Kwaziwa nangokuthi: i- PCD, i-PCD kunye ne-situs solitus kunye ne-immotile cilia syndrome
I-ciliary priysary dyskinesia (i-PCD) yintlupheko yesifo somzimba esichaphazelekayo echaphazelekayo malunga naba-1 kubantu abayi-16,000. I-Cilia iinjongo ezinjengezinwele ezinqamle amacandelo athile omzimba njengombhobho we- eustachian kunye ne-trachea. I-Cilia isebenze umsebenzi obalulekileyo wokuthutha i-mucous kunye nezinye izinto ezivela ngaphandle ngaphandle kwezitho eziza kususwa emzimbeni.
Kwi-PCD, i-cilia ayisasebenzi ngokuqhelekileyo nto ebangela ukulibaziseka ekukhutsheni i-debris okwandisa ingozi yokusuleleka. Kwi-PCD izitho zesisu nesifuba zisesikweni sabo esifanelekileyo.
Kukho neentlobo ze-PCD ezifana ne-Kartagener's syndrome (i-inverus totalisis), ene-PCD, kodwa nayo ibonakalisa iinkalo zamalungu achaphazelekayo emzimbeni. Ngokomzekelo endaweni yepen ekhohlo lwasemzimbeni , lusekunene lomzimba. Ezinye izitho ezichaphazelekayo yi-Kartagener's syndrome ziquka: intliziyo, isibindi, namathumbu. I-Kartagener's syndrome yinto engavumelekanga kunye nesiganeko se-1 kubantu abangama-32,000.
Izinto ezijongene nobungozi kwiDyskinesia yeCiliary Primary
I-ciliary priyary dyskinesia ayiyona ingxaki echaphazelekayo. Unokufumana i-PCD kuphela xa uzalwa ukuba ngaba bobabini abazali bakho bane-disorder okanye baphethe i-disorder. Kuqhelekile ukuba ngumphathiswa we-PCD kuba unomzekelo we- autosomal .
Oku kuthetha ukuba xa omnye umzali ekunika i-gene ejongene ne-PCD kodwa omnye umzali akanikeli i-gene, awuyi kuba nefa le-PCD kodwa uyaziwa ngokuba ngumthwali.
Kukho izifo ezininzi zephene ezingabangela i-PCD nangona ungeke ukwazi ukuxilonga abathwali be-ciliary dyskinesia.
Nayiphina imfuza echaphazela iiprotheni ze-cilia inokubangela ukuba i-PCD iqhube. Ukutshintshela kwiiprotheyini ezinxulumene ne-cilia kunokunciphisa, ukwandisa, okanye umise ukunyakaza okunjengomtsalane okuhambisana nomsebenzi weciliary oqhelekileyo. Utshintsho kumsebenzi we-cilia lunokubangela ukuba iimpawu ezilandelayo zihlobene ne-ciliary dyskinesia:
- ukuxinwa kwamasipha angapheliyo, i- rhinitis , okanye i- sinus infections
- engapheliyo ye-otitis media ( izifo zendlebe ) okanye ukulahlekelwa kwindlebe
- ukungabikho
- Izifo eziphefumula rhoqo ezifana ne-pneumonia
- ukukhwehlela
- i-atelectasis (iphosi epholileyo)
Ukuchonga i-Ciliary Primary Dyskinesia
Ugqirha wakho akakwazi ukujonga ngokuzenzekelayo i-ciliary dyskinesia, njengokuba iimpawu ezinxulumene neengxaki ezininzi ezinokubonwa kubantwana kunye nabantu abadala. Xa i-inversus totalis (izitho ezingekho ngokwempawu kumacandelo athile omzimba) ziphawulwa, ukuxilongwa kungaba lula. Nangona kunjalo, xa ilungu libekwa ngokuqhelekileyo, ugqirha uya kufuneka ukuba enze iimvavanyo ezongezelelweyo. Uvavanyo lwe-Genetic ngenye yezindlela eziphambili ezisetyenziselwa ukuxilonga i-PCD, nangona kukho iindlela ezimbini eziqhelekileyo ezisetyenziselwa ukuvavanya iingxaki nge-cilia yakho: i- electron kunye ne-microscopy yevidiyo . Umehluko phakathi kweemvavanyo ezimbini luhlobo lwe-microscope esetyenzisiweyo.
Zomibini iimvavanyo zidinga ugqirha wakho ukuba athathe isampuli kwi-cality yakho okanye kwindlela yokuhamba emzimbeni ukuhlalutya phantsi kwe-microscope.
Izaziso ezinamathele ezincani ezincinci, ezibizwa ngokuba ngama-particle-shafted particles , zinganyanzeliswa. Ugqirha wakho uya kuhlola ukuba mangaphi amaqhekeza abuyele ngexesha lokuphuma. Xa kuncinci ukuhlawulela iinqununu, iingxaki ze-ciliary ziyakucingelwa. Ugqirha wakho unokukwazi ukuba unyuse i-nitric oxide . Olu vavanyo aluqondwa kakuhle, nangona xa uphumelela ngaphantsi kweziphumo eziqhelekileyo, i-PCD inokukrokrelwa.
Ukusebenza ngokuqhelekileyo cilia kuyafuneka kwakhona kwinkqubo yokuzala enempilo.
Ngenxa yezinga lokungasebenzi kwecilia kwindlela yokuzala, ukuhlalutya isisu kungabonakala kuncedo ekuhloleni i-PCD kubantu abadala. Isampuli yesityalo ihlaziywa phantsi kwe-microscope.
Umgangatho-golide wokuvavanya i-electron microscopy. Oku kucaca ngokucacileyo ukuba ngaba ayenjalo okanye ayisebenzanga kakuhle kunye ne-cilia. I- ENT yakho inokuthatha isampuli ukusuka kwipumlo yakho okanye kwi-airways yakho ukufumana isampuli kule vavanyo. Ukuvavanya i-genetic kunokuxilongwa, nangona kunjalo kuphela ama-60 ekhulwini amatyala e-PCD aneenkcukacha zokubhala ezibonakalayo ezibonakalayo.
Unyango lwe-Ciliary Dyskinesia
Akukho nonyango kwi-ciliary dyskinesia. Unyango luhlobene nokulawula iimpawu kunye nokuzama ukuthintela usulelo. Ukuze uncede ukukhusela izifo zendlebe, i-ENT yakho inokubeka i-tubes indlebe ukuvumela ukuba iindlebe zakho zingene kwi-canal yakho yeendlebe, ekubeni ukuthutha kwi-tube ye-eustachian ayikwazi. Ezinye iindlela zonyango zingabandakanya ukuhlamba kwamanzi amaninzi kunye neentsholongwane ezichasayo.
Unyango lweengxaki zokuphefumula ujolise ekuphuculeni ikhono lakho lokukhwehlela. Ekubeni i-cilia ekhubazekile iyanciphisa amandla akho okuphelisa i-mucus kwi-airways, ukukhwehlela kukuncedisa ukunceda umzimba wakho ekuhambiseni i-mucus ngaphandle kwendlela yakho. Ukuze ukwazi ukufezekisa oku, unokumiselwa:
- I-poet physiotherapy - izixhobo zamatshini okanye iindlela zokuphatha ukukhuthaza ukukhwehlela
- Ukuzivocavoca - i-cardio esebenzayo ibangela ukuba uphefumle kakhulu kunceda ekuhlanganiseni imfihlo kwi-airway
- Imithi - i- bronchodilators kunye ne-anti-inflammatories inceda ukunciphisa ukuvuvukala nokuvula i-airways ukukunceda ukukhwehlela okanye ukuhambisa nayiphi na i-mucus.
Kwiimeko ezinzima kakhulu, i-PCD echaphazela imiphunga ingakhokelela kwi-bronchiectasis. Iziganeko ezinzima aziyi kukwazi ukuphathwa kunye nokufakelwa kwemiphunga kuyimfuneko. Ukutshintshwa kwe-Lung kuyakuphilisa i-PCD kwimiphunga. Nangona kunjalo uya kufuneka ujongane nazo zonke iimfuneko zokunyanga emva kokutshintshwa kunye nezithintelo. Le yonyango enkulu xa kuyimfuneko, kodwa ayilona ndlela efanelekileyo yokuphatha i-PCD.
Imithombo:
Kliegman, Stanton, St Geme & Schor. (2015). Ncwadana yeeNzululwazi. 20th. I-Ciliary Primary Dyskinesia (i-Immotile Cilia Syndrome iKartagener Syndrome). https://www.clinicalkey.com/#!/ (Ubhaliso olufunekayo).
I-Heart Heart, i-Lung, ne-Blood Institute. (2011). Iyiphi i-Ciliary Primary Dyskinesia. https://www.nhlbi.nih.gov/health/health-topics/topics/pcd.
I-National Organisation of Distress Disorders. (2015). I-Ciliary Primary Dyskinesia. http://rarediseases.org/rare-diseases/primary-liary-dyskinesia/