Ngaba wakha waba nentlungu yesisu engachazekiyo, isicaphucaphu, kunye nomchamo omnyama? Ngamanye amaxesha unobangela woku unokuba yimeko engaqhelekanga ekungekho namnye kuthi owakha wayiva. Ekubeni ingaqhelekanga, abantu abaninzi abazi ngayo. Namhlanje sithetha ngesifo esinye esinjalo, i-Acute Hepatic Porphyria (AHP). Ungakhathazeki, siza kuthetha ngale nto ngokulula, ngendlela wonke umntu anokuyiqonda.
Ngamafutshane, yintoni i-Acute Hepatic Porphyria (AHP)?
I-Acute Hepatic Porphyria, okanye i-AHP ngamafutshane, yimeko ebangelwa kukuqokelelwa kakhulu kwee-porphyrins, uhlobo lweekhemikhali eziveliswa emzimbeni wethu, esibindini nakwezinye iindawo zomzimba. Esi asisosifo sinye, kodwa liqela lezifo. Xa ii-porphyrins ziqokelelana ngale ndlela, zinokuchaphazela inkqubo yemithambo-luvo kwaye zibangele ezinye iimpawu ezingathandekiyo.
Nangona kungekho nyango lale sifo okwangoku, unyango olufanelekileyo lunokunceda ukulawula iimpawu kwaye lukuncede uphile ubomi obungcono.
Kutheni esi sifo sisenzeka? Makhe sijonge ngokusondeleyo.
Ukuze sikuqonde kakuhle oku, kufuneka siqale sazi ngento ebaluleke kakhulu emizimbeni yethu. Loo nto ibizwa ngokuba yi - 'Heme ' .
I-Heme yinxalenye ebalulekileyo yeproteni ye -hemoglobin kwiiseli zethu ezibomvu zegazi. Njengoko usazi, i-hemoglobin yiyo ethatha ioksijini emiphungeni ize iyisasaze emzimbeni wonke. Ngoko ke le heme ibalulekile ekusebenzeni kweendawo ezifana nesibindi, izihlunu kunye nomongo wamathambo.
Ngoku khangela, xa umzimba wethu usenza le 'heme', iintlobo ezisi-8 zee-enzymes zinceda ekwenzeni loo nkqubo yenzeke ngokufanelekileyo. Kufana neentlobo ezisi-8 zeziqholo ezifunekayo ukwenza isitya. Ukuba ngesizathu esithile kukho ukusilela okanye ubuthathaka kwenye yezi enzymes zisi-8, inkqubo yokwenza 'heme' iyema phakathi. Okwenzekayo ke kukuba 'ii-porphyrins' esithethe ngazo ngaphambili, ezizizithako ezisetyenziselwa ukwenza 'heme', ziqala ukuqokelelana emzimbeni .
Kwi-AHP, le khemikhali ebizwa ngokuba yi- 'porphyrin ' iqokelelana kakhulu esibindini . Iziphumo zokuqokelelana zizibonakalisa njengeempawu.
| Iimpawu eziqhelekileyo | Okungakumbi malunga noku |
|---|---|
| Intlungu ebuhlungu yesisu | Olu lolona phawu luphambili nolunzima kakhulu. Usenokuba nentlungu yesisu engachazekiyo ngaphandle kwesizathu esicacileyo. |
| Isicaphucaphu nokuhlanza | Le meko inokwenzeka xa kukho intlungu enzulu esiswini. |
| Umchamo omnyama | Umchamo unokuba mdaka okanye ube bomvu ngombala. |
| Uvakalelo lolusu | Kwezinye iintlobo ze-porphyria, ulusu luba novelwano kakhulu kwilanga. |
| Iingxaki zenkqubo yemithambo-luvo | Iimeko ezinzima ezifana nokungaziva mnandi, iintlungu, ubuthathaka bemisipha, kunye nokuxhuzula zinokwenzeka. |
"Uhlaselo" lwe-AHP ludla ngokuqala ngentlungu eqatha yesisu. Oku kungathatha iintsuku. Kwiimeko ezinjalo , ukulaliswa esibhedlele kuyimfuneko . Oku kuthetha ukuya kwiSebe leNgxamiseko (ETU).
Ngubani ofumana esi sifo? Ngubani osemngciphekweni?
Esi sifo sidla ngokuba sisifo esidluliselwa ngemfuza . Oko kukuthi, ufumana ii-genes ezibangela esi sifo kubazali bakho.
Kodwa into ebalulekileyo apha kukuba ayinguye wonke umntu one-gene ebangela esi sifo oza kuba neempawu . Uninzi lwabantu abane-gene ye-AHP banokuhlala besempilweni ubomi babo bonke ngaphandle kweempawu.
Oku kufana nengonyama eleleyo. Nokuba i-gene ikhona, ukuba akukho nto inokubangela ukuba ivuke, okanye "ivuse", ingahlala ingenangxaki.
Ngoko ke zithini izizathu zesi sifo?
Le yinxalenye ebaluleke kakhulu. Ukuba wena okanye umntu omaziyo une-AHP, kubalulekile ukuba uzazi ezi zinto.
| Isixhobo sokubangela ingxaki | Amanqaku abalulekileyo |
|---|---|
| Amanye amayeza | Ezinye iipilisi zokuthomalalisa iintlungu, izithomalalisi, ii-barbiturates, izithomalalisi, kunye neepilisi zokulawula ukuzalwa zinokuba zezona zinto ziphambili ezibangela oku. Ukuba ukrokrela okanye ufunyenwe une-AHP, musa ukuthatha naluphi na unyango ngaphandle kokubonisana nogqirha. |
| Ukusela utywala nokutshaya | Ezi zinokubangela esi sifo kuba zibeka uxinzelelo olukhulu kwisibindi. |
| Ukuzila ukutya okungqongqo okanye ukuzila ukutya | Ukunciphisa ukutya okanye ukungatyi ukutya ngenxa yokuzama ukunciphisa umzimba ngequbuliso kuyingozi kakhulu kwaba baguli. Ukungafumani iikhalori ezifunwa ngumzimba kuyingozi enkulu. |
| Uxinzelelo lwengqondo nolomzimba | Izinto ezifana noxinzelelo olukhulu, utyando, kunye nosulelo nazo zinokubangela esi sifo. |
Ndingenza ntoni?
Ukuba ufumana naziphi na iimpawu ezikhankanyiweyo kweli nqaku, ingakumbi iintlungu zesisu ezinzima nezingenakuchazwa, rhoqo , nceda ungacingi ukuba yinto eqhelekileyo. Bona ugqirha ofanelekileyo ngoko nangoko.
Ugqirha uza kukuxilonga aze, ukuba kuyimfuneko, acele uvavanyo lomchamo, igazi, kunye nolwemfuza ukuze kuqinisekiswe ukuba unesifo na.
Ukutya okunesondlo kubaluleke kakhulu kumntu ophila ne-AHP. Ukuba kuyimfuneko, ugqirha wakho angakuthumela kwingcali yesondlo.
Umyalezo Wokuya Ekhaya
- I-Acute Hepatic Porphyria (AHP) sisifo esingaqhelekanga, esizuzwe njengelifa esibangelwa kukuqokelelana kwekhemikhali ebizwa ngokuba yi-porphyrin esibindini.
- Isizathu esiphambili soku kukungabikho kwe-enzyme efunekayo kwinkqubo yokwenza i-'heme'.
- Uphawu oluphambili ziintlungu zesisu ezinganyamezelekiyo. Zinokubangela iingxaki zenkqubo yemithambo-luvo.
- Nokuba une-gene ebangela esi sifo, ukuba uyaziphepha izinto ezibangela esi sifo, usenokungabi nazimpawu.
- Amanye amayeza, utywala, ukutshaya, ukutya ukutya okungqongqo, kunye noxinzelelo zezona zinto ziphambili ezibangela oku.
- Ukuba unayo nayiphi na kwezi mpawu okanye ukrokrela ukuba une-AHP, thetha nogqirha wakho. Ungaze uphume uye kuzithengela amayeza.
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