I-Marfan syndrome yiyona nto ixhaphakileyo yintlupheko yesisombululo esivumelanayo, esichaphazela isistim skeletal, intliziyo, imithwalo yegazi kunye namehlo. Abantu abaneMarfan syndrome bahlala bephakame kwaye bancinci, kwaye iingalo kunye nemilenze yabo lide kunexesha eliqhelekileyo. Kananjalo baninzi banominwe omude (imeko yoogqirha bayabiza i-arachnodactyly), i-curvature engavamile yesihlingo ( kyphoscoliosis ), kunye nokutshintshwa kwelensi yeso .
Ubunzima obunobungozi bokuphila kwe-Marfan syndrome buhlobene nentliziyo kunye nemithambo yegazi; ngokukodwa, kwi- aneurysm ye-aorta . I-aneurysm i-dilation (i-ballooning-out) yodonga lwesitya segazi. Olu phulo lubuthathaka kakhulu udonga lwe-aorta, kwaye lwenze ukuba luthandwe ngokukhawuleza (imeko apho oogqirha babiza ukutshintshwa kwe-dissection). Ukusabalalisa kwe-Aortic yimeko engxamisekileyo yonyango, kwaye kunokukhokelela ekufeni ngokukhawuleza kubantu abaneMarfan syndrome.
Ekubeni le nkathazo iyakwenzeka ngexesha lexesha xa i-cardiovascular system ixininiswa, ukunyamezela, ngokukodwa, kunokunciphisa i-aortic dissection kumntu one-Marfan syndrome. Ngenxa yoko, abantu abatsha abane-Marfan syndrome kufuneka bawuthintele ukuthatha inxaxheba kwimisebenzi yezemidlalo.
Akunakuze kube lula kubantu abatsha ukuba bakuve ukuba umsebenzi wabo wecala unganciphisa. Ngenhlanhla, abaninzi abantu abaneMarfan syndrome bangakwazi ukuhlala bekhuthele, kodwa banqandekile.
Kubalulekile ukuba aba badlali abancinci baqaphele imida yabo.
Iingcebiso eziqhelekileyo zokuQinisekisa Kwabadlali abaLutsha abaneMarfan Syndrome
Iziphakamiso eziqhelekileyo ngokubandakanyeka kumsebenzi wezemidlalo okhuphisanayo nabadlali abahamba ngeMarfan syndrome zanyatheliswa yiNgqungquthela ye-Bethesda ka-2005 malunga neNyanzeliso yeeNgcaciso zePhuphiswano ngeCardiovascular anomali.
Nantsi isishwankathelo salezo ngcebiso:
Abadlali abadlali abane-Marfan syndrome kufuneka babe ne- echocardiograms nganye kwiinyanga ezintandathu ukujonga ukuhlanjululwa kwengcambu ye-aorta.
Ukuba akukho i-aortic yokuxhoma okanye ezinye izinto ezibi kakhulu zentliziyo, kwaye akukho nentsapho yembali ye-aortic dissection, inokuthi ithathe inxaxheba kwizinto ezibizwa ngokuba yizinto ezisezantsi kwaye zisezingeni elilinganayo lemidlalo yezemidlalo - loo mi sebenzi ayisoloko idinga "imisindo" ephezulu kakhulu ukusetyenziswa ngamandla. Imizekelo yemisebenzi efanelekileyo ibandakanya igalofu, isithsaba, i-hikingl, kunye netafile ye-tennis.
Ukuba kukho ubungqina bokunyuselwa kweengcambu ze-aortic, okanye imbali yentsapho ye-aortic dissection, ngoko kuphela umsebenzi ophantsi kakhulu, njengegalufu okanye isithsaba, kufanelekile.
Abantu abaneMarfan syndrome bayacetyiswa ukuba baphephe imisebenzi ephezulu-mngcipheko - qhagamshelana nemidlalo kunye nemisebenzi efuna uvavanyo lwe-isometric oluvelisa u-Valsalva umqhubi (njengokuphakama kwesisindo).
Abanye abantu abane-Marfan syndrome banokuhlulwa ngabanye oogqirha babo (ukuba ingozi yabo igwetywa kakhulu), ukuba bathathe inxaxheba kwimidlalo yengozi efana nebhokballball, i-baseball, ibhola lebhola, kunye nebhayisikili.
Ingqalelo, iNgqungquthela yaseBetdada ijongene ngqo nabantu ababandakanyekayo kwiimidlalo ezidibeneyo, ezikhuphisanayo.
Ngokukodwa, ibeka izikhokelo ezikolweni nakweminye imibutho apho abadlali abajongene ne-Marfan syndrome banokufuna ukuthatha inxaxheba kwiinkqubo zabo. Akuzange kulandelwe ngqo umdlali wezokuzonwabisa.
Nangona kunjalo, iziphakamiso zeBetdada ziyakwazi ukunika iingcebiso kubadlali bezokuzonwabisa kunye noogqirha babo. Kwinoma ubani onama-marfan syndrome onqwenela ukuzibandakanya kwimidlalo, i-echocardiograms ngezihlandlo zingasetyenziselwa ukukhokela izinga elifanelekileyo lomsebenzi.
ILizwi
Abantu abaneMarfan syndrome banomngcipheko ongapheliyo kwimiba enesifo senhliziyo, kwaye bafuna ukulandelwa rhoqo kwezokwelapha.
Ukuthintela imingcipheko iyacetyiswa nakubani na abane-Marfan syndrome. Nangona kunjalo, umlinganiselo wezithintelo uya kuhluka kumntu kumntu, kwaye abaninzi banako (kwaye bakhuthazwa ukuba) banandipha ubomi bokusebenza ngokufanelekileyo kunye nokulinda okufanelekileyo.
> Imithombo:
> Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 I-ACCF / AHA / AATS / ACA / ACA / ASA / SCA / SCAI / SIR / STS / SVM Izikhokelo zoLwazi kunye noLawulo lwezigulane ezinezifo ze-Thoracic Aortic: ingxelo ye-American College of Cardiology Foundation / i-American Heart Association Task Force on Practice Izikhokelo, i-American Association ye-Thoracic Surgery, i-American College of Radiology, i-American Stroke Association, i-Society of Cardiovascular Anesthesiologists, i-Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, i-Society of Thoracic Surgeons, kunye ne-Society ye-Vascular Medicine. Ukuhamba ngo-2010; 121: e266.
> Maron, BJ, Ackerman, MJ, Nishimura, RA, et al. Umsebenzi we-4: I-HCM kunye nezinye i-Cardiomyopathies, i-Mitral Valve Prolapse, i-Myocarditis, ne-Marfan Syndrome. J Am Coll Cardiol 2005; 45: 1340