Ukuntula alpha-1-antitrypsin: izimpawu, izimbangela, ukuxilongwa kanye nokwelashwa

Ukuntula alpha-1-antitrypsin - isifo yesimo esingokwemvelo, ngocansi ezakhini zofuzo, nge akhulelwe ingane. Izimpawu ziqala ukuvela ezinsukwini zokuqala zokuphila komntwana. Nge izimpawu zokwelapha ziya ngokuya wehlukile, futhi isimo lesiguli kancane kancane siya siba sibi ngokuhamba kwesikhathi. In iziguli eziningi, odokotela ukuxilonga ifomu yamaphaphu izifo. 12% kuphela eziguli izimpawu izicubu isibindi. imithi yesimanje akanawo izindlela eziqondile ukuze ukwelapha izifo. Ngakho-ke, esilinganisweni esikhulu kufanele okuhloswe ngazo ukwehlisa ukuqhubekela phambili kwesifo.

Kuyini Alpha-1 antitrypsin?

Lokhu amaprotheni, okuyinto unomthwalo nokukhiqizwa kwamangqamuzana isibindi. Kusuka lapho, lingena emgudwini wegazi, bese uyiswa emaphashini. Kungani siyidinga le amaprotheni? umsebenzi wayo lomkhulu ukuvikela wamaphaphu izicubu ekulimaleni kwezinye izinhlobo amaprotheni - enzyme. Lezi izinto ezidingekayo ukuze ukusebenza evamile nokuthuthukiswa lonke umzimba. Ngokwesibonelo, proteases banesibopho nokususa amagciwane ovela emaphashini, ekusizeni ukulwa nesifo. Lapho ngidedelwa ejele izinto eziningi ezinkulu, nabo nomthelela lokuvikeleka kwezifiso ezisemqoka ezingumphumela alveoli nokusondelana lentuthu kagwayi. Umsebenzi la ma-enzyme kumelwe silinganiswe. Ngo nemingcele umcimbi ukwandisa izakhi yamaphaphu uqale ancibilike. Alpha-1 antitrypsin ikuvumela ukulungisa umsebenzi enzyme protease.

kuyinto kusilela kanjani?

Kokunye iseli nucleus iqukethe Ama-chromosomes e-46 kuphela. Bonke zihlukaniswe 23 ngazimbili. Ngo pair ngamunye, omunye chromosome ngofuzo uyise, elinye - kusukela kumama. Kuyinto isakhiwo filamentary. Ngakwelinye ihlangothi, isakhi ngasinye siqukethe ukulandelana ezithile deoxyribonucleic acid futhi ithatha indawo eqondile e-chromosome. Iphinde elawula umsebenzi othile noma enze umsebenzi wabo siqu emzimbeni. DNA yakha impahla zofuzo zomuntu.

Esingamahlalakhona Alpha-1-antitrypsin isakhi esithile sisebenza ephelezelwa umonakalo-chromosome №14. Uma ukuphindaphindeka amaprotheni akuyona akhiqizwa izinto eziningi kudingeka, yakheke kusilela. Le into is zibekwe isibindi Asingeni nezicubu wamaphaphu. ukuhlukumeza okunjalo akuhileli ukulimala ukuthuthukiswa alveoli kanye ne-emphysema. amaprotheni asalayo esibindini kusiza kumiswa isibanda izicubu. Ngenxa yalokho asathuthuka yokwehluleka kwesibindi.

yizifo indlela

pathologies Okokuqala libhekene yokuntuleka-alpha-1-antitrypsin, achazwa 1963 udokotela Danish Odongeni Erickson. Namuhla-ke ukuthi unesifo kakhulu kuyaqabukela. Nokho, inani leziguli kwandisa e umkhuba oqhubekayo wokubuka yejeyomethri. Kungani? Zonke daba ubunjalo zofuzo izifo. Lesi sifo Sitholakala lapho bobabili abazali benalesi sakhi sofuzo ongaphilile. Ngokwezibalo, mayelana 1 umuntu 25, isici onjalo sitholakale. Uma abangane ababili wobulili obuhlukile esakhini sofuzo engaphelele anqume ukuba akhulelwe ingane, nge awokuthi ukukhanya kuyoba yinto ingane engenampilo. Kuze kube manje, kuye wonke abantu 5000 kukhona ukuthuthukiswa isithombe esiphelele ukwelashwa kuphela. Vele emashumini ambalwa eminyaka edlule, lezi zibalo ngezikhathi ngezansi. Abantu abayizinkulungwane 20 wahlangana isakhi sofuzo esinephutha eyodwa kuphela. Lezi izibalo afanekise izinga egcwele ekwandeni pathologies.

Izimpawu kanye izimpawu zesifo lokuqala

Isithombe basemtholampilo inqubo sokugembula buvezwa ezakheke nezinga lesion. Kubantu abaningi, izimpawu musa azibonakali amashumi eminyaka. Abanye, kunalokho, i-pathology iqhubeka ngokushesha. Le nqubo ibizwa sihleli izimo ekuphileni lesiguli kanye indlela yakhe yokuphila.

ukubonakaliswa yamaphaphu zingezinye zezifo ezivame kakhulu. Abantu ababhemayo zivela emva kweminyaka 20. Iziguli abanamathela ngendlela enempilo, lokhu embundwini weqiwe cishe 2 izikhathi (iminyaka 40-45). Okokuqala, kukhona iphika emva umsebenzi okukhulu. Kancane kancane kuholela Ukuwohloka inqubo zokuphefumula. Khona-ke ijoyina ukukhwehlela. Lesi sifo njalo libhekene izilonda sokuvuvukala engenhla ipheshana zokuphefumula.

Ukuntula alpha-1-antitrypsin ezinganeni, noma kunalokho izimpawu zakhe isibindi avele ezinsukwini zokuqala zokuphila. Bona azibonakalisa ifomu kwesibindi of omsakazo ezingaziwa, jaundice kwesikhumba. Ngeminyaka 14-16 iningi amacala zokwelapha ukuxazululwa bebheke ukunciphisa izimpawu hepatic. Kwezinye izindawo intsha ukulondolozwa izinhlungu ukubelesela ngezikhathi ezithile kule hypochondrium kwesokudla. Ukubukeka izimpawu hepatic ebungozini omdala yezinkinga asathuthuka. A isibonelo esifanayo libhekene nezibazi ijwabu sitho, kamuva kuholela isifo sokusha ezinzima futhi ukufa yomuntu.

ukuxilongwa umehluko

Amaphrotheni-alpha-1-antitrypsin linendima ebalulekile ekusebenzeni wonke umzimba. ukuhluleka kwakhe kuhambisana eziyingozi, futhi ngezinye izikhathi kuholela ukufa lomuntu. I kwengozi isifo zihlanganisa:

• ukubhema abantu emphysema, wabonakala iminyaka engu-45;
• iziguli ezine panniculitis;
• non-ababhemayo abanale emphysema kutholakala ukuthi uphethwe yini emva kweminyaka 45;
• izinsana ne-jaundice ezinzima kanye enzyme isibindi ephakeme;
• Iziguli bronchiectasis of omsakazo engaziwa.

Ukuze ukuxilongwa umehluko isiguli kumele anikeze igazi alpha-1 antitrypsin. Uma ukuhlaziywa kuqinisekisa ukuntula amaprotheni, ilunga lomndeni isiguli ngasinye kudingeka ahlolwe ngokugcwele yezokwelapha ukuthola isakhi sofuzo engaphelele. Zonke ezinye imisebenzi ukuhlola kunqophe ukulinganisa izinga lomonakalo izicubu. Ngezikhathi, udinga i-:

• ekuhloleni ukuphila wamaphaphu ivolumu Izinga lokuphefumula;
• kwencazelo spirometry;
• ukuhlolwa kwegazi ku umsebenzi ukuhlolwa isibindi.

izinkombandlela ukwelashwa

Emphysema kuphakamisa izinguquko engelapheki. Ngakho-ke, noma ikuphi ukwelapha osetshenziswayo akusho ukubuyisela isakhiwo wamaphaphu noma ncono kusebenta kwato, kodwa ungakwazi ukumisa ukuqhubeka kwesifo. I kwelashwa ngempela eqolo futhi kuphela Kunconywa inani elandayo leziguli ezingebona ukubhema abanale ngokulinganisela ezinzima isitho ukungasebenzi. Lolo thando ungalubonisa ukuthi uma ukuhlaziywa Alpha-1 antitrypsin luphakathi umkhawulo ephansi.

Ukuyeka ukubhema, nokusebenzisa bronchodilators - izingxenye ezibalulekile yokwelapha. ukwelashwa kokulinganisa kusetshenziswa phenylbutyric acid, okuvumela ukudweba kumiswa eziphukile amaprotheni ku hepatocytes namanje ngaphansi ukuthuthukiswa. Iziguli eziningi ezingaphansi kuka eminyakeni engu-60, odokotela basikisela wamaphaphu ukufakelwa. Ulwazi mayelana nezinzuzo ukuhlinzwa ukunciphisa ivolumu umzimba nge-emphysema ziyangqubuzana kakhulu.

Ukwelashwa kwezifo isibindi siyefana. Ukushintshwa enzyme iningi amacala ingaphumeleli, njengoba lesi sifo kubangelwa inqubo sokugembula. Iziguli yokwehluleka kwesibindi kuboniswe kwezitho zomzimba. Ukuze ekwelapheni panniculitis esetshenziswa corticosteroids futhi tetracyclines.

ukuba nombono ongafani ngeke

Okufanele ikulindele lapho ithandana iziguli kwathiwa unesifo "nokuntuleka-alpha-1-antitrypsin"? Ukwelashwa lesi sifo kuhilela kuphela ngempumuzo isimo lesiguli. Ngokuphelele unqobe lo zokugula akunakwenzeka. Onalesi sifo incike izinga lomonakalo isitho. Ezimweni sina ngokukhethekile, ke okungekuhle. Abantu bayafa ngenxa emphysema noma isifo sokusha.

Ososayensi ezivela emhlabeni wonke bayaqhubeka ngenkuthalo ukuhlola lesi sifo, funa umuthi jikelele. Ngokwesibonelo, e-US kanye kwamanye amazwe aseYurophu, isiguli ngaphambi kokuqala ukwelashwa zokuhlola ahlinzekwa ukuze nginikeze ukuhlaziya eqinisekisa isifo. Alpha-1-antitrypsin, etholakala kuyi-plasma yegazi labantu ngokuphelele enempilo, iqondiswa ngemithambo yegazi. Ukwelashwa Kwenziwa kwi izikimu eziklanyelwe. inqubo okunjalo liphindwe izikhathi eziningana ngenyanga. Le ndlela yokwelashwa kuyabiza futhi inkanuko. Ngaphezu kwalokho, asifanele iziguli ukubonakaliswa hepatic lesi sifo, lapho inqubo sokugembula abathintekayo akulula. Ezimweni ezinjalo, kwelashwa ukususa izimpawu zokugula.

izinyathelo

izindlela Specific ekuvimbeleni izifo, ezokwelapha zesimanje banganikeza. Odokotela batusa ukuthi imibhangqwana ehlela okukhulelwa ingane, ukuya zofuzo ukubonisana. Lokhu kuyiqiniso ikakhulukazi abazali, esinye sazo asibiza usunayo i isakhi sofuzo esinephutha.

Kufinyeta

Lesi sifo libhekene yokuntuleka protease yamaphaphu futhi inqwaba kule antitrypsin isibindi sokugembula, kuba abavamile. Nokho, njalo ngonyaka inani leziguli nge izimpawu kwandisa exponentially. Ngeshwa, ukuvimbela ukuthuthukiswa yayo akunakwenzeka.