Tamariki Progeria: E whai koe tamariki te tupono?

Tamariki Progeria - he mate o huru ira, ārahi ki huringa tina heke i roto i te whekau o te pūnaha e tika ana ki te hoholo taimi halá, o te rauropi katoa. Hoki te wā tuatahi kua te mate kua tāutuhia, ka whakaahuatia i roto i te 1889 e Hoani. Hutchinson me takitahi i roto i 1904 e J. Guilford. Ahakoa te meka e tamariki progeria - he mate tino onge, mai whakaahuatia tona kitea neke atu i 150 take. A āta ako ia o ratou he maha nui o pūtaiao.

tetahi o te 7 miriona hou tākuta anake te tātari i te syndrome o Hutchinson-Gilford. Kei te whakawhanake ana ngā tamariki Progeria tere - noa te tau tau tinana o te manawanui 5-9 tau. Ko te tamariki i roto i te wā onge ora ki 23 tau o tau, mate i te mate atu angamaheni o te feia ruhiruhia. Ko te tohu tuatahi o "koroheketanga, a ngā tamariki" puta i roto i te tamariki i te wawe tau (2-3 tau).

Ka rite taua maimoatanga e kore e tīari, e te, kua whakaritea rongoā anake mo te maimoatanga symptomatic o mate taha. Ko pūtaiao huri noa te ao e ako i te raruraru, me te e rapu mō huarahi whai hua ki te whakatutuki i te reira.

progeria e whai ake nei mau faaiteraa a ngā tamariki āhuatanga:

• iti roa;
• marama (13-22 kg);
• pai kiri, i roto i nei oko kitea;
• puhoi-neke hononga o nga ringa, me waewae;
• upoko nui, engari i te wa ano - he tangata iti;
• reo nui-noho.

Ko te take o te mate

whakaae kaipūtaiao katoa e kore te mea tamariki progeria he mate, riro te. mohiotia te ao anake ki tetahi utuafare, te wahi riro katoa 3 tamariki te syndrome.

E kore e pera roa i mua i whakaaro ai e anake te take mo te "tau o ngā tamariki" - te irakētanga o te ira kotahi wāhi e te roto i te hanganga o te A. pūmua lamin e hanga nga karihi pūtau i runga i te pūtake o te pūmua. I roto i te tukanga o te hurihanga i roto i prelamina te rahunga pūmua he pakeke. Ko te kupu, kia rite ki te lamin hua te tino rerekē i te pūmua noa. arata'i tenei ki te momo o huringa pathological i roto i te tinana o te tamaiti, āhuatanga nei i te mate Hutchinson-Gilford. Tamariki Progeria timata ki te ahu whakamua, meinga hoholo taimi halá.

Ko kitea etahi pūtaiao i tetahi ira i roto i te DNA, te irakētanga e arata'i ki te mate. tukanga tina heke i roto i te BAF-1 ira hē whanaketanga -prichina i roto i te pūtau membrane. Mai pāhekoheko hoki BAF-1 ki ētahi atu pūmua, te irakētanga i roto i tona hanganga ai raruraru i roto i te tahi atu pūmua.

He reira he tupono?

Ki te rā, pūtaiao kanohi he mahi uaua whakaoti i te ako whānui o te tukanga o te irakētanga ira. A, ki te angitu ratou, ka ka whai i te whai wāhi tūturu ki te riro i te progeria tamariki, me kaiwhakaako nga tamariki mate - ki te ārai i tona tupu.

Me kï reira e maha tūroro ki te tumanako hemo o te ora. Kua tīmata US pūtaiao tamataraa haumanu o anti-tenei mate. Ko taea, Heoi, e kore e taea e te tamaiti katoa ora ake ki tenei wa. Otiia i roto i tetahi take, ki te he angitu nga whakamātautau me patua te progeria tamariki te, ka waiho hei wikitoria mo te hunga katoa e mahi nga mea katoa i roto i te tikanga ki te whakaora ratou tamariki i te taua mate e wehingia ana.