Ingaba unesifo esingaqhelekanga ongasiqondiyo? Sisenokuba yiAcute Hepatic Porphyria!

Ngaba uziva ngathi unesisu esibuhlungu ngendlela engenakucingelwa, ngamanye amaxesha unentliziyo enzima, kwaye amalungu akho abuthathaka? Mhlawumbi noogqirha bayadideka ukuba yintoni na. Enye yezi mpawu zingaqhelekanga, ezibonakala zingadibaniyo esiza kuthetha ngazo namhlanje yi-Acute Hepatic Porphyria (AHP). Ungakhathazeki, oku kunzima kancinci, kodwa masikuqonde ngokulula.

Yintoni kanye kanye i-acute hepatic porphyria (AHP)?

Ngamafutshane, i-AHP sisifo esingaqhelekanga kakhulu esibangelwa yimfuza . Siqala esibindini sakho. Kodwa sikwachaphazela nenkqubo yakho yemithambo-luvo, sibangele iimpawu emzimbeni wakho wonke. Cinga ngayo, kukho into ebizwa ngokuba yi-heme, eyinxalenye ye-hemoglobin egazini lethu. Umzimba wethu udinga ii-enzyme ezahlukeneyo ukwenza le heme. Umntu one-AHP unesiphene, okanye uswele, ezinye zee-enzymes ezifunekayo ukwenza le heme.

Kwenzeka ntoni xa oku kusenzeka? Ezinye zeekhemikhali ebekufanele ukuba zisetyenziselwe ukwenza i-heme ziyasala. Sizibiza ngokuba zizinto eziseleyo njenge-porphyrin precursors . Xa ezi zinto ziqokelelana, ziyingozi emzimbeni. Kwi-AHP, ezi zinto zinobungozi ziqala ziqokelelane esibindini. Emva koko, xa zingena egazini kwaye zidibana nemithambo-luvo, iimpawu ziqala ukubonakala. Uyaqonda?

Ngaba kukho iintlobo ezahlukeneyo ze-AHP?

Ewe, kukho iintlobo ezine eziphambili ze-AHP. Ezi ntlobo zine zahlulwe ngokweentlobo zee-enzymes endizikhankanyileyo ngaphambili, kuxhomekeke ekubeni yeyiphi i-enzyme esweleyo. Uhlobo ngalunye lubizwa ngegama lenxalenye ebizwa ngokuba yi-"hepatic" kuba iqala esibindini, kwaye inxalenye ebizwa ngokuba yi-"acute" kuba iimpawu zivela ngequbuliso. Ukusuka kolona hlobo luqhelekileyo ukuya kolungaxhaphakanga, nazi iintlobo:

1. I-Acute Intermittent Porphyria (AIP): Le yeyona ixhaphakileyo. Utshintsho kwi-gene ye-HMBS lubangela ukunqongophala kwi-enzyme i-hydroxymethylbilane synthase (HMBS) (ekwaziwa ngokuba yi-porphobilinogen deaminase (PBGD)). Olu tshintsho lungaba lutshintsho lwe-genetic olusandula ukwenzeka, okanye lunokufunyanwa njenge-autosomal dominant character (oko kukuthi, esi sifo sinokwenzeka nokuba ngumzali omnye kuphela ozuza i-gene).

2. I-Variegate Porphyria (VP): Utshintsho kwi-gene ye-PPOX lubangela ukunqongophala kwi-enzyme protoporphyrinogen oxidase (PPO okanye PPOX). Oku kunokufunyanwa njenge-autosomal dominant okanye i-autosomal recessive.

3. I-Hereditary Coproporphyria (HCP): Utshintsho kwi-gene `CPOX` lubangela ukunqongophala kwe-enzyme `coproporphyrinogen oxidase (CPOX).` Oku kunokufunyanwa njenge-autosomal dominant okanye i-autosomal recessive.

4. I-Porphyria yokuNqanda i-ALAD (i-Porphyria yokuNqanda i-ALAD - ADP):Utshintsho kwi-gene ye-'ALAD' lubangela ukunqongophala kwi-enzyme ye-'delta-aminolevulinic acid dehydratase (ALAD).` Ifunyanwa njengelifa ngendlela ye-'autosomal recessive' (oko kukuthi, esi sifo sinokwenzeka kuphela ukuba abazali bobabini bayasizuza njengelifa i-gene).

I-AHP indichaphazela njani?

Oku kuyamangalisa, kuba i-AHP ayichaphazeli wonke umntu ngokufanayo. Abanye abantu banotshintsho lwezakhi zofuzo kodwa abanazo iimpawu . Abanye banokuba "nohlaselo" lweempawu kube kanye okanye kabini ebomini babo. Kodwa abanye abantu bahlaselwa rhoqo, kwaye abanye bahlaselwa rhoqo , oko kuthetha ukuba bayaqhubeka benazo iimpawu ezichaphazela ubomi babo bemihla ngemihla. Ngamanye amaxesha uhlaselo lunokuba nzima kangangokuba unokufuna ukukhawuleziswa kwigumbi likaxakeka esibhedlele. Unokuba neentlungu ezinkulu zemithambo-luvo, utshintsho lolusu, kunye neempawu ezinxulumene nenkqubo yemithambo-luvo.

Ngubani onokuthi afumane oku?

I-AHP inokuchaphazela abantu balo naluphi na uhlanga okanye umbala. Ingafunyanwa kumzali omnye okanye bobabini abanotshintsho lwezakhi zofuzo. Okumangalisayo kukuba, abantu abaninzi abanazo iimpawu . Ukuze iimpawu zivele, utshintsho lwezakhi zofuzo lodwa alwanelanga. Kufuneka kubekho nezinye izinto ezininzi ezibangela oku .

Ziziphi ezi zinto zibangela oku?

• Utshintsho lweehomoni (ingakumbi ngexesha lokufikisa, ukukhulelwa, kunye nomjikelo wokuya esikhathini)
• Amanye amayeza
• Ukusetyenziswa kotywala
• Ukutshaya
• Uxinzelelo oluqatha
• Ulawulo olungqongqo lokutya (ingakumbi ukuncitshiswa kweekhalori)

Kwakhona kuyaphawuleka ukuba ama-80% abo babonakalisa iimpawu ngabafazi abakwiminyaka yokuzala .

Ixhaphake kangakanani i-AHP?

Kunzima ukuqiniseka, kuba esi sifo asisoloko sichongwa ngokufanelekileyo. Kwihlabathi liphela, kuqikelelwa ukuba malunga nabantu aba-5 kwabayi-100,000 banokuba naso. Kwiintlobo ezikhankanyiweyo ngaphambili, i-'Acute Intermittent Porphyria (AIP)' luhlobo olubonwa kwi-80% kuphela yeemeko ezixeliweyo. I-'ALAD-Deficiency Porphyria (ADP)' lolona hlobo lungaqhelekanga, kwaye zi-9 kuphela iimeko ezixeliweyo kwihlabathi liphela. Khawuthelekelele, ngumntu omnye kuphela kwabayi-10 abane-gene mutation enxulumene ne-AHP oya kuba neempawu.

Zithini iimpawu ze-AHP?

Iimpawu ze-AHP zingaqhelekanga kakhulu. Zinokuba nzima ukuba izigulana kunye noogqirha baziqonde, kuba iimpawu zinokuvela ngequbuliso, zibe nzima, kwaye zibonakale zinganxulumananga.

Nazi ezinye zezi mpawu:

Intlungu yemithambo-luvo

Le ntlungu inokwenzeka kwiindawo ezahlukeneyo emzimbeni:

• Intlungu ebuhlungu esiswini – Olu luphawu lokuqala oluvela kubantu abaninzi.
• Iintlungu zesifuba
• Umqolo obuhlungu
• Intlungu ezandleni nasezinyaweni

Iingxaki zenkqubo yokugaya ukutya

Ngenxa yokuba imithambo-luvo iyachaphazeleka, iingxaki kwinkqubo yokugaya ukutya nazo zinokubakho:

• Isicaphucaphu nokuhlanza
• Ukungagayi kakuhle kokutya
• Ukuqhinwa
• Urhudo

Iimpawu zenkqubo ye-nervous central

Ezi zinto zinxulumene ngqo nengqondo:

• Ixhala
• Ukungalali
• Uxinzelelo
• I-Delirium
• Izinto ezingabonakaliyo – ukubona okanye ukuva izinto ezingekhoyo ngokwenene
• Isimo sokuxhuzula/ukuxhuzula (`Ukuxhuzula`)

Iimpawu zenkqubo ye-nervous system

Ezi zidibene nemithambo-luvo kwezinye iindawo zomzimba:

• Ubuthathaka bemisipha
• Ukungaziva mnandi kunye nokurhawuzelela kwamalungu omzimba
• Ukudinwa
• Ukulahleka kweemvakalelo
• Ukukhubazeka kwemisipha
• Ukukhubazeka kokuphefumla – Oku kuyingozi kakhulu kwaye kunokubangela ubunzima bokuphefumla.

Iimpawu zenkqubo ye-nervous ezimeleyo

Ezi zizinto ezenzeka ngokuzenzekela, izinto esingenalawulo kuzo:

• Ukubetha kwentliziyo
• Igazi elonyukayo

Iimpawu zolusu (ingakumbi kwi-'Variegate Porphyria' kunye ne-'Heroditary Coproporphyria')

Abantu abanezi ntlobo zimbini banokufumana iingxaki zolusu xa bechatshazelwa lilanga :

• Uvelwano elangeni
• Amaqhuqhuva aqhumayo
• Ukutshintsha kombala wolusu (Ukutshintsha kombala)
• Amanxeba

Eyona nto ibalulekileyo kukuba xa une-porphyria, umchamo wakho unokutshintsha umbala . Xa ezo zinto ziphambili ze-porphyrin endizikhankanyileyo ziqokelelana emzimbeni wakho kwaye ziphuma kumchamo wakho, umchamo wakho ungajika ube bomvu-mfusa . Igama elithi "porphyria" livela kwigama lesiGrike elithi "porphura," elithetha "mfusa." Ii-porphyrins ezikwiiseli zethu ezibomvu zegazi zizo ezenza igazi libe nombala obomvu.

Yintoni uhlaselo lwe-AHP?

Kwabaninzi abantu abaneempawu ze-AHP, ezi ziza ngohlobo "lokuhlaselwa" ngamanye amaxesha. Abanye abantu bazifumana rhoqo, abanye abazifumana rhoqo. Abanye abantu bazifumana kakhulu, ngelixa abanye bengazifumani. Ngokwesiqhelo, ezi ziganeko zihlala iintsuku ezimbalwa, zize zinyuke kancinci kancinci. Ukuba uneempawu eziqatha nezoyikisayo, kufuneka uye esibhedlele. Njengoko benditshilo, izinto ezifana nokuphefumla, okwenza kube nzima ukuphefumla, ziimeko ezifuna unyango olungxamisekileyo.

Uphawu oluqhelekileyo lohlaselo lwe-AHP yintlungu yesisu engachazwanga, eqatha.. Abantu abangaphezu kwama-90% abane-AHP baya kugqirha ngenxa yale ntlungu yesisu. Nangona kunjalo, ekubeni zininzi izizathu zentlungu yesisu, kwaye ekubeni i-X-ray okanye i-CT scan ingenakukwazi ukufumana unobangela wale ntlungu (kuba yintlungu yemithambo-luvo), kunzima kakhulu ukuyifumanisa. Ngenxa yokuba le ntlungu yesisu ihamba nezinto ezifana nokucaphuka, ukuhlanza, kunye nokuqhina, oogqirha banokucinga ukuba sisifo sokugaya ukutya. Ukuba nawe uneengxaki zengqondo nezeemvakalelo, banokucinga ukuba sisifo semithambo-luvo. Xa ezi mpawu zingabonakaliyo zidibana, kuyimfuneko ukubona ugqirha owaziyo ngaso ukuze aqonde ukuba isenokuba yi-porphyria.

Zeziphi iimpawu ezingapheliyo ezinokubangela i-AHP?

Abantu abanesifo esincinci (ukungabikho kwee-enzyme ezininzi) abafane babe neempawu ezingapheliyo. Basenokuba nohlaselo olunye okanye ezimbini ebomini babo. Ukuba bayaziqonda izinto ezibangela ezi mpawu, banokuziphepha. Nangona kunjalo, abanye abantu bahlaselwa rhoqo, kwaye ezinye iimpawu zihlala zixhaphakile kangangokuba zinokuthathwa njengezingapheliyo. Ezi mpawu ziquka:

• Intlungu
• Ukudinwa
• Isicaphucaphu
• I-Neuropathy ( ukungaziva mnandi, iintlungu, okanye ubuthathaka kwimilenze)

Zithini iingxaki ze-AHP?

Iimpawu ezinxulumene nenkqubo ye-nervous central (ezifana nokuxhalaba, ukubona izinto ezingekhoyo, kunye nokuxhuzula) zihlala zenzeka kuphela ngexesha lohlaselo. Nangona kunjalo, iimpawu ezinxulumene nenkqubo ye-nervous peripheral (ezifana nobuthathaka bemisipha kunye nokukhubazeka) zinokwenzeka rhoqo, kwaye umonakalo osisigxina unokwenzeka emva kohlaselo olunzima. Ukuqokelelana kwezi porphyrin precursors kunokubangela umonakalo wexesha elide kwizitho ezithile zomzimba. Ezi zinokubandakanya:

• Uxinzelelo lwegazi olungapheliyo
• Isifo sesibindi esingapheliyo
• Isifo sezintso esingapheliyo
• Umhlaza wesibindi oyintloko
• Ukudakumba kunye noxinzelelo

Yintoni ebangela i-AHP?

Eyona nto ibangela i-AHP kukuguquka kwezakhi zofuzo . Kodwa njengoko benditshilo ngaphambili, iimpawu azibonakali kwangoko emva kokuba utshintsho lwezakhi zofuzo lukhona. Kwiimeko ezininzi, iimpawu ziqala ukubonakala ngexesha lokufikisa, kunye notshintsho lweehomoni. Ukongeza, amayeza athile, iziyobisi, ukusela utywala ngokugqithisileyo, ukunqongophala kweekhalori, kunye nezifo ezibeka uxinzelelo olukhulu emzimbeni nazo zinokubangela loo nto.

Zonke ezi zinto zibangela ukuba kubekho i-heme esibindini. Nangona kunjalo, kwisibindi somntu one-AHP, imveliso ye-heme (ezifana nee-porphyrin precursors) ayisetyenziswa kakuhle, oko kukuthi, ayisetyenziswanga ngokupheleleyo. Ke ngoko, ezi zinto ziseleyo ze-porphyrin precursors ziqokelelana esibindini. Kuphela xa ezi zinto ziqokelelana ukuya kwinqanaba elithile apho ziqala ukuchaphazela inkqubo yemithambo-luvo kwaye zibangele iimpawu.

Ifunyaniswa njani i-AHP? (Ukuxilongwa)

Kwabo bane-AHPUkufumana uxilongo oluchanekileyo kunokuba nzima, kuba iimpawu azikho kule meko kuphela kwaye zinokubonakala zinganxulumananga. Nangona kunjalo, ugqirha oqhelene ne-AHP unokurhanela i-AHP ukuba unentlungu yesisu kunye neempawu zenkqubo yemithambo-luvo esembindini kunye neye-peripheral. Ukudibana kwezi mpawu zintathu kuthathwa njenge "classic triad" ye-AHP.

Ukuba ugqirha urhanela ukuba une-AHP, angenza iimvavanyo ezininzi ukuqinisekisa oko.

• Uvavanyo lomchamo: Oku kungenziwa ngokukhawuleza ngexesha lohlaselo. Umchamo usenokuba yinto eqhelekileyo xa ungenahlaselo, kodwa ngexesha lohlaselo, umchamo uya kubonisa amanqanaba aphezulu e-porphyrin precursors (PBG kunye ne-ALA). Olu asilulo uvavanyo olwenziwa kuphela kwi-AHP, kodwa luvavanyo olusisiseko olunokunceda oogqirha ukuba bakubonise kwicala elifanelekileyo.
• Uvavanyo lwe-genetic: Le yeyona ndlela ilungileyo necacileyo yokuxilonga i-AHP ('umgangatho wegolide') . Nokuba uneempawu okanye awunazo, inokuqinisekisa ukuba une-gene mutation enxulumene ne-AHP. Ingakuxelela nokuba loluphi uhlobo lwe-AHP onalo kunye nokuba ukungabikho kwe-enzyme kungakanani na. Oku kuquka ukuthatha isampuli yegazi okanye yamathe. Isibhedlele sakho sasekuhlaleni sinokufuna ukuthumela isampuli kwilabhoratri ekhethekileyo.

Inyangwa njani i-AHP?

Unyango lwe-AHP lugxile ekuthinteleni nasekulawuleni uhlaselo . Usenokufuna ukulaliswa esibhedlele ngexesha lohlaselo. Usenokufuna iintlobo ezahlukeneyo zamayeza ukunciphisa iimpawu zakho. Ngamaxesha apho ungenazo iintlaselo, unokufuna amanye amayeza okulawula izinto ezibangela ukuba iimpawu zakho zibe zimbi kakhulu. Ukuba wena nogqirha wakho nisebenzisana ukuchonga izinto ezibangela ukuba uhlaselo luchaphazeleke kakhulu, unyango lwakho luya kusebenza ngakumbi.

Unyango lohlaselo oluqatha:

• Inaliti yeHemin: Xa uhlaselwe kakhulu, ugqirha angakunika inaliti yehemin emthanjeni. Oku kunceda ukunciphisa ubungakanani beporphyrin egazini lakho. IHemin yinto ethathwa kwiiseli ezibomvu zegazi kwaye inciphisa ubungakanani beporphyrin emzimbeni wakho.
• Ukuphelisa iintlungu: Xa uhlaselo lunzima, kufuneka amayeza okudambisa iintlungu anamandla afana nee-opioid.
• IiPhenothiazines: Ezi zisetyenziselwa ukulawula isicaphucaphu esinzima kunye nokuhlanza.
• Ulwelo lwe-IV kunye nesondlo: Iimpawu ezifana nentlungu yesisu, isicaphucaphu, ukuhlanza, urhudo, kunye nokuqhina kwesisu zinokubangela ukuba umzimba ulahlekelwe ziikhalori namanzi ngexesha lokuhlaselwa. I-AHP inokubangela ukulahleka kwezinto ezifana ne-sodium kunye ne-magnesium. Ke ngoko, ulwelo oluqulethe iicarbohydrate kunye nee-electrolytes lunokunikwa ngemithambo yegazi.
• Amayeza okuxhuzula:Malunga nama-20% ezigulana zinokufuna unyango lwesifo sokuwa ngexesha lohlaselo.

Iindlela zonyango lwexesha elide:

• I-hemin yokuthintela: Ukunika abantu abahlaselwa rhoqo iidosi ezincinci ze-hemin kanye ngeveki kunokuthintela ukuqokelelwa kwee-porphyrins.
• I-Givosiran (GIVLAARI®): Olu luhlobo lonyango lwezakhi zofuzo . Lunciphisa ukuveliswa kwezinto ezibangela i-porphyrin. Ngoku luvunyiwe njengenaliti yenyanga ukuthintela iimpawu ze-AHP kubantu abanokuhlaselwa rhoqo.
• Unyango lweehomoni: Ukuba uhlaselo lwakho lwe-AHP lubangelwa kukujikeleza kwakho kokuya exesheni, ugqirha wakho unokukunika amayeza afana ne-GnRH (i-Gonadotropin-releasing hormone) analogs. La mayeza anciphisa ukuveliswa kweehomoni ze-estrogen kunye ne-progesterone emzimbeni.
• Amayeza oxinzelelo lwegazi oluphezulu: Ukuba uxinzelelo lwegazi oluphezulu olungapheliyo luvela, kuya kufuneka lulawulwe ngamayeza athile.
• Ukufakelwa kwesibindi: Abantu abahlaselwa rhoqo, nto leyo esongela ubomi kwaye abangazange baphendule kwezinye iindlela zonyango banokucinga ngokufakelwa isibindi. Oku kunokusinyanga ngokupheleleyo esi sifo.

Ngaba i-AHP ingathintelwa?

Akunakwenzeka ukuthintela ukuba esi sifo singabikho. Nangona kunjalo, unokunceda ekuthinteleni iimpawu ukuba zingabikho ngokuphepha izinto ezibangela esi sifo . Ukuba awukaze ube nohlaselo, kungcono ukunqanda zonke izinto ezinokubangela esi sifo. Ukuba ukhe waba nohlaselo kwaye wazichonga izinto ezibangele esi sifo, kunokwanela ukuphepha ezo zinto zibangela esi sifo.

Nazi ezinye zezinto ezibangela ukuba umntu aqalise ukucaphuka:

Iintlobo zamayeza:

• Ii-Antihistamines (amayeza athile omkhuhlane kunye nee-allergies)
• Izithomalalisi
• Izithinteli zokukhulelwa ezithethwa ngomlomo
• Unyango lokutshintsha ihomoni

Ukusetyenziswa kwezinto:

• Icuba (`Icuba`)
• Intsangu
• Utywala
• Iziyobisi zokuzonwabisa

Uxinzelelo:

• Iintsholongwane
• Utyando
• Ukutya iikhalori eziphantsi (ingakumbi ukutya iicarbohydrate eziphantsi) ('Ukungabi nakhalori`)
• Uxinzelelo lwengqondo

Liyintoni ikamva labantu abane-AHP? (Ingqikelelo)

Oku kuyahluka kakhulu kumntu nomntu. Abantu abaninzi abaze babe neempawu . Kwabo banazo, uninzi lwabantu lunehlaselo elinye okanye ezimbalwa ebomini babo. Nangona uhlaselo lunokuba yingozi ebomini, uninzi lwabantu luyaphola ngokupheleleyo ngonyango olukhawulezileyo . Malunga ne-5% yabantu abane-AHP baneempawu ezixhaphakileyo okanye ezingapheliyo. Kwaba bantu, amayeza okuthintela ahlala eluncedo, kwaye ukufakelwa isibindi kunokuthathwa njengento yokugqibela.

Ndingazinyamekela njani xa ndiphila ne-AHP?

• Ziphephe izinto ezibangela le ngxaki. Utywala, ukutshaya, kunye neziyobisi kufuneka zibe sentloko kuluhlu lwezinto omele uziphephe. ​​Ungathetha nogqirha wakho ngezinye iindlela onokuzisebenzisa endaweni yamanye amayeza owasebenzisayo.
• Yitya ukutya okunempilo nokunesondlo esifanelekileyo. Kuphephe ukutya okune-carbohydrate engaphantsi kakhulu kunye nokutya okune-carbohydrate ephantsi. Ukuba uzila ukutya ngaphambi kovavanyo okanye utyando, thetha nogqirha wakho ngako.
• Fumana uhlolo lwezonyango rhoqo. Nokuba awunazo iimpawu, ugqirha wakho uza kuhlola rhoqo isibindi sakho, izintso kunye noxinzelelo lwegazi.

I-Acute hepatic porphyria sisifo esingaqhelekanga, ngoko ke uluntu ngokubanzi alunalo ulwazi ngaso. Xa uhlaselo lufika ngequbuliso luneempawu ezinzima, luyadida kwaye luyoyikisa. Abanye abantu bachitha iminyaka bezama ukufumanisa ukuba yintoni engalunganga ngabo kunye noko banokukwenza. Ukuba ukrokrela ukuba une-AHP, uvavanyo lwemfuza lunokuqinisekisa oko. Wakuba uyazi, kuya kuba luncedo olukhulu kuwe ukuthintela uhlaselo kunye nokuzilawula.

Izinto ezibaluleke kakhulu ekufuneka uzazi (Umyalezo oya nawo ekhaya)

Kulungile, makhe ndishwankathele ezinye zezinto ekufuneka uzikhumbule koko sithethe ngako:

• I-AHP sisifo esingaqhelekanga semfuza esiqala esibindini kwaye sichaphazele inkqubo yemithambo-luvo .
• Oku kungenxa yokunqongophala kwee-enzymes ezifunekayo kwinkqubo yokwenza i-heme , nto leyo ebangela ukuba izinto ezinobuthi ezibizwa ngokuba zii-porphyrin precursors ziqokelele emzimbeni.
• Iimpawu zingahluka, ukusuka kwiintlungu zesisu ezingadibaniyo, iintlungu zemithambo-luvo, iingxaki zengqondo, iingxaki zolusu, kwanotshintsho kumbala womchamo wakho.
• Nokuba kukho utshintsho lwezakhi zofuzo, iimpawu azifane zivele ngaphandle kwezinto ezibangela oku ( ezifana neehomoni, amayeza athile, utywala, kunye noxinzelelo).
• Uvavanyo lomchamo kunye novavanyo lwemfuza zibalulekile ekuxilongweni.
• Eyona nto iphambili kukulawula nokuthintela ukuhlaselwa ngonyango. Kusetyenziswa iyeza lokugonya i-`Hemin`, amayeza okudambisa iintlungu, kunye namayeza athile akhethekileyo. Kwiimeko ezinzima kakhulu, ukufakelwa kwesibindi kukwasisisombululo.
• Ungaphila kakuhle nesi sifo ngokuphepha izinto ezisibangela esi sifo, uphile ubomi obusempilweni, kwaye uhlale ujongwa rhoqo ngugqirha .

Ndiyathemba ukuba olu lwazi luza kukunceda. Khumbula, ukuba unayo nayiphi na kwezi mpawu, ungoyiki ukubona ugqirha uze uthethe ngayo.

I- Acute hepatic porphyria, i-AHP, isibindi, inkqubo yemithambo-luvo, izifo zemfuza, ii-porphyrins, iimpawu, unyango