Cabanga uzwa ubuhlungu besisu obungazelelwe, obubuhlungu ngaphandle kwesizathu esibonakalayo, obuhambisana nokuqaqamba noma ukungazweli ezithweni zakho, ubuthakathaka bemisipha, kanye nokucasuka. Ngisho nangemva kokubona odokotela abaningana nokwenza izivivinyo eziningi, imbangela eyinhloko isalokhu ingatholakali. Ingabe wena noma othile omaziyo uke wabhekana nalokhu? Kungaba yisimo esingavamile sofuzo esivame ukunganakwa. Namuhla, sihlola lesi simo: I-Acute Hepatic Porphyria , noma i-AHP.
Iyini ngempela i-Acute Hepatic Porphyria (AHP)?
I-AHP ibhekisela emndenini wezifo zofuzo ezingavamile, ezizuzwe njengefa ezithinta isimiso sakho sezinzwa futhi ngezinye izikhathi isikhumba sakho. Akuvamile kakhulu, kuthinta cishe abantu abahlanu kubantu abayi-100,000. Lesi simo singabangela izinkinga zempilo ezingazelelwe, ezinzima, futhi ezingase zisongele impilo.
Ukuze siqonde ukuthi kungani, ake sibheke igazi lakho. Amaseli ethu abomvu egazi aqukethe iphrotheni ebizwa ngokuthi i-Hemoglobin . Umsebenzi wawo oyinhloko ukucosha umoya-mpilo emaphashini akho bese uwuletha ezithweni nasezicutshini zakho—njengenkonzo yokulethwa komoya-mpilo ezinikele.
Ukuze udale le phrotheni, umzimba wakho udinga isakhi esibalulekile esibizwa ngokuthi i-Heme .
Kubantu abane-AHP, i-enzyme ethile edingekayo ekukhiqizweni kwe-Heme iyantuleka noma ayikho. Cabanga ngakho njengokuzama ukubhaka iresiphi kodwa ulahlekelwe isithako esiyinhloko; umkhiqizo wokugcina awukwazi ukwenziwa ngendlela efanele.
Lokhu kuntuleka kwama-enzyme kwenzeka kakhulu esibindini , okubizwa nangokuthi uhlelo lwesibindi . Yingakho lesi simo sibizwa ngokuthi i-Acute Hepatic Porphyria .
Uma umzimba wakho ungakwazi ukukhiqiza i-Heme, izakhi zamakhemikhali—ikakhulukazi izakhi ezinobuthi ezifana ne-Porphobilinogen (PBG) kanye ne-Aminolevulinic acid (ALA)—ziqala ukuqongelela esibindini. Lezi zinobuthi zingena egazini futhi zingahamba emzimbeni wonke, ikakhulukazi zilimaze uhlelo lwakho lwezinzwa. Lokhu kulimala kwezinzwa yikona okuholela ezimpawini ezifana nobuhlungu obukhulu , ukungazweli , kanye nokucasuka .
Yiziphi Izinhlobo Eziyinhloko ze-AHP?
Kunezinhlobo ezine eziyinhloko ze-AHP. Nakuba ngayinye ihilela ukuntuleka kwe-enzyme ehlukile enqubweni yokukhiqizwa kwe-Heme, zonke ezine zithinta kakhulu isibindi kanye nesistimu yezinzwa.
I-Acute Intermittent Porphyria (AIP)
Lolu uhlobo oluvame kakhulu, oluthinta ama-80% eziguli ze-AHP. Lubangelwa ukuntuleka kwe-enzyme ye-Hydroxymethylbilane synthase (HMBS), okubangelwa ukuguqulwa kwezakhi zofuzo ze-HMBS. Lokhu kungenziwa njengefa kumzali, noma kungenzeka njengoshintsho olungavamile lwezakhi zofuzo ngaphandle komlando womndeni wangaphambilini.
I-AHP Izizwa Kanjani?
Izimpawu ze-AHP ziyahlukahluka kakhulu. Abanye abantu bangase bangabi nazo izimpawu, kanti abanye bangase babe nokuhlaselwa okunzima njalo. Ukuqaphela lezi zimpawu kubalulekile.
| Uhlelo Lomzimba Oluthintekile | Izimpawu Ezivamile |
|---|---|
| Isisu | Ubuhlungu besisu obukhulu, obungachazeki ( uphawu oluyinhloko ), isicanucanu, ukuhlanza, kanye nokuqunjelwa. |
| Uhlelo Lwezinzwa | Ukuba ndikindiki noma ukuqaqamba kwamalungu, ubuthakathaka bemisipha, ubuhlungu bemisipha, kanye nokukhubazeka okuvamile. |
| Impilo Yengqondo | Ukungahlaliseki, ukukhathazeka, ukudideka, kanye nokubona izinto ezingekho. |
| Inhliziyo Nomfutho Wegazi | Inhliziyo ishaya ngamandla (i-tachycardia) kanye nomfutho wegazi ophakeme. |
| Okunye | Umchamo omnyama, obomvu, noma onsundu (ikakhulukazi ngesikhathi sokuhlaselwa). |
Ngenxa yokuthi i-AHP ayivamile, izimpawu zayo zivame ukuphambaniswa nezimo ezivame kakhulu, ezingabambezela ukuxilongwa.
Yini Ebangela Ukuhlaselwa?
Abantu abaningi abane-AHP baphila impilo evamile, kodwa ezinye izinto ezibangela lokhu zingabangela ukuhlaselwa okungazelelwe. Kubalulekile ukuqaphela lokhu:
- Imithi Ethile: Ikakhulukazi ama-barbiturates nama -antibiotic e-sulfonamide. Uma une-AHP, hlala uthintana nodokotela wakho ngaphambi kokuqala noma yimuphi umuthi omusha.
- Utshwala: Imbangela enkulu yokuhlaselwa yi-AHP.
- Ukunqunyelwa/Ukuzila Ukudla Okune-Calorie: Ukudla okunama-calories nama-carbohydrate aphansi kakhulu kungabangela izimpawu.
- Izinguquko zama-hormone: Ikakhulukazi lezo ezihambisana nomjikelezo wokuya esikhathini kwabesifazane.
- Ukucindezeleka kanye Nezifo: Ukucindezeleka ngokomzimba, umkhuhlane, noma izifo ezivamile njengomkhuhlane kungabangela isiqephu.
Kufanele Ubone Udokotela Nini?
Uma uhlangabezana nalokhu okulandelayo, ungazilahli. Funa iseluleko sezokwelapha uma:
- Unezinhlungu zesisu ezingapheli ezingachazeki.
- Uba nezimpawu ezihlobene nemizwa njengokuthuthumela noma ubuthakathaka bemilenze.
- Unomlando womndeni owaziwayo we-Porphyria.
- Uyabona ukuthi umchamo wakho usuphenduke umbala obomvu noma onsundu.
Uma ubhekene nesimo esiphuthumayo, njengobuhlungu obungabekezeleleki, ubunzima bokuphefumula, ukudideka okungazelelwe, noma ukungakwazi ukuhambisa izitho zakho zomzimba, funa ukunakekelwa okusheshayo emnyangweni wezimo eziphuthumayo oseduze nawe noma ushayele u-911.
Ingabe Kukhona Ukwelashwa Kwe-AHP?
Nakuba kungekho ikhambi lalesi simo sofuzo, kunezindlela zokwelapha eziphumelela kakhulu ezitholakalayo zokulawula nokuvimbela ukuhlaselwa.
Izigameko ezinzima zidinga ukulaliswa esibhedlela ukuze kuqiniswe isimo.
- Indlela eyinhloko ukuthola nokususa isibangeli.
- Ukuphathwa kobuhlungu okunamandla kusetshenziselwa ukulawula ukungakhululeki okukhulu.
- Imithi inqunyelwe ukwelapha isicanucanu nokuhlanza.
- Ukuphathwa kwe-glucose nge-intravenous kungasiza ekuvimbeleni ukukhiqizwa kwezinto ezinobuthi esibindini.
- Ukwelashwa Okuqondile: Ukufakwa kwe-Hemin kusebenza njengokufaka esikhundleni okuqondile, okubonisa isibindi ukuthi siyeke ukukhiqiza ngokweqile izandulela ze-Heme eziyingozi.
- Ngaphezu kwalokho, kulabo ababhekana nokuhlaselwa njalo, izindlela zokwelapha ezithuthukisiwe zokuthulisa izakhi zofuzo seziyatholakala.
Udokotela wakho uzonquma uhlelo lokwelashwa olufanelana nezidingo zakho ezithile.
Umlayezo Wokuya Nawe Ekhaya
- I-Acute Hepatic Porphyria (AHP) iyisifo esingavamile sofuzo, kodwa ngokuphathwa, abantu abaningi baphila impilo eyanelisayo nejwayelekile.
- Uphawu oluphawulekayo ubuhlungu obukhulu besisu obuphindaphindayo obuhambisana nezinkinga zezinzwa (ukuba ndikindiki, ubuthakathaka).
- Ukugwema izinto ezibangela lokhu—njengemithi ethile, utshwala, nokuzila ukudla isikhathi eside—kubalulekile.
- Uma usola ukuthi unezimpawu, noma unomlando womndeni, thintana nodokotela wakho ngokushesha.
- Kukhona izindlela zokwelapha ezisebenzayo zokulawula izimpawu nokuvimbela ukuhlaselwa esikhathini esizayo. Asikho isidingo sokwesaba; sebenzisana eduze nomhlinzeki wakho wezempilo.
I-AHP, i-Acute Hepatic Porphyria, i-Porphyria, ubuhlungu besisu, izinkinga zezinzwa, izifo zofuzo, i-heme, i-hemoglobin, i-porphyrin, izifo ezingavamile
