Kartagener chirwere kuvana: chirwere, photo, kurapwa

Kartagener kuti chirwere - isingawanzowanikwi vachiberekwa chirwere yakakonzerwa kutyorwa cilia motility, riri pamusoro pemvura masero akawanda epithelial. The zvizere chimiro kuremara uku akaora kunoratidzwa ne vanamwari vatatu vatatu zviratidzo:

• bronchiectasis;
• hypoplasia (hypoplasia), paranasal sinuses, icho pamwe idzokororo sinusitis;
• zvinopesana neizvi urongwa zvomukati (Situs inversus).

The chirwere zita pashure Kartagener chiremba Swiss, uyo akapa hwakadzama rondedzero mamiriro muna 1933. Zvino, ngationei kurukura muudzame hukuru zvikuru chirwere.

Inokonzera Kartagener urwere

Zvino zvakava kuti Kartagener chirwere ine majini hunhu ane autosomal recessive kwadzinoita nhaka. Hwaro chirwere vari kuremara munyika dzinoverengeka majini kuti encode zvimwe mapuroteni, yakagadzirirwa nechokwadi mashandiro cilia.

Somugumisiro mufambo ravo ipfupi kana vakarasika zvachose - pane chirwere inonzi "chikuru ciliary dyskinesia." Adzidza kupfuura makumi maviri majini anokonzera kukura Kartagener syndrome. Kutyora basa chero kwavari utero kungatungamirira zvokurwara.

How zvakafanana ndiyo chirwere?

Sezvataurwa pamusoro apa, Kartagener chirwere kuvana hariwanikwi -only mune chimwe chete achangoberekwa kubva 16,000.

Zvinofanira kucherechedzwa kuti vana vanogona zvokurwara hakuiti unooneka, uye zvokuongorora anoitwa chete pashure pemwedzi mishomanana kana kunyange makore, kana kuva rakajeka kunotoda mufananidzo.

Zviratidzo Kartagener urwere

Kartagener chirwere zviratidzo anosiyana kubva pasina zviratidzo kuti vakasiyana kunotoda mufananidzo.

Kana asymptomatic yokuvira yakarurama chirwere zvakaoma zvikuru, uye rikauya kakawanda dzakaonekwa netsaona kana Thando zvomukati panguva imwe ongororo yakaitwa pamusoro mamwe zvirwere.

bronchiectasis

Kazhinji Árbol muti (huru chiedza puranga) ane chimiro branching tubules vane dzakafanana rutapudzo pamusoro dhayamita zviri lumen nenzira kubva overlying kuti dambudziko zvikamu chinoratidza korona zvachose-pasi.

Bronchiectasis (kana bronchiectasis) - iri munharaunda pakuwedzera lumen ari principales mhando mabhegi, spindles kana sepombi. In akaremara principales dzakadaro zvisisakwanisiki zvakajairika kufamba secretion. He stagnates, izvo vaizongoguma kutungamirira utachiona uye kukura anogumbura mhinduro.

Akaora, bronchiectasis pamwe Kartagener chirwere akaratidza chikosoro pamwe expectoration greenish purulent sputum, fivha kusvika 38 madhigirii uye yakakwirira, utera, nomusoro, dzimwe nguva unogona kubatana svoto uye kurutsa. Simba inorwisa mabhakitiriya kurapa achibvumira zvokunze kupora, asi mishonga izvi hazvigoni kubvisa chikuru chinokonzera kuzvimba vari emo bronchiectasis. Naizvozvo, chiremba mufananidzo anorondedzerwa kakawanda akadzokorora, rakatumbuka ne "chiedza" asymptomatic zvikamu. It anova refu uye kuramba tichishanda kosi. Chirwere relapsing nzira mamiriro rinonzi bronchiectasis.

sinusitis

Chimwe kuratidzwa Kartagener chirwere ndiye sinusitis, kana kuzvimba paranasal sinuses. Chaiye ciliary epithelium pamusoro nasal mucosa uye sinus nokuda nokufamba ayo anopa sangano secretion ngarigare pamusoro paro seguruva uye utachiona. Kartagener chirwere Kana cilia basa iri vasingadzoreki, uye zviri stagnates iri sinuses, zvichiita kuzvimba.

Kakawanda kuva sinusitis, kana kuzvimba maxillary sinuses, kanenge - mberi (frontal sinus), ethmoiditis (masero ane trellised labyrinth) uye sphenoiditis (sphenoid sinus). inoti zvose izvi kunoratidzwa nefivha, kutemwa uye runny mhino chete pus. Sinusitis zvakare hwokuratidza marwadzo kumativi wamapapiro mhuno uye pamatama.

The neizvi urongwa zvomukati

The neizvi urongwa zvomukati, kana Situs viscerusim ne - ichi ndicho chinonyanya hunhu kuoneka Kartagener urwere, izvo, zvisinei, rinowanikwa muna isingasviki pahafu varwere. Kuenda kufamba zvomukati maererano mhando mirroring. Kusiyanisa kwakakwana uye kukwana neizvi urongwa nhengo dzomukati.

Kukwana neizvi urongwa vari swapped mumapapu. Izvi angaperekedzwa sokunge girazi kuratidzwa mwoyo ine chaishanda kwayo rworudyi pamusoro pechipfuva mhango (dextrocardia).

With zvizere neizvi urongwa anobata girazi kufamba zvose zvomukati. Panyaya iyi, chiropa uri kuruboshwe, kuti chirwatata - kurudyi. Netsaona kuziva miedzo zvokurwara uyu Ultrasound panguva purogiramu kuongororwa uye anobvumira kufungidzira Kartagener chirwere kana asymptomatic.

The neizvi urongwa zvomukati imhaka kutyora yokutama masero embryo uye nyama panguva embryogenesis. masangano akawanda havawanzodi akakura zvokuti apo vari pashure aberekwa. Saka, itsvo vakaradzikwa pelvic nharaunda uye zvishoma nezvishoma musimukire padanho XI-XII mbabvu.

Kufambira (kana yokutama) kuti mitumbi panguva pamuviri nguva iri kuitwa cilia, Kartagener chirwere iyo kana asina kushanda, izvo zvinokonzera neizvi urongwa zvomukati. Sezvineiwo, sokunge kutyisidzira ichi haana kutarira, zviri kakawanda haitungamiriri nouchapupu kutyorwa upenyu muviri.

Other kuratidzwa Kartagener urwere

Pakati pezvimwe zviratidzo chirwere ndiyo inokosha mukono kushaya mbereko. It kunokonzerwa kutadza kufamba pamusoro dzisingaoneki nokuti kuti cilia dzavo rega kushanda.

Varwere zvinogona kuvhiringidza idzokororo nzeve noutachiona uye kusanzwa. Izvi nokuda kurega kunotanga kubva secretions pakati nzeve, izvo kazhinji anofanira kubviswa epithelial masero ciliated mucosal.

Kartagener chirwere kuongorora chirwere muvana

Kana mwana, kutanga kumativi zera mwedzi mumwe, anogara chirwere nemabayo, rhinitis uye sinusitis zvinofanira vanofungidzirwa Kartagener urwere, nechirwere izvo zvisiri kunzwisisa chaizvo. Rinosanganisira vanoverengeka richishanda uye rabhoritari zvidzidzo:

• ongororo mumuviri, kana vanhuwo kuongororwa navanachiremba, zvicharatidza kuoma nasal kufema, maduramazwi auscultatory mufananidzo mumapapu uye mwoyo, uyewo kuchinja chinouraya phalanges kuti "drumsticks" mhando iri refu nzira kwacho.
• X-ray kuongororwa acharatidza pathological maronda mumapapu uye neizvi urongwa mwoyo (dextrocardia). Nzira iyi ndiyo nyore uye ngozi zvakakwana, saka, anoita kuti tikwanise kuita chirwere kubva Kartagener chirwere muchipatara.
• Bronchoscopy - nzira yakarurama kwazvo okuongorora kuziva bronchiectasis. Uyezve, chete kuburikidza bronchoscopy kunogona kutora biopsy pakati Árbol mucosa.
• mucosal biopsies vanoratidza kwokuomerwa kushoma uye kuongorora cilia marongerwo.

Mukusarudza zvipatara uye mumayunivhesiti munyika vari kukurukura kwenguva refu nguva kare Kartagener urwere, mufananidzo zviri munyaya ino. Kuwana nyanzvi dzakakurumbira inogona kudzidzisa chizvarwa muduku vanachiremba vazive isingawanzowanikwi chirwere chakadai.

Vachiona Kartagener urwere

Ndingava kubvisa ichi? Pari, Mabatirwo Kartagener kuti chirwere kunobva zviratidzo. Hapana mushonga kuti aizodzosa kushanda cilia masero epithelial, asi mushonga ano ane akapfuma zvombo maturusi kuti zviite weboka chirwere. Uchibatsirwa wavo, murwere anogona kukanganwa pamusoro wake isingawanzowanikwi chirwere.

The chikuru nzira kurapwa:

• Mishonga. zvinodhaka aya nachiremba kuzvimba mumapapu akaita bronchiectasis, uye sinusitis. Shandisa chekare mishonga kuti penicillin, macrolides uye zvinodhaka kubva boka "kufema" fluoroquinolones.
• Nzira kuvandudza abudise kushanda principales - postural abudise, masaja, kushanda mucokinetic uye mucolytic zvinodhaka, etc ...
• Physiotherapy.

Kana kuvapo iziviswe bronchiectasis pamwe kazhinji idzokororo bronchitis uye nemabayo kushanda kurapwa - kubviswa (resection) The kupfuura njodzi nzvimbo yemapapu. Mushure yakadaro kuvhiyiwa mwoyo varwere akavandudzika substantially.