Reka twige ku ndwara idakunze kugaragara ifata amagufwa yawe n'uruhu rwawe? (Maffucci Syndrome)

Ese wigeze wumva indwara idasanzwe itera ibibazo mu magufwa yawe, rimwe na rimwe uruhu rwawe? Wenda wabonye ikintu nk'igufwa risohoka mu kuboko cyangwa ku kuguru kwawe, cyangwa utudomo dutukura cyangwa tw'umuhengeri ku ruhu rwawe. Ibyo nibyo tugiye kuvugaho uyu munsi, indwara igoye gato, kandi idakunze kugaragara. Yitwa `(Maffucci Syndrome)`. Ntugahangayike, tuzabivugaho mu buryo bworoshye, mu buryo ushobora gusobanukirwa.

None se, iyi ndwara ya Maffucci ni iki?

Muri make, Maffucci Syndrome ni indwara idakunze kugaragara cyane ifata amagufwa n'uruhu rwawe. Ikubiyemo ibintu bitatu by'ingenzi:

1. Indwara y'urusenda ikura cyane mu magufwa yawe. Iyi ndwara ni ingingo ikomeye kandi yoroshye itwikira ibice nk'ingingo zawe. Iyo izi ndwara zikura zikaba ibibyimba mu magufwa yawe, tuyita 'enchondromas'. Izi ndwara si kanseri (zitari mbi), bivuze ko zitangiza mu ntangiriro. Ariko, amagana, cyangwa ndetse n'ibihumbi, by'izi ndwara zishobora kurwara.

2. Izi '(enchondromas)' zitera ubumuga bw'amagufwa. Tekereza, niba hari ikintu gikurira mu igufwa bitari ngombwa, imiterere y'iryo gufwa irahinduka, sibyo? Uko ni ko bimeze. Ibintu nko kugabanya amaguru n'amaboko, kunanura no kuvunika bishobora kubaho .

3. Hemangiomas ni utubyimba tugaragara nk'imitsi idasanzwe y'amaraso ifatanye ku ruhu. Ibi bishobora kugaragara nk'umutuku, ubururu, cyangwa umuhengeri.

Izi ndwara zo mu magufwa y'uruti rw'umugongo, zitwa enchondroma, zikunze kugaragara mu magufwa y'intoki n'amano. Ariko, zishobora no kugira ingaruka ku bice bikurikira:

• Amaboko
• Amaguru
• Imbavu
• Agahanga
• Udukoko tw'umugongo (vertebrae)

Icy'ingenzi ni uko izi enchorroma mu ntangiriro zitari kanseri (zitari mbi), ariko rimwe na rimwe zishobora kuba mbi (zishobora kurwara). Umubare munini w'abantu barwaye Maffucci syndrome (kugeza kuri 50% nk'uko ubushakashatsi bumwe na bumwe bubivuga) bafite ibyago byinshi byo kurwara kanseri y'amagufwa yitwa chondrosarcoma, itangira mu turemangingo tw'imitsi. Bafite kandi ibyago byinshi byo kurwara izindi kanseri.

Indwara ya Maffucci ikunze kugaragara gute?

Mu by’ukuri, iyi ni indwara idasanzwe. Hari amagana make gusa y’abantu barwaye yavuzwe mu nyandiko z’abaganga ku isi yose, bityo ntibitangaje niba utarigeze ubyumva mbere.

Ni ibihe bimenyetso bya Maffucci Syndrome?

Ibimenyetso bya Maffucci syndrome bishobora gutandukana bitewe n'umuntu ku giti cye. Hari abantu bashobora kugira ibimenyetso byoroheje cyane, abandi bakaba bafite ibimenyetso bikomeye kurushaho. Ibi bimenyetso bishobora kugaragara mu gihe cyo kuvuka, cyangwa bigatangira kugaragara mu bwana.

Ibimenyetso by'ingenzi biterwa na bya bibyimba bya karitsiye twavugaga byitwa "enchondromas." Bishobora gutera ibintu nk'ibi:

• Kubabara amagufwa
• Gucika kw'amagufwa - Igufwa rishobora kuvunika nubwo ryagwa gato.
• Amaboko cyangwa amaguru yunamye
• Amagufwa abyimbye - asa n'ibibyimba.
• Uruti rw'umugongo rugoramye - bisa na scoliosis.
• Osteolysis ( gucika kw'amagufwa )
• Mugufi
• Amaboko cyangwa amaguru adahuje – rimwe rigaragara nk'aho ari maremare cyangwa magufi kurusha irindi.

Ibibyimba byo mu mitsi y'amaraso, bizwi nka hemangiomas, bishobora gutera ibimenyetso nk'ibi:

• Amaraso apfutse
• Lymphangiomas – Ibi bimeze nk'udusimba twuzuyemo amazi y'imitsi.
• Utubyimba dutukura, umuhengeri, cyangwa ubururu ku ruhu (hemangiomas) - dukunze kugaragara ku maboko. Uko igihe kigenda gihita, dushobora gukomera no gucikamo ibice.
• Imitsi idakura neza - mu gace kagize ikibazo.

Ni iki gitera indwara ya Maffucci?

Indwara ya Maffucci ni indwara iterwa n’uturemangingo. Ibi bivuze ko iterwa n’impinduka (mutation) mu turemangingo twacu. By’umwihariko, iterwa n’impinduka zitunguranye muri imwe muri jeni ebyiri yitwa `IDH1` (ikunze kugaragara cyane) cyangwa `IDH2` (idasanzwe). Abashakashatsi bemeza ko iyi mpinduka iterwa n’uturemangingo ibaho umwana akiri mu nda, ni ukuvuga mbere yo kuvuka. Izi jeni za `IDH` zifasha gukora `(enzymes)` zifitiye akamaro imikorere y’uturemangingo mu mubiri wacu. Ariko, abahanga mu bya siyansi ntibarabasha kumenya isano nyayo iri hagati y’izi `(enzymes)` na Maffucci syndrome.

Igikuru ni uko iyi atari indwara ikomoka ku ruragi. Ni ukuvuga ko atari indwara ishobora kwanduzwa mu gisekuru kimwe ijya mu kindi kuko ababyeyi bayirwaye. Iyi ni ihinduka ry’uturemangingo mu buryo butunguranye.

Indwara ya Maffucci ipimwa ite?

Ibi bizamini bishobora gufasha muganga gusuzuma indwara ya Maffucci:

• Isuzuma ry'umubiri: Muganga azagusuzuma kandi arebe ibimenyetso byose bigaragara (nk'ibibyimba cyangwa ubumuga). Azakubaza kandi ibimenyetso byawe ndetse niba hari umuntu wo mu muryango wawe wigeze kugira ibibazo nk'ibyo.
• Gupima X-ray cyangwa CT scans: Ibi bishobora gufata amashusho meza y'amagufwa yawe. Hanyuma bashobora kureba niba hari enchondromas, aho zakwirakwiriye, ndetse niba hari n'ibyangiritse mu magufwa.
• Gufata ingero z'ingingo mu gihe cyo kubaga no kuzisuzuma (`Biopsy`):Hari igihe agace gato k'umubiri kabagwa mu gace gakekwaho (nk'igufwa cyangwa ikibyimba cy'uruhu) kagasuzumwa hakoreshejwe mikorosikopi. Ibi nibyo bituma gakira neza.

Ni ubuhe bwoko bw'inzobere buvura indwara ya Maffucci?

Niba urwaye indwara ya Maffucci, itsinda ry'inzobere zitandukanye rishobora guterana kugira ngo rikuvure. Urugero:

• Inzobere mu by'uruhu: Ifasha kuvura, kugabanya, cyangwa, bibaye ngombwa, gukuraho ibibyimba by'uruhu nka hemangiomas.
• Umuganga w’amagufwa: Avura imvune, akabaga agakosora ubumuga bw’amagufwa, ndetse akanabagwa agakuraho enchondromas.
• Umuganga w’indwara zo mu magufwa: Iyo kanseri nka chondrosarcoma twavuze mbere igaragaye, aba baganga babafasha kuyikuraho no gukurikirana indwara mu buzima bwabo bwose kugira ngo barebe niba izongera kubaho.
• Inzobere mu by’imirasire: Baziga mu gufata amashusho y’ubuvuzi, nka X-rays na CT scans, kandi ni ingenzi mu gusuzuma impinduka mu magufwa uko igihe kigenda gihita.

Indwara ya Maffucci ivurwa ite?

Mu by'ukuri, nta buryo bwo gukiza burundu indwara ya Maffucci buhari kugeza ubu. Kubwibyo, intego nyamukuru zo kuvura ni izi zikurikira:

• Gupima kanseri hakiri kare no gutanga ubuvuzi bwiza kurushaho.
• Kurinda cyangwa kuvura kuvunika kw'amagufwa.
• Kugabanya ibimenyetso nk'ububabare.

Itsinda ry'abaganga bawe rikunze gutumiza ibizamini by'ubuvuzi (nk'imirasire ya X) kugira ngo bakurikirane amagufwa yawe. Niba ibibazo bya echondroma cyangwa amagufwa bibangamira imirimo yawe ya buri munsi, ubuvuzi bushobora kuba burimo:

• Kwambara imishumi cyangwa kubagwa kugira ngo bakosore scoliosis.
• Ingufu, imitsi yo gufunga, no kubagwa amagufwa yavunitse.
• Gabanya itandukaniro mu burebure bw'amaguru ukoresheje inkweto zidasanzwe (inkweto ziterura), imiti, cyangwa kubagwa.
• Kubagwa kugira ngo hakosorwe ubumuga mu ntoki.
• (Enchondromas) ishobora gukurwaho hakoreshejwe uburyo bwo kubaga. Ariko, izi zishobora kongera kugaruka.

Niba hemangiomas ku ruhu rwawe ikubangamiye, muganga wawe ashobora kukugira inama yo kuvura yitwa sclerotherapy. Ibi bikubiyemo gutera umuti mu ruhu kugira ngo ugabanye ibibyimba. Iyo bibaye ngombwa, hemangiomas zishobora gukurwaho.

Ese indwara ya Maffucci ishobora kwirindwa?

Nkuko twabiganiriyeho mbere, abahanga mu bya siyansi ntibarasobanukirwa neza uburyo ihinduka ry’uturemangingo ritera ibi ribaho. Kubwibyo, nta buryo bwihariye bwo kubikumira buriho ubu.

Ejo hazaza h'umuntu urwaye indwara ya Maffucci ni he?

Indwara ya Maffucci ni indwara idakira kandi ibaho ubuzima bwose. Hari abantu bashobora kugira ibibazo byoroheje by’umubiri, bishobora kutagira ingaruka nyinshi ku mirimo yabo ya buri munsi. Ariko, ku bandi, ibibazo by’amagufwa bishobora kuba bikomeye kurushaho, kandi ubumuga bw’umubiri bushobora kwiyongera.

Icy'ingenzi ni uko iyi ndwara idakora ku bwenge. Ariko, kanseri iramutse ibaye, hari amahirwe ko ishobora kugabanya igihe cyo kubaho.

Ni gute nakwiyitaho nk'umuntu ufite indwara ya Maffucci?

Kubera ko abantu barwaye indwara ya Maffucci baba bafite ibyago byinshi byo kurwara kanseri, ni ngombwa ko basuzumwa buri gihe nk'uko byasabwe n'itsinda ry'abaganga. Uko kanseri igaragaye hakiri kare, ni ko iba ishobora kuvurwa.

Ni irihe tandukaniro riri hagati ya Ollier Syndrome na Maffucci Syndrome?

Indwara ya Ollier na Maffucci syndrome byombi ni indwara zitera kanseri y'uruti rw'umugongo yitwa enchondroma. Ariko, muri Maffucci syndrome, hari n'ibibyimba by'imitsi y'amaraso yitwa hemangiomas. Indwara ya Ollier ntabwo igira hemangiomas. Iryo ni ryo tandukaniro rikomeye.

Ese hari andi mazina ya Maffucci Syndrome?

Kubera ko ari gake cyane, iyo ndwara rimwe na rimwe yitwa andi mazina, ariko ntabwo ikunze kugaragara. Dore ingero zimwe na zimwe:

• `(Chondrodysplasia hamwe na hemangioma)`
• `(Dyschondroplasia na cavernous hemangioma)`
• `(Enchondromatosis hamwe na hemangiomata)`
• `(Indwara ya Kast)`

Nubwo hari amazina menshi nk'aya, izina rikunze gukoreshwa cyane ni Maffucci Syndrome.

Amaherezo, ubutumwa bwo kujyana mu rugo

Niba wowe cyangwa umwana wawe murwaye Maffucci Syndrome, bishobora kuba biremereye gato. Ugomba guhangayikishwa n'imvune na kanseri y'amagufwa ubuzima bwawe bwose. Bishobora kandi kunaniza kubonana n'inzobere zitandukanye no gupimwa kenshi.

Ariko, kwitonda muri ubu buryo, cyane cyane kwipimisha kanseri hakiri kare, bishobora gufasha cyane mu kurokora cyangwa kongera igihe cyo kubaho. Gutahura kanseri hakiri kare no kuyivura ni bwo buryo bwiza bwo kunoza ubuzima bwawe. Muganga wawe azakubwira ibimenyetso ugomba kwitondera.

Wihangayika. Ntabwo uri wenyine. Hari abaganga n'amatsinda y'ubufasha yo gufasha no kuyobora abantu babana n'izi ndwara zidasanzwe. Icy'ingenzi ni ukubona amakuru nyayo no gukurikiza inama za muganga wawe.

Indwara ya Maffucci , indwara z'amagufwa, cartilage, hemangioma, ibyago byo kurwara kanseri, indwara zikomoka ku ngirabuzimafatizo, indwara zidasanzwe