi.

Ɔkwan bɛn so na wɔsa AHP?

AHP ayaresa twe adwene si ntua a wobesiw ano na wɔadi so so . Ebia ɛho behia sɛ wɔde wo kɔ ayaresabea bere a ntua bi reba no. Ebia wubehia nnuru ahorow na ama wo yare no ho sɛnkyerɛnne so atew. Wɔ mmere a wunnya ntua mu no, ebia wubehia nnuru afoforo a wode bedi nneɛma a ɛkanyan wo a ɛma wo sɛnkyerɛnne no mu yɛ den no so. Sɛ wo ne wo duruyɛfo bom hu nneɛma a ɛkanyan wo a ɛka wo kɛse a, w’ayaresa no bɛyɛ nea etu mpɔn kɛse.

Ayaresa a wɔde sa ntua a emu yɛ den:

• Hemin a wɔde twitwiw nipadua no mu: Sɛ ɛba sɛ obi nya ntua a emu yɛ den a, oduruyɛfo betumi de hemin ahyɛ wo ntini bi mu. Eyi boa ma porphyrin dodow a ɛwɔ wo mogya mu no so tew. Hemin yɛ ade a wogye fi mogya mu nkwammoaa kɔkɔɔ mu na ɛtew porphyrin dodow a ɛwɔ wo nipadua mu no so.
• Ɛyaw ano aduru: Wɔ ntua a emu yɛ den mu no, ebia ɛho behia sɛ wɔde nnuru a ano yɛ den a wɔde kum ɛyaw te sɛ opioid.
• Phenothiazines: Wɔde eyinom di dwuma de siw akisikuru ne ɔfe a emu yɛ den ano.
• Nsu a wɔde fa IV mu ne aduannuru a wɔde ma: Nsɛnkyerɛnne te sɛ yafunu mu yaw, akisikuru, ɔfe, akisikuru, ne akisikuru betumi ama nipadua no ahwere calories ne nsu bere a ntua aba no. AHP nso betumi ama nneɛma te sɛ sodium ne magnesium ayera. Enti, wobetumi de nsu a carbohydrates ne electrolytes wom ahyɛ ntini mu.
• Nnuru a wɔde sa akisikuru:Ebia ayarefo bɛyɛ 20% behia ayaresa ama akisikuru bere a ntua rekɔ so no.

Ayaresa a wɔde bere tenten apaw:

• Hemin a wɔde siw ano: Sɛ wɔde hemin dodow a ɛba fam ma nnipa a wɔtaa nya ntua pɛnkoro dapɛn biara a, ebetumi asiw porphyrins a ɛbɛboaboa ano no ano.
• Givosiran (GIVLAARI®): Eyi yɛ awosu mu ayaresa bi . Ɛtew porphyrin a edi kan a wɔyɛ no so. Mprempren wɔapene so sɛ wɔde twitwiw wɔn ɔsram biara de siw AHP ho sɛnkyerɛnne ano wɔ nnipa a wɔtaa nya ntua no so.
• Hormone ayaresa: Sɛ wo AHP ntua no fi wo brayɛ mu a, ebia wo duruyɛfo bɛkyerɛw nnuru te sɛ GnRH (Gonadotropin-releasing hormone) analogs. Eyinom brɛ nipadua no mu ahoɔdennuru a wɔfrɛ no estrogen ne progesterone no ase.
• Mogya mmoroso nnuru: Sɛ mogya mmoroso a enni sabea ba a, ebehia sɛ wɔde nnuru pɔtee bi siw ano.
• Berɛbo a wɔde ma: Nnipa a wɔtaa nya ntua a ɛde wɔn nkwa to asiane mu na wɔnyɛ ayaresa afoforo ho biribi no betumi asusuw mmerɛbo a wɔde bɛma afoforo ho. Eyi betumi asa yare no koraa mpo.

So wobetumi asiw AHP ano?

Ɛrentumi nyɛ yiye sɛ wobesiw yare no ano. Nanso, wubetumi aboa ma wɔasiw sɛnkyerɛnne ahorow ano denam nneɛma a ɛkanyan no a wobɛkwati so . Sɛ woanhyia ntua da a, ɛbɛyɛ papa sɛ wobɛkwati nneɛma a ebetumi akanyan wo nyinaa. Sɛ woanya ntua na woahu nneɛma a ɛkanyan wo a, ebia ɛbɛdɔɔso sɛ wobɛkwati saa nneɛma pɔtee a ɛkanyan wo no nkutoo.

Nneɛma a ɛkanyan obi a wɔtaa hu no bi ni:

Nnuru ahorow a wɔde di dwuma:

• Antihistamines (nnuru bi a wɔde sa atiridiinini ne akisikuru) .
• Nnuru a ɛma obi dwo
• Nnuru a wɔde siw nyinsɛn ano a wɔnom
• Ayaresa a wɔde si hormone ananmu

Nneɛma a wɔde di dwuma:

• Tawa (`Tawa`) .
• Wii a wɔde di dwuma
• Nsaden
• Nnubɔne a wɔde gye wɔn ani

Ɔbrɛ:

• Nyarewa a wɔde nyarewa ba
• Sɛɛgyiri
• Calorie a ɛba fam a wodi (titiriw carbohydrate a ɛba fam) (`Calorie a wonnya`) .
• Adwene mu nhyɛso

Dɛn ne daakye ma nnipa a wɔwɔ AHP? (Nkɔmhyɛ) .

Eyi gu ahorow kɛse wɔ onipa biara mu. Nnipa pii nnya yare no ho sɛnkyerɛnne biara da . Wɔn a wɔyɛ saa no mu no, nnipa dodow no ara nya ntua biako anaa kakraa bi pɛ wɔ wɔn nkwa nna mu. Ɛwom sɛ ntua betumi de wɔn nkwa ato asiane mu de, nanso nnipa dodow no ara ho tɔ wɔn koraa denam ayaresa a wɔde ma ntɛm so . Nnipa a wɔwɔ AHP no mu bɛyɛ 5% na wɔtaa nya sɛnkyerɛnne ahorow anaasɛ enni sabea. Wɔ saa nnipa yi fam no, nnuru a wɔde siw yare ano no taa boa, na wobetumi abu mmerɛbo a wɔde bɛma obi foforo sɛ ɔkwan a etwa to.

Mɛyɛ dɛn ahwɛ me ho bere a me ne AHP te no?

• Kwati nneɛma a ɛtaa kanyan obi. Ɛsɛ sɛ nsa, sigaretnom, ne nnubɔne di kan wɔ nneɛma a ɛsɛ sɛ wokwati no mu. Ebia wobɛpɛ nso sɛ wo ne wo duruyɛfo kasa fa nnuru foforo a wode besi nnuru a wonom no bi ananmu ho.
• Di aduan a ahoɔden wom na ɛkari pɛ. Kwati adidi a ɛtra so ne aduan a carb kakraa bi nnim ho nhyehyɛe. Sɛ woredi mmuada ansa na woayɛ aduruyɛ mu nhwehwɛmu anaa oprehyɛn a, wo ne wo duruyɛfo nka ho asɛm.
• Kɔ aduruyɛ mu nhwehwɛmu daa. Sɛ wunni sɛnkyerɛnne biara mpo a, wo duruyɛfo bɛhwɛ wo mmerɛbo, w’asaabo, ne wo mogya mmoroso daa.

Hepatic porphyria a emu yɛ den yɛ yare a ɛntaa nsi, enti nnipa dodow no ara nnim ho hwee. Sɛ ntua bi ba mpofirim a yare no ho sɛnkyerɛnne a emu yɛ den ba a, ɛyɛ basaa kɛse na ɛyɛ hu. Nnipa binom de mfe pii bɔ mmɔden sɛ wobehu nea ɛhaw wɔn ne nea wobetumi ayɛ. Sɛ wususuw sɛ wowɔ AHP a, awosu mu nhwehwɛmu betumi asi so dua. Sɛ wuhu wie a, ɛbɛyɛ mmoa kɛse ama wo sɛ wubesiw ntua ano na woadi so.

Nneɛma a ɛho hia sen biara a ɛsɛ sɛ wuhu (Take-Home Message) .

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

• AHP yɛ awosu mu yare a ɛntaa nsi a efi ase wɔ mmerɛbo mu na ɛka ntini ahorow .
• Eyi fi enzyme ahorow a ehia wɔ heme , a ɛma nneɛma a awuduru wom a wɔfrɛ no porphyrin precursors boaboa ano wɔ nipadua no mu no a enni hɔ.
• Nsɛnkyerɛnne no betumi egu ahorow, efi yafunu mu yaw a emu yɛ den, ntini mu yaw, adwenemhaw, honam ani haw, ne nsakrae a ɛba wo nsu mu kɔla mpo so.
• Sɛ awosu mu nsakrae no wɔ hɔ mpo a, ɛntaa mma sɛ sɛnkyerɛnne ahorow no da adi a biribiara a ɛkanyan no ( te sɛ hormone, nnuru bi, nsa, ne adwennwen).
• Nsu mu nhwehwɛmu ne awosu mu nhwehwɛmu ho hia na ama wɔahu yare no.
• Ade titiriw ne sɛ wɔbɛfa ayaresa so adi ntua so na asiw ano . Wɔde `Hemin` vaccine, nnuru a wɔde kum ɛyaw, ne nnuru titiriw bi na edi dwuma. Wɔ tebea horow a emu yɛ den sen biara mu no, mmerɛbo a wɔde ma obi foforo nso yɛ ano aduru.
• Wubetumi ne yare no atra ase yiye denam nneɛma a ɛkanyan no a wobɛkwati, asetra pa a wobɛbɔ, ne aduruyɛ mu nhwehwɛmu a wobɛkɔ so daa no so .

Mewɔ anidaso sɛ saa nsɛm yi bɛboa wo. Kae sɛ, sɛ wowɔ saa sɛnkyerɛnne yi bi a, nsuro sɛ wobɛkɔ oduruyɛfo nkyɛn akɔka ho asɛm.

` Acute hepatic porphyria, AHP, mmerɛbo, ntini nhyehyɛe, awosu mu nyarewa, porphyrins, sɛnkyerɛnne, ayaresa