Kpɔe ɖa le susu me be èse ƒodo me vevesese kpata le susu aɖeke manɔmee, si kpe ɖe wò abɔwo kple afɔwo ƒe ʋuʋu alo lãmetutudɔ, lãmekawo ƒe gbɔdzɔgbɔdzɔ, kple ʋuʋudedi ŋu. Le ɖɔkta geɖe gbɔ yiyi kple dodokpɔ geɖe wɔwɔ vɔ megbe gɔ̃ hã la, womenya nusi gbɔ wòtso o. Ðe wò ŋutɔ alo ame aɖe si nènya la to nusia me kpɔa? Ate ŋu anye domenyiŋusẽfianu ƒe nɔnɔme si mebɔ o si dzi woŋea aɖaba ƒuna zi geɖe. Egbea la, míele nɔnɔme sia me dzrom: Acute Hepatic Porphyria , alo AHP.
Nuka Tututue Nye Acute Hepatic Porphyria (AHP)?
AHP fia domenyiŋusẽfianu ƒe dɔléle siwo mebɔ o, siwo ƒe dome wonyi, siwo gblẽa nu le wò lãmekawo kple wò ŋutigbalẽ ŋu ɣeaɖewoɣi. Ƒã ŋutɔ, eye wòdzea ame atɔ̃ le ame 100,000 ɖesiaɖe me. Nɔnɔme sia ate ŋu ahe lãmesẽkuxiwo vɛ kpata, siwo nu sẽ, eye woate ŋu ade ame ƒe agbe afɔku me.
Be míase nusitae gɔme la, mina míalé ŋku ɖe wò ʋu ŋu. Protein aɖe si woyɔna be Hemoglobin le míaƒe ʋumenugbagbevi dzĩwo me. Eƒe dɔ vevitɔe nye be wòafɔ ɔksidzin tso wò lãkusi me atsɔe ayi wò ŋutinuwo kple lãmekawo gbɔ—abe oxygen nana dɔwɔƒe tɔxɛ aɖe ene.
Be wò ŋutilã nate ŋu awɔ protein sia la, ehiã na akpa vevi aɖe si woyɔna be Heme .
Le ame ɖekaɖeka siwo ŋu AHP le gome la, enzyme aɖe koŋ si hiã le Heme wɔwɔ me la mehiã o alo mebu o. Bu eŋu abe agbagbadzedze be yeaɖa nuɖaɖa aɖe gake nu vevi aɖe to ŋuwò ene; womate ŋu awɔ nu mamlɛtɔ si wowɔ la nyuie o.
Enzyme sia ƒe anyimanɔmanɔ dzɔna le aklã me koŋ , si woyɔna hã be aklã ƒe dɔwɔwɔ. Esia tututu tae wotsɔ ŋkɔ na dɔlélea be Acute Hepatic Porphyria .
Ne wò ŋutilã mete ŋu wɔ Heme o la, atike siwo tua ame ɖo—vevietɔ aɖi siwo do ŋgɔ na wo abe Porphobilinogen (PBG) kple Aminolevulinic acid (ALA) ene—dzea ƒoƒo ɖe aklãa me gɔme. Aɖi siawo gena ɖe ʋukawo me eye woate ŋu ato ŋutilã bliboa me, eye woagblẽ nu le wò lãmekawo ŋu koŋ. Lãmetsiŋusẽ ƒe gbegblẽ siae hea dzesiwo abe vevesese sesẽ , lãmetutudɔ , kple ʋuʋudedi ene vɛ .
Nukae Nye AHP Ƒomevi Veviwo?
AHP ƒomevi vevi enee li. Togbɔ be wo dometɔ ɖesiaɖe lɔ enzyme vovovo aɖe ƒe anyimanɔmanɔ ɖe eme le Heme wɔwɔ me hã la, wo ame eneawo katã kpɔa ŋusẽ ɖe aklã kple lãmekawo dzi koŋ.
Porphyria si Dzɔna le Ɣeyiɣi aɖe Me (AIP) .
Esiae nye esi bɔ wu, si gblẽa nu le AHP dɔnɔwo ƒe 80% ŋu. Hydroxymethylbilane synthase (HMBS) enzyme ƒe anyimanɔmanɔ gbɔe wòtsona, si tsoa tɔtrɔ si va le HMBS domenyiŋusẽfianu me gbɔ. Esia ate ŋu anyi tso dzila gbɔ, alo ate ŋu adzɔ abe domenyiŋusẽfianu ƒe tɔtrɔ si dzɔ le vome ene si me ƒome ŋutinya aɖeke mele do ŋgɔ o.
Aleke AHP Se le eɖokui me?
AHP ƒe dzesiwo to vovo ŋutɔ. Ðewohĩ ame aɖewo makpɔ dzesi aɖeke gbeɖe o, evɔ ame bubuwo ya ate ŋu adze ame dzi enuenu vevie. Dzesi siawo nyanya le vevie ŋutɔ.
| Ŋutilã ƒe Dɔwɔɖoɖo si Ŋu Ŋusẽ Le | Dzesi Siwo Bɔ |
|---|---|
| Ƒodo | Ƒodo ƒe vevesese sesẽ si womate ŋu aɖe o (si nye dzesi vevitɔ ), ʋuʋudedi, ʋuʋudedi, kple dɔmeɖui. |
| Lãmetsiŋusẽ ƒe Dɔwɔɖoɖo | Abɔ kple afɔwo ƒe ʋuʋu alo ʋuʋudedi, lãmekawo ƒe gbɔdzɔgbɔdzɔ, lãmekawo ƒe vevesese, eye ƒã hafi wozu lãmetutudɔ. |
| Susu ƒe Lãmesẽ | Dzimaɖitsitsi, dzimaɖitsitsi, tɔtɔ, kple susumenuwo kpɔkpɔ. |
| Dzi & Ʋu ƒe sisi | Dzi ƒe tsotso kabakaba (tachycardia) kple ʋusɔgbɔdɔ. |
| Bubu | Aɖuɖɔ si do viviti, si biã, alo aŋutiɖiɖi (vevietɔ le amedzidzedze me). |
Esi wònye be AHP mebɔ o ta la, zi geɖe la, wobua eƒe dzesiwo vodadatɔe be wonye nɔnɔme siwo bɔ wu, si ate ŋu ahe dɔlélea didi ɖe megbe.
Nukae Hea Amedzidzedze?
Ame geɖe siwo ŋu AHP le la nɔa agbe si sɔ, gake nu aɖewo siwo hea dɔlélea vɛ ate ŋu ahe amedzidzedze kpata vɛ. Esiawo nyanya le vevie ŋutɔ:
- Atike Aɖewo: Vevietɔ barbiturates kple sulfonamide dɔlékuiwutikewo . Ne AHP le ŋuwò la, yi ɖɔkta gbɔ ɣesiaɣi hafi nàdze atike yeye ɖesiaɖe zazã gɔme.
- Ahanono: Nu vevi aɖe si hea AHP ƒe amedzidzedze vɛ.
- Nuɖuɖumeŋusẽ ƒe Mɔxexeɖenu/Nutsitsidɔ: Nuɖuɖu siwo me calorie kple carbohydrates mesɔ gbɔ ɖo o ate ŋu ahe dzesiawo vɛ.
- Lãmetsiŋusẽ ƒe Tɔtrɔ: Vevietɔ esiwo do ƒome kple nyɔnuwo ƒe dzinukpɔkpɔ.
- Nuteɖeamedzi Kple Dɔlékuiwo: Ŋutilã me nuteɖeamedzi, dzoxɔxɔ, alo dɔléle siwo bɔ abe dzamezã ene ate ŋu ahe nudzɔdzɔ aɖe vɛ.
Ɣekaɣie Wòle Be Nàkpɔ Ðɔkta?
Ne nu siwo gbɔna la dzɔ ɖe dziwò la, mègaɖe asi le wo ŋu o. Di atikewɔlawo ƒe aɖaŋuɖoɖo ne:
- Ƒodo me vevesese sesẽ si womate ŋu aɖe o si gbugbɔna vaa dziwò.
- Èkpɔa dzesi siwo do ƒome kple lãmekawo abe ʋuʋudedi alo abɔ kple afɔ ƒe gbɔdzɔgbɔdzɔ ene.
- Porphyria ƒe ƒome ŋutinya si wonya le asiwò.
- Ède dzesii be wò aɖuɖɔ trɔ zu dzĩ alo blɔ goglo.
Ne èto nɔnɔme kpata aɖe me, abe vevesese si womate ŋu ado dzi atsɔ o, gbɔgbɔ sesẽ, tɔtɔ kpata, alo wò abɔwo kple afɔwo ƒe ʋuʋu ƒe ŋutetemanɔamesi ene la, di beléle na wò enumake le dɔwɔƒe si te ɖe ŋuwò le nudzɔdzɔ kpatawo gbɔ kpɔƒe si te ɖe ŋuwò alo nàƒo ka na 911.
Ðe Atikewɔwɔ Li Na AHP?
Togbɔ be atike aɖeke meli na domenyiŋusẽfianu ƒe nɔnɔme sia o hã la, atikewɔmɔnu siwo wɔa dɔ ŋutɔ li siwo dzi woato akpɔ amedzidzedzewo gbɔ ahaxe mɔ ɖe enu.
Nudzɔdzɔ sesẽwo bia be woaxɔ ame ɖe kɔdzi be wòali ke.
- Mɔnu vevitɔe nye be woade dzesi nusi aʋãe ahaɖee ɖa.
- Wozãa vevesesenutsitike sesẽ tsɔ ɖua vevesese sesẽ dzi.
- Woŋlɔa atikewo na amewo be woatsɔ akpɔ ʋuʋudedi kple ʋuʋudedi gbɔ.
- Suklidɔ dodo ɖe ʋuka me ate ŋu akpe ɖe ame ŋu wòaxe mɔ ɖe aɖinuwo ƒe wɔwɔme nu le aklãa me.
- Atikewɔwɔ Tɔxɛ: Hemin dodo ɖe lãme na ame wɔa dɔ abe nusi axɔ ɖe aklã teƒe tẽ ene, si naa dzesi aklãa be wòadzudzɔ Heme ƒe ŋgɔdonya siwo gblẽa nu le ame ŋu la wɔwɔ fũ akpa.
- Tsɔ kpe ɖe eŋu la, le amesiwo dzea ame dzi enuenu gome la, domenyiŋusẽfianu ƒe ɖoɖo deŋgɔwo li fifia.
Wò ɖɔkta akpɔ atikewɔwɔ ƒe ɖoɖo si sɔ nyuie wu ɖe wò nuhiahiã tɔxɛwo nu.
Gbedasi si Wotsɔ Yi Aƒeme
- Acute Hepatic Porphyria (AHP) nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o, gake ne wokpɔ edzi la, ame geɖe nɔa agbe si me dzidzeme le, si sɔ.
- Dzesi si ɖe dzesi la nyea ƒodo ƒe vevesese sesẽ, si gbugbɔna yia edzi eye ahɔhɔ̃mekawo ƒe kuxiwo (ŋutete, gbɔdzɔgbɔdzɔ) kpena ɖe eŋu.
- Ƒo asa na nusiwo ʋãa ame—abe atike aɖewo, aha sesẽ nono, kple nutsitsidɔ ɣeyiɣi didi ene—le vevie ŋutɔ.
- Ne èsusu be dzesiwo le ye ŋu, alo yeƒe ƒome ŋutinya le ye si la, yi wò ɖɔkta gbɔ enumake.
- Atikewɔwɔ nyuiwo li be woatsɔ akpɔ dzesiawo gbɔ eye woaxe mɔ ɖe amedzidzedze siwo ava dzɔ le etsɔme nu. Mehiã be nàtsi dzodzodzoe o; wɔ dɔ kplikplikpli kple wò lãmesẽdɔwɔla.
AHP, Acute Hepatic Porphyria, Porphyria, ƒodo ƒe vevesese, ahɔhɔ̃mekawo ƒe dɔlélewo, domenyiŋusẽfianu ƒe dɔlélewo, heme, hemoglobin, porphyrin, dɔléle siwo mebɔ o
