Children Progeria: Une vana mukana?

Children Progeria - chirwere nemagene hunhu, zvichitungamirira kusingarapiki kuchinja zvomukati wegadziriro nokuda vakurumidze kukwegura yose muviri. Kokutanga chirwere rakazivikanwa uye zvinotsanangurwa muna 1889 naJohani. Hutchinson yakazvimiririra muna 1904 naJ Guilford. Pasinei chokwadi kuti vana progeria - chirwere apo neapo, kubva chichikosha akatsanangura nyaya anopfuura 150. Uye mumwe nomumwe wavo vainyatsoongorora akawandisa masayendisiti.

mumwe miriyoni 7 vachangoberekwa vanachiremba chete rokuongorora kuti urwere pakati Hutchinson-Gilford. Children Progeria iri kukura nokukurumidza - chete gore murwere muviri mazera makore 5-9. Vana zvisingawanzoitiki nyaya vararame makore 23 okuberekwa, kufa kubva chirwere zvinowanzoitika vakwegura. Zviratidzo zvokutanga "akwegura, zvevana" kuoneka muvana kubvira vachiri vaduku (makore 2-3).

Sezvo kurapa kwakadaro hakuwaniki, ndiko, mishonga vanogadzwa chete nokuda symptomatic dambudziko divi zvirwere. Asi masayendisiti munyika yose vari kudzidza dambudziko uye vari kutsvaka nzira dzinobatsira kugadzirisa nayo.

Ichazivikanwa chevana progeria zvinotevera zvinoratidza:

• miduku;
• chiedza (13-22 kg);
• wakanakisisa ganda, kuburikidza iyo midziyo zvinoonekwa;
• anononoka-zvinokambaira zvenguvo maoko nemakumbo;
• musoro guru, asi panguva imwe chete - zvishoma munhu;
• soro-ndokudzika izwi.

Zvinokonzera chirwere

masayendisiti ose anobvumirana kuti vana progeria hachisi chirwere, nhaka. Nyika anozivikanwa chete imwe mhuri, apo vana vose 3 nhaka syndrome.

Handizvo kare zvaifungwa kuti kwega "zera vana" - kuti mutation a Gene chete kuti zvinobatanidzwa paumbwe puroteni lamin A. Sero nuclei Makavakwa pachishandiswa puroteni. In nedanho kwokushanduka prelamina A mature mapuroteni rine kukundikana. Saka, somugumisiro lamin A yekudyidzana kwakasiyana hwandaiva mapuroteni. Izvi zvinotisvitsa zvakasiyana Kumwe kuchinja kunotanga mumuviri mwana, iyo inozivikanwa nokuva chirwere Hutchinson-Gilford. Vana Progeria vanotanga mberi, zvichikonzera kukurumidza kuchembera.

Asi mamwe masayendisiti vakanga vawana chimwe Gene ari DNA, mumwe mutation iyo vanotozvikuvadza kunotungamirira kuti chirwere. Kusingarapiki pezvakaitwa muna BAF-1 Gene -prichina kukura kuremara ari Kamudzira mumasero. Sezvo BAF-1 wo interacts nevamwe mapuroteni, kuti mutation racho kuchivako kunokonzera kuvhiringidzika nedzimwe mapurotini.

Pane mukana?

To musi, masayendisiti kutarisana rakaoma kupedzisa misoro kudzidza kwauchaita kuti Gene mutation. Uye kana vachida kubudirira, ipapo vana vanorwara vachava mukana chaiye kuhwina vana progeria, uye vanachiremba - kudzivirira chiitiko chacho.

Zvinofanira kucherechedzwa varwere kuti vakawanda vane tariro simba yeruponeso. Masayendisiti US akatanga miedzo kurapwa pane Anti-ichi chirwere. Zvisinei Zvinogoneka,, kuti haana mwana wose anogona kurarama panguva ino. Asi chero zvakadaro, kana miedzo vakabudirira uye vana progeria yakurirwa, ichava kukunda vose vaya vanoita zvinhu zvose kuitira kuti aponese vana vavo kubva yakadaro chirwere chakaipisisa.