Ingaba umntwana wakho unethumba entliziyweni yakhe? Masifunde ngeCardiac Rhabdomyoma!

Njengomama okanye utata, xa ucinga ngempilo yomntwana wakho, nokuba yinto encinci ivakala inkulu, akunjalo? Ingakumbi xa ugqirha esithi, "Kukho into efana nethumba elincinci entliziyweni yomntwana wakho," kunokuba buhlungu kakhulu. Kodwa ungakhathazeki . Namhlanje siza kuthetha ngemeko oye uve ngayo ngamanye amaxesha anje, kodwa idla ngokuba yingozi. Le yiCardiac Rhabdomyoma .

Yintoni iCardiac Rhabdomyoma?

Ngamafutshane, i-cardiac rhabdomyoma sisimila esingenabungozi, esingengomhlaza esikhula kwimisipha yentliziyo yomntwana wakho. Ezi azixhaphakanga kakhulu. Kodwa, ngokumangalisayo, zezona ntlobo zixhaphakileyo zesifo sentliziyo esikhula ngexesha lokukhulelwa, oko kukuthi, ngelixa umntwana esesesibelekweni sikanina ('gestation'). Uninzi lwexesha, ezi rhabdomyomas zentliziyo zivela ngelixa umntwana esesesibelekweni okanye kunyaka wokuqala wobomi.

Ezi thumba zivela kwimisipha yentliziyo ngokwayo. Okukhethekileyo kukuba azibonwa zodwa, kodwa zibonwa ngamaqoqo . Yile nto izenza zahluke kwi -fibromas, olunye uhlobo lwethumba olungenabungozi olukhula entliziyweni. Kuba ii-fibromas nazo zivela kwimisipha yentliziyo ngokwayo, kodwa zivela njengethumba elinye, kungekhona ngamaqoqo.

Into ebalulekileyo kukuba ii-cardiac rhabdomyomas aziyongozi okanye zingumhlaza . Oku kuthetha ukuba azisasazeki kwezinye iindawo zomzimba. Eyona ndlela zinokuba yingozi ngayo kukuba ziphazamisana nokusebenza kwentliziyo yomntwana wakho. Kodwa oko kunqabile kakhulu. Uninzi lwexesha, ezi thumba ziyashwabana kwaye ziphele zodwa kwaye azibangeli ngxaki zinkulu.

Ezi thumba zinokukhula kwicala lasekunene okanye lasekhohlo lentliziyo yomntwana. Zifumaneka kakhulu kwiindawo ezisezantsi zentliziyo , kwi-ventricle yasekunene okanye kwi-ventricle yasekhohlo . Ngamanye amaxesha zinokukhula nakwiindawo eziphezulu zentliziyo, kwi-atria , okanye eludongeni olwahlula la magumbi mabini. Ubungakanani bezi thumba bungahluka ukusuka kwiimilimitha ezimbalwa ukuya kwiisentimitha ezininzi. Zibonakala zimthubi okanye zimhlophe emehlweni.

I-cardiac rhabdomyoma sisimila esingenabungozi, esingengomhlaza esakha amaqela kwizihlunu zentliziyo.

Ngubani onokuthi aphuhlise oku ngakumbi?

Iintsana kunye nabantwana abane- tuberous sclerosis basengozini enkulu yokuba ne-cardiac rhabdomyomas. Enyanisweni, malunga nabantwana abasi-8 kwabalishumi abane-cardiac rhabdomyomas nabo bane-tuberous sclerosis. I-tuberous sclerosis nayo yi-genetic disorder engaqhelekanga kakhulu. Umzekelo, ichaphazela malunga ne-1 kwi-6,000 yeentsana ezisandula ukuzalwa eMelika.

Uphando alufumananga bungqina bokuba isini, uhlanga, okanye uhlanga luchaphazela ukwenzeka kwale meko.

Ixhaphake kangakanani le meko?

Iirhabdomyomas zentliziyo zezona ntlobo zixhaphakileyo zesifo sentliziyo kwiintsana nabantwana abancinci. Nangona kunjalo, ngokubanzi, iirhabdomyomas zentliziyo zinqabile kangangokuba iirhabdomyomas zentliziyo zithathwa njengezingaqhelekanga . Iirhabdomyomas eziqala entliziyweni ngokwayo (ezibizwa ngokuba yi-'primary cardiac tumors') zichaphazela abantu abangaphantsi komnye kubantu abangama-2,000.

Kutheni i-rhabdomyoma yentliziyo ikhula?

Kukholelwa ukuba ii-cardiac rhabdomyomas zibangelwa lutshintsho lwemfuza olwenzeka ngaphambi kokuzalwa . Olu tshintsho lwemfuza lukwakhokelela kwimeko ebizwa ngokuba yi-tuberous sclerosis. Abantu abane-tuberous sclerosis banotshintsho kwi-genes `TSC1` okanye `TSC2`. Umsebenzi ophambili wezi gene zimbini kukulawula ukwakheka kwee-tumor emzimbeni. Ngoko ke, abantwana abaninzi abane-cardiac rhabdomyomas nabo bane-tuberous sclerosis.

Nangona kunjalo, abanye abantwana banokuba ne-cardiac rhabdomyoma ngaphandle kwe-tuberous sclerosis. Kwiimeko ezinjalo, unobangela ochanekileyo wale tumor awukafunyanwa .

Ingaba le yinto evela ekuzalweni? Ngaba yimfuza?

I-rhabdomyoma yentliziyo idla ngokuba yinto yokuzalwa . Ke ngoko, ithathwa njengeyokuzalwa. Nangona kunjalo, ayifani neyokuzalwa . Ukuba imeko idluliselwa kwilifa, inokudluliselwa nge-genes yosapho.

I-rhabdomyoma yentliziyo inokuba yimfuza, kodwa ayiqhelekanga. Oku kungenxa yokuba imeko yemfuza ebizwa ngokuba yi-tuberous sclerosis, enxulunyaniswa nale thumba, idla ngokuzenzekela, ngaphandle konxibelelwano lwemfuza.

Umzali one-tuberous sclerosis unethuba elingama-50% lokuyidlulisela kumntwana wakhe. Nangona kunjalo, eli "lifa" libonwa kuphela kwisithathu sezigulane ezine-tuberous sclerosis. Kwiimeko ezininzi, utshintsho lwemfuza lwenzeka okokuqala kumntwana osandul' ukuxilongwa , ongenalo imbali yosapho. Oku kuthetha ukuba i-cardiac rhabdomyoma ngamanye amaxesha inokuvela njengoko bekulindelwe, kwaye ngamanye amaxesha njengesiganeko esingacwangciswanga ngaphandle kweempawu zesilumkiso.

Oku kunokuvakala kuyinkimbinkimbi, kodwa khumbula, ii-genes ziyinxalenye nje yebali. Ukuba unemibuzo okanye iinkxalabo malunga ne-cardiac rhabdomyoma okanye i-tuberous sclerosis, qiniseka ukuba uthetha nogqirha wakho . Xoxa ngembali yosapho lwakho kunye nendlela enokukuchaphazela ngayo wena okanye umntwana wakho.

Zithini iimpawu zoku?

Uninzi lwexesha, i-cardiac rhabdomyoma ayibangeli zimpawu.Nangona kunjalo, kunqabile kakhulu, ukuba ithumba okanye iqela leethumba liba likhulu, linokubangela iingxaki ngaphakathi entliziyweni yomntwana kwaye libangele iimpawu. Umzekelo, iithumba ezinkulu zinokuthintela ukuhamba kwegazi okanye ziphazamise isigqi sentliziyo. Ukuba oku kwenzeka ngexesha lokukhulelwa, kunokuba yingozi kakhulu kumntwana ongekazalwa. Kungakhokelela ekusileleni kwentliziyo okanye kwimeko embi ebizwa ngokuba yi-hydrops .

Ugqirha wakho uhlala ejonga ezi ngxaki ngexesha lokukhulelwa ngokuvavanya imifanekiso.

Ukuba ezi thumba kwiintsana nakubantwana abancinci azipheli zodwa kwaye ziyaqhubeka, okanye ukuba zikhula zibe nkulu, zinokubangela ukungasebenzi kakuhle kwentliziyo okanye ukubetha kwentliziyo okungahambelaniyo (arrhythmias). Oku nako kunqabile kakhulu. Kodwa ugqirha womntwana wakho uya kujonga le meko ukuze abone ukuba kukho naziphi na iingxaki eziphakamayo.

Ukuba umntwana wakho ubonakalisa naziphi na kwezi mpawu zilandelayo, fowunela u-1990 ngoko nangoko, okanye umse esibhedlele esikufutshane:

• Utshintsho kwisigqi sentliziyo.
• Ukubetha kwentliziyo ngokukhawuleza ( i-tachycardia ).
• Ubunzima bokuphefumla okanye ukuphefumla kancinci, ingakumbi xa ulele phantsi ( ukuphelelwa ngamandla ).
• Intlungu yesifuba okanye ukuxinana okuthomalaliswa kukuhlala phantsi.
• Ukukhwehlela.
• Ukudinwa okanye ukuquleka.
• Ukuziva udiniwe okanye ubuthathaka.
• Ukudumba kwemilenze, amaqatha, okanye isisu.

Uyibona njani le nto?

I-rhabdomyoma yentliziyo ifunyaniswa ngovavanyo lwemifanekiso . Olu vavanyo lunokwenziwa ngexesha lokukhulelwa okanye emva kokuba umntwana ezelwe.

Uvavanyo lokufumanisa isifo sentliziyo i-rhabdomyoma

Le thumba idla ngokubonwa okokuqala ngexesha lokuhlolwa kwe-ultrasound ngaphambi kokukhulelwa. Le thumba iqala ukubonakala kwi-ultrasound phakathi kweeveki ezingama-20 ukuya kwezingama-30 zokukhulelwa. Ingabonwa nangokuhlolwa okukhethekileyo okubizwa ngokuba yi-fetal echocardiogram . Olu luhlobo lwe-ultrasound, kodwa ijonga ngokukodwa naziphi na iingxaki zentliziyo ngelixa umntwana esakhula esibelekweni.

Uvavanyo olusetyenziselwa ukuxilonga nokuvavanya i-cardiac rhabdomyoma emva kokuba umntwana ezelwe luquka:

• I-Electrocardiogram (EKG/ECG)
• I-Echocardiogram (isandi)
• Umzobo weMagnetic Resonance (MRI)

Umntwana wakho usenokufuna ukuvavanywa rhoqo, njenge-EKG okanye i-echo. Ugqirha uza kujonga ama-cysts ukuze abone ukuba ayancipha ngokwawo na . Naziphi na ii-cysts ezingapheli ngokwazo ziya kufuneka zinyangwe.

I-rhabdomyoma yentliziyo idla ngokubhidaniswa ne -tuberous sclerosis.Isenokuba luphawu lokuqala lwesi sifo. Ngenxa yoko, kunokufuneka uvavanyo olongezelelekileyo ukuze kubonwe ukuba umntwana uneempawu ze-tuberous sclerosis kwezinye iindawo zomzimba.

Ukuba umntwana wakho ufunyaniswe ene-tuberous sclerosis, thetha nogqirha wakho malunga novavanyo lwezakhi zofuzo . Ngamanye amaxesha abazali banokuba nale meko kancinci kakhulu kwaye bangaze bafunyaniswe benayo. Ngoko ke, ugqirha wakho unokufuna ukubona ukuba wena okanye iqabane lakho ninayo na i-gene mutation ebangela esi sifo.

Zithini iindlela zonyango?

Iindaba ezimnandi zezokuba ii-cardiac rhabdomyomas zihlala zishwabana zize zinyamalale zodwa , ngoko ke akukho nyango lufunekayo . Kwiimeko ezininzi, ezi zicubu zifikelele kubukhulu bazo obuphezulu ngexesha lokuzalwa komntwana. Emva koko, ezi zicubu zishwabana zodwa , ngaphandle kokubangela naziphi na iingxaki.

Nangona kunjalo, kunqabile kakhulu ukuba ezi thumba ziphazamise ukusebenza kwentliziyo kwaye zifune unyango. Ngamanye amaxesha oku kunokwenzeka ngexesha lokukhulelwa. Kwiimeko ezinjalo, umama unokufuna ukuthatha amayeza okunciphisa ithumba.

Ngamanye amaxesha iingxaki zinokubakho emva kokuba umntwana ezelwe. Ukuba i-cyst okanye i-cyst cluster ayizipheli yodwa , okanye ukuba ikhula ibe nkulu, inokuphazamisana nokusebenza kwentliziyo yomntwana. Oku kunokubangela ukungasebenzi kakuhle kwentliziyo okanye ukubetha kwentliziyo okungahambelaniyo (arrhythmia).

Ukuba kunjalo, umntwana wakho usenokufuna amayeza anjengala:

• Izithinteli ze-enzyme eguqula i-angiotensin (ACE)
• I-Diuretics (la ngamayeza asusa ulwelo olugqithisileyo emzimbeni)
• Amayeza okulwa nokuphazamiseka kwentliziyo

Ugqirha wakho uza kuxoxa nawe ngeendlela zonyango aze akuncede ukhethe isicwangciso esilungele umntwana wakho. Utyando alufuneki rhoqo .

Iyintoni imbono yabo banale meko? (Imbono)

Uninzi lwabantu abane-cardiac rhabdomyoma abadingi nyango . Nangona kunjalo, ukuba banezinye iimeko, ezifana ne-tuberous sclerosis, inokuchaphazela impilo yabo yexesha elizayo.

Ukuba umntwana wakho une-tuberous sclerosis, uya kufuna ukuvavanywa rhoqo ngugqirha kunye novavanyo . Kwabanye abantu, le meko incinci kakhulu, kwaye isenokungaqatshelwa. Kwabanye, inokubangela iingxaki ezinkulu kwaye ichaphazele kakubi umgangatho wobomi. Ugqirha wakho uya kuthetha nawe ngeemfuno zonyango ezithile zomntwana wakho kunye noqikelelo lwesifo. Kunye, ninokwenza isicwangciso sokhathalelo esingcono kakhulu somntwana wakho.

Ndingayibuza ntoni ugqirha?

Thetha nogqirha wakho ngemeko yomntwana wakho kunye nendlela azibona ngayo izinto. Ukuba ufunyaniswe une-cardiac rhabdomyoma ngexesha lokukhulelwa, usenokungazi ukuba uqale phi. Ugqirha wakho usenokuchaza izinto ezininzi ngaphambi kokuba ubuze nemibuzo. Nangona kunjalo, kunokuba luncedo ukubuza imibuzo efana nale ukuze ufumane ulwazi oluthe kratya:

• Singawujonga njani umkhuhlane womntwana wam?
• Ngaba umntwana wam uya kufuna amayeza okanye utyando?
• Ngaba umntwana wam une-tuberous sclerosis?
• I-tuberous sclerosis iza kumchaphazela njani umntwana wam?
• Ngaba kufuneka ndifumane uvavanyo lwemfuza lwe-tuberous sclerosis?
• Ungandidibanisa neqela lenkxaso elinceda abantu abane-tuberous sclerosis?

Ukufumanisa ukuba umntwana wakho unemeko yezonyango kunokuba yinto eyoyikisayo kunokuba nayo ngokwakho. Kodwa ngolwazi olufanelekileyo kunye nezixhobo, ungamnceda umntwana wakho ukuba alawule le meko aze aphile . Ukuba umntwana wakho une-cardiac rhabdomyoma, thetha nogqirha wakho ukuze ufunde okungakumbi. Ugqirha wakho unokucebisa icebo elibizwa ngokuba yi- "waiting waiting," elibandakanya ukujonga amaqhuma nokujonga ukuba ayaphela na . Kwiimeko ezininzi, le ndlela kuphela efunekayo. Nangona kunjalo, ukuba unyango luyadingeka, ugqirha wakho uya kukwazisa.

Isishwankathelo (Umyalezo Wokuya Ekhaya)

Mama noTata abathandekayo, Nangona igama elithi Cardiac Rhabdomyoma lisenokuvakala lisoyikisa, khumbula ukuba lidla ngokuba sisimila esingenabungozi, esingengomhlaza . Liqhelekile kakhulu kwiintsana, kwaye lihlala liziphelela ngaphandle konyango .

Eyona nto ibalulekileyo kukuhlala uzolile, ulandele iingcebiso zonyango, kwaye unxibelelane rhoqo nogqirha wakho malunga nemeko yomntwana wakho. Qaphela iimeko ezinxulumene noko ezifana ne-tuberous sclerosis, kwaye wenze uvavanyo olufunekayo kunye nokulandelelana.

Ndikunqwenelela umntwana wakho ukuba aphile ngokukhawuleza!

I- Rhabdomyoma yentliziyo, I-Rhabdomyoma yentliziyo, Iithumba zentliziyo, Isifo sentliziyo sabantwana abancinci, I-Tuberous Sclerosis, I-Tuberous Sclerosis, Isifo sentliziyo sabantwana abangekazalwa