Ukuntuleka kwe-AAT, noma i-Alpha-1-antitrypsin ukuntuleka, yisimo sezakhi zofuzo ezibangelwa ukungabi nalutho lweprotheni evikelayo, i-Alpha-1-antitrypsin (AAT), evame ukukhiqizwa yisibindi. Ngenxa yokuthi iningi lethu elinamanani alinganayo we-AAT, le phrotheni encane idlala indima ebalulekile ekugcineni umsebenzi ovamile wamaphaphu. Kodwa kulabo abanesifo se-AAT, indaba ithatha isimo esihlukile.
Kubantu abanempilo, amaphaphu aqukethe i-neutrophil elastase, i-enzyme yemvelo ukuthi - ngaphansi kwezimo ezivamile - kusiza amaphaphu aphule amaseli awonakele, amangqamuzana asekhulile namabhaktheriya. Le nqubo ikhuthaza ukuphulukiswa kwezicubu zamaphaphu. Ngeshwa, la ma-enzyme awazi ukuthi uzoyeka nini, futhi ekugcineni ahlasele izicubu zamaphaphu esikhundleni sokuthi aphilise. Yilapho i-AAT ingena khona. Ngokubhubhisa i-enzyme ngaphambi kokuthi ingabangela umonakalo emathangeni amaphaphu enempilo, amaphaphu aqhubeke asebenza ngokujwayelekile. Uma kungekho AAT eyanele, izicubu zamaphaphu zizoqhubeka zibhujiswa ngezinye izikhathi eziholela emphysema.
Ngingayithola Kanjani Ukuntuleka Kwe-AAT?
Uma ingane izalwa, izuza amaqoqo amabili e-AAT, eyodwa evela kumzali ngamunye. Ingane izoba nokuntuleka kwe-AAT kuphela uma kokubili amasethi wegciwane le-AAT engavamile. Uma kuphela igazi le-AAT elingajwayelekile futhi elinye livamile, khona-ke ingane iyoba "othwala" wesifo, kodwa empeleni ngeke ibe nesifo ngokwayo.
Uma zombili izigcawu zezakhi zofuzo zivamile, ingane ngeke ihlushwa lesi sifo, futhi ngeke ibe yithwala.
Uma uthola ukuthi unesifo se-AAT, kubalulekile ukuthi ukhulume nodokotela wakho mayelana nokuba nabanye abantu emndenini wakho bahlolwe lesi sifo, kuhlanganise nezingane zakho.
Uma ungenayo izingane, udokotela wakho angancoma ukuthi ufune ukwelulekwa kwezakhi zofuzo ngaphambi kokwenza leso sinqumo.
Ukuze uthole olunye ulwazi xhumana ne-Alpha-1 Foundation Website noma ushayele ucingo lwazo lwesiguli: 1-800-245-6809.
Izibalo
Ukuntuleka kwe-AAT kuye kwabonakala cishe kuwo wonke umuntu, isiko kanye neqembu lobuzwe. I-American Lung Association ilinganisela ukuthi kunabantu abangaba ngu-100 000 e-United States abazalelwa ukulahlekelwa kwe-AAT. Phakathi kwabaningi balaba bantu, i-AAT emphysema ehlobene nayo ingaba yande kakhulu. Ngaphezu kwalokho, njengoba ukulahlekelwa kwe-AAT kuvame ukuthi kungatholakali noma kungaqondakali kahle, okungenani ama-3% kuwo wonke amacala e-emphysema ahlobene nokuntuleka kwe-AAT ayitholakali.
Emhlabeni wonke, abantu abayizigidi ezingu-116 bangabathwali bokuntuleka kwe-AAT. Kulezi, cishe izigidi ezingu-25 zihlala e-United States. Ngesikhathi abathwali bengenalo ngempela isifo ngokwabo, bangadlulisela lesi sifo ezinganeni zabo. Ngalokhu engqondweni, i-World Health Organization (WHO) incoma ukuthi bonke abantu abane- COPD , kanye nabantu abadala nabasha abane- asthma , bahlolwe ukuntuleka kwe-AAT.
Ingozi yokuthuthukisa i-AAT ehlobene ne-emphysema ekhuphuka kakhulu kubantu ababhema. I-American Lung Association ithi ukubhema akugcini nje kwandisa ingozi yakho yokuphefumula uma unesifo se-AAT, kodwa singanciphisa nokuphila kwakho iminyaka engaba ngu-10.
Izimpawu Nezibonakaliso Zokuntuleka Kwe-AAT
Ngenxa yokuthi ama-emphysema ahlobene okuhlobene ne-AAT ngezinye izikhathi athatha isinyathelo esilandelayo emafomu ahlonishwa kalula a-COPD, kwenziwa umzamo omkhulu phakathi neminyaka ukuthola izindlela zokwahlukanisa. Ngokusho kweChest , izici ezimbili eziyinhloko ze-emphysema ezihlobene nokuntuleka okukhulu kwe-AAT kufanele zihoxise ukuhlaselwa kwe-AAT emphysema.
Okokuqala, izimpawu ze-emphysema kubantu abangenalo i-AAT ngokuvamile akenzeki kuze kube yiminyaka eyishumi noma yesishiyagalombili yokuphila. Ngokusho kwe-American Lung Association, lokhu akulona iqiniso kubantu abane-emphysema ehlobene ne-AAT. Kulaba bantu, ukuqala kwezimpawu kwenzeka kakhulu kakhulu, ngokuvamile phakathi kweminyaka engu-32 kuya ku-41.
Esinye isici esibalulekile esihlukanisa i-AAT emphysema ehlobene ne-non-AAT engalingani okulingana nayo indawo endaweni yamapayipi lapho kuvela khona isifo. Kulabo abane-emphysema ehlobene ne-AAT, lesi sifo sivame kakhulu engxenyeni engezansi yamaphaphu, kuyilapho ku-emphysema engeyona i-AAT ehlobene, lesi sifo sithinta isifunda samaphaphu aphezulu. Zombili lezi zici zingasiza umhlinzeki wakho wokunakekelwa kwezempilo enze ukuxilongwa okunembile.
Izimpawu ezivamile kakhulu ze-AAT ukuphelelwa amandla kwe-emphysema yizi:
- Dyspnea
- Ukushisa
- Ukukhwehlela okungapheli nokukhiqizwa kwe-mucus
- Izikhukhula eziphindaphindiwe
- I-Jaundice
- Ukuvuvukala kwesisu noma imilenze
- Ukunciphisa ukuvivinya umzimba
- I-asthma engaphenduliyo noma i-round-round allergies
- Izinkinga ezingabonakali zesibindi noma izinyimba ezinamandla zesibindi
- Bronchiectasis
Ukuxilongwa nokuhlolwa
Ukuhlolwa kwegazi okulula kungatshela udokotela wakho uma unesifo se-AAT. Ngenxa yokubaluleka kokuxilongwa kokuqala, i-University of South Carolina iye yaqala uhlelo, ngosizo lwe-Alpha-1 Foundation, evumela ukuhlolwa okukhululekile, okuyimfihlo kulabo abasengozini yesifo.
Ukuxilongwa okusheshayo kubalulekile ngoba ukuyeka ukubhema nokuphathwa kwangaphambili kungasiza ekunciphiseni ukuqhuma kwe-AAT emphysema.
Ukuze uthole olunye ulwazi mayelana nokuhlolwa, sicela uthintane ne-Alpha-1 Registry Research ku-Medical University yaseNingizimu Carolina ngo-1-877-886-2383 noma vakashela i-Alpha-1 Foundation.
Funda kabanzi mayelana nokuthi ukuhlolwa kwesifo se-AAT kwenziwa kanjani nokuthi ubani okufanele ahlolwe.
Ukwelashwa kokuntuleka kwe-AAT
Kubantu abaye baqala ukukhombisa izimpawu ze-AAT ehlobene ne-emphysema, ukwelashwa esikhundleni (ukukhushulwa), kungaba ukhetho lwezokwelapha olungasiza ukuvikela amaphaphu ngokumelene ne-enzyme ebulalayo, i-neutrophil elastase.
Ukwelapha okufakelwayo kubandakanya ukunikeza ifomu eligxile le-AAT elitholakala eplasma yomuntu. Iphakamisa izinga le-AAT egazini legazi. Uma usuqala ukwelapha okunye esikhundleni, kufanele ube nemithi yokuphila. Lokhu kungenxa yokuthi uma uyeka, amaphaphu akho azobuyela ezingeni lawo langaphambilini lokungasebenzi futhi i-neutrophil elastase izoqala futhi ukubhubhisa amathishu akho emaphaphu.
Akukona kuphela ukuthi ukwelashwa okuthintekayo kunciphisa ukulahlekelwa komsebenzi wamaphaphu kubantu abane-emphysema ehlobene ne-AAT, kodwa kungasiza ekunciphiseni imvamisa yezifo zamaphaphu. Esicwaningweni esashicilelwe e- Chest , ukwelashwa okuphindaphindiwe kuboniswe ukuba nobuhlobo obuqinile nokunciphisa okuphawulekayo emvuthwandaba kanye nobukhulu bezinsana zamaphaphu ezihlobene ne-AAT ehlobene ne-emphysema. Ucwaningo lwaphetha ngokuthi ngenkathi i-emphysema isalokhu ingenakuphikisana, izifo ezincane kakhulu, izifo ezincane zamapayipi zingasiza ekunciphiseni ukuqhubekela phambili kwe-AAT emphysema ehlobene nokuholela emgangathweni wokuphila ophakeme.
Ukuze uthole ulwazi oluthe xaxa mayelana nokuntuleka kwe-AAT noma ukwelashwa okunye, thintana nomhlinzeki wakho wezempilo noma uvakashele i-Alpha-1 Foundation.
Imithombo:
I-American Lung Association. I-Alpha-1 ehlobene ne-Emphysema http://www.lungusa.org. Novemba, 2006.
I-American Thoracic Society. Isitatimende se-American Thoracic Society / European Respiratory Society: Amazinga okuHlola nokuPhathwa kwabantu ngabanye nge-Alpha-1 Antitrypsin Ukuntuleka ". ATS: Disemba 2002. ERS: February, 2003.
I-Alpha-1 Isisekelo. https://www.alpha1.org/
U-Lieberman, J. "Ukwelapha Ukwenyuka Kwegazi Kunciphisa Ukuvama Kwezifo Zama-Lung ku-Antitrypsin Ukuntuleka: I-Hypothesis Entsha Ngokusekela Idatha". I-Chest 2000; 118; 1480-1485.
I-Stoller, J., MD, FCCP. "Izici zezokwelapha kanye nomlando wemvelo we-Alpha-1 Antitrypsin". I-Chest 1997; 111: 123S-128S.