Ukuntuleka kwe-AAT, okanye u-Alpha-1-antitrypsin ukusilela, yimeko yokuzalwa yemvelo ebangelwa ukungabikho kwiprotheni yokukhusela, i-Alpha-1-antitrypsin (AAT), evame ukuveliswa ngesibindi. Ngenxa yobuninzi bethu abanemali eyaneleyo ye-AAT, le proteinyini inendima ebalulekileyo ekugcineni umsebenzi oqhelekileyo wemiphunga. Kodwa kulabo abanokwanela kwe-AAT, ibali lithatha imeko eyahlukileyo.
Abantu abaneempilweni, imiphunga iqukethe i-neutrophil elastase, inzyme yemvelo e-phantsi kweemeko eziqhelekileyo-inceda imiphunga igawula izonakaliso ezonakalisiweyo, iintsholongwane eziguga kunye neebhaktheriya. Le nkqubo ikhuthaza ukuphulukiswa kwamathambo emiphunga. Ngelishwa, la ma-enzyme awaziyo ukuba amise nini, kwaye ekugqibeleni ahlasele amathambo emiphunga endaweni yokunceda ukuphilisa. Yilapho i-AAT ingena khona. Ngokutshabalalisa i-enzyme ngaphambi kokuba ibangele umonakalo kwimisipha yamaphaphu enempilo, imiphunga iyaqhubeka isebenza ngokuqhelekileyo. Xa kungekho AAT ngokwaneleyo, izicubu zomphunga ziya kuqhubeka zichithwa ngamanye amaxesha zikhokelela ekumphysema.
Ndingayifumana njani ifa le-AAT?
Xa umntwana ezalwa, uzuza ilifa ezimbini zee-AAT zegciwane, enye evela kumzali ngamnye. Umntwana uya kuba ne-AAT engenako kuphela ukuba iisethi zee-AAT zegciwane aziqhelekanga. Ukuba kuphela i-AAT yegciwane engavamile kwaye enye iyinto eqhelekileyo, ngoko umntwana uya kuba "ngumphathiswa" wesifo, kodwa akayi kuba nesifo ngokwalo.
Ukuba zombini iisethi zeendiza ziqhelekile, ngoko umntwana akayi kuhlushwa ngesifo, kwaye akayi kuba ngumthwali.
Ukuba uye wafumanisa ukuba unesifo se-AAT, kubalulekile ukuba uthethe nodokotela wakho malunga nokuba nabanye abantu kwiintsapho zakho bavavanyelwa eso sifo, kuquka nabantwana bakho.
Ukuba awunabo abantwana, ugqirha wakho unokuncoma ukuba ufune ukululekwa ngezofuzo ngaphambi kokuba wenze eso sigqibo.
Ukuze uthole olunye ulwazi qha ga mshelana ne-Alpha-1 Foundation Website okanye ushayele umnxeba wesigulane: 1-800-245-6809.
Izibalo
Ukunqongophala kwe-AAT kuye kwafunyanwa kuwo wonke umntu, inkcubeko kunye neentlanga. I-American Lung Association iqikelela ukuba kukho abantu abangama-100 000 e-United States abazalelwe nge-AAT. Phakathi kwabaninzi bala bantu, i-AAT ehlobene ne-emphysema inokuthi ixhaphake. Ukongezelela koko, ekubeni u-AAT unqongophala udlalwa okanye awufumani kakuhle, amaninzi ama-3% kuwo onke amacala e-emphysema anxulumene nokusilela kwe-AAT ayifumananga.
Ehlabathini lonke, abantu abayizigidi ezili-116 bathwala ubunzima be-AAT. Kulezi zigidi ezingama-25 zihlala eUnited States. Ngelixa abathwali bengenayo isifo ngokwabo, banokudlulisela eso sifo kubantwana babo. Ngaloo ngqondweni, i-World Health Organisation (WHO) incomela ukuba bonke abantu abaneCOPD , kunye nabantu abadala kunye nabaselula abane- asthma , bavavanywe ukusilela kwe-AAT.
Umngcipheko wokuphuhlisa i-AAT enxulumene ne-emphysema ngokunyuka kakhulu kubantu abashushu. I-American Lung Association ithi ukutshaya kungekhona nje kwandisa umngcipheko we-emphysema xa unesifo se-AAT, kodwa sinokunciphisa nokuphila kwakho iminyaka engama-10.
Iimpawu kunye nezibonakaliso zokunqongophala kwe-AAT
Ngenxa yokuba i-AAT enxulumene ne-emphysema ngezinye iinkhathi ithatha isithuba esilandelayo kwiifom zeCOPD, iinjongo ezininzi zenziwe malunga neminyaka ukufumana iindlela zokwahlula. Ngokutsho kweChest , iimbali ezimbini ezibalulekileyo ze-emphysema ezinxulumene nokulahleka kwe-AAT enzima kufuneka zikholise ukukhankanywa kwe-AAT emphysema.
Okokuqala, iimpawu ze-emphysema kubantu abangenalo i-AAT bavame ukuba zenzeke kude kube yintandathu yeshumi okanye yeshumi leminyaka yobomi. Ngokwe-American Lung Association, oku akunjalo kubantu abane-AAT ehlobene ne-emphysema. Kuba bantu, ukuqala kweempawu kubakho kakhulu ngaphambili, kaninzi phakathi kweminyaka eyi-32 ukuya ku-41.
Enye into ebalulekileyo ehlula i-AAT enxulumene ne-emphysema ephuma kwi-non-AAT engekho mlingane ongeyena mnye ummandla kwimipuphu apho isifo sifana khona. Kulabo abane-emphysema ene-AAT engenako ukukhubazeka, eso sifo sixhaphake kakhulu kwingxenyana engaphantsi kwemiphunga, kanti kwi-emphysema engahambelani ne-AAT, lesi sifo sichaphazela ummandla wamaphaphu angaphezulu. Zomibini zale mpawu zinokukunceda umboneleli wakho wezempilo ukuba enze uvavanyo oluchanekileyo.
Iimpawu eziqhelekileyo kunye neempawu ze-AAT ukukhubazeka kwe-emphysema zi:
- Dyspnea
- Ukugubha
- Ukukhwehlela okungapheliyo kunye nokwanda kwemveliso ye-mucus
- Iifubho eziphinda zikhukhulayo
- Jaundice
- Ukuvuvuka kwesisu okanye imilenze
- Ukunyamezela ukunyamezela
- I-asthma okanye i-round-round allergies
- Ingxaki ezingabonakaliyo zesibindi okanye i- enzyme yesibindi ephakamileyo
- Bronchiectasis
Ukuxilongwa novavanyo
Uvavanyo olulula lwegazi lunokuxelela ugqirha ukuba unako ukukhubazeka kwe-AAT. Ngenxa yokubaluleka kokuxilongwa kwangaphambili, iYunivesithi yeSouth Carolina iye yaphuhlisa inkqubo, ngoncedo lwe-Alpha-1 Foundation, evumela ukuvavanywa okukhululekile, okuyimfihlo kwabo basengozini yesifo.
Ukuxilongwa kwangaphambili kubalulekile kuba ukuyeka ukutshaya kunye nokunyango kwangaphambili kunokuncedisa ukunyuka kwe-AAT emphysema enxulumene ne-AAT.
Ukufumana ulwazi oluthe xaxa malunga nokuvavanya, nceda uqhagamshelane ne-Alpha-1 yoRejista yoPhando kwiYunivesithi yezoLimo yaseSouth Carolina ngo-1-877-886-2383 okanye u tyelele iSiseko se-Alpha-1.
Funda ngokubanzi malunga nendlela ukuxilongwa kwe-AAT engenakwenziwa ngayo kwaye ngubani omele ahlolwe.
Unyango lwe-AAT
Kubantu abaye baqala ukubonakalisa iimpawu ze-AAT ezinxulumene ne-emphysema, ukutshintshwa (ukunyuswa kwamayeza) kwindlela yokwenza unyango onokukunceda ukukhusela imiphunga ngokumelene ne-enzyme ewonakalisayo, i-neutrophil elastase.
Ukuguqulwa kwamathambo kubandakanya ukunika ifom ye-AAT ejoliswe kwi-plasma yabantu. Iphakamisa izinga le-AAT kwigazi. Emva kokuba uqale unyango oluthathe indawo, kufuneka ufumane unyango lobomi. Oku kungenxa yokuba xa uyeka, imiphunga yakho iya kubuya kwinqanaba labo langaphambili le-dysfunction kwaye i-neutrophil elastase iya kuphinda iqalise ukutshabalalisa izicubu zakho zemiphunga.
Ukuphela kwonyango oluthathayo kunokunciphisa ukulahleka komsebenzi wamaphaphu kubantu abane-AAT emphysema enxulumene ne-AAT, kodwa inokukunceda ukunciphisa imvama yezifo zamaphaphu. Kwiphononongo eshicilelwe kwi- Chest , unyango oluthathelweyo lwaboniswa ukuba lunxulumene oluchanekileyo kunye nokunciphisa okukhulu kwimizimba kunye nobukhulu bezonyango ezichaphazelekayo kunye ne-AAT ehlobene ne-emphysema. Uphononongo luphelile ukuba ngelixa i-emphysema ihlala ingavumelekanga, ukungaphantsi kwexesha, izifo ezinzima zamaphaphu zingancedisa ukunyuka kwe-AAT emphysema enxulumene ne-AAT kwaye ikhokelela kumgangatho ophezulu wobomi.
Ukufumana ulwazi oluninzi malunga nokusilela kwe-AAT okanye unyango oluthathe indawo, thetha nomboneleli wakho wezempilo okanye u tyelele iSiseko se-Alpha-1.
Imithombo:
I-American Lung Association. I-Alpha-1 ehlobene ne-Emphysema http://www.lungusa.org. Novemba, 2006.
I-American Thoracic Society. Inkcazo yaseMelika yaseTranski / I-European Respiratory Society Inkcazo: Imigangatho yokuHlola kunye noLawulo lwaBantu ngabanye nge-Alpha-1 Antitrypsin Ukuntula ". I- ATS: Disemba 2002. I-ERS: NgoFebruwari, 2003.
I-Alpha-1 Foundation. https://www.alpha1.org/
ULieberman, J. "Ukunyuswa kwamayeza okunciphisa ukwanda kwama-Lung Infections ku-Antitrypsin Ukusilela: I-Hypothesis entsha kunye neNkxaso-nkcukacha". I-Chest 2000; 118; 1480-1485.
I-Stoller, J., MD, FCCP. Iziganeko zeKliniki kunye neNkcubeko yeNdalo ye-Alpha-1 ye-Antitrypsin Deficiency ". I-Chest 1997; 111: 123S-128S.