Chii chinonzi VATER Syndrome?

Zvemukati

• Mhedziso
• Chii chinokonzera?
• Ndezvipi zviratidzo?
• Vertebral kukanganisa
• Kukanganisa kweanal
• Kukanganisa kwemoyo
• Tracheoesophageal fistula
• Kukanganisa kwetsvo
• Kuremara kwemaoko
• Zvimwe zviratidzo
• Inoonekwa sei?
• Ndedzipi nzira dzekurapa?
• Outlook

Mhedziso

VATER syndrome, inowanzonzi kusangana kweVATER, iboka rekuremara kwekuzvarwa kunowanzoitika pamwe chete. VATER chidimbu.Tsamba imwe neimwe inomiririra chikamu chemuviri chakakanganiswa:

• vertebrae (musana mapfupa)
• anus
• tracheoesophageal (trachea uye esophagus)
• itsvo (itsvo)

Sangano rinodaidzwa kunzi VACTERL kana moyo (moyo) nemitezo zvikanganiswawo. Sezvo izvi zvinowanzoitika, VACTERL inowanzo iri izwi rakanyatsojeka.

Kuti uonekwe uine VATER kana VACTERL mubatanidzwa, mwana anofanira kunge aine hurema hwekuzvara mune dzinenge nhatu dzeidzi nzvimbo.

VATER / VACTERL kushamwaridzana hakuwanzo. Anofungidzirwa 1 kubva pavane zviuru gumi kusvika makumi mana evana vanozvarwa vaine iri boka remamiriro.

Chii chinokonzera?

Vanachiremba havazive chaizvo chinokonzeresa kusangana kweVATER. Vanotenda kuti kukanganisa kunoitika pakutanga kwekubata.

Iko kusanganiswa kwemajini uye zvakatipoteredza zvinhu zvinogona kuve zvinobatanidzwa. Hapana rimwe geni rakaonekwa, asi vaongorori vakawana mashoma echromosomal zvisizvo uye geni shanduko (shanduko) inoenderana nemamiriro acho. Dzimwe nguva vanopfuura munhu mumwe chete mumhuri imwechete vanozobatwa.

Ndezvipi zviratidzo?

Zviratidzo zvinoenderana nekuti mwana ane hurema hupi.

Vertebral kukanganisa

Kusvika pamakumi masere muzana yevanhu vane mubatanidzwa weVATER vane hurema mumapfupa emusana wavo (vertebrae). Aya matambudziko anogona kusanganisira:

• kushaya mabhonzo muzongoza
• mamwe mapfupa musana
• mapfupa akaumbwa zvisina kujairika
• mapfupa akasunganidzwa pamwechete
• musana wakakombama (scoliosis)
• mbabvu dzekuwedzera

Kukanganisa kweanal

Pakati pe60 ne90 muzana yevanhu vane musangano weVATER vane dambudziko neanus yavo, senge:

• chifukidzo chakaonda pamusoro penzvimbo inovhara kuvhura
• hapana nzira inoenda pakati pezasi kwemukati ura (rectum) uye anus, saka chigaro hachigone kupfuura kubva ura kunze kwemuviri

Matambudziko neanus anogona kukonzera zviratidzo senge:

• dumbu rakazvimba
• kurutsa
• hapana ura, kana mashoma kwazvo ura

Kukanganisa kwemoyo

"C" muVACTERL inomirira "moyo." Zvinetso zvemwoyo zvinokanganisa makumi mana kusvika makumi masere muzana muzana yevanhu vane chirwere ichi. Izvi zvinogona kusanganisira:

• Ventricular septal chirema (VSD). Iri igomba pamadziro rinopatsanura dzimba dzekurudyi nekuruboshwe dzemoyo (ventricles).
• Atrial septal kuremara. Apa ndipo apo gomba pamadziro rinopatsanura makamuri maviri ekumusoro emoyo (atrium).
• Tetralogy yekunyepedzera. Uku ndiko kusanganiswa kweane moyo kukanganisa: VSD, yakawedzera aortic vharuvhu (inodarika aorta), kudzikira kwepulmonary vharuvhu (pulmonary stenosis), uye kuomesa kweiyo kurudyi ventricle (kurudyi ventricular hypertrophy).
• Hypoplastic yakasara moyo syndrome. Apa ndipo apo divi reruboshwe remoyo risinganoumbike zvakanaka, richidzivirira ropa kubva kuyerera kuburikidza nemoyo.
• Patent ductus arteriosus (PDA). PDA inoitika kana paine kuvhurika kusingaenzanisike mune imwe yemidziyo yeropa yemwoyo inodzivirira ropa kubva kuenda kumapapu kunotora oxygen.
• Shanduko yetsinga huru. Tsinga mbiri huru dzinobuda mumoyo dzakadzokera kumashure (dzakashandurwa).

Zviratidzo zvezvinetso zvemwoyo zvinosanganisira:

• kunetseka kufema
• mweya wekufema
• ruvara rwebhuruu kune ganda
• kuneta
• isina kujairika moyo mutinhimira
• kukurumidza kurova kwemoyo
• moyo kugunun'una (whooshing kurira)
• kusadya zvakanaka
• hapana kuwedzera uremu

Tracheoesophageal fistula

Fistula kubatana kusinganzwisisike pakati petrachea (windpipe) uye esophagus (iyo chubhu inotakura chikafu kubva pamuromo kuenda mudumbu). Aya maumbirwo maviri haawanzo kubatana zvachose. Iyo inokanganisa chikafu chinopfuura kubva pahuro kuenda mudumbu, kuchinjisa chimwe chikafu mumapapu.

Zviratidzo zvinosanganisira:

• kufemera chikafu mumapapu
• kukosora kana kudzipa paunenge uchidya
• kurutsa
• ruvara rwebhuruu kune ganda
• kunetseka kufema
• dumbu rakazvimba
• kuwedzera mashoma uremu

Kukanganisa kwetsvo

Vanenge makumi mashanu muzana yevanhu vane VATER / VACTERL vane hurema hwetsvo. Izvi zvinogona kusanganisira:

• itsvo isina kuumbwa (s)
• itsvo dziri munzvimbo isiriyo
• kuvhara kwerini kubva muitsvo
• kuchengetedza weti kubva mudundira kupinda muitsvo

Itsvo kukanganisika kunogona kukonzera kugara urinary turakiti hutachiona. Vakomana vanogona zvakare kuve nehurema umo kuvhurwa kwenhengo yavo kuri pazasi, panzvimbo pekumusoro (hypospadias).

Kuremara kwemaoko

Kusvika makumi manomwe kubva muzana evacheche vane VACTERL vane mitezo yakaremara. Izvi zvinogona kusanganisira:

• kushayikwa kana zvigunwe zvisina kugadzirwa zvakanaka
• mimwe minwe kana zvigunwe (polydactyly)
• minwe yewebhu kana zvigunwe (syndactyly)
• maoko akasimba akasimba

Zvimwe zviratidzo

Zvimwe, zvimwe zvakajairika zviratidzo zveVATER kusangana zvinosanganisira:

• kunonoka kukura
• kutadza kuwedzera uremu
• zvisina kufanira kumeso maficha (asymmetry)
• nzeve kuremara
• mapapu akaremara
• matambudziko nemukadzi kana nhengo yemurume

Izvo zvakakosha kuti uzive kuti VATER / VACTERL kusangana hakukanganise kudzidza kana kusimudzira kwehungwaru.

Inoonekwa sei?

Nekuti kusangana kweVATER isumbu remamiriro, hapana bvunzo imwe inogona kuiongorora. Vanachiremba vanowanzo gadzira chirwere ichi zvichienderana nezviratidzo nezviratidzo zvekiriniki. Vacheche vane chirwere ichi vane zvisungo zvitatu zveVATER kana VACTERL. Izvo zvakakosha kuti ubvise mamwe magene syndromes uye mamiriro anogona kugovana maficha nesangano reVATER / VACTERL.

Ndedzipi nzira dzekurapa?

Kurapa kunoenderana nemhando dzipi dzehurema hwekuzvarwa hunobatanidzwa. Kuvhiya kunogona kugadzirisa akawanda akaremara, kusanganisira matambudziko nekuvhura anal, mapfupa emusana, moyo, neitsvo. Kazhinji maitiro aya anoitwa achangoberekwa mwana.

Nekuti kusangana kweVATER kunosanganisira masystem akati wandei, vanachiremba vashoma vanoirapa, kusanganisira:

• cardiologist (matambudziko emwoyo)
• gastroenterologist (GI turakiti)
• nyanzvi yemapfupa (mapfupa)
• urologist (itsvo, dundira, uye zvimwe zvikamu zveiyo weti system)

Vana vane musangano weVATER vanowanzoda kuongororwa kweupenyu hwese uye kurapwa kudzivirira matambudziko anotevera. Ivo vangangodawo rubatsiro kubva kuna nyanzvi senge chiremba wepanyama uye wezvekurapa.

Outlook

Maonero anoenderana nemhando dzipi dzekukanganisa munhu anadzo, uye kuti matambudziko aya anorapwa sei. Kazhinji vanhu vane musangano weVACTERL vanove nezviratidzo muhupenyu hwavo hwese. Asi nemabatirwo akanaka, vanogona kurarama hupenyu hune hutano.