Chii chinonzi Noonan Syndrome? Usatya, ngatizivei izvi

Wakamboona chimwe chinhu chinoshamisa pachiso chemwana wako here? Pamwe maziso ake ari kure zvishoma, kana kuti mutsipa wake mupfupi zvishoma... Kana kukura kwemwana wako kuchiita sekunonoka pamwe chete nezvinhu izvi, chikonzero chingave chirwere chausati wambonzwa chinonzi 'Noonan Syndrome.' Usazvidya moyo, ichi hachisi chinhu chinozivikanwa nevanhu vazhinji. Saka ngatitaure nezvazvo nhasi.

Chii chaizvo chinonzi Noonan Syndrome?

Zvichitaurwa zviri nyore, Noonan syndrome chirwere chemajini chinoberekwa mwana nacho. Izvi zvinogona kukanganisa kukura kwenhengo dzakasiyana dzemuviri wemwana. Vamwe vana vane zviratidzo zvishoma nekuda kwechirwere ichi. Kureva kuti, hazvionekwe kunze. Zvisinei, vamwe vana vanogona kunge vaine matambudziko ehutano akakomba .

Mumamiriro ezvinhu aya, mwana anogona kunge aine chimiro chechiso chakasiyana (semuenzaniso, huma yakafara, maziso akapararira), chimiro chipfupi (chimiro chipfupi), matambudziko emaziso, uye matambudziko emwoyo aakaberekwa nawo.

Ndiani anowana chirwere ichi? Chinowanzoitika sei?

Chirwere cheNoonan chirwere chinogona kuitika pakuzvarwa kwemunhu wese. Hachikonzerwi nemhosva yemunhu.

• Nhaka kubva kuvabereki: Vana vanosvika 50% vane chirwere cheNoonan vane mubereki ane chirwere ichi. Izvi zvinoreva kuti kana mumwe wevabereki vane chirwere ichi, mwana ane mukana we50% wekugara nhaka.
• Kuitika kwechirwere ichi zvisina tsarukano: Dzimwe nguva, chirwere ichi chinogonawo kuitika nekuda kwekuchinja-chinja kwemajini emwana, pasina mumwe munhu mumhuri ane chirwere ichi.

Ichi chirwere hachiwanzoitika sezvaungafunga. Paavhareji, mwana mumwe chete pavana 1,000 kusvika 2,500 vanoberekwa anobatwa nechirwere ichi.

Chii chinokonzera chirwere cheNoonan?

Kune majini chaiwo anoudza nhengo dzemuviri wedu kuti dzikure nekupatsanurana. Noonan syndrome inowanzo kukonzerwa nekuchinja ('mutations') mumajini aya. Nekuda kwekuchinja uku, mapuroteni anogadzirwa nemajini iwayo anoramba achishanda kwenguva yakareba kupfuura yaanofanira kuita. Zvakafanana nechiedza chinogara chakabatidzwa panzvimbo pekudzima pazvinofanira kuitwa. Izvi zvinokanganisa kukura nekupatsanurana kwemasero.

Pane mamwe mamiriro akafanana neaya here?

Ehe, chirwere cheNoonan chirwere chinowanikwa muboka rezvirwere zvinonzi `RASopathies`. Zvimwe zvirwere zviri muboka iri zvine zvikonzero zvakafanana zvemajini. Nokudaro, zviratidzo zvazvo zvinowanzofanana.

Heano mamwe mamiriro ezvinhu akadaro:

• "Dambudziko remwoyo"
• `Costello syndrome`
• "Neurofibromatosis rudzi 1 (NF1)"
• `Legius syndrome`
• "Turner syndrome"

Ndezvipi zviratidzo zveNoonan syndrome?

Zviratidzo zvinogona kusiyana zvikuru kubva kumwana mumwe kuenda kune mumwe. Vamwe vana vanogona kunge vaine zviratidzo zvisina kunyanya kuipa, nepo vamwe vanogona kunge vaine zviratidzo zvakakomba, zvinouraya. Zviratidzo zvakawanda zvinotanga mudumbu kana kuonekwa vasati vasvitsa makore gumi nerimwe.

Asi chinhu chakanaka ndechekuti mwana paanokura, zvakawanda zvezviso zvake zvinopera zvishoma nezvishoma.

Kuti tinzwisise zviri nyore, ngatigovanei hunhu uhwu mumatafura akati wandei.

Matambudziko ane chekuita nemoyo

Vana vazhinji vane chirwere cheNoonan syndrome vanogona kunge vaine chirwere chemwoyo chekuzvarwa nacho. Vamwe vana vangada kurapwa nekukasika . Vamwe vana vanogonawo kuva nechirwere chemwoyo gare gare muhupenyu hwavo. Matambudziko emwoyo anowanzo sanganisira:

• Buri riri pakati peatria yemoyo (atrial septal defect).
• Kukora kwetsandanyama dzemwoyo (Hypertrophic cardiomyopathy).
• Stenosis yetsinga dzemapapu.

Zvimwe zvinetso zvehutano zvinogona kuitika

• Matambudziko ekufema: Semuenzaniso, zvirwere zvakaita se `laryngomalacia`.
• Kuzvimba kwemaoko netsoka: Kuungana kwemvura (lymphedema) nekuda kwedambudziko re lymphatic system.
• Kunonoka kukura kwemwana .
• Kubuda ropa kana mavanga zviri nyore .
• Kuomerwa nekuyamwisa mwana achiri mudiki.
• Machende asina kudzika muvakomana . Kana akasarapwa, izvi zvinogona kukonzera kusabereka mune ramangwana.
• Kurwara kwemusoro (scoliosis ).
• Kutadza kuona kana kunzwa zvakanaka.
• Zvirwere zvekuzvarwa nazvo zvine chekuita neitsvo.

Ndezvipi zvimwe zvinetso zvinouya nazvo?

Vana vazhinji vane chirwere cheNoonan vanokura zvishoma nezvishoma pane zvakajairwa, kunyanya panguva yekuyaruka. Uyewo, vanenge 25% yevana vanogona kunge vaine matambudziko ekudzidza. Zvisinei, vashoma chete ndivo vane matambudziko epfungwa. Izvi zvinoreva kuti vana vazhinji vanogona kudzidza zvakanaka. Vanenge 10-15% yevana vangangoda rubatsiro rwedzidzo rwakakosha.

Pamusoro pezvo, vamwe vana vane mukana mudiki wekuwana chirwere cheleukemia chisingawanzoitiki muvana chinonzi `juvenile myelomonocytic leukemia (JMML)` kana mamwe magomarara evana. Asi musazvidya moyo, njodzi iyi yakaderera zvikuru, pa4 % pazera remakore makumi maviri.

Chiremba anoongorora sei izvi? (Kuongororwa)

Chiremba wenyu anogona kufungidzira kuti Noonan syndrome mushure mekuongorora mwana wenyu mumuviri uye kubvunza nezvezviratidzo zvenyu. Kuti asimbise chirwere ichi uye kuti asave nezvimwe zvirwere, chiremba wenyu anogona kuraira bvunzo dzakasiyana-siyana.

Miedzo iyi inogona kusanganisira:

• Kuverengwa kweropa rose (CBC)
• X-ray yechipfuva
• Kuongororwa kweCT
• Kuongororwa kwe 'Echocardiogram' kunoongorora mashandiro emoyo
• Bvunzo ye `Electrocardiogram (EKG)` inoongorora mashandiro emagetsi emwoyo.
• Kuongororwa kwemajini
• Kuongororwa kweUltrasound (`Ultrasound`)

Pane mushonga weNoonan syndrome here?

Hapana mushonga weNoonan syndrome parizvino. Zvisinei, kune nzira dzakawanda dzinoshanda dzinogona kubatsira kudzora zviratidzo uye kubatsira mwana kurarama hupenyu hune hutano .

Chikwata chevanachiremba chiri kurapa mwana wenyu chichagadzira hurongwa hwekurapa hunoenderana nezviratidzo zvemwana wenyu uye kuoma kwazvo. Hurongwa hwacho hunogona kusanganisira:

• Zvishandiso zvekubatsira: Zvinhu zvakaita semagirazi emaziso kana zvinhu zvinobatsira kunzwa.
• Kurapa kwemaitiro kana kutaura .
• Rutsigiro rwedzidzo: Kupa rutsigiro rwakakosha kune vane matambudziko ekudzidza.
• Mishonga: Mishonga yekurapa matambudziko emwoyo, kudzora kubuda ropa, kana kuwedzera kukura kunonoka.
• Kurapa kwehomoni yekukura .
• Mishonga yekuwedzera: Zvinhu zvakaita sekurapa kwekumanikidzwa kwe lymphedema (kuzvimba).

Dzimwe nguva, chiremba wako anogona kukurudzira kuvhiyiwa. Kuongororwa kwechirwere nekukurumidza kwakakosha kuti kurapwa kubudirire uye kuteverwa.

Remangwana richave rakaita sei? Uye izvi zvinogona kudzivirirwa here?

Ichi ndicho chinhu chakakosha. Vanhu vazhinji vane chirwere cheNoonan vanorarama hupenyu hwakanaka uye hwakazvimiririra. Chikwata chevanachiremba vemwana wenyu chichakubatsirai kugadzirisa zviratidzo zvemwana wenyu uye kudzivirira matambudziko.

Sezvo chirwere ichi chichikonzerwa nekuchinja kwemajini, hapana chatingaita kuti chisaitike. Zvisinei, kana mumwe munhu mumhuri yako aine chirwere cheNoonan, unogona kutaura nachiremba wako nezvekuongororwa kwemajini epamuviri.

Zvakajairika kunzwa kutya kana mwana akaonekwa aine chirwere chakadai. Asi yeuka, vana vazhinji vane chirwere cheNoonan vane zviratidzo zvishoma. Vanogona kurarama hupenyu hwakazara, hune simba. Saka taura nachiremba wako nezvekurapa kwakanakira mwana wako. Kutanga kurapwa nekukurumidza kunogona kubatsira mwana wako kuti ave nemigumisiro yakanaka pahutano.