Kartagener maʻi pale i loko o nā keiki:ʻike, photo, lapaʻau

Kartagener ka maʻi pale - he ke nānā congenital maʻi, kena ae la ia e ka 'aʻe' ana o cilia motility, Aia ma luna o ka ili o na mea he nui epithelial keena. Ka piha ano o keia kina ua clinically hoikeia aku ai e kekahi triad o symptoms:

• bronchiectasis;
• hypoplasia (hypoplasia), paranasal sinuses, i ua pu ma recurrent sinusitis;
• ka mea nana e hoole hoʻoponoponoʻana o Kamehameha o ka na nā loko (situs inversus).

Ka maʻi pale ua kapaʻia ma hope o Kartagener Hawaiian kauka, nana i haawi mai i ka piha ano o ka moku'āina i ka makahiki 1933. Ano, e ka kūkākūkā i oi aku au mamuli o ka maʻi.

Ke kumu no Kartagener maʻi pale

Ano ka mea, ua hoao aku au ia ia i ka Kartagener maʻi pale i ka aaiaoe maoli i autosomal recessive pololei o ka'āina hoʻoili me ka. Ke kumu o ka maʻi, he mā ma ka helu o nā'ōewe i encode kekahi nā polokina, ke hoʻololi e hōʻoia i ka hana o ka cilia.

E like me ka hopena o ko lākou ka hoʻoneʻe ua hoemi paha lilo i mea a pau - aia no he ano i kapaia "iniiaiie ciliary dyskinesia." Aʻo hou aku he iwakalua nā'ōewe kuleana no ka hooulu ana o Kartagener maʻi pale. 'Aʻe' ana i ka hana ana i kekahi o ia mau mea i loko o utero e alakai i ka pathology.

Pehea, he pono ole o ka maʻi?

E like me ka olelo ma luna, Kartagener maʻi pale i na keiki mea pohihihi keia -only i loko o hoʻokahi wale nō keiki mai o 16.000.

It E e kaulana i na keiki i pathology 'aʻole i hoike ia iho, a me ka ua i kaʻike, wale nō ma hope o kekahi mau mahina paha hiki makahiki, ka wā ulu he ao alohilohi i lapaʻau kiʻi.

Symptoms o Kartagener maʻi pale

Kartagener maʻi pale symptoms lauwili mai ole symptoms a hiki i ka okoa lapaʻau kiʻi.

Ma ka hihia o asymptomatic okoʻa no ka manawa pololeiʻike, mea paʻakikī loa, a me ka mākaukau kaua paha ma ka hapanui pinepine ia akulla 'ma ka paha ka wā reversing ka na nā loko i ke ana e pili ana i kekahi mau maʻi'ē aʻe.

bronchiectasis

Maʻamau, ka bronchial laau (ka papa kuhikuhiE malamalama maoli) i ke ano o ka ai manamana ai tubules me kaʻaʻahu ka ho'ēmiʻana o ke anawaena o ka lumen ma ke ala e hiki aku, mai ka overlying a hiki i ka hp'pnphp mau pauku i kohu i lalo ke-ia kalaunu.

Bronchiectasis (ole bronchiectasis) - he kūloko? Anoe o ka lumen o ka bronchi type mauʻeke, manamana elua a me nā paukūʻolokaʻa. Ma na mea ino bronchi i hiki ole maʻamau neʻe secretion. He stagnates, a e inevitably e alakai i ka maʻiʻaʻai a me ka hooulu ana i ka hoʻoʻino pane.

Clinically, bronchiectasis me Kartagener maʻi pale hoike aku cough me ka expectoration he omaomao iki purulent sputum, kuni mai i ka 38 degere a me luna, nawaliwali, headache, i kekahi manawa, hiki ke hui nausea a me ka luaʻi. Ikaika antibiotic Inc e leie aku no mawaho koi, akā, hiki mai kēia mau ai 'hoʻopau i ka papa kuhikuhiE kumu o ka li nui i kūloko bronchiectasis. No laila, i ka lapaʻau kiʻi hōʻike Hoomau hou, alternating me ka "mālamalama" asymptomaticʻanuʻu. Ua lilo i ka papa lōʻihi, a protracted. Maʻi relapsing papa o ke kulana i kapaia o bronchiectasis.

sinusitis

Kekahi kīnā o Kartagener maʻi pale ka sinusitis, a me ka li nui o ka paranasal sinuses. Ma maʻamau ciliary epithelium o ka nasal mucosa a me ka sinus no i kona fluctuations i ana secretion noho ma luna o ia me ka lepo (particles) a me ka koʻohune. Kartagener maʻi pale I cilia kuleana pili i ka hihia, a me kahi stagnates i loko o nā sinuses, hoʻomakaʻu li nui.

Loa pinepine hoʻomōhala sinusitis, a me ka li nui o ka maxillary sinuses, ma ka liʻiliʻi loa - alo (frontal sinus), ethmoiditis (keena o ka trellised Kimi) a me ka sphenoiditis (sphenoid sinus). A pau kēia mau Hawaii i hoikeia aku ai e ke kuni, a me ka headache runny ihu me ka pus. Sinusitis ua i wehewehe aku e kaʻeha ma luna o nāʻaoʻao o nā'ēheu o ka ihu, a me ka papalina.

Ka aʻe hoʻoponoponoʻana o Kamehameha o na nā loko

Ka aʻe hoʻoponoponoʻana o Kamehameha no ka mea na nā loko, a situs viscerusim kū'ē - keia mea i ka loa ano hoike ana o Kartagener maʻi pale, a, nae, ia lŘlŘ i emi ma mua o ka hapalua o nā mea maʻi. Hele ana o na nā loko, e like me ke 'ano o ka mirroring. Hoomaopopo piha a kāpili aʻe hoʻoponoponoʻana o Kamehameha o na nā loko.

Kāpili aʻe hoʻoponoponoʻana o Kamehameha i swapped ia maʻiʻaʻai. Ua hiki e mai ana, e like me ka mea e noho ana he aniani manaʻo o ka naʻau a me ka hoʻololi i loko o kona luna'ākauʻaoʻao o ka pahu lua (dextrocardia).

Me ka piha aʻe hoʻoponoponoʻana o Kamehameha paʻa i ka aniani e hoʻoneʻe i nā maloko nā loko. Ma keia hihia, ke akepaʻa mea ma ka hema, i ka spleen - i ka pono. Accidental ka loaʻaʻana o kēia pathology ma ultrasound ma ka oaeouee? Hookolokolo, a e leie aku i ka manao Kartagener maʻi pale ka wā asymptomatic.

Ka aʻe hoʻoponoponoʻana o Kamehameha no ka mea na nā loko mea ku pono i ka 'aʻe o ka poʻe Polenekia o embryonic keena a me nā'aʻaʻa i embryogenesis. He nui nā keʻena ma lalo nei maʻamau mai i hoʻomōhala i ka wahi, kahi a lakou i ma hope o ka hanau ana. Penei, hakuʻala waiho i loko o ka pelvic māhele 'āina a me ke kiʻekiʻe o ku e mai i kaʻilikai XI-XII iwi aoao.

E neʻe ( 'ole ka neʻekau) o na kino i ka prenatal wā ua lawe mai ma cilia, Kartagener maʻi pale i ka wā ole functioning, i kumu no ka hoʻoponoponoʻana o Kamehameha aʻe o ka na nā loko. Mea pōmaikaʻi, me he mea hoʻoweliweli keia kulana i ole nana, ia mea i loko o ka hapanui hihia aole i alakai i paa aʻe 'ana o ke ola o ka meaola.

Other hoikeana o Kartagener maʻi pale

Mawaena o nā symptoms o ka maʻi o ka mea nui kāne infertility. Ua ua kena ae la ma immobility o ka sperm aie i ka mea i kā lākou mau cilia mai i kuleana pili i.

Hoʻomanawanui iki mai i kekahi hoopilikia recurrent pepeiao nā mea palahēhē a me ka poho lohe. Keia mea aie a hiki i ka stagnation o secretions ma ka waena pepeiao, a maʻamau pono e wehe 'ia ciliated epithelial mucosal keena.

Kartagener maʻi paleʻike, i loko o na keiki

Inā he keiki, e hoʻomaka ana a puni ka makahiki o kekahi malama, mau poino mai pneumonia, rhinitis a me ka sinusitis E e kēia Kartagener maʻi pale, ma kaʻike, a i mea ole loa huikau. Ua nā He helu o vocal a me ka keʻena hoʻokolohua haʻawina:

• Helu ninaninau, a ano 'inikua ninaninau, e hoike mai i ka pilikia o ka nasal hanu, ka mea i ho'ākāka' auscultatory kiʻi i loko o ka maʻiʻaʻai a me ka naʻau, e like me ka loli i ka maʻi phalanges o "drumsticks" 'ano i loko o ka papa lōʻihi o ke kaʻina.
• X-Ray keia hookolokolo ana , e hoike i ka pathological lesions ma ka maʻiʻaʻai, a me ka hoʻoponoponoʻana o Kamehameha aʻe o ka naau (dextrocardia). Kēia papa hana Ua puni hoʻi a me ka maluhia kupono, no laila,ʻo ia ka mea hiki ke lawe maiʻike, o Kartagener maʻi pale i loko o ka haukapila.
• Bronchoscopy - ka loa pololei hana o ka hoʻokolokolo 'ana, e huai ae bronchiectasis. A, wale ma ka mea o ka bronchoscopy hiki ke lawe i ka biopsy o ka bronchial mucosa.
• mucosal biopsies hōʻike i ke oolea o ka hoʻoʻino kaʻina a Loiloi i cilia 'ole.

Alakai Ho 'Oponopono a me nā kulanui a puni ka honua e kūkā ana muli o kona kulana he lōʻihi manawa mua Kartagener maʻi pale, he Hawaii photo o i i hōʻike mai iā ia i loko o kēia' atikala. 'Ike i ka loa kaulana poe akamai i ka mea hiki ke lńkou i ke kaikaina hanauna o nā kauka, eʻike ia i ka nānā maʻi.

Ana Kartagener maʻi pale

Hiki au pakele o kēiaʻano? Currently, lapaʻau o Kartagener ka maʻi pale hilinaʻi nui ma luna o nā symptoms. Aia mea i lapaau i makemake i ka hana o ka cilia o epithelial aeee hoihoi, akā, laekahi lapaʻau i ka waiwai arsenal o na mea paahana, e kōkua i ka papa o ka mea maʻi. Me kā lākou kōkua, ke ahonui e hoopoina e pili ana i kona pohihihi keia maʻi.

I ka papa kuhikuhiE ano o ka lapaʻau.

• Antibiotics. Mau ai 'i hoakaka ia no ka li nui o na ia maʻiʻaʻai kena bronchiectasis, a me ka sinusitis. Pili panina antibiotics penicillin, macrolides a me ka ai 'mai ka pae mai o "naehana" fluoroquinolones.
• Nā Papahana O ka hoʻomaikaʻi i ka papa nōkahea o na bronchi - postural nōkahea, lomilomi, noi mucokinetic a me ka mucolytic ai ', a pela aku ...
• Physiotherapy.

A hiki i ke alo o ka haʻi bronchiectasis me pinepine recurrent bronchitis a me ka pneumonia operative hana - ana (resection) The loa hoʻohuli ai i nā 'āpana o ke akemāmā. Ma hope o ia ka hana ana o na nā mea maʻi hou kūpono kou.