Ingabe ingane yakho inesimila enhliziyweni yayo? Ake sifunde nge-Cardiac Rhabdomyoma!

Njengomama noma ubaba, uma ucabanga ngempilo yengane yakho, ngisho nento encane kakhulu izwakala inkulu, akunjalo? Ikakhulukazi uma udokotela ethi, "Kukhona into efana nesimila esincane enhliziyweni yengane yakho," kungaba buhlungu kakhulu. Kodwa ungakhathazeki . Namuhla sizokhuluma ngesimo osizwa ngaso ngezinye izikhathi ezinjengalezi, kodwa ngokuvamile asiyona ingozi. Leso yi -Cardiac Rhabdomyoma .

Kuyini i-Cardiac Rhabdomyoma?

Kalula nje, i-cardiac rhabdomyoma iyisimila esingenabungozi, esingesona umdlavuza esikhula emisipheni yenhliziyo yengane yakho. Lezi azivamile. Kodwa, ngokumangazayo, ziyizinhlobo ezivame kakhulu zesimila senhliziyo esikhula ngesikhathi sesigaba somntwana, okungukuthi, ngesikhathi ingane isesesibelethweni sikanina ('ukukhulelwa'). Esikhathini esiningi, lawa ma-cardiac rhabdomyoma avela ngesikhathi ingane isesesibelethweni noma phakathi nonyaka wokuqala wokuphila.

Lezi zimila zivela emisipheni yenhliziyo uqobo. Okukhethekile ukuthi ngokuvamile azibonwa zodwa, kodwa zibonakala ngamaqoqo . Yilokhu okuzihlukanisa nama -fibroma, olunye uhlobo lwesimila olungenangozi oluvela enhliziyweni. Ngoba ama-fibroma nawo avela emisipheni yenhliziyo uqobo, kodwa avela njengesimila esisodwa, hhayi ngamaqoqo.

Into ebalulekile ukuthi ama-rhabdomyoma enhliziyo awawona amabi noma angewona umdlavuza . Lokhu kusho ukuthi awasabalali kwezinye izingxenye zomzimba. Okuwukuphela kwendlela angaba yingozi ngayo uma ephazamisa ukusebenza okuvamile kwenhliziyo yengane yakho. Kodwa lokho akuvamile kakhulu. Esikhathini esiningi, lawa mathumba ayashwabana futhi aphele ngokwawo futhi awabangeli izinkinga ezinkulu.

Lezi zimila zingakhula ohlangothini lwesokudla noma lwesobunxele lwenhliziyo yengane. Zivame ukutholakala emakamelweni aphansi enhliziyo , e-ventricle yesokudla noma e-ventricle yesokudla . Ngezinye izikhathi zingakhula nasemakamelweni aphezulu enhliziyo, e-atria , noma odongeni oluhlukanisa amakamelo amabili. Ubukhulu bezimila bungahluka kusukela kumamilimitha ambalwa kuya kumasentimitha amaningana. Zibonakala ziphuzi noma zimhlophe emehlweni.

I-rhabdomyoma yenhliziyo iyisimila esingenabungozi, esingesona umdlavuza esakheka ngamaqoqo emisipheni yenhliziyo.

Ubani onamathuba amaningi okuthuthukisa lokhu?

Izinsana nezingane ezinesimo sofuzo esibizwa ngokuthi i-tuberous sclerosis zisengozini enkulu yokuthola i-cardiac rhabdomyomas. Eqinisweni, cishe izingane ezingu-8 kwezingu-10 ezine-cardiac rhabdomyomas nazo zine-tuberous sclerosis. I-tuberous sclerosis nayo iyisimo sofuzo esingavamile kakhulu. Isibonelo, ithinta cishe ingane eyodwa kwezingu-6,000 ezisanda kuzalwa e-United States.

Ucwaningo alutholanga bufakazi bokuthi ubulili, uhlanga, noma ubuzwe kuthinta ukwenzeka kwalesi simo.

Sivame kangakanani lesi simo?

Ama-rhabdomyoma enhliziyo ayindlela evame kakhulu yesimila senhliziyo ezinsaneni nasezinganeni ezincane. Kodwa-ke, ngokuvamile, izimila zenhliziyo azivamile kangangokuthi ama-rhabdomyoma enhliziyo abhekwa njengengavamile . Izimila eziqala enhliziyweni uqobo (ezibizwa ngokuthi 'izimila zenhliziyo eziyinhloko') zithinta abantu abangaphansi koyedwa kwabangu-2,000.

Kungani i-rhabdomyoma yenhliziyo ikhula?

Kucatshangwa ukuthi ama-rhabdomyomas enhliziyo abangelwa izinguquko zofuzo ezenzeka ngaphambi kokuzalwa . Lezi zinguquko zofuzo ziholela esimweni esibizwa ngokuthi i-tuberous sclerosis. Abantu abane-tuberous sclerosis banoguquko ku-gene `TSC1` noma `TSC2`. Umsebenzi oyinhloko walezi zakhi zofuzo ezimbili ukulawula ukwakheka kwezimila emzimbeni. Ngakho-ke, izingane eziningi ezine-cardiac rhabdomyomas nazo zine-tuberous sclerosis.

Kodwa-ke, ezinye izingane zingaba ne-cardiac rhabdomyoma ngaphandle kwe-tuberous sclerosis. Ezimweni ezinjalo, imbangela eqondile yalesi simila ayikatholakali .

Ingabe lokhu kuyinto evela ekuzalweni? Ingabe kuyifa?

I-rhabdomyoma yenhliziyo ivame ukuzalwa nayo . Ngakho-ke, ibhekwa njengeyokuzalwa nayo. Kodwa-ke, ayifani neyokuzalwa nayo . Uma isimo siwufuzo, singadluliselwa ngezakhi zofuzo zomndeni.

I-rhabdomyoma yenhliziyo ingaba yifa, kodwa ayivamile. Lokhu kungenxa yokuthi isimo sofuzo esibizwa ngokuthi i-tuberous sclerosis, esihlotshaniswa nalesi simila, sivame ukwenzeka ngokuzenzakalelayo, ngaphandle kokuxhumana nofuzo.

Umzali one-tuberous sclerosis unethuba elingu-50% lokuyidlulisela enganeni yakhe. Kodwa-ke, leli "fa" libonakala cishe engxenyeni eyodwa kwezintathu yeziguli ezine-tuberous sclerosis. Ezimweni eziningi, ukuguquka kwezakhi zofuzo kwenzeka okokuqala enganeni esanda kutholakala ukuthi inayo, engenawo umlando womndeni. Lokhu kusho ukuthi i-cardiac rhabdomyoma ngezinye izikhathi ingavela njengoba kulindelekile, futhi ngezinye izikhathi njengesenzakalo esingahleliwe ngaphandle kwezimpawu eziyisixwayiso.

Lokhu kungase kuzwakale kuyinkimbinkimbi, kodwa khumbula, izakhi zofuzo ziyingxenye nje yendaba. Uma unemibuzo noma ukukhathazeka mayelana ne-cardiac rhabdomyoma noma i-tuberous sclerosis, qiniseka ukuthi ukhuluma nodokotela wakho . Xoxa ngomlando womndeni wakho nokuthi ungakuthinta kanjani wena noma ingane yakho.

Ziyini izimpawu zalokhu?

Esikhathini esiningi, i-cardiac rhabdomyoma ayibangeli zimpawu.Kodwa-ke, akuvamile kakhulu, uma isimila noma iqoqo lezimila liba likhulu, lingabangela izinkinga ngaphakathi kwenhliziyo yengane futhi libangele izimpawu. Isibonelo, izimila ezinkulu zingavimba ukugeleza kwegazi noma ziphazamise isigqi senhliziyo. Uma lokhu kwenzeka ngesikhathi sokukhulelwa, kungaba yingozi kakhulu embungwini. Kungaholela ekuhlulekeni kwenhliziyo noma esimweni esibi esibizwa ngokuthi ama-hydrops .

Udokotela wakho uzovame ukuhlola lezi zinkinga ngesikhathi sokukhulelwa ngezivivinyo zezithombe.

Uma lezi zimila ezinganeni ezincane zingapheli zodwa futhi ziqhubeka, noma uma zikhula, zingabangela ukwehluleka kwenhliziyo noma ukushaya kwenhliziyo okungajwayelekile (ama-arrhythmia). Lokhu nakho akuvamile kakhulu. Kodwa udokotela wengane yakho uzoqapha lesi simo ukuze abone ukuthi kukhona yini izinkinga eziphakamayo.

Uma ingane yakho ikhombisa noma yiziphi izimpawu ezilandelayo, shayela u-1990 ngokushesha, noma uyiyise esibhedlela esiseduze:

• Ushintsho ekushayweni kwenhliziyo.
• Inhliziyo esheshayo ( i-tachycardia ).
• Ubunzima bokuphefumula noma ukuphelelwa umoya, ikakhulukazi uma ulele phansi ( i-dyspnea ).
• Ubuhlungu besifuba noma ukuqina okudambisa ukuhlala phansi.
• Ukukhwehlela.
• Ukuzizwa unesizungu noma ukuphelelwa yithemba.
• Ukuzizwa ukhathele noma ubuthakathaka.
• Ukuvuvukala emilenzeni, emaqakaleni, noma esiswini.

Ukubona kanjani lokhu?

I-rhabdomyoma yenhliziyo itholakala ngokuhlolwa kwezithombe . Lokhu kuhlolwa kungenziwa ngesikhathi sokukhulelwa noma ngemva kokuzalwa komntwana.

Ukuhlolwa kokuxilonga i-rhabdomyoma yenhliziyo

Lesi simila sivame ukutholakala okokuqala ngesikhathi sokuhlolwa kwe-ultrasound kwangaphambi kokukhulelwa ngesikhathi sokukhulelwa. Lesi simila siqala ukubonakala ku-ultrasound phakathi kwamasonto angama-20 nama-30 okukhulelwa. Singatholakala futhi nge-scan ekhethekile ebizwa ngokuthi i-fetal echocardiogram . Lolu futhi uhlobo lwe-ultrasound, kodwa lubheka ngqo noma yiziphi izinkinga zenhliziyo ngenkathi umbungu usakhula esibelethweni.

Ukuhlolwa okusetshenziselwa ukuxilonga nokuhlola i-rhabdomyoma yenhliziyo ngemva kokuzalwa komntwana kufaka phakathi:

• I-Electrocardiogram (EKG/ECG)
• I-Echocardiogram (i-echo)
• Isithombe Sokuzwakala Kwe-Magnetic (MRI)

Ingane yakho ingadinga ukuhlolwa njalo, njenge-EKG noma i-echo. Udokotela uzoqapha ama-cyst ukuze abone ukuthi ayancipha yini ngokwawo . Noma yimaphi ama-cyst angapheli ngokwawo kuzodingeka elashwe.

I-rhabdomyoma yenhliziyo ivame ukuphambaniswa ne- tuberous sclerosis.Kungaba uphawu lokuqala lwesifo. Ngakho-ke, kungase kudingeke ukuhlolwa okwengeziwe ukuze kubonakale ukuthi ingane inezimpawu ze-tuberous sclerosis kwezinye izingxenye zomzimba.

Uma ingane yakho itholakale ine-tuberous sclerosis, khuluma nodokotela wakho mayelana nokuhlolwa kwezakhi zofuzo . Ngezinye izikhathi abazali bangase babe nalesi simo kancane kakhulu futhi bangaze batholakale benaso. Ngakho-ke, udokotela wakho angase afune ukubona ukuthi wena noma umlingani wakho ninayo yini i-gene mutation ebangela lesi sifo.

Yiziphi izindlela zokwelapha?

Izindaba ezinhle ukuthi ama-rhabdomyomas enhliziyo avame ukushwabana bese enyamalala ngokwawo , ngakho-ke akukho ukwelashwa okudingekayo . Ezimweni eziningi, lawa mathumba asuke esefinyelele usayizi wawo ophezulu ngesikhathi umntwana ezalwa. Ngemva kwalokho, amathumba ashwabana ngokwawo , ngaphandle kokubangela izinkinga.

Kodwa-ke, akuvamile kakhulu ukuthi la mathumba aphazamise ukusebenza kwenhliziyo futhi adinga ukwelashwa. Ngezinye izikhathi lokhu kungenzeka ngesikhathi sokukhulelwa. Ezimweni ezinjalo, umama angadinga ukuthatha imithi yokunciphisa isimila.

Ngezinye izikhathi izinkinga zingavela ngemva kokuzalwa komntwana. Uma i-cyst noma i-cyst cluster ingahambi yodwa , noma uma ikhula, ingaphazamisa ukusebenza kwenhliziyo yomntwana. Lokhu kungabangela ukwehluleka kwenhliziyo noma ukushaya kwenhliziyo okungajwayelekile (i-arrhythmia).

Uma kunjalo, ingane yakho ingadinga imithi efana nale:

• Izithibi ze-enzyme eguqula i-angiotensin (ACE)
• Ama-diuretics (lena imithi esusa uketshezi oluningi emzimbeni)
• Imithi yokulwa nokuphazamiseka kwemizwa

Udokotela wakho uzoxoxa nawe ngezindlela zokwelapha futhi akusize ukhethe uhlelo olufanele ingane yakho. Ukuhlinzwa akuvamile ukwenziwa .

Iyini ikusasa kulabo abanalesi simo? (Ikusasa)

Iningi labantu abane-cardiac rhabdomyoma abadingi ukwelashwa . Kodwa-ke, uma benezinye izimo, njenge-tuberous sclerosis, kungathinta impilo yabo yesikhathi esizayo.

Uma ingane yakho ine-tuberous sclerosis, izodinga ukuhlolwa njalo kwezokwelapha kanye nokuhlolwa . Kwabanye abantu, lesi simo sincane kakhulu, futhi singase singanakwa. Kwabanye, singabangela izinkinga ezinkulu futhi sithinte kakhulu ikhwalithi yokuphila. Udokotela wakho uzokhuluma nawe ngezidingo ezithile zezokwelapha zengane yakho kanye nokubikezela kwayo. Ndawonye, ​​ningakha uhlelo lokunakekela olungcono kakhulu lwengane yakho.

Yini okufanele ngiyibuze udokotela?

Khuluma nodokotela wakho ngesimo somntwana wakho kanye nendlela abukeka ngayo. Uma kutholakale ukuthi une-cardiac rhabdomyoma ngesikhathi sokukhulelwa, ungase ungazi ukuthi uzoqala kuphi. Udokotela wakho cishe uzochaza izinto eziningi ngaphambi kokuba ubuze imibuzo. Kodwa-ke, kungaba usizo ukubuza imibuzo efana nalena ukuze uthole ulwazi olwengeziwe:

• Singaqapha kanjani i-rhabdomyoma yengane yami?
• Ingabe ingane yami izodinga imithi noma ukuhlinzwa?
• Ingabe ingane yami ine-tuberous sclerosis?
• I-tuberous sclerosis izoyithinta kanjani ingane yami?
• Ingabe kufanele ngithole ukuhlolwa kwezakhi zofuzo kwe-tuberous sclerosis?
• Ungangixhumanisa neqembu lokusekela elisiza abantu abane-tuberous sclerosis?

Ukuthola ukuthi ingane yakho inesimo sezokwelapha kungaba yinto eyesabekayo kakhulu kunokuba nayo wena ngokwakho. Kodwa ngolwazi olufanele kanye nezinsizakusebenza, ungasiza ingane yakho ukuthi iphathe lesi simo futhi ibe ngcono . Uma ingane yakho ine-cardiac rhabdomyoma, khuluma nodokotela wakho ukuze ufunde kabanzi. Udokotela wakho angase ancome isu elibizwa ngokuthi "ukulinda ngokuqapha," elihilela ukubheka amaqhubu nokubona ukuthi ayaphela yini . Ezimweni eziningi, le ndlela iyona kuphela edingekayo. Kodwa-ke, uma kudingeka ukwelashwa, udokotela wakho uzokwazisa.

Isifinyezo (Umyalezo Wokuya Nawe Ekhaya)

Mama noBaba abathandekayo, Nakuba igama elithi Cardiac Rhabdomyoma lingase lizwakale lesabisa, khumbulani ukuthi livame ukuba yisifo esingenabungozi, esingesona umdlavuza . Livame kakhulu ezinganeni, futhi livame ukuphela lodwa ngaphandle kokwelashwa .

Into ebaluleke kakhulu ukuhlala uzolile, ulandele iseluleko sezokwelapha, futhi uxoxe njalo nodokotela wakho ngesimo sengane yakho. Qaphela izimo ezihlobene njenge-tuberous sclerosis, bese wenza izivivinyo ezidingekayo kanye nokulandelela.

Ngifisela ingane yakho ukululama okusheshayo!

I- Rhabdomyoma Yenhliziyo, I-Rhabdomyoma Yenhliziyo, Amathumba Enhliziyo, Isifo Senhliziyo Sezingane, I-Tuberous Sclerosis, I-Tuberous Sclerosis, Isifo Senhliziyo Sengane Esanda Kuzalwa