Potter syndrome: take, tohu, tātaritanga

huaina Potter syndrome ko te koha nui o te kukune. timata kiripuaki ki te whakamāramatanga o tenei Pākatokato.

He aha te mea Potter Syndrome?

syndrome o Potter - he koha congenital o te kukune, i roto i nei e ia whatukuhu tino ngaro, i te mea te putanga o te whakaiti i te rōrahi o te waikahu.

Ko te hua o te scarcity wai ko te kōpeketanga o te kukune i roto i te kopu. E kitea ana hoki ngā tamariki painga whakawhanakehia pūkahukahu, angamaheni mata mingimingi me angaanga notia, waewae i hira ake.

I roto i te 1964 whaki godu Potter āhuatanga he koha whanaketanga i agenesis (kore o) e rua tākihi fetal aplasia ranei (ngaro o tetahi okana tinana ranei wahi), ngā ki abnormalities kanohi. Kei te tuku i tēnei syndrome ki roto i te tuhinga hauora me te syndrome Potter ki agenesis tākihi 2, tākihi-kanohi syndrome große, kanohi dysplasia tākihi.

syndrome o Potter i te kukune: Take

e kore tonu e matau tino te aetiology (take) syndrome. I roto i te 50% o te wā i kitea ai e whakakitea te pīari tuatahi te tika ki te kore o te waikahu (wai fetal) i roto i te wahine hapu. Kōpeketanga o te kōpū fetal arata'i ki te whanaketanga o kōhikohiko tākihi fehangahangai (flattening me te flattening), ngakau, rectal, taihemahema, pūkahukahu (hypoplasia), pito (clubfoot).

Puta Potter syndrome i roto i nga kohungahunga, tamariki i raro i te tau o te marama torutoru, i te iti rawa - i roto i ngā tamariki pakeke ake i te tau kotahi, me te ake maha i roto i te mau tamaroa. Ko te pā o te koha :. 1 ki te 50 mano whakatupuranga.

He rangahau o ngā mātua

He aha e haere tahi i te tohu o te syndrome e Potter i te kukune? tākihi white nui - ko tenei te mea e taea te kite i runga i ultrasound. Ka taea e tēnei agenesis hei whakarerenga iho mo dominantly (whakapapa o te tahi atu mātua e kore e taea e te pehi i te reira), arā e kore e pā ana ki te nui o te waikahu. Ko tētahi o nga mātua kia underdevelopment ngaro o tetahi o nga whatukuhu, e taea te wareware i roto i ngā whakamātautau hauora mua ranei.

agenesis tākihi riro te dominantly, e te tikanga e reira ko te 50% tūponotanga o whanau o te kukune, ka e kia kitea Potter syndrome.

Potter syndrome ki 75 ōrau tūponotanga kia puta i roto i te tamaiti, ka kitea e tona matua he irakētanga i roto i te PKHD1 ira. puta ratou i runga i toharite 1 i roto i te 50 iwi. e kore e ēnei irakētanga arahi ki te whanaketanga tere o te koha i te pāpāho, e taea hoki tekau haere ratou i runga i i whakatupuranga ki whakatupuranga.

Ko te mōrea o te whanau o te tamaiti ki te syndrome taea faaite mai Potter tupu o irakētanga i ngā mātua e rua. Ka Ki te ka haere nga huringa iranga te kukune te papa anake te whaea ranei, ki te tupono noa 50 ōrau e whanau ia hauora. 25% o ngā tamariki e kore e whiwhi PKHD1 irakētanga i ngā mātua e rua, whai i tetahi huringa i te taumata ira.

tohutanga syndrome

syndrome o Potter i te tātaritanga kukune heheu te mau faaiteraa e whai ake nei:

• tino whāiti rau āputa;
• parautanga āhuatanga i raro i te rārangi o te rau tau;
• hypoplasia mandibular (mikrognatsiya);
• ihu parehe;
• tawhiti tomuri nui i waenganui i nga okana takirua (pērā i kanohi) - hypertelorism;
• epicanthus koropuku ( "puninga Monokōriana") - he puninga motuhake i te kokonga i roto rawa o te kanohi, te uhi i te tubercle lacrimal;
• āhua rerekē ngohengohe taringa nui.

disinfection

A, no te whanau te tamaiti ko te tohu matua - nui mamae romahā, e faaite ake ki te meneti tuatahi o te ora motuhake. A faatere ventilation pneumothorax faingataa (mirumiru hau i roto i te wāhi pleural e arai atu kaupapa naki marama).

Kia aroha mai, te nuinga rawa o nga kohungahunga pāngia ki te syndrome o Potter mate he torutoru haora i muri i whanau. A, no te nga puka mama e pā ana digestivnogo (kūnatu) tuleki - cloaca urogenital (te pūhui o te tau mahani whero me te urogenital i roto i tetahi), kahore he perforation o te nono (rēhitatanga tika) - whakamahia te pokanga.

Ora syndrome tamaiti Potter

I te 2013, korerotia te pāpāho i runga i te kotiro hou Apikaira Butler, tamahine o te mema o te Whare o te United States Whare o ngā Māngai, Republican Congresswoman Jaime Herrera Beutler. i taea e ki te ora ki tenei syndrome e ārai te mahi noa o te pūnaha romahā te tamaiti.

I te 5 o nga marama o te hapūtanga, ako te wahine e kore e te kotiro e tu i roto i te mimi ki te ngaro oti o whatukuhu. Ko te take he kaimahi o waikahu i roto i te hapu Jaime Herrera Beutler. Ahakoa whakapuakina te mutunga o nga kaiwhakaako tenei take mate, te wahine, me tana tane faaoti ki tonu i te hapūtanga. I roto i te tikanga ki te utu i te nui iti o te waikahu i roto i te kōpū whakahaere Jaime saline motuhake. John Hopkins - te tākuta nei i mahi te therapy, kihai i taea e tino kī te hoa faaipoipo, e maimoatanga ka arahi ki te hua pai.

Ko te Hōngongoi 15, whanau 2013 Little Apikaira i ora. pūmahara Mother o, kihai te kotiro i karanga i te whanautanga, engari i muri i te ia ka anga ki te tangi - pūkahukahu o pēpi mahi. I muri i te whanautanga, i whakawhitia tonu ia ki te tātari - te pūnaha e tino whakakapi tākihi mahi e whakaatu nga hua o te pākia i te tinana. He tau i muri mai, kua whakakapi ia e te tākihi kaituku.

Kei te whakaaro tonu te whai wāhi hoki te whanautanga o te tamaiti syndrome ora Potter me tona whakanohonoho angitu muri i roto i te tikanga o mau tupuraa mau hou te semeio. Na reira, tohe kaiwhakaako i runga i te materoto i roto i te tātaritanga o te kukune te koha taua. e kore e taea e akiaki Tauira Apikaira Butler, engari he mea e tika ana ki te matau e ko he take tino tenei.