Kartagener syndrome kubantwana: sifo, photo, unyango

Kartagener kaThixo syndrome - isifo esinqabileyo naso esibangelwa kophulwa umthetho isiliya motility, ibekwe phezu iiseli ezininzi epithelial. Ifom ngokupheleleyo eli isiphako ngokwezonyango olwaboniswa oneziqu iimpawu:

• bronchiectasis;
• hypoplasia (hypoplasia), sinuses paranasal, nto leyo sikhatshwa sinusitis nezolo;
• ilungiselelo reverse enyama yangaphakathi (sokuSebenza inversus).

Esi sifo ithiywe emva Kartagener ugqirha Swiss, ngubani wabanika eneenkcukacha inkcazo karhulumente ngo-1933. Ngoku, makhe sixubushe ngokweenkcukacha ezininzi sifo.

Ubangela Kartagener syndrome

Ngoku kuvunyiwe ukuba Kartagener syndrome unalo uhlobo yemfuza kunye autosomal imo sofuzo zelifa. Isiseko kwesifo iziphako eziliqela izakhi encoder iiproteni ezithile, eyenzelwe ukuqinisekisa umsebenzi isiliya.

Ngenxa ukuhamba kwazo zancitshiswa okanye ezilahlekileyo kwaphela - kukho imeko ebizwa ngokuba "ciliary zaseprayimari zeDyskinesia." Sasifunda zofuzo ngaphezu amabini noxanduva lokuphuhlisa Kartagener syndrome. Ukophulwa umsebenzi nayiphi na kuzo Esibelekweni kunokukhokelela ngezifo.

Indlela eqhelekileyo esi sifo?

Njengoko kukhankanyiwe ngasentla, Kartagener syndrome ebantwaneni kunqabile kuphela xa usana enye kuphela ngaphandle 16,000.

Kufuneka kuqatshelwe ukuba abantwana Iisampulu akuthethi ukuzibonakalisa, kwaye uxilongo senziwa kuphela emva kweenyanga ezimbalwa okanye iminyaka, xa ukuphuhlisa umfanekiso eliqaqambileyo clinical.

Iimpawu Kartagener syndrome

Iimpawu syndrome Kartagener ziyahluka ukusuka akukho mpawu kumfanekiso abahlukene clinical.

Kwimeko isicwangciso nazimpawu uphethwe oluchanekileyo kunzima kakhulu, yaye kwenzeka amaninzi ukubonwa ngengozi xa uguqula izibilini ngexesha uphando malunga ethile nezinye izifo.

bronchiectasis

Ngokwesiqhelo, le umthi inyumoniya (isakhelo ukukhanya engundoqo) na uhlobo eyayizalwa tubules kunye ukuncitshiswa efanayo ubukhulu be mithambo kwicala ukusuka overlying kumacandelo ezona ifana oguqulweyo isithsaba.

Bronchiectasis (okanye bronchiectasis) - nokongezwa yasekuhlaleni mithambo ye bronchi uhlobo iibhegi, zokuluka okanye iisilinda. Xa bronchi likhubazeke ezinjalo kube nzima eqhelekileyo intshukumo secretion. Yena stagnates, nto leyo eya ekugqibeleni kukhokelela ekubeni usuleleko kunye nophuhliso impendulo arhabaxa.

Ngokwezonyango, bronchiectasis ne Kartagener syndrome wabonakalisa ukhohlokhohlo kunye expectoration ubovu ezibuhlazarha isikhohlela, ifiva ukuya degrees 38 nangaphezulu, ubuthathaka, intloko ebuhlungu, maxa wambi ukujoyina isicaphucaphu nokugabha. unyango enamandla zintsholongwane ivumela recovery yangaphandle, kodwa la machiza ayikwazi ukubeka ecaleni oyena nobangela ukudumba zezi bronchiectasis zasekuhlaleni. Ngoko ke, lo mfanekiso lwezonyango ekuthethwa ngazo apha ngokuphindaphindiweyo ngokuphindaphindiweyo, ngelinye kunye amathuba "ukukhanya" nazimpawu. Irubrikhi izifundo elide kwaye uthathe. Ikhosi relapsing Chronic imeko ekuthiwa bronchiectasis.

sinusitis

Enye ukubonakaliswa Kartagener syndrome na sinusitis, okanye ukudumba sinuses paranasal. Ngokwesiqhelo ciliary epithelium le isifo ngeempumlo kunye sinus ngenxa ukuhla yayo ukunika intshukumo secretion ndizihlalise phezu kuyo amasuntswana othuli neentsholongwane. Kartagener syndrome Xa umsebenzi isiliya kuphazamisekile, kwaye imixholo stagnates kwi sinuses, ebangela komtshiso oko.

Amaninzi ukuphuhlisa sinusitis, okanye ukudumba sinuses maxillary, ubuncinane - ephambili (sinus ngokungqalileyo), ethmoiditis (iiseli ze ekulo trellised) kunye sphenoiditis (sinus sphenoid). Zonke ezi States sibonakalalisiwe yi ifiva, intloko ebuhlungu kunye nempumlo esihambisayo kunye ubomvu. Sinusitis linophawu iintlungu emacaleni kwamaphiko impumlo kunye nemihlathi.

Amalungiselelo umva ngamalungu zangaphakathi

Amalungiselelo reverse enyama yangaphakathi, okanye isiza viscerusim versus - oku ukubonakalisa eyona uphawu Kartagener syndrome, leyo, Noko ke, kwenzeka ngaphantsi kwesiqingatha kwizigulane. Ukuya intshukumo ngamalungu angaphakathi ngokutsho uhlobo Ibukusa. Ukwahlula ilungiselelo epheleleyo kunye engaphelelanga reverse nezibilini.

ilungiselelo umva ezingagqitywanga ukuba uhlele nemiphunga. Oku kuhamba kunye, ngathi ngumfanekiso isibuko intliziyo kukho utshintsho yayo ecaleni ephezulu ngasekunene lweHo- kwesifuba (dextrocardia).

Ilungiselelo ngokupheleleyo reverse ubambe isipili ukuba onke amalungu zangaphakathi. Kulo mzekelo, isibindi ngasekhohlo, lo udakada - ilungelo. Ubhaqo ngengozi le ngezifo ultrasound ngexesha loviwo lwesiqhelo kwaye ivumela ukuba ukrokrela Kartagener syndrome xa nazimpawu.

Amalungiselelo reverse izibilini kungenxa kukwaphula yokufuduka iiseli osandul kunye nezihlunu ngexesha embryogenesis. arhente abaninzi ngokuqhelekileyo musa zikhula ukuya kwinqanaba apho emva kokuzalwa. Ngenxa yoko, zezintso babekwa kummandla zangasese yaye ngokuthe ngcembe kunyuka ukuya kwinqanaba XI-XII ezimbanjeni.

Ukushukumisa (okanye kwenye indawo) izidumbu ngexesha siswini ikhutshwa phandle isiliya, Kartagener syndrome leyo xa isebenza, nto leyo ebangela ukuba ilungiselelo reverse izibilini. Ngethamsanqa, ngokungathi zisongele le meko ayizange khangela, ukuba kwiimeko ezininzi akukho kungakhokelela ephule ebonakalayo ibubomi kumzimba.

Ezinye kubonakaliswa Kartagener syndrome

Phakathi kwezinye iimpawu zesi sifo yeyona ubudlolo ibalulekileyo eyindoda. Oku kubangelwa nokungakwazi madlozi ngenxa yokuba isiliya zabo musa ukusebenza.

Izigulane kwakhona uphazamise usulelo indlebe nezolo kunye nokulahlekelwa ukuva. Oku kungenxa kokoma of netuwa kwi zendlebe, leyo ngokwesiqhelo kufuneka isuswe iiseli ciliated epithelial mucosal.

Kartagener syndrome uphethwe ebantwaneni

Ukuba umntwana, ukususela ngeenxa ubudala kwinyanga enye, unesifo rhoqo yingqele, nokuthimliswa kunye sinusitis kufuneka kukrokrelwa Kartagener syndrome, uxilongo ingekuko inzima kakhulu. Oku kuquka inani lezifundo negalelo and laboratory:

• ukuxilongwa ngokwasemzimbeni, okanye uvavanyo lonyango zesiqhelo, ndibatyhilele nobunzima ngeempumlo ukuphefumla, umfanekiso auscultatory ezithile imiphunga kunye nentliziyo, kwakunye utshintsho phalanges ukuphela "drumsticks" uhlobo ngexesha elide inkqubo.
• iimviwo X-ray ndibatyhilele izitshanguba zophendlo lwezifo emiphungeni kunye nelungiselelo umva intliziyo (dextrocardia). Le ndlela ilula kwaye ekhuselekileyo ngokwaneleyo, ngoko ke, kwenza ukuba kungenzeka ukuba afeze une Kartagener syndrome esibhedlele.
• Bronchoscopy - indlela echanekileyo yophando ekubhaqeni bronchiectasis. Ngaphezu koko, kuphela ngokusebenzisa bronchoscopy kungathatha biopsy le isifo inyumoniya.
• biopsies mucosal ebonisa ubukhali inkqubo yokukrala nokuvavanya nesakhiwo isiliya.

izibhedlele Ukukhokela kunye neeyunivesiti jikelele axoxa kakhulu ixesha elide sele Kartagener syndrome, ifoto apho thaca kweli nqaku. Amava izazi edume ngayo ukuqeqesha isizukulwana omnci oogqirha ukuqaphela isifo esinqabileyo enjalo.

Ukuphatha Kartagener syndrome

Njani ukulahla le meko? Okwangoku, unyango Kartagener engqondweni kuxhomekeke iimpawu. Akukho yeza eyayiza ukubuyisela umsebenzi isiliya iiseli epithelial, kodwa iyeza mihla linembali yokugcina izixhobo ukwenza lula indlela yalo sifo. Ngoncedo lwabo, isigulane usenokulibala kwisigulo sakhe ezinqabileyo.

Iindlela eziphambili zonyango:

• Antibiotics. La machiza lalathelwe ukudumba imiphunga yabangela bronchiectasis, kunye sinusitis. Faka classical antibiotics penicillin, macrolides kunye neziyobisi ukusuka iqela 'zemiphunga "fluoroquinolones.
• Iindlela yokuphucula umsebenzi kutsalwe le bronchi - yokuhambisa amanzi postural, yokuthanjiswa, isicelo iziyobisi mucokinetic kwaye mucolytic, etc ...
• Ukuyifundisa.

Xa ubukho bronchiectasis ucaca bronchitis nezolo rhoqo kunye nonyango yentsebenziswano pneumonia - ukususwa (resection) Le ndawo uchaphazelekayo kwemiphunga. Emva i enjalo imeko nokusebenza izigulane yaphucuka kakhulu.