Deficiency o Alpha-1-antitrypsin: symptoms, ke kumu,ʻike, a me ka lapaʻau

Deficiency o Alpha-1-antitrypsin - he maʻi o ka innate ano, i hiki i ka aaiaoe pae, me ka hapai ana o ke keiki. Symptoms hoʻomaka e hōʻike i nā lā mua o ko pēpē ka ola. Me ka lapaʻau hoailona e lilo hou like, a me ke ahonui o ke ano i nā worsens ma manawa. Ma ka hapanui o nā mea maʻi, kauka diagnose pulmonary ano o ka maʻi. Wale 12% o nā mea maʻi i nā symptoms o ke akepaʻa'aʻaʻa. Hoikehonua, lapaau Aʻole ole i kekahi mea e pono maʻi. No laila, e ke pahuna i ka papa kuhikuhi ana ma ka meheu o maʻi Holomua.

He aha ka Alpha-1 antitrypsin?

Kēia kumuʻiʻo, i mea kuleana no ka? Iecaianoaaiiie o ke akepaʻa keena. Mai laila, e komo i ke koko, a laila, kaʻi aku i na ia maʻiʻaʻai. No ke aha la makou e pono ai keia kumuʻiʻo? Kona papa kuhikuhiE kuleana pili i mea e hoʻopale akemāmā'aʻaʻa mai ka poho i nāʻano o nā polokina - nā'ūhū. Mau waiwai e pono ai ka mea maʻamau functioning a me ka ulu ana o ka a pau meaola. No ka laʻana, proteases i kuleana no ka wehe koʻohune mai na maʻiʻaʻai, kōkua i ke kaua i ka maʻiʻaʻai. I ka hookuu ae i ka nui na mea, ka mea i hoʻonui i ka hoomalu ana o na alveoli mai ka waiū a hiki i ka paka uwahi. pono e 'O ke kaulike o ka ha awina o kēia nā'ūhū. Ma ka hanana kiko'î e hoomahuahua i pulmonary hehee wale hoʻomaka e hehee. Alpha-1 antitrypsin e leie aku oe e hoʻomaʻa i ka ha awina o ka protease'ūhū.

Pehea o ka nele?

I kekahi aeea nucleus he wale 46 chromosomes. A pau o ia e māheleʻia i loko o 23 mau hui. Ma kēlā me kēia mau, hoʻokahi chromosome ili mai o ka makua kāne, ka mea 'ē aʻe - mai ka makuahine. He He filamentary 'ole. I ka huli, kēlā'ōewe ninoieo o ka manaʻo kaʻina o deoxyribonucleicʻakika a ninoaaeyao i ka henua i loko o ka chromosome. Ua hoi he mau 'papa hoʻi iā a hana lākou mau hana i loko o ke kino. DNA ano he kanaka aaiaoe mea.

Insufficiency Alpha-1-antitrypsin'ōewe ua pu ma ka poho i ka chromosome №14. Inā no ka duplication o ke kumuʻiʻo ua i hua mai i loko o ke koi 'ia mea, ua hana nele. Kēia waiwai a ua waihoia ma ke ake, a aole i komo i loko o ke akemāmā'aʻaʻa. Oia kue entails poino i ka alveoli a me ka emphysema ulu. Koena kumuʻiʻo ma ke akepaʻa kōkua ai i nā ke kahua o ka me ka makaʻu no'aʻaʻa. E like me ka hopena o ka ulu akepaʻa ole.

maʻi EYIʻano papa hana

First pathologies wehewehe aku ma ka nele ana o Alpha-1-antitrypsin, i ho'ākāka 'ia ma 1963 ma ka Denemaka kauka ke Wall Erickson. I kēia ka mea, ua diagnosed loa kākaʻikahi. Naʻe, i ka helu ana o nā mea maʻi maoli i loko o ka geometrical Holomua. No ke aha? A pau i ka mea i loko o ka aaiaoe ano o ka maʻi. I ka maʻi ua ili mai iā ia ka wā nā makua e lawe aku i ka'āno'ē'ōewe. Wahi a ka 'ikepili helu, ma kahi o 1 kanaka 25, e like me ka kina ua diagnosed. Inā ka poe hui mau o ke ku pono anal me ka hemahema'ōewe olelo e hapai i ke keiki, me ka pahiki kiʻekiʻe o ka malamalama, e lilo ia i keiki unhealthy. I ka lā, no ka mea, o kela mea keia 5000 kanaka he mea wale nō kekahi me ka hooulu ana o ka piha lapaʻau kiʻi. Pono he mau makahiki aku nei, i kēia mau kii a pau ma nā manawa ma lalo nei. hālāwai 20 tausani kanaka me kekahi wale hemahema'ōewe. Kēia mau 'ikepili helu kahaki ii ka piha loa o ka hoʻonuiʻana pathologies.

Symptoms a me na hoailona o ka maʻi mua

Ka lapaʻau kiʻi o ka pathological kaʻina ua pili kēia nānā 'ia e kona helehelena a me ka loa o ka lesion. No ka hapanui o kanaka, na symptoms mai e hoike ia lakou iho no ka makahiki. Others, ma ka mea ku ole, ka pathology progresses hikiwawe loa. Kēia kaʻina ua imprinted rula i ke ahonui o ka ola a me konaʻaoʻao o ke ola.

Pulmonary hoike ana i ka loa, he pono ole ka maʻi. Paka ka mea, eʻikeʻia ma hope o 20 makahiki. E hoomanawanui i ka poe i hoopili i ke ola i ke Aloha Hawaii, keia paepae ua oi ma ka aneane 2 manawa (40-45 makahiki). Mua, he mea pokole o ka hanu ma hope o ka, pili hoounauna. Ua i nā hiki aku ai i ka mea hōʻemi o ka naehana kaʻina. A laila, hui cough. Kēia maʻi ua pinepine wehewehe aku ma ka hoʻoʻino lesions o ke keena naehana'āpana.

Deficiency o Alpha-1-antitrypsin i loko o na keiki, a he oiaio, i kona ake symptoms hōʻike i nā lā mua o ke ola. Ka mea, hoike mai ia lakou iho i loko o ka palapala o ka hepatitis o ike etiology, jaundice o ka ili. Ma 14-16 makahiki i ka hapanui o ka lapaʻau hihia hoʻonā i loko o ke ala e hiki aku o minimizing hepatic symptoms. Ma kekahi o na nā pōkiʻi hoolaia occasional nagging eha ma ka hypochondrium akau. Keʻano o ka hepatic symptoms i kanaka makua pilikia o ka ulu complications. A like kumu ua wehewehe aku ma scarring o ka hui,'aʻaʻa, i a laila hiki aku ai i loa cirrhosis a me ka make ana o ke kanaka.

palena pauʻike,

Kumuʻiʻo Alpha-1-antitrypsin Radio nui i kūlana ma ka functioning o ka mea a pau meaola. Kona ui ua pu e kaumaha complications, a me kekahi manawa hele aku ai i ka make ana o ke kanaka. Ka māhuahua pilikia o ka maʻi nā:

• momoku kanaka me ka emphysema, ikea mai la i ka 45 makahiki;
• nā mea maʻi me ka panniculitis;
• ole-paka, ka poe i emphysema diagnosed ma hope o 45 makahiki;
• keiki keiki me ka loa jaundice a me ke kiekie ake nā'ūhū;
• Nā mea maʻi me ka bronchiectasis o ka ike ole etiology.

No ka palena pauʻike, e haawi aku i ke ahonui o ke koko no ka Alpha-1 antitrypsin. Inā ka Ikepili keʻae i ke kumuʻiʻo deficiency, kēlā me kēia lālā o ka ohana o ka hoʻomanawanui Pono e undergo i ka piha 'inikua ninaninau, e huai ae i ka hemahema'ōewe. A pau nā hana lapaʻau 'nona ma hoomaopopo ana i ke degere o ka'aʻaʻa ka pōʻino. Kekahi manawa, e pono e kekahi mea loiloi no:

• ke kālailaiʻana ola akemāmā ka leo a me ka ÷ naehana hanu kōmi;
• ka wehewehena o ka spirometry;
• koko e ho'āʻo ai ma akepaʻa kuleana pili i ho'āʻo.

lapaʻau Nā Kūlana

Emphysema hoʻonāukiukiʻana mai o irreversible loli. No laila, i kekahi pili Inc 'aʻole i hoihoi i ka akemāmā' ole a me ka hoʻoikaika 'ana i kā lākou mau hana, akā, ka mea hiki ke hooki i ka Holomua o ka maʻi. Ka iapaau ana He nō pipiʻi a wale e waiho no ole-momoku e hoomanawanui i ka poe i moderately loa hui, dysfunction. Ua Ua hoike ia ina ka Ikepili o ka Alpha-1 antitrypsin mea i loko o ka lalo palena.

Quitting uiki, hoʻohana a bronchodilators - nui eiiiiiaiou o Inc. Hoʻokolohua lapaʻau hoʻohana 'ana i ka phenylbutyricʻakika, e leie aku, e kikoo aku i ka haʻihaʻi i kahua o nā polokina i loko o nā hepatocytes mea nō ma lalo o? Acaeoey. He nui nā mea maʻi kaikaina ma mua o 60 makahiki, kauka paipai akemāmā transplantation. Information e pili ana i ka pomaikai o kaʻoki kino, e ho'ēmi i ke kino ka leo me ka emphysema mea nui contradictory.

Lapaʻau o ke akepaʻa maʻi mea symptomatic. Hakahaka o nā'ūhū i loko o ka hapa nui o ka hoopii mea ineffective, mahope mai o ka maʻi, kena ae la ia e ka pathological kaʻina. Nā mea maʻi i ake e loaa hōʻike hui, transplantation. No ka iapaau ana o ka panniculitis hoʻohana corticosteroids a me tetracyclines.

Outlook

He aha e manaʻo ai ma nā mea maʻi me kaʻike, o "deficiency o Alpha-1-antitrypsin"? Lapaau ana i ka maʻi komo ai wale i ke kokua ana o ka hoʻomanawanui ka kulana. Loa maluna o keia pathology mea,ʻaʻole hiki. Ke prognosis hilinaʻi nui ma luna o ka degere o ka hui, ka pōʻino. Ma pakahi aku la ia mea hoopii, ia ka noho lakou malaila. People make mai emphysema paha cirrhosis.

Epekema mai a puni ka honua hoʻomau i ka 'Imi makaikai i ka maʻi, nana no i ao lāʻau. No ka laʻana, i loko o ka US a me Europa aina kekahi, i ka hoʻomanawanui ma mua e hoʻomaka ana i ka mana iapaau ana i kaumaha aku ai, e haawi aku ma ka Ikepili e hookupaa aku ana i ka maʻi. Alpha-1-antitrypsin, loko mai o ka wai koko o ka loa ola kanaka, ka mea, ua lawelawe intravenously. Lapaʻau ua lawe mai ma luna o ka pela 'ia manaʻo. Oia ke kaʻina hana no ua hai kekahi mau manawa no ka malama. Kēia hana o ka lapaau mea pipiʻi a me ka proactive. Eia hou kekahi, ia mea e pono ai nā mea maʻi me ka hepatic hoike ana mai o ka maʻi, ka wā i loko o ka pathological kaʻina āpau i komo i mea e maʻalahi. Ma na hoopii, lapaʻau mea e wehe i ka symptoms o ka maʻi.

kāohi ana

I Kekahi Mau maʻi Kāohi epekema, laekahi lapaʻau hiki ke kaumaha. Kauka paipai aku i Leomakana hoʻolalaʻo e hapai i ke keiki, e hele ana i ke kūkā aaiaoe?. He oi loa oiaio o na makua, i kekahi o ka mea kāinoa mua i ka hemahema'ōewe.

e hōʻuluʻulu

I ka maʻi ua wehewehe aku ma ka nele ana o pulmonary protease a me ka hoʻomāhuahua i loko o ke akepaʻa pathological antitrypsin, ia mea he kŘpa a pohihihi keia. Eia naʻe, i kela makahiki i ka helu o nā mea maʻi me ka symptoms maoli he exponentially. Eia naʻe, e ole kona ulu mea,ʻaʻole hiki.