Ðe dɔléle wɔnuku aɖe le ŋuwò si gɔme màte ŋu ase oa? Ate ŋu anye Acute Hepatic Porphyria!

Ðe nèsena le ɖokuiwò me be yeƒe dɔgbo le vevem si womate ŋu asusu o, eye ɣeaɖewoɣi la, dzi le kpekpem, eye wò abɔwo kple afɔwo hã vuvua? Ðewohĩ ɖɔktawo gɔ̃ hã bu be yewoanya nusi wònye. Dzesi wɔnuku siawo siwo dze abe ɖe womedo ƒome kplii o ene siwo ŋu míaƒo nu tsoe egbea dometɔ ɖekae nye Acute Hepatic Porphyria (AHP). Mègatsi dzi o, esia sesẽ vie, gake mina míase egɔme bɔbɔe.

Nuka tututue nye aklã me ʋumenugbagbeviɣidɔ sesẽ (AHP)?

Kpuie ko la, AHP nye domenyiŋusẽfianu ƒe dɔléle si mebɔ kura o . Edzea egɔme tso wò aklã me . Gake ekpɔa ŋusẽ ɖe wò lãmekawo hã dzi , si wɔnɛ be dzesiwo dzena le wò ŋutilã katã me. Bu eŋu kpɔ, nane li si woyɔna be heme, si nye hemoglobin si le míaƒe ʋu me ƒe akpa aɖe. Míaƒe ŋutilã hiã enzyme vovovowo be wòate ŋu awɔ heme sia. Enzyme siwo hiã be woatsɔ awɔ heme sia dometɔ aɖewo mesɔ gbɔ ɖe amesi ŋu AHP le si o, alo mehiãe o.

Nukae dzɔna ne esia dzɔ? Atike siwo wòle be woazã atsɔ awɔ heme hafi la dometɔ aɖewo susɔ. Míeyɔa nusiwo susɔ siawo be porphyrin ƒe ŋgɔdonyawo . Ne nusiawo ƒo ƒu la, wonyea aɖi na ŋutilãa. Le AHP me la, aɖi siawo ƒoa ƒu ɖe aklã me gbã. Emegbe ne woge ɖe ʋua me eye wowɔ nu kple lãmekawo la, dzesiawo dzea dzedze gɔme. Èse egɔmea?

Ðe AHP ƒomevi vovovowo lia?

Ẽ, AHP ƒomevi vevi enee li. Woma ƒomevi ene siawo ɖe enzyme ƒomevi siwo meyɔ va yi me, le enzyme si mehiã o nu. Wotsɔ ŋkɔ na ƒomevi ɖesiaɖe tso akpa si woyɔna be "aklã" elabena edzea egɔme tso aklã me, kple akpa si woyɔna be "acute" elabena dzesiawo dzena kpata. Le ɖoɖo si nu tso ƒomevi si bɔ wu dzi va ɖo esi mebɔ o dzi la, ƒomeviawoe nye esi:

1. Acute Intermittent Porphyria (AIP): Esiae nye esi bɔ wu. HMBS domenyiŋusẽfianu ƒe tɔtrɔ nana be enzyme si nye hydroxymethylbilane synthase (HMBS) (si woyɔna hã be porphobilinogen deaminase (PBGD)) meganɔa anyi o. Esia ateŋu anye domenyiŋusẽfianu ƒe tɔtrɔ yeye si dzɔ, alo ateŋu anyi eƒe dome abe autosomal dominant trait ene (i.e., dɔlélea ateŋu adzɔ ne dzila ɖeka koe nyi domenyiŋusẽfianu la dome gɔ̃ hã).

2. Variegate Porphyria (VP): PPOX domenyiŋusẽfianu ƒe tɔtrɔ nana be enzyme protoporphyrinogen oxidase (PPO alo PPOX) meganɔa anyi o. Woate ŋu anyi esia hã ƒe dome abe autosomal dominant alo autosomal recessive ene.

3. Domenyinu ƒe Coproporphyria (HCP): Dzɔdzɔmeŋusẽ ƒe tɔtrɔ le domenyiŋusẽfianu `CPOX` me hea enzyme `coproporphyrinogen oxidase (CPOX) ƒe anyimanɔmanɔ vɛ.` Woate ŋu anyi esia hã ƒe dome abe autosomal dominant alo autosomal recessive ene.

4. ALAD-Manɔamesi Porphyria (ALAD-Manɔamesi Porphyria - ADP):Dzɔdzɔmeŋusẽ ƒe tɔtrɔ le `ALAD` domenyiŋusẽfianu me hea enzyme `delta-aminolevulinic acid dehydratase (ALAD) ƒe anyimanɔmanɔ vɛ.` Wonyia dome le `autosomal recessive` mɔnu nu (i.e., ne dzila evea siaa nyi domenyiŋusẽfianu la dome ko hafi dɔlélea ate ŋu adzɔ).

Aleke AHP kpɔa ŋusẽ ɖe dzinyee?

Esia wɔ nuku ŋutɔ, elabena AHP mekpɔa ŋusẽ ɖe amesiame dzi sɔsɔe o. Domenyiŋusẽfianu ƒe tɔtrɔ le ame aɖewo ŋu na esia gake dzesi aɖeke medzea wo ŋu gbeɖe o . Ame bubuwo ateŋu akpɔ dzesi siwo dzena zi ɖeka alo zi eve ko le woƒe agbenɔɣi ƒe "amedzidzedze"wo. Gake ame aɖewo dzea amedzidzedze siawo enuenu, eye ame bubuwo hã xɔa wo atraɖii , si fia be dzesi siwo gblẽa nu le woƒe gbesiagbegbenɔnɔ ŋu la yia edzi nɔa wo ŋu. Ɣeaɖewoɣi la, amedzidzedze ate ŋu asẽ ale gbegbe be ahiã be woatsɔ wò ayi kɔdzi ƒe xɔ si me wokpɔa dɔnɔwo gbɔ kpata le. Àte ŋu ase veve vevie le lãmekawo me, ŋutigbalẽ ƒe tɔtrɔ, kple dzesi siwo do ƒome kple lãmekawo ƒe dɔwɔwɔ.

Amekae ate ŋu axɔ nusia wu?

AHP ate ŋu akpɔ ŋusẽ ɖe ameƒomevi alo amadede ɖesiaɖe me tɔwo dzi. Woate ŋu anyie tso dzila ɖeka alo evea siaa siwo si domenyiŋusẽfianu ƒe tɔtrɔ le gbɔ. Nukutɔe la, ame geɖe mekpɔa dzesi aɖeke o . Be dzesiawo nadze egɔme la, domenyiŋusẽfianu ƒe tɔtrɔ ɖeɖe mesɔ gbɔ o. Ele be `nu siwo ʋãa nu` bubu geɖewo hã nanɔ anyi.

Nukawoe nye nu siawo siwo ʋãa ame?

• Lãmetsiŋusẽ ƒe tɔtrɔ (vevietɔ le sɔhɛmenɔɣi, fufɔfɔ, kple dzinukpɔkpɔ me) .
• Atike aɖewo
• Ahanononono
• Sigaretnono
• Nuteɖeamedzi sesẽ
• Nuɖuɖu dzi ɖuɖu vevie (vevietɔ nuɖuɖumeŋusẽ ƒe agbɔsɔsɔ dzi ɖeɖe kpɔtɔ) .

Eɖe dzesi hã be amesiwo dzea dzesiawo ƒe 80% nye nyɔnu siwo xɔ ƒe si woate ŋu adzi vi .

Aleke gbegbee AHP bɔe?

Esesẽ be míagblɔe kple kakaɖedzi, elabena zi geɖe la, womedea dzesi dɔléle sia nyuie o. Le xexea me godoo la, wobu akɔnta be ame siwo ade 5 le 100,000 ɖesiaɖe me ate ŋu axɔe. Le ƒomevi siwo míeyɔ va yi dome la, `Acute Intermittent Porphyria (AIP)` ye nye ƒomevi si wokpɔna le dɔléle siwo ŋu woka nya ta le ƒe 80% ko me. `ALAD-Deficiency Porphyria (ADP)` ye nye ƒomevi si mebɔ o, eye ame 9 koe wogblɔ le xexeame katã. Bu eŋu kpɔ be ame 10 ɖesiaɖe si me domenyiŋusẽfianu ƒe tɔtrɔ si do ƒome kple AHP le la dometɔ ɖeka koe axɔ dzesiwo.

Nukae nye AHP ƒe dzesiwo?

AHP ƒe dzesiwo wɔ nuku ŋutɔ. Wo gɔmesese ate ŋu asesẽ na dɔnɔwo kple ɖɔktawo, elabena dzesiawo ate ŋu adze kpata, asẽ, eye wòadze abe ɖe womedo ƒome kple wo nɔewo o ene.

Nu siawo dometɔ aɖewoe nye esi:

Lãmetsiŋusẽ ƒe vevesese

Vevesese sia ate ŋu adzɔ le ŋutilãa ƒe teƒe vovovowo:

• Ƒodo ƒe vevesese sesẽ – Esiae nye dzesi gbãtɔ si dzena na ame akpa gãtɔ.
• Akɔtave
• Akɔtave
• Vevesese le asiwo kple afɔwo ŋu

Nuɖuɖumeŋusẽ ƒe kuxiwo

Esi wònye be lãmekawo gblẽa nu le ame ŋu ta la, kuxiwo ate ŋu ado mo ɖa le nuɖuɖumeŋusẽ ŋu hã:

• Aɖuɖɔtoe kple ʋuʋudedi
• Nuɖuɖumadzroame
• Miléle
• Dɔmetɔtrɔ

Titina lãmekawo ƒe dɔwɔwɔ ƒe dzesiwo

Esiawo do ƒome kple ahɔhɔ̃ kple susu tẽ:

• Dzitsitsi
• Alɔ̃ madɔmadɔ
• Teteɖeanyi
• Delirium ƒe dɔléle
• Hallucinations – nusiwo mele afima ŋutɔŋutɔ o kpɔkpɔ alo sese
• Nɔnɔme epilepticus/alɔ̃madɔmadɔ (`Alɔ̃madɔmadɔ`) .

Lãmetsiŋusẽ si le gota ƒe dzesiwo

Esiawo do ƒome kple lãmeka siwo le ŋutilã ƒe akpa bubuwo:

• Lãmetsiwo ƒe gbɔdzɔgbɔdzɔ
• Afɔ kple afɔwo ƒe ʋuʋu kple ʋuʋudedi
• Ɖeɖiteameŋu
• Sidzenuwo ƒe bublu
• Lãmetsiwo ƒe lãmetutudɔ
• Yagbɔgbɔ ƒe lãmetutudɔ – Esia nye afɔku gã aɖe eye ateŋu ana gbɔgbɔ nasesẽ.

Dzesi siwo dzena le lãmekawo ƒe dɔwɔwɔ ŋu le wo ɖokui si

Esiawo nye nusiwo dzɔna le wo ɖokui si, nusiwo dzi míate ŋu akpɔ ŋusẽ ɖo o:

• Dzi ƒe tsotso
• Ʋusɔgbɔdɔ

Ŋutilã ƒe dzesiwo (vevietɔ le `Variegate Porphyria` kple `Hereditary Coproporphyria` me)

Ame siwo ŋu ƒomevi eve siawo le ate ŋu akpɔ ŋutigbalẽ me kuxiwo ne wole ŋdɔ me :

• Ɣe ƒe keklẽ ƒe seselelãme
• Blistering ʋuʋudedi
• Ŋutilã ƒe amadede ƒe tɔtrɔ (Pigmentation) .
• Abidede ame ŋu

Nu vevitɔe nye be ne porphyria le ŋuwò la, wò aɖuɖɔ ate ŋu atrɔ amadede . Ne porphyrin ƒe ŋgɔdonya mawo siwo ŋu meƒo nu tsoe la ƒo ƒu ɖe wò ŋutilã me eye woɖe wo do goe le wò aɖuɖɔ me la, wò aɖuɖɔ ate ŋu atrɔ azu amadede dzĩ-dzẽ . Nya "porphyria" tso Helagbe me nya "porphura," si gɔmee nye "aɖabɛ dzĩ" me. Porphyrin siwo le míaƒe ʋumenugbagbevi dzĩwo me ye nana ʋu ƒe amadede biã.

Nukae nye AHP ƒe amedzidzedze?

Le ame geɖe siwo ŋu AHP ƒe dzesiwo le gome la, esiawo va le "amedzidzedze" ɣeaɖewoɣi ƒe nɔnɔme me. Ame aɖewo toa wo me enuenu, eye ame aɖewo hã metoa wo me edziedzi o. Ame aɖewo toa wo me vevie wu, evɔ ame bubuwo ya metoa wo me o. Zi geɖe la, amedzidzedze siawo nɔa anyi ŋkeke ʋɛ aɖewo, eye emegbe wodzina ɖe edzi vivivi heɖea wo dzi kpɔtɔna. Ne dzesi sesẽ siwo doa vɔvɔ̃ na ame le ŋuwò la, ele be nàyi kɔdzi. Abe alesi megblɔe ene la, nuwo abe gbɔgbɔtsixetsixe, si nana gbɔgbɔ sesẽna ene, nye nɔnɔme siwo hiã be woakpɔ egbɔ kpata.

AHP ƒe amedzidzedze ƒe dzesi si bɔ wue nye dɔgbo ƒe vevesese sesẽ si womeɖe o.. Ame siwo ŋu AHP le ƒe 90% kple edzivɔ yia ɖɔkta gbɔ le dɔgbo ƒe vevesese sia ta. Gake esi wònye be nu geɖe li siwo gbɔ dɔgbo ƒe veve tsona, eye esi wònye be X-ray alo CT scan mate ŋu akpɔ nusi gbɔ vevesese sia tso o ta (elabena lãmekawo ƒe vevesesee wònye) ta la, esesẽna ŋutɔ be woanya dɔlélea. Esi wònye be dɔgbomeve sia kpena ɖe nuwo abe aɖuɖɔtoe, aɖuɖɔtoe, kple dɔmeɖui ene ŋu ta la, ɖɔktawo ate ŋu abui be nuɖuɖumeŋusẽdɔe wònye. Ne susu kple seselelãme ƒe kuxiwo hã le ŋuwò la, woate ŋu abui hã be ahɔhɔ̃mekawo ƒe dɔlélee. Ne dzesi siawo siwo dze abe ɖe womedo ƒome kple wo nɔewo o ene va ƒo ƒu la, ehiã be woayi ɖɔkta si nya nu tso eŋu be wòakpɔe adze sii be ate ŋu anye porphyria.

Dzesi siwo nɔa anyi didi kawoe AHP ate ŋu ahe vɛ?

Ƒã hafi ame siwo ŋu dɔléle si nu mesẽ o le (enzyme ƒe agbɔsɔsɔ si mede ame dzi o) la kpɔa dzesi siwo nɔa anyi didi. Ðewohĩ amedzidzedze ɖeka alo eve koe adze wo dzi le woƒe agbenɔɣi. Ne wode dzesi nusiwo hea wo vɛ la, woate ŋu aƒo asa na wo. Gake ame aɖewo dzea ame dzi enuenu, eye dzesi aɖewo dzena enuenu ale gbegbe be woate ŋu abu wo be wonɔa anyi didi. Dzesi mawo dometɔ aɖewoe nye:

• Vevesese
• Ðeɖiteameŋu
• Xeɖeɖe
• Neuropathy ( abɔ kple afɔwo ƒe ʋuʋu, vevesese, alo gbɔdzɔgbɔdzɔ) .

Nukawoe nye kuxi siwo dona tso AHP me?

Zi geɖe la, ne ame aɖe dze ame dzi ko hafi dzesi siwo do ƒome kple lãmekawo ƒe dɔwɔwɔ (abe dzimaɖitsitsi, susumenuwo, kple alɔ̃madɔmadɔ ene). Gake dzesi siwo do ƒome kple lãmeka siwo le gota (abe lãmekawo ƒe gbɔdzɔgbɔdzɔ kple lãmetutudɔ ene) ate ŋu ado mo ɖa enuenu, eye woate ŋu agblẽ nu tegbee le amedzidzedze sesẽ aɖe megbe. Porphyrin ƒe ŋgɔdonya siawo ƒe ƒuƒoƒo ate ŋu agblẽ nu le ŋutinu aɖewo hã ŋu ɣeyiɣi didi. Esiawo ate ŋu anye:

• Ʋusɔgbɔdɔ si nɔa anyi didi
• Aklãdɔléle si nɔa anyi didi
• Ayikudɔ si nɔa anyi didi
• Aklã me kansa gbãtɔ
• Blanuiléle kple dzimaɖitsitsi

Nukae hea AHP vɛ?

Nusi koŋ gbɔ AHP tsonae nye domenyiŋusẽfianu ƒe tɔtrɔ . Gake abe alesi megblɔe va yi ene la, dzesiawo medzena enumake ne domenyiŋusẽfianu ƒe tɔtrɔa va o. Zi geɖe la, dzesiawo dzena zi gbãtɔ le sɔhɛmenɔɣi, tsɔ kpe ɖe lãmetsiŋusẽ ƒe tɔtrɔ ŋu. Tsɔ kpe ɖe eŋu la, atike aɖewo, atike muamewo, ahanono fũu akpa, nuɖuɖumeŋusẽ ƒe mɔxexeɖenu sesẽ, kple dɔléle siwo dea nuteɖeamedzi geɖe ŋutilã me hã ate ŋu aʋãe.

Nusi sɔ kple nusiwo katã ʋãa ame siawoe nye be wodoa ŋusẽ aklãa ƒe heme wɔwɔ. Gake le amesi ŋu AHP le ƒe aklã me la, wometrɔa nusiwo dona tso heme wɔwɔ me (abe porphyrin ƒe ŋgɔdonyawo ene) nyuie o, si fia be wozãa wo vɔ. Eyata porphyrin ƒe ŋgɔdonya siawo siwo susɔ la ƒoa ƒu ɖe aklãa me. Ne esiawo ƒo ƒu va ɖo afi aɖe ko hafi wodzea ŋusẽkpɔkpɔ ɖe lãmekawo dzi eye wohea dzesiwo vɛ.

Aleke wowɔna dea dzesi AHP? (Dɔléle ƒe dzesi) .

Le amesiwo ŋu AHP le gomeDɔlélea didi nyuie ate ŋu asesẽ, elabena dzesiawo menye nɔnɔme sia ɖeɖeko tɔ o eye ate ŋu adze abe ɖe womedo ƒome o ene. Gake ɖɔkta si nya AHP nyuie ate ŋu asusu be AHP le ŋuwò ne ƒodo me veve tsɔ kpe ɖe lãmeka siwo le titina kple gota gome ƒe dzesiwo ŋu. Wobua dzesi etɔ̃ siawo ƒe ƒuƒoƒo be enye AHP ƒe "classic triad".

Ne ɖɔkta aɖe ɖi ɖase be AHP la, woate ŋu awɔ dodokpɔ geɖe atsɔ aɖo kpe edzi.

• Aɖuɖɔ dodokpɔ: Woate ŋu awɔ esia kaba le amedzidzedze me. Aɖuɖɔ ate ŋu asɔ ne mèle dɔ lém o, gake le amedzidzedze me la, aɖuɖɔtoea aɖe porphyrin precursors (PBG kple ALA) ƒe agbɔsɔsɔ si dzi ɖe edzi afia. Esia menye dodokpɔ si wowɔna na AHP ɖeɖeko o, gake enye dodokpɔ vevi aɖe si ate ŋu akpe ɖe ɖɔktawo ŋu woafia mɔ nyuitɔ wò.
• Domenyiŋusẽfianu dodokpɔ: Esiae nye mɔ nyuitɔ kple esi ŋu kakaɖedzi le wu si dzi woato anya AHP (`sikadzidzenu`) . Dzesiwo ɖale ŋuwò alo mèkpɔe o, ate ŋu aɖo kpe edzi nenye be domenyiŋusẽfianu ƒe tɔtrɔ si do ƒome kple AHP le ŋuwò. Ate ŋu agblɔ AHP ƒomevi si le ŋuwò kple alesi enzyme ƒe anyimanɔmanɔ nu sẽe hã na wò. Esia bia be woaɖe ʋu alo aɖuɖɔ. Ðewohĩ ahiã be kɔdzi si le mia gbɔ hã naɖo kpɔɖeŋua ɖe dodokpɔxɔ tɔxɛ aɖe.

Aleke wowɔa atike na AHP?

AHP ƒe atikewɔwɔ ku ɖe mɔxexe ɖe amedzidzedzewo nu kple wo dzi ɖuɖu ŋu . Ðewohĩ ahiã be woaxɔ wò ɖe kɔdzi le amedzidzedze aɖe me. Ðewohĩ ahiã be nàzã atike vovovowo be wòaɖe wò dzesiwo dzi akpɔtɔ. Le ɣeyiɣi siwo me mèle amedzidzedze kpɔm o me la, àte ŋu ahiã atike bubuwo be nàtsɔ aɖu nusiwo hea wò dzesiawo vɛ dzi. Ne mia kple wò ɖɔkta miewɔ dɔ ɖekae de dzesi nusiwo aʋã wò siwo gblẽa nu le ŋuwò wu la, wò atikewɔwɔa awɔ dɔ nyuie wu.

Atikewɔwɔ na amedzidzedze sesẽ aɖe:

• Hemin dodo: Ne ame aɖe dze ame dzi vevie la, ɖɔkta ate ŋu ado hemin na wò ɖe ʋuka aɖe me. Esia kpena ɖe ŋuwò be porphyrin ƒe agbɔsɔsɔ si le wò ʋu me dzi ɖena kpɔtɔna. Hemin nye nusi woɖena tso ʋumenugbagbevi dzĩwo me eye wòɖea porphyrin ƒe agbɔsɔsɔ si le wò ŋutilã me dzi kpɔtɔna.
• Vevesese dzi ɖeɖe kpɔtɔ: Le amedzidzedze sesẽ aɖe me la, ate ŋu ahiã vevesesenutsitike sesẽwo abe opioid ene.
• Phenothiazines: Wozãa esiawo tsɔ léa ʋuʋudedi kple ʋuʋudedi sesẽwo nu.
• Lãmetsi kple nunyiame: Dzesiwo abe dɔgbo ƒe vevesese, ʋuʋudedi, ʋuʋudedi, aɖuɖɔtoe, kple dɔmeɖui ate ŋu ana ŋutilãa nabu nuɖuɖumeŋusẽ kple tsi le amedzidzedze me. AHP ate ŋu ana nusiwo abe sodium kple magnesium ene hã nabu. Eyata woate ŋu ado tsi siwo me carbohydrates kple electrolytes le ɖe ʋuka me.
• Atike siwo wozãna na alɔ̃madɔmadɔ:Dɔnɔ siwo ade 20% ate ŋu ahiã atikewɔwɔ na asrãdɔ le amedzidzedze aɖe me.

Atikewɔwɔ ƒe tiatia siwo woate ŋu azã ɣeyiɣi didi:

• Hemin si xea mɔ na dɔlélea: Hemin ƒe agbɔsɔsɔ sue aɖe nana amesiwo dzea ame dzi enuenu zi ɖeka le kwasiɖa me ate ŋu axe mɔ na porphyrins ƒe ƒuƒoƒo.
• Givosiran (GIVLAARI®): Esia nye domenyiŋusẽfianu ƒe dɔdamɔnu ƒomevi aɖe . Eɖea porphyrin ƒe ŋgɔdonyawo wɔwɔ dzi kpɔtɔna. Fifia woda asi ɖe edzi be woadoe ɣleti sia ɣleti be woatsɔ axe mɔ ɖe AHP ƒe dzesiwo nu le amesiwo dzea ame dzi enuenu ŋu.
• Lãmetsiŋusẽtike: Ne wò dzinukpɔkpɔ gbɔe wò AHP ƒe amedzidzedzewo tso la, wò ɖɔkta ate ŋu aŋlɔ atikewo abe GnRH (Gonadotropin-releasing hormone) analogs ene na wò. Esiawo ɖea lãmetsi siwo nye estrogen kple progesterone siwo ŋutilãa wɔna dzi kpɔtɔna.
• Ʋusɔgbɔdɔ ƒe atikewo: Ne ʋusɔgbɔdɔ si nɔa anyi didi va do mo ɖa la, ahiã be woatsɔ atike aɖewo koŋ aɖu edzi.
• Aklã dodo na ame: Ame siwo dzea ame dzi enuenu, si dea woƒe agbe afɔku me, eye womewɔa nu ɖe ​​atike bubuwo ŋu o la ate ŋu abu aklã dodo ŋu. Esia ate ŋu ada dɔlélea keŋkeŋ gɔ̃ hã.

Ðe woate ŋu axe mɔ ɖe AHP nua?

Manya wɔ be woaxe mɔ ɖe dɔlélea nu be wòagadzɔ o. Gake àte ŋu akpe ɖe ŋuwò nàxe mɔ ɖe dzesiawo nu ne èƒo asa na nusiwo hea dɔlélea vɛ . Ne amedzidzedze aɖeke medze dziwò kpɔ o la, anyo wu be nàƒo asa na nusiwo katã ate ŋu aʋã wò. Ne amedzidzedze aɖe dze dziwò eye nède dzesi nusiwo gbɔ wòtso la, ate ŋu asɔ gbɔ be nàƒo asa na nu mawo koŋ siwo ʋã wò.

Nusiwo wokpɔna zi geɖe siwo hea dɔlélea vɛ dometɔ aɖewoe nye esi:

Atike ƒomeviwo:

• Antihistamines (atike aɖewo na dzamezã kple nulɔdɔwo) .
• Atike siwo naa ame ƒe dzi dzea eme
• Mɔxexeɖefufɔfɔnu siwo woano
• Lãmetsiŋusẽ ɖɔliɖɔli ƒe atikewɔwɔ

Nusiwo wozã:

• Atama (`Ataama`) .
• Marijuana zazã
• Ahasesẽ
• Modzakaɖetikewo zazã

Nuteɖeamedzi:

• Dɔlékuiwo
• Amekoko
• Calorie si mesɔ gbɔ o ɖuɖu (vevietɔ carbohydrate si mebɔ o) (`Clorie ƒe anyimanɔmanɔ`)
• Susu me nuteɖeamedzi

Nukae nye etsɔme na amesiwo ŋu AHP le? (Nyagblɔɖi) .

Esia toa vovo ŋutɔ tso ame ɖeka gbɔ yi bubu gbɔ. Ame geɖe mekpɔa dzesi aɖeke gbeɖe o . Le amesiwo wɔa esia dome la, ame akpa gãtɔ ƒe amedzidzedze zi ɖeka alo ʋɛ aɖewo koe dzea wo dzi le woƒe agbenɔɣi. Togbɔ be amedzidzedze ate ŋu ade ame ƒe agbe afɔku me hã la, ame akpa gãtɔ hayana bliboe ne wowɔ atike na wo kaba . Ame siwo ŋu AHP le la dometɔ siwo ade 5% ƒe dzesiwo dzena enuenu alo nɔa anyi didi. Le ame siawo gome la, mɔxexeɖedɔléle nu ƒe atikewo kpena ɖe ame ŋu zi geɖe, eye woate ŋu abu aklã dodo na ame be enye mɔnu mamlɛtɔ.

Aleke malé be na ɖokuinye esime mele AHP gbɔ?

• Ƒo asa na nusiwo ʋãa ame zi geɖe. Ele be ahanono, atamanono, kple atike muamewo nanɔ nu siwo wòle be nàƒo asa na la ƒe tatɔ. Àte ŋu aƒo nu kple wò ɖɔkta hã tso atike siwo nàzã ɖe atike aɖewo teƒe ŋu.
• Ƒo nuɖuɖu si naa lãmesẽ, si da sɔ. Ƒo asa na nuɖuɖu si gbɔ eme kple nuɖuɖu si me carb mesɔ gbɔ ɖo o ƒe ɖoɖowo. Ne èle nu tsim do ŋgɔ na atikewɔwɔ ƒe dodokpɔ alo amekoko la, ƒo nu tso eŋu na wò ɖɔkta.
• Di atikewɔlawo ƒe dodokpɔ edziedzi. Ne dzesi aɖeke mele ŋuwò o hã la, wò ɖɔkta alé ŋku ɖe wò aklã, ayiku, kple ʋu ƒe sisi ŋu edziedzi.

Aklã me ʋu ƒe sisi kpata nye dɔléle si mebɔ o, eyata amewo katã menya nu tso eŋu o. Ne amedzidzedze aɖe va kpata kple dzesi sesẽwo la, etɔtɔa ame ŋutɔ eye wòdoa vɔvɔ̃ na ame. Ame aɖewo zãa ƒe geɖe tsɔ dzea agbagba be yewoanya nusi gblẽ le yewo ŋu kple nusi yewoate ŋu awɔ. Ne èsusu be AHP le ye ŋu la, domenyiŋusẽfianu ƒe dodokpɔ ate ŋu aɖo kpe edzi. Ne ènya nya ko la, anye kpekpeɖeŋu gã aɖe na wò be nàxe mɔ ɖe amedzidzedzewo nu ahaɖu wo dzi.

Nu vevitɔ kekeake siwo wòle be nànya (Take-Home Message) .

Enyo, eyata mina maƒo nu tso nusiwo dzi wòle be nàɖo ŋkui tso nusiwo ŋu míeƒo nu tsoe me la dometɔ aɖewo ŋu kpuie:

• AHP nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o si dzea egɔme tso aklã me eye wògblẽa nu le lãmekawo ŋu .
• Esia tso enzyme siwo hiã le heme wɔwɔ me ƒe anyimanɔmanɔ gbɔ , si nana aɖinuwo siwo woyɔna be porphyrin precursors ƒoa ƒu ɖe ŋutilãa me.
• Dzesiawo ate ŋu ato vovo, tso esiwo medo ƒome kple wo nɔewo o, dɔgbo ƒe vevesese sesẽ, lãmekawo ƒe vevesese, tagbɔdɔléle, ŋutigbalẽ ƒe kuxiwo, kple wò aɖuɖɔ ƒe amadede ƒe tɔtrɔ gɔ̃ hã dzi.
• Ne domenyiŋusẽfianu ƒe tɔtrɔa le eme gɔ̃ hã la, ƒã hafi dzesiawo dzena nusiwo hea dɔlélea vɛ manɔmee ( abe lãmetsiŋusẽ, atike aɖewo, aha sesẽ, kple nuteɖeamedzi ene).
• Aɖuɖɔtoe dodokpɔ kple domenyiŋusẽfianu dodokpɔ le vevie hena dɔlélea didi.
• Nu vevitɔe nye be woaɖu amedzidzedzewo dzi ahaxe mɔ ɖe enu to atikewɔwɔ me. Wozãa `Hemin` ƒe atike, vevesesenutsitikewo, kple atike tɔxɛ aɖewo. Le dɔléle siwo nu sẽ wu me la, aklã dodo na ame hã nyea egbɔkpɔnu.
• Àte ŋu anɔ agbe nyuie kple dɔlélea ne èƒo asa na nusiwo hea dɔlélea vɛ, nènɔa agbe le lãmesẽ me, eye nèdoa wò kpɔ edziedzi le atikewɔƒea .

Mele mɔ kpɔm be nyatakaka sia akpe ɖe ŋuwò. Ðo ŋku edzi be ne dzesi siawo dometɔ aɖe le ŋuwò la, mègavɔ̃ be yeayi ɖɔkta gbɔ aƒo nu tso eŋu o.

` Acute hepatic porphyria, AHP, aklã, lãmekawo ƒe dɔwɔwɔ, domenyiŋusẽfianu ƒe dɔlélewo, porphyrins, dzesiwo, atikewɔwɔ