Iyini i-VATER Syndrome?

-Delile

• Ukubuka konke
• Kubangelwa yini?
• Ziyini izimpawu?
• Amaphutha we-Vertebral
• Ukukhubazeka kwesidoda
• Ukukhubazeka kwenhliziyo
• I-fistula ye-Tracheoesophageal
• Ukukhubazeka kwezinso
• Ukukhubazeka kwezitho zomzimba
• Ezinye izimpawu
• Kutholakala kanjani?
• Yiziphi izinketho zokwelashwa?
• Umbono

Ukubuka konke

I-VATER syndrome, evame ukubizwa ngokuthi yinhlangano ye-VATER, iyiqembu lokukhubazeka kokuzalwa okuvame ukwenzeka ndawonye. I-VATER isichazamazwi.Uhlamvu ngalunye lumele ingxenye yomzimba ethintekile:

• amathambo omgogodla (amathambo omgogodla)
• ingquza
• i-tracheoesophageal (i-trachea ne-esophagus)
• izinso (izinso)

Usoseshini ubizwa nge-VACTERL uma inhliziyo (inhliziyo) nezitho zomzimba nazo zithintekile. Njengoba lokhu kuvame ukwenzeka, i-VACTERL imvamisa iyigama elinembe kakhulu.

Ukuze kutholakale ukuthi unenhlangano ye-VATER noma ye-VACTERL, ingane kufanele ibe nezinkinga zokuzalwa okungenani kulezi zindawo ezintathu.

Inhlangano yeVATER / VACTERL ayivamile. Kulinganiselwa ukuthi ingane eyodwa kwezingu-10 000 kuya ku-40 000 izalwa naleli qembu lezimo.

Kubangelwa yini?

Odokotela abazi kahle ukuthi yini ebanga ukuhlangana kweVATER. Bakholelwa ukuthi ukukhubazeka kwenzeka ekuqaleni kokukhulelwa.

Inhlanganisela yezakhi zofuzo nezici zemvelo kungabandakanyeka. Asikho isakhi sofuzo esisodwa esitholakele, kepha abacwaningi bathole ukungajwayelekile kwe-chromosomal nokushintsha kwezakhi zofuzo okuhlobene nalesi simo. Kwesinye isikhathi kuzothinteka abantu abangaphezu koyedwa emndenini ofanayo.

Ziyini izimpawu?

Izimpawu zincike ekutheni ingane inamaphutha anjani.

Amaphutha we-Vertebral

Kuze kube ngamaphesenti angama-80 abantu abanenhlangano ye-VATER abanokukhubazeka emathanjeni omgogodla wabo (i-vertebrae). Lezi zinkinga zingafaka:

• ulahlekile amathambo emgogodleni
• amathambo engeziwe emgogodleni
• amathambo abunjwe ngokungajwayelekile
• amathambo ahlanganiswe ndawonye
• umgogodla ogobile (scoliosis)
• izimbambo ezingeziwe

Ukukhubazeka kwesidoda

Phakathi kwabantu abangamaphesenti angama-60 kuya kwangama-90 abanenhlangano yeVATER banenkinga ngendunu yabo, efana nale:

• isembozo esincane phezu kwendunu esivimba ukuvuleka
• ayikho indlela yokungena phakathi kwezansi lamathumbu amakhulu (i-rectum) ne-anus, ngakho-ke indle ayinakudlula iphume emathunjini iphume emzimbeni

Izinkinga nge-anus zingadala izimpawu ezifana nalezi:

• isisu esivuvukile
• ukuhlanza
• akukho ukunyakaza kwamathumbu, noma ukunyakaza kwamathumbu okumbalwa kakhulu

Ukukhubazeka kwenhliziyo

I- “C” ku-VACTERL imele “inhliziyo.” Izinkinga zenhliziyo zithinta amaphesenti angama-40 kuye kwangama-80 abantu abanalesi simo. Lokhu kungafaka:

• Isici se-septal se-Ventricular (VSD). Lona umgodi odongeni ohlukanisa amakamelo aphansi angakwesokudla nangakwesobunxele enhliziyo (ama-ventricles).
• Isici se-atrial septal. Yilapho umgodi odongeni uhlukanisa amakamelo amabili aphezulu enhliziyo (i-atrium).
• I-Tetralogy of Fallot. Lokhu kuyinhlanganisela yezinkinga zenhliziyo ezine: i-VSD, i-aortic valve eyandisiwe (i-aorta engaphezulu), ukuncipha kwe-valve yamaphaphu (i-pulmonary stenosis), nokujiya kwe-ventricle efanele (i-ventricular hypertrophy yangakwesokudla).
• Isifo senhliziyo esine-hypoplastic. Yilapho uhlangothi lwenxele lwenhliziyo lungakheki kahle, luvimbela igazi ukuba lingagobhozi lingene enhliziyweni.
• I-Patent ductus arteriosus (i-PDA). I-PDA yenzeka lapho kunokuvuleka okungavamile komunye wemithambo yegazi yenhliziyo evimbela igazi ukuthi liye emaphashini liyothatha umoya-mpilo.
• Ukushintshwa kwemithambo emikhulu. Imithambo yegazi emibili ephuma enhliziyweni ibuyele emuva (iguquliwe).

Izimpawu zezinkinga zenhliziyo zifaka:

• inkinga yokuphefumula
• ukuphelelwa umoya
• umbala oluhlaza okwesibhakabhaka esikhunjeni
• ukukhathala
• isigqi senhliziyo esingajwayelekile
• ukushaya kwenhliziyo okusheshayo
• ukububula kwenhliziyo (umsindo oshisayo)
• ukungadli kahle
• akukho ukuzuza kwesisindo

I-fistula ye-Tracheoesophageal

I-fistula ukuxhumana okungavamile phakathi kwe-trachea (i-windpipe) ne-esophagus (ishubhu elithwala ukudla lisuke emlonyeni liye esiswini). Lezi zakhiwo ezimbili ngokuvamile azixhunyiwe nakancane. Iphazamisa ukudla okudlula kusuka emphinjeni kuya esiswini, kugudlulela okunye ukudla emaphashini.

Izimpawu zifaka:

• ukuphefumulela ukudla emaphashini
• ukukhwehlela noma ukuklinya ngenkathi udla
• ukuhlanza
• umbala oluhlaza okwesibhakabhaka esikhunjeni
• inkinga yokuphefumula
• isisu esivuvukile
• ukuzuza kwesisindo esibi

Ukukhubazeka kwezinso

Cishe amaphesenti angama-50 abantu abane-VATER / VACTERL banokukhubazeka kwezinso. Lokhu kungafaka:

• izinso ezakhiwe kabi
• izinso ezisendaweni engafanele
• ukuvinjelwa komchamo ngaphandle kwezinso
• ukulondolozwa komchamo esinyeni ungene ezinso

Ukukhubazeka kwezinso kungadala ukutheleleka kaningi komchamo. Abafana futhi bangaba nenkinga lapho kuvulwa khona ipipi labo ngezansi, esikhundleni sethiphu (hypospadias).

Ukukhubazeka kwezitho zomzimba

Kufika kumaphesenti angama-70 ezingane ezine-VACTERL ezinokukhubazeka kwezitho. Lokhu kungafaka:

• izithupha ezingekho noma ezingakhuli kahle
• iminwe eyengeziwe noma izinzwane (polydactyly)
• iminwe noma izinzwane ezine-webbed (syndactyly)
• izingalo ezingakhuli kahle

Ezinye izimpawu

Okunye, ezinye izimpawu ezijwayelekile zenhlangano ye-VATER zifaka:

• ukukhula kancane
• ukwehluleka ukukhuluphala
• izici zobuso ezingalingani (i-asymmetry)
• ukukhubazeka kwezindlebe
• ukukhubazeka kwamaphaphu
• izinkinga zesitho sangasese sowesilisa noma sowesilisa

Kubalulekile ukuqaphela ukuthi inhlangano ye-VATER / VACTERL ayithinti ukufunda noma ukukhula kwengqondo.

Kutholakala kanjani?

Ngoba inhlangano ye-VATER iyiqoqo lezimo, akukho sivivinyo esisodwa esingayihlola. Odokotela bavame ukwenza ukuxilongwa kususelwe kwizimpawu nezimpawu zomtholampilo. Izingane ezinalesi simo okungenani zinamaphutha amathathu e-VATER noma e-VACTERL. Kubalulekile ukukhipha amanye ama-syndromes ezakhi zofuzo nemibandela engabelana ngezici nenhlangano ye-VATER / VACTERL.

Yiziphi izinketho zokwelashwa?

Ukwelashwa kusekelwe kuziphi izinhlobo zokukhubazeka kokuzalwa okuhilelekile. Ukuhlinzwa kungalungisa ukukhubazeka okuningi, kufaka phakathi izinkinga ngokuvulwa kwendunu, amathambo omgogodla, inhliziyo, nezinso. Imvamisa lezi zinqubo zenziwa ngemuva nje kokuzalwa kwengane.

Ngoba inhlangano ye-VATER ifaka amasistimu amaningi omzimba, odokotela abambalwa abahlukene bayayelapha, kufaka phakathi:

• isazi senhliziyo (izinkinga zenhliziyo)
• i-gastroenterologist (ipheshana le-GI)
• uchwepheshe wamathambo (amathambo)
• i-urologist (izinso, isinye, nezinye izingxenye zohlelo lomchamo)

Izingane ezinenhlangano yeVATER zizodinga ukuqashwa nokwelashwa impilo yazo yonke ukuvimbela izinkinga ezizayo. Bangadinga nosizo kochwepheshe abanjengochwepheshe bomzimba kanye nodokotela osebenza emsebenzini.

Umbono

Umbono uncike ekutheni yiziphi izinhlobo zokukhubazeka umuntu anazo, nokuthi lezi zinkinga ziphathwa kanjani. Imvamisa abantu abanenhlangano ye-VACTERL bazoba nezimpawu kukho konke ukuphila kwabo. Kepha ngokwelashwa okufanele, bangaphila impilo enempilo.