Menkes chirwere: tsananguro uye kuongorora chirwere

Menkes urwere, rinonziwo chirwere rakamonana bvudzi - isingawanzowanikwi uye dzakakomba zvikuru magene chirwere. Zvinokanganisawo vakomana vaduku uye zvinosuwisa, haagoni kurapwa.

The zvinokonzera chirwere

Menkes chirwere mugumisiro kukodzera kushanda ATR7A Gene. Somugumisiro yacho anomaly muviri asingadi rishandise mhangura, riri iiswe itsvo, uye zvimwe mitumbi yose vari kusangana kuzvidya mwoyo mvura, achirapwa kuparadza nokukurumidza. Chokutanga, vanotambura mwoyo, uropi, mapfupa, arteries, uye bvudzi. The chirwere nhaka uye hariwanikwi chaizvo. Ngozi anenge 1 muna mamiriyoni 50-100 uye vakagovera kunyanya varume. In munhukadzi vana chirwere kushanda zvikasaitika.

The zviratidzo Menkes urwere rokutanga

A mwana akaberekwa aine chirwere ichi, anotarira uye anonzwa zvachose zvakajairika mumavhiki zvoupenyu rokutanga. Yakanga chete romwedzi zviratidzo kuonekwa wechitatu, zvichiratidza kuti mwana ave Menkes urwere:

• Curly bvudzi riri panzvimbo rawa pasi zvishoma mucheche. Vane mashoma chaizvo uye yakajeka. On ukanyatsozvitarisa zviri pachena kuti bvudzi riri nyore chaizvo uye zvakanyatsobatana. Vanotarisa vavhiringidzika. Zvakanyatsobatana sezvo bvudzi uye nevagari vayo.
• The ganda chinhu sokurwarirwa pallor.
• Baby vanotarisana zvikuru dzizere.
• Bridge mhino ndiko sandarara.
• Chichiburutswa tembiricha.
• Fudzai mwana anova matambudziko. Nzara iri zvachose haazovipo. Uyezve, pane zvakasiyana bowel tsika.
• Kid pusa, hope uye ane usimbe, chiso chake zvinoratidza anenge asina manzwiro. Pane zvose nezviratidzo nokusakendenga.
• Baby kazhinji achirwadzisa chinyamunhari chinorwadza.
• Mwana yekudyidzana lagging shure vezera ravo uye handichisiri kuita kunyange icho wakatobatsira vakakwanisa kudzidza.

chirwere of urwere

Zvinofanira kucherechedzwa kuti kushoma chirwere zhinji rakaoma rokuongorora. Chimwe chikonzero ichi - murombo kuziva vanachiremba vana. Kana ari Menkes chirwere wevana anogona kuona kujairika mhando mbudzana bvudzi. Pachena zviratidzo ndivo chinyamunhari chinorwadza, iyo nguva nenguva kuoneka mune mwana.

Vakawanda kupfuura zviratidzo rwakavapo nezvimwe zvirwere, kure zvishoma ngozi. Naizvozvo, kuti tiwane navo mwana wenyu vanetseka hakusi zvakakodzera. Asi zvinokosha kuongorora izvozvo. The Mhando chikuru Diagnostics Menkes chirwere ane muedzo kwevari kuvapo mairi emhangura uye maekisirei mapfupa, izvo zvinogona kuratidza kuchinja dzavo noruzhinji ropa.

dziviriro chirwere

Zvinosuruvarisa, kudzivisa chirwere nzira Menkes mushonga harisati tega. Pangozi - varume vana vaiva nehama pamwe chirwere. Kuona chirwere achiri padanho mudumbu kukura, uye zvakawanda zviri anenge zvisingaiti kudzivirira. Chokwadi, vamwe vakadzi vari nokuzvibvunza mubvunzo kana zvichibvira kuronga pamuviri pamwe Menkes urwere mumhuri murume? Mhinduro iyi inogona chete kupa geneticist akanaka, ndiani ichaongorora zvayakanakira nezvayakaipira, ndichaita rokuongorora, tara "magene muti" uye chete ipapo kuzivisa mwero ngozi. Zvinokurudzirwa kutora chisarudzo pamusoro pekuronga mwana chete pashure zano nyanzvi.

Treatment Curly bvudzi chirwere

Zvinosuruvarisa Menkes chirwere chiri chirwere chisingarapiki. Achatanda panguva zvakaoma chaizvo. The chirwere mberi nokukurumidza, kukonzera kusingarapiki kuchinja mumuviri. Kuwedzera hwepfungwa retardation, iyo inenge nguva dzose achiperekedzwa ane chirwere, kuchititadzisa kukura tsinga, havachanyatsogoni mwoyo basa, mapfupa mamiriro kuchinja.

Life pamwe Menkes chirwere ipfupi. Vana Most kufa pashure maviri matatu pashure pokuberekwa. Kazhinji zvinoitika nokukurumidza - iri pakanyorwa vakawanda pakusimba - nemabayo, zvirwere kana atsamwira rwengarava ropa.

Kunyange kurapwa sezvo mushonga kwakadaro haazivi, kune nzira aivabatsira murwere ezvinhu. Ichi chinhu hazvizivi romugove yemhangura muviri, iyo chinotarisirwa nomutsinga. mushonga Izvi yakati kunokutadzisa kukura chirwere pamwe anobvisa zviratidzo. Asi chete pasi nenguva kutanga kwake - iko pamazuva okutanga kana pane mavhiki akawanda upenyu (kusvikira yava kusingarapiki kuchinja muuropi). Izvo, nhamo, kwakaoma zvisingaiti - chaizvoizvo sokuti symptomatic mufananidzo chete mwedzi mitatu. Mune zvimwe zviitiko, vanachiremba vanogona kupa rutsigiro chete mushonga.

Very kashoma, asi zvinoitika kuti Menkes chirwere kunoitika nounyoro chimiro. mhando iyi chirwere chinonzi occipital nyanga chirwere uye kunoratidzwa kuparadzwa connective tishu. Varwere chirwere ichi havasi lagging mumashure kukura mupfungwa, asi kuguma mugumisiro achiri tsitsi. The chete chirwere kachitanga papfuura makore anenge gumi okuberekwa.