Ingabe unesifo esingajwayelekile ongasiqondi? Kungaba yi-Acute Hepatic Porphyria!

Uzizwa sengathi unesifo sesisu esingenakucatshangwa, ngezinye izikhathi unenhliziyo esindayo, kanye nokungazweli ezithweni zakho? Mhlawumbe ngisho nodokotela bayahluleka ukuthola ukuthi siyini. Esinye salezi zimpawu ezingavamile, ezibonakala zingahlobene esizokhuluma ngazo namuhla yi-Acute Hepatic Porphyria (AHP). Ungakhathazeki, lokhu kuyinkimbinkimbi kancane, kodwa masikuqonde kalula.

Iyini ngempela i-acute hepatic porphyria (AHP)?

Kalula nje, i-AHP iyisifo esingavamile kakhulu esibangelwa izakhi zofuzo . Siqala esibindini sakho. Kodwa futhi sithinta uhlelo lwakho lwezinzwa, okubangela izimpawu emzimbeni wakho wonke. Cabanga ngakho, kukhona into ebizwa ngokuthi i-heme, eyingxenye ye-hemoglobin egazini lethu. Umzimba wethu udinga ama-enzyme ahlukahlukene ukwenza le heme. Umuntu one-AHP unesifo, noma ukuntuleka, kwamanye ama-enzyme adingekayo ukwenza le heme.

Kwenzekani uma lokhu kwenzeka? Amanye amakhemikhali okwakufanele asetshenziswe ukwenza i-heme asele. Sibiza lawa ma-porphyrin precursors asele. Uma lawa enqwabelana, ayingozi emzimbeni. Ku-AHP, lawa ma-toxin aqala ukunqwabelana esibindini. Bese kuthi, uma engena egazini futhi esebenzisana nemizwa, izimpawu ziqale ukubonakala. Uyaqonda?

Ingabe kunezinhlobo ezahlukene ze-AHP?

Yebo, kunezinhlobo ezine eziyinhloko ze-AHP. Lezi zinhlobo ezine zihlukaniswe ngezinhlobo zama-enzyme engikhulume ngawo ekuqaleni, kuye ngokuthi iyiphi i-enzyme entulekayo. Uhlobo ngalunye luqanjwe ngengxenye ebizwa ngokuthi "isibindi" ngoba iqala esibindini, kanti ingxenye ibizwa ngokuthi "i-acute" ngoba izimpawu zivela ngokuzumayo. Kusukela kohlobo oluvame kakhulu kuya kolungavamile, nansi izinhlobo:

1. I-Acute Intermittent Porphyria (AIP): Lena yiyo evame kakhulu. Ukuguquka kwezakhi zofuzo ze-HMBS kubangela ukuntuleka kwe-enzyme i-hydroxymethylbilane synthase (HMBS) (eyaziwa nangokuthi i-porphobilinogen deaminase (PBGD)). Lokhu kungaba ukuguquka kwezakhi zofuzo okusha, noma kungazuzwa njengesici esiyinhloko se-autosomal (okungukuthi, lesi sifo singavela ngisho noma umzali oyedwa kuphela ozuza i-gene).

2. I-Variegate Porphyria (VP): Ukuguquka kwezakhi zofuzo ze-PPOX kubangela ukuntuleka kwe-enzyme protoporphyrinogen oxidase (PPO noma PPOX). Lokhu kungazuzwa njenge-autosomal dominant noma i-autosomal recessive.

3. I-Hereditary Coproporphyria (HCP): Ukuguquka kwezakhi zofuzo i-'CPOX' kuphumela ekuntulekeni kwe-enzyme i-'coproporphyrinogen oxidase' (CPOX). Lokhu kungazuzwa njenge-autosomal dominant noma i-autosomal recessive.

4. I-Porphyria Yokuntuleka kwe-ALAD (i-Porphyria Yokuntuleka kwe-ALAD - ADP):Ukuguquka kwezakhi zofuzo ze-'ALAD' kubangela ukuntuleka kwe-enzyme ye-'delta-aminolevulinic acid dehydratase (ALAD).` Izuzwa ngendlela ye-'autosomal recessive' (okungukuthi, lesi sifo singavela kuphela uma bobabili abazali bezuza izakhi zofuzo).

I-AHP ingithinta kanjani?

Lokhu kuyamangaza, ngoba i-AHP ayithinti wonke umuntu ngendlela efanayo. Abanye abantu bane-gene mutation yayo kodwa abakaze babe nezimpawu . Abanye bangase babe "nokuhlaselwa" kwezimpawu ezenzeka kanye noma kabili kuphela empilweni yabo. Kodwa abanye abantu bavame ukuba nalezi zihlaselo, kanti abanye bazithola zingamahlalakhona , okusho ukuthi bayaqhubeka nokuba nezimpawu ezithinta impilo yabo yansuku zonke. Ngezinye izikhathi ukuhlaselwa kungaba kubi kakhulu kangangokuthi kungadingeka uphuthunyiswe egumbini lezimo eziphuthumayo esibhedlela. Ungase ube nobuhlungu obukhulu bemizwa, izinguquko zesikhumba, kanye nezimpawu ezihlobene nohlelo lwezinzwa.

Ubani okungenzeka kakhulu ukuthi athole lokhu?

I-AHP ingathinta abantu banoma yiluphi uhlanga noma umbala. Ingazuzwa njengefa kumzali oyedwa noma bobabili abanokuguqulwa kwezakhi zofuzo. Ngokumangazayo, abantu abaningi ababi nezimpawu . Ukuze izimpawu zivele, ukuguqulwa kwezakhi zofuzo kuphela akwanele. Kumelwe kube nezinye 'izimbangela' eziningana.

Yiziphi lezi zinto ezibangela lokhu?

• Izinguquko zama-hormone (ikakhulukazi ngesikhathi sokuthomba, ukukhulelwa, kanye nomjikelezo wokuya esikhathini)
• Eminye imithi
• Ukusetshenziswa kotshwala
• Ukubhema
• Ukucindezeleka okukhulu
• Ukulawula ukudla okuqinile (ikakhulukazi ukunqunywa kwama-calories)

Kuyaphawuleka futhi ukuthi ama-80% alabo abathola izimpawu ngabesifazane abaseminyakeni yokuzala .

Ivame kangakanani i-AHP?

Kunzima ukusho ngokuqinisekile, ngoba lesi sifo sivame ukungatholakali kahle. Emhlabeni jikelele, kulinganiselwa ukuthi cishe abantu aba-5 kwabayi-100,000 bangaba naso. Kulezi zinhlobo ezishiwo ngaphambilini, i-'Acute Intermittent Porphyria (AIP)' uhlobo olubonakala kuma-80% kuphela ezimo ezibikiwe. I-'ALAD-Deficiency Porphyria (ADP)' uhlobo olungavamile kakhulu, kanti amacala ayi-9 kuphela abikiwe emhlabeni jikelele. Cabanga nje, oyedwa kuphela kwabayi-10 abantu abane-gene mutation ehlobene ne-AHP abazoba nezimpawu.

Ziyini izimpawu ze-AHP?

Izimpawu ze-AHP zixakile kakhulu. Kungaba nzima ngeziguli nodokotela ukuziqonda, ngoba izimpawu zingavela ngokuzumayo, zibe zimbi kakhulu, futhi zibonakale zingahlobene.

Nazi ezinye zalezi zici:

Ubuhlungu bemizwa

Lobuhlungu bungenzeka ezindaweni ezahlukene emzimbeni:

• Ubuhlungu obukhulu besisu - Lolu uphawu lokuqala oluvela kubantu abaningi.
• Ubuhlungu besifuba
• Ukuhlungu obusemhlane
• Ubuhlungu ezandleni nasezinyaweni

Izinkinga zesistimu yokugaya ukudla

Ngenxa yokuthi izinzwa ziyathinteka, izinkinga ngohlelo lokugaya ukudla nazo zingavela:

• Isicanucanu nokuhlanza
• Ukungagayeki kahle kokudla
• Ukuqunjelwa
• Uhudo

Izimpawu zesistimu yezinzwa ephakathi

Lokhu kuhlobene ngqo nobuchopho nengqondo:

• Ukukhathazeka
• Ukungalali
• Ukucindezeleka
• Ukuphambana kwengqondo
• Ukubona izinto ezingekho - ukubona noma ukuzwa izinto ezingekho ngempela
• Isimo sokuwa/ukuquleka (`Ukuquleka`)

Izimpawu zesistimu yezinzwa yangaphandle

Lokhu kuxhumene nemizwa kwezinye izingxenye zomzimba:

• Ubuthakathaka bemisipha
• Ukuba ndikindiki nokuthuthumela emilenzeni
• Ukukhathala
• Ukulahlekelwa yizinzwa
• Ukukhubazeka kwemisipha
• Ukukhubazeka kokuphefumula - Lokhu kuyingozi kakhulu futhi kungabangela ubunzima bokuphefumula.

Izimpawu zesistimu yezinzwa ezizenzakalelayo

Lezi yizinto ezenzeka ngokuzumayo, izinto esingenakulawula kuzo:

• Ukushaya kwenhliziyo
• Umfutho wegazi ophakeme

Izimpawu zesikhumba (ikakhulukazi ku-'Variegate Porphyria' kanye ne-'Heroditary Coproporphyria')

Abantu abanalezi zinhlobo ezimbili bangase babe nezinkinga zesikhumba uma bechayeke elangeni :

• Ukuzwela elangeni
• Ukuqhuma okuqhumayo
• Ukushintsha kombala wesikhumba (Ukushintsha kombala)
• Izibazi

Into ebaluleke kakhulu ukuthi uma une-porphyria, umchamo wakho ungashintsha umbala . Lapho lezo zinto ezandulela i-porphyrin engizishilo ziqoqana emzimbeni wakho futhi zikhishwa emchameni wakho, umchamo wakho ungaphenduka umbala obomvu-onsomi . Igama elithi "porphyria" livela egameni lesiGreki elithi "porphura," elisho "onsomi." Ama-porphyrins asemangqamuzaneni ethu abomvu egazi yiwo anika igazi umbala walo obomvu.

Kuyini ukuhlaselwa kwe-AHP?

Kubantu abaningi abanezimpawu ze-AHP, lezi ziza ngesimo "sokuhlaselwa" ngezikhathi ezithile. Abanye abantu babhekana nazo kaningi, abanye kaningi. Abanye abantu babhekana nazo kakhudlwana, kanti abanye abazizwa. Ngokuvamile, lezi zihlaselo zihlala izinsuku ezimbalwa, bese zikhula kancane kancane futhi zinciphe. Uma unezimpawu ezinkulu nezesabekayo, kufanele uye esibhedlela. Njengoba ngishilo, izinto ezifana nokukhubazeka kokuphefumula, okwenza kube nzima ukuphefumula, yizimo ezidinga ukunakekelwa okuphuthumayo kwezokwelapha.

Uphawu oluvame kakhulu lokuhlaselwa yi-AHP ubuhlungu obukhulu besisu obungachazeki.Abantu abangaphezu kuka-90% abane-AHP baya kudokotela ngenxa yalobu buhlungu besisu. Kodwa-ke, njengoba kunezimbangela eziningi zobuhlungu besisu, futhi njengoba i-X-ray noma i-CT scan ingakwazi ukuthola imbangela yalobu buhlungu (ngoba bubuhlungu bemizwa), kunzima kakhulu ukubuthola. Ngenxa yokuthi lobu buhlungu besisu buhambisana nezinto ezifana nokucanuzela kwenhliziyo, ukuhlanza, nokuqunjelwa, odokotela bangase bacabange ukuthi kuyisifo sokugaya ukudla. Uma nawe unezinkinga zengqondo nezinzwa, bangase bacabange ukuthi kuyisifo sezinzwa. Lapho lezi zimpawu ezibonakala zingahlobene zihlangana, kubalulekile ukubona udokotela owaziyo ngaso ukuze aqaphele ukuthi kungaba yi-porphyria.

Yiziphi izimpawu ezingamahlalakhona ezingabangelwa yi-AHP?

Abantu abanesifo esincane (ukuntuleka kwama-enzyme amaningi) abavamile ukuba nezimpawu ezingamahlalakhona. Bangase babe nokuhlaselwa okukodwa noma okubili kuphela empilweni yabo. Uma bebona izimbangela zazo, bangazigwema. Kodwa-ke, abanye abantu bahlaselwa njalo, kanti ezinye izimpawu zivame kakhulu kangangokuthi zingabhekwa njengezingamahlalakhona. Izimpawu ezinjalo zifaka:

• Ubuhlungu
• Ukukhathala
• Isicanucanu
• I-neuropathy ( ukungabi namandla, ubuhlungu, noma ubuthakathaka emalungeni)

Yiziphi izinkinga ze-AHP?

Izimpawu ezihlobene nohlelo lwezinzwa oluphakathi (njengokukhathazeka, ukubona izinto ezingekho, kanye nokuxhuzula) zivame ukwenzeka kuphela ngesikhathi sokuhlaselwa. Kodwa-ke, izimpawu ezihlobene nohlelo lwezinzwa olungaphandle (njengobuthakathaka bemisipha kanye nokukhubazeka) zingavela njalo, futhi umonakalo ohlala njalo ungenzeka ngemva kokuhlaselwa okunzima. Ukuqongelela kwalezi zinto ezandulela i-porphyrin nakho kungabangela umonakalo wesikhathi eside ezithweni ezithile zomzimba. Lokhu kungafaka:

• Umfutho wegazi ophakeme ongapheli
• Isifo sesibindi esingamahlalakhona
• Isifo sezinso esingamahlalakhona
• Umdlavuza wesibindi oyinhloko
• Ukucindezeleka nokukhathazeka

Yini ebangela i-AHP?

Imbangela eyinhloko ye-AHP ukuguquka kwezakhi zofuzo . Kodwa njengoba ngishilo ngaphambili, izimpawu azibonakali ngokushesha ngemva kokuba ukuguquka kwezakhi zofuzo sekukhona. Ezimweni eziningi, izimpawu ziqala ukubonakala ngesikhathi sokuthomba, kanye nokushintsha kwama-hormone. Ngaphezu kwalokho, imithi ethile, izidakamizwa, ukuphuza utshwala ngokweqile, ukuncishiswa kwama-calories amaningi, kanye nezifo ezibeka ukucindezeleka okukhulu emzimbeni nakho kungayibangela.

Konke lokhu okufana ngakho ukuthi kuvuselela inqubo yokukhiqizwa kwe-heme yesibindi. Kodwa-ke, esibindini somuntu one-AHP, imikhiqizo ephuma ekukhiqizweni kwe-heme (njengezinto ezandulela i-porphyrin) ayigaywa kahle, okungukuthi, isetshenziswe yonke. Ngakho-ke, lezi zinto ezandulela i-porphyrin ezisele ziqoqana esibindini. Kulapho kuphela lapho lezi ziqoqana zifinyelela ezingeni elithile lapho ziqala khona ukuthinta isimiso sezinzwa futhi zibangele izimpawu.

Itholakala kanjani i-AHP? (Ukuxilongwa)

Kulabo abane-AHPUkuthola ukuxilongwa okufanele kungaba yinselele, njengoba izimpawu azifani nalesi simo futhi zingase zibonakale zingahlobene. Kodwa-ke, udokotela ojwayelene ne-AHP angase asole i-AHP uma unobuhlungu besisu kanye nezimpawu zesistimu yezinzwa ephakathi kanye neyangaphandle. Ukuhlanganiswa kwalezi zimpawu ezintathu kubhekwa njenge-"classic triad" ye-AHP.

Uma udokotela esola i-AHP, angenza izivivinyo eziningana ukuze aqinisekise lokho.

• Ukuhlolwa komchamo: Lokhu kungenziwa ngokushesha ngesikhathi sokuhlaselwa. Umchamo ungase ube ojwayelekile uma ungahlaselwa, kodwa ngesikhathi sokuhlaselwa, umchamo uzobonisa amazinga aphezulu e-porphyrin precursors (PBG kanye ne-ALA). Lokhu akulona ukuhlolwa okwenziwa kuphela kwe-AHP, kodwa kuwukuhlolwa okuyisisekelo okungasiza odokotela ukukukhomba endleleni efanele.
• Ukuhlolwa kwezakhi zofuzo: Lena yindlela engcono kakhulu necacile yokuxilonga i-AHP ('izinga legolide') . Kungakhathaliseki ukuthi unezimpawu noma cha, kungaqinisekisa ukuthi une-gene mutation ehlotshaniswa ne-AHP. Kungakutshela nokuthi yiluphi uhlobo lwe-AHP onalo nokuthi ukuntuleka kwe-enzyme kukhulu kangakanani. Lokhu kuhilela ukuthatha isampula yegazi noma yamathe. Isibhedlela sakho sendawo singadinga futhi ukuthumela isampula elabhorethri ekhethekile.

Iphathwa kanjani i-AHP?

Ukwelashwa kwe-AHP kugxile ekuvimbeleni nasekulawuleni ukuhlaselwa . Kungadingeka ukuthi ulaliswe esibhedlela ngesikhathi sokuhlaselwa. Kungadingeka ukuthi udinge izinhlobo ezahlukene zemithi yokunciphisa izimpawu zakho. Phakathi nezikhathi lapho ungenazo izihlaselo, ungadinga eminye imithi yokulawula izimbangela ezenza izimpawu zakho zibe zimbi kakhulu. Uma wena nodokotela wakho nisebenzisana ukuthola izimbangela ezikuthinta kakhulu, ukwelashwa kwakho kuzosebenza kangcono.

Ukwelashwa kokuhlaselwa okunzima:

• Umjovo we-Hemin: Uma kwenzeka ukuhlaselwa okukhulu, udokotela angakunika umjovo we-hemin emthanjeni. Lokhu kusiza ukunciphisa inani le-porphyrin egazini lakho. I-Hemin yinto ethathwa kumaseli abomvu egazi futhi yehlisa inani le-porphyrin emzimbeni wakho.
• Ukuqeda ubuhlungu: Ekuhlaselweni okukhulu, kungase kudingeke imithi enamandla yokudambisa izinhlungu njenge-opioid.
• Ama-Phenothiazines: Lawa asetshenziselwa ukulawula isicanucanu esinzima nokuhlanza.
• Uketshezi oluphuma emzimbeni kanye nokudla okunempilo: Izimpawu ezifana nobuhlungu besisu, isicanucanu, ukuhlanza, isifo sohudo, kanye nokuqunjelwa kungabangela ukuthi umzimba ulahlekelwe amakhalori namanzi ngesikhathi sokuhlaselwa. I-AHP ingabangela nokulahlekelwa izinto ezifana ne-sodium ne-magnesium. Ngakho-ke, uketshezi oluqukethe ama-carbohydrate nama-electrolyte lunganikezwa ngemithambo yegazi.
• Imithi yokuquleka:Cishe ama-20% eziguli angadinga ukwelashwa kwesifo sokuwa ngesikhathi sokuhlaselwa.

Izinketho zokwelapha zesikhathi eside:

• I-hemin yokuvimbela: Ukunikeza imithamo ephansi ye-hemin kanye ngesonto kubantu abanokuhlaselwa njalo kungavimbela ukunqwabelana kwama-porphyrins.
• I-Givosiran (GIVLAARI®): Lolu uhlobo lokwelashwa kwezakhi zofuzo . Lunciphisa ukukhiqizwa kwezakhi zofuzo ze-porphyrin. Manje seluvunyelwe njengomjovo wanyanga zonke ukuvimbela izimpawu ze-AHP kubantu abanokuhlaselwa njalo.
• Ukwelashwa ngama-hormone: Uma ukuhlaselwa kwakho yi-AHP kubangelwa umjikelezo wakho wokuya esikhathini, udokotela wakho angase akunikeze imithi efana ne-GnRH (i-Gonadotropin-releasing hormone) analogues. Lokhu kunciphisa ukukhiqizwa kwama-hormone i-estrogen ne-progesterone emzimbeni.
• Imithi yokucindezeleka kwegazi okuphezulu: Uma umfutho wegazi ophezulu ongapheli uvela, kuzodingeka ulawulwe ngemithi ethile.
• Ukufakelwa isibindi: Abantu abahlaselwa njalo futhi abasongela ukuphila futhi abangasabelanga kwezinye izindlela zokwelapha bangase bacabangele ukufakelwa isibindi. Lokhu kungasiqeda lesi sifo ngokuphelele.

Ingabe i-AHP ingavinjelwa?

Akunakwenzeka ukuvimbela lesi sifo ukuthi singenzeki. Kodwa-ke, ungasiza ekuvimbeleni izimpawu ukuthi zingenzeki ngokugwema izimbangela . Uma ungakaze uhlaselwe, kungcono ukugwema zonke izimbangela ezingaba khona. Uma uke wahlaselwa futhi wazi ukuthi yiziphi izimbangela ezibangele lokhu, kungase kwanele ukugwema lezo zimbangela ezithile kuphela.

Nazi ezinye zezinto ezivame ukubonakala:

Izinhlobo zemithi:

• Ama-antihistamine (eminye imithi yomkhuhlane kanye nokungezwani komzimba)
• Imithi yokudambisa ubuhlungu
• Izindlela zokuvimbela inzalo ngomlomo
• Ukwelashwa kokufaka esikhundleni sama-hormone

Ukusetshenziswa kwezinto:

• Ugwayi (`Ugwayi`)
• Insangu
• Utshwala
• Izidakamizwa zokuzijabulisa

Ukucindezeleka:

• Izifo
• Ukuhlinzwa
• Ukudla okunama-calorie aphansi (ikakhulukazi ukudla okunama-carbohydrate aphansi) ('Ukuntuleka kwama-calorie')
• Ukucindezeleka kwengqondo

Liyini ikusasa labantu abane-AHP? (Isibikezelo)

Lokhu kuyahlukahluka kakhulu kumuntu nomuntu. Abantu abaningi abakaze babe nezimpawu . Kulabo abanazo, iningi labantu linokuhlaselwa okukodwa noma okumbalwa empilweni yabo. Nakuba ukuhlaselwa kungaba yingozi empilweni, iningi labantu liyalulama ngokugcwele ngokwelashwa okusheshayo . Cishe u-5% wabantu abane-AHP banezimpawu ezivamile noma ezingamahlalakhona. Kulaba bantu, imithi yokuvimbela ivame ukuba usizo, futhi ukufakelwa isibindi kungabhekwa njengesinyathelo sokugcina.

Ngizinakekela kanjani ngenkathi ngiphila ne-AHP?

• Gwema izinto ezivame ukubangela ukucindezeleka. Utshwala, ukubhema, nezidakamizwa kufanele kube phezulu ohlwini lwakho lwezinto okufanele uzigweme. Ungase ufune nokukhuluma nodokotela wakho ngezinye izindlela zokusebenzisa imithi ethile esikhundleni seminye oyiphuzayo.
• Yidla ukudla okunempilo nokulinganiselayo. Gwema ukudla ngokweqile kanye nezinhlelo zokudla okunama-carbohydrate aphansi. Uma uzila ukudla ngaphambi kokuhlolwa noma ukuhlinzwa, khuluma nodokotela wakho ngakho.
• Thola ukuhlolwa njalo kwezokwelapha. Ngisho noma ungenazo izimpawu, udokotela wakho uzohlola njalo isibindi sakho, izinso kanye nomfutho wegazi.

I-Acute hepatic porphyria iyisifo esingavamile, ngakho-ke ulwazi emphakathini jikelele luphansi ngaso. Uma ukuhlaselwa kuvela ngokuzumayo ngezimpawu ezinzima, kuyadida kakhulu futhi kuyesabisa. Abanye abantu bachitha iminyaka bezama ukuthola ukuthi yini engalungile ngabo nokuthi yini abangayenza. Uma usola ukuthi une-AHP, ukuhlolwa kofuzo kungakuqinisekisa lokho. Uma usukwazi, kuzoba usizo olukhulu kuwe ukuvimbela ukuhlaselwa nokuzilawula.

Izinto ezibaluleke kakhulu okudingeka uzazi (Umyalezo Wokuya Ekhaya)

Kulungile, ake ngifingqe ezinye zezinto okudingeka uzikhumbule kulokho esikhulume ngakho:

• I-AHP yisifo esingavamile esibangelwa izakhi zofuzo esiqala esibindini futhi sithinte isimiso sezinzwa .
• Lokhu kungenxa yokuntuleka kwama-enzyme adingekayo enkambisweni yokwenza i-heme , okubangela ukuba izinto ezinobuthi ezibizwa ngokuthi ama-porphyrin precursors ziqongelelane emzimbeni.
• Izimpawu zingahlukahluka, kusukela ebuhlungwini besisu obukhulu, ubuhlungu bemizwa, izinkinga zengqondo, izinkinga zesikhumba, ngisho nokushintsha kombala womchamo wakho.
• Ngisho noma kukhona ukuguquka kwezakhi zofuzo, izimpawu azivamile ukuvela ngaphandle kwezimbangela ( njengama-hormone, imithi ethile, utshwala, kanye nokucindezeleka).
• Ukuhlolwa komchamo kanye nokuhlolwa kofuzo kubalulekile ekuxilongweni.
• Into eyinhloko ukulawula nokuvimbela ukuhlaselwa ngokwelashwa. Kusetshenziswa umuthi wokugomela i-`Hemin`, imithi yokubulala izinhlungu, kanye neminye imithi ekhethekile. Ezimweni ezimbi kakhulu, ukufakelwa kwesibindi nakho kuyisisombululo.
• Ungaphila kahle nalesi sifo ngokugwema izimbangela, ukuphila impilo enempilo, nokuhlolwa njalo kwezokwelapha .

Ngiyethemba ukuthi lolu lwazi luzokusiza. Khumbula, uma unezinye zalezi zimpawu, ungesabi ukubona udokotela bese ukhuluma ngakho.

I - porphyria yesibindi esibukhali, i-AHP, isibindi, isimiso sezinzwa, izifo zofuzo, ama-porphyrins, izimpawu, ukwelashwa