Wake waba nobuhlungu obukhulu besisu obungachazeki, isicanucanu, kanye nomchamo omnyama? Ngezinye izikhathi imbangela yalokhu ingaba yisimo esingavamile esingakaze sizwakale kithi. Njengoba singavamile, abantu abaningi abazi ngaso. Namuhla sikhuluma ngesifo esisodwa esinjalo, i-Acute Hepatic Porphyria (AHP). Ungakhathazeki, sizokhuluma ngalokhu kalula, ngendlela wonke umuntu angayiqonda.
Kalula nje, iyini i-Acute Hepatic Porphyria (AHP)?
I-Acute Hepatic Porphyria, noma i-AHP ngamafuphi, yisimo esibangelwa ukunqwabelana ngokweqile kwama-porphyrins, uhlobo lwamakhemikhali akhiqizwa emzimbeni wethu, esibindini nakwezinye izingxenye zomzimba. Lesi akusona isifo esisodwa ngempela, kodwa yiqembu lezifo. Lapho ama-porphyrins eqoqana ngale ndlela, angathinta isimiso sezinzwa futhi abangele ezinye izimpawu eziningi ezingakhululekile.
Nakuba kungekho ikhambi lalesi sifo okwamanje, ukwelashwa okufanele kungasiza ekulawuleni izimpawu futhi kukusize uphile impilo engcono.
Kungani lesi sifo sivela? Ake sibheke kabanzi.
Ukuze sikuqonde kahle lokhu, sidinga ukwazi okokuqala ngento ebaluleke kakhulu emizimbeni yethu. Lokho kubizwa ngokuthi ' Heme ' .
I-Heme iyingxenye ebalulekile yeprotheni i-hemoglobin emaseli ethu abomvu egazi. Njengoba wazi, i-hemoglobin yiyo ethatha umoya-mpilo emaphashini bese iwusabalalisa emzimbeni wonke. Ngakho-ke le heme ibalulekile ekusebenzeni kwezindawo ezifana nesibindi, imisipha kanye nomnkantsha wamathambo.
Manje bheka, lapho umzimba wethu wenza le 'heme', izinhlobo ezingu-8 zama-enzyme zisiza ekwenzeni leyo nqubo yenzeke kahle. Kufana nezinhlobo ezingu-8 zezinongo ezidingekayo ukwenza isidlo. Uma ngesizathu esithile kukhona ukuntuleka noma ubuthakathaka kunoma iyiphi yalezi enzyme ezingu-8, inqubo yokwenza 'heme' iyama phakathi. Okwenzekayo-ke ukuthi 'ama-porphyrins' esikhulume ngawo ngaphambili, okuyizithako ezisetshenziselwa ukwenza 'heme', aqala ukuqongelela emzimbeni .
Ku-AHP, leli khemikhali elibizwa ngokuthi ' i-porphyrin ' liqongelela kakhulu esibindini . Imiphumela yalokhu kuqongelela yiyo ebonakala njengezimpawu.
| Izimpawu ezivame ukubonakala | Okunye okwengeziwe ngalokhu |
|---|---|
| Ubuhlungu obukhulu besisu | Lolu uphawu oluyinhloko nolunzima kakhulu. Ungase ube nobuhlungu besisu obungachazeki ngaphandle kwesizathu esicacile. |
| Isicanucanu nokuhlanza | Lesi simo singase senzeke ngobuhlungu obukhulu esiswini. |
| Umchamo omnyama | Umchamo ungaba nsundu noma ube bomvu ngombala. |
| Ukuzwela kwesikhumba | Kwezinye izinhlobo ze-porphyria, isikhumba siba nomuzwa omkhulu elangeni. |
| Izinkinga zesistimu yezinzwa | Izimo ezinzima ezifana nokungazweli, ubuhlungu, ubuthakathaka bemisipha, kanye nokuxhuzula kungase kube khona. |
"Ukuhlaselwa" kwe-AHP kuvame ukuqala ngobuhlungu obukhulu besisu. Lokhu kungahlala izinsuku eziningi. Ezimweni ezinjalo , ukulaliswa esibhedlela kuyimpoqo . Lokhu kusho ukuya eMnyangweni Wezimo Eziphuthumayo (ETU).
Ubani othola lesi sifo? Ubani osengozini?
Ngokuvamile, lesi sifo siwufuzo . Okusho ukuthi, uthola izakhi zofuzo ezibangela lesi sifo kubazali bakho.
Kodwa into ebalulekile lapha ukuthi akuwona wonke umuntu one-gene ebangela lesi sifo ozoba nezimpawu . Iningi labantu abane-gene ye-AHP lingahlala liphilile impilo yalo yonke ngaphandle kwezimpawu.
Lokhu kufana nebhubesi elilele. Ngisho noma i-gene ikhona, uma kungekho nto yokuyivusa, noma "ukuyivusa", ingahlala ngaphandle kwezinkinga.
Ngakho-ke yiziphi izimbangela zalesi sifo?
Lena yingxenye ebaluleke kakhulu. Uma wena noma othile omaziyo ene-AHP, kubalulekile ukuqaphela lezi zinto.
| Isiqalisi | Amaphuzu abalulekile |
|---|---|
| Eminye imithi | Eminye imithi yokunciphisa ubuhlungu, imithi yokudambisa ubuhlungu, imithi yokunciphisa ubuhlungu, imithi yokunciphisa ubuhlungu, kanye namaphilisi okulawula ukubeletha kungaba yizimbangela ezinkulu. Uma usola noma utholakale une-AHP, ungathathi noma yimuphi umuthi ngaphandle kokubonisana nodokotela. |
| Ukuphuza utshwala nokubhema | Lokhu kungabangela kalula lesi sifo ngoba kubeka ingcindezi enkulu esibindini. |
| Ukudla okuqinile noma ukuzila ukudla | Ukunciphisa ukudla noma ukweqa ukudla ngenxa yokuzama ukwehlisa isisindo ngokuzumayo kuyingozi kakhulu kulezi ziguli. Ukungatholi amakhalori adingwa ngumzimba kuyimbangela enamandla. |
| Ukucindezeleka kwengqondo nokwenyama | Izinto ezifana nokucindezeleka okukhulu, ukuhlinzwa, kanye nezifo nazo zingadala lesi sifo. |
Kumele ngenzeni?
Uma uhlangabezana nanoma yiziphi izimpawu ezishiwo kulesi sihloko, ikakhulukazi ubuhlungu besisu obukhulu, obungachazeki, njalo , sicela ungacabangi ukuthi kuyinto evamile. Bheka udokotela oqeqeshiwe ngokushesha.
Udokotela uzokuxilonga, futhi uma kudingeka, acele ukuhlolwa komchamo, igazi, kanye nofuzo ukuze kutholakale ukuthi unalesi sifo.
Ukudla okunomsoco kubaluleke kakhulu kumuntu ophila ne-AHP. Uma kudingeka, udokotela wakho angakuthumela kuchwepheshe wezokudla.
Umlayezo Wokuya Nawe Ekhaya
- I-Acute Hepatic Porphyria (AHP) yisifo esingavamile, esizuzwe njengefa esibangelwa ukunqwabelana kwekhemikhali ebizwa ngokuthi i-porphyrin esibindini.
- Imbangela eyinhloko yalokhu ukuntuleka kwe-enzyme edingekayo enkambisweni yokwenza 'i-heme'.
- Uphawu oluyinhloko ubuhlungu besisu obungabekezeleleki. Bungabangela nezinkinga zesimiso sezinzwa.
- Ngisho noma unezakhi zofuzo ezibangela lesi sifo, uma ugwema izimbangela ezibangela lesi sifo, ungase ungabi nazo izimpawu.
- Eminye imithi, utshwala, ukubhema, ukudla okuqinile, kanye nokucindezeleka kuyizimbangela ezinkulu.
- Uma unezinye zalezi zimpawu noma usola ukuthi une-AHP, qiniseka ukuthi ukhuluma nodokotela. Ungalokothi uphume uyothenga imithi wedwa.
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