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Yu gɛt wan strenj sik we yu nɔ ebul fɔ ɔndastand? I kin bi Akyu Ɛpatik Pɔfyria!

Yu gɛt wan strenj sik we yu nɔ ebul fɔ ɔndastand? I kin bi Akyu Ɛpatik Pɔfyria!

Yu kin fil lɛk se yu bɛlɛ kin at we yu nɔ go ebul fɔ imajin, sɔntɛnde yu at kin ebi, ɛn yu an ɛn fut dɛn kin swɛ? Sɔntɛm ivin dɔktɔ dɛn nɔ kin no wetin i bi. Wan pan dɛn strenj, we tan lɛk se nɔ gɛt natin fɔ du wit dɛnsɛf, we wi go tɔk bɔt tide na Acute Hepatic Porphyria (AHP). Nɔ wɔri, dis na tin we kɔmplikt smɔl, bɔt lɛ wi ɔndastand am simpul wan.

Wetin rili na akyu ɛpatik pɔfyuria (AHP)?

Fɔ tɔk am simpul wan, AHP na wan sik we nɔ kin bɔku we pɔsin kin gɛt we i kam pan jɛnɛtiks . I kin bigin na yu liva . Bɔt i kin afɛkt yu nervɔs sistɛm bak , we kin mek yu gɛt sɔm sayn dɛn ɔlsay na yu bɔdi. Tink bɔt am, sɔntin de we dɛn kɔl hεm, we na pat pan di ɛmoglobin we de na wi blɔd. wi bכdi nid difrεn εnzym dεm fכ mek dis hεm. Pɔsin we gɛt AHP nɔ gɛt bɛtɛ, ɔ i nɔ gɛt sɔm pan di ɛnzaym dɛn we i nid fɔ mek dis hεm.

Wetin kin apin we dis kin apin? Sɔm pan di kemikal dɛn we dɛn fɔ dɔn yuz fɔ mek hεm, kin lɛf. Wi kin kɔl dɛn tin ya we lɛf na di bɔdi pɔfyurin prɛkursɔ . We dɛn tin ya bɔku, dɛn kin gɛt pɔyzin to di bɔdi. insay AHP, dεn tכxin dεm ya kin fכs bכku na di liva. Dɔn, we dɛn go insay di blɔd ɛn miks wit di nerv dɛm, di sayn dɛm kin bigin fɔ sho. Yu ɔndastand?

Difrɛn kayn AHP dɛn de?

Yes, 4 men kayn AHP de. dεn fכ tכp dεm ya dεn sheb to di kayn εnzym dεm we a bin tכk bכt, i dipכnt pan us εnzym dεm we nכ de. εvri tכp dεn gi in nem afta di pat we dεn kכl "hεpatik" biכs i de stat na di liva, εn di pat we dεn kכl "acute" biכs di simptom dεm de sho wan wan. Fɔ mek dɛn go ɔda frɔm di kayn we we kɔmɔn pas ɔl to di wan we nɔ kɔmɔn, na di kayn dɛn ya:

1. Akyu Intamitεnt Pכfyria (AIP): Dis na di wan we kכmכn pas כl. wan mכtεshכn na di HMBS jin de mek di εnzym haydroksimεtilbilan sεntez (HMBS) (dεn kכl am bak pכrfobilinojen diaminaz (PBGD)) nכ de. dis kin bi nyu jεnεtik mכtεshכn we de apin, כ i kin inhεrit as כtosom dכminant trayt (i.e., di sik kin apin ivin if na wan mama εn papa nכmכ gεt di jin).

2. Variegate Porphyria (VP): wan mכtεshכn na di PPOX jin de mek di εnzym protoporphyrinogen oxidase (PPO כ PPOX) nכ de. dis kin inhεrit bak as כtosom dכminant כ כtosom rεsεsiv.

3. Hεriditary Coproporphyria (HCP): wan mכtεshכn na di jin `CPOX` de rizulta in dεfisit fכ di εnzym `coproporphyrinogen oxidase (CPOX).` dis kin inhεrit bak as כtosomal dכminant כ כtosomal rεsεsiv.

4. ALAD-Dεfisiεns Pכfyuria (ALAD-Dεfisiεns Pכfyuria - ADP):mכtεshכn na di `ALAD` jin de mek di εnzym `delta-aminolevulinic acid dehydratase (ALAD) dεfichεns.` i de inhεrit insay wan `autosomal recessive` we (i.e., di sik kin כnli apin if di mama εn papa tu inhεrit di jin).

Aw AHP de afɛkt mi?

Dis kin mek wi sɔprayz, bikɔs AHP nɔ kin afɛkt ɔlman di sem we. Sɔm pipul dɛn gɛt di jin muteshɔn fɔ am bɔt dɛn nɔ kin ɛva gɛt ɛni sayn . Ɔda pipul dɛn kin gɛt "atak" fɔ di sayn dɛm we kin apin wan ɔ tu tɛm nɔmɔ insay dɛn layf. Bɔt sɔm pipul dɛn kin gɛt dɛn kayn atak ya ɔltɛm, ɛn ɔda wan dɛn kin gɛt am fɔ lɔng tɛm , we min se dɛn kin kɔntinyu fɔ gɛt sɔm sayn dɛn we kin afɛkt dɛn layf ɛvride. Sɔntɛnde, atak kin so bad dat yu go nid fɔ kɛr yu go na di ɔspitul imejensi rum. Yu kin gɛt siriɔs pen na yu nerv, yu skin kin chenj, ɛn yu kin gɛt sɔm kayn sik wae gɛt fɔ du wit yu nervɔs sistɛm.

Udat go mɔs gɛt dis?

AHP kin afɛkt pipul dɛn we gɛt ɛni kɔlɔ ɔ ɛni kɔlɔ. I kin kɔmɔt frɔm wan ɔ ɔl tu mama ɛn papa dɛn we gɛt di jin muteshɔn. I sɔprayz fɔ no se bɔku pipul dɛn nɔ kin gɛt dis sik . Fɔ mek di simptom dɛn bigin, di jin we de chenj nɔmɔ nɔ go du fɔ am. Sεvεra כda `trigεr` dεm mכst de de bak.

Wetin na dɛn tin ya we kin mek pɔsin gɛt dis sik?

  • di chenj dεm we de apin na di כmon dεm (εspεshali we uman de bכn, we uman bεlε, εn di mεnstral saykl) .
  • Sɔm mɛrɛsin dɛn
  • Yuz rɔm
  • Fɔ smok
  • Siriɔs strɛs
  • Strikt kɔntrol fɔ it (espɛshali fɔ stɔp di kalori) .

I fayn bak fɔ no se 80% pan di wan dɛn we gɛt di sik na uman dɛn we dɔn ol fɔ bɔn pikin .

Aw kɔmɔn tin fɔ AHP?

I nɔ kin izi fɔ tɔk fɔ tru, bikɔs bɔku tɛm dɛn nɔ kin no dis sik fayn fayn wan. Ɔlsay na di wɔl, dɛn se na lɛk 5 pan 100,000 pipul dɛn kin gɛt am. pan di kayn dεm we wi bin dכn tכk bכt, `Acute Intermittent Porphyria (AIP)` na di tכp we dεn kin si pan כnli 80% pan di kεys dεm we dεn ripot. `ALAD-Deficiency Porphyria (ADP)` na di kayn we we nɔ kɔmɔn, wit 9 kes dɛm nɔmɔ we dɛn ripɔt ɔlsay na di wɔl. Imajin, na wan pan ɛvri 10 pipul dɛm wae gɛt di jin muteshon wae gɛt fɔ du wit AHP go gɛt sɔm kayn sik.

Wetin na di sayn dɛm fɔ AHP?

Di sayn dɛm fɔ AHP rili strenj. Dɛn kin tranga fɔ di sikman ɛn dɔktɔ fɔ ɔndastand, bikɔs di sik kin kam wantɛm wantɛm, dɛn kin tranga, ɛn i kin tan lɛk se dɛn nɔ gɛt natin fɔ du wit dɛnsɛf.

Na sɔm pan dɛn tin ya:

Nɛv pen

Dis pen kin apin na difrɛn say dɛn na di bɔdi:

  • Siriɔs bɛlɛ pen – Dis na di fɔs sayn we kin apin to bɔku pipul dɛn.
  • Chɛst de pen
  • Bak pen
  • Pen na di an ɛn fut

Prɔblɛm dɛn we de na di dijestiv sistɛm

Bikɔs di nerv dɛn kin afɛkt, prɔblɛm kin apin bak wit di dijestiv sistɛm:

  • Nɔs ɛn vɔmit
  • Di we aw pɔsin nɔ de it fayn
  • Banbɛlɛ
  • Rɔnbɛlɛ

Sεntri nεv sεstem simptom dεm

Dɛn tin ya gɛt fɔ du wit di bren ɛn maynd:

  • Wɔri
  • Nɔ ebul slip
  • Pwɛl at
  • Delirium we gɛt di sik
  • Hallucinations – fɔ si ɔ yɛri tin dɛn we nɔ rili de
  • Status epilepticus/sɛiz (`Sɛiz`) .

Di simptom dɛm fɔ di periferik nɛvɔ sistɛm

dis dεm kin kכnεkt to di nεv dεm na כda pat dεm na di bכdi:

  • Di mɔsul dɛn we wik
  • Di an ɛn fut dɛn kin swɛla ɛn tingling
  • Taya
  • Di sɛns we pɔsin kin gɛt we i nɔ de fil fayn
  • Mɔsul dɛn we de paralayz
  • Respiratory paralysis – Dis kin rili denja ɛn i kin mek i nɔ izi fɔ blo.

Di simptom dɛm fɔ di ɔtonomik nervɔs sistɛm

Dis na tin dɛn we kin apin fɔ dɛnsɛf, tin dɛn we wi nɔ ebul fɔ kɔntrol:

  • Di at we de blo
  • Di ay blɔd prɛshɔn

Di simptom dɛm na di skin (espɛshal wan na `Variegate Porphyria` ɛn `Hereditary Coproporphyria`)

Pipul wae gɛt dis tu kayn kin gɛt prɔblɛm wit dɛn skin wae dɛn de na di san :

  • Fɔ fil we di san de shayn
  • Rash we de blistering
  • Di kɔlɔ we di skin kin chenj (Pigmentation) .
  • Skaring we de mek pɔsin gɛt skata

Di tin we impɔtant pas ɔl na dat we yu gɛt pɔfyuria, yu urine kin chenj in kɔlɔ . We dɛn pɔfyurin prɛkursɔ dɛn we a bin tɔk bɔt gɛda na yu bɔdi ɛn kɔmɔt na yu urine, yu urine kin tɔn rɛd-pɔpul kɔlɔ . Di wɔd "porphyria" kɔmɔt frɔm di Grik wɔd "porphura," we min "pɔpul." Di pɔfyurin dɛn we de na wi rɛd blɔd sɛl dɛn na dɛn de gi blɔd in rɛd kɔlɔ.

Wetin na AHP atak?

Fɔ bɔrku pipul dɛm wae gɛt AHP simptom dɛm, dɛn tin ya kin kam insay di fɔm wae dɛn kin gɛt "atak" wan wan tɛm. Sɔm pipul dɛn kin gɛt dɛn mɔ ɛn mɔ, sɔm nɔ kin gɛt am. Sɔm pipul dɛn kin gɛt dɛn bad bad wan, ɛn ɔda wan dɛn nɔ kin gɛt am. Bɔrku tɛm, dɛn atak ya kin las fɔ sɔm dez, dɔn smɔl smɔl dɛn kin bɔku ɛn stɔp. If yu gɛt siriɔs, frayd sik, yu fɔ go na ɔspitul. As a bin se, tin dɛm lɛk respiratory paralysis, we kin mek i nɔ izi fɔ blo, na kɔndishɔn dɛm we nid fɔ gɛt mɛrɛsin kwik kwik wan.

Di sayn we kin sho se pɔsin gɛt AHP atak na we i nɔ kin ɛksplen, we i kin gɛt bad bad bɛlɛ pen.. Mɔr pas 90% pan di pipul dɛm wae gɛt AHP kin go to dɔktɔ bikɔs ɔf dis bɛlɛ pen. Bɔt bikɔs bɔku tin dɛn de we kin mek pɔsin fil pen na in bɛlɛ, ɛn bikɔs ɛkstrem rayt ɔ CT skan nɔ kin ebul fɔ fɛn di tin we kin mek i fil pen (bikɔs na pen na di nerve), i kin rili at fɔ no. Bikɔs dis bɛlɛ pen kin kam wit tin dɛn lɛk nɔys, vɔmit, ɛn kɔnstipɛshɔn, dɔktɔ dɛn kin tink se na sik we de mek pɔsin digest. If yu gɛt prɔblɛm wit yu maynd ɛn sɛns bak, dɛn kin tink bak se na nyurolɔjik sik. We dɛn sik ya we tan lɛk se nɔ gɛt natin fɔ du wit dɛnsɛf kam togɛda, i nid fɔ go to dɔktɔ we no bɔt am fɔ no se i kin bi pɔfyuria.

Us sik dɛn we nɔ de mɛn we AHP kin mek pɔsin gɛt?

Pipul wae gɛt dis sik wae nɔr kin pasmak (we nɔr kin gɛt bɛtɛ ɛnzaym) nɔr kin gɛt dis sik wae nɔr de dɔn. Dɛn kin jɔs gɛt wan ɔ tu atak insay dɛn layf. If dɛn no di tin dɛn we kin mek dɛn du dat, dɛn kin avɔyd dɛn. Bɔt sɔm pipul dɛn kin gɛt atak ɔltɛm, ɛn sɔm sayn dɛn kin so ɔltɛm dat dɛn kin tek am se dɛn nɔ de mɛn. Dɛn kayn sayn ya na:

  • Pen
  • We pɔsin taya
  • Vɔmit
  • Nyuropathy ( we yu bɔdi nɔ gɛt bɛtɛ trɛnk, pen, ɔ wik) .

Wetin na di kɔmplikɛshɔn dɛm we AHP kin gɛt?

Di simptom dɛm wae gɛt fɔ du wit di sɛntral nervous system (lɛk wae yu de wɔri, wae yu de si tin, ɛn yu kin gɛt sik) kin apin nɔmɔ wae pɔrsin gɛt atak. Bɔt di sayn dɛm we gɛt fɔ du wit di periferik nervɔs sistɛm (lɛk we di mɔsul dɛn wik ɛn paralayz) kin apin bɔku tɛm, ɛn i kin pwɛl fɔ ɔltɛm afta dɛn dɔn atak am bad bad wan. di akyumyuleshכn fכ dεn pכfyurin prεkursכr dεm kin mek bak sכm כgan dεm de damej fכ lכng tεm. Dɛn tin ya kin bi:

  • Krɔnik haypatɛyshɔn
  • Di sik we nɔ de mɛn na di liva
  • Kidni sik we nɔ de mɛn
  • Praymari liva kansa
  • Pwɛl hat ɛn wɔri

Wetin kin mek pɔsin gɛt AHP?

Di men tin we kin mek pɔsin gɛt AHP na di we aw pɔsin kin chenj in jɛnɛtiks . Bɔt lɛk aw a bin dɔn tɔk bifo tɛm, di sayn dɛn nɔ kin apin wantɛm wantɛm afta di jenɛtik muteshɔn dɔn de. Bɔrku tɛm, di sayn dɛm kin fɔs kin kam wae pɔrsin de bɔn, wit di chenj dɛm na di ɔmon dɛm. Apat frɔm dat, sɔm mɛrɛsin dɛn, drɔgs, fɔ drink pasmak, fɔ stɔp di kalori bad bad wan, ɛn sik dɛn we de put bɔku strɛs pan di bɔdi kin mek bak i gɛt am.

wetin כl dεn trig dεm ya gεt fכ kכmכn na dat dεn de mek di liva in hεm prodakshכn prכsεs. כltu, insay di liva fכ pכsin we gεt AHP, di bayprodukt dεm we di hεm prodakshכn de mek (lεk di pכfyurin prεkursכr dεm) nכ de mεtabolayz fayn, dat na fכ yus כp. fכ dat, dεn lεfεf pכfyurin prεkursכr dεm ya kin kכmכt na di liva. Na we dɛn tin ya gɛda to wan patikyula lɛvul nɔmɔ dɛn kin bigin fɔ afɛkt di nervɔs sistɛm ɛn mek dɛn gɛt sɔm sayn dɛn.

Aw dɛn kin no se pɔsin gɛt AHP? (Diagnosis) .

Fɔ di wan dɛn we gɛt AHPFɔ gɛt kɔrɛkt diagnosis kin tranga, bikɔs di sayn dɛm nɔr kin jɔs bi fɔ dis sik ɛn i kin tan lɛk se dɛn nɔr gɛt ɛnitin fɔ du wit am. Bɔt dɔktɔ we sabi AHP kin sɔprayz se AHP de if yu gɛt bɛlɛ pen wit di sɛntral ɛn periferik nɛvɔ sistɛm simptom dɛm. di kכmbaynshכn fכ dεn tri simptom dεm ya dεn kin kכnsidr am as di "klasik triad" fכ AHP.

If dɔktɔ sɔspɛkt se AHP, dɛn kin du bɔku tɛst fɔ kɔnfirm am.

  • Yurin tɛst: Dɛn kin du dis kwik kwik wan we pɔsin gɛt atak. Yurin kin bi nכmal we yu nכ de gεt atak, bכt we yu atak, di urine go sho εlevεt lεvεl dεm fכ di pכfyurin prεkursכr (PBG εn ALA). Dis nɔto tɛst we dɛn kin du fɔ AHP nɔmɔ, bɔt na bɛsik tɛst we kin ɛp dɔktɔ dɛn fɔ sho yu di rayt say.
  • Jεnεtik tεst: Dis na di bεst εn mכst difinitiv we fכ no AHP (di `gold standad`) . Ilɛksɛf yu gɛt di sik ɔ yu nɔ gɛt di sik, i kin kɔnfɔm if yu gɛt wan jin muteshon we gɛt fɔ du wit AHP. I kin tɛl yu bak us kayn AHP yu gɛt ɛn aw di ɛnzaym dɛfisiɛns de bad bad wan. Dis min se dɛn fɔ tek blɔd ɔ saliva sɛmpul. Yu lokal ɔspitul kin nid bak fɔ sɛn di sampul to spɛshal lab.

Aw dɛn kin trit AHP?

Di tritmɛnt fɔ AHP de pe atɛnshɔn fɔ mek dɛn nɔ atak ɛn kɔntrol di atak dɛn . Yu kin nid fɔ go na ɔspitul we dɛn atak yu. Yu kin nid difrɛn kayn mɛrɛsin fɔ ridyus yu sik. Na di tɛm dɛm wae yu nɔr de gɛt atak, yu kin nid ɔda mɛrɛsin fɔ kɔntrol di tin dɛm wae kin mek yu sik wɔs. If yu ɛn yu dɔktɔ wok togɛda fɔ no di tin dɛn we de ambɔg yu mɔ, yu tritmɛnt go wok fayn.

Tritmɛnt fɔ pɔsin we gɛt siriɔs atak:

  • Hemin injection: If yu atak yu bad bad wan, dɔktɔ kin gi yu injection of hemin insay wan vein. Dis kin ɛp fɔ ridyus di pɔfyrin we de na yu blɔd. Ɛmin na sɔntin we dɛn kin tek frɔm di rɛd blɔd sɛl dɛn ɛn i kin ridyus di pɔfyrin we de na yu bɔdi.
  • Fɔ mek pɔsin nɔ fil pen: We pɔsin gɛt siriɔs atak, dɛn kin nid strɔng mɛrɛsin fɔ mɛn pen lɛk opioid.
  • Phenothiazines: Dɛn kin yuz dɛn tin ya fɔ kɔntrol di bad bad nɔys ɛn vɔmit.
  • IV wata ɛn it: Di sayn dɛm lɛk bɛlɛ pen, nɔs, vɔmit, dayarɛa, ɛn kɔnstipɛshɔn kin mek di bɔdi lɔs kalori ɛn wata we pɔsin atak. AHP kin mek bak yu lɔs tin dɛm lɛk sɔdiɔm ɛn magnɛsiɔm. So, dɛn kin gi wata we gɛt kabɔhaydrɛt ɛn ilɛktrɔlayt insay di bɛlɛ.
  • Di mɛrɛsin dɛn we dɛn kin yuz fɔ mek pɔsin sik:Na lɛk 20% pan di sik pipul dɛn kin nid tritmɛnt fɔ ɛpilepsi we dɛn atak dɛn.

Di tritmɛnt dɛn we yu kin pik fɔ lɔng tɛm:

  • Prɔfylaktik ɛmin: If yu gi smɔl dos fɔ ɛmin wan tɛm insay di wik to pipul dɛn we gɛt atak ɔltɛm, dat kin mek di pɔfyrin nɔ gɛda.
  • Givosiran (GIVLAARI®): Dis na wan kayn jin tɛrapi . i de ridyus di prodakshכn fכ di pכfyurin prεkursכr dεm. Naw dɛn dɔn gri fɔ mek dɛn gi am ɛvri mɔnt fɔ mek dɛn nɔ gɛt AHP pan pipul dɛn we kin gɛt atak ɔltɛm.
  • Ɔmon tɛrapi: If yu AHP atak na yu menstrual saykl, yu dɔktɔ kin gi yu mɛrɛsin lɛk GnRH (Gonadotropin-releasing hormone) analogs. dis dεm de ridyus di bכdi in prodakshכn כmon dεm we dεn kכl εstrojen εn prכjεstεron.
  • Ha blɔd prɛshɔn mɛrɛsin: If yu gɛt ay blɔd prɛshɔn we nɔ de dɔn, dɛn go nid fɔ kɔntrol am wit sɔm patikyula mɛrɛsin dɛn.
  • Liva transplant: Pipul dɛn we kin gɛt atak ɔltɛm, we kin mek dɛn layf de pan denja ɛn we nɔ kin du ɔda tritmɛnt dɛn kin tink bɔt fɔ transplant liva. Dis kin ivin mɛn di sik kpatakpata.

Yu tink se dɛn kin mek dɛn nɔ gɛt AHP?

I nɔ pɔsibul fɔ mek di sik nɔ kam. Bɔt yu kin ɛp fɔ mek yu nɔ gɛt di sik bay we yu avɔyd tin dɛn we kin mek yu gɛt dis sik . If yu nɔ ɛva gɛt atak, i bɛtɛ fɔ avɔyd ɔl di tin dɛn we kin mek yu atak yu. If yu dɔn gɛt atak ɛn yu dɔn no di tin dɛn we mek yu atak am, i go fayn fɔ avɔyd jɔs dɛn patikyula tin dɛn de we mek yu atak am.

Na sɔm pan di tin dɛn we dɛn kin si we kin mek pɔsin sik:

Di kayn mɛrɛsin dɛn we dɛn kin yuz:

  • Antihistamines (sɔm mɛrɛsin fɔ kol ɛn alɛji) .
  • Di tin dɛn we de mek pɔsin fil fayn
  • Di tin dɛn we dɛn kin yuz fɔ mek uman nɔ gɛt bɛlɛ
  • Ɔmon riplesmɛnt tɛrapi

Di we aw dɛn de yuz di tin dɛn we dɛn de yuz:

  • Tabak (`Tobacco`) .
  • Marijuana we dɛn kin yuz
  • Rum
  • Drug dɛn fɔ ɛnjɔy yusɛf

Strɛs:

  • Infεkshכn dεm
  • Ɔpreshɔn
  • If yu it smɔl kalori (espɛshali we yu it smɔl kabɔhaydrɛt) (`Calorie deprivation`)
  • Saikɔlɔjik strɛs

Wetin na di fiuja fɔ pipul dɛm wae gɛt AHP? (Prɔgnosis) .

Dis kin difrɛn bad bad wan frɔm wan pɔsin to ɔda pɔsin. Bɔrku pipul nɔr kin ɛva gɛt dis sik . Pan di wan dɛn we kin du dat, bɔku pipul dɛn kin gɛt wan ɔ sɔm atak dɛn nɔmɔ insay dɛn layf. Pan ɔl we atak kin mek dɛn layf de pan denja, bɔku pipul dɛn kin wɛl ful wan if dɛn tek mɛrɛsin kwik kwik wan . Na lɛk 5% pan di pipul dɛm wae gɛt AHP kin gɛt sɔm kayn sik wae dɛn kin gɛt ɔltɛm ɔr kin gɛt dis sik. Fɔ dɛn pipul ya, bɔku tɛm, di mɛrɛsin dɛn we dɛn kin gi fɔ mek dɛn nɔ gɛt di sik kin ɛp, ɛn dɛn kin tek di liva transplant as di las tin we dɛn fɔ du.

Aw a kin tek kia ɔf misɛf we a de liv wit AHP?

  • Avɔyd tin dɛn we kin mek yu nɔ gɛt prɔblɛm. Rɔk, smok, ɛn drɔgs fɔ de bifo pan di tin dɛn we yu fɔ avɔyd. Yu kin want fɔ tɔk bak to yu dɔktɔ bɔt ɔda tin dɛn we yu go yuz pas sɔm pan di mɛrɛsin dɛn we yu de tek.
  • It fayn it ɛn balans it. Nɔ it bɔku bɔku it dɛn ɛn nɔ fɔ it tin dɛn we nɔ gɛt bɔku kab. If yu de fast bifo yu du mɛdikal tɛst ɔ ɔpreshɔn, tɔk to yu dɔktɔ bɔt am.
  • Gɛt dɔktɔ chɛk-ap ɔltɛm. Ivin if yu nɔ gɛt di sik, yu dɔktɔ go de chɛk yu liva, yu kidni, ɛn yu blɔd prɛshɔn ɔltɛm.

Akyu epatik pɔfyuria na sik we nɔ kin bɔku, so di pipul dɛn nɔ kin no bɔt am bɛtɛ. We atak kam wantɛm wantɛm wit bad bad sayn dɛm, i kin rili kɔnfyus ɛn mek yu fred. Sɔm pipul dɛn kin spɛn bɔku ia fɔ tray fɔ no wetin bad wit dɛn ɛn wetin dɛn go ebul fɔ du. If yu sɔspɛkt se yu gɛt AHP, wan jenɛtik tɛst kin kɔnfɔm am. We yu dɔn no, i go rili ɛp yu fɔ mek dɛn nɔ atak yu ɛn kɔntrol dɛn.

Di impɔtant tin dɛn we yu nid fɔ no (Take-Home Message) .

Okay, so mek a sɔma sɔm pan di tin dɛn we yu nid fɔ mɛmba frɔm wetin wi dɔn tɔk bɔt:

  • AHP na wan sik we nɔ kin apin so ɔltɛm we kin bigin na di liva ɛn i kin afɛkt di nervɔs sistɛm .
  • dis na bi כs di εnzym dεm we dεn nid fכ mek hεm , we de mek tכxik tin dεm we dεn kכl pכfyurin prεkursכr dεm de kכlכm na di bכdi.
  • Di sayn dɛm kin difrɛn, frɔm wae yu nɔr gɛt ɛnitin fɔ du wit am, yu bɛlɛ de at bad bad wan, yu nerv pen, yu maynd prɔblɛm, yu skin prɔblɛm, ɛn ivin yu urine in kɔlɔ kin chenj.
  • Ivin if di jεnεtik mכtεshכn de, di sayn dεm nכ kin apia witout trigεr ( lεk כmon, sכm mεdikeshכn, alcohol, εn strεs).
  • Yurin tɛst ɛn jenɛtik tɛst impɔtant fɔ no if pɔsin gɛt di sik.
  • Di men tin na fɔ kɔntrol ɛn protɛkt atak tru tritmɛnt. `Hemin` vaysin, pen kil, ɛn sɔm spɛshal mɛrɛsin dɛn kin yuz. Insay di kes dɛm we kin tranga pas ɔl, fɔ transplant liva na sɔlv bak.
  • Yu kin liv fayn wit dis sik bay wae yu nɔr de du tin wae de mek yu nɔr gɛt dis sik, liv yu layf fayn fayn wan, ɛn wae yu de chɛk yu ɔltɛm .

A op se dis infɔmeshɔn go ɛp yu. Mɛmba se if yu gɛt ɛni wan pan dɛn sik ya, nɔ fred fɔ go to dɔktɔ ɛn tɔk bɔt am.


` Akyu hεpatik pכfyuria, AHP, liva, nεv sεstem, jεnεtik sik dεm, pכfyurin, simptom dεm, tritmεnt

⚠️ Important: The medical articles and information on Nirogi Lanka are for general awareness only, and are by no means a substitute for professional medical advice, diagnosis, or treatment. For any medical problem you have, consult a qualified physician immediately.

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Yu gɛt wan strenj sik we yu nɔ ebul fɔ ɔndastand? I kin bi Akyu Ɛpatik Pɔfyria!

Yu gɛt wan strenj sik we yu nɔ ebul fɔ ɔndastand? I kin bi Akyu Ɛpatik Pɔfyria!

Yu kin fil lɛk se yu bɛlɛ kin at we yu nɔ go ebul fɔ imajin, sɔntɛnde yu at kin ebi, ɛn yu an ɛn fut dɛn kin swɛ? Sɔntɛm ivin dɔktɔ dɛn nɔ kin no wetin i bi. Wan pan dɛn strenj, we tan lɛk se nɔ gɛt natin fɔ du wit dɛnsɛf, we wi go tɔk bɔt tide na Acute Hepatic Porphyria (AHP). Nɔ wɔri, dis na tin we kɔmplikt smɔl, bɔt lɛ wi ɔndastand am simpul wan.

Wetin rili na akyu ɛpatik pɔfyuria (AHP)?

Fɔ tɔk am simpul wan, AHP na wan sik we nɔ kin bɔku we pɔsin kin gɛt we i kam pan jɛnɛtiks . I kin bigin na yu liva . Bɔt i kin afɛkt yu nervɔs sistɛm bak , we kin mek yu gɛt sɔm sayn dɛn ɔlsay na yu bɔdi. Tink bɔt am, sɔntin de we dɛn kɔl hεm, we na pat pan di ɛmoglobin we de na wi blɔd. wi bכdi nid difrεn εnzym dεm fכ mek dis hεm. Pɔsin we gɛt AHP nɔ gɛt bɛtɛ, ɔ i nɔ gɛt sɔm pan di ɛnzaym dɛn we i nid fɔ mek dis hεm.

Wetin kin apin we dis kin apin? Sɔm pan di kemikal dɛn we dɛn fɔ dɔn yuz fɔ mek hεm, kin lɛf. Wi kin kɔl dɛn tin ya we lɛf na di bɔdi pɔfyurin prɛkursɔ . We dɛn tin ya bɔku, dɛn kin gɛt pɔyzin to di bɔdi. insay AHP, dεn tכxin dεm ya kin fכs bכku na di liva. Dɔn, we dɛn go insay di blɔd ɛn miks wit di nerv dɛm, di sayn dɛm kin bigin fɔ sho. Yu ɔndastand?

Difrɛn kayn AHP dɛn de?

Yes, 4 men kayn AHP de. dεn fכ tכp dεm ya dεn sheb to di kayn εnzym dεm we a bin tכk bכt, i dipכnt pan us εnzym dεm we nכ de. εvri tכp dεn gi in nem afta di pat we dεn kכl "hεpatik" biכs i de stat na di liva, εn di pat we dεn kכl "acute" biכs di simptom dεm de sho wan wan. Fɔ mek dɛn go ɔda frɔm di kayn we we kɔmɔn pas ɔl to di wan we nɔ kɔmɔn, na di kayn dɛn ya:

1. Akyu Intamitεnt Pכfyria (AIP): Dis na di wan we kכmכn pas כl. wan mכtεshכn na di HMBS jin de mek di εnzym haydroksimεtilbilan sεntez (HMBS) (dεn kכl am bak pכrfobilinojen diaminaz (PBGD)) nכ de. dis kin bi nyu jεnεtik mכtεshכn we de apin, כ i kin inhεrit as כtosom dכminant trayt (i.e., di sik kin apin ivin if na wan mama εn papa nכmכ gεt di jin).

2. Variegate Porphyria (VP): wan mכtεshכn na di PPOX jin de mek di εnzym protoporphyrinogen oxidase (PPO כ PPOX) nכ de. dis kin inhεrit bak as כtosom dכminant כ כtosom rεsεsiv.

3. Hεriditary Coproporphyria (HCP): wan mכtεshכn na di jin `CPOX` de rizulta in dεfisit fכ di εnzym `coproporphyrinogen oxidase (CPOX).` dis kin inhεrit bak as כtosomal dכminant כ כtosomal rεsεsiv.

4. ALAD-Dεfisiεns Pכfyuria (ALAD-Dεfisiεns Pכfyuria - ADP):mכtεshכn na di `ALAD` jin de mek di εnzym `delta-aminolevulinic acid dehydratase (ALAD) dεfichεns.` i de inhεrit insay wan `autosomal recessive` we (i.e., di sik kin כnli apin if di mama εn papa tu inhεrit di jin).

Aw AHP de afɛkt mi?

Dis kin mek wi sɔprayz, bikɔs AHP nɔ kin afɛkt ɔlman di sem we. Sɔm pipul dɛn gɛt di jin muteshɔn fɔ am bɔt dɛn nɔ kin ɛva gɛt ɛni sayn . Ɔda pipul dɛn kin gɛt "atak" fɔ di sayn dɛm we kin apin wan ɔ tu tɛm nɔmɔ insay dɛn layf. Bɔt sɔm pipul dɛn kin gɛt dɛn kayn atak ya ɔltɛm, ɛn ɔda wan dɛn kin gɛt am fɔ lɔng tɛm , we min se dɛn kin kɔntinyu fɔ gɛt sɔm sayn dɛn we kin afɛkt dɛn layf ɛvride. Sɔntɛnde, atak kin so bad dat yu go nid fɔ kɛr yu go na di ɔspitul imejensi rum. Yu kin gɛt siriɔs pen na yu nerv, yu skin kin chenj, ɛn yu kin gɛt sɔm kayn sik wae gɛt fɔ du wit yu nervɔs sistɛm.

Udat go mɔs gɛt dis?

AHP kin afɛkt pipul dɛn we gɛt ɛni kɔlɔ ɔ ɛni kɔlɔ. I kin kɔmɔt frɔm wan ɔ ɔl tu mama ɛn papa dɛn we gɛt di jin muteshɔn. I sɔprayz fɔ no se bɔku pipul dɛn nɔ kin gɛt dis sik . Fɔ mek di simptom dɛn bigin, di jin we de chenj nɔmɔ nɔ go du fɔ am. Sεvεra כda `trigεr` dεm mכst de de bak.

Wetin na dɛn tin ya we kin mek pɔsin gɛt dis sik?

  • di chenj dεm we de apin na di כmon dεm (εspεshali we uman de bכn, we uman bεlε, εn di mεnstral saykl) .
  • Sɔm mɛrɛsin dɛn
  • Yuz rɔm
  • Fɔ smok
  • Siriɔs strɛs
  • Strikt kɔntrol fɔ it (espɛshali fɔ stɔp di kalori) .

I fayn bak fɔ no se 80% pan di wan dɛn we gɛt di sik na uman dɛn we dɔn ol fɔ bɔn pikin .

Aw kɔmɔn tin fɔ AHP?

I nɔ kin izi fɔ tɔk fɔ tru, bikɔs bɔku tɛm dɛn nɔ kin no dis sik fayn fayn wan. Ɔlsay na di wɔl, dɛn se na lɛk 5 pan 100,000 pipul dɛn kin gɛt am. pan di kayn dεm we wi bin dכn tכk bכt, `Acute Intermittent Porphyria (AIP)` na di tכp we dεn kin si pan כnli 80% pan di kεys dεm we dεn ripot. `ALAD-Deficiency Porphyria (ADP)` na di kayn we we nɔ kɔmɔn, wit 9 kes dɛm nɔmɔ we dɛn ripɔt ɔlsay na di wɔl. Imajin, na wan pan ɛvri 10 pipul dɛm wae gɛt di jin muteshon wae gɛt fɔ du wit AHP go gɛt sɔm kayn sik.

Wetin na di sayn dɛm fɔ AHP?

Di sayn dɛm fɔ AHP rili strenj. Dɛn kin tranga fɔ di sikman ɛn dɔktɔ fɔ ɔndastand, bikɔs di sik kin kam wantɛm wantɛm, dɛn kin tranga, ɛn i kin tan lɛk se dɛn nɔ gɛt natin fɔ du wit dɛnsɛf.

Na sɔm pan dɛn tin ya:

Nɛv pen

Dis pen kin apin na difrɛn say dɛn na di bɔdi:

  • Siriɔs bɛlɛ pen – Dis na di fɔs sayn we kin apin to bɔku pipul dɛn.
  • Chɛst de pen
  • Bak pen
  • Pen na di an ɛn fut

Prɔblɛm dɛn we de na di dijestiv sistɛm

Bikɔs di nerv dɛn kin afɛkt, prɔblɛm kin apin bak wit di dijestiv sistɛm:

  • Nɔs ɛn vɔmit
  • Di we aw pɔsin nɔ de it fayn
  • Banbɛlɛ
  • Rɔnbɛlɛ

Sεntri nεv sεstem simptom dεm

Dɛn tin ya gɛt fɔ du wit di bren ɛn maynd:

  • Wɔri
  • Nɔ ebul slip
  • Pwɛl at
  • Delirium we gɛt di sik
  • Hallucinations – fɔ si ɔ yɛri tin dɛn we nɔ rili de
  • Status epilepticus/sɛiz (`Sɛiz`) .

Di simptom dɛm fɔ di periferik nɛvɔ sistɛm

dis dεm kin kכnεkt to di nεv dεm na כda pat dεm na di bכdi:

  • Di mɔsul dɛn we wik
  • Di an ɛn fut dɛn kin swɛla ɛn tingling
  • Taya
  • Di sɛns we pɔsin kin gɛt we i nɔ de fil fayn
  • Mɔsul dɛn we de paralayz
  • Respiratory paralysis – Dis kin rili denja ɛn i kin mek i nɔ izi fɔ blo.

Di simptom dɛm fɔ di ɔtonomik nervɔs sistɛm

Dis na tin dɛn we kin apin fɔ dɛnsɛf, tin dɛn we wi nɔ ebul fɔ kɔntrol:

  • Di at we de blo
  • Di ay blɔd prɛshɔn

Di simptom dɛm na di skin (espɛshal wan na `Variegate Porphyria` ɛn `Hereditary Coproporphyria`)

Pipul wae gɛt dis tu kayn kin gɛt prɔblɛm wit dɛn skin wae dɛn de na di san :

  • Fɔ fil we di san de shayn
  • Rash we de blistering
  • Di kɔlɔ we di skin kin chenj (Pigmentation) .
  • Skaring we de mek pɔsin gɛt skata

Di tin we impɔtant pas ɔl na dat we yu gɛt pɔfyuria, yu urine kin chenj in kɔlɔ . We dɛn pɔfyurin prɛkursɔ dɛn we a bin tɔk bɔt gɛda na yu bɔdi ɛn kɔmɔt na yu urine, yu urine kin tɔn rɛd-pɔpul kɔlɔ . Di wɔd "porphyria" kɔmɔt frɔm di Grik wɔd "porphura," we min "pɔpul." Di pɔfyurin dɛn we de na wi rɛd blɔd sɛl dɛn na dɛn de gi blɔd in rɛd kɔlɔ.

Wetin na AHP atak?

Fɔ bɔrku pipul dɛm wae gɛt AHP simptom dɛm, dɛn tin ya kin kam insay di fɔm wae dɛn kin gɛt "atak" wan wan tɛm. Sɔm pipul dɛn kin gɛt dɛn mɔ ɛn mɔ, sɔm nɔ kin gɛt am. Sɔm pipul dɛn kin gɛt dɛn bad bad wan, ɛn ɔda wan dɛn nɔ kin gɛt am. Bɔrku tɛm, dɛn atak ya kin las fɔ sɔm dez, dɔn smɔl smɔl dɛn kin bɔku ɛn stɔp. If yu gɛt siriɔs, frayd sik, yu fɔ go na ɔspitul. As a bin se, tin dɛm lɛk respiratory paralysis, we kin mek i nɔ izi fɔ blo, na kɔndishɔn dɛm we nid fɔ gɛt mɛrɛsin kwik kwik wan.

Di sayn we kin sho se pɔsin gɛt AHP atak na we i nɔ kin ɛksplen, we i kin gɛt bad bad bɛlɛ pen.. Mɔr pas 90% pan di pipul dɛm wae gɛt AHP kin go to dɔktɔ bikɔs ɔf dis bɛlɛ pen. Bɔt bikɔs bɔku tin dɛn de we kin mek pɔsin fil pen na in bɛlɛ, ɛn bikɔs ɛkstrem rayt ɔ CT skan nɔ kin ebul fɔ fɛn di tin we kin mek i fil pen (bikɔs na pen na di nerve), i kin rili at fɔ no. Bikɔs dis bɛlɛ pen kin kam wit tin dɛn lɛk nɔys, vɔmit, ɛn kɔnstipɛshɔn, dɔktɔ dɛn kin tink se na sik we de mek pɔsin digest. If yu gɛt prɔblɛm wit yu maynd ɛn sɛns bak, dɛn kin tink bak se na nyurolɔjik sik. We dɛn sik ya we tan lɛk se nɔ gɛt natin fɔ du wit dɛnsɛf kam togɛda, i nid fɔ go to dɔktɔ we no bɔt am fɔ no se i kin bi pɔfyuria.

Us sik dɛn we nɔ de mɛn we AHP kin mek pɔsin gɛt?

Pipul wae gɛt dis sik wae nɔr kin pasmak (we nɔr kin gɛt bɛtɛ ɛnzaym) nɔr kin gɛt dis sik wae nɔr de dɔn. Dɛn kin jɔs gɛt wan ɔ tu atak insay dɛn layf. If dɛn no di tin dɛn we kin mek dɛn du dat, dɛn kin avɔyd dɛn. Bɔt sɔm pipul dɛn kin gɛt atak ɔltɛm, ɛn sɔm sayn dɛn kin so ɔltɛm dat dɛn kin tek am se dɛn nɔ de mɛn. Dɛn kayn sayn ya na:

  • Pen
  • We pɔsin taya
  • Vɔmit
  • Nyuropathy ( we yu bɔdi nɔ gɛt bɛtɛ trɛnk, pen, ɔ wik) .

Wetin na di kɔmplikɛshɔn dɛm we AHP kin gɛt?

Di simptom dɛm wae gɛt fɔ du wit di sɛntral nervous system (lɛk wae yu de wɔri, wae yu de si tin, ɛn yu kin gɛt sik) kin apin nɔmɔ wae pɔrsin gɛt atak. Bɔt di sayn dɛm we gɛt fɔ du wit di periferik nervɔs sistɛm (lɛk we di mɔsul dɛn wik ɛn paralayz) kin apin bɔku tɛm, ɛn i kin pwɛl fɔ ɔltɛm afta dɛn dɔn atak am bad bad wan. di akyumyuleshכn fכ dεn pכfyurin prεkursכr dεm kin mek bak sכm כgan dεm de damej fכ lכng tεm. Dɛn tin ya kin bi:

  • Krɔnik haypatɛyshɔn
  • Di sik we nɔ de mɛn na di liva
  • Kidni sik we nɔ de mɛn
  • Praymari liva kansa
  • Pwɛl hat ɛn wɔri

Wetin kin mek pɔsin gɛt AHP?

Di men tin we kin mek pɔsin gɛt AHP na di we aw pɔsin kin chenj in jɛnɛtiks . Bɔt lɛk aw a bin dɔn tɔk bifo tɛm, di sayn dɛn nɔ kin apin wantɛm wantɛm afta di jenɛtik muteshɔn dɔn de. Bɔrku tɛm, di sayn dɛm kin fɔs kin kam wae pɔrsin de bɔn, wit di chenj dɛm na di ɔmon dɛm. Apat frɔm dat, sɔm mɛrɛsin dɛn, drɔgs, fɔ drink pasmak, fɔ stɔp di kalori bad bad wan, ɛn sik dɛn we de put bɔku strɛs pan di bɔdi kin mek bak i gɛt am.

wetin כl dεn trig dεm ya gεt fכ kכmכn na dat dεn de mek di liva in hεm prodakshכn prכsεs. כltu, insay di liva fכ pכsin we gεt AHP, di bayprodukt dεm we di hεm prodakshכn de mek (lεk di pכfyurin prεkursכr dεm) nכ de mεtabolayz fayn, dat na fכ yus כp. fכ dat, dεn lεfεf pכfyurin prεkursכr dεm ya kin kכmכt na di liva. Na we dɛn tin ya gɛda to wan patikyula lɛvul nɔmɔ dɛn kin bigin fɔ afɛkt di nervɔs sistɛm ɛn mek dɛn gɛt sɔm sayn dɛn.

Aw dɛn kin no se pɔsin gɛt AHP? (Diagnosis) .

Fɔ di wan dɛn we gɛt AHPFɔ gɛt kɔrɛkt diagnosis kin tranga, bikɔs di sayn dɛm nɔr kin jɔs bi fɔ dis sik ɛn i kin tan lɛk se dɛn nɔr gɛt ɛnitin fɔ du wit am. Bɔt dɔktɔ we sabi AHP kin sɔprayz se AHP de if yu gɛt bɛlɛ pen wit di sɛntral ɛn periferik nɛvɔ sistɛm simptom dɛm. di kכmbaynshכn fכ dεn tri simptom dεm ya dεn kin kכnsidr am as di "klasik triad" fכ AHP.

If dɔktɔ sɔspɛkt se AHP, dɛn kin du bɔku tɛst fɔ kɔnfirm am.

  • Yurin tɛst: Dɛn kin du dis kwik kwik wan we pɔsin gɛt atak. Yurin kin bi nכmal we yu nכ de gεt atak, bכt we yu atak, di urine go sho εlevεt lεvεl dεm fכ di pכfyurin prεkursכr (PBG εn ALA). Dis nɔto tɛst we dɛn kin du fɔ AHP nɔmɔ, bɔt na bɛsik tɛst we kin ɛp dɔktɔ dɛn fɔ sho yu di rayt say.
  • Jεnεtik tεst: Dis na di bεst εn mכst difinitiv we fכ no AHP (di `gold standad`) . Ilɛksɛf yu gɛt di sik ɔ yu nɔ gɛt di sik, i kin kɔnfɔm if yu gɛt wan jin muteshon we gɛt fɔ du wit AHP. I kin tɛl yu bak us kayn AHP yu gɛt ɛn aw di ɛnzaym dɛfisiɛns de bad bad wan. Dis min se dɛn fɔ tek blɔd ɔ saliva sɛmpul. Yu lokal ɔspitul kin nid bak fɔ sɛn di sampul to spɛshal lab.

Aw dɛn kin trit AHP?

Di tritmɛnt fɔ AHP de pe atɛnshɔn fɔ mek dɛn nɔ atak ɛn kɔntrol di atak dɛn . Yu kin nid fɔ go na ɔspitul we dɛn atak yu. Yu kin nid difrɛn kayn mɛrɛsin fɔ ridyus yu sik. Na di tɛm dɛm wae yu nɔr de gɛt atak, yu kin nid ɔda mɛrɛsin fɔ kɔntrol di tin dɛm wae kin mek yu sik wɔs. If yu ɛn yu dɔktɔ wok togɛda fɔ no di tin dɛn we de ambɔg yu mɔ, yu tritmɛnt go wok fayn.

Tritmɛnt fɔ pɔsin we gɛt siriɔs atak:

  • Hemin injection: If yu atak yu bad bad wan, dɔktɔ kin gi yu injection of hemin insay wan vein. Dis kin ɛp fɔ ridyus di pɔfyrin we de na yu blɔd. Ɛmin na sɔntin we dɛn kin tek frɔm di rɛd blɔd sɛl dɛn ɛn i kin ridyus di pɔfyrin we de na yu bɔdi.
  • Fɔ mek pɔsin nɔ fil pen: We pɔsin gɛt siriɔs atak, dɛn kin nid strɔng mɛrɛsin fɔ mɛn pen lɛk opioid.
  • Phenothiazines: Dɛn kin yuz dɛn tin ya fɔ kɔntrol di bad bad nɔys ɛn vɔmit.
  • IV wata ɛn it: Di sayn dɛm lɛk bɛlɛ pen, nɔs, vɔmit, dayarɛa, ɛn kɔnstipɛshɔn kin mek di bɔdi lɔs kalori ɛn wata we pɔsin atak. AHP kin mek bak yu lɔs tin dɛm lɛk sɔdiɔm ɛn magnɛsiɔm. So, dɛn kin gi wata we gɛt kabɔhaydrɛt ɛn ilɛktrɔlayt insay di bɛlɛ.
  • Di mɛrɛsin dɛn we dɛn kin yuz fɔ mek pɔsin sik:Na lɛk 20% pan di sik pipul dɛn kin nid tritmɛnt fɔ ɛpilepsi we dɛn atak dɛn.

Di tritmɛnt dɛn we yu kin pik fɔ lɔng tɛm:

  • Prɔfylaktik ɛmin: If yu gi smɔl dos fɔ ɛmin wan tɛm insay di wik to pipul dɛn we gɛt atak ɔltɛm, dat kin mek di pɔfyrin nɔ gɛda.
  • Givosiran (GIVLAARI®): Dis na wan kayn jin tɛrapi . i de ridyus di prodakshכn fכ di pכfyurin prεkursכr dεm. Naw dɛn dɔn gri fɔ mek dɛn gi am ɛvri mɔnt fɔ mek dɛn nɔ gɛt AHP pan pipul dɛn we kin gɛt atak ɔltɛm.
  • Ɔmon tɛrapi: If yu AHP atak na yu menstrual saykl, yu dɔktɔ kin gi yu mɛrɛsin lɛk GnRH (Gonadotropin-releasing hormone) analogs. dis dεm de ridyus di bכdi in prodakshכn כmon dεm we dεn kכl εstrojen εn prכjεstεron.
  • Ha blɔd prɛshɔn mɛrɛsin: If yu gɛt ay blɔd prɛshɔn we nɔ de dɔn, dɛn go nid fɔ kɔntrol am wit sɔm patikyula mɛrɛsin dɛn.
  • Liva transplant: Pipul dɛn we kin gɛt atak ɔltɛm, we kin mek dɛn layf de pan denja ɛn we nɔ kin du ɔda tritmɛnt dɛn kin tink bɔt fɔ transplant liva. Dis kin ivin mɛn di sik kpatakpata.

Yu tink se dɛn kin mek dɛn nɔ gɛt AHP?

I nɔ pɔsibul fɔ mek di sik nɔ kam. Bɔt yu kin ɛp fɔ mek yu nɔ gɛt di sik bay we yu avɔyd tin dɛn we kin mek yu gɛt dis sik . If yu nɔ ɛva gɛt atak, i bɛtɛ fɔ avɔyd ɔl di tin dɛn we kin mek yu atak yu. If yu dɔn gɛt atak ɛn yu dɔn no di tin dɛn we mek yu atak am, i go fayn fɔ avɔyd jɔs dɛn patikyula tin dɛn de we mek yu atak am.

Na sɔm pan di tin dɛn we dɛn kin si we kin mek pɔsin sik:

Di kayn mɛrɛsin dɛn we dɛn kin yuz:

  • Antihistamines (sɔm mɛrɛsin fɔ kol ɛn alɛji) .
  • Di tin dɛn we de mek pɔsin fil fayn
  • Di tin dɛn we dɛn kin yuz fɔ mek uman nɔ gɛt bɛlɛ
  • Ɔmon riplesmɛnt tɛrapi

Di we aw dɛn de yuz di tin dɛn we dɛn de yuz:

  • Tabak (`Tobacco`) .
  • Marijuana we dɛn kin yuz
  • Rum
  • Drug dɛn fɔ ɛnjɔy yusɛf

Strɛs:

  • Infεkshכn dεm
  • Ɔpreshɔn
  • If yu it smɔl kalori (espɛshali we yu it smɔl kabɔhaydrɛt) (`Calorie deprivation`)
  • Saikɔlɔjik strɛs

Wetin na di fiuja fɔ pipul dɛm wae gɛt AHP? (Prɔgnosis) .

Dis kin difrɛn bad bad wan frɔm wan pɔsin to ɔda pɔsin. Bɔrku pipul nɔr kin ɛva gɛt dis sik . Pan di wan dɛn we kin du dat, bɔku pipul dɛn kin gɛt wan ɔ sɔm atak dɛn nɔmɔ insay dɛn layf. Pan ɔl we atak kin mek dɛn layf de pan denja, bɔku pipul dɛn kin wɛl ful wan if dɛn tek mɛrɛsin kwik kwik wan . Na lɛk 5% pan di pipul dɛm wae gɛt AHP kin gɛt sɔm kayn sik wae dɛn kin gɛt ɔltɛm ɔr kin gɛt dis sik. Fɔ dɛn pipul ya, bɔku tɛm, di mɛrɛsin dɛn we dɛn kin gi fɔ mek dɛn nɔ gɛt di sik kin ɛp, ɛn dɛn kin tek di liva transplant as di las tin we dɛn fɔ du.

Aw a kin tek kia ɔf misɛf we a de liv wit AHP?

  • Avɔyd tin dɛn we kin mek yu nɔ gɛt prɔblɛm. Rɔk, smok, ɛn drɔgs fɔ de bifo pan di tin dɛn we yu fɔ avɔyd. Yu kin want fɔ tɔk bak to yu dɔktɔ bɔt ɔda tin dɛn we yu go yuz pas sɔm pan di mɛrɛsin dɛn we yu de tek.
  • It fayn it ɛn balans it. Nɔ it bɔku bɔku it dɛn ɛn nɔ fɔ it tin dɛn we nɔ gɛt bɔku kab. If yu de fast bifo yu du mɛdikal tɛst ɔ ɔpreshɔn, tɔk to yu dɔktɔ bɔt am.
  • Gɛt dɔktɔ chɛk-ap ɔltɛm. Ivin if yu nɔ gɛt di sik, yu dɔktɔ go de chɛk yu liva, yu kidni, ɛn yu blɔd prɛshɔn ɔltɛm.

Akyu epatik pɔfyuria na sik we nɔ kin bɔku, so di pipul dɛn nɔ kin no bɔt am bɛtɛ. We atak kam wantɛm wantɛm wit bad bad sayn dɛm, i kin rili kɔnfyus ɛn mek yu fred. Sɔm pipul dɛn kin spɛn bɔku ia fɔ tray fɔ no wetin bad wit dɛn ɛn wetin dɛn go ebul fɔ du. If yu sɔspɛkt se yu gɛt AHP, wan jenɛtik tɛst kin kɔnfɔm am. We yu dɔn no, i go rili ɛp yu fɔ mek dɛn nɔ atak yu ɛn kɔntrol dɛn.

Di impɔtant tin dɛn we yu nid fɔ no (Take-Home Message) .

Okay, so mek a sɔma sɔm pan di tin dɛn we yu nid fɔ mɛmba frɔm wetin wi dɔn tɔk bɔt:

  • AHP na wan sik we nɔ kin apin so ɔltɛm we kin bigin na di liva ɛn i kin afɛkt di nervɔs sistɛm .
  • dis na bi כs di εnzym dεm we dεn nid fכ mek hεm , we de mek tכxik tin dεm we dεn kכl pכfyurin prεkursכr dεm de kכlכm na di bכdi.
  • Di sayn dɛm kin difrɛn, frɔm wae yu nɔr gɛt ɛnitin fɔ du wit am, yu bɛlɛ de at bad bad wan, yu nerv pen, yu maynd prɔblɛm, yu skin prɔblɛm, ɛn ivin yu urine in kɔlɔ kin chenj.
  • Ivin if di jεnεtik mכtεshכn de, di sayn dεm nכ kin apia witout trigεr ( lεk כmon, sכm mεdikeshכn, alcohol, εn strεs).
  • Yurin tɛst ɛn jenɛtik tɛst impɔtant fɔ no if pɔsin gɛt di sik.
  • Di men tin na fɔ kɔntrol ɛn protɛkt atak tru tritmɛnt. `Hemin` vaysin, pen kil, ɛn sɔm spɛshal mɛrɛsin dɛn kin yuz. Insay di kes dɛm we kin tranga pas ɔl, fɔ transplant liva na sɔlv bak.
  • Yu kin liv fayn wit dis sik bay wae yu nɔr de du tin wae de mek yu nɔr gɛt dis sik, liv yu layf fayn fayn wan, ɛn wae yu de chɛk yu ɔltɛm .

A op se dis infɔmeshɔn go ɛp yu. Mɛmba se if yu gɛt ɛni wan pan dɛn sik ya, nɔ fred fɔ go to dɔktɔ ɛn tɔk bɔt am.


` Akyu hεpatik pכfyuria, AHP, liva, nεv sεstem, jεnεtik sik dεm, pכfyurin, simptom dεm, tritmεnt

⚠️ Important: The medical articles and information on Nirogi Lanka are for general awareness only, and are by no means a substitute for professional medical advice, diagnosis, or treatment. For any medical problem you have, consult a qualified physician immediately.

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