Huʻi intermittent porphyria: symptoms,ʻike, lapaʻau

Aaiaoe maʻi i ka loa a me ka luna 'pilikia, no ka mea, i loa e pohaku uinihepa, no ia mea hiki ole. Kekahi mau maʻi He huʻi intermittent porphyria. Keia ua kena ae la ia e hereditary maʻi ma i ka helu ana o porphyrins i loko o ka piʻi pū koko. Kēiaʻano o ka pathology ua manaoia kekahi o ka loa hanohano i waena o nā mea a pau ano o aaiaoe porphyria.

I loko o ka maʻi he mea i hoʻomāhuahua oʻawahia 'ona i loko o kanaka nā loko. Loa pinepine diagnosed pathology o ka maikai ke keka. A me ka mea hiki ke triggered ma ka hāpai keiki, a hānau.

symptomatology o ka maʻi

Inā he hoomanawanui e hoʻomōhala ana i huʻi intermittent porphyria, symptoms loaʻa:

• Kekahi numbness o ka ili.
• Polyneuritis (mau nā aʻalolo pōʻino).
• Wikiwiki.
• Redness a me ka nani o ka ili.
• Pigmentation a blisters ma luna o kaʻili mau mea i mea maʻamau hamama.
• Microcysts (hoemiia bladder nona iho, kahi kaulana hoʻohuneʻaihue o kona oihana).
• Psychosis.
• Ke aloha pathological loli ma ke akepaʻa e aloha ia kona functionality.
• Anemia.
• Coma.
• Pain i loko o ka abdomen, a me ia mea nui ikaika. ka mea, i hele pu me ia e hou nausea a me ka luaʻi.
• I ka make ana o ke kui una honua.
• E hoomahuahua oe i puʻe.
• urination kāna hana.
• Ka hilahila, uunu nui, hallucinations.
• Lōlō o naehana nāʻiʻo.

It E e hoʻomaopopo i ka mea mua symptoms malama i loko o nā mea maʻi elemakule 20 a hiki i 40 makahiki ma ka palapala o ka ho'āhewa wale. No hoi, ka mea hiki ke hai pinepine a hiki wale i kekahi manawa, i loko o ka wā e ola ana. Inā ka pepehi maʻi e hoʻomōhala ai, ka mea e hōʻike i ka 'aʻe' ana ma ka aoao o ka brainstem, cranial nā aʻalolo (oculomotor a me ka lehalehu t), autonomic NS.

Nā symptoms o porphyria e hoikea mai oia, i nā mea a pau nā moku halihali mokulele. No ka laʻana, 80% o ia hiki loa aku e ike ole aku o ka pilikia. A hiʻona o ka maʻi ho'āhewa wale 'o ia i loko o nā mea a pau kona ano apau loa i loko o ka hoʻomanawanui ka mimi hoʻololi waihoʻoluʻu a me ka lilo a pink, eleele a me ka ulaula.

Huʻi ano o ka porphyria mea pohihihi keia. Symptomatology o ka maʻi ua olelo okoa.

pathology lapaʻau '

Ma ka hihia o ka maʻi e like me huʻi intermittent porphyria,ʻike, E e akahele. Mau hoʻohana keʻena hoʻokolohua ho'āʻo ma o ka mimi a me ke koko, a nana e hoike aku i ke dala o ke porphobilinogen, porphyrin pae i ka wai koko. Inā ma ka liʻiliʻi loa hoʻokahi hāpana ua'āno'ē, ke hoʻomanawanui i ka lanakila i hou ninaninau.

No ka laʻana, ka mea, ua nui ia e ka radiography o ka abdominal lua, a kokua i ka hōʻike ileus. Eia hou, aia nō kekahi PalekanaEND_LINK ana hoʻohālike e kōkua diagnose like pololei i hiki:

1. Iloko o ka hoouka: ka mimi ua loa hoʻomaka koke excreted PBG a me ALA.
2. I loiloi no ka degere o ka ho'ēmiʻana deaminase ha awina hiki ke i i kala.

Ma kekahi hihia, i ka palena pauʻike, e ia i ka maʻi lapaʻau ua maoli ka hoʻokō. Inā keia hookolokolo ana i ua i pono, a me ka hana ua ineffective a me ke ahonui ka iini nui no ka make (60% o nā hihia).

Ua mea i nui ia i hala i nā ho'āʻo ma na lala a pau o ka hoʻomanawanui ka ohana. Kēia e kōkua i loko o ka wā e hiki mai, e pale aku i ke kūpono 'ana o ke porphyria i loko o ke keiki kakou nana.

Pehea o ka maʻi?

Keʻano papa hana o ka? Acaeoey o ka pathology mea he mea. Heme - 'ole-kumuʻiʻo hapa o ka hemoglobin - ma lalo o ka aoao o kekahi aʻe hiki ke hoano hou ana ia i ka lahuiʻawahia waiwai. Ia, ma ka huli,'aʻaiawā hoʻi ke kahakai ka'aʻaʻa ma lalo o kaʻili.

Nolaila, ke uhi hoʻomaka e lilo i mea eleele, thinning. Over manawa, ua uhiia i ka ili o ka hoʻomanawanui me ka eha a me ka ulcers, o ka oi aku ina mea ua paku ole ia i Map. Ka mea i ultraviolet malamalama ke kumu poino, a no laila, palupalu fabrics.

? Ac? Aaioea, ka maʻi kaʻina e loli ai,ʻaʻole wale na epidermis. Kekahi iʻa a cartilaginous nā'aʻaʻa o ka ihu, pepeiao. Maoli ai, ka mea deforms ia. I mea huʻi intermittent porphyria lawe ole wale kino ai akā, i ka noʻonoʻo 'hoomanawanui like me ka ke kanaka hiki ole nana i kona hoinoino maka i loko o ke aniani.

kumu o ka

Huʻi intermittent porphyria - ka maʻi ka luna 'a me ka pilikia. Kumu no kona ulu ana, ua like penei:

1. Aaiaoe predisposition: pathology ua ili mai iā ia.
2. Nui loa ka mämä holo o ka lipid peroxidation.
3. ake maʻi. He oi loa oiaio o hepatitis.
4. Ikaika Nine Inch kaʻapu kōheoheo a paʻakai o ka mea kaumaha ke hooheheeia.
5. Siderosis Kupffer keena.
6. A hailonaʻana o ka hao i loko o ke koko.
7. Hooloihiia hoʻohana o ka mea kaumaha lapaʻauʻia: barbiturates, hormones.
8. Maʻi alcoholism.

He aha kumumea ke hoʻonui i ka pilikia o ka maʻi?

Porphyria (aha ia mea - ua ike no oe, ua) hiki ke triggered e:

• A ke kanana 'ʻai. Ua, pū, mea he nui mea kaumaha ma luna o ke ake, a ua koiia e hana hou ma mua o mau.
• Ka hoʻonaʻauao.
• Loaa ana he nui loa o ka ai '.
• Pinepine hui 'me ka pilikia Nine Inch.
• Hormonal loli aie i ka hāpai keiki a menstruation i loko o na wahine.
• Complex infectious pathologies e like me hepatitis C.
• Excessive hokii o ka waiʻona.

I ka pathology mea,ʻaʻole nui kou manaʻo e pili ana ma, a ma luna o hou, oe wale pono e hoʻopau a pau o nā kumumea helu 'ia ma luna, e like me ka pono me ke ola i ke Aloha Hawaii.

Outlook

Inā ka hoʻomanawanui ua diagnosed me ka huʻi intermittent porphyria, ka prognosis hilinaʻi nui ma luna o ka degere o ke aʻalolo a hāʻule pahū wale. Manaʻoi hoʻokoe 'hoʻokumu pū Inc eliminates symptoms loko 2-4 lā.

Inā ke hoʻomanawanui? Ac? Loa Hawaii neuropathy, alaila, aole e e ole symptoms e nalo no ka mahina a me ka makahiki. I ka mua he kanaka i, ka emi ikepili koʻikoʻi i kumumea i mea maʻi. He ua hoemiia ke alapine (frequency) o ka ho'āhewa wale.

Hiʻona o ka lapaʻau

Inā ka hoʻomanawanui ua diagnosed me ka huʻi intermittent porphyria, i ka iapaau ana pono paka i ka hematologist a me ka endocrinologist. O ka holo ana, Inc E e noniakahi. It nā:

• Plasmapheresis (koko maʻemaʻe o toxins me ke kōkua o kūikawā lako a pan).
• Hoomalu mai pololei Map.
• Splenectomy (ana o ka spleen).
• E hoʻohana i analgesic ai '.
• Ka hoʻohanaʻana i ai 'lakou ma ke koko,.
• E hoʻohana i ka monakō koko pāʻoihana no infusion.
• и "Рибоксин". Ke ano o ka ai 'i pono denozinmonofosfat a "Riboksin".
• E ho ohana i ka hana e hoʻonui ana i ka wehe 'ana o ka ili o porphyrins i loko o ka mimi.

Porphyria (aha mea keia - i ho'ākāka 'ia ma luna) pono i noniakahi kokoke i ka lapaau. Loa Inc noaia hiki e:

1. E hoʻomaka, i ka hoʻomanawanui i ke komo i ka haukapila. Eia ka mea i pono, e mau kanaka hoʻoponopono i ka naau ana, ke koko, a me nā pōʻino hoailona.
2. He nui ke hookaawale ia e pāpā ikaika a pau nā kumumea i kāhea i ka worsening o ka maʻi.
3. Inā ka mau kumu o ka pathological hoi i ole hoi, ua paa kona manao, i ka hoʻomanawanui hiki e ka infusion o ka monakō koko. Naʻe, i kēia hana e kokua wale ina porphyria mea ole ka ikaika hoouka.
4. Ua mea i nui ke koke hoʻomaka ahonui kanaka hoʻohana 'hemin. Hou ana ma keia hihia, e hele mai ma hope o 2-4 kūlana kanaka.
5. Hoʻopau o ka symptoms mea i nui ka hapa o ka iapaau ana kaʻina. He mea pono e hahai i ka inu regimen i ka hoʻomanawanui 'ole e hana dehydration. No kaʻeha Pono paracetamol a narcotic analgesics (kuhikuhi o ke kauka). No ka iapaau ana o ka naau kāna hana, a me ka pilikia a me ke koko, hiki ke hoʻohana Hoʻokolohua-blockers. Me ka maʻiʻaʻai E e hana me ka penicillin paha cephalosporins.

General Mea Maluna

Huʻi intermittent porphyria mea ia mau ano:

1. Pathology kauoha ae la ia e ka deficiency dehydratase.
2. Hereditary coproporphyria.
3. Variegated porphyria.

He aha lapaau hiki ole ai?

Huʻi intermittent porphyria kānāwai ma muli o ai i ka hopena o kekahi ai '. No ia mea, ia mea e pono ke ike i ka ia e ole e lawe no laila, i ole e hoʻonāukiuki i ka hoouka.

No laila, ina he kanaka i Ua huʻi intermittent porphyria, pāpā lāʻau papa inoa mea pono e ike ma ka naau:

• Mau mea pilikia loa ai ', "dapsone", "Danazol," "Difenilgidanton" "Diclofenac", "carbamazepine", "meprobamate," "Novobiocin" "Chloroquine."
• Palena papaha pilikia loa "clonazepam" "Ketamine", "Clonidine", "Nortriptyline", "Rifampicin" "Theophylline" "Erythromycin", "Spironolactone".

maʻi Kāohi

Eia naʻe, kauka hiki ole e kaumaha aku i ka laulā o ke kāohi ana i hiki ole i ka maʻi. Naʻe, i kekahi mau kōkua hiki ke hana no ka pono kākoʻo mai o ke kino.

1. He pono, e haawi i pūnuku puhi a me ka inu ana.
2. He nui ka ai i kela mau ai, e like me ka hapa o ka i pihaʻi me nui dala o nā wikamina, o ka oi aku B pae
3. Hoʻopale ai i kou ili mai ka pololei Map: makaaniani, SafeSearch kapa, pālule a papale wā i komo i ke alanui koi 'ia!
4. E aho e pale a pau o ka poe kumumea i ke kāhea i ke flare.
5. He nui e nīnau aku i ka dietitian, no laila ia i kokua mai, e koho i ka maikaʻi loaʻai.

Aia nō kekahi mau mea kōkua ma koi o kauka i ka hoʻomanawanui pono mau hooko. No ka laʻana, ina he e mau ia i ke kupee, a e ke palapala, i ke kanaka o ka maʻi.

A pau nā hiʻona i hoakaka ia e ka pathology. E hoʻokaʻaʻike ola!