I-Marfan syndrome iyisifo esivame kakhulu esizuzwe njengezicubu ezixhumayo , evame ukuthinta uhlelo lwamathambo, inhliziyo, imithwalo yegazi namehlo. Abantu abane-Marfan syndrome bavame ukude kakhulu futhi bancane, futhi izingalo nemilenze yabo sekude kunejwayelekile. Kaningi futhi baneminwe ende (isimo odokotela babiza i-arachnodactyly), isivunguvungu esingavamile somgogodla ( kyphoscoliosis ), nokuhlukaniswa kwe- lens iso .
Izinkinga ezisongela impilo kaMarfan syndrome zihlobene nenhliziyo nemithambo yegazi; ikakhulukazi, ku- aneurysm ye-aorta . I-aneurysm i-dilation (ibhaloning-out) kodonga lwesitsha segazi. Lokhu kuncibilikisa kunciphisa kakhulu udonga lwe-aorta, futhi kwenza kube lula ukuphuka ngokungazelelwe (isimo odokotela ababiza ngokuthi dissection). Ukususwa kwe-Aortic kuyisimo esiphuthumayo sezokwelapha, futhi kungaholela ekufeni okungazelelwe kubantu abaneMarfan syndrome.
Njengoba le nhlekelele ingase ibe khona phakathi kwezikhathi lapho uhlelo lwezinhliziyo lucindezelwa, ukuzivocavoca, ikakhulukazi, kunganciphisa ukuhlukaniswa kwe-aortic kumuntu one-Marfan syndrome. Ngenxa yalesi sizathu, abantu abasha abane-Marfan syndrome kumele bavame ukunciphisa ukuhlanganyela kwabo emisebenzini yezemidlalo.
Akulula neze kubantu abasha ukuzwa ukuthi umsebenzi wabo womzimba kufanele ulinganiselwe. Ngenhlanhla, iningi labantu abane-Marfan syndrome likwazi ukuhlala lisebenza, kodwa ngemingcele.
Kubalulekile kulaba badlali abasha ukuba baqaphele imingcele yabo.
Izincomo Zokujwayelekile Zokuzivocavoca Kwabadlali Abasha NeMarfan Syndrome
Izincomo ezihlelekile ekubandakanyekeni komdlalo wokuncintisana kwabadlali abadlali abane-Marfan syndrome zanyatheliswa yiNgqungquthela ka-Bethesda ka-2005 mayelana Nezizathu Zokufaneleka Zokuncintisana Abadlali Abanokuncintisana Nezinkinga Zezinhliziyo.
Nasi isifinyeto salezo zincomo:
Abagijimi abane-Marfan syndrome kufanele babe nama- echocardiograms njalo ezinyangeni eziyisithupha ukuze bafune ukuhlenga impande ye-aorta.
Uma kungekho ukuxhuma kwe-aortic noma ukunye okungahle kwenzeke komzimba, futhi akukho mlando womndeni we-aortic dissection, bangabamba iqhaza kulokho okubizwa ngokuthi imisebenzi ephansi futhi elinganiselayo yemidlalo - leyo miisebenzi ngokuvamile ayidingi "ukuqhuma" okuphezulu kakhulu Ukuzivocavoca umzimba. Izibonelo zemisebenzi efanele kuhlanganisa igalofu, bowling, hikingl, kanye netafula tennis.
Uma kukhona ubufakazi be-aortic impande yokuxhuma, noma umlando womndeni we-aortic dissection, khona-ke umsebenzi kuphela ophansi kakhulu, njengegalufu noma isitsha se-bowling, kufanelekile.
Abantu abane-Marfan syndrome bayelulekwa ukuba bagweme imisebenzi engozini kakhulu - thintana nemidlalo nemisebenzi edinga ukuzivocavoca kwe-isometric okukhiqiza ukuhamba kwe-Valsalva (njengokuphakamisa isisindo).
Abanye abantu abane-Marfan syndrome bangasuswa ngabanye odokotela babo (uma ingozi yabo ibhekwa njengephansi kakhulu), ukuba bahlanganyele emidlalweni yemidlalo engozini efana ne-basketball, baseball, touch football, kanye nebhayisikili.
Ngokuphawulekayo, iNgqungquthela yaseBethesda iqondise ngqo abantu abahlanganyela emidlalweni ehleliwe, ekhuphisanayo.
Ikakhulukazi, ibeke iziqondiso ezikoleni nakwezinye izinhlangano lapho abagijimi abaneMarfan syndrome bebheka ukuhlanganyela kuzo izinhlelo zabo. Akuzange iqondane ngqo nomdlali wezokuzilibazisa.
Kodwa-ke, izincomo zeBethesda zisengakhipha isiqondiso sabathameli bezokuzijabulisa kanye nodokotela babo. Kunoma ubani onama-Marfan syndrome ofisa ukuhlanganyela ezemidlalo, ama-echocardiograms ngezikhathi ezithile angasetshenziswa ukuqondisa izinga elifanele lomsebenzi.
Izwi elivela
Abantu abane-Marfan syndrome banomngcipheko ohlala njalo emzimbeni we-cardiovascular, futhi badinga ukulandelwa njalo kwezokwelapha.
Imikhawulo yokuzivocavoca iyalulekwa kunoma ubani one-Marfan syndrome. Noma kunjalo, izinga lokuvinjelwa lizohlukahluka komunye nomuntu, futhi abaningi bayakwazi (futhi bakhuthazwa ukuba) bajabulele izindlela zokuphila ezikhuthele kanye nezinyathelo ezifanele zokuqapha.
> Imithombo:
> Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 I-ACCF / AHA / AATS / ACR / ASA / SCA / SCAI / SIR / STS / SVM Izikhombandlela Zokuthola Ukuxilongwa Nokuphathwa Kweziguli ezinezifo ze-Thoracic Aortic: umbiko we-American College of Cardiology Foundation / i-American Heart Association Task Force on Practice Imihlahlandlela, i-American Association ye-Thoracic Surgery, i-American College of Radiology, i-American Stroke Association, i-Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, kanye neSocial for Vascular Medicine. Ukujikeleza ngo-2010; 121: e266.
> Maron, BJ, Ackerman, MJ, Nishimura, RA, et al. Umsebenzi we-4: I-HCM nezinye izinto ze-Cardiomyopathies, i-Mitral Valve Prolapse, i-Myocarditis, ne-Marfan Syndrome. J Am Coll Cardiol 2005; 45: 1340