Franceschetti ciwo: Yanã, cututtuka da kuma lura da cutar

Franceschetti ciwo ne autosomal rinjaye cuta halin da musakai-fascial fuska yankin. An farko aka bayyana a cikin 1900 ta Birtaniya ophthalmologist Treacher Collins.

Pathology halayyar

A cutar halin gaban haƙuri da mahara na fuska musakai. Tun da Pathology tasowa a utero, bayan haihuwa za a iya kafa Treacher Collins ciwo, Franceschetti.

Wannan cuta na da cututtuka da kuma kusan gaba daya yanke yaro da al'umma, yin ransa mafi wuya da kuma m. Raya ci gaban cuta da kashi da nama da kuma ossification dalili mai tsanani fuska jeri na bangaren, saboda abin da auditory da na gani AIDS zama marar sakewa tsarin Pathology.

Franceschetti ciwo - a rare kwayoyin cuta da ya auku a 1 a 50,000 kowacce haihuwa. Duk da yake magani nepodvlasten iko da kwayoyin maye gurbi, don haka idan kana da wani munanan a cikin iyali, dole ne ka tabbata ka ziyarci halittar jini, wanda zai taimaka wajen kirga yiwuwar samun ci gaban da ba a haifa ba daga wani kayyade munanan.

A Sanadin cutar

Ci gaban da cutar da ake dangantawa da illa fetal ci gaba a tsawon 6-7 makonni. Keta suna sarrafa a cikin tayi abubuwa na farko gill baka.

Franceschetti ciwo iya samun sãɓãwar launukansa digiri na cututtuka, daga m ga karfi da bayyana pathologies daga cikin kwanyar. A ciwo da aka gada a wani autosomal rinjaye hali da kuma yana da babban kashi na phenotypic manifestations na irin pathologies a yara.

ÃyõyinSa na ciwo

Azahiri ido ciwo ne da aka nuna ta hanyar QFontDatabase slits, cikin matsanancin kusurwa wanda aka tsallake lokaci-lokaci lura coloboma karni (babu wani ɓangare na ƙananan ko babba fatar ido), nakasar cataracts, microphthalmia, paresis tsokoki alhakin ido motsi.

A maxillofacial tsarin lura hypoplasia na da zygomatic kashi, manya da ƙananan muƙamuƙi. A sakamakon haka, wani gagarumin jeri na bangaren na fuska, wani lokacin ba a marasa lafiya kogon palate, watakila turawa da harshe, bayar da tasu gudunmuwar toshewa da oropharynx da kuma haddasa numfashi cututtuka.

Hakora ne sau da yawa underdeveloped, yadu kafa, akwai wata matsala tare da cizo. Hypoplasia daga cikin ƙananan ɓangare na fuska ba shi da wani irin tsuntsu. A rare lokuta, akwai halakar manyan jini ganga, zuciya, underdevelopment, ciki hydrocephalus.

Franceschetti ciwo za a iya bambanta daga dysplasia Goldenhaara. Domin wannan Pathology halayyar karuwa a yawan thoracic ko lumbar vertebrae, wuyansa synostosis, ji hasara, pathologies daga cikin tsarin da auditory meatus, forked harshe, kogon palate.

M ciwo yana da kusan babu tsarin canje-canje a cikin fuska yankin, da wani talakawan nauyi - jera cututtuka da aka bayyana selectively. Mai tsananin ciwo ne halin da cikakken rashi na zaba fuska fasali a cikin yara. Bisa kididdigar da, matsakaici ciwo sau da yawa bayyana, amma a mafi yawan lokuta zai yiwu don kawar da lahani ta roba tiyata.

Franceschetti ciwo: Jiyya

Don bi marasa lafiya na bukatar ha] in gwiwar kiwon lafiya m. Babban matsalar ne take hakkin da airway, hadiya, hangen nesa da kuma ji. A wasu lokuta kuma zai zama lalle tracheostomy - da aiki na dissection agara tracheal bango.

Bayan gastrostomy (fitsari kafa) kwashe abinci mai gina jiki da kuma haƙuri numfashi. Iya inganta bayyanar da roba tiyata, amma shi ne yake aikata a kan bukatar da kawai kan kai wani shekaru.

Surgery bukatar a hankali a hankali canji na bayyanar da magani mataki. Lahani za a iya shafe ta a cikin 'yan shekaru, ko ma shekarun da suka gabata. A wasu lokuta, ba shi yiwuwa don kammala gyara da fuska musakai, don haka likitoci ta hanyar tiyata iya kawai dan kadan rage bayyanar cututtuka da kuma inganta haƙuri da ingancin rayuwa.

Kokarin mayar ji ta hanyar tiyata (don daidai tsarin da auditory ossicles) a cikin marasa lafiya basu da kyau sakamako, saboda haka shi ne mafi alhẽri ga wannan dalili don amfani da jin AIDS, zaba bisa mutum Pathology da ciki tsarin da tsakiyar kunne.

Franceschetti ciwo: photos na marasa lafiya

A hoto ya nuna cewa mutane da ciwo Treacher Collins ba kasa aiki rayuwa fiye da lafiya mutane. Sun koyi yarda da kanka, son da fuskarka, okin ya taimaka a wannan yara wanda basu da sha'awar yin rayuwa da kuma polontsenno jin tsoro ka tafi waje saboda izgili da sauran yara.

Ko a gaba daya lafiya iyaye iya samun wani yaro da kwayoyin cutar.

A cikin hotuna kafin da kuma bayan roba tiyata, da yarinya gani wani gagarumin bambanci. Saboda haka, kada ka yi baƙin kafin lokacin da za a sa hannuwanku. Za ka iya ko da yaushe samun wata hanya ta inganta su bayyanar.