Kartagener's syndrome yog ib yam kab mob uas tsis tshua muaj tshwm sim los ntawm kev ua haujlwm tsis zoo ntawm cilia nyob rau saum npoo ntawm ntau lub hlwb epithelial. Nyob rau hauv nws daim ntawv tag nrho, qhov tsis xws luag yog kuaj pom los ntawm triad ntawm cov tsos mob:
- bronchiectasis;
- kev txhim kho (hypoplasia) ntawm paranasal sinuses, uas yog nrog los ntawm kev rov ua dua sinusitis;
- reverse arrangement of internal organ (siteus inversus).
Tus mob no muaj npe tom qab tus kws kho mob Swiss Kartagener, uas tau muab cov lus piav qhia ntxaws txog tus mob hauv xyoo 1933. Tam sim no cia peb tham txog tus kab mob kom ntxaws ntxiv.
Causes of Kartagener's Syndrome
Tam sim no, nws tau raug pov thawj tias Kartagener's syndrome muaj cov caj ces nrog rau hom autosomal recessive yam qub txeeg qub teg. Tus kab mob no yog raws li qhov tsis xws luag ntawm ntau cov noob uas encode qee cov proteins uas tsim los muab kev ua haujlwm ntawm cilia.
Vim li ntawd, lawv txoj kev mus los tau txo qis lossis ploj tag nrho - ib qho mob hu ua "primary ciliary dyskinesia" tshwm sim. Ntau tshaj nees nkaum cov noob lub luag haujlwm rau kev loj hlob ntawm Kartagener's syndrome tau kawm. Ua txhaum hauvKev ua hauj lwm ntawm ib qho ntawm lawv nyob rau lub sij hawm ntawm kev loj hlob intrauterine tuaj yeem ua rau pathology.
Tus kab mob no tshwm sim li cas?
Raws li tau hais los saum no, Kartagener syndrome hauv cov menyuam yaus tsis tshua muaj - tsuas yog ib tus menyuam yug tshiab hauv 16,000.
Nws yuav tsum tau muab sau tseg tias hauv cov menyuam mos cov kab mob yuav tsis tshwm sim nws tus kheej hauv ib txoj kev, thiab kev kuaj mob tsuas yog tom qab ob peb lub hlis thiab ntau xyoo, thaum daim duab kho mob tshwm sim.
Cov tsos mob ntawm Kartagener's Syndrome
Kev tshwm sim sab nraud ntawm Kartagener's syndrome sib txawv los ntawm qhov tsis muaj cov tsos mob ua tiav mus rau cov duab pom zoo.
Rau cov chav kawm asymptomatic, qhov kev kuaj mob kom raug yog qhov nyuaj heev thiab feem ntau tshwm sim los ntawm kev sib tsoo thaum pom qhov chaw rov qab ntawm lub cev hauv nruab nrog cev thaum kuaj xyuas qee yam kab mob.
Bronchiectasis
Feem ntau, tsob ntoo bronchial (lub ntsiab lus ntawm lub ntsws) zoo li cov tubules uas muaj qhov sib txawv hauv txoj kab uas hla ntawm lawv cov lumen nyob rau hauv kev coj los ntawm cov seem overlying mus rau hauv lub hauv paus, uas zoo li ib tug inverted crown.
Bronchiectasias (los yog bronchiectasis) yog lub zos expansions ntawm lub bronchial lumen nyob rau hauv daim ntawv ntawm lub hnab, spindles los yog cylinders. Nyob rau hauv xws li deformed bronchi, ib txwm txav ntawm qhov zais cia yuav ua tsis tau. Nws stagnates, uas inevitably ua rau tus kab mob thiab kev loj hlob ntawm ib tug inflammatory tshuaj.
kho mob, bronchiectasis hauv Kartagener's syndrome yog tshwm sim los ntawm hnoos nrog tawm ntawm ntsuab purulenthnoos qeev, kub taub hau txog 38 degrees thiab siab dua, tsis muaj zog, mob taub hau, qee zaum xeev siab thiab ntuav tuaj yeem koom nrog. Cov tshuaj tua kab mob muaj zog tso cai rau koj kom ua tiav kev rov zoo sab nraud, tab sis cov tshuaj zoo li no yuav tsis tuaj yeem tshem tawm qhov ua rau mob loj - hauv zos nthuav dav ntawm bronchi. Yog li ntawd, daim duab piav qhia kev kho mob yog rov ua dua, alternating nrog "lub teeb" asymptomatic intervals. Nws kis tau ib tug ntev thiab protracted ntws. Cov kab mob rov qab mus ntev ntawm tus mob no feem ntau hu ua bronchiectasis.
Lwm qhov tshwm sim ntawm Kartagener's syndrome yog sinusitis, lossis o ntawm paranasal sinuses. Nquag, cilia ntawm lub epithelium ntawm cov mucous daim nyias nyias ntawm lub qhov ntswg thiab sinuses, vim lawv vibrations, xyuas kom meej lub zog ntawm lub zais cia nrog cov hmoov av thiab cov kab mob uas tau tswm ntawm nws. Hauv Kartagener's syndrome, kev ua haujlwm ntawm cilia yog qhov tsis zoo, thiab cov ntsiab lus stagnate hauv paranasal sinuses, ua rau mob.
Feem ntau feem ntau tshwm sim sinusitis, lossis o ntawm maxillary sinuses, tsawg zaus - frontal sinusitis (frontal sinus), ethmoiditis (cells ntawm ethmoid labyrinth) thiab sphenoiditis (sphenoid sinus). Tag nrho cov mob no yog tshwm sim los ntawm kub taub hau, mob taub hau thiab los ntswg qhov ntswg nrog tso kua paug. Sinusitis kuj tshwm sim los ntawm qhov mob ntawm ob sab ntawm tis ntawm lub qhov ntswg thiab hauv cov pob txha.
Rov qab lub cev hauv nruab nrog
Qhov kev rov qab los ntawm cov kab mob hauv nruab nrog cev, lossis situs viscerusim piv yog qhov tshwm sim zoo tshaj plaws ntawm tus mob. Kartagener, uas, txawm li cas los xij, tshwm sim tsawg dua ib nrab ntawm cov neeg mob. Muaj kev txav ntawm cov kabmob hauv nruab nrog cev raws li hom ntawm lawv daim iav duab. Muab faib ua kom tiav thiab tsis tiav rov qab kho ntawm lub cev hauv nruab nrog cev.
Thaum qhov kev thim rov qab tsis tiav, lub ntsws hloov chaw. Qhov no yuav ua tau nrog ib hom iav duab ntawm lub plawv nrog kev hloov ntawm nws apex mus rau sab xis ib nrab ntawm lub hauv siab kab noj hniav (dextrocardia).
Nrog rau kev rov qab tag nrho, muaj daim iav txav ntawm txhua lub cev hauv nruab nrog cev. Hauv qhov no, daim siab nyob rau sab laug, tus po - sab xis. Kev tshawb pom qhov tshwm sim ntawm cov kab mob no thaum lub sij hawm ultrasound thaum lub sij hawm kuaj xyuas niaj hnub thiab tso cai rau xav tias Kartagener's syndrome hauv nws cov chav kawm asymptomatic.
Kev rov qab kho ntawm cov kabmob hauv nruab nrog cev yog piav qhia los ntawm kev ua txhaum ntawm kev tsiv teb tsaws ntawm embryonic hlwb thiab cov ntaub so ntswg hauv embryogenesis. Ntau lub cev ib txwm tsis loj hlob nyob rau tib qhov chaw uas lawv nyob tom qab yug me nyuam. Yog li ntawd, lub raum muab tso rau hauv lub plab mog thiab maj mam nce mus rau theem ntawm lub XI-XII tav.
Kev txav mus los (lossis kev tsiv teb tsaws) ntawm cov kabmob hauv lub cev xeeb tub yog ua los ntawm cilia, uas tsis ua haujlwm hauv Kartagener's syndrome, uas ua rau rov qab kho cov kabmob sab hauv. Hmoov zoo, txawm tias qhov kev hem thawj zoo li no zoo li cas, feem ntau ntawm cov xwm txheej nws tsis ua rau muaj kev cuam tshuam loj ntawm lub cev lub luag haujlwm tseem ceeb.
Lwm yam tshwm sim ntawm Kartagener's syndrome
Ntawm lwm cov tsos mob ntawm tus kab mob, qhov tseem ceeb tshaj plawsyog txiv neej infertility. Nws yog tshwm sim los ntawm immobility ntawm cov phev vim lub fact tias lawv flagella tsis ua hauj lwm.
Cov neeg mob kuj tuaj yeem hnov mob otitis media thiab hnov lus tsis zoo. Qhov no yog vim lub stagnation ntawm qhov zais cia nyob rau hauv nruab nrab pob ntseg, uas ib txwm yuav tsum tau muab tshem tawm los ntawm cilia ntawm lub epithelial hlwb ntawm mucous daim nyias nyias.
Kev kuaj mob ntawm Kartagener syndrome hauv cov menyuam yaus
Yog ib tug me nyuam, pib txij li ib hlis, tsis tu ncua los ntawm mob ntsws, dej ntswg thiab sinusitis, Kartagener's syndrome yuav tsum tau xav tias, qhov kev kuaj mob uas tsis nyuaj heev. Nws suav nrog ntau txoj hauv kev tshawb fawb thiab kev sim:
- Kev kuaj lub cev, lossis kev kuaj mob niaj hnub, yuav qhia pom qhov ua tsis taus pa ntawm lub qhov ntswg, ib daim duab auscultatory tshwj xeeb ntawm lub ntsws thiab lub plawv, nrog rau kev hloov pauv ntawm lub davhlau ya nyob twg phalanges ntawm tus ntiv tes zoo li "drumsticks" nrog ntev. chav kawm ntawm tus txheej txheem.
- X-ray kuaj yuav qhia pom cov kab mob foci hauv lub ntsws thiab qhov chaw rov qab ntawm lub plawv (dextrocardia). Txoj kev no yooj yim thiab muaj kev nyab xeeb txaus, yog li ntawd, nws ua rau nws tuaj yeem kuaj xyuas Kartagener's syndrome hauv tsev kho mob maternity.
- Bronchoscopy yog txoj kev kuaj xyuas qhov tseeb tshaj plaws rau kev kuaj mob bronchiectasis. Tsis tas li ntawd xwb, tsuas yog los ntawm bronchoscopy tuaj yeem kuaj pom ntawm bronchial mucosa.
- Ib qho mucosal biopsy yuav qhia qhov hnyav ntawm cov txheej txheem inflammatory thiab tshuaj xyuas cov qauv ntawm cilia.
Top chaw kho mob thiabCov tsev kawm qib siab hauv ntiaj teb tau sib tham txog Kartagener's syndrome tau ntev heev, cov duab uas tau nthuav tawm hauv kab lus no. Qhov kev paub ntawm cov kws tshaj lij uas muaj npe nrov tshaj plaws ua rau nws muaj peev xwm cob qhia cov tub ntxhais hluas ntawm cov kws kho mob kom paub txog tus kab mob tsawg tsawg.
Kev Kho Mob Kartagener's Syndrome
Puas muaj peev xwm tshem tau tus mob no? Tam sim no, kev kho mob rau Kartagener syndrome nyob ntawm cov tsos mob. Tsis muaj cov tshuaj uas yuav rov ua haujlwm ntawm cilia ntawm cov hlwb epithelial, txawm li cas los xij, cov tshuaj niaj hnub muaj cov cuab yeej nplua nuj ntawm cov cuab yeej uas txo cov kab mob. Nrog lawv pab, tus neeg mob tuaj yeem hnov qab txog nws tus kab mob tsis tshua muaj sijhawm ntev.
Kev kho mob tseem ceeb:
- Tshuaj tua kab mob. Cov tshuaj no tau sau tseg rau kev mob ntsws ntawm lub ntsws vim bronchiectasis thiab sinusitis. Cov tshuaj tua kab mob classic ntawm penicillin series, macrolides, nrog rau cov tshuaj los ntawm cov pab pawg "ua pa" fluoroquinolones yog siv.
- Txoj kev uas txhim kho bronchial kua - postural kua, zaws, siv cov tshuaj mucolytic thiab mucokinetic, thiab lwm yam.
- Physiotherapy.
Nyob rau hauv lub xub ntiag ntawm pronounced bronchiectasis nrog feem ntau recurrent bronchitis thiab pneumonia, kev kho mob phais yog qhia - tshem tawm (resection) ntawm cov feem ntau cuam tshuam cheeb tsam ntawm lub ntsws. Tom qab ua haujlwm zoo li no, cov neeg mob zoo dua qub.
