Potter chirwere: zvinokonzera, zviratidzo, kuongorora chirwere

Potter chirwere chinonzi chakakomba kuremara azvarwa. Characterization anotanga tsanangudzo anomaly ichi.

Chii Potter Syndrome?

Wehari chirwere - a vachiberekwa kuremara ari mudumbu, umo iye zvakakwana ndisipo itsvo, inova mhedzisiro kuderedza Vhoriyamu Líquido amniótico.

Mugumisiro mvura chishoma ari compression ari mudumbu mudumbu. Vana vari vakacherechedzawo havaratidzi dzakabudirira nomumapapu, kwese Enrugado chiso yaidzipira dehenya, akaremara maoko nemakumbo.

Muna 1964 godu Potter haareururi kuwedzera chakaremara akanga dzinoratidzwa agenesis (kushayikwa) vaviri fetus itsvo kana aplasia (kusavapo chero nhengo kana dzomuviri), pamwe chete chiso abnormalities. chirwere ichi kunotaurwa mune zvokurapa mabhuku uyewo chirwere Potter pamwe renal agenesis 2, renal-chiso great urwere, chiso renal dysplasia.

Wehari chirwere ari mudumbu: Zvinokonzera

The aetiology (kwakabva) chirwere haisi aizvinzwisisa. Muna 50% kubva kacho Vakawana kuti chikuru abnormality kunoratidzwa nokuda kushayikwa Líquido mvura (fetus mvura) ari mukadzi pamuviri. Compression of fetus dumbu zvinotungamirira chitange renal anomalies vanosangana (flattening uye flattening), mwoyo, rectal, pasikarudzi, zvemapapu (hypoplasia), migumo (clubfoot).

Potter chirwere kunoitika muna vacheche, vana pasi pemakore mishomanana, dzinenge - Vana vakuru gore rimwe, uye kakawanda vakomana. Kuitika ndiko kuremara :. 1 kusvika 50 zviuru kuzvarwa.

Imwe ongororo yakaitwa vabereki

Chii zviratidzo zviri chirwere achabatsirwa Potter ari mudumbu? Large chena itsvo - ndicho chii chinogona kuoneka Ultrasound. agenesis Izvi zvinogona dominantly nhaka (nhodzera mumwe mubereki haagoni vasachiziva), kureva haana chokuita uwandu Líquido amniótico. Mumwe vabereki vangave underdevelopment kana kusavapo mumwe itsvo, izvo zvinogona kuregeredzwa zvokurapa bvunzo adarika.

Renal agenesis nhaka dominantly, izvo zvinoreva kuti pane 50% ndingangokuvara kuberekwa mudumbu, izvo zvichaita kuitwa Potter syndrome.

Potter chirwere pamwe 75 muzana mukana zvingaitika mune mwana apo vabereki vake akawana mutation iri Gene PKHD1. Zvaitika Paavhareji 1 muna 50 vanhu. kunotouraya izvi haanamati kukurumidza kukura ndiko kuremara iri nhau, vanogona kwemakumi emakore apfuura kubva kumarudzi namarudzi.

Ngozi Kuberekwa mwana pamwe chirwere anogona kuratidza Potter nhaka kunotouraya kuvabereki vose. Kana mudumbu nemagene kuchinja achapiwa vakapfuura chete baba kana mai, ane 50 muzana mukana iye achaberekwa vatano. 25% yevana regai nhaka PKHD1 mutation kubva vabereki vose, vane chero kuchinja panguva majini chechetere.

urwere symptomatology

chirwere Potter ari mudumbu Diagnostics anoratidza zvinotevera zvinoratidza:

• zvikuru yakamanikana maburi remakore;
• maitiro muforo pasi mutsetse remakore;
• mandibular hypoplasia (mikrognatsiya);
• sandara mhino;
• kure zvisinganzwisisiki guru pakati aiwanzopiwa wokushanda nhengo (akadai nameso) - hypertelorism;
• convex epicanthus ( "Mongolian ukagunzva") - chaiyo mudanga iri romukati kukona ziso, kufukidza lacrimal tubercle;
• zvemamiriro chimiro nyoro nzeve hombe.

Disinfection

Kana mwana azvarwa huru chiratidzo - zvakakomba kufema nenhamo, izvo hworatidza pacharo pamwe maminitsi yakazvimirira upenyu chokutanga. Kuitisa kufefetedza kuoma pneumothorax (mhepo matema iri pleural munharaunda kuchidzivisa chiedza mutinhimira kufamba).

Zvinosuruvarisa, ruzhinji kwevacheche chirwere Potter kwakaita chirwere kufa maawa mashomanana pashure pokuberekwa. Kana vakareruka siyana uchirondedzera digestivnogo (zvomudumbu) turakiti - urogenital cloaca (mukomboni pamusoro mumagaro uye urogenital mutsinga dzinofamba nemukaka rimwe), hapana perforation pamusoro horo (akarurama kunyoresa) - rinoshandiswa kuvhiyiwa.

Kupukunyuka mwana chirwere Potter

Muna 2013, nhepfenyuro akashuma musikana achangoberekwa Abigail Butler, mwanasikana nhengo House of United States House of Representatives, Republican Congresswoman Jaime Herrera Beutler. Mwana akakwanisa kurarama ne chirwere ichi kuti kunodzivisa kunowanzoitika kushanda kufema hurongwa.

Panguva 5th mwedzi yenhumbu, mukadzi akadzidza kuti musikana haamiri kubva weti kuti kusavapo itsvo rakakwana. Chikonzero chaiva kuperevedza Líquido mvura mune pamuviri Jaime Herrera Beutler. Pasinei kwemamiriro vanachiremba akazivisa iyi dzinouraya, mukadzi uye murume wake akasarudza kuramba pamuviri. Kuti kuripa shoma Líquido mvura iri dumbu Jaime raitongwa chinokosha sesaline. John Hopkins - chiremba vaichengeta kurapa, asingagoni zvachose pave vakaroorana, kuti kurapwa hunotungamirira inogutsa mugumisiro.

Asi July 15, 2013 Little Abigail akaberekwa mupenyu. Ndangariro kwaAmai, musikana haana mhere pakuzvarwa, asi pashure penguva yakati vakatanga kuchema - vomwana mumapapu kushanda. Mushure aberekwa, akanga pakarepo kune dialysis - hurongwa kuti zvachose unotsiva itsvo basa kuti anoratidza zvibereko muviri mumuviri. Kwapera gore, iye ari chikatsiviwa anyatsoronga itsvo.

A mukana kuberekwa mhenyu mwana chirwere ane Potter uye ake hunotevedzana akabudirira vapore ari ezvinhu mazuva ano ichiri kunzi chishamiso. Naizvozvo, vanachiremba chandagwinyira pamuviri iri nechirwere ari mudumbu zvakakanganisika yakadaro. Muenzaniso Abigail Butler haagoni zvinokurudzira, asi zvakakosha kunzwisisa kuti uyu asina mhosva.