Tangoganizirani mukumva kupweteka kwa m'mimba mwadzidzidzi komanso kopweteka popanda chifukwa chomveka, limodzi ndi kumva kuwawa kapena dzanzi m'miyendo yanu, kufooka kwa minofu, komanso nseru. Ngakhale mutapita kwa madokotala angapo ndikuyesedwa kangapo, chomwe chimayambitsa vutoli sichikudziwika. Kodi inuyo kapena munthu wina amene mumamudziwa adakumanapo ndi izi? Ikhoza kukhala vuto la majini losowa lomwe nthawi zambiri limanyalanyazidwa. Lero, tikuyang'ana vutoli: Acute Hepatic Porphyria , kapena AHP.
Kodi Acute Hepatic Porphyria (AHP) ndi chiyani kwenikweni?
AHP ndi matenda obadwa nawo omwe amakhudza mitsempha yanu yamagazi komanso nthawi zina khungu lanu. Ndi osowa kwambiri, ndipo amakhudza anthu pafupifupi asanu pa anthu 100,000 aliwonse. Matendawa angayambitse mavuto azaumoyo mwadzidzidzi, oopsa, komanso omwe angawononge moyo.
Kuti timvetse chifukwa chake, tiyeni tiwone magazi anu. Maselo athu ofiira amagazi ali ndi puloteni yotchedwa Hemoglobin . Ntchito yake yayikulu ndikutenga mpweya kuchokera m'mapapo anu ndikuupereka ku ziwalo ndi minofu yanu—monga ntchito yapadera yoperekera mpweya.
Kuti mupange puloteni iyi, thupi lanu limafunika chinthu chofunikira kwambiri chotchedwa Heme .
Kwa anthu omwe ali ndi AHP, enzyme inayake yomwe imafunika popanga Heme imakhala yoperewera kapena ikusowa. Taganizirani ngati kuyesa kuphika chakudya koma osapeza chinthu chofunikira; chinthu chomaliza sichingapangidwe bwino.
Kusowa kwa ma enzyme kumeneku kumachitika makamaka m'chiwindi , chomwe chimatchedwanso kuti hepatic system. Ichi ndichifukwa chake matendawa amatchedwa Acute Hepatic Porphyria .
Thupi lanu likalephera kupanga Heme, zinthu zomwe zimapanga mankhwala—makamaka zinthu zoopsa monga Porphobilinogen (PBG) ndi Aminolevulinic acid (ALA)—zimayamba kusonkhana m'chiwindi. Ma poizoni amenewa amalowa m'magazi ndipo amatha kuyenda m'thupi lonse, makamaka kuwononga mitsempha yanu. Kuwonongeka kwa mitsempha kumeneku ndi komwe kumabweretsa zizindikiro monga kupweteka kwambiri , dzanzi , ndi nseru .
Kodi Mitundu Yaikulu ya AHP Ndi Chiyani?
Pali mitundu ikuluikulu inayi ya AHP. Ngakhale kuti iliyonse imakhala ndi kusowa kwa enzyme yosiyana pakupanga Heme, yonse inayi imakhudza kwambiri chiwindi ndi dongosolo lamanjenje.
Porphyria Yosatha (AIP)
Imeneyi ndi njira yofala kwambiri, yomwe imakhudza 80% ya odwala AHP. Imayamba chifukwa cha kusowa kwa enzyme ya Hydroxymethylbilane synthase (HMBS), yomwe imachitika chifukwa cha kusintha kwa majini a HMBS. Izi zitha kuchitika kuchokera kwa kholo, kapena zitha kuchitika ngati kusintha kwa majini mwachisawawa popanda mbiri ya banja.
Kodi AHP Imamveka Bwanji?
Zizindikiro za AHP zimasiyana kwambiri. Anthu ena sangakumane ndi zizindikiro, pomwe ena angavutike pafupipafupi komanso moopsa. Kuzindikira zizindikirozi ndikofunikira kwambiri.
| Thupi Lokhudzidwa | Zizindikiro Zodziwika |
|---|---|
| Mimba | Kupweteka kwambiri m'mimba ( chizindikiro chachikulu ), nseru, kusanza, ndi kudzimbidwa. |
| Dongosolo la Mitsempha | Kusanza kapena kumva kuwawa m'miyendo, kufooka kwa minofu, kupweteka kwa minofu, komanso, nthawi zina, kufooka kwa ziwalo. |
| Thanzi la Maganizo | Kusakhazikika, nkhawa, chisokonezo, ndi kuona zinthu molakwika. |
| Mtima ndi Kuthamanga kwa Magazi | Kuthamanga kwa mtima mofulumira (tachycardia) ndi kuthamanga kwa magazi. |
| Zina | Mkodzo wakuda, wofiira, kapena wofiirira (makamaka panthawi ya kuukira). |
Popeza AHP ndi yosowa, zizindikiro zake nthawi zambiri zimasokonezedwa ndi matenda ofala kwambiri, zomwe zingachedwetse kuzindikira matendawa.
Kodi N’chiyani Chimayambitsa Kuukira?
Anthu ambiri omwe ali ndi AHP amakhala ndi moyo wabwinobwino, koma zinthu zina zomwe zimayambitsa matendawa zingayambitse matenda adzidzidzi. Kudziwa izi ndikofunikira:
- Mankhwala Ena: Makamaka ma barbiturates ndi maantibayotiki a sulfonamide. Ngati muli ndi AHP, nthawi zonse funsani dokotala wanu musanayambe mankhwala atsopano.
- Mowa: Choyambitsa chachikulu cha kuukira kwa AHP.
- Kuchepetsa/Kusala Kudya Zakudya Zopatsa Ma calories: Zakudya zochepa kwambiri za calories ndi carbohydrates zingayambitse zizindikiro.
- Kusintha kwa mahomoni: Makamaka zomwe zimayenderana ndi msambo mwa akazi.
- Kupsinjika maganizo ndi matenda: Kupsinjika maganizo, malungo, kapena matenda wamba monga chimfine zingayambitse vuto.
Kodi Muyenera Kuonana ndi Dokotala Liti?
Ngati mukukumana ndi zotsatirazi, musawachotse. Funsani upangiri wa dokotala ngati:
- Mumakhala ndi ululu waukulu m'mimba womwe umabwerezedwabwerezedwa komanso wosafotokozeka.
- Mumakumana ndi zizindikiro zokhudzana ndi mitsempha monga kumva kuwawa kapena kufooka kwa miyendo.
- Muli ndi mbiri yodziwika bwino ya banja la Porphyria.
- Mukuwona kuti mkodzo wanu wasanduka wofiira kwambiri kapena bulauni.
Ngati mukukumana ndi vuto ladzidzidzi, monga kupweteka kosapiririka, kuvutika kupuma, kusokonezeka mwadzidzidzi, kapena kulephera kusuntha miyendo yanu, funani chithandizo mwamsanga ku dipatimenti yadzidzidzi yapafupi kapena imbani 911.
Kodi Pali Chithandizo cha AHP?
Ngakhale kuti palibe mankhwala a matendawa, pali mankhwala othandiza kwambiri omwe alipo kuti athetse ndikuletsa kuukira.
Matenda oopsa amafunika kuti munthu agonekedwe kuchipatala kuti akhazikike bwino.
- Njira yoyamba ndiyo kuzindikira ndi kuchotsa choyambitsa.
- Kuchepetsa ululu mwamphamvu kumagwiritsidwa ntchito poletsa kusasangalala kwambiri.
- Mankhwala amaperekedwa kuti achepetse nseru ndi kusanza.
- Kupereka shuga m'mitsempha kungathandize kuchepetsa kupanga kwa poizoni m'chiwindi.
- Chithandizo Chapadera: Kulowetsedwa kwa Hemin kumagwira ntchito ngati njira yolowera mwachindunji, kuwonetsa chiwindi kuti chisiye kupanga mopitirira muyeso zinthu zoopsa za Heme.
- Kuphatikiza apo, kwa iwo omwe amakumana ndi ziwopsezo pafupipafupi, njira zamakono zoletsa majini tsopano zikupezeka.
Dokotala wanu adzasankha njira yochizira yomwe ikugwirizana ndi zosowa zanu.
Uthenga Wopita Nawo Kunyumba
- Matenda a Acute Hepatic Porphyria (AHP) ndi matenda osowa kwambiri omwe amapezeka m'majini, koma ndi chithandizo, anthu ambiri amakhala ndi moyo wabwinobwino komanso wokhutiritsa.
- Chizindikiro chodziwika bwino ndi kupweteka kwambiri m'mimba komwe kumabwerezabwereza komwe kumayenderana ndi mavuto amitsempha (kusanza, kufooka).
- Kupewa zinthu zomwe zimayambitsa vuto la kudya—monga mankhwala enaake, mowa, ndi kusala kudya kwa nthawi yayitali—n’kofunika kwambiri.
- Ngati mukukayikira kuti muli ndi zizindikiro, kapena muli ndi mbiri ya banja, funsani dokotala wanu mwamsanga.
- Pali mankhwala othandiza omwe angathandize kuchepetsa zizindikiro ndikupewa kuukira kwamtsogolo. Palibe chifukwa chochita mantha; gwirani ntchito limodzi ndi dokotala wanu.
AHP, Acute Hepatic Porphyria, Porphyria, kupweteka m'mimba, matenda amitsempha, matenda a majini, heme, hemoglobin, porphyrin, matenda osowa
