Ese uruhu rw'umwana wawe ruratukura kandi rurabyimbye? Reka twige ku ndwara ya Klippel-Trenaunay Syndrome (KTS)!

Ese wigeze ubona ko hari abana bavuka bafite ikimenyetso kinini gitukura ku ruhande rumwe rw'umubiri wabo, cyane cyane ku kuboko cyangwa ku kuguru, kandi ukuboko/ukuguru kukaba kunini gato kuruta urundi ruhande? Cyangwa rimwe na rimwe ushobora kubona imitsi kuri urwo ruhande? Ni ibisanzwe kumva ufite ubwoba gato iyo ubonye ikintu nk'iki. Uyu munsi tugiye kuvuga ku ndwara igaragaza ibimenyetso bisa, bidasanzwe, bivuze ko atari buri wese uyirwara, kandi akaba yaravukanye. Ibi byitwa Klippel-Trenaunay Syndrome (KTS) . Ntugahangayike, tuzabivugaho mu buryo bworoshye, mu buryo ushobora gusobanukirwa.

Indwara ya Klippel-Trenaunay (KTS) ni iki? Mu magambo make...

Tekereza, KTS ni indwara idakunze kugaragara ijyana n'umwana wacu iyo aje muri iyi si (ni ukuvuga indwara yo kuvukana ). Ibi bitera impinduka nto mu buryo ingingo zoroshye z'umwana, amagufwa ye, n'imitsi ye y'amaraso ikura. Kimwe mu bimenyetso by'ingenzi by'ibi ni ukugaragara kw'ikimenyetso cy'umutuku n'umuhengeri, akenshi ku maboko cyangwa ku maguru, cyitwa 'port-wine stain' . Akenshi, abantu bafite iyi KTS bagira ibibazo bimwe na bimwe mu bijyanye n'imitsi yabo. Iyi sisitemu y'imitsi ni sisitemu y'ingenzi ifasha mu gutuma amazi yo mu mubiri wacu aguma mu rugero rwiza.

Kugeza ubu nta muti uhari wa KTS. Ariko, ntugatinye. Hari uburyo bwinshi bwiza bwo kuvura ibimenyetso . Iyo iyi ndwara imenyekanye kandi ikavurwa hakiri kare, nko nyuma yo kuvuka k'umwana, ingorane zishobora guterwa na KTS zishobora kugabanuka cyane.

Abaganga bamwe na bamwe bita KTS condition CLVM . Iyi ni inyuguti ya mbere y'amagambo ane y'Icyongereza.

• C ni iy'imitsi mito y'amaraso ihuza imitsi n'imitsi yacu.
• L isobanura sisitemu y'imitsi - Iki ni kimwe mu bigize ubudahangarwa bwacu bw'umubiri. Itwara amazi yitwa imitsi mu mubiri wose.
• V isobanura imitsi - iyi ni imitsi ijyana amaraso mu mutima wacu.
• Inyuguti M isobanura ko hari igice cy'umubiri kidakura neza, cyangwa ko hari ubumuga .

KTS yitiriwe abaganga babiri b'Abafaransa, Maurice Klippel na Paul Trenaunay, bavumbuye iyi ndwara mu 1900. Impuguke zivuga ko hafi umuntu 1 ku bantu 100.000 ku isi arwaye iyi ndwara. Ishobora kwibasira umuntu uwo ari we wese, hatitawe ku bwoko cyangwa igitsina.

Ni ibihe bimenyetso bya KTS? Ubimenya ute?

Ibimenyetso bya Klippel-Trenaunay Syndrome (KTS) bigira ingaruka cyane cyane ku mitsi, imitsi yo mu bwonko, ingirangingo zoroshye, amagufwa, n'imitsi itwara amaraso mu mubiri wacu. Reka turebe uko bigenda:

1. Kurema nabi kw'imitsi yo mu kanwa (CM):

Aha niho hagaragara 'ibara rya port-wine' twavuze mbere. Ibi biterwa no kubyimba kw'imitsi iri munsi y'uruhu rwacu. Ibi bimenyetso by'amavuko bishobora kuba kimwe mu bimenyetso bya mbere byerekana ko ushobora kuba ufite KTS. Ibi bimenyetso bishobora kuza mu mabara atandukanye, kuva ku ibara ry'umutuku woroshye kugeza ku ibara ry'umutuku wijimye (umutuku wa divayi). Uko ugenda usaza, ibi bimenyetso bishobora guhinduka nk'ibisebe, byoroshye cyangwa byijimye.

2. Kumererwa nabi kw'imitsi (VM):

Hafi ya buri wese urwaye KTS afite ubumuga bw'imitsi. Ibi bishobora kugira ingaruka ku mitsi iri hejuru gato y'uruhu, bigatera imitsi ishyushye mu maguru, cyane cyane mu kibuno no mu matako. Bishobora kandi kugira ingaruka ku mitsi miremire inyura mu mubiri. Ibi bishobora gutuma amaraso agwa mu mitsi miremire (Deep Vein Thrombosis - DVT) . DVT ni indwara iteje akaga.

3. Imitsi yoroshye n'amagufwa bikura cyane:

Iyi ni indwara aho, kuva mu bwana, ukuboko kumwe cyangwa ukuguru kumwe bikunze gukura kuruta ukundi. Akenshi, ibi bigira ingaruka ku kuboko kumwe cyangwa ukuguru kumwe gusa, kandi akenshi bikunze kugira ingaruka ku kuguru kumwe gusa. Hari igihe ukuguru kumwe gushobora kuba kurenza ukundi. Ibi bishobora gutuma umuntu atabasha kuringaniza imiterere ye, kugenda bigoranye, ndetse no kugira aho anyura hato.

4. Kutagira imiterere myiza y'imitsi yo mu maraso (LM):

Bamwe mu bantu barwaye KTS bashobora kuba bafite imiyoboro y’amaraso ya lymph mu buryo bw’imitsi yabo, cyangwa imiyoboro y’amaraso ya lymph isanzwe ishobora kuba ifite imiterere idasanzwe. Kubera ko iyi miyoboro y’amaraso ya lymph idakora neza, amazi ya lymphatic ashobora kuva , bigatera kubyimba amaguru, cyane cyane ibirenge, cyangwa ibibazo mu kibuno, mu ruhago, cyangwa mu mara yo hasi.

Ni iki gitera KTS? Kuki ibi bibaho?

Akenshi, KTS iterwa n'ihindagurika rya gene yitwa PIK3CA . Iyi mihindagurikire y'uturemangingo ibaho rimwe na rimwe, bivuze ko nta mpamvu izwi ihari. Ntiragwa n'ababyeyi . Ibi bivuze ko nubwo nta mubyeyi n'umwe wagiraga KTS, umwana ashobora kurwara KTS kubera iyi mihindagurikire y'uturemangingo.

Hari abantu bafite KTS badafite iyi mpinduka ya PIK3CA. Kubwibyo, abashakashatsi bizera ko KTS ishobora guterwa n'impinduka mu zindi ngirabuzimafatizo nyinshi. Ubushakashatsi kuri ibi buracyakomeje.

Ni izihe ngorane zishobora guterwa na KTS?

KTS ishobora gutera ingorane zitandukanye. Niyo mpamvu ubuvuzi bwihuse ari ingenzi. Reka turebere hamwe izo ngorane:

• Amaraso agwa: Ashobora kubaho, cyane cyane mu mitsi miremire (DVT).
• Selulite: Iyi ni indwara ya bagiteri ibera munsi y'uruhu.
• Kubyimba no gukurura amazi (Lymphedema): Biterwa n'imikorere mibi y'urusobe rw'amaraso.
• Kuva amaraso imbere: Kuva amaraso bishobora kubaho mu nzira y'igifu (GI), mu ruhago, cyangwa mu buryo bw'imyororokere bw'abagore.
• Kuziba kw'imitsi y'ibihaha: Iyi ni indwara ishobora gushyira ubuzima mu kaga aho amaraso agwa mu mitsi yo mu bihaha agacika burundu.

Ni gake cyane abantu barwaye KTS bashobora kugira ibibazo bavukana mu biganza cyangwa mu birenge. Urugero:

• Kugira intoki z'inyongera (Polydactyly)
• Intoki zafatanye (Syndactyly)

Ese KTS itera ububabare?

Yego, KTS ishobora kubabaza.

• Imitsi y'imitsi ishobora gutera uburyaryate cyangwa ububabare.
• Ibibazo byo gutembera kw'amaraso bishobora gutera kubyimba no kubabara, cyane cyane mu maguru yo hasi.
• Gukura cyane kw'urugingo, nk'ukuguru, bishobora gutera kumva uremereye kandi ubabara muri urwo rugingo.

Ni gute abaganga basuzuma indwara ya KTS? (Isuzuma)

Abaganga babanza gusuzuma KTS bashingiye ku bimenyetso by'umubiri. KTS ikekwaho niba hari ibibazo nibura mu bice bibiri by'ingenzi twavuze mbere - imitsi yo mu bwonko, imitsi, cyangwa imikurire y'ingingo. Kubera ko ibimenyetso byinshi bya KTS bigaragara igihe umwana avuka, birashoboka ko wasuzuma KTS mbere yuko umwana wawe ava mu bitaro.

Ni ibihe bipimo bikoreshwa mu gusuzuma KTS?

Bitewe n'ibimenyetso, muganga ashobora gukora ibizamini nk'ibi kugira ngo yemeze neza ikibazo kandi amenye ingano yacyo:

• Gupima CT cyangwa MRI: Reba imiterere y'uturemangingo tworoshye n'amagufwa.
• Angiogramu ya Magnetic Resonance (MR angiogramu): Iki ni ikizamini cya MRI cyihariye gishobora kureba neza imiterere y'imitsi n'amaraso.
• Doppler ultrasound: Ibi bifasha kubona amaraso atembera mu mitsi no mu mitsi.

Ni ubuhe buryo bwo kuvura KTS?

Uburyo bwo kuvura indwara ya Klippel-Trenaunay Syndrome (KTS) butandukana bitewe n'ibimenyetso buri muntu afite.. Si abantu bose bahabwa ubuvuzi bumwe. Intego nyamukuru y'ubuvuzi ni ukugenzura ibimenyetso no kugabanya ibyago byo kugira ingorane. Reka turebe uburyo bw'ingenzi bwo kuvura:

• Imiti igabanya amaraso / Imiti igabanya amaraso: Urugero, imiti nka Heparin . Iyi igabanya ibyago byo kubyimba kw'amaraso mu maguru (DVT) no kuzura kw'amaraso mu bihaha.
• Sirolimus: Uyu muti ukunze gukoreshwa mu kubuza umubiri kwanga urugingo rwatewe. Ariko kandi, byagaragaye ko ubuza imitsi kumererwa nabi kurushaho.
• Amasogisi yo gukanda: Aya ni ubwoko bwihariye bw'amasogisi afasha amaraso kuva ku maguru kugera ku mutima. Ibi bishobora kugabanya kubyimba, ububabare, ndetse n'ibyago byo kubyimba kw'amaraso.
• Gukoresha ubushyuhe bwo mu mubiri: Ibi bikubiyemo gukoresha ingufu zigaragara mu gufunga imiyoboro y'amaraso ifite ikibazo imbere. Ibi bikorwa imitsi ikiri aho. Ibi bituma igihe cyo gukira kigabanuka kandi ububabare bugagabanuka.
• Uburyo bwo kuvura hakoreshejwe laser: Ingufu zikomeye kandi zihambaye zishobora gukoreshwa mu gusenya cyangwa gukuraho ingingo zidakenewe. Ubu buryo bwo kuvura hakoreshejwe laser bukoreshwa mu gukabya ibara ry'ikimenyetso cy'amavuko cya 'port-wine'.
• Ubuvuzi bwa sclerotherapy: Muri ubu buryo, umuti wihariye uterwa mu mitsi ifite ikibazo, mu mitsi ifunga imitsi, cyangwa mu mitsi ya lymphatic. Hanyuma imitsi irafunga. Ubu ni uburyo bwiza cyane bwo kuvura imitsi ifunga imitsi.
• Guterura inkweto: Niba amaguru yawe adahwanye, ushobora gushyiramo inkweto nyinshi kugira ngo ukosore ibi. Ibi bishobora kandi gufasha kwirinda indwara ya scoliosis .
• Kubaga: Kubaga bishobora gukorwa kugira ngo hakosorwe ibibazo by'imitsi no kuringaniza uburebure bw'amaguru. Cyangwa, kubaga bishobora gukorwa kugira ngo hakureho ibinure cyangwa ingingo zirenze urugero kugira ngo hagabanywe ingano y'ukuboko cyangwa ukuguru gukabije. Ni gake cyane, iyo ikirenge kinini kidasanzwe kidakora neza bigatuma bigorana kwambara inkweto cyangwa kugenda, ikirenge gishobora kubagwa.

Ese KTS ishobora kwirindwa?

Ikibabaje ni uko kubera ko KTS ibaho ku buryo butunguranye, nta buryo bwo kuyirinda . Ariko, nk'uko byavuzwe mbere, ubuvuzi bukwiye bushobora gufasha abantu barwaye KTS kubaho ubuzima bwiza.

Ni ubuhe buryo bw'ejo hazaza umuntu urwaye KTS yakwitega?

Nubwo nta muti uhari wa KTS, ibimenyetso birashoboka kubikemura. Akenshi, abantu bafite iyi ndwara babaho ubuzima busanzwe .

Ariko, uko indwara ya KTS ihagaze bishobora gutandukana bitewe n'umuntu ku giti cye. Biterwa n'ubukana bw'indwara z'imitsi yawe. Ibi bishobora kwiyongera uko igihe kigenda gihita. Kubwibyo, niba urwaye indwara yo kuva amaraso mu nda, indwara yo mu mitsi itemba cyane, cyangwa indwara yo mu bihaha , ugomba kwihutira gushaka abaganga . Kuziba kw'amaraso cyangwa kuva amaraso menshi bishobora kwica.

Guhabwa inama n'ubuvuzi buri gihe bishobora kugabanya cyane ibyago byo kurwara.

Niba mfite indwara ya KTS, niyitaho nte? / Ni gute nitaye ku mwana wanjye?

Niba wowe cyangwa umwana wawe afite indwara ya KTS, menya ibi bintu:

• Wite ku ruhu rwawe neza: Ni ngombwa kwirinda kwandura indwara.
• Uburyo bwo kuboneza urubyaro bugizwe n'imisemburo irimo estrogen muri rusange ntibusabwa: Bushobora kongera ibyago byo kuvura amaraso. Vugana na muganga wawe kuri ibi.
• Fata imiti yo kwirinda kubyimba kw'amaraso mu gihe utwite: Muganga wawe azakugira inama kuri ibi.
• Fata imiti igabanya amaraso mbere yo kubagwa: Gabanya ibyago byo kubyimba kw'amaraso.

Ni ryari ngomba kubonana na muganga?

Jyana na muganga wawe buri gihe mu buzima bwawe bwose kugira ngo agusuzumishe . Ibi bizatuma muganga wawe akurikirana indwara yawe kandi agufashe kuvura ibibazo bishya bishobora kuvuka. Gusuzumisha buri gihe bizafasha muganga wawe kubona uburyo ubuvuzi bwawe bukora neza no guhindura gahunda yawe yo kuvura nibiba ngombwa.

Ni ryari nkwiye kujya mu Ishami ry’Ubuvuzi bw’Ingoboka (ETU) ?

Niba utekereza ko ufite amaraso apfutse (urugero: DVT, ibimenyetso bya Pulmonary Embolism) cyangwa niba uva amaraso menshi (kuva amaraso menshi), ugomba guhita ujya kwa muganga wita ku barwayi b’ingoboka.

Ni ibihe bibazo by'ingenzi ugomba kubaza muganga?

Niba wowe cyangwa umwana wawe afite indwara ya KTS, ushobora kubaza muganga wawe ibibazo nk'ibi:

• Ni ubuhe buryo bwo kuvura bubereye njye/umwana wanjye?
• Ni kangahe nkwiye kuza kwisuzumisha?
• Ukurikije uko meze ubu, ni iki wavuga ku hazaza hawe (ibiteganyijwe/ibiteganyijwe)?

Ese KTS irashyira ubuzima mu kaga?

KTS ubwayo ntabwo igira ingaruka ku buryo butaziguye ku gihe umuntu azamara. Ariko, zimwe mu ngorane zishobora guterwa na KTS, nko kuva amaraso imbere mu mubiri cyangwa gutemba kw'ibihaha, zishobora gushyira ubuzima mu kaga . Kuvura ibi bintu bishobora kugabanya ibyago byo kurwara.

Ese KTS ni ubumuga?

Bishobora kubaho. Niba udashobora gukora bitewe n'ingorane za KTS, nko kuvura imitsi yo mu bwonko (DVT) cyangwa kuvura ibihaha, ushobora kuba wemerewe guhabwa amafaranga y'ubumuga. Ushobora kandi guhabwa amafaranga nk'agapapuro ko guhagarara ku bafite ubumuga bitewe n'ibibazo nko kugenda nabi bitewe n'amaguru menshi.

Bishobora kugorana gusobanukirwa ibimenyetso byose n'ingorane za KTS icyarimwe. Ariko, muganga wawe ashobora kubigusobanurira byose no kugufasha kuvura ibibazo byawe. Ntutinye kumenyesha muganga wawe uko wiyumva n'ibimenyetso bishya ufite . Kuvugana nawe mu buryo bweruye muri ubu buryo bizoroshya gusaba ubufasha niba ufite ikibazo.

Icyo dushaka kujyana muri iyi nkuru (Ubutumwa bwo kujyana mu rugo)

Sawa, twavuze byinshi kuri KTS, si byo se? Dore ibintu bike ugomba kuzirikana:

• KTS ni indwara idakunze kugaragara, ivukanwa . Ntugahangayike rero, ntabwo uri wenyine.
• Ibimenyetso by'ingenzi ni ikimenyetso cy'amavuko cya 'port-wine stain', ibibazo byo kuboko/ukuguru, n'imitsi .
• Nubwo nta muti wuzuye uhari, hari uburyo bwiza cyane bwo kuvura ibimenyetso .
• Ni ngombwa cyane gusuzuma indwara no gutangira kuyivura vuba bishoboka .
• Ni ngombwa ko umuntu akurikiranwa n'abaganga buri gihe kandi agahabwa ubuvuzi bukenewe mu buzima bwe bwose.
• Komeza kuvugana neza na muganga wawe. Baza ikibazo icyo ari cyo cyose ufite, kandi umubwire uko wumva umeze.

Ndizera ko aya makuru yagufashije gusobanukirwa neza KTS. Niba wowe cyangwa undi muntu uzi afite ibi bimenyetso, ni byiza gushaka inama kwa muganga.

` Indwara ya Klippel-Trenaunay, KTS, ibara rya port-wine, ikimenyetso cy'amavuko, ubumuga bw'imitsi, sisitemu ya limfu, gukura cyane kw'ingingo, indwara yo kuvukana, ikimenyetso cy'amavuko gitukura, ubumuga bw'imitsi, sisitemu ya limfu, gukura cyane kw'ingingo, indwara y'uturemangingo, CLVM