Porphyria - he maʻi o kēia kanaka a pau

porphyria maʻi mea he kūikawāʻano o ke akepaa pathologies ma ka aaiaoe pae, ma iʻaʻole loa pololei ole hemoglobin hoʻololi kemikala. Ua Ua manaoio ia, he ewalu ka mea i hahai ana ka mea mamuli o kekahi no laila,-kapaia enzymatic kapuwaʻi i loko o ka biosynthesis o hemoglobin. Naha a ma ke kahua o kekahi o ia mau mea i mua e alakai i ka pilikia e like me porphyria. Ka maʻi ana e hoʻomōhala ana i hikiwawe loa. Ma kēia 'atikala e kūkākūkā mākou i ka loa au mamuli ia mea i kēia maʻi, a mea mau i ka laekahi' ano o ka nohoʻana.

Porphyria - he maʻi o kēia kanaka. He aha ka keʻano papa hana o ka maʻi?

Initially, ai '-kumuʻiʻo kekahi hapa o ka hemoglobin (i ole ia - heme) ua hoohuliia i kaʻawahia waiwai a, ma ka huli, kūpono e ai ana aku e pāpā ikaika a pau i ka subcutaneous'aʻaʻa. No laila, i kaʻili i nā loaʻa iā ia kekahi he brownish tint, e lilo ana thinner me ka manawa, a me ka pololei Map nahae. I mea no ke aha nā mea maʻi i kēia maʻi, e hiki ke loaʻa mai hehe lehulehu a me ka scars. No laila, ke kiʻekiʻe o ka ulu 'porphyria. Maʻi, ua nana i ka wa e kaawale aku o ka 'eleu lapaʻau, i hookuu ka hopuʻia a pau o ka hou like o ka ili.

kumu

Sequential kue o ka heme biosynthesis, i i kūkākūkā i kekahi mau mea ma luna,ʻo ia excessive hoʻomāhuahua o porphyrins i loko o ka meaola, like hoʻi me no laila, kapaʻia precursors (e laʻa me, ALA a me porphobilinogen). Ka mea, ua i ka hope loaʻa pākeu, he ikaikaʻawahia ia ma luna o ka meaola, like me ka hopena o nā kumu, ua ulu lapaʻau symptoms. Ke kumu no ka 'aʻe' ia nei he mutation o ka'ōewe kuleana no ka ha awina o kekahi nā'ūhū komo i loko o ka nunui-ʻanuʻu hoʻololi kemikala o ka heme. Ma ka manawa, e hana ka noiʻi i loko o ka oi piha study o kēia mau keʻano o ka hanaʻana a me keia mai e like me porphyria.

maʻi symptoms

• Poino i ka Central ko mākou hopohopo nenoaiu.
• Mimi he pinkish hue.
• Lehulehu na mai hehe, a scars ma luna o ke kino.
• Pain a discomfort i loko o ka abdominal wahi.
• Hoʻonuiʻia ke koko.

He aha e e hana?

Eia naʻe, ma ka manawa, poe akamai i hiki ole kaumaha ka hoʻokō ano o pathogenetic Inc o keia maʻi. Ma kekahi hihia (e laʻa me, erythropoietic uroporfiriya) lapaʻau hiki e hoʻokō. Intermittent porphyria (i ka maʻi i loko o ka huʻi ke kahua) loa e ole ae i ka pono o ka dipyrone a me tranquilizers, e like me ka mea, i ka exacerbation o symptoms. Ma ke alo o ka eha i loko o ka maʻi i porphyria, e like me ka rula, kupua mai i ka ai 'ikaika.

ka hopena

Porphyria maʻi (kiʻi helu 1 hōʻike kekahi o kona hoike ana), Eia naʻe ua diagnosed i loko o kēia mau lā hou pinepine. Ma ka hopena, ka mea e e kaulana ia i kēia lā i kēiaʻano o ka maʻi nō koe poorly i ike. Epekema mai a puni ka honua hoʻomau e noiʻi ana i ke kumu kumu o, iniiaiie lapaʻau symptoms, e like me kāu kiʻina hana e hakakā ia kaʻawaʻawa loa pilikia. E hoʻokaʻaʻike ola!