Cystic fibrosis mwana: zviratidzo, zviratidzo, kurapwa

Chimwe chezvinhu kwakapararira nezvirwere kuti vari nhaka ndiyo cystic fibrosis. Mwana zvinoberekwa pathogenic chakavanzika inotungamirira nokuvhiringidzirwa ari zvomudumbu turakiti, kufema hurongwa. Characteristically, hosha iri chirwere uye kazhinji hworatidza pacharo, kana vabereki vose vane yakati chakaremara Gene. Kana nzvimbo ine mutation mune chete mumwe vabereki, vana havazogari nhaka chirwere. Kazhinji, cystic fibrosis kunokwanisa kuonekwa mune rokutanga rokubata upenyu (kana kunyange mudumbu).

Nhoroondo chirwere

Cystic fibrosis muvana anova nokuda nokuvhiringidzirwa sechivako chromosome 7 (papfudzi rake). Panyaya iyi, iyo madzihwa mumuviri hunowedzera viscous. Zvinozivikanwa kuti chakavanzika ichi mhando vari kuchizvivanza zvinenge zvose zvomukati. Nokuda pamusoro ichi wakanyorova pavo, muraurwe, nemamwe madzihwa inochenesa izvozvo. Asi kana imi zvichaitika kuti anorovera, ari viscous secretion stagnates rinoungana. Mumamiriro ezvinhu akadaro, uye pathogenic tupukanana tusingaoneki muwande, zvichitungamirira chirwere zvachose. Naizvozvo sei nhengo yacho zvomudumbu ino, nhengo anotambura nokushayiwa okisijeni. Kokutanga Mwana cystic fibrosis paakarondedzerwa muna 1938 na D. Anderson. Kusvikira panguva iyoyo, vakawanda vana vanofa nemabayo uye nezvimwe zvirwere zvinokonzerwa cystic fibrosis. More ruzivo pamusoro chirwere yakabuda mukupera ramakumi remakore. Zvinenge zvose ramakumi ugere redu - mutakuri of cystic fibrosis Gene kusachinja. kuremara ichi haina chokuita tsika dzakaipa vabereki panguva pamuviri, pamwe kushandisa zvinodhaka. Uyewo pane ayo pamberi asingadi kukanganisa vanoshorwa zvipenyu nezvakazvipoteredza mamiriro ezvinhu. Vanotambura kubva chete nevasikana nevakomana.

chimiro cystic fibrosis

The chirwere angava zvitarisiko inokosha matatu.

• Wokutanga - zvemapapu. It unowanikwa anenge 15-20% of nhamba dzose nyaya. Dzinoratidzwa ndechokuti principales kuva tsinga pamwe gobvu madzihwa. Kwapera nguva, vanokwanisa tsinga zvachose. The chakavanzika wokuva shasha maberekero pasi kuti utachiona uye utachiona. Nokufamba kwenguva, mukuru zvemapapu rinenge rakanamwa, kuva nesimba. Cysts kuoneka. Uyezve basa pulmonary chete kuipa. Munhu anogona kufa kubva kutadza kufema.
• Cystic fibrosis, mwana ave uye intestinal chimiro. In ichi kwakakanganiswa ari zvomudumbu hurongwa, zvokudya havaratidzi ozeyewa. ezvinhu akadaro anoita kukura cheshuga, peptic urikanwi, chiropa cirrhosis uye zvichingodaro. N. Rinowanikwa 5% nyaya.
• The inonyanya fomu - yakavhenganiswa. Zvinonzi chirwere muna 75% varwere chirwere ichi majini.
• Very kushoma (anenge 1%) kunoitika, uye atypical chimiro.

Cystic fibrosis pakati mumapapu muvana

Ichi chimiro chirwere zvinowanzooneka nezvavo savakuru kufema. Kazhinji, zviratidzo zviri wakaratidzwa achangoberekwa pakarepo. Cystic fibrosis pakati mapapu muvana ane zviratidzo chikosoro kwakadaro, vakawanda simba, ganda akacheneruka. Nokufamba kwenguva, kunowedzerwa chikosoro, zviri pamwe pakabudiswa gobvu madzihwa. Broken gasi chinja pezvakaitwa. Anogona thicken kumuromo kwematanda minwe. Kazhinji vana cystic fibrosis, uye kutambura kubva nemabayo, kunoitika nenzira zvakanaka kwazvo. kufema zvinyama sei purulent kuzvimba. Mabayo anova chisingaperi. Zvichida kuwandisa connective zvinyama mumapapu. Nokufamba kwenguva, pane zviitiko pakuonekwa "mwoyo pulmonary".

Pulmonary cystic fibrosis zviratidzo muvana uye vakuru zvakafanana: zemlyanistogo ganda ruvara, chipfuva anowana bochkopodobnuyu kuumba akaremara nyore. Uyewo, pane kupfupika mweya kunyange zororo. Nokuda rutapudzo kuti havi uye kuderedzwa kurema murwere. Zvisinei, Zviratidzo zvinogona kuonekwa zvikuru gare gare. Ichi - a nani. chimiro kuti murwere.

Stage cystic fibrosis bapu aibuda

Pane matanho anoverengeka kosi chirwere.

1. Danho rokutanga kunoratidzwa nechitarisiko chikosoro, kazhinji yakaoma uye pasina sputum. uye kupfupika kufema zvingaitika mune zvimwe zviitiko. Chimwe chinhu chinokosha Danho ichi ndechokuti zvinogona kugara kunyange makore mashoma (kusvika 10).
2. Danho rechipiri - ndiko kwaita bronchitis ari chirwere chimiro, kuchinja mune phalanges. Kuoneka yavaive vangakosora. dhigirii ichi chirwere uyewo kwazvinogumira chaizvo kwenguva refu - kusvika makore 15.
3. In pachikuva rechitatu matambudziko kukudziridza. Yaiitwa yakapfupikiswa zvemapapu rinenge cysts kuoneka. Against ichi nechekumashure, zviri kutambura uye mwoyo. Ya nguva kurwara - 3-5 makore.
4. Danho chechina (mwedzi mishoma) iri ichazivikanwa noku chaizvo rinorema kukundwa ari kufema ezvinhu, mwoyo. Mugumisiro wacho kazhinji zvinouraya.

Zviratidzo intestinal cystic fibrosis

Ichi chimiro chirwere dzinoratidzwa kukundikana zvomudumbu ino. Intestinal cystic fibrosis ari mwana kunooneka pacharo munguva arumurwa zvakajeka kwazvo. Muchiitiko chino, pane murombo Absorção de Água kwemafuta, mapuroteni (makabhohaidhiretsi vari ozeyewa zvishoma nani). Nokuda kukura dzinotsanagura kuora ari dumbu vari kuumbwa chepfu yeimwe, wakazara mudumbu. A chinokosha Nhamba bowel masangano kunowedzera. Kana nechirwere "cystic fibrosis" (enteric chimiro) Vana vanogona uyewo kuona rectal prolapse. Kazhinji varwere vanotsutsumwa mumuromo yakaoma. Utrudnyaetsya kugamuchira zvokudya yakaoma. Mimwe kuchiita hosha ipfupi muviri uremu.

The chirwere chiri dzinoratidzwa polyhypovitaminosis kubva wakakodzera kugaya matambudziko muviri haana mavhitaminzi anenge mapoka ose. Kazhinji, tsandanyama vanorasikirwa matauriro avo, ganda anova zvishoma kwakavharwa nenyama. Mumwe mamwe maitiro zviratidzo cystic fibrosis muvana (intestinal mhando) - marwadzo yakasiyana hunhu mudumbu. Nokufamba kwenguva, zvingava, uye peptic urikanwi chirwere, uye chirwere cheshuga (Isimba rakadini rinogona kubudiswa fomu). The chirwere chinobata itsvo, chiropa. Kana akarova nokuda chiropa, pachigaro kunova vatema. Zvinokachidza vaunganidza mumuviri uye kusvika muropa kuuropi. Vane kunambwa masero etsinga, kutanga encephalopathy. Kunoitawo kuti zvishoma nezvishoma kuwedzera chirwatata intestinal cystic fibrosis vana. Photography pathologically zvakachinjwa bowel (mu muchinjikwa chikamu) iri pasi apa.

Mixed chimiro chirwere

Izvi Chirwere kunoratidzwa zviratidzo zvose inhalational uye intestinal. Kazhinji, vachangozvarwa akacherechedza kakawanda uye kwenguva refu mabayo, bronchitis. Kazhinji kacho, uye chikosoro aripo. Uyezve, zvakavhenganiswa cystic fibrosis mune vana achiperekedzwa bloating, kazhinji chetsoka imvura, ayo ruvara kwaanoda girini. Pane kuwirirana pakati kwokuomerwa chirwere kubva panguva apo zviratidzo kuoneka. Kazhinji, kana zviratidzo zvokutanga aonekwa achiri muduku, asi fungidziro iwayo zvisingafadzi..

meconium ileus

Cystic fibrosis chinokurwadzai kuwedzera viscosity pamusoro muviri zvakavanzika, kusanganisira meconium - chepakutanga chetsoka vana. Izvi inoguma blockage uye ura. Ichi chimiro chirwere kunoitika pakuzvarwa apo meconium hazviperimo. Mwana anova kudzikama, kazhinji inosvipa (kunyange bile marara). Uyezve pane bloating, ganda kunova zvacheneruka. Uyezve zvirwere kuyerera zvinobatsira nechokwadi kuti achangoberekwa yekudyidzana kunoderedza gwenya basa (kana hazvina anomira). Chikonzero ichi ndicho kusavapo trypsin. Meconium ileus rakanyatsojeka ngozi uye kunoda kuvhiya kupindira.

chirwere chirwere

Chirwere kuti cystic fibrosis muvana rinosanganisira kuongorora kuti nhaka uye vachiberekwa abnormalities. Uyewo, bvunzo ropa, weti uye sputum buditsa. Waya uye coprogram. Kunoitawo kuti kuona pamberi inclusions mafuta ari mutsvina yadzo mwana. Akaongorora uye kufema system (radiography, bronchography, bronchoscopy). Zvakakodzera uye spirometry, sezvo anobvumira kuongorora dzichipfekeka mamiriro mumapapu. Kana uchifungidzira munhu cystic fibrosis (Zviratidzo vana varege unooneka) zvidzidzo magene tsvakurudzo. Vanobatsira kumisa kuvapo zviratidzo mutation kuti ndiye anokonzera secretory basa nhengo. Neonatal neonatal kuongorora rinoitwa (trypsin wevasungwa iri kuongororwa mune yaoma ropa nzvimbo). It chinodzidzisa zvakakwana uye ziya bvunzo. Kana muhari kwakaratidza yavaibvisa ions, kuiisa chlorine mu yakawanda, zvinokwanisika kutaura kuvapo chirwere rurefu mukana. Kana mukadzi, umo mhuri vaiva vanhu vane chirwere che "cystic fibrosis", vachitarisira mwana, vanachiremba vanoti kuongorora mashoko Líquido mvura pamusoro zera vhiki vanenge 18-20.

Mabatirwo cystic fibrosis

Zvinokosha kucherechedza kuti ndeyokubvisa chirwere hazvibviri. Zvisinei, mushonga unogona yekudyidzana kuvandudza upenyu yayo refu. Kare, varwere vakawanda yakafanana chirwere kufa vasati makore 20. Zvino, zvisinei, zvakakodzera uye nenguva kunokwanisa kurarama yekudyidzana yakareba. Intestinal chimiro kunoda chinokosha kudya. Food vanofanira akapfuma mapuroteni (hove, mazai). Uyezve yakatarwa uye mavhitaminzi yakaoma. Aidawo kugamuchirwa makemikari ( "Creon", "pantsitrat", "wokupembera" and said.). Zvinokosha kucherechedza kuti zvinodhaka izvi zvinofanira muupenyu hwake hwose. Chokwadi chokuti marapirwo anopa mugumisiro uchava anoratidza normalization pamusoro chetsoka, uremu uye kushayikwa kunyange kukura kwayo. Abdominal marwadzo kunyangarika, uye mutsvina yadzo zvisina dzakaonekwa nechekuita mukugarisana mafuta.

Kana pulmonary zvinodhaka chimiro chirwere panodiwa kuti zvichaita kufambisa liquefaction pamusoro sputum uye Árbol kudzorera functionality ( "Mukosolvin", "Mukaltin"). Zvakakosha zvikuru pakurapa pulmonary cystic fibrosis kudzivirira kukura zvinotapuriranwa pezvakaitwa mumapapu. Good zvazviri uye rinokosha kufema kurovedza. Zvinofanira kuitwa nguva dzose. Nokuti kunokwanisa kupiwa uye mishonga inorwisa mabhakitiriya. Kanganwa pamusoro chirwere kwenguva rebei achibatsira zvikuru nzira yakadaro, sezvo transplantation chepasi chemapapu. Zvisinei, rine zvainovhiringidzawo yayo: pangozi kurambwa, kwokubatsira mishonga tunodzivisa zvirwere. Mukuwedzera, murwere inofanira kuva utano nakei. Vazhinji kuisirwa izvi akaita kunze.

Kunenge kwaitwa kuti varwere cystic fibrosis

Vanachiremba vanoti zvakasimba kuti varwere chirwere ichi kuti yakakodzera majekiseni pamusoro whooping chikosoro, uye zvirwere zvakafanana. Zvakakosha kubvisa zvinogona allergens: inovaraidza dander, nezvifukidzo nemagumbeze kubva dzakafanana shiri. Zvinonzi zvakare zvachose hazvitenderwi kuti pusa kusvuta. chirwere kwakadaro kunoda Spa kurapwa vana. Kana mwero kwokuomerwa hosha hakukoshi, mwana kupinda chikoro, mitambo netsvimbo, ane zvakanaka kushingaira mararamiro. Mabatirwo cystic fibrosis muvana vane makore pasi gore rimwe kunosanganisira kushandisa dzinokosha wemisanganiswa (Dietta Extra, Dietta Plus). Uyezve, tinofanira kuwedzera uye chimwe dose kuti 1.5 nguva. Uyezve chakaunzwa nomwana zvinodyiwa shoma munyu (ichi kunokosha muzhizha).

Special kutarisirwa kuva anoshivirira Muterere. Zvinofanira kuva akapfuma mafuta (ruomba, ruomba, nyama) sezvo Absorção de Água muviri akavhuniwa. Kunwa vanodawo zvakawanda. Zvinokosha kuyeuka kuti kutora enzayemu gadziriro kunodiwa daily. Kuwedzera inokosha kunokwanisa kushandiswa, uye tsika mishonga. Hwezvekukurukurirana expectoration miriwo yakadai marshmallow, mai-uye-naamainini. Good zvainoita zvomudumbu ezvinhu ane voikukka, elecampane. Uye zvinokosha anonhuwirira rinoshandiswa kuti inhalations (lavender, Basil, nehisopi). Uyewo kubatsira uye kusimbisa zvinhu zvakadai uchi.

Zvaifungidzirwa

Zvinosuruvarisa kuti upenyu hunotarisirwa kuti kupiwa chirwere haisi yakakwirira chaizvo. Paavhareji, varwere kurarama kwemakore anenge 30 (mu Russia), kana kusvika 40 kana kupfuura (kunze). Zvisinei, nenguva chirwere uye kurapwa yekudyidzana kuvandudza murwere ezvinhu. Chinonyanya vanoshorwa fungidziro - iri kuratidzwa cystic fibrosis okutanga (mu vacheche). Asi zvinozivikanwa uye nyaya apo murwere nehosha zvakafanana akararama kwemakore anopfuura 70. musiyano uyu muupenyu hunotarisira kuRussia uye dzimwe nyika nokuda zvemari chokuita. Varwere mhiri kwemakungwa kutsigirwa pamusoro pehurumende upenyu-refu. Nokuda ichi vanogona kurarama sezvinongoita, kudzidza, kuti asike mhuri uye vane vana. Asi Russia haagoni zvakakwana kupa varwere mishonga zvakafanira (uye ichi enzayemu kugadzirira, uye anokosha nemishonga, uye mucolytics). Chete vashoma vana vanowana vakasununguka kurapwa nemishonga zvakakodzera. Varwere kugara chaiyo nhoroondo hwake hwose. Kuti haabatanidzi panowanikwa chirwere yakadai vanofanira kutanga geneticist pane imwe padanho nokuronga pamuviri.

Psychological mazano kuvabereki

A yakawanda mabhuku akagadzirirwa kutsigira vabereki vane mwana anotambura cystic fibrosis. Chokutanga, musavhunduka. Tinofanira kuwana ruzivo sezvo zvakawanda pamusoro chirwere kuti zvinobudirira kubatsira mwana akurire nemigumisiro yayo. Zvakakosha kuti kugara naye rudo wake.

The chirwere yekudyidzana chinobata kwete chete mumuviri ezvinhu, asi kune vamwe zvakadini pamusoro mupfungwa. Saka, zvinetso hazvingadziviswi (Zvisinei, ivo varipo uye zvokurera vana utano). Vamwe kuchinja zvinogona kuvimbwa kuita uye gotwe murwere. Nyanzvi dzinoti apo vana chete kwete kudzidza chirwere wavo, asi kutarisira pachavo, nani zvikuru kunzwa.

Vabereki havana kunzwa oga mukurwisa cystic fibrosis, unofanira kutaura nemhuri dzavo, vari nematambudziko akafanana dambudziko. Zvinogona kuitwa uye chinokosha Internet forum. Pane zvakawanda mari, vanogona kuenda zvose mupfungwa uye mune zvemari rubatsiro. Zvakakosha kuyeuka kuti chirwere chakadai - kwete mutongo. Vazhinji vanhu vakakurumbira kutambura kubva kuvabereki chirwere, asi izvi hazvina kuvatadzisa kubudirira muupenyu. Muimbi Gregory Lemarchal, enyambo mutambi Bob Flanagan (akararama kusvika makore 43) - izvi zvinongova mienzaniso sei kurarama uye kukura pamwe chirwere chakadai. Uyezve, mushonga hakuiti imire; imwe ongororo pose uchiitirwa pamusoro Gene kurapa pane cystic fibrosis muUnited States. Zvisinei, kana, kuti kukurira manzwiro avo akaoma, unogona nguva dzose kubatsirwa kubva wepfungwa.