Chakuchepa kwa alpha-1-antitrypsin: zizindikiro osiyanasiyana, matenda ndi mankhwala

Chakuchepa kwa alpha-1-antitrypsin - matenda a chikhalidwe titadziwa, opatsirana pa mlingo majini, ndi pakati pa mwanayo. Zizindikiro kuyamba kuonekera m'masiku oyambirira a moyo wamwanayo. Ndi zizindikiro matenda akukhala kwambiri osiyanasiyana, ndipo alili pang'onopang'ono worsens pa nthawi. Odwala kwambiri, madokotala oterewa mawonekedwe m'mapapo mwanga matenda. 12% odwala zizindikiro za minofu chiwindi. mankhwala amakono alibe njira yeniyeni kuchiza matenda. Choncho, muyeso waukulu ayenera umalimbana liwiro kupitilira matenda.

Kodi Alpha-1 antitrypsin?

mapuloteni izi, zomwe ziri udindo yopanga maselo chiwindi. Kuyambira pamenepo, izo akulowa m'magazi, kenako kusamutsidwa m'mapapo. N'chifukwa chiyani tiyenera mapuloteni izi? Ntchito yake yaikulu ndi kuteteza m'mapapo minofu zisaonongeke ndi kawambidwe kena mapuloteni - michere. zinthu zimenezi ndi zofunika uziyenda wabwinobwino ndi chitukuko cha lake lonse. Mwachitsanzo, proteases ali ndi udindo kuchotsa mabakiteriya ku mapapo, kuthandiza kulimbana ndi matenda. Pamene anamasulidwa mu lalikulu zedi, iwo amathandiza kuti chitetezo cha alveoli kuchokera kukhudzana ndi utsi fodya. Ntchito ya michere imeneyi tiziligwirizanitsa. Mu magawo chochitika kuonjezera elements m'mapapo mwanga kuyamba kusungunuka. Alpha-1 antitrypsin amalola kusintha ntchito za protease enzyme.

Kodi kuchepekedwa?

Mu selo limodzi phata lili DNA 46 okha. Onse a iwo anawagawa awiriawiri 23. Mugulu khromozomu wina tinatengera kwa atate, ena - kuchokera kwa amake. Ndi dongosolo filamentary. Nayenso jini iliyonse imakhala ndi ndondomeko yoyenera ya deoxyribonucleic acid ndi ali ndi malo apadera khromozomu lapansi. Komanso, asaaonerera pa ntchito kapena kuchita ntchito zawo mu thupi. DNA ndipamene anthu zinthu chibadwa.

Insufficiency Alpha-1-antitrypsin jini limodzi ndi kuwonongeka kwa khromozomu №14. Ngati mwendo mapuloteni si opangidwa zedi chofunika, aumbike kuchepekedwa. mankhwala uyikidwa mu chiwindi ndi samalowa mu minofu ya m'mapapo. kuphwanya amenewa kumatanthauza kuwonongeka kwa alveoli ndi ndiko kumayambitsa chitukuko. Yotsalira mapuloteni mu chiwindi facilitates mapangidwe minofu yovulazidwa. Chifukwa amayamba kulephera chiwindi.

limagwirira matenda HIV

Choyamba pathologies yodziwika ndi kupanda alpha-1-antitrypsin, amanenedwa mu 1963 ndi Chidanishi dokotala Wall Erickson. Lero amawapeza kawirikawiri. Komabe, chiwerengero cha odwala kumawonjezera mu kupitilira geometrical. Chifukwa chiyani? nkhani zonse mu chikhalidwe majini a matenda. Matendawa tinatengera pamene makolo onse kunyamula jini nthenda. Malinga ndi kafukufuku, 1 munthu 25, chotero chilema amawapeza. Ngati mwamuna ndi mkazi wa anyamata ndi jini zosalongosoka kusankha pakati mwana, ndi Mwina mkulu wa kuwala adzakhala mwana zosafunikira. Pakali pano, kwa anthu onse 5000 pali wina ndi kukula chithunzi zonse matenda yekha. Zaka zingapo zapitazo, Chiwerengerochi nthaŵi pansipa. anthu 20 zikwi anakumana ndi chimodzi chokha zosalongosoka mbewu. Zowerengera zimenezi zikusonyeza kuchuluka kwa kuchuluka pathologies.

Zizindikiro ndi zizindikiro za matenda oyamba

Chithunzi matenda a ndondomeko pathological makamaka anatsimikiza ndi mawonekedwe ake ndi mmene chotupa cha. Kwa anthu ambiri, zizindikiro alibe zimapezeka kwa zaka zambiri. Ena M'malo mwake, ndi kudwala amakula mwamsanga. Zimenezi okhala zinthu moyo wa wodwala ndi moyo wake.

mawonetseredwe m'mapapo mwanga ndi matenda ambiri. Osuta iwo kuonekera patatha zaka 20. Odwala amene amatsatira moyo wathanzi, pakhomo izi kuposa pafupifupi maulendo 2 (zaka 40-45). Choyamba, pali kufupika kwa mpweya pambuyo ntchito kwambiri. Pang'onopang'ono akutitsogolera kuti kuwonongeka kwa ndondomeko kupuma. Ndipo amayamba chifuwa. matenda pafupipafupi yodziwika ndi zotupa yotupa wa kapepalako chapamwamba kupuma.

Chakuchepa kwa alpha-1-antitrypsin ana, kapena m'malo ake zizindikiro chiwindi kuonekera mu masiku oyamba a moyo. Iwo zimapezeka mu mawonekedwe a chiwindi a etiology wosadziwika, matenda a chikasu ndiyo wa khungu. Ndi zaka 14-16 ambiri a milandu matenda anatsimikiza malangizo a kuchepetsa zizindikiro kwa chiwindi. Mu ena a achinyamata opulumutsidwa nthawi zina ululu kulongolola mu hypochondrium bwino. Maonekedwe zizindikiro kwa chiwindi mu chiopsezo wamkulu matendawo osauka. A chimodzimodzi amakhala ndi amamuyambitsa timabala a minofu limba, amene kenako kumayambitsa matenda enaake aakulu ndi imfa ya munthu.

matenda masiyanidwe

Mapuloteni alpha-1-antitrypsin amagwira ntchito yofunika kwambiri mu ntchito kwa chamoyo wonse. kulephera kwake limodzi ndi mavuto aakulu, ndipo nthawizina kumabweretsa imfa ya munthu. Ngozi ya matenda kuchuluka monga:

• kusuta anthu ndi ndiko kumayambitsa anaonekera kwa zaka 45;
• odwala panniculitis;
• sanali osuta amene ndiko kumayambitsa matenda pambuyo zaka 45;
• makanda ndi matenda a chikasu ndiyo kwambiri ndi okwera chiwindi michere;
• Odwala bronchiectasis wa etiology osadziwika.

Pakuti matenda masiyanidwe wodwala ayenera kupereka magazi alpha-1 antitrypsin. Ngati kuwunika chinatsimikizira akusowa zakudya zomanga thupi, aliyense wa banja la wodwalayo ayenera kukumana ndi mankhwala Kupenda zonse kudziwa jini zosalongosoka. zinthu zina zonse diagnostics umalimbana kudziwa mlingo wa kuwonongeka minofu. Nthawi muyenera kuti ndisanabadwe kuti:

• potaya moyo m'mapapo voliyumu ndi mlingo kupuma;
• tanthauzo la spirometry;
• MAGAZI pa mayesero ntchito chiwindi.

Malangizo chithandizo

Ndiko kumayambitsa amakwiya inasinthiratu. Choncho, mankhwala alionse Akutsatira si kubwezeretsa kapangidwe m'mapapo kapena kusintha ntchito zawo, koma chingaimitse kupitirira kwa matenda. Mankhwalawa ndi okwera mtengo kwambiri ndipo okha akulimbikitsidwa odwala kosasuta amene ali aakulu amtengo limba kukanika. Timauzidwa kuti ngati Kupenda Alpha-1 antitrypsin uli mkati malire m'munsi.

Kusiya kusuta fodya, kugwiritsa ntchito bronchodilators - zigawo zikuluzikulu zofunika mankhwala. Experimental mankhwala ntchito asidi phenylbutyric, amene amalola kudzatunga mapangidwe wosweka mapulotini mu hepatocytes akadali pansi chitukuko. odwala ambiri wamng'ono kuposa zaka 60, madokotala amati m'mapapo kupatsidwa zina. Information za ubwino opaleshoni kuchepetsa voliyumu thupi ndiko kumayambitsa zimatsutsana kwambiri.

Chithandizo cha matenda chiwindi ndi symptomatic. M'malo michere mu ambiri a milandu ndi operewera, kuyambira matenda chifukwa ndondomeko pathological. Odwala ndi kulephera chiwindi wasonyeza kupatsidwa zina chiwalo. Zochizira corticosteroids panniculitis ntchito ndi tetracyclines.

kaonedwe

Kodi mungayembekezere odwala ndi matenda a "akusowa wa alpha-1-antitrypsin"? Chithandizo cha matenda amatenda yekha thandizo la alili. kugonjetsa kwathunthu kudwala zimenezi sizingatheke. Kuti madokotala ananena zawo zimadalira mlingo wa kuwonongeka limba. Nthawi makamaka kwambiri, ndi zovuta. Anthu amafa ndiko kumayambitsa kapena matenda enaake.

Asayansi padziko lonse kupitiriza mwachangu kufufuza matenda, yang'anani kwa mankhwala chilengedwe. Mwachitsanzo, mu US ndi mayiko ena a ku Ulaya, wodwalayo musanayambe mankhwala experimental zoperekedwa kwa pobwezera kusanthula kutsimikizira matenda. Alpha-1-antitrypsin linachokera ku madzi a m'magazi a anthu athanzi mwamtheradi, chikuyendetsedwera m'nsinga. Chithandizo ikuchitika pa ziwembu ndi mwapadera. Ndondomeko otere mobwerezabwereza kangapo pa mwezi. Njira imeneyi mankhwala okwera mtengo ndi proactive. Komanso, si abwino kwa odwala ndi mawonetseredwe kwa chiwindi matenda, pamene mu ndondomeko pathological nawo si kophweka. Zikatero, chithandizo ndi kuchotsa zizindikiro za matenda.

zodzitetezera

Specific njira zopewera matenda, mankhwala amakono angapereke. Madokotala amalangiza kuti akukonzekera pakati mwana, kupezeka upo chibadwa. Zimenezi ndi zoona makamaka kwa makolo, amene kale jini zosalongosoka.

kuti adzimvera

Matendawa ali ndi makhalidwe kupanda protease m'mapapo mwanga ndipo kudzikundikira mu chiwindi pathological antitrypsin, ndi n'chosoŵa. Komabe, chaka chilichonse chiwerengero cha odwala ndi zizindikiro kumawonjezera exponentially. Mwatsoka, kupewa chitukuko sizingatheke.