He maʻi ʻē paha kāu āu e hoʻomaopopo ʻole ai? He Acute Hepatic Porphyria paha ia!

Ke manaʻo nei ʻoe he ʻeha ʻōpū kou i hiki ʻole ke noʻonoʻo ʻia, i kekahi manawa me ka puʻuwai kaumaha, a me ka mae ʻana o kou mau lālā? Malia paha ʻaʻole hiki i nā kauka ke ʻike i ke ʻano o ia mea. ʻO kekahi o kēia mau hōʻailona ʻē, me he mea lā ʻaʻole pili a mākou e kamaʻilio ai i kēia lā, ʻo ia ka Acute Hepatic Porphyria (AHP). Mai hopohopo, he paʻakikī iki kēia, akā e hoʻomaopopo maʻalahi kākou.

He aha ke ʻano o ka acute hepatic porphyria (AHP)?

I ka ʻōlelo maʻalahi, he maʻi genetic kakaikahi loa ka AHP. Hoʻomaka ia i loko o kou ake . Akā hoʻopilikia pū ia i kāu ʻōnaehana nerve, e hana ana i nā hōʻailona ma kou kino holoʻokoʻa. E noʻonoʻo e pili ana, aia kekahi mea i kapa ʻia ʻo heme, ʻo ia kekahi hapa o ka hemoglobin i loko o ko mākou koko. Pono ko mākou kino i nā enzymes like ʻole e hana i kēia heme. Loaʻa i ke kanaka me AHP kahi hemahema, a i ʻole ka nele, o kekahi o nā enzymes e pono ai e hana i kēia heme.

He aha ka mea e hana ʻia ke hana ʻia kēia? Ua koe kekahi o nā kemika i pono e hoʻohana ʻia no ka hana ʻana i ka heme. Kāhea mākou i kēia mau koena he mau mea mua o ka porphyrin . Ke hōʻiliʻili kēia mau mea, he mea ʻawahia lākou i ke kino. I loko o AHP, hōʻiliʻili mua kēia mau mea ʻawahia i loko o ke akepaʻa. A laila, ke komo lākou i loko o ke koko a launa pū me nā aʻalolo, hoʻomaka nā hōʻailona e ʻike ʻia. Maopopo iā ʻoe?

Aia kekahi mau ʻano like ʻole o ka AHP?

ʻAe, ʻehā ʻano nui o ka AHP. Ua māhele ʻia kēia mau ʻano ʻehā i nā ʻano enzyme aʻu i ʻōlelo ai ma mua, ma muli o ka enzyme i nele. Ua kapa ʻia kēlā me kēia ʻano ma hope o ka ʻāpana i kapa ʻia ʻo "hepatic" no ka mea hoʻomaka ia i loko o ke akepaʻa, a ʻo ka ʻāpana i kapa ʻia ʻo "acute" no ka mea ʻike koke ʻia nā hōʻailona. Ma ke kaʻina mai ke ʻano maʻamau loa a i ka mea liʻiliʻi loa, eia nā ʻano:

1. ʻO ka Porphyria Intermittent Acute (AIP): ʻO kēia ka mea maʻamau. ʻO ka mutation i loko o ka gene HMBS ke kumu o ka hemahema o ka enzyme hydroxymethylbilane synthase (HMBS) (i ʻike ʻia hoʻi ʻo porphobilinogen deaminase (PBGD). Hiki i kēia ke lilo i mutation genetic hou, a i ʻole hiki ke hoʻoilina ʻia ma ke ʻano he ʻano autosomal dominant (ʻo ia hoʻi, hiki ke loaʻa ka maʻi ʻoiai inā hoʻokahi makua wale nō ka hoʻoilina o ka gene).

2. Variegate Porphyria (VP): ʻO kahi mutation i loko o ka gene PPOX ke kumu o ka hemahema o ka enzyme protoporphyrinogen oxidase (PPO a i ʻole PPOX). Hiki ke hoʻoilina ʻia kēia ma ke ʻano he autosomal dominant a autosomal recessive paha.

3. Hoʻoilina Coproporphyria (HCP): ʻO ka hoʻololi ʻana i loko o ka gene CPOX e hopena i ka nele o ka enzyme coproporphyrinogen oxidase (CPOX). Hiki ke hoʻoilina ʻia kēia ma ke ʻano he autosomal dominant a autosomal recessive paha.

4. ALAD-Deficiency Porphyria (ALAD-Deficiency Porphyria - ADP):ʻO ka hoʻololi ʻana i loko o ka gene ALAD ke kumu o ka nele i ka enzyme delta-aminolevulinic acid dehydratase (ALAD). Ua hoʻoilina ʻia ma ke ʻano autosomal recessive (ʻo ia hoʻi, hiki ke loaʻa ka maʻi inā hoʻoilina nā mākua ʻelua i ka gene).

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Pehea ka hopena o ka AHP iaʻu?

He mea kupanaha kēia, no ka mea, ʻaʻole like ka hopena o ka AHP i nā kānaka a pau. Loaʻa i kekahi poʻe ka hoʻololi ʻana o ka gene no ia mea akā ʻaʻole loaʻa kekahi mau hōʻailona . Loaʻa paha i kekahi poʻe nā "hoʻouka" o nā hōʻailona e kū hoʻokahi a ʻelua paha i ko lākou ola. Akā loaʻa pinepine kēia mau hoʻouka i kekahi poʻe, a loaʻa i kekahi poʻe ma ke ʻano mau , ʻo ia hoʻi ke hoʻomau nei lākou i nā hōʻailona e hoʻopilikia i ko lākou ola o kēlā me kēia lā. I kekahi manawa hiki ke koʻikoʻi loa kahi hoʻouka a pono paha ʻoe e wikiwiki i ka lumi pilikia o ka haukapila. Hiki iā ʻoe ke ʻike i ka ʻeha nui o ke aʻalolo, nā loli o ka ʻili, a me nā hōʻailona e pili ana i ka ʻōnaehana nerve.

ʻO wai ka mea kūpono loa e loaʻa i kēia?

Hiki i ka AHP ke hoʻopilikia i nā kānaka o kēlā me kēia lāhui a i ʻole ke kala. Hiki ke hoʻoilina ʻia mai kekahi a i ʻole nā ​​mākua ʻelua i loaʻa ka hoʻololi ʻana o ka gene. ʻO ka mea kupanaha, ʻaʻole nui ka poʻe e loaʻa nā hōʻailona . No ka ulu ʻana o nā hōʻailona, ​​ʻaʻole lawa ka hoʻololi ʻana o ka gene wale nō. Pono nō hoʻi kekahi mau "mea hoʻonāukiuki" ʻē aʻe.

He aha kēia mau mea hoʻonāukiuki?

• Nā loli hormonal (ʻoi aku hoʻi i ka wā ʻōpio, ka hāpai ʻana, a me ke kaʻina hana menstrual)
• Kekahi mau lāʻau lapaʻau
• Ka hoʻohana ʻana i ka waiʻona
• Puhipaka
• Kaumaha nui
• Kaohi ʻana i ka ʻai pono (ʻoiai ke kaupalena ʻana i ka calorie)

He mea kūpono hoʻi ke ʻike ʻia he 80% o ka poʻe i loaʻa nā hōʻailona he mau wahine hānau keiki .

Pehea ka maʻamau o ka AHP?

He mea paʻakikī ke ʻōlelo maopopo, no ka mea, ʻaʻole pinepine i ʻike pono ʻia kēia maʻi. Ma ka honua holoʻokoʻa, ua manaʻo ʻia aia ma kahi o 5 o 100,000 mau kānaka e loaʻa iā ia. Ma waena o nā ʻano i ʻōlelo ʻia ma mua, ʻo 'Acute Intermittent Porphyria (AIP)' ke ʻano i ʻike ʻia ma 80% wale nō o nā hihia i hōʻike ʻia. ʻO 'ALAD-Deficiency Porphyria (ADP)' ke ʻano liʻiliʻi loa, me 9 mau hihia i hōʻike ʻia ma ka honua holoʻokoʻa. E noʻonoʻo, hoʻokahi wale nō o kēlā me kēia 10 mau kānaka me ka hoʻololi ʻana o ka gene e pili ana i ka AHP e loaʻa nā hōʻailona.

He aha nā hōʻailona o ka AHP?

He mea ʻē loa nā hōʻailona o ka AHP. Hiki ke paʻakikī i nā mea maʻi a me nā kauka ke hoʻomaopopo, no ka mea, hiki ke ʻike koke ʻia nā hōʻailona, ​​​​​​ʻoi aku ka koʻikoʻi, a me he mea lā ʻaʻole pili kekahi i kekahi.

Eia kekahi o kēia mau hiʻohiʻona:

ʻEha aʻalolo

Hiki ke loaʻa kēia ʻeha ma nā wahi like ʻole o ke kino:

• ʻEha nui o ka ʻōpū - ʻO kēia ka hōʻailona mua e kū mai ana i ka hapa nui o nā kānaka.
• ʻEha umauma
• ʻEha hope
• ʻEha ma nā lima a me nā wāwae

Nā pilikia o ka ʻōnaehana hoʻoheheʻe

Ma muli o ka hoʻopilikia ʻia ʻana o nā aʻalolo, hiki ke loaʻa nā pilikia me ka ʻōnaehana hoʻoheheʻe:

• Ka luaʻi a me ka luaʻi
• ʻAʻai ʻole
• Ka paʻa ʻana o ka ʻōpū
• Ka maʻi pākī

Nā hōʻailona o ka ʻōnaehana nerve waena

Pili pono kēia i ka lolo a me ka noʻonoʻo:

• Hopohopo
• ʻAʻole hiki ke hiamoe
• Kaumaha
• Delirium
• ʻO Hallucinations - ka ʻike ʻana a i ʻole ka lohe ʻana i nā mea i ʻike ʻole ʻia ma laila
• Status epilepticus/seizure (Seizure)

Nā hōʻailona o ka ʻōnaehana nerve peripheral

Hoʻopili ʻia kēia mau mea i nā aʻalolo ma nā wahi ʻē aʻe o ke kino:

• Nāwaliwali o nā ʻiʻo
• ʻO ka mae a me ke kani ʻana i nā lālā
• Luhi
• Pohō ʻike
• Ka lolo ʻana o nā ʻiʻo
• ʻO ka maʻi lolo hanu - He mea weliweli loa kēia a hiki ke hoʻopilikia i ka hanu ʻana.

Nā hōʻailona o ka ʻōnaehana nerve autonomic

ʻO kēia nā mea e hana koke ʻia ana, nā mea i hiki ʻole iā mākou ke kaohi:

• Nā palpitations o ka puʻuwai
• Ke koko kiʻekiʻe

Nā hōʻailona ʻili (ʻoi aku hoʻi ma ka 'Variegate Porphyria' a me ka 'Hereditary Coproporphyria')

Hiki i nā kānaka me kēia mau ʻano ʻelua ke loaʻa nā pilikia ʻili ke hōʻike ʻia i ka lā :

• Ka ʻike i ka lā
• ʻŌhūhū pehu
• ʻO ke kala ʻili (Pigmentation)
• Ka ʻeha

ʻO ka mea nui loa, ke loaʻa iā ʻoe ka porphyria, hiki ke loli ke kala o kou mimi . Ke hōʻiliʻili kēlā mau mea mua o ka porphyrin aʻu i ʻōlelo ai i loko o kou kino a hoʻokuʻu ʻia i loko o kou mimi, hiki i kou mimi ke lilo i kala ʻulaʻula-poni . Loaʻa ka huaʻōlelo "porphyria" mai ka huaʻōlelo Helene "porphura," ʻo ia hoʻi "poni." ʻO nā porphyrins i loko o ko kākou mau ʻulaʻula koko ke hāʻawi i ke koko i kona kala ʻulaʻula.

He aha ka hoʻouka kaua AHP?

No nā poʻe he nui me nā hōʻailona AHP, hele mai kēia ma ke ʻano o nā "hoʻouka kaua" i kekahi manawa. ʻIke pinepine kekahi poʻe iā lākou, ʻoi aku ka liʻiliʻi o kekahi. ʻIke nui kekahi poʻe iā lākou, aʻo kekahi ʻaʻole. ʻO ka maʻamau, mau kēia mau hoʻouka kaua no kekahi mau lā, a laila hoʻonui mālie a emi iho. Inā loaʻa iā ʻoe nā hōʻailona koʻikoʻi a weliweli, pono ʻoe e hele i ka haukapila. E like me kaʻu i ʻōlelo ai, ʻo nā mea e like me ka paralysis hanu, ka mea e paʻakikī ai ka hanu ʻana, he mau kūlana e pono ai ka mālama lapaʻau koke.

ʻO ka hōʻailona maʻamau o ka hoʻouka kaua AHP, ʻo ia ka ʻeha ʻōpū nui i wehewehe ʻole ʻia.ʻOi aku ma mua o 90% o ka poʻe me AHP e hele i ke kauka ma muli o kēia ʻeha ʻōpū. Eia nō naʻe, no ka mea he nui nā kumu o ka ʻeha ʻōpū, a ʻoiai ʻaʻole hiki i kahi X-ray a i ʻole kahi CT scan ke loaʻa ke kumu o kēia ʻeha (no ka mea he ʻeha aʻalolo ia), he mea paʻakikī loa ke ʻike. No ka mea ua hele pū kēia ʻeha ʻōpū me nā mea e like me ka nausea, ka luaʻi ʻana, a me ka constipation, manaʻo paha nā kauka he maʻi ʻōpū ia. Inā loaʻa iā ʻoe nā pilikia noʻonoʻo a me nā sensory, manaʻo paha lākou he maʻi neurological ia. Ke hui pū kēia mau hōʻailona pili ʻole, pono e ʻike i ke kauka i ʻike e pili ana i ia mea e ʻike ai he porphyria paha.

He aha nā hōʻailona mau loa e hiki ai i ka AHP ke hana?

ʻAʻole pinepine ka poʻe me ka maʻi ʻoluʻolu (ʻaʻole i lawa ka enzyme) i nā hōʻailona mau loa. Hoʻokahi a ʻelua paha mau hoʻouka kaua i ko lākou ola. Inā ʻike lākou i nā mea hoʻonāukiuki, hiki iā lākou ke pale aku iā lākou. Eia nō naʻe, loaʻa pinepine nā hoʻouka kaua i kekahi poʻe, a pinepine loa kekahi mau hōʻailona a hiki ke manaʻo ʻia he mau maʻi mau. ʻO ia mau hōʻailona:

• ʻEha
• Luhi
• Ka luaʻi
• Neuropathy ( ka ʻeha, ka ʻeha, a i ʻole ka nāwaliwali o nā lālā)

He aha nā pilikia o ka AHP?

ʻO nā hōʻailona e pili ana i ka ʻōnaehana nerve waena (e like me ka hopohopo, nā hallucinations, a me nā hopu ʻana) e ʻike pinepine ʻia i ka wā o ka hoʻouka kaua. Eia nō naʻe, hiki ke loaʻa pinepine nā hōʻailona e pili ana i ka ʻōnaehana nerve peripheral (e like me ka nāwaliwali o nā ʻiʻo a me ka maʻi lolo), a hiki ke loaʻa ka hōʻino mau loa ma hope o ka hoʻouka kaua koʻikoʻi. ʻO ka hōʻiliʻili ʻana o kēia mau precursors porphyrin hiki ke hana i ka hōʻino lōʻihi i kekahi mau ʻōpū. Hiki i kēia mau mea ke komo pū me:

• Ke koko kiʻekiʻe mau loa
• Maʻi akepaʻa mau loa
• Maʻi puʻupaʻa mau loa
• Ka maʻi ʻaʻai ate mua
• Kaumaha a me ka hopohopo

He aha ke kumu o ka AHP?

ʻO ke kumu nui o AHP, ʻo ia nā hoʻololi genetic . Akā e like me kaʻu i ʻōlelo ai ma mua, ʻaʻole ʻike koke ʻia nā hōʻailona ma hope o ka loaʻa ʻana o ka hoʻololi genetic. I ka hapanui o nā hihia, ʻike mua ʻia nā hōʻailona i ka wā ʻōpio, me nā loli hormonal. Eia kekahi, ʻo kekahi mau lāʻau lapaʻau, nā lāʻau lapaʻau, ka inu ʻona nui, ka palena nui o ka calorie, a me nā maʻi e kau nui ana i ke kaumaha ma ke kino hiki ke hoʻoulu iā ia.

ʻO ka mea like o kēia mau mea hoʻonāukiuki a pau, ʻo ia ka hoʻoulu ʻana i ke kaʻina hana heme o ke ake. Eia nō naʻe, i loko o ke ake o kekahi me AHP, ʻaʻole i hoʻopili pono ʻia nā huahana o ka hana heme (e like me nā precursors porphyrin), ʻo ia hoʻi, hoʻohana ʻia. No laila, hōʻiliʻili kēia mau precursors porphyrin i koe i loko o ke ake. ʻO ka wā wale nō e hōʻiliʻili ai kēia mau mea i kahi pae e hoʻomaka ai lākou e hoʻopilikia i ka ʻōnaehana nerve a hoʻoulu i nā hōʻailona.

Pehea e ʻike ʻia ai ka AHP? (Diagnosis)

No ka poʻe me AHPHiki ke paʻakikī ka loaʻa ʻana o kahi hōʻailona pololei , ʻoiai ʻaʻole kū hoʻokahi nā hōʻailona i kēia kūlana a me he mea lā ʻaʻole pili. Eia nō naʻe, hiki i ke kauka i kamaʻāina i ka AHP ke kānalua i ka AHP inā loaʻa iā ʻoe ka ʻeha o ka ʻōpū me nā hōʻailona o ka ʻōnaehana nerve waena a me ke kikowaena. ʻO ka hui pū ʻana o kēia mau hōʻailona ʻekolu ua manaʻo ʻia ʻo ia ka "classic triad" o AHP.

Inā kānalua ke kauka i ka AHP, hiki iā lākou ke hana i kekahi mau hoʻokolohua e hōʻoia iā ia.

• Hoʻāʻo mimi: Hiki ke hana koke ʻia kēia i ka wā o ka hoʻouka kaua. He maʻamau paha ka mimi ke ʻole ʻoe e loaʻa ana i ka hoʻouka kaua, akā i ka wā o ka hoʻouka kaua, e hōʻike ka mimi i nā pae kiʻekiʻe o nā precursors porphyrin (PBG a me ALA). ʻAʻole kēia he hoʻāʻo i hana wale ʻia no AHP, akā he hoʻāʻo maʻamau ia e hiki ke kōkua i nā kauka e kuhikuhi iā ʻoe ma ke ala kūpono.
• Ka hoʻāʻo ʻana i ka genetic: ʻO kēia ke ala maikaʻi loa a maopopo loa e ʻike ai i ka AHP (ke 'kūlana gula') . Inā he mau hōʻailona kou a ʻaʻole paha, hiki iā ia ke hōʻoia inā he hoʻololi gene kou e pili ana me AHP. Hiki iā ia ke haʻi iā ʻoe i ke ʻano o ka AHP āu i loaʻa ai a pehea ke koʻikoʻi o ka hemahema enzyme. Pili kēia i ka lawe ʻana i kahi laʻana koko a i ʻole ka lawai. Pono paha kāu haukapila kūloko e hoʻouna i ka laʻana i kahi lab kūikawā.

Pehea e mālama ʻia ai ka AHP?

ʻO ka mālama ʻana no AHP e kālele ana i ka pale ʻana a me ka kaohi ʻana i nā hoʻouka kaua . Pono paha ʻoe e hoʻokomo ʻia i ka haukapila i ka wā o ka hoʻouka kaua. Pono paha ʻoe i nā ʻano lāʻau like ʻole e hōʻemi i kāu mau hōʻailona. I nā manawa āu e loaʻa ʻole ai i nā hoʻouka kaua, pono paha ʻoe i nā lāʻau ʻē aʻe e kaohi i nā mea hoʻonāukiuki e hoʻonui ai i kou mau hōʻailona. Inā hana pū ʻoe a me kāu kauka e ʻike i nā mea hoʻonāukiuki e hoʻopilikia nui iā ʻoe, e ʻoi aku ka maikaʻi o kāu mālama ʻana.

Lapaʻau no kahi hoʻouka kaua koʻikoʻi:

• ʻO ka inikini Hemin: Inā loaʻa kahi hoʻouka kaua koʻikoʻi, hiki i ke kauka ke hāʻawi iā ʻoe i kahi inikini hemin i loko o kahi aa. Kōkua kēia i ka hōʻemi ʻana i ka nui o ka porphyrin i loko o kou koko. ʻO Hemin kahi mea i lawe ʻia mai nā hunaola koko ʻulaʻula a hōʻemi i ka nui o ka porphyrin i loko o kou kino.
• Hoʻomaha ʻeha: I ka hoʻouka kaua koʻikoʻi, pono paha nā lāʻau ʻeha ikaika e like me nā opioids.
• Phenothiazines: Hoʻohana ʻia kēia mau mea e kāohi i ka nausea nui a me ka luaʻi ʻana.
• Nā wai IV a me ka meaʻai: ʻO nā hōʻailona e like me ka ʻeha o ka ʻōpū, ka nausea, ka luaʻi ʻana, ka maʻi diarrhea, a me ka constipation hiki ke hoʻolilo i ke kino i nā calorie a me ka wai i ka wā o ka hoʻouka kaua. Hiki i ka AHP ke hoʻolilo i ka nalowale o nā mea e like me ka sodium a me ka magnesium. No laila, hiki ke hāʻawi ʻia nā wai i loaʻa nā carbohydrates a me nā electrolytes ma ke ʻano intravenous.
• Nā lāʻau lapaʻau hopu:Ma kahi o 20% o nā maʻi e pono ai ka mālama ʻana no ka epilepsy i ka wā o kahi hoʻouka kaua.

Nā koho lapaʻau lōʻihi:

• ʻO ka hemin prophylactic: ʻO ka hāʻawi ʻana i nā mahele haʻahaʻa o ka hemin i hoʻokahi manawa i ka pule i ka poʻe i loaʻa pinepine i nā hoʻouka kaua hiki ke pale i ka hōʻiliʻili ʻana o nā porphyrins.
• ʻO Givosiran (GIVLAARI®): He ʻano lāʻau lapaʻau gene kēia. Hoʻemi ia i ka hana ʻana o nā precursors porphyrin. Ua ʻae ʻia i kēia manawa he injection mahina e pale aku i nā hōʻailona AHP i ka poʻe i loaʻa pinepine i nā hoʻouka kaua.
• Hoʻōla hormone: Inā hoʻokumu ʻia kāu mau hoʻouka ʻana o AHP e kāu pōʻaiapuni menstrual, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau e like me nā analog GnRH (Gonadotropin-releasing hormone). Hoʻemi kēia i ka hana ʻana o ke kino i nā hormones estrogen a me progesterone.
• Lāʻau lapaʻau koko kiʻekiʻe: Inā ulu ka koko kiʻekiʻe mau loa, pono e kaohi ʻia me nā lāʻau lapaʻau kikoʻī.
• Ke hoʻoili ʻana i ke akepaʻa: ʻO ka poʻe i loaʻa pinepine i nā hoʻouka kaua e hoʻoweliweli ana i ke ola a ʻaʻole i pane i nā lāʻau ʻē aʻe, e noʻonoʻo paha lākou i kahi hoʻoili ʻana i ke akepaʻa. Hiki i kēia ke ho'ōla loa i ka maʻi.

Hiki ke pale ʻia ka AHP?

ʻAʻole hiki ke pale aku i ka maʻi mai ka hanana ʻana. Eia nō naʻe, hiki iā ʻoe ke kōkua i ka pale ʻana i nā hōʻailona mai ka hanana ʻana ma ka pale ʻana i nā mea hoʻonāukiuki . Inā ʻaʻole ʻoe i loaʻa i kahi hoʻouka kaua, ʻoi aku ka maikaʻi o ka pale ʻana i nā mea hoʻonāukiuki āpau. Inā ua loaʻa iā ʻoe kahi hoʻouka kaua a ua ʻike ʻoe i nā mea hoʻonāukiuki i hoʻokumu ai, ua lawa paha ka pale ʻana i kēlā mau mea hoʻonāukiuki kikoʻī.

Eia kekahi o nā mea hoʻonāukiuki i ʻike pinepine ʻia:

Nā ʻano lāʻau lapaʻau:

• Nā antihistamines (kekahi mau lāʻau lapaʻau no ke anu a me nā maʻi allergies)
• Nā lāʻau hoʻomaha
• Nā mea pale waha
• Lapaʻau hoʻololi hormone

Ka hoʻohana ʻana i nā mea:

• Paka (ʻO ka Paka)
• Paʻi mariuana
• ʻAila
• Nā lāʻau lapaʻau leʻaleʻa

Ke kaumaha:

• Nā maʻi
• ʻoki kino
• Ka ʻai ʻana i ka calorie haʻahaʻa (ʻoi aku ka ʻai ʻana i ka carbohydrate haʻahaʻa) ('Ka nele i ka calorie')
• Ke kaumaha noʻonoʻo

He aha ka wā e hiki mai ana no ka poʻe me AHP? (Prognosis)

ʻOkoʻa loa kēia mai kēlā kanaka a i kēia kanaka. ʻAʻole loa e loaʻa i ka nui o ka poʻe nā hōʻailona . No ka poʻe i loaʻa, hoʻokahi a ʻuʻuku wale nō paha nā hoʻouka ʻana o ka poʻe i ko lākou ola. ʻOiai hiki i nā hoʻouka ke hoʻoweliweli i ke ola, hoʻōla piha ka hapa nui o ka poʻe me ka mālama koke ʻana i ka lāʻau lapaʻau . Ma kahi o 5% o ka poʻe me AHP he mau hōʻailona pinepine a mau paha. No kēia poʻe, he kōkua pinepine nā lāʻau pale, a hiki ke manaʻo ʻia he koho hope loa ka hoʻoili ʻana i ke akepaʻa.

Pehea wau e mālama ai iaʻu iho i koʻu noho ʻana me AHP?

• E pale i nā mea hoʻonāukiuki maʻamau. ʻO ka waiʻona, ka puhipaka, a me nā lāʻau lapaʻau e pono ke kau ʻia ma luna o kāu papa inoa o nā mea e pale aku ai. Hiki paha iā ʻoe ke kamaʻilio me kāu kauka e pili ana i nā koho ʻē aʻe i kekahi o nā lāʻau lapaʻau āu e lawe ai.
• E ʻai i ka meaʻai olakino a kaulike. E pale i ka ʻai nui loa a me nā hoʻolālā meaʻai haʻahaʻa-carb. Inā ʻoe e hoʻokē ʻai ana ma mua o kahi hoʻokolohua lapaʻau a ʻoki kino paha, e kamaʻilio me kāu kauka e pili ana i ia mea.
• E hana i nā hoʻokolohua lapaʻau maʻamau. ʻOiai inā ʻaʻohe ou hōʻailona, ​​e nānā mau kāu kauka i kou ake, nā puʻupaʻa, a me ke koko.

He maʻi laha ʻole ka porphyria hepatic koʻikoʻi, no laila ua haʻahaʻa ka ʻike e pili ana i ia mea i waena o ka lehulehu. Ke hiki koke mai kahi hoʻouka kaua me nā hōʻailona koʻikoʻi, he mea huikau loa ia a he mea weliweli hoʻi. Hoʻolilo kekahi poʻe i nā makahiki e hoʻāʻo nei e ʻike i ka mea hewa me lākou a me ka mea hiki iā lākou ke hana. Inā kānalua ʻoe he AHP kou, hiki i kahi hoʻāʻo genetic ke hōʻoia iā ia. Ke ʻike ʻoe, he kōkua nui ia iā ʻoe e pale aku i nā hoʻouka kaua a kāohi iā lākou.

Nā mea nui e pono ai ʻoe e ʻike (Leka Lawe-Home)

ʻAe, e hōʻuluʻulu wau i kekahi o nā mea e pono ai ʻoe e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai:

• He maʻi hoʻoilina laha ʻole ka AHP e hoʻomaka ana ma ke akepaʻa a hoʻopilikia i ka ʻōnaehana nerve .
• ʻO kēia ma muli o ka nele o nā enzymes e pono ai i ke kaʻina hana o ka heme , kahi e hōʻiliʻili ai i nā mea ʻawahia i kapa ʻia ʻo porphyrin precursors i loko o ke kino.
• Hiki ke ʻokoʻa nā hōʻailona, ​​mai ka ʻeha ʻōpū nui ʻole, ka ʻeha aʻalolo, nā pilikia noʻonoʻo, nā pilikia ʻili, a me nā loli i ke kala o kou mimi.
• ʻOiai inā loaʻa ka loli ʻana o ka genetic, ʻaʻole pinepine e ʻike ʻia nā hōʻailona me ka ʻole o nā mea hoʻoulu ( e like me nā hormones, kekahi mau lāʻau lapaʻau, ka waiʻona, a me ke kaumaha).
• He mea nui nā hoʻāʻo mimi a me nā hoʻāʻo genetic no ka ʻike ʻana.
• ʻO ka mea nui, ʻo ia ke kāohi a pale aku i nā hoʻouka ʻana ma o ka lāʻau lapaʻau. Hoʻohana ʻia ka lāʻau pale ʻo 'Hemin', nā lāʻau ʻeha, a me kekahi mau lāʻau lapaʻau kūikawā. I nā hihia koʻikoʻi loa, he hopena nō hoʻi ka hoʻoili ʻana i ke akepaʻa.
• Hiki iā ʻoe ke ola maikaʻi me ka maʻi ma ka pale ʻana i nā mea hoʻonāukiuki, ke alakaʻi ʻana i kahi nohona olakino, a me ka loaʻa ʻana o nā hoʻokolohua lapaʻau maʻamau .

Manaʻolana wau he kōkua kēia ʻike iā ʻoe. E hoʻomanaʻo, inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona, ​​mai makaʻu e ʻike i ke kauka a kamaʻilio e pili ana.

ʻO ka porphyria hepatic koʻikoʻi, AHP, akepaʻa, ʻōnaehana nerve, nā maʻi genetic, nā porphyrins, nā hōʻailona, ​​​​​​lāʻau lapaʻau