Ukuthi yini i-Klippel-Trenaunay Syndrome? Izimpawu, Izimbangela, Nokwelapha

I-Klippel-Trenaunay syndrome iyisimo esingavamile esithinta indlela imithambo yegazi namalungu omzimba aphuhliswayo ngaphambi kokuzalwa. Ngokuvamile ihlanganisa izici ezintathu eziyinhloko: uphawu lokuzalwa olufana newayini elibomvu, imithambo yegazi emikhulu, nokukhula okukhulu kwamalunga, ngokuvamile kuthonya uhlangothi olulodwa lomzimba.

Lesi simo senzeka ngesikhathi sokuthuthukiswa kwasekuqaleni esibelekweni lapho imithambo yegazi ingakhiwa ngendlela efanele. Nakuba kungase kuzwakale kukhulu, abantu abaningi abane-Klippel-Trenaunay syndrome baphila impilo egcwele, esebenzayo ngokunakekelwa okulungile nokuphathwa.

Ukuthi yini i-Klippel-Trenaunay Syndrome?

I-Klippel-Trenaunay syndrome, ngokuvamile ifushaniswe yi-KTS, iyisimo lapho imithambo yegazi ithuthuka ngendlela ehlukile kunokujwayelekile. Lokhu kuthonya indlela igazi eligeleza ngayo ezingxenyeni ezithile zomzimba wakho, ikakhulukazi engalweni noma emilenzeni.

Lesi simo sibizwa ngamagama odktri ababili abachaze okokuqala ngo-1900. Yilokho odokotela abakubiza ngokuthi “i-vascular malformation syndrome,” okusho nje ukuthi imithambo yegazi yakhiwa ngendlela engajwayelekile ngesikhathi sokuthuthuka.

Abantu abaningi abane-KTS bazalwa nayo, futhi ayidluliselwa kubazali kubantwana ezimweni eziningi. Lesi simo sithinta cishe umuntu omunye ku-100,000, okwenza kube yinto engavamile kodwa ayisiyona into engaziwa.

Ziyini izimpawu ze-Klippel-Trenaunay Syndrome?

Izimpawu ze-KTS ngokuvamile zivele ekuzalweni noma ziqale ukubonakala ngesikhathi sobuntwana. Ngokuvamile uzobona ukuxubana kokushintsha kwesikhumba, izinkinga zemithambo yegazi, nokuhlukana ngobukhulu bomlenze.

Nazi izimpawu eziyinhloko ongaziqaphela:

• I-port-wine stain birthmark - uphawu oluqhele ngokwebala, olufana newayini elibomvu esikhumbeni
• Imithambo yegazi emikhulu noma eqhelekile ongayibona phansi kwesikhumba
• Uhlangothi olulodwa lomzimba olukhulu kunolunye (ngokuvamile umlenze noma ingalo)
• Ukukhula komlenze othonyeke, ikakhulukazi ngemuva kokuma noma umsebenzi
• Ubuhlungu noma ukucasuka emilenzeni emikhulu
• Isikumba esifudumele uma siqondwa endaweni ethonyeke

Abanye abantu nabo baye baba nezimpawu ezingavamile njengokuphuma kwegazi emithambweni yegazi emikhulu noma ukutheleleka kwesikhumba. Ubunzima bungashintsha kakhulu kusuka kumuntu komunye - abanye abantu banezimpawu ezilula kanti abanye badinga ukunakekelwa okukhulu.

Yini ebangela i-Klippel-Trenaunay Syndrome?

I-KTS iyenzeka ngenxa yokushintsha kokuthuthuka kwemithambo yegazi ezingxenyeni zokuqala zokukhulelwa. Ososayensi bakholelwa ukuthi kwenzeka ngenxa yokushintsha kwezinto zakwa-genetic okuyenzeka ngokungahleliwe ngesikhathi sokuthuthuka, hhayi ngenxa yanoma yini abazali abayenzile noma abangayenzanga.

Lesi simo sibonakala sihilela izinkinga ngezinto zakwa-genetic eziqondisa indlela imithambo yegazi ekhula futhi ixhumeka ngayo. Lezi zinguquko zithinta ukuthuthuka okujwayelekile kwemithambo yegazi, imithambo yegazi, kanye nemithambo ye-lymphatic ezingxenyeni ezithile zomzimba.

Ezimweni eziningi, i-KTS iyinto engavamile, okusho ukuthi kwenzeka ngengozi kunokudluliselwa kubazali. Noma kunjalo, kube khona izimo ezimbalwa kakhulu lapho kubonakala sengathi kuqhubeka emindenini, okuphakamisa ukuthi izici zakwa-genetic ngezinye izikhathi zingadlala indima.

Nini okumele ubona udokotela nge-Klippel-Trenaunay Syndrome?

Kufanele ubona udokotela uma uqaphela noma yikuphi ukuxubana kwezimpawu ezijwayelekile - uphawu lokuzalwa olufana newayini elibomvu, imithambo yegazi emikhulu ebonakalayo, nokuhlukana ngobukhulu bomlenze. Ukubheka okusheshayo kungasiza ukuvimbela izinkinga nokwenza impilo ibe ngcono.

Funani ukunakekelwa kwezokwelapha ngokushesha uma uhlangabezana nalokhu:

• Ubuhlungu obukhulu ngokushesha emilenzeni ethonyeke
• Izimpawu zokutheleleka njengobushushu, ubomvu, noma ukufudumeka
• Ukuphuma kwegazi emithambweni yegazi emikhulu
• Ukukhuluphala kokuphila noma ukusebenzisa umlenze othonyeke
• Ukwanda okusheshayo kokukhula

Ngisho noma izimpawu zibonakala zingenazinkinga, kufanele uhlolwe udokotela oqeqeshwe ngezimo zemithambo yegazi. Bangakusiza ukuqonda ukuthi yini okufanele uyilandele futhi bakhe uhlelo lokulawula isimo sakho esithile.

Ziyini izici ezingozi ze-Klippel-Trenaunay Syndrome?

Njengoba i-KTS ngokuvamile iyenzeka ngokungahleliwe ngesikhathi sokuthuthuka, azikho izici eziningi ezingozi ongaziqondisa. Lesi simo sibonakala senzeka ngengozi ezimweni eziningi.

Noma kunjalo, abacwaningi baye baqaphela izindlela ezimbalwa okufanele bazazi:

• Ezimo eziningi zenzeka ngokungavamile ngaphandle komlando womndeni
• Ithonya amadoda nabesifazane ngokulinganayo
• Iminyaka yabazali ayibonakali ithonya ingozi
• Izici zemvelo ngesikhathi sokukhulelwa azibonakali zidlala indima

Ezimeni ezimbalwa lapho i-KTS iqhubeka khona emindenini, ukuba nomzali onalesi simo kungase kukhulise ingozi kancane. Kodwa lokhu akukho njalo, futhi abantu abaningi abane-KTS abanamalungu omndeni athintekile.

Ziyini izinkinga ezingase zenzeke nge-Klippel-Trenaunay Syndrome?

Nakuba abantu abaningi abane-KTS baphila impilo evamile, kubalulekile ukwazi ngezinkinga ezingase zenzeke ukuze ukwazi ukubheka izimpawu zokuqaphela futhi uthole usizo uma kudingeka.

Izinkinga ezivamile zihlanganisa:

• Ama-blood clots emithambweni yegazi emikhulu (thrombosis)
• Ukutheleleka kwesikhumba ngenxa yokungabi khona kokugeleza kwegazi
• Ukuphuma kwegazi emithambweni yegazi ebuthakathaka, emikhulu
• Ubuhlungu obuhlala njalo ngenxa yokukhula komlenze
• Ukukhuluphala kokuthola izicathulo noma izingubo ezifanele
• Izinselele zomzwelo ngenxa yokuhlukana okubonakalayo

Izinkinga ezingavamile kodwa ezingathi sína zingabandakanya i-pulmonary embolism (i-blood clot emaphashini) noma ukuphuma kwegazi okukhulu. Abanye abantu nabo bathuthukisa i-arthritis ezinhlanganweni ezithintekile ngokukhula komlenze.

Izindaba ezinhle ukuthi ngokunakekelwa kwezokwelapha okulungile nokubhekwa, izinkinga eziningi zingavikeleka noma ziphathwe kahle. Iqembu lakho lokunakekelwa kwezempilo lizokusebenza nawe ukuze kunciphisa ingozi.

I-Klippel-Trenaunay Syndrome ihlonishwa kanjani?

Odokotela ngokuvamile bahlonishwa i-KTS ngokubuka izimpawu zakho nomlando wakho wezokwelapha. Ukuxubana kwe-port-wine stain, imithambo yegazi emikhulu, nokukhula komlenze ngokuvamile kwenza ukuxilongwa kube sobala.

Udokotela wakho angase asebenzise ukuhlolwa okuningi ukuze athole isithombe esiphelele:

• Ukuhlolwa komzimba ukuhlola ama-birthmarks, imithambo yegazi, nokulinganisa komlenze
• I-ultrasound ukubuka ukugeleza kwegazi nokwakhiwa kwemithambo yegazi
• I-MRI noma i-CT scans ukubona izithombe eziningiliziwe zemithambo yegazi namalungu
• I-X-rays ukuhlola umehluko wokukhula kwamalunga
• Ukuhlolwa kwegazi ukuvimbela izifo zokugqwala

Ngezinye izikhathi ukuhlolwa kwe-genetic kucabangelwa, ikakhulukazi uma kukhona umlando womndeni wezimo ezifanayo. Noma kunjalo, ukushintsha okuthile kwe-genetic akutholakali kuzo zonke izimo ze-KTS.

Yini ukwelapha kwe-Klippel-Trenaunay Syndrome?

Ukwelapha i-KTS kugxile ekulawuleni izimpawu nokunciphisa izinkinga kunokwelapha lesi simo. Iqembu lakho lokunakekelwa lizokwenza uhlelo olufanele olusekelwe ekutheni iziphi izimpawu ezikuthinta kakhulu.

Izindlela zokwelapha ezivamile zihlanganisa:

• Izingubo zokucindezela ukuze kunciphisa ukukhula nokwenza ukugeleza kwegazi kube ngcono
• I-physical therapy ukuze kulondolozwe amandla nokukwazi ukuhamba
• I-Laser therapy ye-port-wine stains
• I-Sclerotherapy ukuvala imithambo yegazi enezinkinga
• Ukuhlinzwa ngenxa yokwehluka okukhulu kobubanzi bomlenze
• I-blood thinners uma ungozini yama-clots

Iqembu lakho lokwelapha lingase lihlanganise ochwepheshe bezinto zakwa-vascular, odokotela besikhumba, ochwepheshe bokuhlinzwa kwamalunga, kanye nochwepheshe be-physical therapy. Bazosebenza ndawonye ukuze baqonde izidingo zakho futhi bakusize ukuba ube nekhwalithi yempilo engcono kakhulu.

Ukuthi unganakekela kanjani ekhaya ngesikhathi se-Klippel-Trenaunay Syndrome?

Ukulawula i-KTS ekhaya kuhilela imikhuba yansuku zonke esekela ukugeleza kwegazi okuhle nokunciphisa izinkinga. Izinyathelo ezincane, ezihlala njalo zingenza umehluko omkhulu endleleni ozizwa ngayo.

Nanku ongakwenza ekhaya:

• Gqoka izokisi noma izingubo zokucindezela njengoba kunconywe
• Phakamisa umlenze othonyeke uma ukhululekile
• Hlala usebenza ngemisebenzi engathinti umzimba kakhulu njengokubhukuda
• Gcina isikhumba sakho sihlanzekile futhi sinamanzi
• Gwema ukuhlala noma ukuhlala endaweni eyodwa isikhathi eside
• Vikela isikhumba sakho ekuthungeni nasekulimala

Qaphela izinguquko ezimpawu zakho futhi ugcine ilogi elula uma kuwusizo. Lolu lwazi lungaba lubalulekile ngesikhathi sokuvakashela udokotela futhi lukusiza ukubamba izinkinga ezingase zenzeke ngokushesha.

Ukuthi kufanele ulungiselele kanjani i-aphoyintimenti yakho nodokotela?

Ukuba ulungile nge-aphoyintimenti yakho kukusiza ukuthola okuningi ngesikhathi sakho nodokotela. Ukulungiselela kancane kungase kuholele ekunakekelweni okungcono nezimpendulo ezicacile zemibuzo yakho.

Ngaphambi kokuya kwakho, buthana lolu lwazi:

• Uhlu lwezimpawu zakho zamanje nokuthi zaqala nini
• Izithombe ezibonisa izinguquko esimweni sakho ngokuhamba kwesikhathi
• Imithi namasuplimenti amanje
• Umlando womndeni wezimo ezifanayo
• Imibuzo mayelana nemisebenzi yansuku zonke nendlela yokuphila

Unganqikazi ukubuza noma yini ekukhathazayo, kusuka ezindleleni zokwelapha kuya kubukhazikhazi besikhathi eside. Iqembu lakho lokunakekelwa kwezempilo lifuna ukukusiza ukuqonda isimo sakho futhi uzizwe ukhululekile ngokulilawula.

Yini eyinhloko mayelana ne-Klippel-Trenaunay Syndrome?

I-Klippel-Trenaunay syndrome iyisimo esilawuleka esithinta umuntu ngamunye ngokuhlukile. Nakuba idinga ukunakekelwa okuqhubekayo nokunakekelwa, abantu abaningi abane-KTS baphila impilo egcwele, esebenzayo ngokusekelwa okulungile nokwelashwa.

Into ebaluleke kakhulu ukusebenza nabaphakeli bezempilo abaqeqeshwe abaqonda izimo zemithambo yegazi. Bangakusiza ukuzulazula izindlela zokwelapha, ukuvimbela izinkinga, nokubhekana nanoma yiziphi izinkinga njengoba zivela.

Khumbula ukuthi ukuba ne-KTS akuchazi imingcele yakho. Ngokunakekelwa okulungile, abantu abaningi bathola izindlela zokuzilungisa nokukhula ngenkathi bephethe izimpawu zabo kahle.

Imibuzo Evamile Mayelana ne-Klippel-Trenaunay Syndrome

Q1: Ingabe i-Klippel-Trenaunay syndrome izuzwa?

Ezimeni eziningi, i-KTS ayizuzwanga futhi iyenzeka ngokungahleliwe ngesikhathi sokuthuthuka. Noma kunjalo, kunezimo ezimbalwa kakhulu lapho kubonakala sengathi kuqhubeka emindenini. Uma une-KTS futhi uhlela umndeni, ukwelulekwa kwe-genetic kungakunikeza ulwazi olufanele mayelana nengozi.

Q2: Ingabe izimpawu zami ziyoba zimbi ngokuhamba kwesikhathi?

Izimpawu ze-KTS zingashintsha njengoba ukhula futhi ugoduke, kodwa lokhu kuyahluka kakhulu kusuka kumuntu komunye. Abanye abantu baqaphela izinguquko ezihamba kancane kanti abanye bahlala beqinile iminyaka eminingi. Ukubhekwa okuqhubekayo neqembu lakho lokunakekelwa kwezempilo kusiza ukulandelela noma yiziphi izinguquko nokwenza ukwelashwa kube ngcono njengoba kudingeka.

Q3: Ngingasebenzisa futhi ngidlale imidlalo nge-KTS?

Abantu abaningi abane-KTS bangasebenzisa futhi bahlanganyele emidlalweni ngezinye izindlela. Imisebenzi engathinti umzimba kakhulu njengokubhukuda ngokuvamile kunconywa, kanti imidlalo yokuxhumana ingadinga ukucabangela okuningi. Udokotela wakho angakusiza ukuthola izindlela ezifanele zokuhlala usebenza ngokusekelwe ezimpawu zakho ezithile.

Q4: Ingabe kukhona ukwelapha i-Klippel-Trenaunay syndrome?

Okwamanje ayikho impilo ye-KTS, kodwa ukwelashwa kungalawula izimpawu nokunciphisa izinkinga. Ucwaningo luyaqhubeka ekuqondeni kangcono lesi simo nokwenza izindlela ezintsha zokwelapha. Ukugxila kukusizeni ukuba uphile ngokunethezeka ngalesi simo.

Q5: I-KTS iyokuthinta kanjani impilo yami yansuku zonke?

Uthinta lwe-KTS empilweni yansuku zonke luyahluka kakhulu kuye ngezimpawu zakho ezithile nobunzima bazo. Abantu abaningi benza izinguquko ezilula njengokugqoka izingubo zokucindezela noma ukukhetha izicathulo ezisekelayo futhi baqhubeka nemisebenzi evamile. Iqembu lakho lokunakekelwa kwezempilo lingakusiza ukuthola amasu asebenza ngendlela yakho yokuphila.