YiMarfan Syndrome: Ibangela, Iimpawu kunye Iindlela Treatment

YiMarfan syndrome - isifo esiyingozi, nto leyo ehambisana kunye kuguquka leqela sofuzo elithile. Abantu unesifo onjalo ubunzima ngenxa yokuphazamiseka isakhiwo kwizicwili. Ngokuba lixesha lokuqala sifo kuchazwa ngo-1876 yi-Williams, kodwa kuphela 1896, wezifo French yiMarfan A Ufundo ngakumbi iimpawu kunye unobangela wale ngxaki, wayinika igama.

syndrome yiMarfan: Oonobangela

Njengoko sele kukhankanyiwe, sisifo ilifa, nto leyo inxulunyaniswa izakhi zofuzo ikhowudi protein fibrin. Yiyo le iproteni inoxanduva isakhiwo eqhelekileyo kwithishyu nokuqina. Umntu kuguquka onjalo bazalwa iziphene. Nakuba kunjalo, iimpawu inokuchazwa ngeendlela ezahlukeneyo. Umzekelo, kukho izigulane lowo sifo emiselwe ngengozi, kuba akukho zimpawu zibonakale azizange. Kwelinye icala, kukho abantu abaphethwe kakhulu sesi sifo, kuba oko kukhatshwa sengozini ubunzima kunye nezifo.

Syndrome yiMarfan: Iimpawu

kwizicwili - i njengesakhi esibalulekileyo senkqubo yonikezelo phantse onke amalungu kunye neenkqubo emzimbeni womntu. Ngoko ke, yiMarfan syndrome ngazo ngokwayo ngeendlela ezahlukeneyo - esi sifo kuchaphazela amathambo, izihlunu, luvo kunye senhliziyo:

• Kaninzi, umntu ogulayo bungabonakala isakhiwo umzimba. Izigulane nalo syndrome idla eziphakamileyo kakhulu, engamarhoqorhoqo, kunye nemilenze omkhulu evuthelweyo. Abantu abaninzi bayahlupheka ukusuka iziphene ezifana zokufota, iinyawo tyaba, yokuphazamiseka kwisakhiwo esifubeni.
• YiMarfan syndrome Idla yi izitshanguba iso - umntu, ukutshintsha lens elinye okanye amabini ngaxeshanye. Abantu abanjalo ezithandwa ukunyanga iglaucoma agalelwa, cataract kunye lamabutho semehlo.
• Njengoko umthetho, esi sifo sikhatshwa kwaphula isakhiwo kunye nokusebenza kwenkqubo sentliziyo - ingakumbi, igcwalisa buthathaka eludongeni aortic, nto leyo eyingozi kakhulu ubomi.
• Izigulane basoloko amanqaku ukuzolula, nasemva kokuphela inkqubo yokukhula kunye nophuhliso. Ukongeza, ukuba ngokuxhomekeke hernias inguinal kunye ventral.

YiMarfan syndrome: isifo kwaye afumane unyango

Ukuza kuthi ga ngoku, akukho na ethile indlela diagnostic. Apha esiyidingayo indlela ebanzi. Okokuqala, ugqirha uqokelela imbali yezonyango epheleleyo, ize emva koko inike iimvavanyo kunye nezifundo lweenkqubo musculoskeletal kwaye yokujikeleza, kwakunye ukuhlolwa amehlo.

Ke yona indlela unyango, ngoko njengezininzi izifo yemfuza, Marfina syndrome uhlala ne umntu ngonaphakade. Kodwa ngoncedo ubuchule amayeza namhlanje kungandisa ubomi nokuqinisekisa intuthuzelo yomguli kunye lula. Isigulane kufuneka rhoqo esweni ngoogqirha. Ngokomzekelo, xa kukho iingxaki kunye neengcali mbono ukwabela izibuko okanye lenses. Ngoncedo iziyobisi asuswe naziphi na iimpawu ze kwinkqubo yokujikeleza kwegazi, yaye orthotics uncedo kuphuhliso eyiyo asemzimbeni.

Namhlanje, kulo lonke ihlabathi bethatha kuqhutywa uphando kwesi sifo. abasebenzi Scientific ukuzama ukuqonda ukuba kwenzeka ntoni xa kuguquka zofuzo kwaye ukuba kunokwenzeka ukumisa inkqubo okanye ngandlel nempembelelo ngayo. Ukongeza, oosokhemisti abasebenza kwi ekuvelisweni, amayeza amatsha olusebenzayo ngakumbi ephelisa iimpawu kunye ekupheliseni ingozi yokufa.