Cystic fibrosis ingane: izimpawu, izimpawu, ukwelashwa

Esinye sezifo obanzi kunayo ukuthi zizuzwe kuyinto cystic fibrosis. Ingane ezivezwa imfihlo pathogenic eholela ukuphazamiseka ipheshana wokugaya ukudla, umgudu wokuphefumula. Akumangalisi lesi sifo singumbulali ezingamahlalakhona futhi ngokuvamile kubonakala, uma bobabili abazali isakhi esithile engaphelele. Uma isayithi nge ngokuguqula izakhi zofuzo eyodwa kuphela usizi olungapheli kubazali, izingane ngeke balizuze ifa sifo. Ngokuvamile, cystic fibrosis sitholakale onyakeni wokuqala wokuphila (noma ngisho esibelethweni).

Umlando isifo

cystic fibrosis ezinganeni eba ngenxa ukuphazamiseka isakhiwo chromosome 7 (ehlombe). Kulokhu, lo isikhwehlela emzimbeni kuba viscous ngaphezulu. Kuyaziwa ukuthi imfihlo yalolu hlobo imbozwe cishe zonke izitho zangaphakathi. Ngenxa yalesi ebusweni oswakanyiswe kubo, nibe ukwehlukanisa, isikhwehlela extra ahlanze ke. Kodwa uma kwenzeka iphahlazeke, le uketshezi viscous stagnates aqoqana. Esimweni esinjalo, nezinto ezincane eziphilayo pathogenic nande, okuholela ukutheleleka unomphela. Ngemva kwalokho kuthinteka izitho wokugaya ukudla, umzimba uhlushwa ukuntuleka komoya-mpilo. Ngokokuqala ngqá ingane cystic fibrosis achazwa 1938 by D. Anderson. Kuze kube yileso sikhathi, inani elikhulu izingane yizifo ezinjenge-pneumonia nezinye izifo ezibangwa-cystic fibrosis. Ulwazi oluthe xaxa ngalesi sifo kwavela kwekhulu lamashumi amabili. Cishe zonke izakhamuzi wamashumi amabini iplanethi yethu - esiphathiswa we-cystic Fibrosis isakhi sofuzo liziguquguqule. Lokhu kungapheleli awahlobene imikhuba emibi abazali ngesikhathi ekhulelwa, nge izidakamizwa. Futhi ku khona layo akuphazamisi isimo okungekuhle semvelo. They jeza kusukela kokubili amantombazane nabafana efanayo.

uhlobo cystic fibrosis

Lesi sifo angase abe Umumo ezintathu zezinhlayiya eziyisisekelo.

• Esokuqala - wamaphaphu. Senzeka cishe 15-20% of the number of zonke izimo. Libhekene iqiniso lokuthi bronchi baba avimbekile izimbici obukhulu. Ngemva kwesikhathi esithile, bayakwazi avimbekile ngokuphelele. Imfihlo yokuba enhle kakhulu yokuzalela umhlabathi bacterium namagciwane. Ngokuhamba kwesikhathi, izicubu wamaphaphu uphawu, ibe lukhuni. Izinhlumba ukuvela. umsebenzi owengeziwe yamaphaphu liwohloka kuphela. Umuntu ungafa kusuka umoya.
• cystic fibrosis, ingane ingase ibe futhi ifomu emathunjini. Kulesi kwaphazamisa wokugaya ukudla, ukudla ayiqondakali kugaywe. isimo esinjalo kubangela ukuthuthukiswa sikashukela, isilonda peptic, isibindi sibe sesomile nokunye. N. Sitholakala 5% of amacala.
• Uhlobo oluvame kakhulu i - elixutshwe. It sitholakale 75% eziguli nale isifo esidluliselwa ngofuzo.
• ezingavamile kakhulu (1%) kwenzeka, kanye nefomu atypical.

cystic fibrosis kwamaphaphu ezinganeni

Le ndlela lesi sifo ngokuvamile aye abizwa ngokuthi zokuphefumula. Ngokuvamile, izimpawu ziyabonakala osanda kuzalwa ngokushesha. cystic fibrosis kwamaphaphu ezinganeni inezimpawu ukukhwehlela ezinjalo, ubuthakathaka jikelele, isikhumba ugodo. Ngokuhamba kwesikhathi, ukukhwehlela ethuthukisiwe, kuhambisana kukhululwe isikhwehlela obukhulu. Broken ukudluliswa igesi izinqubo. Can ukushwaqa amathiphu neminwe. Ngokuvamile izingane ezine-cystic fibrosis futhi abaphethwe i-pneumonia, okuyinto eyenzeka ngo-kahle ezinzima. izicubu zokuphefumula abathintekayo ubovu ukuvuvukala. Isifo samaphaphu kuba amahlala khona. Mhlawumbe ekwandeni ezicutshini emaphashini. Ngokuhamba kwesikhathi, kuba nokukhononda ukubonakala "inhliziyo yamaphaphu".

Yamaphaphu fibrosis izimpawu cystic ezinganeni nakubantu abadala kuyafana: zemlyanistogo ibala lesikhumba, esifubeni uthola bochkopodobnuyu balolonge kalula likhubazeke. Futhi, kukhona iphika ngisho uphumule. Ngenxa ukunciphisa ukudla isisindo encishisiwe isiguli. Nokho, izimpawu ingavela kamuva kakhulu. Lena - ifomu ezikahle ukuze isiguli.

Isiteji cystic Fibrosis wamaphaphu ukugeleza

Kunezigaba eziningana inkambo isifo.

1. Isigaba sokuqala libhekene ukubukeka ukukhwehlela, ngokuvamile eyomile futhi ngaphandle isikhwehlela. futhi iphika kungenzeka kwezinye izimo. Isici esikhethekile sale esiteji ukuthi ingahlala ngisho eminyakeni embalwa (kufika ku-10).
2. Esesibili - kuyinto ukuvela ukucinana kwemigudu yokuphefumula efomini okungamahlalakhona, ushintsho phalanges. Vela Kwabiwa ukukhwehlela. Lokhu degree zokugula futhi ihlala Adalberto isikhathi eside - kuze iminyaka engu-15.
3. In esigabeni sesithathu yezinkinga ukuthuthukisa. Kuncibilikile wamaphaphu izicubu, izinhlumba ukuvela. Ngenxa yalokhu, kuba ukuhlupheka futhi inhliziyo. Ubude Isikhathi sokugula - iminyaka 3-5.
4. Isigaba sesine (kwezinyanga ezimbalwa) libhekene i ukunqotshwa esindayo kakhulu umgudu wokuphefumula, inhliziyo. Umphumela ngokuvamile ebulalayo.

Izimpawu ze-emathunjini cystic fibrosis

Le ndlela lesi sifo libhekene ukwahluleka isimiso sokugaya ukudla bungene. Emathunjini cystic fibrosis e ingane ububonisa ngokucacile kakhulu ngokwayo esikhathini nokulunyulwa. Kulokhu, kukhona ukumuncwa ompofu kwamafutha, amaprotheni (carbohydrate basuke kugaywe kancane kangcono). Ngenxa ukuthuthukiswa izinqubo zokuwohloka esiswini akhiwa compounds ezinobuthi, siyakhukhumala esiswini. Inani elikhulu of wamathumbu ukunyakaza kwandisa. Uma ethola ukuthi unesifo "cystic fibrosis" (ifomu enteric) ezinganeni futhi bangezwa prolapse rectal. Ngokuvamile iziguli esekhala nomlomo owomile. Utrudnyaetsya ethola ukudla eyomile. Nge inchubekelembili kwaleso sifo kuyehla isisindo emzimbeni.

Lesi sifo futhi libhekene polyhypovitaminosis kusukela ngenxa yezinkinga ukugaya umzimba sintula amavithamini cishe wonke amaqembu. Njengomthetho, imisipha ziphelelwe yisimo sazo, isikhumba kuba ngaphansi nokunwebeka. Omunye ngaphezulu izimpawu nobuntu cystic fibrosis ezinganeni (uhlobo emathunjini) - ubuhlungu yinto ehlukile esiswini. Ngokuhamba kwesikhathi, kungase kube nzima, futhi peptic isilonda isifo, kanye nesifo sikashukela (ifomu esicashile). Lesi sifo izinso, isibindi. Uma usindwa isibindi, isitulo iba abamnyama. Ubuthi zanqwabelana emzimbeni futhi ufinyelele egazini ebuchosheni. Sinenkosi nomphumela ongemuhle ezinzwa, ukuthuthukisa encephalopathy. Futhi kunomthelela ukwenyuka kancane ubende emathunjini cystic fibrosis ezinganeni. Photography wamathumbu pathologically ezishintshwe (esigabeni cross) kuboniswa ngezansi.

ifomu ezixutshiwe lesi sifo

Lolu hlobo isifo libhekene izimpawu kokubili inhalational kanye emathunjini. Ngokuvamile, izinsana waphawula inyumoniya njalo isikhathi eside, ukucinana kwemigudu yokuphefumula. Cishe kuzo zonke izimo, nokukhwehlela likhona. Ngaphezu kwalokho, okuxubile cystic fibrosis ezinganeni kuhambisana ukuvuvukala, sezinyawo ngokuvamile uketshezi, umbala lwakhe luphenduka oluhlaza. Kukhona ukuhlanganisa of kobukhulu isifo kusukela esikhathini lapho izimpawu zokuqala yavela. Ngokuvamile, uma izimpawu zokuqala kutholwa esemncane, onalesi sifo kuyinto ngempela okungekuhle.

meconium ileus

cystic fibrosis kuphakamisa ukwanda viscosity izimfihlo umzimba, kuhlanganise meconium - yasekuqaleni sezinyawo ezinganeni. Lokhu kuphumela ukuvaleka kanye emathunjini. Le ndlela lesi sifo kwenzeka ngesikhathi sokuzalwa lapho meconium akusho nimuke. Ingane iba udlubulunda, ngokuvamile regurgitates (ngisho ne Bile ukungcola). Ngaphezu kwalokho kukhona umoya esiswini, ukuqunjelwa, isikhumba kuba okhanyayo. izifo Eminye ukugeleza kusiza ukuqinisekisa ukuthi usana iyehlisa motor umsebenzi (uma eyeka). Isizathu salokhu simo ukungabi khona trypsin. Meconium ileus kuyingozi ngempela futhi edinga kuhlinzwa.

sitholakele isifo

Ukuxilongwa cystic fibrosis ezinganeni kuhlanganisa ukuhlolwa kwezinso zenani elikhulu eziyifa kanye azalwa nako. Futhi, ukuhlolwa kwegazi, umchamo kanye isikhwehlela khulula. Izintambo futhi coprogram. It ikuvumela ukuba kutholakale khona inclusions fat in the indle yengane. Ucwaninge futhi zokuphefumula uhlelo (esimweni, bronchography, bronchoscopy). Kudingekile futhi spirometry, njengoba kuvumela ukuhlola isimo sokusebenza kwamaphaphu. Uma usola i-cystic fibrosis (izimpawu ezinganeni kungenzeka ukuzibonakalisa) ungenza ucwaningo zofuzo. Asiza ukusungula khona kuguquka isakhi unesibopho nomsebenzi secretory umzimba. Neonatal Ukuhlolwa ezisanda kubelethwa wenziwa (trypsin lokuhlushwa uphenyo endaweni omisiwe igazi spot). Kuyinto ulwazi ngokwanele futhi isivivinyo umjuluko. Uma imbiza wembula sodium ion, chlorine e isamba anda, kungenzeka ukuba ngikhulume ngokukhululeka ngokukhubazeka khona isifo awokuthi. Uma owesifazane, lapho mndeni abantu kwathiwa unesifo "cystic fibrosis", alindele ukuba nengane, odokotela batusa ukuba sihlole -amniotic fluid yobudala mayelana 18-20 amasonto.

ekwelapheni cystic fibrosis

Kuyaphawuleka ukuthi balahle lesi sifo ayinakwenzeka. Nokho, ukwelashwa kungaba ngokuphawulekayo ngcono izinga lempilo kanye nesikhathi salo. Ngaphambili, iziguli eziningi unesifo efanayo yokufa ngaphambi kweminyaka eli-20. Nokho, manje, ukwelashwa okufanele futhi ifike ngesikhathi angaphila isikhathi eside ngokuphawulekayo. ifomu emathunjini kudinga uhlobo olukhethekile lokudla. Ukudla kufanele kube ocebile amaprotheni (inhlanzi, amaqanda). Ngaphezu kwalokho esimisiwe namavithamini eziyinkimbinkimbi. Futhi kudingeka reception enzyme ( "Creon", "pantsitrat", "zemikhosi" et al.). Kuyaphawuleka ukuthi le mithi kufanele kuthathwe zonke izinsuku zokuphila kwakhe. Iqiniso ukuthi imithi unika umphumela izobonisa ukuzinza of sezinyawo, kwesisindo kanye nokuntuleka ngisho ekukhuleni kwayo. Ubuhlungu besisu zinyamalale, futhi ngemva kwalokho angene endleni kungukuthi ibanjiwe ukufakwa amafutha.

Lapho izidakamizwa ifomu yamaphaphu lesi sifo kudingeka ezokwenza lula liquefaction ka isikhwehlela futhi bronchial ukubuyisela ukusebenza ( "Mukosolvin", "Mukaltin"). Kubalulekile kakhulu ekwelapheni yamaphaphu-cystic fibrosis ukuvimbela ukuthuthukiswa izinqubo ezithathelwanayo emaphashini. umphumela omuhle, nasekukholweni okungazenzisiyo ukuphefumula izivivinyo ezikhethekile. Kufanele kwenziwe njalo. Ngoba impatho ingasatshenziswa nemithi elwa namagciwane. Bayakhohlwa isifo isikhathi eside ukusiza indlela ezinkulu, njengoba ukufakelwa yamaphaphu. Nokho, it has nezihibe: ingozi senqatshwa ekuphathweni izidakamizwa ukuthi zivimbela izivikeli mzimba. Ngaphezu kwalokho, isiguli kufanele abe sesimweni enhle ngokomzimba. Eziningi zalezi Ukufakelwa wenziwa phesheya.

Izincomo ezenziwe for iziguli ezine-cystic fibrosis

Odokotela batusa kakhulu ukuthi iziguli nale Ihlahlubo ukwenza imigomo ngesikhathi esifanele ngokumelene ukukhwehlela whooping, futhi izifo ezifanayo. Kubalulekile ukuqeda nomzimba ezingaba: dander isilwane, imicamelo nezingubo zokulala kusukela uphaphe inyoni. Futhi akuvumelekile ukuthi intuthu kagwayi. kokutshelwa ngokukhubazeka kwengane kudinga ukwelashwa spa kwezingane. Uma degree of kobukhulu isifo singakabi ezibucayi, ingane ukuya esikoleni, amaqembu ezemidlalo, has a yokuphila sasivumela asebenzayo. Ukwelashwa nokuvikelwa cystic fibrosis ezinganeni ezineminyaka engaphansi konyaka owodwa kuhilela ukusetshenziswa Izingxube ekhethekile (Dietta engaphezulu, Dietta Plus). Ngaphezu kwalokho, isidingo ukwandisa futhi umthamo owodwa we izikhathi 1.5. Ngaphezu kwalokho busuka ekudleni komntwana kuyinto esincane usawoti (lokhu kubalulekile ehlobo).

ngokukhethekile kufanele kube imenyu nesineke. Kufanele bacebe amafutha (ukhilimu, ibhotela, inyama) njengoba ukumuncwa kwezakhi ngobugebengu. Ukuphuza futhi kudingeka luningi. Kubalulekile nokukhumbula ukuthi ukuthatha enzyme amalungiselelo adingekayo nsuku zonke. Ngaphezu ukwelashwa eyisisekelo ingasetshenziswa, kanye nezinhlelo zokwelashwa ngama bendabuko. Kusiza expectoration amakhambi ezifana lwebhece, umama-futhi-nosingamama. nomphumela omuhle isimiso sokugaya ukudla bungene abe dandelion, elecampane. Futhi amafutha abalulekile isetshenziselwa inhalations (i-lavender, basil, nehisopi). Futhi ewusizo futhi ekuqiniseni imikhiqizo efana noju.

Isibikezelo sezulu sezinsuku

Ngeshwa, isilinganiso sobude bokuphila for ukuxilongwa inikezwe akuyona eliphezulu kakhulu. Ngokwesilinganiso, iziguli siphile iminyaka engaba ngu-30 (eRussia), noma kuze kube 40 noma ngaphezulu (phesheya). Nokho, uphethwe esifike ngesikhathi futhi ukwelashwa okufanelekile kakhulu ngcono isimo lesiguli. I ezingezinhle kakhulu Izibikezelo - e ukubonakaliswa okuqala e-cystic fibrosis (ezinsaneni). Kodwa futhi eyaziwa icala lapho isiguli isifo esifanayo waphila iminyaka engaphezu kuka-70. Lo mehluko wenza sokuphila eRussia nakwamanye amazwe ngenxa isici zezimali. iziguli Overseas ungisekela impilo isimo. Ngenxa yalesi bangakwazi ukuphila okuvamile, ukufunda, ukudala umndeni futhi ibe nabantwana. Kodwa Russia ayikwazi ngokwanele ukuhlinzeka iziguli ngemithi ezidingekayo (futhi lokhu enzyme amalungiselelo, futhi imithi elwa namagciwane ekhethekile, futhi mucolytics). Kuphela inani elilinganiselwe izingane zithole ukunakekelwa kwezokwelashwa kwamahala nemithi edingekayo. Iziguli ukuhlala ku-akhawunti yakhe ekhethekile lonke. Ukuze ususe okuvela leso sifo kumele uxhumane isazi sezakhi zofuzo kwenye esiteji bekuhlela sokukhulelwa.

amathiphu kwenqondo abazali

Kuningi izincwadi iklanyelwe ukusekela kubazali ingane unesifo-cystic fibrosis. Okokuqala, ningatatazeli. Sidinga ukuthola ulwazi oluningi mayelana nezifo ukuze usize ngempumelelo ingane ukuba unqobe nemiphumela yaso. Kubalulekile njalo umkhumbuze luthando lwakhe.

Lesi sifo kakhulu asithinti nje kuphela isimo somzimba, kodwa futhi ngezinga elithile phezu ngokomzwelo. Ngakho-ke, ubunzima akunakugwenywa (Nokho, zikhona futhi bekhulisa izingane enempilo). Ezinye zokukhwabanisa uthembeke ngokwanele ukuba afeze futhi isiguli omncane. Ochwepheshe bathi ngenkathi izingane musa nje ufunde ukugula kwazo, kodwa futhi nginakekela ngokwabo, umuzwa ongcono kakhulu.

Abazali abazange bazizwe bebodwa ekulweni cystic fibrosis, udinga ukuxhumana nemikhaya yabo, ebhekene nenkinga efanayo. It kungenziwa futhi ekhethekile izinkundla inthanethi. Kukhona okuningi izimali, bengathola kokubili usizo ezingokwengqondo zezimali. Kubalulekile ukukhumbula ukuthi kokutshelwa ngokukhubazeka kwengane - hhayi umusho. Abantu abaningi abadumile nalesi sifo zofuzo, kodwa lokhu ngeke iyeke kubo siphumelele ekuphileni. Umculi Gregory Lemarchal, umlingisi yamahlaya Bob Flanagan (waphila ngokuvumelana neminyaka engu-43) - lezi ezinye nje izibonelo zendlela ukuphila futhi akhule kanye kokutshelwa ngokukhubazeka kwengane. Ngaphezu kwalokho, imithi akemi namanje: ucwaningo emhlabeni wonke kwenziwa ukwelapha ngezakhi zofuzo ka-cystic fibrosis e-United States. Nokho, Uma, ukuze ubhekane nzima imizwelo yabo, ungahlala bafune usizo wezengqondo.