Kupisa kunogona dzimwe nguva kusave kwakasiyana kubva pakuberekwa. Mune chimiro chisina kujairika chimiro, chinowanzonzi kutukwa kwaOndine, izvi zvinogona kunge zvakakomba uye zvine migumisiro inokosha. Chii chine kutuka kwaOndine? Ongorora zvimwe zvezvikonzero zvinokonzerwa ne congenital central hypoventilation syndrome uye kuti inogona sei kurapwa zvakanaka.
Chii Chinonzi Kutuka Kwemhuka?
Ondine's cursor (inozivikanwawo se congenital central hypoventilation syndrome kana CCHS) inoratidzika, iyo yakakomba yomuitiro wokurara wepabonde umo munhu akabatwa akarega zvachose kufema paanovata.
Inowanzotanga kuberekwa, kureva kuti iripo kubva pakuberekwa. Zvinogona kuonekwa muNeonatal unit mushure mokuberekwa. Central apnea yokurara inoratidzirwa neuropi inotadza kukurumidza kufema kufema. Izvi zvinoratidzika kuti ndezvikonzero nekuda kwekuderera kwekutadza kusvika kune yakakwirira ye carbon carbon and low oxygen levels in the blood. Izvi zvinowanzova nengozi munguva yekurara.
Kutuka kwaOndine kunonzi mushure mezvinyorwa zvenhoroondo pamusoro pemvura inokanganiswa nymph inotuka murume wake asina kutendeka kuti arege kufema kana akazorora zvakare. Mumashoko ekurapa, central hypoventilation inomirira nzira yakanyanyisa yekupona kwepabonde.
Chii Chinokonzera Central Hypoventilation?
Central hypoventilation inosangana neyese mumamiriyoni makumi matatu evanhu, izvo zvinoreva kuti mazana mashoma chete vanhu vanezvo munyika. Sezvo zvakadaro, inofungidzirwa seicho chisingawanzoitiki. A genetic mutation inoratidzika seicho chikonzero chinokonzera.
Inofungidzirwa kuti inoitika kana uropi husingakwanisi kukurudzira kupemha, sezvinogona kuonekwawo mukati mekurara kwepabonde .
Izvi zvinogona kunge zviripo kubva pakuberekwa kana zvinogona kukura mushure mekukuvara kweuropiyo, iyo inotungamirira kufamba kunofema. Central hypoventilation inogona kusanganiswa nekutambudzika kukumedza, matambudziko emukati anonzi chirwere cheHirschsprung , kana matumire anonzi neuroblastoma .
Kunyange zvazvo mamiriro acho ezvinhu anowanzoitika zvishoma nezvishoma, pangava nehutano hwemazwi hunotungamirira mumhuri. Hama dzinogona kunge dzine hutsika hwakaoma hwekusava nehuno hunoita hutachiona hwehurumende. Dambudziko guru rinoratidzika kunge rakakonzerwa nekuchinja kwePHOX2B (4q13) geni. Yakawanda inosanganisira kupararira kwemazwi ekudzoka kubva mabheti makumi maviri (anonzi alanine) kusvika kuma25 kusvika ku33 zvigadziko. Munenge 10% yezviitiko, dzimwe shanduko pane imwe nzvimbo dzinoita seinoita basa.
Ko Central Hypoventilation inotariswa sei?
Vanhu vazhinji vakavhiringidzika vanotanga nguva pfupi mushure mekuberekwa. Izvo zvave zvichitaurirwa kuti zvitange gare gare, zvisinei, pamwe nematambudziko makuru akanyorwa. Zviratidzo zvinogona kubudiswa mumatambudziko akaoma nekushandiswa kweanesthesia kana sedatives.
Kurapa kwechiitiko ichi kunosanganisira kushandiswa kwemhepo inosangana ne tracheostomy tube iri mberi kwehuro apo chero munhu akabatwa anoenda kunorara, kunyange panguva yepamusoro. Kana izvi zvisina kushandiswa, mumwe munhu ane chirwere ichi aigona kufa chero nguva yavanorara.
Pamusana pemhando yekurapa, mhuri dzeavo vanotambura dzinowanzova nyanzvi pakuchengetedza midziyo inofanirwa kuchengetedza kufema kufema. Iko kunotanga kuita sekutyisa, asi rubatsiro mukati mekugadzirwa kwechipatara inobvumira kushanduka kwakanaka kune kurapa kumba.
Nhungamiro kubva kumapfupa ekupora, kusanganisira pamba-pfuma, inogona kugadzirisa kuchinja uku.
Shoko Rinobva
Kana iwe uchida kudzidza zvakawanda pamusoro pekutukwa kwaOndine, kunyanya kana iwe une mwana akaremara, zvinokurudzirwa kuti uone kubvunzurudza nevana vepmatologologist pachikoro chekurapa. Nokuda kwehuwandu hwemamiriro acho ezvinhu, uye kuipa kwemigumisiro inogona kuitika, kutarisira kwenyanzvi kunotanga kudiwa. Izvo zvinogonawo kuve nyore kugadzirisa pamwe nedzimwe mhuri dzinotambura dzinotarisira chirwere chacho. Izvi zvinogona kubatsira kune zvikonzero zvakawanda.
Shingairira kuwana rubatsiro rwaunoda kubva kune nyanzvi yekuvandudza mararamiro uye hupenyu hwemwana wako nemhuri.
> Sources:
> Amiel J et al . "Polyalanine kuwedzera uye kuchinja kwemafashoni emapuranga anonzi homeobox gene PHOX2B mu congenital central hypoventilation syndrome." Nat Genet 2003; 33 (4): 459-461.
> Chen ML, Keens TG. "Congenital central hypoventilation syndrome: kwete chimwe chinetso chisingawanzoitiki." Paediatr Respir Rev 2004; 5 (3): 182-189.
> Faraco J uye Mignot E. "Mazinza Okurara Uye Kurara Kwevanhu." MuMitemo neMabasa ekurapa Mishonga , Elsevier, 2011, mapeji 93-94.