Zovuta

Porphyrias ndi gulu la zovuta zobadwa nazo. Gawo lofunika kwambiri la hemoglobin, lotchedwa heme, silinapangidwe bwino. Hemoglobin ndi mapuloteni m'maselo ofiira amwazi omwe amanyamula mpweya. Heme amapezekanso mu myoglobin, mapuloteni omwe amapezeka muminyewa ina.

Nthawi zambiri, thupi limapanga heme m'njira zingapo. Porphyrins amapangidwa munthawi zingapo za njirayi. Anthu omwe ali ndi porphyria akusowa ma enzyme ena ofunikira pantchitoyi. Izi zimayambitsa ma porphyrins kapena mankhwala ofanana kuti azikhala mthupi.

Pali mitundu yambiri ya porphyria. Mtundu wofala kwambiri ndi porphyria cutanea tarda (PCT).

Mankhwala osokoneza bongo, matenda, mowa, ndi mahomoni monga estrogen amatha kuyambitsa mitundu ina ya porphyria.

Porphyria ndi yotengera. Izi zikutanthauza kuti vutoli limaperekedwa kudzera m'mabanja.

Porphyria imayambitsa zizindikilo zazikulu zitatu:

• Kupweteka m'mimba kapena kupweteka (kokha mwa mitundu ina ya matenda)
• Kuzindikira kuwunika komwe kumatha kuyambitsa ziphuphu, matuza, ndi mabala a khungu (photodermatitis)
• Mavuto amanjenje ndi minofu (kugwidwa, kusokonezeka kwamaganizidwe, kuwonongeka kwa mitsempha)

Kuukira kumachitika mwadzidzidzi. Nthawi zambiri amayamba ndikumva kuwawa m'mimba pambuyo pake kusanza ndi kudzimbidwa. Kukhala panja padzuwa kumatha kupweteketsa, kumva kutentha, matuza, komanso khungu lofiira ndi kutupa. Matuza amachira pang'onopang'ono, nthawi zambiri amakhala ndi zipsera kapena khungu. Chipsera chimatha kukhala chowononga. Mkodzo ukhoza kukhala wofiira kapena wofiirira mukatha kuukira.

Zizindikiro zina ndizo:

• Kupweteka kwa minofu
• Minofu kufooka kapena ziwalo
• Dzanzi kapena kumva kulasalasa
• Kupweteka kwa mikono kapena miyendo
• Ululu kumbuyo
• Umunthu umasintha

Kuukira nthawi zina kumatha kukhala koopseza moyo, ndikupanga:

• Kuthamanga kwa magazi
• Kusamvana kwakukulu kwa electrolyte
• Chodabwitsa

Wothandizira zaumoyo wanu adzakuyesani, zomwe zimaphatikizapo kumvera mtima wanu. Mutha kukhala ndi vuto logunda kwamtima (tachycardia). Wothandizira angapeze kuti ma tendon reflexes anu (mawondo kapena ena) sagwira bwino ntchito.

Kuyezetsa magazi ndi mkodzo kumatha kuwonetsa mavuto a impso kapena mavuto ena. Mayeso ena omwe angachitike ndi awa:

• Mpweya wamagazi
• Zowonjezera zamagetsi
• Magulu a Porphyrin ndi kuchuluka kwa mankhwala ena okhudzana ndi izi (kuyang'aniridwa m'magazi kapena mkodzo)
• Ultrasound pamimba
• Kupenda kwamadzi

Ena mwa mankhwala omwe amagwiritsidwa ntchito pochiza mwadzidzidzi (pachimake) ku porphyria atha kukhala:

• Hematin amaperekedwa kudzera mumitsempha (kudzera m'mitsempha)
• Mankhwala opweteka
• Propranolol kuwongolera kugunda kwa mtima
• Njira zokuthandizani kuti mukhale chete komanso musakhale ndi nkhawa zambiri

Mankhwala ena atha kukhala:

• Beta-carotene amathandizira kuchepetsa photosensitivity
• Chloroquine wambiri kuti muchepetse kuchuluka kwa ma porphyrins
• Zamadzimadzi ndi shuga zimathandizira kuchuluka kwamahydrohydrate, omwe amathandiza kuchepetsa kupanga ma porphyrins
• Kuchotsa magazi (phlebotomy) kuti muchepetse kuchuluka kwa ma porphyrins

Kutengera mtundu wa porphyria womwe muli nawo, omwe amakupatsani akhoza kukuwuzani kuti:

• Pewani mowa wonse
• Pewani mankhwala omwe angayambitse
• Pewani kuvulaza khungu
• Pewani kuwala kwa dzuwa momwe mungathere ndikugwiritsa ntchito zotchingira dzuwa mukakhala panja
• Idyani zakudya zamagulu azakudya zambiri

Zida zotsatirazi zitha kupereka zambiri pa porphyria:

• American Porphyria Foundation - www.porphyriafoundation.org/for-patients/patient-portal
• National Institute of Diabetes and Digestive and Impso Diseases - www.niddk.nih.gov/health-information/liver-disease/porphyria
• National Organisation for Rare Disways - rarediseases.org/rare-diseases/porphyria

Porphyria ndimatenda amoyo wokhala ndi zizindikilo zomwe zimabwera ndikutha. Mitundu ina ya matenda imayambitsa zizindikiro zambiri kuposa zina. Kulandila chithandizo choyenera ndikukhala kutali ndi zoyambitsa kumatha kutalikitsa nthawi pakati pa ziwopsezo.

Zovuta zingaphatikizepo:

• Coma
• Miyala
• Kufa ziwalo
• Kulephera kupuma (chifukwa chofooka kwa minofu pachifuwa)
• Kutupa kwa khungu

Pezani chithandizo chamankhwala mukangokhala ndi zodandaula. Lankhulani ndi omwe akukuthandizani za chiopsezo chanu ngati muli ndi mbiri yayitali yakumva kupweteka m'mimba, mavuto am'mimba ndi mitsempha, komanso kuzindikira kwa dzuwa.

Upangiri wa chibadwa ungapindulitse anthu omwe akufuna kukhala ndi ana komanso omwe ali ndi mbiri yabanja yamtundu uliwonse wa porphyria.

Porphyria cutanea tarda; Porphyria yapakatikati; Cholowa coproporphyria; Kobadwa nako erythropoietic porphyria; Erythropoietic protoporphyria

• Porphyria cutanea tarda m'manja

Bissell DM, Anderson KE, Bonkovsky HL. Zovuta. N Engl J Med. 2017; 377 (9): 862-872. PMID: 28854095 www.ncbi.nlm.nih.gov/pubmed/28854095. (Adasankhidwa)

Wodzaza SJ, Wiley JS. Heme biosynthesis ndi zovuta zake: porphyrias ndi sideroblastic anemias. Mu: Hoffman R, Benz EJ, Silberstein LE, et al, olemba. Hematology: Mfundo Zoyambira ndi Zochita. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 38.

Khalani TP. Matenda okhudzana ndi kuwala ndi zovuta zamatenda amtundu. Mu: Habif TP, mkonzi. Clinical Dermatology: Buku Lopangira Kuzindikira ndi Chithandizo. Lachisanu ndi chimodzi. Philadelphia, PA: Elsevier; 2016: mutu 19.

Kwezani RJ. The porphyrias. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. Wolemba 25. Philadelphia, PA: Elsevier Saunders; 2016: chap 210.