Kartagener syndrome nyob rau hauv cov me nyuam: mob, yees duab, kev kho mob

Kartagener lub syndrome - ib tug tsawg congenital kab mob tshwm sim los ntawm ib tug ua txhaum ntawm cilia kom, nyob rau ntawm qhov chaw uas muaj ntau epithelial hlwb. Cov tag nrho daim ntawv ntawm no defect yog clinically manifested los ntawm ib tug triad ntawm cov tsos mob:

• bronchiectasis;
• hypoplasia (hypoplasia), paranasal sinuses, uas yog nrog los ntawm recurrent sinusitis;
• qhov rov qab kho ntawm lub hauv nruab nrog cev (situs inversus).

Lub syndrome yog muaj npe tom qab Kartagener Swiss tus kws kho mob, uas tau muab ib tug ncauj lus kom ntxaws piav qhia ntawm lub xeev nyob rau hauv 1933. Tam sim no, wb sib tham txog nyob rau hauv ntau dua kom meej tus kab mob no.

Ua Kartagener syndrome

Tam sim no nws yog muaj pov thawj hais tias tus Kartagener syndrome muaj ib tug kev tshuaj ntsuam genetic xwm nrog ib tug autosomal recessive hom ntawm qub txeeg qub teg. Lub hauv paus ntawm tus kab mob yog tsis xws luag nyob rau hauv ib tug xov tooj ntawm cov noob uas encode tej cov nqaijrog, tsim los kom cov kev ua ntawm cilia.

Raws li ib tug tshwm sim ntawm lawv muaj yog txo los yog poob ntawm tag nrho - muaj yog ib tug mob hu ua "tus thawj kws ciliary dyskinesia." Kawm ntau tshaj nees nkaum noob lub luag hauj lwm rau txoj kev loj hlob ntawm Kartagener syndrome. Kev ua txhaum ntawm lub chaw ua hauj lwm ntawm tej yam ntawm lawv nyob rau hauv utero tej zaum yuav ua rau pathology.

Ntau npaum li cas yog muaj tus kab mob?

Raws li hais saum toj no, Kartagener syndrome nyob rau hauv cov me nyuam yuav tsis tshua muaj -only nyob rau hauv tsuas yog ib qho me nyuam mos tawm ntawm 16,000.

Nws yuav tsum tau muab sau tseg tias cov me nyuam tej zaum yuav Pathology tsis manifest nws tus kheej, thiab tus mob no yog ua los tsuas yog tom qab ib tug ob peb lub hlis los yog txawm xyoo, thaum tsim ib tug kaj soj ntsuam daim duab.

Cov tsos mob ntawm Kartagener syndrome

Kartagener syndrome cov tsos mob sib txawv los ntawm tsis muaj cov tsos mob rau ib cov kev soj ntsuam daim duab.

Nyob rau hauv cov ntaub ntawv ntawm asymptomatic chaw kho mob yog ib yam nyuaj heev, thiab tshwm sim feem ntau kuaj tau los ntawm lub caij nyoog thaum reversing lub hauv nruab nrog cev thaum lub sij hawm ib tug daim ntawv ntsuam xyuas txog ib co lwm yam kab mob.

bronchiectasis

Feem ntau, lub bronchial tsob ntoo (lub ntsiab teeb ncej) muaj hauv daim ntawv ntawm branching tubules nrog niaj hnub yuav txo tau ntawm txoj kab uas hla ntawm lub lumen nyob rau hauv cov kev taw qhia los ntawm cov overlying rau qhov pib seem uas tsa ib tug upside-down yas.

Bronchiectasis (los yog bronchiectasis) - ib lub zos expansion ntawm lub lumen ntawm lub bronchi hom hnab, koob los yog thooj voos kheej kheej. Nyob rau hauv xws deformed bronchi yuav tsis yooj yim sua qub zog secretion. Nws stagnates, uas yuav inevitably ua rau tus kab mob thiab cov kev loj hlob ntawm lub inflammatory teb.

Clinically, bronchiectasis nrog Kartagener syndrome manifested hnoos nrog expectoration greenish purulent hnoos qeev, ua npaws mus rau 38 degrees thiab ntau dua, tsis muaj zog, mob taub hau, tej zaum yuav tuaj koom nrog kev xeev siab thiab ntuav. Haib tshuaj tua kab mob pub rau lwm rov qab, tab sis cov tshuaj yuav tsis tshem tawm cov tseem ceeb ua o yog lub zos bronchiectasis. Yog li ntawd, tus soj ntsuam daim duab piav pheej hais dua, alternating nrog lub "teeb" asymptomatic Team sib. Nws ua ib tug ntev thiab protracted kawg. Mob relapsing chav kawm ntawm lub mob hu ua bronchiectasis.

sinusitis

Lwm manifestation ntawm Kartagener syndrome yog sinusitis, los yog o ntawm lub paranasal sinuses. Nyob rau hauv ib txwm ciliary epithelium ntawm lub qhov ntswg mucosa thiab qhov ntswg vim nws hloov mus hloov los muab lub zog secretion khom rau nws nrog plua tshauv hais thiab cov kab mob. Kartagener syndrome Thaum cilia muaj nuj nqi yog kev zoo, thiab tus txheem stagnates nyob rau hauv lub sinuses, ua o.

Feem ntau cov feem ntau tsim sinusitis, los yog o ntawm lub maxillary sinuses, yam tsawg kawg - pem hauv ntej (frontal qhov ntswg), ethmoiditis (hlwb ntawm ib tug trellised labyrinth) thiab sphenoiditis (sphenoid qhov ntswg). Tag nrho cov xeev no tau raug ua los ntawm kub taub hau, mob taub hau thiab los ntswg qhov ntswg nrog kua paug. Sinusitis yog tseem yus muaj los ntawm qhov mob ntawm lub sab ntawm cov tis ntawm lub qhov ntswg thiab lub puab tsaig.

Cov rov qab kho ntawm hauv nruab nrog cev

Cov rov qab kho ntawm lub hauv nruab nrog cev, los yog situs viscerusim tiv tiag - qhov no yog feem ntau cov yam ntxwv ntawm tus manifestation ntawm Kartagener syndrome, uas, li cas los xij, tshwm sim nyob rau hauv tsawg tshaj li ib nrab ntawm cov neeg mob. Mus zog ntawm hauv nruab nrog cev raws li lub hom mirroring. Paub qhov txawv kom tiav thiab tsis tiav rov qab kho ntawm hauv nruab nrog cev.

Uas ua tsis tiav rov qab kho yog swapped ntsws. Qhov no tej zaum yuav nrog, raws li nws yog ib tug iav rog xav txog ntawm lub plawv nrog ib tug ua haujlwm nyob rau hauv nws cov sab saum toj sab xis ntawm lub hauv siab muaj kab noj hniav (dextrocardia).

Nrog rau tag nrho rov qab kho tuas rau hauv daim iav kom tag nrho cov hauv nruab nrog cev. Nyob rau hauv cov ntaub ntawv no, lub siab yog nyob rau sab laug, tus po - txoj cai. Accidental nrhiav kom tau ntawm no pathology los ntawm ultrasound thaum lub sij hawm ib tug niaj hnub soj ntsuam thiab tso cai rau kom xav Kartagener syndrome thaum asymptomatic.

Cov rov qab kho ntawm lub hauv nruab nrog cev yog vim ua txhaum ntawm lub tsiv teb tsaws ntawm embryonic hlwb thiab cov nqaij mos thaum lub sij hawm embryogenesis. Muaj ntau cov koom haum feem ntau tsis tsim mus rau kis twg lawv yog tom qab lub hnub yug. Yog li, lub raum pw nyob rau hauv lub pelvic cheeb tsam thiab maj sawv mus rau theem XI-XII ribs.

Tsiv (los yog tsiv teb tsaws) ntawm lub cev thaum lub sij hawm thaum lub sij hawm yog nqa tawm los ntawm cilia, Kartagener syndrome uas thaum tsis tau hauj lwm, uas ua rau ib tug rov qab kho ntawm lub hauv nruab nrog cev. Qhov zoo ces, raws li yog hais hem tus mob no tsis tau saib, nws yog nyob rau hauv feem ntau tus neeg mob tsis ua rau ntau yam pauv loj ua txhaum ntawm lub neej ntawm tus kab mob.

Lwm yam ces Kartagener syndrome

Cov lwm tsos mob ntawm tus kab mob no yog cov tseem ceeb tshaj plaws txiv neej ntxiv lawm tshob. Nws yog tshwm sim los ntawm immobility ntawm cov phev vim lub fact tias lawv cilia tsis muaj nuj nqi.

Cov neeg mob yuav kuj thab recurrent mob pob ntseg thiab tsis hnov lus. Qhov no yog vim lub stagnation ntawm kua nyob rau hauv nruab nrab pob ntseg, uas feem ntau yuav tsum tau muab tshem tawm ciliated epithelial mucosal hlwb.

Kartagener syndrome mob nyob rau hauv cov me nyuam

Yog hais tias ib tug me nyuam, pib ib ncig ntawm lub hnub nyoog ntawm ib lub hlis, tsis tu ncua suffers los ntawm mob ntsws, txhaws ntswg thiab sinusitis yuav tsum tau xav tias Kartagener syndrome, tus mob uas yog tsis heev tham. Nws muaj xws li ib tug xov tooj ntawm instrumental thiab kuaj kev tshawb fawb:

• Kuaj ib ce, los yog dog dig kev xeem, yuav qhia cov teeb meem ntawm qhov ntswg ua tsis taus pa, cov kev auscultatory daim duab nyob rau hauv lub ntsws thiab lub plawv, zoo li cov kev hloov hauv davhlau ya nyob twg phalanges ntawm "drumsticks" hom nyob rau hauv lub ntev chav kawm ntawm lub txheej txheem.
• X-ray xeem yuav qhia cov pathological txhab nyob rau hauv lub ntsws thiab lub rov qab kho ntawm lub plawv (dextrocardia). Qhov no txoj kev yog yooj yim thiab muaj kev nyab xeeb txaus, yog li ntawd, ua rau nws tau mus nqa tawm mob ntawm Kartagener syndrome nyob rau hauv lub tsev kho mob.
• Bronchoscopy - lub feem ntau muaj tseeb qauv ntawm cov kev tshawb nrhiav mus ntes bronchiectasis. Ntxiv mus, tsuas yog los ntawm txoj kev bronchoscopy yuav coj ib tug me ntawm lub bronchial mucosa.
• mucosal biopsies qhia cov heev ntawm lub inflammatory txheej txheem thiab ntsuam xyuas cilia qauv.

Ua tsev kho mob thiab universities nyob ib ncig ntawm lub ntiaj teb no yog tham txog ib tug heev lub sij hawm ntev twb Kartagener syndrome, ib tug yees duab ntawm cov uas yog nyob rau hauv no tsab xov xwm. Ua tus nto moo tshaj plaws cov kws txawj nws yuav qhia cov hluas tiam ntawm cov kws kho mob kom paub txog xws li ib tug tsawg tus kab mob.

Kho Kartagener syndrome

Kuv tau tshem ntawm tus mob no? Tam sim no, kev kho mob ntawm Kartagener lub syndrome nyob ntawm cov tsos mob. Muaj yog tsis muaj cov tshuaj uas yuav rov qab kho cov nuj nqi ntawm lub cilia ntawm epithelial hlwb, tab sis niaj hnub tshuaj muaj ib tug nplua nuj arsenal ntawm cov cuab yeej los pab txhawb rau thaum kawg ntawm tus kab mob no. Nrog lawv pab, tus neeg mob tej zaum yuav tsis nco qab txog nws tsawg mob.

Lub ntsiab txoj kev kho mob:

• Tshuaj tua kab mob. Cov tshuaj kws kho rau o ntawm lub ntsws tshwm sim los bronchiectasis, thiab sinusitis. Thov classical tshuaj tua kab mob penicillin, macrolides thiab tshuaj los ntawm cov pab pawg neeg ntawm "ua pa" fluoroquinolones.
• Txoj kev ntawm kev txhim kho cov kua muaj nuj nqi ntawm lub bronchi - postural kua, massage, daim ntawv thov mucokinetic thiab mucolytic tshuaj, thiab lwm yam ...
• Physiotherapy.

Thaum lub xub ntiag ntawm cov lus hais bronchiectasis nrog nquag recurrent ntsws thiab mob ntsws muaj dej operative kev kho mob - tshem tawm (resection) Lub feem ntau cuam tshuam rau thaj tsam ntawm lub ntsws. Tom qab xws li ib lub lag luam kev mob ntawm tus neeg mob khees me ntsis lawm tshuam.