Woumba syndrome: zimayambitsa, zizindikiro, matenda

syndrome woumba amatchedwa chilema kwambiri mwana wosabadwayo. Mapangidwe kuyamba ndi tanthauzo la anomaly izi.

Woumba Syndrome ndi chiyani?

Woumba mbiya matenda - ndi kobadwa nako chilema cha mwana wosabadwayo, limene palibe kwathunthu impso, amene ndi zotsatira za kuchepetsa voliyumu ya madzimadzi amniotic.

The chifukwa cha wosaperewera madzi ndi psinjika wa mwana wosabadwayo m'mimba. Ana anatinso mapapo bwino akupangidwa lililonse wamakwinya nkhope ndi zopotola Chigaza, opunduka miyendo.

Mu 1964 godu Muumbi akuwulula zinthu chilema anali yodziwika agenesis (kusowa) onse yoopsa impso kapena aplasia (pakalibe chiwalo kapena thupi mbali iliyonse), pamodzi ndi kuvutika nkhope. syndrome Izi akutchulidwa mu mabuku mankhwala komanso matenda Wowumba ndi aimpso agenesis 2, aimpso-nkhope Grosse syndrome, nkhope aimpso dysplasia.

syndrome woumba mbiya mu mwana wosabadwayo: Zoyambitsa

The aetiology (chiyambi) syndrome akadali asanamvetsetse. 50% milandu chinapezeka kuti chosayenerera chachikulu akuwonekera chifukwa kupanda madzimadzi amniotic (yoopsa madzi) mwa mkazi wapakati. Psinjika a chiberekero yoopsa kumabweretsa chitukuko cha anomalies aimpso maso (flattening ndi flattening), mtima, thumbo, kumaliseche, m'mapapo (hypoplasia), malekezero (clubfoot).

Woumba syndrome amapezeka makanda, ana osakwana zaka miyezi ingapo, osachepera - ana wamkulu kuposa chaka chimodzi, ndipo nthawi zambiri anyamata. Kuchuluka kwa chilema :. kubadwa 1 okwana 50.

A kafukufuku wa makolo

Kodi zizindikiro za matendawa limodzi ndi Muumbi mu mwana wosabadwayo? Large woyera impso - izi ndi zimene Tingaone pa ultrasound. agenesis Zimenezi zingakhale dominantly adzalandira (chibadwa cha kholo sangathe kuletsa izo), mwachitsanzo zosagwirizana ndi kuchuluka kwa madzimadzi amniotic. Mmodzi wa makolo angakhale underdevelopment kapena kusapezeka kwa impso, amene akhoza analekerera mayeso zachipatala kale.

Aimpso agenesis ndi tinatengera dominantly, kutanthauza kuti pali 50% Mwina kubadwa kwa mwana wosabadwayo, zomwe anaona syndrome Muumbi.

Woumba syndrome ndi 75 peresenti Mwina angagwe mu mwana ngati makolo ake anapeza kusintha mu PKHD1 jini. Zidzachitika pafupifupi 1 50 anthu. masinthidwe izi kungathandize kuti apange mofulumira chilema pa TV, iwo akhoza kwa zaka zapita pa ku mibadwomibadwo.

Chiopsezo kubadwa kwa mwana ndi matendawa angasonyeze Muumbi cholowa cha masinthidwe kwa makolo onse. Ngati mwana wosabadwayo kusintha chibadwa adzakhala zapita okha bambo kapena mayi, ndi mwayi 50 peresenti iye adzabadwa wathanzi. 25% ya ana sadzalowa PKHD1 kusintha kwa makolo onse, ndi kusintha kulikonse pa mlingo chibadwa.

syndrome symptomatology

syndrome woumba mbiya mu diagnostics wosabadwa limasonyeza mawonetseredwe zotsatirazi:

• mipata yopapatiza kwambiri m'zaka;
• khalidwe mzere pansi pa mzere wa m'ma;
• mandibular hypoplasia (mikrognatsiya);
• lathyathyathya mphuno;
• Mtunda abnormally lalikulu pakati pa ziwalo wophatikizidwa (monga maso) - hypertelorism;
• otukukira kunja epicanthus ( "Mongolian khola") - ndi khola wapadera pa ngodya lamkati la diso, kuphimba tubercle lacrimal;
• nthenda mawonekedwe zofewa makutu chachikulu.

disinfection

Pamene mwana wabadwa ndi chizindikiro chachikulu - aakulu kupuma mavuto, amene kumaonekera ndi mphindi loyamba la ufulu. Kuchititsa mpweya kobvuta pneumothorax (mpweya thovu m'dera pleural amene amachepetsa kuwala rhythmic kayendedwe).

Mwatsoka, namtindi wa makanda anapezeka ndi matenda Muumbi akafa maola ochepa pambuyo pa kubadwa. Pamene mitundu ya mbandakucha zokhudza digestivnogo (m'mimba) thirakiti - urogenital cloaca (pawiri wa kumatako ndi urogenital ritsa mu umodzi), palibe perforation wa chotulukira (kulembetsa zolondola) - ntchito opaleshoni.

Kupulumuka syndrome mwana Muumbi

Mu 2013, atolankhani lipoti la mtsikana akhanda Abigail Butler, mwana wamkazi wa membala wa Nyumba ya United States Nyumba ya oimira, Republican Congresswoman Jaime HERRERA Beutler. Mwana anali ndi moyo ndi matenda izi zoletsa kuti uziyenda yachibadwa a dongosolo kupuma.

Pa mwezi 5 mimba, mkazi anamva kuti mtsikanayo alibe kuima kuchokera mkodzo kwa anasiya impso. chifukwa anali kusowa kwa amniotic madzimadzi mu mimba Jaime HERRERA Beutler. Ngakhale mapeto a madokotala analengeza Pankhaniyi oopsa, mwamuna ndi mkazi wake anaganiza kupitiriza mimba. Kuti azilipira pang'ono madzimadzi amniotic mu chiberekero Jaime kutumikiridwa saline wapadera. John Hopkins - dokotala amene anachita mankhwala, sindinakhoze mwamtheradi mutsimikizira pabanja, kuti chithandizo adzatsogolera zotsatira zokhutitsa.

Koma July 15, 2013 Little Abigail anabadwa moyo. chokumbukira mayi, mtsikana sikankalira pobadwa, koma patapita kanthawi anayamba kulira - mapapo mwana ntchito. Mwana akabadwa, iye anali pomwepo kunditumiza ku dialysis - dongosolo kuti kwathunthu m'malo impso ntchito imene amasonyeza katundu wa kagayidwe thupi. Chaka chotsatira, iye m'malo mwa impso mwiniwake.

A mwayi kwa kubadwa kwa moyo mwana syndrome Wowumba ndi bwino pambuyo pake konzanso mu zinthu zenizeni ano chimaonedwabe chozizwitsa. Choncho, madokotala amaumirira kuchotsa mimba ndi matenda a mwana wosabadwayo ngati chilema. Mwachitsanzo Abigail Butler simungathe olimbikitsa, koma m'pofunika kumvetsa kuti ndi nkhani yapadera.