Children Progeria: anei oe i na keiki i ka manawa?

Children Progeria - he maʻi o aaiaoe ano, e alakai i ka irreversible loli ma ka na nā loko o ka'ōnaehana aie i ka premature poe oo hoi o ka mea a pau meaola. No ka mea manawa mua i ka maʻi, ua hōʻike 'a i ho'ākāka' ia ma 1889 ma John. Hutchinson, a kū kaʻawale ia ma 1904 ma ka J. Guilford. I loko nō o ka mea i na keiki progeria - he maʻi like pohihihi keia, mahope mai o kona loaʻa ia i ho'ākāka 'oi ma mua o 150 hihia. A me ka pono aʻo kēlā me kēia no ia i ka nui helu o nā kānakaʻepekema.

Wale nō kekahi o ka 7 miliona nā pēpē i kauka diagnose i ka maʻi pale o Hutchinson-Gilford. Children Progeria ua ulu koke - e ka makahiki o ka hoʻomanawanui ka kino makahiki 5-9 makahiki. Nā keiki i loko o pohihihi keia manawa ola mahope aku o ka 23 makahiki o ka makahiki, ka make mai ka maʻi hou typical o ka kūpuna. Ka mua hoailona o ka "wa elemakule, na keiki o ka" hōʻike i na keiki, mai kekahi kakahiaka nui elemakule (2-3 makahiki).

E like me ka mea lapaʻau hakuʻole, i ua, pau i waeia i laau lapaau wale no ka symptomatic iapaau ana o kaʻaoʻao illnesses. Akā,ʻepekema a puni ka honua i ka pilikia i këia a me ka mea imi i ka hoʻokō aoao e hoʻoponopono 'ia.

Wehewehe aku na keiki i ka progeria kēia mau hoike ana:

• uuku ke kino o Iesu;
• malamalama (13-22 kg);
• oiʻili, ma i ipu hiki;
• kali-e neʻe ami o na lima a me na wawae;
• nui poo, akā, i ka mea ia manawa - he wahi kanaka;
• kiʻekiʻe-hoomoana iho la leo.

Nā kumu o ka maʻi

Allʻepekemaʻae aku i nā kamaliʻi progeria mea i ka maʻi, ua ili mai iā ia. Ke ao nei ike wale i kekahiʻohana, ma nā mea a pau 3 keiki ke loaa i ka maʻi pale.

'Aʻole no laila lōʻihi aku nei ka mea i manaʻo i ka mea wale nō kumu no na "keiki ka makahiki" - ka mutation o ka hookahi'ōewe i komo i loko o ke kahua o ke kumuʻiʻo lamin A. i kūkuluʻia ka halepaahao nuclei ma ke kumu o ke kumuʻiʻo. Ma ke kaʻina hana o ka hou 'ana ma prelamina A oʻo kumuʻiʻo ka hoʻolako' ole. Penei, e like me ka hopena lamin A me ka nui loa i kekahi, mai ka maʻamau kumuʻiʻo. Ua hiki aku i keʻano o ka pathological loli i loko o ke kino o ke keiki, a ua wehewehe aku ma ka maʻi Hutchinson-Gilford. Children Progeria hoʻomaka i ka holomua, e kauoha ana premature poe oo hoi.

Akā, kekahi mau kānakaʻepekema i ike ai i kekahi'ōewe i loko o ka DNA, he mutation a inevitably hiki aku ai i ka maʻi. Irreversible keʻano o ka hanaʻana i loko o BAF-1'ōewe -prichina kūpono 'mā i loko o kaʻili lahilahi keena. No ka BAF-1 kekahi hana me nā nā polokina, ke mutation i loko o kona 'ole ke kumu ka haunaele ma ka'ē aʻe nā polokina.

Ua loaʻa kekahi manawa?

I ka lā, kńnaka maka i ka paakiki hana ke hoʻokō 'ia ka hulina study o ke kaʻina hana o ka'ōewe mutation. A ina e loaa ia oe, alaila, na keiki maʻi e i ka maoli e loaa ai i ka poe mamo progeria, a me na kumu - e ole kona hanana.

It E e kaulana ua nui loa nā mea maʻi me ka maule manaolana o ke ola. USʻepekema Ua hoʻomaka 'ana lapaʻau hookolokolo o Anati-i kēia maʻi. He hiki, naʻe, i ole na keiki ke ola mai a hiki i keia manawa. Akā, ma kekahi hihia, ina o ka ho'āʻo eʻana, a me nā keiki progeria ua hee o, ka mea, e lilo ka lanakila no a pau ka poe i hana nā mea a pau ma ka mea e hoola ia kā lākou mau keiki, mai ia i ka maʻi weliweli.