Kartagener syndrome na ụmụ: nchoputa, foto, ọgwụgwọ

Kartagener si syndrome - a obere congenital ọrịa mere site a mebiri cilia motility, emi odude n'elu ọtụtụ epithelial mkpụrụ ndụ. The zuru ụdị a nkwarụ na-clinically gosipụtara a chi atọ n'ime mgbaàmà:

• bronchiectasis;
• hypoplasia (hypoplasia), paranasal sinuses, nke na-esonyere ntighari Sinusitis;
• agbara ndokwa nke esịtidem akụkụ (situs inversus).

The syndrome na-aha ya bụ mgbe Kartagener Swiss dọkịta, onye nyere a zuru ezu nkọwa nke ala na 1933. Ugbu a, ka-atụle n'ụzọ sara mbara karị ọrịa.

Na-eme Kartagener syndrome

Ugbu a, ọ na-egosi na Kartagener syndrome nwere mkpụrụ ndụ ihe nketa uwa na ihe autosomal recessive mode nke nketa. Ndabere nke ọrịa na-ntụpọ na a ọnụ ọgụgụ nke mkpụrụ ndụ ihe nketa na encode ụfọdụ na-edozi, e mere iji hụ na ọrụ nke cilia.

Dị ka a n'ihi nke ha agagharị na-ebelata ma ọ bụ furu efu na niile - e nwere a ọnọdụ a na-akpọ "isi ciliary dyskinesia." Mụọ ihe karịrị iri abụọ mkpụrụ ndụ ihe nketa maka mmepe nke Kartagener syndrome. Mebiri nke ọrụ nke ọ bụla n'ime ha na utero pụrụ iduga daa ọrịa.

Olee nkịtị bụ ọrịa?

Dị ka e kwuru n'elu, Kartagener syndrome na ụmụaka bụ obere -only na naanị otu nwa ọhụrụ nke 16,000.

Ọ ga-kwuru na ụmụaka nwere ike daa ọrịa anaghị egosipụta onwe, na nchoputa na-ahụ nanị mgbe a ọnwa ole na ole ma ọ bụ ọbụna ọtụtụ afọ, mgbe na-emepe emepe a na-egbuke egbuke-adakarị picture.

Mgbaàmà nke Kartagener syndrome

Kartagener syndrome mgbaàmà ịdị iche iche site dịghị mgbaàmà ka a pụrụ iche na-adakarị picture.

N'ihe banyere asymptomatic ọnọdụ arịa bụ oké ike, na etịbe Ọtụtụ ahụrụ site ohere mgbe reversing esịtidem akụkụ n'oge a nnyocha e mere banyere ụfọdụ ọrịa ndị ọzọ.

bronchiectasis

Nọmalị, na bronchial osisi (isi ìhè eku) nwere ụdị branching tubules na edo Mbelata dayameta nke lumen na ntụziaka si overlying na-apụtaghị ìhè na ngalaba na yiri ihe isi ala okpueze.

Bronchiectasis (ma ọ bụ bronchiectasis) - a obodo mmụba nke lumen nke bronchi ụdị akpa, spindles ma ọ bụ cylinders. Na ndị dị otú ahụ nwere nkwarụ bronchi na-aghọ-agaghị ekwe omume nkịtị ije secretion. Ọ stagnates, nke ga-apụghị izere ezere na-eduga ọrịa na mmepe nke mkpali omume.

Clinically, bronchiectasis na Kartagener syndrome gosipụtara ụkwara na expectoration greenish purulent sputum, fever ruo 38 degrees na elu, ike, isi ọwụwa, mgbe ụfọdụ nwere ike iso ọgbụgbọ na vomiting. Ike ogwu mgbochi ọgwụ na-enye ohere maka mpụga mgbake, ma ndị a ọgwụ ọjọọ nwere ike ghara isi na-akpata mbufụt bụ obodo bronchiectasis. Ya mere, na-adakarị picture kọwara ugboro ugboro ugboro ugboro, e nwetụ na "ìhè" asymptomatic etiti oge. Ọ na-aghọ a ogologo na protracted N'ezie. Adịghị ala ala relapsing N'ezie nke ọnọdụ a na-akpọ bronchiectasis.

Sinusitis

Ọzọ pụta ìhè nke Kartagener syndrome bụ Sinusitis, ma ọ bụ mbufụt nke paranasal sinuses. Na nkịtị ciliary epithelium nke gọzie gị mucosa na imi ruru ya fluctuations enye ije secretion dozie na ya ájá ahụ na bacteria. Kartagener syndrome Mgbe cilia ọrụ bụ isi, na ọdịnaya stagnates na sinuses, na-eme mbufụt.

Ọtụtụ mgbe ịzụlite Sinusitis, ma ọ bụ mbufụt nke maxillary sinuses, ọ dịkarịa ala - n'ihu (frontal imi), ethmoiditis (mkpụrụ ndụ nke a trellised labyrinth) na sphenoiditis (sphenoid imi). All ndị a na-ekwu, na-egosipụta site fever, isi ọwụwa na runny imi na abu. Sinusitis na-ji mgbu ndị dị n'akụkụ nku imi na cheeks.

Agbara ndokwa nke esịtidem akụkụ

Agbara ndokwa nke esịtidem akụkụ, ma ọ bụ situs viscerusim vesos - nke a bụ ihe ndị kasị mara ngosipụta nke Kartagener syndrome, nke Otú ọ dị, pụtara na-erughị ọkara nke ndị ọrịa. Aga ije nke esịtidem akụkụ dị ka ụdị mirroring. Ịmata ihe dị iche zuru ezu na ezughị ezu reverse ndokwa nke esịtidem akụkụ.

Ezughị ezu reverse ndokwa na-swapped akpa ume. Nke a nwere ike so, dị ka ọ bụ ndị na a mirror echiche nke obi na a mgbanwe ya n'elu nri n'akụkụ nke obi oghere (dextrocardia).

Na-na reverse ndokwa esetịpụ mirror ịkwaga niile esịtidem akụkụ. Na nke a, na imeju na-bụ n'aka ekpe, na splin - nri. Mberede nchọpụta nke a daa ọrịa site ultrasound n'oge a na-eme nnyocha na-enye ohere ka chee Kartagener syndrome mgbe asymptomatic.

Agbara ndokwa nke esịtidem akụkụ bụ n'ihi na mebiri nke Mbugharị nke na-amalite amalite na mkpụrụ ndụ na anụ ahụ n'oge embryogenesis. Ọtụtụ ụlọ ọrụ na-ejikarị adịghị ịzụlite ebe ha mgbe nwa. N'ihi ya, akụrụ tọgbọrọ pelvic mpaghara na-eji nwayọọ nwayọọ na-ebili ka larịị XI-XII ọgịrịga.

N'ịga (ma ọ bụ Mbugharị) nke aru nke n'oge prenatal oge a rụrụ site cilia, Kartagener syndrome nke mgbe bụghị na-arụ ọrụ, nke na-akpata a reverse ndokwa nke esịtidem akụkụ. Ọ dabara nke ọma, dị ka ma ọ bụrụ na egwu ọnọdụ a adịghị anya, ọ bụ na ọtụtụ mgbe adịghị eduga ọkpụrụkpụ imebi nke ndụ nke organism.

Ndị ọzọ bụ ngosipụta nke Kartagener syndrome

Tinyere ihe ndị ọzọ mgbaàmà nke ọrịa bụ ihe kasị mkpa nwoke infertility. Ọ e mere site ịnọrọ spam n'ihi na eziokwu na ha cilia adịghị arụ ọrụ.

Ọrịa nwekwara ike ichegbu ntighari ntị na-efe efe na ntị ọnwụ. Nke a bụ n'ihi na ọnọdụ na-achọghị nke secretions n'etiti ntị, nke na-ejikarị ga-ewepụ ciliated epithelial mucosal mkpụrụ ndụ.

Kartagener syndrome nchoputa na ụmụ

Ọ bụrụ na a na nwa, na-amalite gburugburu afọ otu ọnwa, mgbe nile na-ata ahụhụ site na oyi baa, rhinitis na Sinusitis ga-enyo enyo Kartagener syndrome, nchoputa nke nke bụ nnọọ mgbagwoju anya. Ọ na-agụnye a ọnụ ọgụgụ nke instrumental na laabu ọmụmụ:

• Physical nnyocha, ma ọ bụ nkịtị ọgwụ nnyocha, ga-ekpughe na ike nke gọzie gị ume, na-kpọmkwem auscultatory picture na akpa ume na obi, nakwa dị ka mgbanwe na ọnụ phalanges nke "drumsticks" ụdị na ogologo N'ezie nke usoro.
• X-ray nnyocha ga-ekpughe na-enweghị nchịkwa na ọnya na akpa ume na agbara ndokwa nke obi (dextrocardia). Nke a na usoro dị mfe na mma-ezu, ya mere, na-eme ka o kwe omume na-ebu nchoputa nke Kartagener syndrome n'ụlọ ọgwụ.
• Bronchoscopy - kasị ezi usoro nke nchoputa ịchọpụta bronchiectasis. Ọzọkwa, nanị site n'aka bronchoscopy nwere ike na-a biopsy nke bronchial mucosa.
• mucosal biopsies egosi ịdị njọ nke mkpali usoro na-amata cilia Ọdịdị.

Eduga n'ụlọ ọgwụ na mahadum gburugburu ụwa na-atụle nnọọ ogologo oge na-ama Kartagener syndrome, a foto nke na-e kwuru n'isiokwu a. Erite kasị ama ndị ọkachamara na ọ nwere ike ịzụ tọrọ ọgbọ nke ndị dọkịta na-aghọta dị otú ahụ a obere ọrịa.

Imeso Kartagener syndrome

Enwere m ike tufuo a ọnọdụ? Ugbu a, ọgwụgwọ nke Kartagener si syndrome na-adabere na ihe mgbaàmà. E nweghị nkà mmụta ọgwụ na ga weghachi ọrụ nke cilia nke epithelial mkpụrụ ndụ, ma nkà mmụta ọgwụ ọgbara nwere a ọgaranya kpụrụ nke ngwaọrụ ikwado N'ezie nke ọrịa. Na enyemaka ha, onye ọrịa nwere ike ichefu ya obere ọrịa.

Isi ụzọ nke ọgwụgwọ:

• Ọgwụ nje. Ọgwụ ndị a na-kenyere mbufụt nke ngụgụ mere bronchiectasis, na Sinusitis. Tee oge gboo ọgwụ nje penicillin, macrolides na ọgwụ ọjọọ si na ìgwè nke "akụkụ okuku ume na" fluoroquinolones.
• Ụzọ nke rụọ ọrụ ndị drainage ọrụ nke bronchi - postural drainage, ịhịa aka n'ahụ, ngwa mucokinetic na mucolytic ọgwụ ọjọọ, wdg ...
• Physiotherapy.

Mgbe ọnụnọ nke akpọ bronchiectasis na ugboro ugboro ntighari bronchitis na oyi baa ịkpa ọgwụgwọ - mwepụ (resection) The kasị akpa ebe nke akpa ume. Mgbe ihe dị otú ahụ ime ihe ọnọdụ nke ndị ọrịa mma n'ụzọ.