Neurofibromatosis shine kwayoyin halitta, ciwon kwayoyin cuta fiye da yadda ya shafi idanu, har ma kwakwalwa, jijiyoyi, kasusuwa, da fata. Akwai nau'i biyu na neurofibromatosis: NF1 (nau'in 1) da NF2 (nau'in 2) . NF1 shine mafi yawan yawan cutar, mafi yawan kashi 90 cikin dari.
Mutanen da ke da NF1 suna da alamun alamomi masu yawa waɗanda zasu iya kasancewa a lokacin haihuwar haihuwa, amma yawancin alamun da bayyanar cututtuka sun nuna a farkon yarinya.
Wadanda ke tare da NF2 sukan ci gaba da alamu da bayyanar cututtuka a cikin rayuwar, tsakanin shekarun 20 zuwa 30. NF1 wani lokaci ana kiransa Von Recklinghausen ko neurofibromatosis. Ana kiran NF2 a wasu lokuta azaman tsakiya neurofibromatosis.
Alamai da cututtuka
Mutanen da suke da nau'in neurofibromatosis 1 zai iya samun siffofin da ke gaba:
- Cafe-au-lait macules: Cafe-au-lait macules ne pigmented fata fata da aka gani a cikin 95% mutanen da neurofibromatosis. Wadannan ƙananan hanyoyi suna karuwa a cikin girman da lambar a lokacin shekaru 10 na rayuwa.
- Ƙunƙarar launi na fata: An yi amfani da takalmin ƙwallon fata kamar yadda Crowe ya shiga. Mutane da ke da neurofibromatosis sau da yawa suna da jerin tarin fuka a cikin yanki da kuma yankuna. A wasu, ana samun waɗannan ƙananan sama a sama da eyelids, karkashin ƙirjin, da kuma kusa da wuya.
- Canjin fata: Mutanen da ke da neurofibromatosis sun kasance suna haifuwa da ci gaban ƙananan ƙananan kuma wani lokaci dysplasia korangwal. Dysplasia na skeletal yana haifar da gajeren rauni da makamai da ƙafafu don kada su kasance tare da sauran jiki.
Neurofibromatosis da Eyes
Mutane da ke da neurofibromatosis sau da yawa sukan haifar da halayen da suke shafar idanu.
- Neurofibromas cututtukan: Neurofibroma cututtuka shi ne farar fata da aka sa hannu a cikin NF1. Neurofibromas suna daukaka, zagaye da ciwon ƙwayar fata. Neurofibromas sun bayyana a cikin ƙuruciyar ƙuruciya kuma suna karuwa a girman da lambar a duk rayuwarsu. Neurofibromas na iya zama babba, wani lokaci sukan kai santimita biyar. Suna yawanci ba mai ciwo ba amma suna iya zamawa kuma suna jin daɗin tabawa. Lokacin da suke faruwa a fatar ido zasu iya haifar da kumburi da cututtuka irin su blepharitis . Hakanan kuma zasu iya haifar da raunin ido na ido kamar ptosis, inda fatar ido ke rataye da ƙananan yadda ya kamata, da kuma ectropion, inda fatar ido ta kasa ya fadi daga idanu kuma zai iya fallasa shi a yanayin da ba shi da haɗari.
- Lisch nodules: Ludch nodule an tashe shi, raunuka masu alade wanda ya bayyana a cikin iris . Likitocin likita zasu iya ganin Lisch nodules a idon mutanen da ke da neurofibromatosis a matsayin matashi a matsayin shekaru shida. Sun yi kama da ƙananan ƙwayoyin cuta, kananan, madauri, masu tsalle-tsalle masu tsalle daga iris. Wadannan na iya zama mahimmanci don dalilai na ganowa. Wasu lokuta, ana gano Lisch nodules a lokacin gwajin ido kafin an gano mutumin da neurofibromatosis.
- Magungunan ƙwayoyin astrocytic na asibiti: Dabbobi masu tarin fuka da ƙwayoyi masu tarin fuka ne na ciwon sukari wanda zai iya ci gaba a duka idanu. Zasu iya haɗawa da jijiyar ƙwayar ido amma yana iya yadawa don tasiri macula, tsakiyar cibiyar kwakwalwa. Idan waɗannan bangarorin suna da hannu, za'a iya rage hangen nesa kuma strabismus (ido ido) na iya kasancewa. Irin wannan ƙwayar yana tsaye a cikin ƙananan kwalliya, ko
- Hanya mafi kyau gliomas: Yawancin kashi 15 cikin dari na yara tare da NF1 suna da ciwon kwakwalwa wanda ke shafar hanyar hanya, wanda ya hada da jijiyar ido kamar yadda yake fitowa bayan ido kuma ya koma cikin kwakwalwa. Wani lokaci waɗannan ciwace-ciwacen zai iya haifar da protososis. Tsarin ƙwayoyin cuta shine yanayin da yaduwar take haifar da ido zuwa gaba. Strabismus (ido ido) zai iya faruwa ne sakamakon sakamakon gliomas.
Jiyya
Neurofibromas na iya zama masu yawa a lamba ko kuma faruwa a kananan bunches. Idan waɗannan da ke tattare da nodules suna faruwa a wurare masu ban sha'awa wadanda ke haifar da matsalolin, misali tare da haikalin, ana iya cire su ta jiki. Wani neurofibroma tare da haikalin zai iya haifar da matsala mai yawa amma kokarin ƙoƙarin yin takalma ko takalma. Rikicin m ya yi aiki sosai don raunuka marasa lafiya amma ba zai yiwu ba a mafi yawan lokuta don cire babban adadin su.
Mutanen dake da ƙananan neurofibromas a kusa da idanu da fatar ido ya buƙaci kulawa da kyau wajen amfani da tsafta mai kyau a cikin wadannan yankunan. Tsaftacewa a kusa da neurofibromas a kusa da fatar ido zai iya hana zubar da jini da sauran cututtukan fata.
Yara da suke inganta gliomas na al'ada ba sa bukatar magani sai dai idan ciwon sukari ya nuna girma ko ci gaba. Idan wannan ya faru, chemotherapy shine jiyya na zabi. Wani lokaci magungunan kwakwalwa na iya haifar da rashin ci gaba ko ciwon hankali. Wadannan yara suna buƙatar kulawa ta musamman kamar yadda waɗannan raunuka zasu iya haifar da lahani na sararin samaniya, matsalar gurguntaccen matsala da matsalolin harshe da kuma matsalolin motsa jiki.
Matsaloli
Wasu matsaloli na iya tashi a cikin mutane tare da NF2.
- Ƙananan neuromas: Neurofibromatosis type 2, NF2, an lalacewa ta hanyar maye gurbi cikin kwayar da take hana ci gaban ciwace-ciwacen ƙwayoyi. Wannan yana haifar da ƙananan ciwon nervous da ciwon tumo. Daya daga cikin alamun da aka fi sani da NF2 shine ƙananan neuromas, ko ƙwayar da ke shafar cutar da ke ba mu damar ji. Mutanen da suke shan wahala daga wannan na iya yin korafin matsalolin rashin daidaituwa, jihohi da jiji.
- Cataracts : Kashi arba'in daga cikin mutane tare da NF2 zasu bunkasa nau'i biyu na takardu, ƙananan subcapsular da cataracts. Wadannan yawanci suna da ƙananan cataracts amma kimanin kashi 20 cikin dari sun sami hangen nesa saboda su.
- Mafi yawan ciwon daji na ƙwararrun ciwon daji: kimanin kashi 27 cikin dari na mutane da NF2 zasu sami ciwace-ciwacen da ke shafar suturar fata. Magungunan da kansu sukan sabawa amma suna iya matsawa da kuma sanya suturar jijiya da jini a cikin jijiyar jiki, haifar da asarar hangen nesa. Wadannan magungunan na iya haifar da ido ga ido da kuma sanya ƙuntataccen tsoka.
- Magungunan asibiti: Mutanen da ke sha wahala daga NF2 kuma suna ganin suna iya samar da ƙwayoyin cutar. Magungunan rubutun su ne membranes da suke ci gaba a kan farfajiyar. Yawancin mutum yana da yawa a kan macula. Idan wadannan kwangilar sun yi musayar, za su iya haifar da raguwa ko ɓata.
Abin da Ya kamata Ka sani
Neurofibromatosis wani cututtuka ne mai tsanani wanda ke haifar da ciwon tumatir a cikin tsarin da yawa cikin jiki. Dukansu NF1 da NF2 na iya haifar da alamun ido wanda likitocin ido zasu iya samowa akan wannan zai haifar da ganewar asali. Duk wanda ake zargi da ciwon neurofibromatosis ya kamata ya yi nazarin ido na shekara daya ciki har da dilar ido.
Girman matsalolin da ke tattare da ido na ido na neurofibromatosis zai iya bambanta sosai, har ma a cikin membobi guda daya. Duk da haka, saboda yana rinjayar tsarin tsarin jiki, wadannan mutane suna ziyarci masu sana'a daban daban ciki har da neurosurgeons, biologists, audiologists, optometrists / ophthalmologists , da neuroradiologists.
> Sources:
> Sowka, Yusufu, Gurwood, Andrew, Kabat, Alan, Neurofibromatosis, p13-15, Yuni 2016, Binciken Abubuwan da ake amfani da ita, littafin jagorancin maganin cututtuka, 18th edition.