Kupweteka kwambiri m'mimba komanso kutaya chidziwitso popanda chifukwa... Tiyeni tiphunzire za matenda osowa awa? (Acute Hepatic Porphyria)

Tangoganizirani, popanda chifukwa chomveka, mwadzidzidzi mukumva kupweteka m'mimba kosapiririka. Pamodzi ndi zimenezo, miyendo yanu imafooka, mukumva ngati mukomoka, ndipo mukumva nseru. Ngakhale mutapita kwa madokotala angapo ndikuchita mayeso ambiri, chifukwa chenicheni cha matendawa sichingapezeke. Kodi inuyo kapena munthu wina amene mumamudziwa adakumanapo ndi izi? Mwina chifukwa chake ndi matenda osowa kwambiri a majini omwe anthu ambiri m'dziko lathu sanamvepo. Lero tikambirana za vuto limodzi lotere, Acute Hepatic Porphyria , kapena AHP.

Mwachidule, kodi Acute Hepatic Porphyria (AHP) ndi chiyani?

AHP ndi matenda osowa kwambiri omwe amakhudza mitsempha yathu komanso nthawi zina khungu lathu. Ndi osowa kwambiri moti amakhudza anthu 5 pa anthu 100,000. Angayambitse zizindikiro zadzidzidzi, zoopsa, komanso zopweteka. Nthawi zina, izi zimatha kupha munthu.

Kuti timvetse izi, tiyeni tikambirane pang'ono za magazi m'thupi lathu. Maselo athu ofiira a m'magazi ali ndi puloteni yotchedwa " Hemoglobin ". Ntchito yake yayikulu ndikutenga mpweya womwe umabwera m'mapapo tikamapuma ndikuupereka ku ziwalo zina zonse ndi minofu m'thupi. Zili ngati 'ntchito yotumizira' yomwe imanyamula mpweya.

Pali chinthu china chofunikira popanga puloteni iyi chotchedwa ``Hemoglobin'', ndipo timachitcha ``Heme'' .

Chomwe chimachitika m'thupi la munthu amene ali ndi matenda a AHP ndi kuchepa kapena kutayika kwa enzyme yomwe imafunika panthawi inayake popanga chinthu ichi chotchedwa ``Heme''. Zili ngati pophika mbale, ngati chosakaniza chimodzi sichikupezeka, mbaleyo singathe kukonzedwa bwino.

Kusowa kwa enzyme iyi kumachitika makamaka m'chiwindi chathu. Timatchanso chiwindi kuti `(Chiwindi)`. Ichi ndichifukwa chake matendawa amatchedwa `Acute Hepatic Porphyria` .

Tsopano, chimachitika ndi chiyani tikalephera kupanga `(Heme)`? Zinthu zopangira zomwe zinagwiritsidwa ntchito, zomwe ndi `(Porphobilinogen – PBG)` ndi `(Aminolevulinic acid – ALA)`, zimayamba kusonkhana m'chiwindi. Ma poizoni amenewa amasonkhana m'magazi ndikuyenda m'thupi lonse, makamaka kuwononga dongosolo lathu la mitsempha. Chifukwa cha kuwonongeka kwa mitsempha kumeneku, zizindikiro monga kupweteka kwambiri , dzanzi , ndi nseru zimabuka.

Kodi mitundu ikuluikulu ya matenda a AHP ndi iti?

Pali mitundu ikuluikulu inayi ya AHP. Mtundu uliwonse umayamba chifukwa cha kusowa kwa enzyme yosiyana popanga ``(Heme)``. Koma zonse zinayi zimakhudza kwambiri chiwindi ndi dongosolo lamanjenje.

Porphyria Yokhazikika Pakati Pafupi (AIP)

Uwu ndi mtundu womwe umakhudza 80% ya odwala AHP, kutanthauza ambiri. Pachifukwa ichi, pali kusowa kwa enzyme `(Hydroxymethylbilane synthase - HMBS)`. Chifukwa cha izi ndi kusintha kwa jini ya `(HMBS)`. Mu zamankhwala, timatcha jini iyi `(mutation)` . Izi nthawi zina zimatha kutengera kuchokera kwa makolo kupita kwa ana. Kapena nthawi zina zimatha kuchitika mwangozi mwa munthu wopanda mbiri ya banja.

Kodi zizindikiro za AHP zimamveka bwanji?

Si aliyense amene ali ndi AHP amene ali ndi zizindikiro zofanana. Anthu ena sangakhale ndi zizindikiro zilizonse pa moyo wawo wonse. Koma ena akhoza kukhala ndi matenda oopsa pafupipafupi. Ndikofunikira kuzindikira zizindikirozi.

Kodi ndi zinthu ziti zomwe zimayambitsa matendawa?

Munthu amene ali ndi matenda a AHP akhoza kukhala moyo wabwinobwino. Komabe, pali zinthu zina zomwe zingayambitse matendawa. Ndikofunikira kwambiri kudziwa izi.

• Mankhwala ena: Makamaka mankhwala ena monga maantibayotiki okhala ndi `(Barbiturates)` ndi `(Sulfa)`. Ngati muli ndi AHP, ndikofunikira kulankhula ndi dokotala wanu musanamwe mankhwala aliwonse.
• Kumwa mowa: Mowa ndiye chinthu chachikulu chomwe chimayambitsa kufalikira kwa matendawa.
• Kusala kudya mopitirira muyeso kapena kudya mopanda mphamvu: Kudya zakudya zokhala ndi ma calories ochepa komanso chakudya chopatsa mphamvu (starch) kungapangitse kuti matendawa achuluke kwambiri.
• Kusintha kwa mahomoni: Kusintha kwa kuchuluka kwa mahomoni, makamaka mwa akazi, komwe kumakhudzana ndi nthawi yawo ya msambo.
• Kupsinjika Maganizo ndi Matenda: Kupsinjika maganizo kulikonse m'thupi, ngakhale matenda opatsirana monga malungo kapena chimfine, kungapangitse kuti matendawa achuluke.

Kodi muyenera kupita kwa dokotala liti?

Ngati muli ndi zizindikiro izi, chonde musamazione ngati zachilendo. Ndikofunikira kwambiri kufunafuna upangiri wa dokotala, makamaka pazochitika izi:

• Ngati muli ndi ululu waukulu m'mimba womwe sunadziwike, womwe umabwerezabwereza.
• Ngati zizindikiro za mitsempha monga dzanzi ndi kufooka zimachitika nthawi imodzi.
• Ngati mukudziwa kuti wina m'banja mwanu ali ndi matendawa `(Porphyria)`.
• Ngati muwona kuti mkodzo wanu ukusintha kukhala wofiira/bulauni wakuda.

Ngati mukukumana ndi zizindikiro zoopsa, monga kupweteka kosapiririka, kuvutika kupuma, kusokonezeka maganizo, kapena kutayika kwa miyendo, pitani ku chipatala cha Emergency Treatment Unit (ETU) nthawi yomweyo.

Kodi pali chithandizo cha AHP?

Popeza iyi ndi matenda obadwa nawo, palibe mankhwala omwe alipo pakadali pano. Komabe, pali mankhwala othandiza kwambiri oletsa ndikuletsa kufalikira kwa matendawa.

Pa milandu yoopsa, kugonekedwa m'chipatala ndi kulandira chithandizo ndikofunikira.

• Chofunika kwambiri ndi kuzindikira chomwe chinayambitsa matendawa ndikuletsa.
• Mankhwala amphamvu ochepetsa ululu amaperekedwa kuti achepetse ululu waukulu.
• Mankhwala amaperekedwa kuti achepetse nseru ndi kusanza.
• Mwa kupatsa thupi shuga (shuga), kutanthauza shuga wokhala ndi mchere kudzera mu mitsempha, kupanga poizoni m'chiwindi kungachepe.
• Monga chithandizo chapadera , jakisoni wa `(Heme)` amachitidwa `(Hemin infusions)`. Izi zimachitika popereka `(Heme)` kunja, kutumiza chizindikiro ku chiwindi kuti, "Muli ndi `(Heme)`, simukufunika kupanga zina," ndikuletsa kupanga poizoni.
• Kupatula izi, tsopano pali mankhwala amakono opangidwa kudzera mu ukadaulo wa majini kuti aletse matendawa kubwereranso kwa iwo omwe ali ndi kukulirakulira pafupipafupi.

Dokotala amene adzakuyang'anirani adzasankha chithandizo choyenera kwambiri kwa inu.

AHP, Acute Hepatic Porphyria, Porphyria, Kupweteka kwa m'mimba, Matenda a mitsempha, Matenda a majini, Heme, Hemoglobin, Porphyrin, Matenda osowa