Kodi VATER Syndrome ndi chiyani?

Zamkati

• Chidule
• Zimayambitsa chiyani?
• Zizindikiro zake ndi ziti?
• Vertebral zopindika
• Zolakwika za kumatako
• Zolakwika zamtima
• Fistula ya Tracheoesophageal
• Zowonongeka
• Kupunduka kwa ziwalo
• Zizindikiro zina
• Kodi amapezeka bwanji?
• Kodi njira zamankhwala ndi ziti?
• Chiwonetsero

Chidule

Matenda a VATER, omwe nthawi zambiri amatchedwa mgwirizano wa VATER, ndi gulu la zolakwika zobadwa zomwe zimachitika limodzi. VATER ndichidule.Kalata iliyonse imayimira gawo la thupi lomwe lakhudzidwa:

• mafupa a msana (mafupa a msana)
• chotulukira
• tracheoesophageal (trachea ndi esophagus)
• impso (impso)

Bungweli limatchedwa VACTERL ngati mtima (mtima) ndi ziwalo zimakhudzidwanso. Monga momwe zimakhalira nthawi zambiri, VACTERL nthawi zambiri imakhala nthawi yolondola kwambiri.

Kuti mupezeke ndi mgwirizano wa VATER kapena VACTERL, mwana ayenera kukhala ndi zolepheretsa kubadwa m'malo atatu mwa awa.

Mgwirizano wa VATER / VACTERL ndi osowa. Pafupifupi mwana m'modzi mwa ana 10,000 mpaka 40,000 amabadwa ndi izi.

Zimayambitsa chiyani?

Madokotala sakudziwa zomwe zimayambitsa kuyanjana kwa VATER. Amakhulupirira kuti zolakwikazo zimachitika msanga ali ndi pakati.

Kuphatikiza kwa majini ndi zinthu zachilengedwe zitha kuphatikizidwa. Palibe jini imodzi yomwe yadziwika, koma ofufuza apeza zovuta zingapo za chromosomal komanso kusintha kwa majini (masinthidwe) okhudzana ndi vutoli. Nthawi zina anthu opitilira m'modzi m'banja limodzi amakhudzidwa.

Zizindikiro zake ndi ziti?

Zizindikiro zake zimadalira zovuta zomwe mwana amakhala nazo.

Vertebral zopindika

Kufikira 80 peresenti ya anthu omwe ali ndi mgwirizano wa VATER ali ndi zopindika m'mafupa a msana wawo (vertebrae). Mavutowa atha kuphatikiza:

• akusowa mafupa mumsana
• mafupa owonjezera pamsana
• mafupa opangidwa modabwitsa
• mafupa olumikizidwa pamodzi
• msana wopindika (scoliosis)
• nthiti zowonjezera

Zolakwika za kumatako

Pakati pa 60 ndi 90 peresenti ya anthu omwe ali ndi mgwirizano wa VATER ali ndi vuto ndi anus yawo, monga:

• chophimba chochepa pamphako chotchinga kutsegula
• palibe njira yopita pakati pamatumbo akulu (rectum) ndi anus, kotero chopondapo sichingadutse kuchokera m'matumbo kutuluka mthupi

Mavuto ndi anus angayambitse zizindikiro monga:

• mimba yotupa
• kusanza
• osayenda matumbo, kapena matumbo ochepa kwambiri

Zolakwika zamtima

"C" mu VACTERL amatanthauza "mtima." Mavuto amtima amakhudza 40 mpaka 80 peresenti ya anthu omwe ali ndi vutoli. Izi zingaphatikizepo:

• Ventricular septal defect (VSD). Ili ndi dzenje pakhoma lomwe limagawa zipinda zam'munsi zamanzere (zamitsempha).
• Matenda osokoneza bongo. Apa ndipomwe bowo pakhoma limagawanitsa zipinda ziwiri zakumtunda za mtima (atrium).
• Zolemba Zachinyengo. Izi ndizophatikizira zopindika zinayi zamtima: VSD, valavu yotakata ya aortic (yopitilira aorta), kuchepa kwa valavu yamapapo (pulmonary stenosis), ndi kukulira kwa ventricle yolondola (ventricular hypertrophy).
• Matenda a mtima otsalira. Apa ndipamene mbali yakumanzere ya mtima siyimangidwe bwino, yopewetsa magazi kuyenda mumtima.
• Maluso a patent ductus arteriosus (PDA). PDA imachitika pakakhala kutseguka kwachilendo m'modzi mwamitsempha yama mtima yomwe imalepheretsa magazi kuti asapite m'mapapu kukatenga mpweya.
• Kusintha kwa mitsempha yayikulu. Mitsempha ikuluikulu iwiri yochokera mumtima ili kumbuyo (yotembenuzidwa).

Zizindikiro za mavuto amtima ndi monga:

• kuvuta kupuma
• kupuma movutikira
• Mtundu wabuluu pakhungu
• kutopa
• nyimbo yachilendo
• kuthamanga kwa mtima
• kung'ung'uza mtima (kumveka phokoso)
• kusadya bwino
• palibe kunenepa

Fistula ya Tracheoesophageal

Fistula ndikulumikizana kwachilendo pakati pa trachea (mphepo yamkuntho) ndi khosi (chubu chomwe chimanyamula chakudya kuchokera mkamwa kupita kumimba). Nyumba ziwirizi sizimalumikizidwa konse. Zimasokoneza chakudya chodutsa pakhosi kupita m'mimba, ndikumapatulira chakudya m'mapapu.

Zizindikiro zake ndi izi:

• kupumira chakudya m'mapapu
• kutsokomola kapena kutsamwa uku mukudyetsa
• kusanza
• Mtundu wabuluu pakhungu
• kuvuta kupuma
• mimba yotupa
• kunenepa pang'ono

Zowonongeka

Pafupifupi 50 peresenti ya anthu omwe ali ndi VATER / VACTERL ali ndi vuto la impso. Izi zingaphatikizepo:

• impso (s) zopangidwa bwino
• impso zomwe zili m'malo olakwika
• kutsekeka kwamkodzo kuchokera mu impso
• kusunga mkodzo kuchokera mu chikhodzodzo mu impso

Zofooka za impso zimatha kuyambitsa matenda opitilira mkodzo pafupipafupi. Anyamata amathanso kukhala ndi chilema momwe kutsegula kwa mbolo yawo kumakhala pansi, m'malo mokhala nsonga (hypospadias).

Kupunduka kwa ziwalo

Mpaka 70 peresenti ya ana omwe ali ndi VACTERL ali ndi zilema zamiyendo. Izi zingaphatikizepo:

• kusowa kapena kusakula bwino kwa thupi
• zala kapena zala zazing'ono (polydactyly)
• zala zazing'ono kapena zala (syndactyly)
• mikono yopanda bwino

Zizindikiro zina

Zina, zizindikilo zowonjezereka za mgwirizano wa VATER ndi izi:

• kukula pang'onopang'ono
• kulephera kunenepa
• nkhope yosagwirizana (asymmetry)
• khutu kupindika
• zopindika m'mapapo
• mavuto ndi nyini kapena mbolo

Ndikofunika kuzindikira kuti mgwirizano wa VATER / VACTERL sukukhudza kuphunzira kapena kukulitsa nzeru.

Kodi amapezeka bwanji?

Chifukwa mgwirizano wa VATER ndi gulu lazikhalidwe, palibe mayeso amodzi omwe angazindikire. Madokotala nthawi zambiri amapanga matendawa kutengera zomwe ali nazo. Ana omwe ali ndi vutoli ali ndi zolakwika zosachepera VATER kapena VACTERL. Ndikofunika kuthana ndi ma syndromes ena amtundu ndi zikhalidwe zomwe zitha kugawana zinthu ndi gulu la VATER / VACTERL.

Kodi njira zamankhwala ndi ziti?

Chithandizo chimatengera mtundu wamatenda obadwa nawo omwe akukhudzidwa. Opaleshoni imatha kukonza zolakwika zambiri, kuphatikiza zovuta zotseguka kumatako, mafupa a msana, mtima, ndi impso. Nthawi zambiri njirazi zimachitika mwana akangobadwa.

Chifukwa mgwirizano wa VATER umakhudza machitidwe angapo amthupi, madokotala angapo amachiza, kuphatikiza:

• katswiri wamatenda (mavuto amtima)
• gastroenterologist (GI thirakiti)
• katswiri wa mafupa (mafupa)
• urologist (impso, chikhodzodzo, ndi mbali zina za mkodzo)

Ana omwe ali ndi mgwirizano wa VATER nthawi zambiri amafunikira kuwunikira ndi chithandizo chamtsogolo kuti ateteze mavuto amtsogolo. Akhozanso kufunikira thandizo kuchokera kwa akatswiri ngati othandizira thupi komanso othandizira pantchito.

Chiwonetsero

Maganizo amatengera mtundu wa zofooka zomwe munthu ali nazo, ndi momwe mavutowa amathandizidwira. Nthawi zambiri anthu omwe ali ndi mgwirizano wa VACTERL amakhala ndi zizindikilo m'miyoyo yawo yonse. Koma ndi chithandizo choyenera, amatha kukhala ndi moyo wathanzi.