Cystic fibrosis yog ib tug me nyuam: tej yam tshwm sim, cov tsos mob, kev kho mob

Ib qho ntawm feem ntau cov kab mob uas yog pub yog cystic fibrosis. Tus me nyuam uas ua los ntawm pathogenic daim card uas ua rau yus muaj kev cuam tshuam ntawm cov hnyuv, ua pa system. Characteristically, tus kab mob no yog ib tug mob thiab feem ntau nws tus kheej, Yog hais tias ob qho tag nrho cov niam txiv muaj ib tug puas noob. Yog hais tias ib qhov chaw nrog ib tug hloov nyob rau hauv tsuas yog ib qho ntawm cov niam txiv, cov me nyuam yuav tsis tau txais tus kab mob no. Feem ntau, cystic fibrosis thiaj paub tias yog nyob rau hauv thawj xyoo ntawm lub neej (los sis txawm nyob rau hauv lub plab).

Lub keeb kwm ntawm tus kab mob

Cystic fibrosis nyob rau hauv cov me nyuam muaj vim muaj kev cuam tshuam ntawm cov qauv ntawm cov chromosome 7 (nws lub xub pwg). Nyob rau hauv cov ntaub ntawv no, cov kua nplaum nyob rau hauv lub cev yuav khov dua. Nws yog lub npe hu hais tias tus daim card los ntawm no hom yuav them los ntawm yuav luag tag nrho cov hauv nruab nrog cev. Vim li no cov nto moistened lawv, thiab yuav nej cais, ntxiv hnoos qeev cleans nws. Tab sis, yog hais tias koj tshwm sim mus tsoo, lub khov secretion stagnates accumulates. Nyob rau hauv xws li ib puag ncig, thiab pathogenic microorganisms muab, ua rau tas mus li kab mob. Tom qab cuam tshuam lub nruab nrog digestive system, tus kab mob suffers los ntawm tsis muaj oxygen. Rau cov thawj lub sij hawm tus me nyuam tau cystic fibrosis piav nyob rau hauv 1938 los ntawm D. Anderson. Kom txog thaum uas lub sij hawm, ib tug loj tus naj npawb ntawm cov me nyuam tuag los ntawm mob ntsws thiab lwm yam kab mob uas yog tshwm sim los ntawm cystic fibrosis. Yog xav paub ntxiv txog tus kab mob sawv nyob rau hauv lub lig thib nees nkaum caug xyoo. Yuav luag txhua txhua thib nees nkaum neeg nyob li ntawm peb ntiaj chaw - cov cab kuj ntawm lub cystic fibrosis noob mutating. Qhov no kho raws yog tsis muaj feem xyuam rau tau phem ntawm cov niam txiv thaum lub sij hawm conception, nrog noj tshuaj. Tsis tas li ntawd nyob rau hauv nws muaj tsis cuam tshuam rau unfavorable ecological teeb meem no. Lawv raug kev txom nyem los ntawm cov tib ob ntxhais thiab cov tub hluas.

daim ntawv ntawm cystic fibrosis

Tus kab mob tej zaum yuav muaj peb tej daim.

• Tus thawj tug - mob ntsws. Nws tshwm sim nyob rau hauv kwv yees li 15-20% ntawm tus xov tooj ntawm txhua tus neeg mob. Yus muaj los ntawm qhov tseeb hais tias lub bronchi ua daig nrog tuab hnoos qeev. Tom qab ib co sij hawm, lawv yuav tau mus kiag li daig. Cov pub leejtwg mus rau ua ib tug zoo heev yog yug me nyuam hauv av rau cov kab mob thiab cov kab mob. Thaum lub sij hawm, lub ntsws cov ntaub so ntswg kaw, ua nyuaj. Hlwv yuav tshwm sim. Ntxiv ua hauj lwm yog koj muaj teebmeem kev xwb deteriorates. Ib tug neeg muaj peev xwm tuag los ntawm txhawm chim.
• Cystic fibrosis, ib tug me nyuam tej zaum yuav muaj, thiab cov plab hnyuv daim ntawv. Nyob rau hauv no cuam tshuam lub digestive system, cov zaub mov yog tsis zoo nqis. Tej yam kev mob ua rau txoj kev loj hlob ntawm cov ntshav qab zib, peptic rwj, daim siab mob npuas paug thiab thiaj li nyob. N. Nws yog pom nyob rau hauv 5% ntawm cov neeg.
• Feem ntau daim ntawv - tov. Nws thiaj paub tias yog nyob rau hauv 75% ntawm cov neeg mob nrog rau qhov no caj kab mob.
• Heev tsawg (txog 1%) tshwm sim, thiab lub atypical daim ntawv.

Cystic fibrosis ntawm lub ntsws nyob rau hauv cov me nyuam

Qhov no daim ntawv ntawm tus kab mob yog feem ntau xa mus rau raws li qhov ua pa. Feem ntau, cov tsos mob no tau raug ua nyob rau hauv tus me nyuam mos tam sim ntawd. Cystic fibrosis ntawm lub ntsws nyob rau hauv cov me nyuam muaj cov tsos mob xws hnoos, general tsis muaj zog, tawv nqaij daj. Thaum lub sij hawm, enhanced hnoos, nws yog nrog los ntawm qhov kev tso tawm ntawm ib tug tuab hnoos qeev. Tawg roj hloov lwm lub tsev muaj dab. Yuav nkoog lub tswv yim ntawm cov ntiv tes. Feem ntau cov me nyuam nrog cystic fibrosis, thiab kev txom nyem los ntawm mob ntsws, uas tshwm sim nyob rau hauv ib tug ncaj loj. pa nqaij mos cuam tshuam purulent o. Mob Ntsws Txheem Dej yuav ntev. Tej zaum qhov loj hlob ntawm connective cov ntaub so ntswg nyob rau hauv lub ntsws. Thaum lub sij hawm, muaj mob cov tsos ntawm "pulmonary lub siab".

Koj muaj teebmeem kev cystic fibrosis cov tsos mob nyob rau hauv cov me nyuam thiab cov laus yog zoo sib xws: zemlyanistogo daim tawv nqaij xim, lub hauv siab tau txais bochkopodobnuyu shape deformed cov ntsis ntiv tes. Tsis tas li ntawd, muaj yog txog siav txawm nyob rau hauv so. Vim cov yuav txo tau ntawm qab los noj mov thiab txo luj ntawm tus neeg mob. Txawm li cas los, cov tsos mob yuav tshwm sim ntau npaum li cas tom qab ntawd. Qhov no yog - ib tug ntau paaj daim ntawv rau tus neeg mob.

Theem cystic fibrosis ntsws txaus

Muaj ntau ntau theem ntawm cov kab mob thiab thaum kawg.

1. Tus thawj theem yog tsiag ntawv los ntawm cov tsos ntawm hnoos, feem ntau qhuav thiab tsis muaj hnoos qeev. thiab ua tsis taus pa tej zaum yuav tshwm sim nyob rau hauv tej rooj plaub. Ib tug feature tshwj xeeb ntawm no nyob ntev yog tias nws muaj peev xwm kav txawm tias ib tug ob peb xyoos (mus txog 10).
2. Qhov thib ob theem - yog lub rov tshwm sim ntawm hlab ntsws nyob rau hauv lub mob hauv daim ntawv, qhov kev hloov nyob rau hauv lub phalanges. Tshwm qee hnoos. Qhov no degree ntawm kev mob nkeeg kuj kav heev ib tug ntev lub sij hawm - mus txog rau 15 xyoo.
3. Nyob rau hauv lub thib peb theem teeb meem tsim. Condensed ntsws ntaub so ntswg, hlwv yuav tshwm sim. Tiv thaiv no tom qab, nws yog kev txom nyem thiab lub plawv. Lub duration ntawm lub sij hawm ntawm tus kab mob - 3-5 lub xyoo.
4. Qhov thib plaub theem (ib tug ob peb lub hlis) yog yus muaj los ntawm ib tug tsis tshua hnyav yeej ntawm lub pa system, lub plawv. Lub sij hawm yog feem ntau neeg tuag taus.

Cov tsos mob ntawm plab hnyuv cystic fibrosis

Qhov no daim ntawv ntawm tus kab mob no yus muaj los ntawm ib tug tsis ua hauj lwm nyob rau hauv lub digestive system. Plab hnyuv cystic fibrosis nyob rau hauv ib tug me nyuam kom meej meej heev manifests nws tus kheej nyob rau hauv lub sij hawm ntawm weaning. Nyob rau hauv cov ntaub ntawv no, muaj yog tsis nqus ntawm cov rog, cov nqaijrog (carbohydrates yog nqis me ntsis zoo dua). Vim txoj kev loj hlob ntawm cov txheej txheem kev lwj nyob rau hauv lub gut yog tsim tshuaj lom tebchaw, swells lub plab. Ib tug xov tooj tseem ceeb ntawm plob tsis so tswj taw tsub kom. Yog hais tias mob "cystic fibrosis" (enteric daim ntawv no) nyob rau hauv cov me nyuam tej zaum yuav kuj ua qhov quav prolapse. Feem ntau cov neeg mob tsis txaus siab ntawm lub qhov ncauj qhuav. Utrudnyaetsya tau txais qhuav zaub mov. Nrog ntxiv kev vam meej ntawm tus kab mob yog txo lub cev hnyav.

Tus kab mob no kuj yus polyhypovitaminosis vim vim zom cov zaub mov cov teeb meem lub cev lacks vitamins zoo tag nrho tej pawg neeg. Raws li ib tug txoj cai, cov nqaij ntshiv tsis tau lawv lub suab, daim tawv nqaij yuav tsis tshua muaj elastic. Ib tug ntau yam ntxwv cov tsos mob ntawm cystic fibrosis nyob rau hauv cov me nyuam (plab hnyuv hom) - qhov mob ntawm ib tug cov xwm sib txawv nyob rau hauv lub plab mog. Thaum lub sij hawm, tej zaum nws yuav tsum, thiab peptic rwj kab mob, thiab mob ntshav qab zib (latent daim ntawv no). Tus kab mob muaj feem xyuam rau lub ob lub raum, lub siab. Yog hais tias ntaus los ntawm lub siab, lub rooj zaum yuav dub. Co toxins sau nyob rau hauv lub cev thiab mus txog hauv cov hlab ntsha mus rau lub hlwb. Lawv muaj ib tug tsis zoo ntxim rau cov hlab ntsha hlwb, tsim encephalopathy. Tseem muaj txhawb rau gradual nce nyob rau hauv tus po plab hnyuv cystic fibrosis nyob rau hauv cov me nyuam. Photography pathologically kho plob tsis so tswj (nyob rau hauv tus ntoo khaub lig seem) yog qhia hauv qab no.

Mixed daim ntawv ntawm tus kab mob

Qhov no hom ntawm tus kab mob yog yus muaj los ntawm tej yam tshwm sim ntawm tag nrho ob qho inhalational thiab plab hnyuv. Feem ntau, cov me nyuam mos cai ntau heev thiab ntev mob ntsws, ntsws. Nyob rau hauv yuav luag txhua tus neeg mob, thiab hnoos yog tam sim no. Nyob rau hauv tas li ntawd, mixed cystic fibrosis nyob rau hauv cov me nyuam yog nrog los ntawm tsam plab, quav feem ntau yog ib cov kua, nws cov xim puv ntsuab. Muaj yog ib tug correlation ntawm heev ntawm tus kab mob los ntawm lub sij hawm thaum cov tsos mob xub tshwm sim. Feem ntau, yog hais tias tus thawj cov cim qhia nrhiav tau nyob rau ntawm ib thaum ntxov lub hnub nyoog, lub raug yog heev unfavorable.

meconium ileus

Cystic fibrosis provokes ib qho kev nce nyob rau hauv viscosity ntawm tus kab mob secrets, xws li meconium - thawj quav nyob rau hauv cov me nyuam. Qhov no los tau nyob rau hauv lub txhaws thiab hnyuv. Qhov no daim ntawv ntawm tus kab mob no tshwm sim nyob rau yug me nyuam thaum meconium tsis ncaim. Tus me nyuam yuav nyob tsis tswm, feem ntau regurgitates (txawm muaj kua tsib impurities). Ntxiv muaj tsam plab, daim tawv nqaij yuav daj ntseg. Ntxiv cov kab mob txaus pab xyuas kom meej tias tus me nyuam mos ho thiaj li tsav ua si (yog hais tias tsis nws tsis). Yog vim li cas rau tus mob no yog qhov uas tsis trypsin. Meconium ileus yog heev txaus ntshai thiab yuav tsum tau phais kev cuam tshuam.

mob ntawm tus kab mob no

Mob ntawm cystic fibrosis nyob rau hauv cov me nyuam muaj xws li kev kuaj rau raws roj ntsha thiab congenital txawv txav. Tsis tas li ntawd, cov ntshav mus kuaj, zis thiab cov hnoos tawm. Nyob ntawm tej thiab coprogram. Nws tso cai rau koj los mus txiav txim hauv lub xub ntiag ntawm inclusions ntawm rog nyob rau hauv cov quav ntawm cov me nyuam. Researched thiab ua pa system (radiography, bronchography, bronchoscopy). Tsim nyog thiab spirometry, raws li nws tso cai rau kom ntsuam xyuas tej lub xeev ntawm lub ntsws. Yog hais tias koj xav tias ib tug cystic fibrosis (cov tsos mob nyob rau hauv cov me nyuam tej zaum yuav tsis manifest nws tus kheej) ua kev tshuaj ntsuam genetic. Lawv pab tsim kom muaj cov muaj ib tug noob hloov uas yog lub luag hauj lwm rau lub secretory kev ua si ntawm tus kab mob. Neonatal neonatal kuaj yog ua (trypsin concentration yog soj ntsuam nyob rau hauv ib tug qhuav ntshav chaw). Nws yog tuav txaus thiab cov hws xeem. Yog hais tias lub lauj kaub qhia sodium ions, tshuaj chlorine ua nyob rau hauv ib tug muaj zog npaum li cas, nws tseem tau mus hais lus ntawm lub hav zoov ntawm tus kab mob nrog ib tug high school cov probability. Yog hais tias ib tug poj niam, nyob rau hauv uas lub tsev neeg cov neeg uas muaj ib tug mob ntawm "cystic fibrosis", yuav ib tug me nyuam, cov kws kho mob pom zoo kom mus xyuas lub amniotic kua rau lub hnub nyoog ntawm txog 18-20 lub lis piam.

kev kho mob ntawm cystic fibrosis

Nws yog ib nqi sau cia hais tias tau tshem ntawm tus kab mob yog tsis yooj yim sua. Txawm li cas los, kev kho yuav zoo heev txhim kho qhov zoo ntawm lub neej thiab nws ntev. Yav tas los, muaj ntau yam mob uas muaj xws li cov kev kuaj mob tuag ua ntej lub hnub nyoog ntawm 20 xyoo. Tam sim no, txawm li cas los, nrog rau txoj kev thiab raws sij hawm kev kho mob muaj peev xwm nyob ho ntev. Plab hnyuv daim ntawv yuav tsum tau ib tug tshwj xeeb cov khoom noj. Khoom noj khoom haus yuav tsum nplua nuj nyob rau hauv protein (ntses, qe). Tsis tas li ntawd tsa thiab complex vitamins. Tsis tas li ntawd yuav tsum tau kev txais tos enzymes ( "Creon", "pantsitrat", "Festal" thiab al.). Nws yog ib nqi sau cia hais tias cov tshuaj yuav tsum tau coj thoob plaws hauv nws lub neej. Qhov tseeb hais tias cov kev kho mob muab cov tshwm sim yuav qhia normalization quav, poob phaus thiab tsis muaj kev txawm nws txoj kev loj hlob. Mob plab ploj, thiab nyob rau hauv cov quav tsis kuaj menyuamuas ntawm roj.

Thaum koj muaj teebmeem kev siv yeeb tshuaj hauv daim ntawv ntawm cov kab mob uas yuav tsum tau hais tias yuav pab txhawb liquefaction ntawm hnoos qeev thiab bronchial restore functionality ( "Mukosolvin", "Mukaltin"). Nws yog ib qho tseem ceeb heev nyob rau hauv cov kev kho mob ntawm pulmonary cystic fibrosis kom tiv thaiv cov kev loj hlob ntawm cov kab dab nyob rau hauv lub ntsws. Zoo nyhuv thiab ib tug tshwj xeeb ua tsis taus pa ce. Nws yuav tsum tau ua tsis tu ncua. Rau cov kev kho mob yuav muab tshuaj tua kab mob thiab. Tsis nco qab txog tus kab mob rau ib tug txiav txim lub sij hawm pab xws li ib tug radical txoj kev, raws li cov kev sib hloov ntawm lub ntsws. Txawm li cas los, nws muaj nws cov drawbacks: kev pheej hmoo ntawm rejection, tus thawj coj ntawm cov tshuaj uas inhibit qhov kev tiv thaiv kab mob. Nyob rau hauv tas li ntawd, tus neeg mob yuav tsum tau nyob rau hauv tubntxhais zoo mob lub cev. Feem ntau ntawm cov hloov ua txawv teb chaws.

Pom zoo ua rau cov neeg mob nrog cystic fibrosis

Cov kws kho mob xav kom koj hais tias cov neeg mob nrog rau qhov no mob yuav ua rau raws sij hawm txhaj tshuaj tiv thaiv hnoos, thiab zoo sib xws cov kab mob. Nws yog ib qho tseem ceeb los mus tshem tawm tej zaum allergens: tus tsiaj dander, tog hauv ncoo thiab pam vov los ntawm ib tug noog plaub. Nws kuj txwv tsis pub rau passive haus luam yeeb. Xws li ib tug mob yuav tsum tau ib tug spa kev kho mob ntawm cov me nyuam. Yog hais tias cov neeg kawm ntawv ntawm hnyav ntawm tus kab mob yog tsis tseem ceeb heev, tus me nyuam mus kawm lub tsev kawm ntawv, cov kev ua si clubs, muaj ib tug ncaj active txoj kev ua neej. Kev kho mob ntawm cystic fibrosis nyob rau hauv cov me nyuam muaj hnub nyoog nyob rau hauv ib xyoos yuav siv cov tshwj xeeb mixtures (Dietta Ntxiv, Dietta Plus). Nyob rau hauv tas li ntawd, qhov yuav tsum tau rau nce thiab ib zaug xwb koob tshuaj ntawm 1.5 lub sij hawm. Tsis tas li ntawd nkag tau rau hauv tus me nyuam txoj kev noj haus yog ib tug me me npaum li cas ntawm cov ntsev (qhov no yog qhov tseem ceeb nyob rau hauv lub caij ntuj sov).

Tshwj xeeb mloog yuav tsum ua siab ntev qhia zaub mov. Nws yuav tsum tau nplua nuj nyob rau hauv roj (lee, butter, nqaij) raws li qhov haum cov as-ham lawm. Haus kuj yuav tsum tshaj. Nws yog ib nqi nco ntsoov tias noj enzyme npaj yuav tsum tau txhua hnub. Nyob rau hauv tas li ntawd mus rau lub yooj yim kev kho mob yuav siv tau, thiab cov tshuaj kho. Facilitates expectoration tshuaj ntsuab xws li marshmallow, niam-thiab-stepmother. Zoo ntxim rau cov digestive system muaj ib tug dandelion, elecampane. Thiab qhov tseem ceeb roj yog siv rau inhalations (lavender, basil, hyssop). Tsis tas li ntawd pab tau thiab fortifying cov khoom xws li zib ntab.

forecasts

Tu siab, lub neej expectancy rau ib tug muab mob yog tsis heev. Nyob rau nruab nrab, cov neeg mob nyob rau txog 30 xyoo (nyob rau hauv Russia), los yog txog li 40 los yog ntau tshaj (txawv teb chaws). Txawm li cas los, raws sij hawm mob thiab kho kom ho zoo rau tus neeg mob tus mob. Qhov tseem unfavorable raug - nyob rau hauv thaum ntxov ces cystic fibrosis (nyob rau hauv cov me nyuam mos). Tab sis nws yog lub npe hu thiab ib cov ntaub ntawv nyob qhov twg ib tug neeg mob nrog zoo xws li cov kab mob nyob rau ntau tshaj 70 xyoo. Qhov no txawv nyob rau hauv lub neej expectancy nyob rau hauv Russia thiab lwm lub teb chaws vim cov nyiaj txiag nam. Txawv teb chaws cov neeg mob tau txais lub neej-ntev kev pab txhawb nqa ntawm lub xeev. Vim li no lawv yuav ua ib lub neej zoo, kom paub, los ua ib tug tsev neeg thiab muaj cov me nyuam. Tab sis Russia yuav tsis txaus muab cov neeg mob uas tsim nyog tshuaj (thiab qhov no enzyme npaj, thiab tshwj xeeb tshuaj tua kab mob, thiab mucolytics). Tsuas yog ib tug tsawg tus naj npawb ntawm cov me nyuam tau txais dawb kev kho mob thiab yuav tsum tau noj tshuaj. Cov neeg mob nyob rau hauv ib tug tshwj xeeb account ntawm nws lub neej tag nrho. Yuav kom tshem tawm cov tshwm sim ntawm xws li ib tug kab mob yuav tsum sab laj nrog ib tug geneticist rau lwm theem ntawm cev xeeb tub npaj.

Puas siab puas ntsws lub tswv yim rau cov niam txiv

Ib tug ntau ntawm cov ntaub ntawv uas tsim los txhawb cov niam txiv uas nws tus me nyuam suffers los ntawm cystic fibrosis. Ua ntej ntawm tag nrho cov, tsis txhob ntshai. Peb yuav tsum tau ntau npaum li cov lus qhia txog tus kab mob no nyob rau hauv thiaj li yuav zoo pab tus me nyuam los mus kov yeej nws cov txim. Nws yog ib qho tseem ceeb rau tsis tu ncua nws nco qab nws txoj kev hlub.

Tus kab mob ho tsis yog lub mob lub cev, tab sis kuj rau ib co raws li nyob rau hauv lub siab ntsws. Yog li ntawd, lub teeb meem yuav tsis tsum zam (txawm li cas los, lawv yog tam sim no thiab nyob rau hauv lub upbringing ntawm kev noj qab nyob cov me nyuam). Ib txhia manipulation yuav thoj mus nqa tawm thiab tus tub ntxawg tus neeg mob. Kws txawj hais tias thaum cov me nyuam tsis tsuas paub lawv cov mob, tab sis kuj tu lawv tus kheej, zoo npaum li cas lawm.

Cov niam txiv tsis xav nyob ib leeg nyob rau hauv sib ntaus tawm tsam cystic fibrosis, koj yuav tsum sib txuas lus nrog rau lawv cov tsev neeg, uas yog kev nyuaj siab nrog ib tug zoo xws li cov teeb meem. Nws yuav ua tau thiab tshwj xeeb Internet forums. Muaj yog ib tug ntau ntawm cov nyiaj, lawv muaj peev xwm tig mus rau ob qho tib si puas siab puas ntsws thiab kev pab nyiaj txiag. Nws yog ib qho tseem ceeb yuav tau nco ntsoov hais tias xws li ib tug mob - tsis yog ib tug kab lus. Muaj ntau cov neeg nto moo kev txom nyem los ntawm cov kev tshuaj ntsuam genetic kab mob, tab sis no twb tsis nres lawv kawm tau ntawv zoo nyob rau hauv lub neej. Singer Gregory Lemarchal, comic actor Bob Flanagan (nyob mus txog rau 43 xyoo) - cov no yog cov cia li ib co piv txwv ntawm yuav ua li cas ua neej nyob thiab loj hlob nrog xws li ib tug mob. Nyob rau hauv tas li ntawd, cov tshuaj tsis sawv tseem: lub ntiaj teb no kev tshawb fawb ua rau noob txoj kev kho ntawm cystic fibrosis nyob rau hauv lub tebchaws United States. Yog hais tias, txawm li cas los, lawv yuav paub tu lawv cov cwj pwm txawv yooj yim, koj yuav yeej ib txwm nrhiav kev pab los ntawm ib tug psychologist.